周期性嘔吐症 autointoxication＝「アセトン血性嘔吐症」
ストレス、風邪などが誘引となて生じる、頻回の嘔吐。
２～６歳ぐらいまでが多い。
大人にも見られる。ADH放出型？なども見られるようだ。
柳沢桂子さんもそう？認められぬ病気のこと？？？
＿＿＿＿＿＿＿＿＿＿＿＿＿＿＿
<1>２～１０才ぐらいの小児にみられる。
<2>尿中・血中ケトン体が急増したための中毒症状。
症状 突然、リンゴがすえたような臭い(アセトン体)の吐物を吐く発作が起きる
特別な原因もなく、元気がなくなり、
食欲不振・悪心・嘔吐があり、
顔面蒼白・血圧低下・全身倦怠を訴え、
重症では、
・激しい嘔吐を頻発、
・胆汁orコーヒー色の血液を吐き、
・脱水症となる。
脳波に異常を認めることもある。
原因不明 私は、３０年あまりにわたって、原因不明の苦しい病気を患っていた。毎月１回は決まって起こる激しい嘔吐発作。そのたびに１週間は起きられない。気分が不快である上に、強い腹痛も伴った。１年の半分を病院で過ごしたこともあるが、体重は減り、点滴で栄養分と水分を補わなければならなかった。
名だたる病院の消化器科内科、消化器外科に次々かかり、徹底したドクターショッピング（医師を次々変えること）をした。しかし、どこへ行っても原因も病名も分からず、気のせいか、性格に悪いところがあるためだと言われた。家族も医師の言葉を信じ、私も自分を責め続けた。その苦しさは、病の苦しさを上回るものだった。
こうした状況が３０年近く続いたある日、金沢の小児科の先生から一通のお手紙をいただいた。
先生は、私が書いた『認められぬ病』という本をお読みになって、私の病気が周期性嘔吐症候群という、脳幹の病気であると確信を持ってお手紙をくださった。先生のご専門は、周期性嘔吐症候群の中でもＡＣＴＨ・ＡＤＨ放出症候群と呼ばれる病気で、やはり激しい嘔吐を伴う症状を呈する。
先生はアメリカとカナダの周期性嘔吐症候群の患者と医師の会があるからと、そこのメールアドレスを教えてくださった。私は早速会員になった。
会が年に２回送ってくれるニューズレターには、病気についての初歩的な知識から、印刷に回している最新論文のタイトルまで載っている。また、独自に募金を募り、医師に研究費を出している。
海のすぐ向こうの国でこのようなことが行われているにもかかわらず、なぜ日本の医師はこの病気を知らなかったのだろうか？特に消化器を扱う医師が誰一人として知らなかったことは、理解しがたい。
最近になって、この病気は三環系抗うつ剤で症状を抑えることが分かった。私もその恩恵に浴している。」（生命科学者・サイエンスライター・柳澤桂子）
疲れ
が引き金
寝る直前まで、元気だったんですが、急に吐き出して。５回も吐いて、顔も真っ青です。大丈夫でしょうか」と武君のお母さんがびっくりして駆け込んできました。どうやら自家中毒です。そうお伝えしたら、お母さんは「えっ、食中毒ですか。それとも何か毒でも」とますますあわててしまいました。
さっきまで元気に飛び跳ねていた子供がこれといった理由もないのに突然、ぐったりして何度も吐くことがあります。こういった状態を一般に自家中毒、または周期性嘔吐症、アセトン血性嘔吐症などと呼びます。
原因ははっきりしませんが、疲れやストレス、興奮、風邪などが引き金となり、過敏になった自律神経が腹痛、嘔吐を引き起こすと考えられています。尿検査をすると代謝が混乱した時に出るケトン体が見つかります。
痩せ型で神経質な子供がなりやすいと言われ、体質的なものとして何度も繰り返します。けれど、小学校高学年になると治っていくので大丈夫です。
軽い場合はスポーツドリンクなどを少しずつ、何回かに分けて与え、水分を補給します。飲ませてもすぐに吐いたり、元気がないようなら病院へいきましょう。点滴を受けると急に元気になることがあります。（橋爪孝雄・市立堺病院小児科部長）
ストレス 最近では自家中毒という言葉は使われずに、周期性嘔吐症と呼ばれることが多い。症状の特徴として、
発作は夜間または寝起きに多い
発作時はぐったりして元気が無く、頻脈・顔面蒼白を伴う。
発作は感染・飢餓などの身体的ストレスや感情的動揺によって誘発される。
発作は１日～数日で治り、発作以外に症状はない。年に数回、発作を繰り返すことが多く、大部分は年長になるにつれ自然に治る。
２～６歳までに多く、体形はやせ形で、性格は情緒不安定で神経質な小児に多い・・・・・などだ。
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原因については、ホルモン異常、神経異常、糖や脂質の代謝異常などいろいろと考えられているが、ハッキリしたことは分かっていない。
治療は脱水状態にならないように、ジュースなどを少量ずつ飲ませる。飲めないようであれば点滴が必要になる。
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予防策としては、
(1)疲労後に空腹のまま寝かせつけないようにすること。
(2)ストレスを取り除くこと。
最後に、重要なことは、嘔吐を反復する別の病気が隠れている可能性があるので、嘔吐を繰り返すからと、安易に周期性嘔吐症と考えないことである。
黄連湯
五苓散
柴胡桂枝湯
大承気湯
調胃承気湯
六君子湯
2009年12月27日日曜日
2009年12月4日金曜日
早朝高血圧とは
血圧が正常な方でも、朝、目覚める前から血圧は上がってきます。この目覚めのときの血圧の上昇が急に起こり、高血圧の状態になってしまう現象を「早朝高血圧」といいます。
降圧薬などで高血圧の治療に取り組み、日中の血圧はコントロールできている方の中にも、早朝高血圧が起こっている場合があります。家庭で、朝起きてすぐに測った血圧が、収縮期血圧135mmHg／拡張期血圧85mmHg以上の場合は、早朝高血圧の疑いがあります。
早朝高血圧の危険性
高血圧が原因のひとつとなって起こると考えられている脳卒中や心筋梗塞の発作は、朝方から午前中にかけて多く発生することが分かっています。これは、早朝高血圧が起こっている時間帯と同じ時間帯です。したがって、午前中の脳卒中や心筋梗塞の発作と、早朝高血圧には関係があると考えられており、その危険性が注目されています。
早朝高血圧の治療
早朝高血圧には、レニンアンジオテンシン系（RA系）という体内の酵素・ホルモン系や交感神経系が深く関係しているといわれています。したがって、早朝高血圧が見つかった患者さんには、RA系に働く薬（アンジオテンシンⅡ受容体拮抗薬〔ARB〕、アンジオテンシン変換酵素阻害薬〔ACE阻害薬〕）や交感神経系に働く薬（α遮断薬）を使うことが勧められます。また、24時間にわたって血圧をコントロールするために、作用時間の長い降圧薬を使ったり、朝夕の2回に分けて降圧薬を飲んだりする方法も勧められています。
血圧日内変動とは
血圧は一日中同じ値を保っているわけではなく、さまざまな要因で上がったり下がったりしています。この血圧の変化を「血圧日内変動」といいます。
24時間自由行動下血圧測定（ABPM）が可能になって、この血圧日内変動に一定のリズムがあることが分かっていますが、このリズムの乱れと、合併症や臓器障害には深い関係があることが注目されています。
血圧日内変動のリズム
一般的に、血圧は睡眠中にはもっとも低く、起床前から起床後に上がります。そして、夕方から夜にかけて下がるという一定のリズムを刻んでいます。
この血圧日内変動には、自律神経の働きが大きくかかわっていると考えられています。自律神経には、身体を活動的な状態にする交感神経と、身体を休めるように働く副交感神経の2つの系統がありますが、朝から昼間にかけては交感神経が強くなるため血圧が高くなり、逆に夜や睡眠中は副交感神経が強くなるため血圧が低くなるものと考えられます。
血圧日内変動のリズム
夜間の血圧
血圧が正常な方は、通常、昼間の血圧に対して夜間の血圧が10～20％低くなるパターン（ディッパー型）を示します。しかし、なかには血圧日内変動のリズムが狂っていて、夜間に血圧が低くならない方（ノン・ディッパー型）や、夜間に血圧が極端に下がりすぎてしまう方（エクストリーム・ディッパー型）がいることが分かってきました。このうち、ノン・ディッパー型の方では脳血管障害や心肥大などの合併症や臓器障害が起こりやすいことが分かっています。またエクストリーム・ディッパー型の方でも、はっきりとした結論にはなっていないものの、症状を伴わない脳梗塞になりやすいという研究結果もあります。
このように血圧の日内変動をみると、合併症の危険性が分かることがありますので、高血圧の治療では家庭血圧の測定や24時間自由行動下血圧測定（ABPM）が勧められます。
ピークフローメータの数値の読み方・考え方
気道過敏性が高まると、日ない変動が多くなる
朝低い ヒル14時高い→20％以内であれば正常。
■ 自己のベストピークフロー値気道に病変を持たない健常者のピークフロー値が発表されています。お子さんの場合ですと、身長に応じた正常のピークフロー値も発表されています。しかし、喘息の患者は必ずしもこれらの数値にとらわれる必要はないとされています。絶えず自己のベストピークフロー値になるようにコントロールされるべきと言われています。では自己のベストピークフロー値とは、どういったものでしょうか。喘息の自覚症状はなく、朝と午後のピークフローの変動が少ない状態が２週間以上続いたときのピークフローを指します。それは先程も述べましたように、健常者のピークフロー値と必ずしも同じになるとは限りません。普段はこの自己ベストピークフロー値を目標にコントロールされます。
■ ピークフロー値の低下
これはすなわち気管支の収縮、つまり喘息発作の前兆を示しています。自覚症状が無くてもピークフローメーターは敏感に反応してきます。
■ピークフロー値の日内変動
ピークフローは一日中一定ではありません。起床時と日中の午後の１日２回の測定が勧められています。喘息発作時を除いて、起床時のピークフローが一日で最も低く、午後から（だいたい２時くらい）のピークフローが最も高いとされています。これは自律神経や外界の影響を受けて気管支の状態が絶えず変化している事を意味しています。２０％以内の変動なら健常者でも観察されます。この変動がそれ以上に大きい場合は、気管支の反応が敏感になっており、喘息発作の起こりやすい状態になっていると考えられています。
→ 参考ページ：JavaScriptによる日内変動率の計算
グローバルストラテジーより
お子さんにグローバルストラテジーを当てはめようとすると、むずかしいところがあります。参考として読んでください。
■ ＷＨＯと米国国立心・肺・血液研究所（National Heart, Lung and Blood Institute）が発表した喘息管理グローバルストラテジー（Global Strategy for Asthma Management and Prevention, GSAMP, グローバルストラテジー）では、喘息さんの自己管理にピークフローメーターの使用を勧めています。その中で患者さんへの分かりやすい説明として、ピークフローメーターの数値を以下の３段階に分けています。
- グリーンゾーン
- 安全： ピークフロー値は、予想値または個人の自己ベストの80～100%。日内変動は20%未満
- 日常生活や睡眠に影響はなく、喘息症状もほとんどない（理想的には全くない）状態です。
- イエローゾーン
- 注意： 喘息症状（夜間症状、活動減弱、咳、喘鳴、運動時または安静時の胸部圧迫感）が認められます。
- ピークフロー値は自己ベストの60～80%
- 日内変動は20～30%
- レッドゾーン
- 喘息警告： 安静時にも喘息の症状があり、日常生活に支障を来す状態。
- ピークフロー値は、予想値または自己ベストの60%未満
2009年11月10日火曜日
ステロイドについて
【表4】各ステロイドの特徴
| 同力化用量 (mg) | 糖質 コルチコイド作用 | 鉱質 コルチコイド 作用 | 蛋白結合 | 血漿 半減期(hr) | 生物学的 半減期(hr) | |
| Short Acting | ||||||
| ヒドロコルチゾン | 25 | 0.8 | 0.8 | ～ | 0.5 | 8～12 |
| Intermediate Acting | ||||||
| メチルプレドニゾロン | 4 | 5 | 0.5 | ～ | >3.5 | 18～36 |
| プレドニゾロン | 5 | 4 | 0.6 | ++ | 2.1～3.5 | 18～36 |
| トリアムシノロン | 4 | 5 | 0 | ++ | 2～5 | 18～36 |
| Long Acting | ||||||
| デキサメサゾン | 0.75 | 20～30 | 0 | ++ | 3～4.5 | 36～54 |
| ベタメサゾン | 0.6 | 20～30 | 0 | ++ | 3～5 | 36～54 |
生理的産生量 コルチゾール 5.7mg/m2/日
American Society of Health-System Pharmacists, Inc. AHFS Drug Information. Bethesda, MD, 200
American Society of Health-System Pharmacists, Inc. AHFS Drug Information. Bethesda, MD, 200
妊婦、授乳中のタミフル
添付文書 ×
ＣＤＣ ○
日本産婦人科学会 ○
妊娠している婦人もしくは授乳中の婦人に対しての
新型インフルエンザ（H1N1）感染に対する対応Q＆A (一般の方対象)
平成21年8月4日
社団法人 日本産科婦人科学会
Q1： 妊娠している人は一般の妊娠していない人に比べて新型インフルエンザに感染した場合、症状が重くなるのでしょうか？
A1： 妊婦は肺炎などを合併しやすく、重症化しやすいことが明らかとなりました。
Q2： 妊婦にインフルエンザ様症状（３８℃以上の発熱と鼻汁や鼻がつまった症状、のどの痛み、咳）が出た場合、どのようにすればよいでしょうか？
A2： かかりつけ産婦人科医を直接受診することは極力避け、地域の一般病院にあらかじめ電話をして受診します。あらかじめ受診する病院を決めておくと安心です。早期の抗インフルエンザ薬（タミフル、あるいはリレンザ）服用（治療目的）開始は重症化防止に効果ありとされていますので、発熱、のどの痛み、咳等の症状出現後はできるだけ早い受診をお勧めします。また、WHOは新型インフルエンザ感染が疑われる場合には確認検査結果を待たずにできるだけ早期のタミフル服用開始を勧めています。かかりつけ産婦人科医受診を避けるのは「感染すると重症化しやすい妊婦から妊婦への感染を防止するため」です。
Q3： 妊婦の新型インフルエンザ感染が確認された場合の対応はどうしたらいいでしょうか？
A3： 早期の抗インフルエンザ薬（タミフル、あるいはリレンザ）服用（治療目的）をお勧めします。WHOは治療目的にはタミフル服用を勧めています。
Q4： 妊娠した女性が新型インフルエンザ感染者と濃厚接触（ごく近くにいたり、閉ざされた部屋に同席した場合）した場合の対応はどうしたらいいでしょうか？
A4： 抗インフルエンザ薬（タミフル、あるいはリレンザ）服用（予防目的）をお勧めします。
Q5： 抗インフルエンザ薬（タミフル、リレンザ）はお腹の中の赤ちゃんに大きな異常を引き起こすことはないのでしょうか？
A5： 2007年の米国疾病予防局ガイドラインには「抗インフルエンザ薬を投与された妊婦および出生した赤ちゃんに有害な副作用（有害事象）の報告はない」との記載があります。また、これら薬剤服用による利益は、可能性のある薬剤副作用より大きいと考えられています。
Q6： 抗インフルエンザ薬（タミフル、リレンザ）の予防投与（インフルエンザ発症前）と治療投与（インフルエンザ発症後）で投与量や投与期間に違いがあるのでしょうか？
A6： 米国疾病予防局の推奨 （http://www.cdc.gov/h1n1flu/recommendations.htm）では以下のようになっていますので、日本でも同様な投与方法が推奨されています。
1. タミフルの場合
予防投与：75mg錠 1日1錠（計75mg)、
治療のための投与：75mg錠 1日2回（計150mg）5日間
なお、日本の2008年Drugs in Japanによれば、治療には上記量を5日間投与、予防には上記量を7日～10日間投与となっています。
2. リレンザの場合
予防投与：10mgを1日1回吸入（計10mg）、
治療のための投与：10mgを1日2回吸入（計20mg）
なお、日本の2008年Drugs in Japanによれば、治療には上記量を5日間吸入、予防には上記量を10日間吸入となっています。
Q7： 予防投与の場合、予防効果はどの程度持続するのでしょうか？
A7： タミフル、リレンザともに2008年Drugs in Japanによれば、これらを連続して服用している期間のみ予防効果ありとされています。
Q8： 予防投与した場合、健康保険は適応されるのでしょうか？
A8： 予防投与は原則として自己負担となりますが、自治体の判断で自己負担分が公費負担となる場合があります。
Q9： 抗ウィルス剤を服用しながら授乳することは可能でしょうか？
A9： 母乳自体による新型インフルエンザ感染の可能性は現在のところ知られていません。季節性インフルエンザでは母乳感染は極めてまれです。授乳期に抗ウィルス薬を使用する場合は、担当の医師と相談の上授乳を続けるかどうか決めてください。なお米国疾病予防局の推奨では抗ウィルス剤を服用しながら、赤ちゃんに授乳することは可能であるとされています。同時に赤ちゃんへの感染リスクを最小限にするため頻繁に手洗いしたりマスクをつけるなどの処置を必要とします。 母児分離を行なうべきとの勧告は今のところなされていません。
添付文書 ×
ＣＤＣ ○
日本産婦人科学会 ○
妊娠している婦人もしくは授乳中の婦人に対しての
新型インフルエンザ（H1N1）感染に対する対応Q＆A (一般の方対象)
平成21年8月4日
社団法人 日本産科婦人科学会
Q1： 妊娠している人は一般の妊娠していない人に比べて新型インフルエンザに感染した場合、症状が重くなるのでしょうか？
A1： 妊婦は肺炎などを合併しやすく、重症化しやすいことが明らかとなりました。
Q2： 妊婦にインフルエンザ様症状（３８℃以上の発熱と鼻汁や鼻がつまった症状、のどの痛み、咳）が出た場合、どのようにすればよいでしょうか？
A2： かかりつけ産婦人科医を直接受診することは極力避け、地域の一般病院にあらかじめ電話をして受診します。あらかじめ受診する病院を決めておくと安心です。早期の抗インフルエンザ薬（タミフル、あるいはリレンザ）服用（治療目的）開始は重症化防止に効果ありとされていますので、発熱、のどの痛み、咳等の症状出現後はできるだけ早い受診をお勧めします。また、WHOは新型インフルエンザ感染が疑われる場合には確認検査結果を待たずにできるだけ早期のタミフル服用開始を勧めています。かかりつけ産婦人科医受診を避けるのは「感染すると重症化しやすい妊婦から妊婦への感染を防止するため」です。
Q3： 妊婦の新型インフルエンザ感染が確認された場合の対応はどうしたらいいでしょうか？
A3： 早期の抗インフルエンザ薬（タミフル、あるいはリレンザ）服用（治療目的）をお勧めします。WHOは治療目的にはタミフル服用を勧めています。
Q4： 妊娠した女性が新型インフルエンザ感染者と濃厚接触（ごく近くにいたり、閉ざされた部屋に同席した場合）した場合の対応はどうしたらいいでしょうか？
A4： 抗インフルエンザ薬（タミフル、あるいはリレンザ）服用（予防目的）をお勧めします。
Q5： 抗インフルエンザ薬（タミフル、リレンザ）はお腹の中の赤ちゃんに大きな異常を引き起こすことはないのでしょうか？
A5： 2007年の米国疾病予防局ガイドラインには「抗インフルエンザ薬を投与された妊婦および出生した赤ちゃんに有害な副作用（有害事象）の報告はない」との記載があります。また、これら薬剤服用による利益は、可能性のある薬剤副作用より大きいと考えられています。
Q6： 抗インフルエンザ薬（タミフル、リレンザ）の予防投与（インフルエンザ発症前）と治療投与（インフルエンザ発症後）で投与量や投与期間に違いがあるのでしょうか？
A6： 米国疾病予防局の推奨 （http://www.cdc.gov/h1n1flu/recommendations.htm）では以下のようになっていますので、日本でも同様な投与方法が推奨されています。
1. タミフルの場合
予防投与：75mg錠 1日1錠（計75mg)、
治療のための投与：75mg錠 1日2回（計150mg）5日間
なお、日本の2008年Drugs in Japanによれば、治療には上記量を5日間投与、予防には上記量を7日～10日間投与となっています。
2. リレンザの場合
予防投与：10mgを1日1回吸入（計10mg）、
治療のための投与：10mgを1日2回吸入（計20mg）
なお、日本の2008年Drugs in Japanによれば、治療には上記量を5日間吸入、予防には上記量を10日間吸入となっています。
Q7： 予防投与の場合、予防効果はどの程度持続するのでしょうか？
A7： タミフル、リレンザともに2008年Drugs in Japanによれば、これらを連続して服用している期間のみ予防効果ありとされています。
Q8： 予防投与した場合、健康保険は適応されるのでしょうか？
A8： 予防投与は原則として自己負担となりますが、自治体の判断で自己負担分が公費負担となる場合があります。
Q9： 抗ウィルス剤を服用しながら授乳することは可能でしょうか？
A9： 母乳自体による新型インフルエンザ感染の可能性は現在のところ知られていません。季節性インフルエンザでは母乳感染は極めてまれです。授乳期に抗ウィルス薬を使用する場合は、担当の医師と相談の上授乳を続けるかどうか決めてください。なお米国疾病予防局の推奨では抗ウィルス剤を服用しながら、赤ちゃんに授乳することは可能であるとされています。同時に赤ちゃんへの感染リスクを最小限にするため頻繁に手洗いしたりマスクをつけるなどの処置を必要とします。 母児分離を行なうべきとの勧告は今のところなされていません。
2009年11月2日月曜日
ヨードとりすぎと 甲状腺機能低下症 ↓↓
ヨード摂取と甲状腺機能について
ねこんぶの常用によってTSHが５０－
海草類やイソジンでのうがい、
習慣をかえてもらってだいたい２ヶ月でTSHが正常化するように
なぜ、
甲状腺の専門医のかたのコメントを期待しています。
一般内科医とすれば、
ヨード摂取と甲状腺機能について
ねこんぶの常用によってTSHが５０－
海草類やイソジンでのうがい、
習慣をかえてもらってだいたい２ヶ月でTSHが正常化するように
なぜ、
甲状腺の専門医のかたのコメントを期待しています。
一般内科医とすれば、
自家中毒 周期性嘔吐症
Cyclic vomiting syndrome
Author
Emily Dulude, MD
David J Desilets, MD, PhD
Richard G Boles, MD
Section Editor
Nicholas J Talley, MD, PhD
Marc C Patterson, MD, FRACP
George D Ferry, MD
Deputy Editor
Peter A L Bonis, MD
Last literature review for version 17.2: 5月 1, 2009 | This topic last updated: 5月 26, 2009
INTRODUCTION — Cyclic vomiting syndrome (CVS) is an idiopathic disorder characterized by recurrent, stereotypical bouts of vomiting with intervening periods of normal health [1]. Although it appears to primarily affect children, it is being recognized increasingly in adults.
CVS was first described in France in 1861 [2]. The first English-language publication, in 1882, described three essential clinical features of the disorder, which still hold true today [3]:
Three or more recurrent discrete episodes of vomiting
Varying intervals of completely normal health between episodes
Episodes are stereotypical with regard to timing of onset, symptoms, and duration
A critical fourth criterion that has been added subsequently includes the absence of an organic cause of vomiting [4].
This topic review will provide an overview of cyclic vomiting syndrome in adults and children. A guideline on this topic has also been published [4].
EPIDEMIOLOGY — CVS is no longer considered to be rare in children. A cross-sectional study of school-age children in Aberdeen, Scotland, estimated that 34 of 2165 children (1.6 percent) fulfilled the diagnostic criteria for CVS [5]. Their average age was 9.6 years at the time of diagnosis, while the average age at the onset of symptoms was 5.3 years. The overall gender ratio was equal, although, among younger children, it was more common in boys. A similar age distribution has been described in other reports of children, although many have suggested that it may be more common in girls [4,6-10]. A family in which CVS appears to be inherited has been described [11].
The disorder appears to be less common in adults, although there are no population-based studies from which to derive estimates of its prevalence. One of the largest studies, which included 17 adult patients seen during a 10-year period at an academic medical center, found that the CVS began at an average age of 35 (range 14 to 73 years) but was not diagnosed until patients were on average 41 years old [12]. The gender distribution was equal. The average length of episodes was six days (range 1 to 21 days), with symptom-free intervals averaging three months (range 0.5 to 6 months). Similar findings have been described in other reports in adults [13,14].
Although there appear to be similarities in CVS across age groups [7], a few differences between adults and children have been observed [12]:
Adults have on average approximately four cycles per year in contrast with 12 cycles per year in children [15].
The age of onset covers a broad span in adults, while in children the age of onset is often in the toddler or preschool years.
Adults have usually been symptomatic for longer prior to diagnosis, possibly reflecting the increased recognition of CVS by pediatricians.
Triggering events are less common in adults.
Nausea between episodes is more common in adults.
PATHOGENESIS — The pathogenesis of CVS remains unknown, although it may represent a heterogeneous group of disorders. An association between CVS and migraine headaches has been most consistently described, suggesting that there may be a common pathophysiologic process. However, CVS has also been linked to food allergy, mitochondrial, metabolic, and endocrine disorders.
CVS and migraines — CVS has been linked to migraine headaches and abdominal migraine (show table 1) [16,17]. This connection is based upon the discreteness of episodes, the progression from cyclic vomiting to migraine headaches in many patients, the presence of a strong family history of migraine headaches in affected children (approximately 80 percent), and the response to antimigraine therapy in up to 80 percent of children [4,18]. Sympathetic autonomic dysfunction may predispose children to both CVS and migraine headaches [19,20]. (See "Pathophysiology, clinical features, and diagnosis of migraine in children").
One hypothesis is that CVS may lead to abdominal migraines, which in turn lead to migraine headaches. However, more children progress directly from CVS to migraines than from CVS to abdominal migraines to migraines. Abdominal migraines can be distinguished from CVS in that the core symptom of abdominal migraines is abdominal pain, not vomiting. Both syndromes may have headache as a feature and respond to antimigraine therapy [21].
Metabolic disorders — Mitochondrial disorders of fatty acid oxidation (eg, medium-chain acyl coenzyme A dehydrogenase deficiency), respiratory chain defects (eg, MELAS: Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Syndrome), and mitochondrial DNA deletions can be associated with episodes of metabolic crisis and vomiting, usually with infection or prolonged fasting [22]. (See "Presenting features of inborn errors of metabolism" and see "Overview of the hereditary ataxias").
One group suggested that about one-half of patients with cyclic vomiting syndrome have evidence for maternal inheritance of a mitochondrial DNA sequence variation [23]. Compared with controls, mothers of patients with cyclic vomiting syndrome were more likely to have a history of migraine, depression, irritable bowel syndrome, and hypothyroidism, disorders that the researchers hypothesized may segregate with cyclic vomiting syndrome in families due to predisposing mitochondrial DNA sequence variants. The authors speculated that cyclic vomiting syndrome represents a rare clinical presentation in individuals who carry the predisposing mitochondrial variants that are more commonly associated with migraine, depression, irritable bowel syndrome, hypothyroidism, and other functional/dysautonomic conditions [24,25].
Dysautonomia — Patients who have a hereditary sensory autonomic neuropathy (such as Riley Day Syndrome) can also have clinical features resembling CVS. (See "Hereditary sensory autonomic neuropathies").
Hypothalamic-pituitary-adrenal axis defects — Elevated corticotropin, cortisol, vasopressin, prostaglandin E2, and catecholamines have been described in a group of children with cyclic vomiting, profound lethargy, and hypertension [26]. Animal studies suggest that CVS may be a brain-gut disorder in which corticotropin-releasing factor induces gastric stasis and vomiting by vagal stimulation [27].
Food allergy — Sensitivity to cow's milk, soy, and egg white protein may be related to CVS in children [6]. Other food triggers include chocolate, cheese, and monosodium glutamate. The relationship between food allergy and CVS is uncertain.
Catamenial CVS — Similar to menstrual migraine headaches, some girls develop catamenial CVS at the onset of their menstrual period. Some respond to treatment with a low-dose estrogen or progesterone-only birth control pill, although oral contraceptives and other steroids can also precipitate vomiting episodes and other symptomatology in some. An association between symptoms and menses has also been described in adults [12].
Chronic cannabis use — Cessation of chronic cannabis use has been associated with resolution of cyclic vomiting in case reports suggesting a causal association [28-30]. In one series, the association was characterized by repetitive washing behavior during vomiting cycles [28]. However, many adults with CVS self-medicate with cannabis to alleviate the nausea.
CLINICAL MANIFESTATIONS — Each of the proposed diagnostic criteria suggests two essential features of cyclic vomiting syndrome. (See "Diagnosis" belowSee "Diagnosis" below).
Stereotypical episodes of vomiting regarding onset (acute) and duration (hours to days)
The absence of nausea and vomiting between episodes
Supportive criteria for the diagnosis of CVS include a history or family history of migraine headaches, the self-limited nature of the attacks, associated symptoms of nausea, abdominal pain, headache, motion sickness, photophobia, and lethargy, and associated signs of fever, pallor, diarrhea, dehydration, excess salivation, and social withdrawal. In children, nausea and possibly lethargy are considered to be key diagnostic features.
The specific pattern of vomiting episodes is variable among patients but, importantly, is stereotypical for an individual patient [31]. In general, CVS episodes tend to begin in the early morning hours (2:00 to 7:00 AM) and may involve a prodromal period of pallor, anorexia, nausea, abdominal pain, and/or lethargy (show table 2). In children, the attacks last an average of 24 to 48 hours. Approximately one-half of children have attacks at regular intervals, commonly occurring every two to four weeks, while the others have an unpredictable temporal pattern of vomiting. Approximately two-thirds of parents can identify a trigger, which is usually infectious (upper respiratory) or psychological (negative or positive) [32].
As noted above, episodes tend to be longer in adults (approximately three to six days) with longer intervening intervals of normal health (approximately three months) [12-14]. Intervening episodes of normal health are important for distinguishing CVS from functional nausea and vomiting in which symptoms are present continuously or nearly continuously (show figure 1).
The characteristics on presentation (and difficulty establishing the diagnosis) were illustrated in a group of 17 adults [12]:
Each patient had been evaluated by at least two physicians (typically with repeated imaging and endoscopic studies) and had been hospitalized at least once. Three patients (18 percent) required nutritional support.
Three patients had undergone surgical exploration and partial gastrectomy, pyloroplasty, or fundoplication without improvement.
Abdominal pain was present during episodes in approximately two-thirds of patients.
Only 20 percent of patients had a psychiatric diagnosis (either anxiety or another affective disorder).
Erosive esophagitis was observed in 50 percent of patients during or shortly after a vomiting episode (including one patient who had a Mallory-Weiss tear). However, in no patients did vomiting episodes improve with antireflux measures.
Only one-third of patients described a prodrome of nausea, epigastric pain, or both, while the others reported the onset of symptoms without warning.
Four of seven reproductive age women described a link to their menstrual cycle; two had severe episodes precipitated by pregnancy.
Sleep was beneficial in relieving symptoms in four patients (23 percent).
A history of migraine headaches was described in only four patients (23 percent).
NATURAL HISTORY — Many children outgrow CVS by their preteen or early teenage years. However, some authors have observed that up to 75 percent of children with CVS will go on to develop migraine headaches by age 18 [18]. A minority of children who progress from CVS to migraine headaches will first pass through a phase of abdominal migraines [33]. One retrospective study of 51 children followed for up to 13 years found that vomiting resolved in 60 percent [34]. However, 42 percent continued to have regular headaches and 37 percent had abdominal pain; these features were present even in patients whose vomiting had resolved.
The natural history in adults has not been well studied. In the series of 17 adults described above [12], 13 had at least a partial response to antidepressant therapy while an additional 3 responded to other types of treatment. One patient had persistent symptoms during two years of follow-up.
DIAGNOSIS — Cyclic vomiting syndrome remains a diagnosis based upon the history and exclusion of alternative diagnoses. At least two sets of criteria have been proposed based upon a consensus of experts.
Rome III criteria — Rome III criteria include the presence of all of the following [35]:
Stereotypical episodes of vomiting regarding onset (acute) and duration (less than one week)
Three or more discrete episodes in the prior year
Absence of nausea and vomiting between episodes
The criteria should be fulfilled for the last three months with symptom onset at least six months before diagnosis.
Supportive criteria include:
History or family history of migraine headaches.
North American Society for Pediatric Gastroenterology Hepatology and Nutrition — A consensus statement issued by the North American Society for Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN) suggests the following diagnostic criteria (all of which must be met) [4]. These recommendations apply to children and adolescents:
At least five attacks in any interval, or a minimum of three attacks during a six-month period
Episodic attacks of intense nausea and vomiting lasting 1 hour to 10 days and occurring at least one week apart
Stereotypical pattern and symptoms in the individual patient
Vomiting during attacks occurs at least four times per hour for at least one hour
Return to baseline health between episodes
Not attributed to another disorder
The recommendations for diagnosis and treatment from NASPGHAN (which apply to children and adolescents) are summarized in the algorithms (show algorithm).
Warning signs — Warning signs alerting to an alternative diagnosis include the presence of severe headaches, altered mental status, gait disturbances or other new "neurological signs", gastrointestinal bleeding, unilateral abdominal pain, weight loss, failure to respond to treatment, progressive worsening, prolonged episodes requiring hospitalization, and a change in pattern or symptoms [4]. An upper gastrointestinal series should be performed in children to exclude intestinal malrotation. (See "Intestinal malrotation"). Ureteropelvic junction obstruction has been reported to be an overlooked mimic of CVS in a case series of four children [36]. CVS should be considered among other diagnoses in patients presenting with nausea and vomiting. (See "Approach to the adult patient with nausea and vomiting" and see "Approach to the infant or child with nausea and vomiting").
One approach to excluding other disorders of recurrent vomiting includes a complete, extensive evaluation of standard blood work (electrolytes, glucose, ALT, GGTP, amylase, lipase), urinalysis, and (in children) evaluation for metabolic disorders (eg, lactate, ammonia, amino acids, urine organic acids) during an acute episode. (See "Approach to the infant or child with nausea and vomiting"). An upper gastrointestinal series with small bowel follow-through X-ray (UGI/SBFT), CT/MRI of the head, and endoscopy can be performed between episodes. As noted above, the presence of esophagitis on endoscopy (performed during or near an episode) does not necessarily imply that reflux is an underlying cause, since as many as one-half of patients will have esophagitis but will not respond to antireflux measures.
Only one in eight children with cyclical episodes of vomiting who were evaluated with this approach has been found to have another underlying disorder that required intervention [4]. However, the cost of initial diagnostic testing and annual ambulatory and hospital treatment of CVS using the above approach was estimated to be $17,035 per child in one report [21]. A decision-analysis concluded that a more cost-effective diagnostic strategy in children was to obtain a UGI/SBFT to rule out malrotation with volvulus, followed by a two-month trial of antimigraine therapy, with further studies reserved for those with continued symptoms [37]. In adults, a CT scan of the abdomen and pelvis has been suggested to exclude malignancy and other structural disorders.
TREATMENT — Patients may require supportive care during severe bouts of cyclic vomiting, which may include admission to a hospital, intravenous fluids, antiemetics, and occasionally analgesics. Children should be referred to a pediatric gastroenterologist, neurologist, or metabolic specialist. Recognized precipitating factors (more commonly observed in children) should be avoided whenever feasible. Examples include physical exhaustion, motion (car rides, amusement park rides), fasting, and certain foods (eg, chocolate, cheese).
No specific therapy has been proven to be effective for CVS in controlled trials. However, several empiric treatments have been effective in case series. Treatment with medications should be guided by three considerations [22]:
Whether there is a family history of migraines
Frequency of episodes
Severity of episodes
Treatment can also be considered as abortive, prophylactic, and supportive.
A trial of antimigraine medications is reasonable in patients with a family history of migraine headaches. Some authors recommend antimigraine therapy even in the absence of a personal or family history of migraines if, after careful evaluation, the diagnosis of CVS seems certain.
The decision to administer abortive and/or prophylactic antimigraine medications depends upon the frequency and severity of the attacks, similar to the principles that guide treatment of patients with migraine headaches. Prophylactic daily therapy with antimigraine medications is warranted if attacks occur more than once every one to two months or are severe enough to require hospitalization or substantial disability. In contrast, abortive therapy can be used if episodes occur less than once every one to two months or are mild. The choice of specific agents is discussed separately. (See "Management of migraine headache in children" and see "Acute treatment of migraine in adults" and see "Preventive treatment of migraine in adults").
Agents that have been used empirically (with variable success) in children include sumatriptan, erythromycin, carnitine, propranolol, cyproheptadine, and tricyclic antidepressants [38-40]. Some authorities advocate use of amitriptyline for prophylaxis in children older than five even if there is no history of headache or a family history of migraine [25]. A common starting dose is 0.5 mg/kg per day at bedtime, although many patients require a higher dose, often 1 mg/kg per day at bedtime. It typically takes one to three months for the effects of amitriptyline to become evident.
Some CVS patients who do not respond to 1 mg/kg per day have very low or undetectable blood levels of amitriptyline, and respond when the amitriptyline dosage is further increased. As a general rule, a blood amitryptyline level should be obtained before attempting to exceed 1 mg/kg/ per day to avoid a toxic level.
Use of amitriptyline may be limited in infants and toddlers under 5 years of age due to side effects (such as personality changes, anticholinergic effects, and tachyarrhythmias). The EKG (particularly the QTc interval) and electrolytes (K+, Mag+) should be monitored. Cyproheptadine and propranolol are often used as alternatives. Cyproheptadine is recommended as first-line treatment in children under age 5 years. Therapies of unproven efficacy that have been used include topiramate and co-enzyme Q10.
Sumatriptan, ketorolac, prochlorperazine, and tricyclic antidepressants have been used in case reports in adults [13,39,41,42]. Low-dose estrogen or progesterone-only birth control pills can be used as directed in females with catamenial CVS in whom vomiting attacks occur at the time of menses; however, birth control pills can also exacerbate symptoms in CVS patients [4,43]. (See "Estrogen-associated migraine"). A variety of antiemetic medications have been used unsuccessfully, including high dose dexamethasone, metoclopramide, ondansetron, and naloxone [42].
A clinical response to tricyclic antidepressants was observed in 13 of 17 adults in the report described above (although only three patients had a complete response) [12]. Antidepressants used included amitriptyline, doxepin, and nortriptyline (median dose for each is 50 mg daily). Two other patients responded to fluoxetine or cyproheptadine. Another report described improvement with zonisamide or levetiracetam (antiepileptic drugs) in adults who had symptoms refractory to tricyclic antidepressants [44]. However, side-effects (such as fatigue, confusion, headache, and poor concentration) were common.
During vomiting episodes, the goal is to abort or shorten the episode. Anecdotal experience in children suggests that intravenous administration of a 10 percent dextrose solution can decrease the frequency and duration of vomiting episodes [25] in about one-half of patients. Between episodes, frequent low fat feedings have been advocated to decrease the frequency of episodes. These observations may have a pathophysiologic basis in the impaired carbohydrate and fat metabolism that has been described in children with mitochondrial variants that may predispose to cyclic vomiting syndrome. (See "Metabolic disorders" above).
In addition to 10 percent intravenous dextrose, anecdotal experience suggests that high dose ondansetron (0.3 to 0.4 mg/kg/dose, maximum about 20 mg/dose), sedation (eg, with diphenhydramine or lorazepam), and a quiet, dark room are often helpful. Drinking water to induce vomiting (and thus lessening nausea) is common, and does not indicate a factitious or psychiatric etiology [45].
SUMMARY AND RECOMMENDATIONS
Definition —
Cyclic vomiting syndrome (CVS) is an idiopathic disorder characterized by recurrent, stereotypical bouts of vomiting with intervening periods of normal health. Although it appears to affect primarily children, it is being recognized increasingly in adults. (See "Introduction" above).
Pathogenesis —
The pathogenesis of CVS remains unknown, although it may represent a heterogeneous group of disorders. An association between CVS and migraine headaches has been most consistently described, suggesting that there may be a common pathophysiologic process. However, CVS has also been linked to food allergy, mitochondrial, metabolic, and endocrine disorders. (See "Pathogenesis" aboveSee "Pathogenesis" above).
Diagnosis — Diagnostic criteria have been proposed based upon consensus of experts. The specific pattern of vomiting is variable among patients but, importantly, is stereotypical for an individual patient. Cyclic vomiting syndrome remains a diagnosis based upon the history and exclusion of alternative diagnoses. (See "Diagnosis" aboveSee "Diagnosis" above).
Treatment — Treatment can be considered as abortive, prophylactic, and supportive. Most treatments have been based upon observational data or clinical experience, forming the basis for the following suggested approach (Grade 2C).
In adults, we generally start antimigraine therapy, especially if there is a family history of migraine headaches. If episodes occur more often than monthly, or if they are prolonged or debilitating, we start daily prophylactic therapy. If episodes are less frequent or are mild, we will usually try abortive therapy at the onset of episodes. If abortive therapy fails after two or three attempts, we switch to prophylactic therapy. (See "Management of migraine headache in children" and see "Acute treatment of migraine in adults" and see "Preventive treatment of migraine in adults").
In children, some authorities advocate use of amitriptyline (0.5 mg/kg per day increased as needed to 1 mg/kg or higher per day at bedtime) for prophylaxis even if there is no history of headache or a family history of migraine. However, its use may be limited due to side effects in infants and toddlers under age 5 years. The EKG (particularly the QTc interval) should be monitored. Cyproheptadine and propranolol are often used as alternatives. (See "Management of migraine headache in children"). In children under age 5 years, cyproheptadine is the first choice (or pizotifen outside of the United States).
Topiramate and coenzyme Q10 are unproven therapies that are gaining in popularity due to anecdotal experiences of efficacy. Abortive therapy with intravenous dextrose (10 percent) and high-dose ondansetron have been found to be helpful in anecdotal reports in children. Comfort measures during episodes include a dark quiet room, and sedation as appropriate. In addition, patients should be instructed to avoid fasting.
Support — Support and counseling may be helpful for selected patients. The Cyclic Vomiting Syndrome Association (CVSA), a national and international organization, was established in 1993 to provide phone support, support groups, regional conferences, a newsletter, and a web site.
For more information on the CVSA, contact:
Cyclic Vomiting Syndrome Association (CVSA-USA/Canada)
2819 West Highland Blvd.
Milwaukee, WI 53208
Phone: 414-342-7880
Fax: 414-342-8980
E-mail: cvsa@cvsaonline.org
Website: www.cvsaonline.org
Additional resources that might be useful are listed in the table (show table 3).
Guidelines — The above recommendations are similar to those issued by the North American Society of Pediatric Gastroenterology Hepatology and Nutrition, which apply to children and adolescents (show algorithm).
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REFERENCES
Li, BU. Cyclic vomiting syndrome: Light emerging from the black box. J Pediatr 1999; 135:276.
Lombard, HC. Evrose de la digestion, caracteriseo par des crises periodiques de vomissements et une profonde modification de l'assimilation. Gazette Medicale de Paris 1861:312.
Gee, S. On fitful or recurrent vomiting. St Bartholomew Hospital Reports 1882; 18:1.
Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379.
Abu-Arafeh, I, Russell, G. Cyclic vomiting syndrome in children: A population-based study. J Pediatr Gastroenterol Nutr 1995; 21:454.
Lucarelli, S, Corrado, G, Pelliccia, A, et al. Cyclic vomiting syndrome and food allergy/intolerance in seven children: A possible association. Eur J Pediatr 2000; 159:360.
Prakash, C, Staiano, A, Rothbaum, RJ, Clouse, RE. Similarities in cyclic vomiting syndrome across age groups. Am J Gastroenterol 2001; 96:684.
Withers, GD, Silburn, SR, Forbes, DA. Precipitants and aetiology of cyclic vomiting syndrome. Acta Paediatr 1998; 87:272.
Hoyt, C, Stickler, G. A study of 44 children with the syndrome of recurrent (cyclic) vomiting. Pediatrics 1960; 25:775.
Lee, WS, Kaur, P, Boey, CC, Chan, KC. Cyclic vomiting syndrome in South-East Asian children. J Paediatr Child Health 1998; 34:568.
Haan, J, Kors, EE, Ferrari, MD. Familial cyclic vomiting syndrome. Cephalalgia 2002; 22:552.
Prakash, C, Clouse, RE. Cyclic vomiting syndrome in adults: Clinical features and response to tricyclic antidepressants. Am J Gastroenterol 1999; 94:2855.
Scobie, BA. Recurrent vomiting in adults. A syndrome?. Med J Aust 1983; 1:329.
Abell, TL, Kim, CH, Malagelada, JR. Idiopathic cyclic nausea and vomiting--a disorder of gastrointestinal motility?. Mayo Clin Proc 1988; 63:1169.
Fleisher, DR. Matar, M. The cyclic vomiting syndrome: a report of 71 cases and literature review. J Pediatr Gastroenterol Nutr 1993; 17:361.
Welch, KM. Scientific basis of migraine: Speculation on the relationship to cyclic vomiting. Dig Dis Sci 1999; 44:26S.
Jernigan, SA, Ware, LM. Reversible quantitative EEG changes in a case of cyclic vomiting: evidence for migraine equivalent. Dev Med Child Neurol 1991; 33:80.
Li, BU, Murray, RD, Heitlinger, LA, et al. Is cyclic vomiting related to migraines? J Pediatr 1999; 134:567.
To, J, Issenman, RM, Kamath, MV. Evaluation of neurocardiac signals in pediatric patients with cyclic vomiting syndrome through power spectral analysis of heart rate variability. J Pediatr 1999; 135:363.
Chelimsky, TC, Chelimsky, GG. Autonomic abnormalities in cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2007; 44:326.
Li, BU, Balint, J. Cyclic vomiting syndrome: The evolution of understanding a brain-gut disorder. Adv Pediatr 2000; 47:117.
Boles, RG, Chun, N, Seadheera, D, Wong, LJ. Cyclic vomiting syndrome and mitochondrial DNA mutations. Lancet 1997; 350:1299.
Boles, RG, Adams, K, Li, BU. Maternal inheritance in cyclic vomiting syndrome. Am J Med Genet A 2005; 133:71.
Wang, Q, Ito, M, Adams, K, et al. Mitochondrial DNA control region sequence variation in migraine headache and cyclic vomiting syndrome. Am J Med Genet A 2004; 131:50.
Boles, RG, Adams, K, Ito, M, Li, BU. Maternal inheritance in cyclic vomiting syndrome with neuromuscular disease. Am J Med Genet A 2003; 120:474.
Sato, T, Igarashi, N, Minami, S, et al. Recurrent attacks of vomiting, hypertension and psychotic depression: a syndrome of periodic catecholamine and prostaglandin discharge. Acta Endocrinol (Copenh) 1988; 117:189.
Tache, Y. Cyclic vomiting syndrome: The corticotropin-releasing-factor hypothesis. Dig Dis Sci 1999; 44:79S.
Allen, JH, de Moore, GM, Heddle, R, Twartz, JC. Cannabinoid hyperemesis: cyclical hyperemesis in association with chronic cannabis abuse. Gut 2004; 53:1566.
Roche, E, Foster, PN. Cannabinoid hyperemesis: not just a problem in Adelaide Hills. Gut 2005; 54:731.
Sontineni, SP, Chaudhary, S, Sontineni, V, Lanspa, SJ. Cannabinoid hyperemesis syndrome: clinical diagnosis of an underrecognised manifestation of chronic cannabis abuse. World J Gastroenterol 2009; 15:1264.
Fleisher, DR, Gornowicz, B, Adams, K, et al. Cyclic Vomiting Syndrome in 41 adults: the illness, the patients, and problems of management. BMC Med 2005; 3:20.
Li, BU, Issenman, RM, Sarna, SK. Consensus statement--2nd International Scientific Symposium on CVS. The Faculty of The 2nd International Scientific Symposium on Cyclic Vomiting Syndrome. Dig Dis Sci 1999; 44:9S.
Symon, DN, Russell, G. Abdominal migraine: A childhood syndrome defined. Cephalalgia 1986; 6:223.
Fitzpatrick, E, Bourke, B, Drumm, B, Rowland, M. Outcome for children with cyclical vomiting syndrome. Arch Dis Child 2007; 92:1001.
Tack, J, Talley, NJ, Camilleri, M, et al. Functional gastroduodenal disorders. Gastroenterology 2006; 130:1466.
Schulte-Bockholt, A, Kugathasan, S, Mesrobian, HG, Werlin, SL. Ureteropelvic junction obstruction: An overlooked cause of cyclic vomiting. Am J Gastroenterol 2002; 97:1043.
Olson, AD, Li, BU. The diagnostic evaluation of children with cyclic vomiting: A cost-effectiveness assessment. J Pediatr 2002; 141:724.
Vanderhoof, JA, Young, R, Kaufman, SS, Ernst, L. Treatment of cyclic vomiting in childhood with erythromycin. J Pediatr Gastroenterol Nutr 1993; 17:387.
Andersen, JM, Sugerman, KS, Lockhart, JR, Weinberg, WA. Effective prophylactic therapy for cyclic vomiting syndrome in children using amitriptyline or cyproheptadine. Pediatrics 1997; 100:977.
Van Calcar, SC, Harding, CO, Wolff, JA. L-carnitine administration reduces number of episodes in cyclic vomiting syndrome. Clin Pediatr (Phila) 2002; 41:171.
Benson, JM, Zorn, SL, Book, LS. Sumatriptan in the treatment of cyclic vomiting. Ann Pharmacother 1995; 29:997.
Pasricha, PJ, Schuster, MM, Saudek, CD, et al. Cyclic vomiting: Association with multiple homeostatic abnormalities and response to ketorolac. Am J Gastroenterol 1996; 91:2228.
Boyle, CA. Management of menstrual migraine. Neurology 1999; 53:S14.
Clouse, RE, Sayuk, GS, Lustman, PJ, Prakash, C. Zonisamide or levetiracetam for adults with cyclic vomiting syndrome: a case series. Clin Gastroenterol Hepatol 2007; 5:44.
Pareek, N, Fleisher, DR, Abell, T. Cyclic vomiting syndrome: what a gastroenterologist needs to know. Am J Gastroenterol 2007; 102:2832.
GRAPHICS
Profile of symptoms in cyclic vomiting syndrome, abdominal migraine, and migraine headaches
Percentage of patients with symptom
CVS Abdominal migraine Migraine headache
Core symptoms
Vomiting 100 39-72 40-69
Abdominal pain 3-81 100 10-55
Headache 38-59 31-50 100
Associated symptoms
Lethargy 91
Pallor 87 90-100 23-88
Anorexia 74 91-98 13-93
Nausea 72 73-91 46-100
Photophobia 32 1-42 27-81
Adapted from: Li, BU, Balint, JP. Cyclic vomiting syndrome: Evolution in understanding of a brain-gut disorder. Adv Pediatr 2000; 47:117.
Clinical/epidemiological features of cyclic vomiting syndrome
Feature Characterization
Female:male ratio 55:45
Age of onset 5.3 years
Morbidity 20 d of missed school/year, 50 percent of patients require intravenous hydration
Symptoms:
Vomiting 6 times/hr at peak, with bile (76 percent) and blood (32 percent)
Autonomic Lethargy (91 percent), pallor (87 percent), fever (29 percent), salivation (13 percent)
GI Abdominal pain (80 percent), retching (78 percent), anorexia (74 percent), nausea (72 percent), diarrhea (36 percent)
Neurologic Headache (40 percent), photophobia (32 percent), phonophobia (28 percent), vertigo (22 percent)
Temporal pattern 24-48 h duration; 47 percent of patients have episodes at regular intervals, usually 2-4 weeks; episodes occur at night or early morning in 34-60 percent of patients; 98 percent of patients show a stereotypical pattern
Precipitating events Infection (41 percent), psychological stress (34 percent), dietary (26 percent), menstrual (13 percent), some trigger identified in 68 percent of patients
Natural history 3.4 yr duration; 28 percent of patients progress to migraine headaches; predicted 75 percent progress to migraines by age 18 yrs
Family history of migraine Present in 82 percent of patients
Adapted from: Li, BU, Balint, JP. Cyclic vomiting syndrome: Evolution in understanding of a brain-gut disorder. Adv Pediatr 2000; 47:117 and Li, BU. Cyclic vomiting: New understanding of an old disorder. Contemp Pediatr 1996; 17:48.
Differences between cyclic and chronic temporal patterns of vomiting
Differences between cyclic and chronic temporal patterns of vomiting can be compared when the number of emeses is plotted over a two-month period. The chronic pattern (red line) is characterized by low-grade, nearly daily episodes whereas the cyclic pattern (black line) is marked by high-intensity episodes every several weeks. Adapted from Li, BU. New hope for children with cyclic vomiting syndrome. Contemp Pediatr 2002; 19:121.
NASPGHN evaluation CVS
Evaluation of cyclic vomiting pattern in children older than 2 years
* May not need metabolic evaluation.
Reproduced with permission from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Lifestyle changes in CVS
Lifestyle changes in CVS
Lifestyle changes (for 1-2 months or 1-2 cycles)
Reassurance (eg, episodes are not self-induced) and anticipatory guidance (eg, natural history)
Avoidance of triggers
Keep a "vomiting diary" of potential precipitating factors
Avoid fasting
Recognize the potential role of excitement as a trigger (eg, downplay big events)
Maintain good sleep hygiene (eg, avoid sleep deprivation)
Avoid triggering foods: chocolate, cheese, monosodium glutamate, antigenic foods
Avoid excessive energy output
Supplemental carbohydrate: for fasting-induced episodes
Provide fruit juices, other sugar-containing drinks
Provide extra snacks between meals, before exertion, or at bedtime
Migraine headache lifestyle interventions
Regular aerobic exercise (avoid overexercising)
Regular meal schedules (ie, avoid skipping meals)
Moderation in consuming or avoidance of caffeine
Reproduced with permission from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Prophylactic medication CVS
Prophylactic or preventive medications* in CVS
Children 2 to 5 years old
Antihistamines: cyproheptadine (first choice) and pizotifen (available in UK, Canada)
Cyproheptadine 0.25-0.5 mg/kg/day orally divided twice daily or three times daily (maximum 12 mg per 24 hours). (Available in 2 mg/5 mL liquid).
Side effects: increased appetite, weight gain, sedation
Alternatives: pizotifen (available in UK, Canada)
Beta-blockers: propranolol (second choice)
Propranolol 0.25-1 mg/kg/day orally divided twice daily or three times daily, most often 10 mg twice daily or three times daily. Initiate at 0.25 mg/kg/day and increase every 1-4 weeks in increments of 0.25 mg/kg/day as needed and tolerated. (<35 kg maximum 60 mg per 24 hours. 35 kg maximum 120 mg per 24 hours). (Available in liquid). Monitor: resting heart rate maintain 60 bpm Side effects: lethargy, reduced exercise intolerance Contraindications: asthma, diabetes, heart disease, depression Discontinuation: tapered for 1-2 weeks Children older than 5 years Tricyclic antidepressants: amitriptyline (first choice) Amitriptyline begin at 0.25-0.5 mg/kg/day orally at bedtime, increase every 1-4 weeks as needed and tolerated by 5-10 mg, until 1-1.5 mg/kg/day (maximum 75 mg per 24 hours). (For dose >1 mg/kg/day, divide twice daily).
Monitor: EKG QTc interval before starting and 10 days after peak dose
Side effects: constipation, sedation, arrhythmia, behavioral changes (especially in young children)
Alternatives: nortriptyline (available in 10 mg/5 mL liquid)
Beta-blockers: propranolol (second choice) - see above
Other agents
Anticonvulsants: phenobarbital
Phenobarbital 2 mg/kg/day orally administered at bedtime (maximum 40 mg per 24 hours)
Side effects: sedation, cognitive impairment
Alternatives: topiramate, valproic acid, gabapentin, levetiracetam - consult Pediatric neurologist
Supplements
L-carnitine 50-100 mg/kg/day orally divided twice daily or three times daily (maximum 1 g three times daily)
Coenzyme Q10 10 mg/kg/day orally divided twice daily or three times daily (maximum 100 mg three times daily)
Side effects: diarrhea, fishy body odor (for L-carnitine)
* All medication recommendations are made for off-label use.
Limited or no published experience regarding efficacy.
Adapted from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Supportive abortive Rx CVS
Supportive and abortive treatment approaches in CVS
Supportive care
Fluid, electrolyte and nutritional management
Infuse 10% dextrose, 0.45% (half normal) saline solution and potassium chloride as appropriate at 1.5 times maintenance fluid rates OR through a Y-connector 10% dextrose infusion at one times maintenance and normal saline at 0.5 times maintenance
If no enteral intake for 3-5 days, initiate peripheral parenteral nutrition with 1.5 g of amino acids/kg/day and energy units above the catabolic threshold of 55-70 kcal/kg/day
Antiemetic (5HT3 antagonist) agents
Ondansetron 0.3-0.4 mg/kg/dose intravenously every 4-6 hours (maximum 20 mg per dose)
Side effects: constipation
Alternatives: granisetron
Sedatives
Diphenhydramine 1-1.25 mg/kg/dose intravenously every 6 hours (maximum 50 mg per dose)
Lorazepam 0.05-0.1 mg/kg/dose intravenously every 6 hours (maximum 2 mg per dose)
Side effects: respiratory depression, hallucinations
Chlorpromazine 0.5-1 mg/kg/dose intravenously every 6 hours + diphenhydramine (see above) intravenously:
<5 years or <23 kg maximum 10 mg per dose, 40 mg per 24 hours chlorpromazine >5 years or 23 kg maximum 18 mg per dose, 75 mg per 24 hours chlorpromazine
Side effects: dystonic reactions with chlorpromazine alone
Analgesics (nonsteroidal and narcotic) agents
Ketorolac 0.4-1 mg/kg intravenously every 6 hours x 48 hours (maximum 30 mg per dose, maximum 120 mg per 24 hours); avoid in renal insufficiency or dehydration
Side effects: gastrointestinal hemorrhage
Alternatives: narcotics, intravenous morphine or fentanyl by bolus or by patient-control infusion
Treatment of specific signs and symptoms: epigastric pain, diarrhea, and hypertension
Epigastric pain: acid suppression by H2RAs or PPIs, (eg, intravenous ranitidine, pantoprazole)
Diarrhea: antidiarrheals (eg, loperamide)
Hypertension: short-acting ACE inhibitors (eg, captopril)
Treatment of specific complications
Dehydration and electrolyte deficit: replace calculated deficits
Metabolic acidosis: determine cause and treat accordingly
SIADH: restrict free water intake
Hematemesis: intravenous H2RAs or PPIs
Weight loss: nasogastric or parenteral nutrition
Abortive care
Antimigraine (triptan) agents
Sumatriptan: 20 mg once intranasally as early as possible at episode onset for children 12 years and older (maximum 40 mg / 24 hours)
Side effects: neck pain/burning, coronary vasospasm
Contraindications: basilar artery migraine
Recovery and refeeding
Feed ad libitum when child declares episode is over
H2RAs: histamine-2 receptor antagonists. PPIs: proton pump inhibitors; ACE: angiotensive converting enzyme; SIADH: syndrome of inappropriate antidiuretic hormone.
Adapted from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Sample treatment protocol order sheet for a child having an acute attack of CVS
In Emergency Department and in-hospital settings, an example of a regimen would include:
Darkened, quiet room, take vital signs every 4-6 hours
If child is dehydrated, rehydrate with initial intravenous fluid bolus of 10 mL/kg normal 0.9% saline and repeat as clinically necessary
Infuse 10% dextrose 0.45% (half normal) saline solution with potassium chloride as appropriate at 1.5 times maintenance rates
Intravenous ondansetron 0.3 mg/kg/dose every 6 hours x 24 hours (maximum 20 mg per dose)
Intravenous lorazepam 0.05 mg/kg/dose every 6 hours x 24 hours (maximum 2 mg per dose)
If child has moderate to severe abdominal pain, intravenous ketorolac 0.5 to 1 mg/kg/dose (maximum 30 mg per dose) every 6 hours x 24 hours (avoid in renal insufficiency or prior to hydration if dehydrated)
Admit child if >5 percent dehydrated, no urine output >12 hours, Na+ <130 mEq/L, anion gap >18 mEq/L, or inability to stop emesis
Allow oral fluid intake
Adapted from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Grade 2C recommendation
A Grade 2C recommendation is a very weak recommendation; other alternatives may be equally reasonable.
Explanation:
A Grade 2 recommendation is a weak recommendation. It means "this is our suggestion, but you may want to think about it." It is unlikely that you should follow the suggested approach in all your patients, and you might reasonably choose an alternative approach. For Grade 2 recommendations, benefits and risks may be finely balanced, or the benefits and risks may be uncertain. In deciding whether to follow a Grade 2 recommendation in an individual patient, you may want to think about your patient's values and preferences or about your patient's risk aversion.
Grade C means the evidence comes from observational studies, unsystematic clinical experience, or from randomized, controlled trials with serious flaws. Any estimate of effect is uncertain.
Recommendation grades
1. Strong recommendation: Benefits clearly outweigh the risks and burdens (or vice versa) for most, if not all, patients
2. Weak recommendation: Benefits and risks closely balanced and/or uncertain
Evidence grades
A. High-quality evidence: Consistent evidence from randomized trials, or overwhelming evidence of some other form
B. Moderate-quality evidence: Evidence from randomized trials with important limitations, or very strong evidence of some other form
C. Low-quality evidence: Evidence from observational studies, unsystematic clinical observations, or from randomized trials with serious flaws
For a complete description of our grading system, please see the UpToDate editorial policy which can be found by clicking "About UpToDate" and then selecting "Policies".
Family support and informational resources
Cyclic Vomiting Syndrome Association (CVSA)
2819 W Highland Blvd, Milwaukee, WI 53208
Phone: 414-342-7880
Fax: 414-342-8980
cvsa@cvsaonline.org, www.cvsaonline.org
Gastroparesis and Dysmotilities Association (GDPA)
5520 Dalhart Hill NW, Calgary, AB, Canada T3A 1S9
Phone: 403-247-3215
www.gpda.net
International Foundation for Functional Gastrointestinal Disorders (IFFGD)
PO Box 170864, Milwaukee, WI 53217
Phone: 414-964-1799
Children: http://www.aboutkidsgi.org
Adults: http://www.iffgd.org
Migraine Awareness Group (MAGNUM)
113 S Saint Asaph, Suite 300, Alexandria, VA 22314
Phone: 703-739-9384
www.migraines.org
National Organization for Rare Disorders
55 Kenosia Ave, PO Box 1968, Danbury, CT 06813-1968
Phone: 203-744-0100, 800-999-6673
TDD number: 203-797-9590
Fax: 203-798-2291
www.nord-rdb.com
United Mitochondrial Disease Foundation
PO Box 1151, Monroeville, PA 15146-1151
Phone: 412-793-8077
Reproduced with permission from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
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Cyclic vomiting syndrome
Author
Emily Dulude, MD
David J Desilets, MD, PhD
Richard G Boles, MD
Section Editor
Nicholas J Talley, MD, PhD
Marc C Patterson, MD, FRACP
George D Ferry, MD
Deputy Editor
Peter A L Bonis, MD
Last literature review for version 17.2: 5月 1, 2009 | This topic last updated: 5月 26, 2009
INTRODUCTION — Cyclic vomiting syndrome (CVS) is an idiopathic disorder characterized by recurrent, stereotypical bouts of vomiting with intervening periods of normal health [1]. Although it appears to primarily affect children, it is being recognized increasingly in adults.
CVS was first described in France in 1861 [2]. The first English-language publication, in 1882, described three essential clinical features of the disorder, which still hold true today [3]:
Three or more recurrent discrete episodes of vomiting
Varying intervals of completely normal health between episodes
Episodes are stereotypical with regard to timing of onset, symptoms, and duration
A critical fourth criterion that has been added subsequently includes the absence of an organic cause of vomiting [4].
This topic review will provide an overview of cyclic vomiting syndrome in adults and children. A guideline on this topic has also been published [4].
EPIDEMIOLOGY — CVS is no longer considered to be rare in children. A cross-sectional study of school-age children in Aberdeen, Scotland, estimated that 34 of 2165 children (1.6 percent) fulfilled the diagnostic criteria for CVS [5]. Their average age was 9.6 years at the time of diagnosis, while the average age at the onset of symptoms was 5.3 years. The overall gender ratio was equal, although, among younger children, it was more common in boys. A similar age distribution has been described in other reports of children, although many have suggested that it may be more common in girls [4,6-10]. A family in which CVS appears to be inherited has been described [11].
The disorder appears to be less common in adults, although there are no population-based studies from which to derive estimates of its prevalence. One of the largest studies, which included 17 adult patients seen during a 10-year period at an academic medical center, found that the CVS began at an average age of 35 (range 14 to 73 years) but was not diagnosed until patients were on average 41 years old [12]. The gender distribution was equal. The average length of episodes was six days (range 1 to 21 days), with symptom-free intervals averaging three months (range 0.5 to 6 months). Similar findings have been described in other reports in adults [13,14].
Although there appear to be similarities in CVS across age groups [7], a few differences between adults and children have been observed [12]:
Adults have on average approximately four cycles per year in contrast with 12 cycles per year in children [15].
The age of onset covers a broad span in adults, while in children the age of onset is often in the toddler or preschool years.
Adults have usually been symptomatic for longer prior to diagnosis, possibly reflecting the increased recognition of CVS by pediatricians.
Triggering events are less common in adults.
Nausea between episodes is more common in adults.
PATHOGENESIS — The pathogenesis of CVS remains unknown, although it may represent a heterogeneous group of disorders. An association between CVS and migraine headaches has been most consistently described, suggesting that there may be a common pathophysiologic process. However, CVS has also been linked to food allergy, mitochondrial, metabolic, and endocrine disorders.
CVS and migraines — CVS has been linked to migraine headaches and abdominal migraine (show table 1) [16,17]. This connection is based upon the discreteness of episodes, the progression from cyclic vomiting to migraine headaches in many patients, the presence of a strong family history of migraine headaches in affected children (approximately 80 percent), and the response to antimigraine therapy in up to 80 percent of children [4,18]. Sympathetic autonomic dysfunction may predispose children to both CVS and migraine headaches [19,20]. (See "Pathophysiology, clinical features, and diagnosis of migraine in children").
One hypothesis is that CVS may lead to abdominal migraines, which in turn lead to migraine headaches. However, more children progress directly from CVS to migraines than from CVS to abdominal migraines to migraines. Abdominal migraines can be distinguished from CVS in that the core symptom of abdominal migraines is abdominal pain, not vomiting. Both syndromes may have headache as a feature and respond to antimigraine therapy [21].
Metabolic disorders — Mitochondrial disorders of fatty acid oxidation (eg, medium-chain acyl coenzyme A dehydrogenase deficiency), respiratory chain defects (eg, MELAS: Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Syndrome), and mitochondrial DNA deletions can be associated with episodes of metabolic crisis and vomiting, usually with infection or prolonged fasting [22]. (See "Presenting features of inborn errors of metabolism" and see "Overview of the hereditary ataxias").
One group suggested that about one-half of patients with cyclic vomiting syndrome have evidence for maternal inheritance of a mitochondrial DNA sequence variation [23]. Compared with controls, mothers of patients with cyclic vomiting syndrome were more likely to have a history of migraine, depression, irritable bowel syndrome, and hypothyroidism, disorders that the researchers hypothesized may segregate with cyclic vomiting syndrome in families due to predisposing mitochondrial DNA sequence variants. The authors speculated that cyclic vomiting syndrome represents a rare clinical presentation in individuals who carry the predisposing mitochondrial variants that are more commonly associated with migraine, depression, irritable bowel syndrome, hypothyroidism, and other functional/dysautonomic conditions [24,25].
Dysautonomia — Patients who have a hereditary sensory autonomic neuropathy (such as Riley Day Syndrome) can also have clinical features resembling CVS. (See "Hereditary sensory autonomic neuropathies").
Hypothalamic-pituitary-adrenal axis defects — Elevated corticotropin, cortisol, vasopressin, prostaglandin E2, and catecholamines have been described in a group of children with cyclic vomiting, profound lethargy, and hypertension [26]. Animal studies suggest that CVS may be a brain-gut disorder in which corticotropin-releasing factor induces gastric stasis and vomiting by vagal stimulation [27].
Food allergy — Sensitivity to cow's milk, soy, and egg white protein may be related to CVS in children [6]. Other food triggers include chocolate, cheese, and monosodium glutamate. The relationship between food allergy and CVS is uncertain.
Catamenial CVS — Similar to menstrual migraine headaches, some girls develop catamenial CVS at the onset of their menstrual period. Some respond to treatment with a low-dose estrogen or progesterone-only birth control pill, although oral contraceptives and other steroids can also precipitate vomiting episodes and other symptomatology in some. An association between symptoms and menses has also been described in adults [12].
Chronic cannabis use — Cessation of chronic cannabis use has been associated with resolution of cyclic vomiting in case reports suggesting a causal association [28-30]. In one series, the association was characterized by repetitive washing behavior during vomiting cycles [28]. However, many adults with CVS self-medicate with cannabis to alleviate the nausea.
CLINICAL MANIFESTATIONS — Each of the proposed diagnostic criteria suggests two essential features of cyclic vomiting syndrome. (See "Diagnosis" belowSee "Diagnosis" below).
Stereotypical episodes of vomiting regarding onset (acute) and duration (hours to days)
The absence of nausea and vomiting between episodes
Supportive criteria for the diagnosis of CVS include a history or family history of migraine headaches, the self-limited nature of the attacks, associated symptoms of nausea, abdominal pain, headache, motion sickness, photophobia, and lethargy, and associated signs of fever, pallor, diarrhea, dehydration, excess salivation, and social withdrawal. In children, nausea and possibly lethargy are considered to be key diagnostic features.
The specific pattern of vomiting episodes is variable among patients but, importantly, is stereotypical for an individual patient [31]. In general, CVS episodes tend to begin in the early morning hours (2:00 to 7:00 AM) and may involve a prodromal period of pallor, anorexia, nausea, abdominal pain, and/or lethargy (show table 2). In children, the attacks last an average of 24 to 48 hours. Approximately one-half of children have attacks at regular intervals, commonly occurring every two to four weeks, while the others have an unpredictable temporal pattern of vomiting. Approximately two-thirds of parents can identify a trigger, which is usually infectious (upper respiratory) or psychological (negative or positive) [32].
As noted above, episodes tend to be longer in adults (approximately three to six days) with longer intervening intervals of normal health (approximately three months) [12-14]. Intervening episodes of normal health are important for distinguishing CVS from functional nausea and vomiting in which symptoms are present continuously or nearly continuously (show figure 1).
The characteristics on presentation (and difficulty establishing the diagnosis) were illustrated in a group of 17 adults [12]:
Each patient had been evaluated by at least two physicians (typically with repeated imaging and endoscopic studies) and had been hospitalized at least once. Three patients (18 percent) required nutritional support.
Three patients had undergone surgical exploration and partial gastrectomy, pyloroplasty, or fundoplication without improvement.
Abdominal pain was present during episodes in approximately two-thirds of patients.
Only 20 percent of patients had a psychiatric diagnosis (either anxiety or another affective disorder).
Erosive esophagitis was observed in 50 percent of patients during or shortly after a vomiting episode (including one patient who had a Mallory-Weiss tear). However, in no patients did vomiting episodes improve with antireflux measures.
Only one-third of patients described a prodrome of nausea, epigastric pain, or both, while the others reported the onset of symptoms without warning.
Four of seven reproductive age women described a link to their menstrual cycle; two had severe episodes precipitated by pregnancy.
Sleep was beneficial in relieving symptoms in four patients (23 percent).
A history of migraine headaches was described in only four patients (23 percent).
NATURAL HISTORY — Many children outgrow CVS by their preteen or early teenage years. However, some authors have observed that up to 75 percent of children with CVS will go on to develop migraine headaches by age 18 [18]. A minority of children who progress from CVS to migraine headaches will first pass through a phase of abdominal migraines [33]. One retrospective study of 51 children followed for up to 13 years found that vomiting resolved in 60 percent [34]. However, 42 percent continued to have regular headaches and 37 percent had abdominal pain; these features were present even in patients whose vomiting had resolved.
The natural history in adults has not been well studied. In the series of 17 adults described above [12], 13 had at least a partial response to antidepressant therapy while an additional 3 responded to other types of treatment. One patient had persistent symptoms during two years of follow-up.
DIAGNOSIS — Cyclic vomiting syndrome remains a diagnosis based upon the history and exclusion of alternative diagnoses. At least two sets of criteria have been proposed based upon a consensus of experts.
Rome III criteria — Rome III criteria include the presence of all of the following [35]:
Stereotypical episodes of vomiting regarding onset (acute) and duration (less than one week)
Three or more discrete episodes in the prior year
Absence of nausea and vomiting between episodes
The criteria should be fulfilled for the last three months with symptom onset at least six months before diagnosis.
Supportive criteria include:
History or family history of migraine headaches.
North American Society for Pediatric Gastroenterology Hepatology and Nutrition — A consensus statement issued by the North American Society for Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN) suggests the following diagnostic criteria (all of which must be met) [4]. These recommendations apply to children and adolescents:
At least five attacks in any interval, or a minimum of three attacks during a six-month period
Episodic attacks of intense nausea and vomiting lasting 1 hour to 10 days and occurring at least one week apart
Stereotypical pattern and symptoms in the individual patient
Vomiting during attacks occurs at least four times per hour for at least one hour
Return to baseline health between episodes
Not attributed to another disorder
The recommendations for diagnosis and treatment from NASPGHAN (which apply to children and adolescents) are summarized in the algorithms (show algorithm).
Warning signs — Warning signs alerting to an alternative diagnosis include the presence of severe headaches, altered mental status, gait disturbances or other new "neurological signs", gastrointestinal bleeding, unilateral abdominal pain, weight loss, failure to respond to treatment, progressive worsening, prolonged episodes requiring hospitalization, and a change in pattern or symptoms [4]. An upper gastrointestinal series should be performed in children to exclude intestinal malrotation. (See "Intestinal malrotation"). Ureteropelvic junction obstruction has been reported to be an overlooked mimic of CVS in a case series of four children [36]. CVS should be considered among other diagnoses in patients presenting with nausea and vomiting. (See "Approach to the adult patient with nausea and vomiting" and see "Approach to the infant or child with nausea and vomiting").
One approach to excluding other disorders of recurrent vomiting includes a complete, extensive evaluation of standard blood work (electrolytes, glucose, ALT, GGTP, amylase, lipase), urinalysis, and (in children) evaluation for metabolic disorders (eg, lactate, ammonia, amino acids, urine organic acids) during an acute episode. (See "Approach to the infant or child with nausea and vomiting"). An upper gastrointestinal series with small bowel follow-through X-ray (UGI/SBFT), CT/MRI of the head, and endoscopy can be performed between episodes. As noted above, the presence of esophagitis on endoscopy (performed during or near an episode) does not necessarily imply that reflux is an underlying cause, since as many as one-half of patients will have esophagitis but will not respond to antireflux measures.
Only one in eight children with cyclical episodes of vomiting who were evaluated with this approach has been found to have another underlying disorder that required intervention [4]. However, the cost of initial diagnostic testing and annual ambulatory and hospital treatment of CVS using the above approach was estimated to be $17,035 per child in one report [21]. A decision-analysis concluded that a more cost-effective diagnostic strategy in children was to obtain a UGI/SBFT to rule out malrotation with volvulus, followed by a two-month trial of antimigraine therapy, with further studies reserved for those with continued symptoms [37]. In adults, a CT scan of the abdomen and pelvis has been suggested to exclude malignancy and other structural disorders.
TREATMENT — Patients may require supportive care during severe bouts of cyclic vomiting, which may include admission to a hospital, intravenous fluids, antiemetics, and occasionally analgesics. Children should be referred to a pediatric gastroenterologist, neurologist, or metabolic specialist. Recognized precipitating factors (more commonly observed in children) should be avoided whenever feasible. Examples include physical exhaustion, motion (car rides, amusement park rides), fasting, and certain foods (eg, chocolate, cheese).
No specific therapy has been proven to be effective for CVS in controlled trials. However, several empiric treatments have been effective in case series. Treatment with medications should be guided by three considerations [22]:
Whether there is a family history of migraines
Frequency of episodes
Severity of episodes
Treatment can also be considered as abortive, prophylactic, and supportive.
A trial of antimigraine medications is reasonable in patients with a family history of migraine headaches. Some authors recommend antimigraine therapy even in the absence of a personal or family history of migraines if, after careful evaluation, the diagnosis of CVS seems certain.
The decision to administer abortive and/or prophylactic antimigraine medications depends upon the frequency and severity of the attacks, similar to the principles that guide treatment of patients with migraine headaches. Prophylactic daily therapy with antimigraine medications is warranted if attacks occur more than once every one to two months or are severe enough to require hospitalization or substantial disability. In contrast, abortive therapy can be used if episodes occur less than once every one to two months or are mild. The choice of specific agents is discussed separately. (See "Management of migraine headache in children" and see "Acute treatment of migraine in adults" and see "Preventive treatment of migraine in adults").
Agents that have been used empirically (with variable success) in children include sumatriptan, erythromycin, carnitine, propranolol, cyproheptadine, and tricyclic antidepressants [38-40]. Some authorities advocate use of amitriptyline for prophylaxis in children older than five even if there is no history of headache or a family history of migraine [25]. A common starting dose is 0.5 mg/kg per day at bedtime, although many patients require a higher dose, often 1 mg/kg per day at bedtime. It typically takes one to three months for the effects of amitriptyline to become evident.
Some CVS patients who do not respond to 1 mg/kg per day have very low or undetectable blood levels of amitriptyline, and respond when the amitriptyline dosage is further increased. As a general rule, a blood amitryptyline level should be obtained before attempting to exceed 1 mg/kg/ per day to avoid a toxic level.
Use of amitriptyline may be limited in infants and toddlers under 5 years of age due to side effects (such as personality changes, anticholinergic effects, and tachyarrhythmias). The EKG (particularly the QTc interval) and electrolytes (K+, Mag+) should be monitored. Cyproheptadine and propranolol are often used as alternatives. Cyproheptadine is recommended as first-line treatment in children under age 5 years. Therapies of unproven efficacy that have been used include topiramate and co-enzyme Q10.
Sumatriptan, ketorolac, prochlorperazine, and tricyclic antidepressants have been used in case reports in adults [13,39,41,42]. Low-dose estrogen or progesterone-only birth control pills can be used as directed in females with catamenial CVS in whom vomiting attacks occur at the time of menses; however, birth control pills can also exacerbate symptoms in CVS patients [4,43]. (See "Estrogen-associated migraine"). A variety of antiemetic medications have been used unsuccessfully, including high dose dexamethasone, metoclopramide, ondansetron, and naloxone [42].
A clinical response to tricyclic antidepressants was observed in 13 of 17 adults in the report described above (although only three patients had a complete response) [12]. Antidepressants used included amitriptyline, doxepin, and nortriptyline (median dose for each is 50 mg daily). Two other patients responded to fluoxetine or cyproheptadine. Another report described improvement with zonisamide or levetiracetam (antiepileptic drugs) in adults who had symptoms refractory to tricyclic antidepressants [44]. However, side-effects (such as fatigue, confusion, headache, and poor concentration) were common.
During vomiting episodes, the goal is to abort or shorten the episode. Anecdotal experience in children suggests that intravenous administration of a 10 percent dextrose solution can decrease the frequency and duration of vomiting episodes [25] in about one-half of patients. Between episodes, frequent low fat feedings have been advocated to decrease the frequency of episodes. These observations may have a pathophysiologic basis in the impaired carbohydrate and fat metabolism that has been described in children with mitochondrial variants that may predispose to cyclic vomiting syndrome. (See "Metabolic disorders" above).
In addition to 10 percent intravenous dextrose, anecdotal experience suggests that high dose ondansetron (0.3 to 0.4 mg/kg/dose, maximum about 20 mg/dose), sedation (eg, with diphenhydramine or lorazepam), and a quiet, dark room are often helpful. Drinking water to induce vomiting (and thus lessening nausea) is common, and does not indicate a factitious or psychiatric etiology [45].
SUMMARY AND RECOMMENDATIONS
Definition —
Cyclic vomiting syndrome (CVS) is an idiopathic disorder characterized by recurrent, stereotypical bouts of vomiting with intervening periods of normal health. Although it appears to affect primarily children, it is being recognized increasingly in adults. (See "Introduction" above).
Pathogenesis —
The pathogenesis of CVS remains unknown, although it may represent a heterogeneous group of disorders. An association between CVS and migraine headaches has been most consistently described, suggesting that there may be a common pathophysiologic process. However, CVS has also been linked to food allergy, mitochondrial, metabolic, and endocrine disorders. (See "Pathogenesis" aboveSee "Pathogenesis" above).
Diagnosis — Diagnostic criteria have been proposed based upon consensus of experts. The specific pattern of vomiting is variable among patients but, importantly, is stereotypical for an individual patient. Cyclic vomiting syndrome remains a diagnosis based upon the history and exclusion of alternative diagnoses. (See "Diagnosis" aboveSee "Diagnosis" above).
Treatment — Treatment can be considered as abortive, prophylactic, and supportive. Most treatments have been based upon observational data or clinical experience, forming the basis for the following suggested approach (Grade 2C).
In adults, we generally start antimigraine therapy, especially if there is a family history of migraine headaches. If episodes occur more often than monthly, or if they are prolonged or debilitating, we start daily prophylactic therapy. If episodes are less frequent or are mild, we will usually try abortive therapy at the onset of episodes. If abortive therapy fails after two or three attempts, we switch to prophylactic therapy. (See "Management of migraine headache in children" and see "Acute treatment of migraine in adults" and see "Preventive treatment of migraine in adults").
In children, some authorities advocate use of amitriptyline (0.5 mg/kg per day increased as needed to 1 mg/kg or higher per day at bedtime) for prophylaxis even if there is no history of headache or a family history of migraine. However, its use may be limited due to side effects in infants and toddlers under age 5 years. The EKG (particularly the QTc interval) should be monitored. Cyproheptadine and propranolol are often used as alternatives. (See "Management of migraine headache in children"). In children under age 5 years, cyproheptadine is the first choice (or pizotifen outside of the United States).
Topiramate and coenzyme Q10 are unproven therapies that are gaining in popularity due to anecdotal experiences of efficacy. Abortive therapy with intravenous dextrose (10 percent) and high-dose ondansetron have been found to be helpful in anecdotal reports in children. Comfort measures during episodes include a dark quiet room, and sedation as appropriate. In addition, patients should be instructed to avoid fasting.
Support — Support and counseling may be helpful for selected patients. The Cyclic Vomiting Syndrome Association (CVSA), a national and international organization, was established in 1993 to provide phone support, support groups, regional conferences, a newsletter, and a web site.
For more information on the CVSA, contact:
Cyclic Vomiting Syndrome Association (CVSA-USA/Canada)
2819 West Highland Blvd.
Milwaukee, WI 53208
Phone: 414-342-7880
Fax: 414-342-8980
E-mail: cvsa@cvsaonline.org
Website: www.cvsaonline.org
Additional resources that might be useful are listed in the table (show table 3).
Guidelines — The above recommendations are similar to those issued by the North American Society of Pediatric Gastroenterology Hepatology and Nutrition, which apply to children and adolescents (show algorithm).
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REFERENCES
Li, BU. Cyclic vomiting syndrome: Light emerging from the black box. J Pediatr 1999; 135:276.
Lombard, HC. Evrose de la digestion, caracteriseo par des crises periodiques de vomissements et une profonde modification de l'assimilation. Gazette Medicale de Paris 1861:312.
Gee, S. On fitful or recurrent vomiting. St Bartholomew Hospital Reports 1882; 18:1.
Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379.
Abu-Arafeh, I, Russell, G. Cyclic vomiting syndrome in children: A population-based study. J Pediatr Gastroenterol Nutr 1995; 21:454.
Lucarelli, S, Corrado, G, Pelliccia, A, et al. Cyclic vomiting syndrome and food allergy/intolerance in seven children: A possible association. Eur J Pediatr 2000; 159:360.
Prakash, C, Staiano, A, Rothbaum, RJ, Clouse, RE. Similarities in cyclic vomiting syndrome across age groups. Am J Gastroenterol 2001; 96:684.
Withers, GD, Silburn, SR, Forbes, DA. Precipitants and aetiology of cyclic vomiting syndrome. Acta Paediatr 1998; 87:272.
Hoyt, C, Stickler, G. A study of 44 children with the syndrome of recurrent (cyclic) vomiting. Pediatrics 1960; 25:775.
Lee, WS, Kaur, P, Boey, CC, Chan, KC. Cyclic vomiting syndrome in South-East Asian children. J Paediatr Child Health 1998; 34:568.
Haan, J, Kors, EE, Ferrari, MD. Familial cyclic vomiting syndrome. Cephalalgia 2002; 22:552.
Prakash, C, Clouse, RE. Cyclic vomiting syndrome in adults: Clinical features and response to tricyclic antidepressants. Am J Gastroenterol 1999; 94:2855.
Scobie, BA. Recurrent vomiting in adults. A syndrome?. Med J Aust 1983; 1:329.
Abell, TL, Kim, CH, Malagelada, JR. Idiopathic cyclic nausea and vomiting--a disorder of gastrointestinal motility?. Mayo Clin Proc 1988; 63:1169.
Fleisher, DR. Matar, M. The cyclic vomiting syndrome: a report of 71 cases and literature review. J Pediatr Gastroenterol Nutr 1993; 17:361.
Welch, KM. Scientific basis of migraine: Speculation on the relationship to cyclic vomiting. Dig Dis Sci 1999; 44:26S.
Jernigan, SA, Ware, LM. Reversible quantitative EEG changes in a case of cyclic vomiting: evidence for migraine equivalent. Dev Med Child Neurol 1991; 33:80.
Li, BU, Murray, RD, Heitlinger, LA, et al. Is cyclic vomiting related to migraines? J Pediatr 1999; 134:567.
To, J, Issenman, RM, Kamath, MV. Evaluation of neurocardiac signals in pediatric patients with cyclic vomiting syndrome through power spectral analysis of heart rate variability. J Pediatr 1999; 135:363.
Chelimsky, TC, Chelimsky, GG. Autonomic abnormalities in cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2007; 44:326.
Li, BU, Balint, J. Cyclic vomiting syndrome: The evolution of understanding a brain-gut disorder. Adv Pediatr 2000; 47:117.
Boles, RG, Chun, N, Seadheera, D, Wong, LJ. Cyclic vomiting syndrome and mitochondrial DNA mutations. Lancet 1997; 350:1299.
Boles, RG, Adams, K, Li, BU. Maternal inheritance in cyclic vomiting syndrome. Am J Med Genet A 2005; 133:71.
Wang, Q, Ito, M, Adams, K, et al. Mitochondrial DNA control region sequence variation in migraine headache and cyclic vomiting syndrome. Am J Med Genet A 2004; 131:50.
Boles, RG, Adams, K, Ito, M, Li, BU. Maternal inheritance in cyclic vomiting syndrome with neuromuscular disease. Am J Med Genet A 2003; 120:474.
Sato, T, Igarashi, N, Minami, S, et al. Recurrent attacks of vomiting, hypertension and psychotic depression: a syndrome of periodic catecholamine and prostaglandin discharge. Acta Endocrinol (Copenh) 1988; 117:189.
Tache, Y. Cyclic vomiting syndrome: The corticotropin-releasing-factor hypothesis. Dig Dis Sci 1999; 44:79S.
Allen, JH, de Moore, GM, Heddle, R, Twartz, JC. Cannabinoid hyperemesis: cyclical hyperemesis in association with chronic cannabis abuse. Gut 2004; 53:1566.
Roche, E, Foster, PN. Cannabinoid hyperemesis: not just a problem in Adelaide Hills. Gut 2005; 54:731.
Sontineni, SP, Chaudhary, S, Sontineni, V, Lanspa, SJ. Cannabinoid hyperemesis syndrome: clinical diagnosis of an underrecognised manifestation of chronic cannabis abuse. World J Gastroenterol 2009; 15:1264.
Fleisher, DR, Gornowicz, B, Adams, K, et al. Cyclic Vomiting Syndrome in 41 adults: the illness, the patients, and problems of management. BMC Med 2005; 3:20.
Li, BU, Issenman, RM, Sarna, SK. Consensus statement--2nd International Scientific Symposium on CVS. The Faculty of The 2nd International Scientific Symposium on Cyclic Vomiting Syndrome. Dig Dis Sci 1999; 44:9S.
Symon, DN, Russell, G. Abdominal migraine: A childhood syndrome defined. Cephalalgia 1986; 6:223.
Fitzpatrick, E, Bourke, B, Drumm, B, Rowland, M. Outcome for children with cyclical vomiting syndrome. Arch Dis Child 2007; 92:1001.
Tack, J, Talley, NJ, Camilleri, M, et al. Functional gastroduodenal disorders. Gastroenterology 2006; 130:1466.
Schulte-Bockholt, A, Kugathasan, S, Mesrobian, HG, Werlin, SL. Ureteropelvic junction obstruction: An overlooked cause of cyclic vomiting. Am J Gastroenterol 2002; 97:1043.
Olson, AD, Li, BU. The diagnostic evaluation of children with cyclic vomiting: A cost-effectiveness assessment. J Pediatr 2002; 141:724.
Vanderhoof, JA, Young, R, Kaufman, SS, Ernst, L. Treatment of cyclic vomiting in childhood with erythromycin. J Pediatr Gastroenterol Nutr 1993; 17:387.
Andersen, JM, Sugerman, KS, Lockhart, JR, Weinberg, WA. Effective prophylactic therapy for cyclic vomiting syndrome in children using amitriptyline or cyproheptadine. Pediatrics 1997; 100:977.
Van Calcar, SC, Harding, CO, Wolff, JA. L-carnitine administration reduces number of episodes in cyclic vomiting syndrome. Clin Pediatr (Phila) 2002; 41:171.
Benson, JM, Zorn, SL, Book, LS. Sumatriptan in the treatment of cyclic vomiting. Ann Pharmacother 1995; 29:997.
Pasricha, PJ, Schuster, MM, Saudek, CD, et al. Cyclic vomiting: Association with multiple homeostatic abnormalities and response to ketorolac. Am J Gastroenterol 1996; 91:2228.
Boyle, CA. Management of menstrual migraine. Neurology 1999; 53:S14.
Clouse, RE, Sayuk, GS, Lustman, PJ, Prakash, C. Zonisamide or levetiracetam for adults with cyclic vomiting syndrome: a case series. Clin Gastroenterol Hepatol 2007; 5:44.
Pareek, N, Fleisher, DR, Abell, T. Cyclic vomiting syndrome: what a gastroenterologist needs to know. Am J Gastroenterol 2007; 102:2832.
GRAPHICS
Profile of symptoms in cyclic vomiting syndrome, abdominal migraine, and migraine headaches
Percentage of patients with symptom
CVS Abdominal migraine Migraine headache
Core symptoms
Vomiting 100 39-72 40-69
Abdominal pain 3-81 100 10-55
Headache 38-59 31-50 100
Associated symptoms
Lethargy 91
Pallor 87 90-100 23-88
Anorexia 74 91-98 13-93
Nausea 72 73-91 46-100
Photophobia 32 1-42 27-81
Adapted from: Li, BU, Balint, JP. Cyclic vomiting syndrome: Evolution in understanding of a brain-gut disorder. Adv Pediatr 2000; 47:117.
Clinical/epidemiological features of cyclic vomiting syndrome
Feature Characterization
Female:male ratio 55:45
Age of onset 5.3 years
Morbidity 20 d of missed school/year, 50 percent of patients require intravenous hydration
Symptoms:
Vomiting 6 times/hr at peak, with bile (76 percent) and blood (32 percent)
Autonomic Lethargy (91 percent), pallor (87 percent), fever (29 percent), salivation (13 percent)
GI Abdominal pain (80 percent), retching (78 percent), anorexia (74 percent), nausea (72 percent), diarrhea (36 percent)
Neurologic Headache (40 percent), photophobia (32 percent), phonophobia (28 percent), vertigo (22 percent)
Temporal pattern 24-48 h duration; 47 percent of patients have episodes at regular intervals, usually 2-4 weeks; episodes occur at night or early morning in 34-60 percent of patients; 98 percent of patients show a stereotypical pattern
Precipitating events Infection (41 percent), psychological stress (34 percent), dietary (26 percent), menstrual (13 percent), some trigger identified in 68 percent of patients
Natural history 3.4 yr duration; 28 percent of patients progress to migraine headaches; predicted 75 percent progress to migraines by age 18 yrs
Family history of migraine Present in 82 percent of patients
Adapted from: Li, BU, Balint, JP. Cyclic vomiting syndrome: Evolution in understanding of a brain-gut disorder. Adv Pediatr 2000; 47:117 and Li, BU. Cyclic vomiting: New understanding of an old disorder. Contemp Pediatr 1996; 17:48.
Differences between cyclic and chronic temporal patterns of vomiting
Differences between cyclic and chronic temporal patterns of vomiting can be compared when the number of emeses is plotted over a two-month period. The chronic pattern (red line) is characterized by low-grade, nearly daily episodes whereas the cyclic pattern (black line) is marked by high-intensity episodes every several weeks. Adapted from Li, BU. New hope for children with cyclic vomiting syndrome. Contemp Pediatr 2002; 19:121.
NASPGHN evaluation CVS
Evaluation of cyclic vomiting pattern in children older than 2 years
* May not need metabolic evaluation.
Reproduced with permission from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Lifestyle changes in CVS
Lifestyle changes in CVS
Lifestyle changes (for 1-2 months or 1-2 cycles)
Reassurance (eg, episodes are not self-induced) and anticipatory guidance (eg, natural history)
Avoidance of triggers
Keep a "vomiting diary" of potential precipitating factors
Avoid fasting
Recognize the potential role of excitement as a trigger (eg, downplay big events)
Maintain good sleep hygiene (eg, avoid sleep deprivation)
Avoid triggering foods: chocolate, cheese, monosodium glutamate, antigenic foods
Avoid excessive energy output
Supplemental carbohydrate: for fasting-induced episodes
Provide fruit juices, other sugar-containing drinks
Provide extra snacks between meals, before exertion, or at bedtime
Migraine headache lifestyle interventions
Regular aerobic exercise (avoid overexercising)
Regular meal schedules (ie, avoid skipping meals)
Moderation in consuming or avoidance of caffeine
Reproduced with permission from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Prophylactic medication CVS
Prophylactic or preventive medications* in CVS
Children 2 to 5 years old
Antihistamines: cyproheptadine (first choice) and pizotifen (available in UK, Canada)
Cyproheptadine 0.25-0.5 mg/kg/day orally divided twice daily or three times daily (maximum 12 mg per 24 hours). (Available in 2 mg/5 mL liquid).
Side effects: increased appetite, weight gain, sedation
Alternatives: pizotifen (available in UK, Canada)
Beta-blockers: propranolol (second choice)
Propranolol 0.25-1 mg/kg/day orally divided twice daily or three times daily, most often 10 mg twice daily or three times daily. Initiate at 0.25 mg/kg/day and increase every 1-4 weeks in increments of 0.25 mg/kg/day as needed and tolerated. (<35 kg maximum 60 mg per 24 hours. 35 kg maximum 120 mg per 24 hours). (Available in liquid). Monitor: resting heart rate maintain 60 bpm Side effects: lethargy, reduced exercise intolerance Contraindications: asthma, diabetes, heart disease, depression Discontinuation: tapered for 1-2 weeks Children older than 5 years Tricyclic antidepressants: amitriptyline (first choice) Amitriptyline begin at 0.25-0.5 mg/kg/day orally at bedtime, increase every 1-4 weeks as needed and tolerated by 5-10 mg, until 1-1.5 mg/kg/day (maximum 75 mg per 24 hours). (For dose >1 mg/kg/day, divide twice daily).
Monitor: EKG QTc interval before starting and 10 days after peak dose
Side effects: constipation, sedation, arrhythmia, behavioral changes (especially in young children)
Alternatives: nortriptyline (available in 10 mg/5 mL liquid)
Beta-blockers: propranolol (second choice) - see above
Other agents
Anticonvulsants: phenobarbital
Phenobarbital 2 mg/kg/day orally administered at bedtime (maximum 40 mg per 24 hours)
Side effects: sedation, cognitive impairment
Alternatives: topiramate, valproic acid, gabapentin, levetiracetam - consult Pediatric neurologist
Supplements
L-carnitine 50-100 mg/kg/day orally divided twice daily or three times daily (maximum 1 g three times daily)
Coenzyme Q10 10 mg/kg/day orally divided twice daily or three times daily (maximum 100 mg three times daily)
Side effects: diarrhea, fishy body odor (for L-carnitine)
* All medication recommendations are made for off-label use.
Limited or no published experience regarding efficacy.
Adapted from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Supportive abortive Rx CVS
Supportive and abortive treatment approaches in CVS
Supportive care
Fluid, electrolyte and nutritional management
Infuse 10% dextrose, 0.45% (half normal) saline solution and potassium chloride as appropriate at 1.5 times maintenance fluid rates OR through a Y-connector 10% dextrose infusion at one times maintenance and normal saline at 0.5 times maintenance
If no enteral intake for 3-5 days, initiate peripheral parenteral nutrition with 1.5 g of amino acids/kg/day and energy units above the catabolic threshold of 55-70 kcal/kg/day
Antiemetic (5HT3 antagonist) agents
Ondansetron 0.3-0.4 mg/kg/dose intravenously every 4-6 hours (maximum 20 mg per dose)
Side effects: constipation
Alternatives: granisetron
Sedatives
Diphenhydramine 1-1.25 mg/kg/dose intravenously every 6 hours (maximum 50 mg per dose)
Lorazepam 0.05-0.1 mg/kg/dose intravenously every 6 hours (maximum 2 mg per dose)
Side effects: respiratory depression, hallucinations
Chlorpromazine 0.5-1 mg/kg/dose intravenously every 6 hours + diphenhydramine (see above) intravenously:
<5 years or <23 kg maximum 10 mg per dose, 40 mg per 24 hours chlorpromazine >5 years or 23 kg maximum 18 mg per dose, 75 mg per 24 hours chlorpromazine
Side effects: dystonic reactions with chlorpromazine alone
Analgesics (nonsteroidal and narcotic) agents
Ketorolac 0.4-1 mg/kg intravenously every 6 hours x 48 hours (maximum 30 mg per dose, maximum 120 mg per 24 hours); avoid in renal insufficiency or dehydration
Side effects: gastrointestinal hemorrhage
Alternatives: narcotics, intravenous morphine or fentanyl by bolus or by patient-control infusion
Treatment of specific signs and symptoms: epigastric pain, diarrhea, and hypertension
Epigastric pain: acid suppression by H2RAs or PPIs, (eg, intravenous ranitidine, pantoprazole)
Diarrhea: antidiarrheals (eg, loperamide)
Hypertension: short-acting ACE inhibitors (eg, captopril)
Treatment of specific complications
Dehydration and electrolyte deficit: replace calculated deficits
Metabolic acidosis: determine cause and treat accordingly
SIADH: restrict free water intake
Hematemesis: intravenous H2RAs or PPIs
Weight loss: nasogastric or parenteral nutrition
Abortive care
Antimigraine (triptan) agents
Sumatriptan: 20 mg once intranasally as early as possible at episode onset for children 12 years and older (maximum 40 mg / 24 hours)
Side effects: neck pain/burning, coronary vasospasm
Contraindications: basilar artery migraine
Recovery and refeeding
Feed ad libitum when child declares episode is over
H2RAs: histamine-2 receptor antagonists. PPIs: proton pump inhibitors; ACE: angiotensive converting enzyme; SIADH: syndrome of inappropriate antidiuretic hormone.
Adapted from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Sample treatment protocol order sheet for a child having an acute attack of CVS
In Emergency Department and in-hospital settings, an example of a regimen would include:
Darkened, quiet room, take vital signs every 4-6 hours
If child is dehydrated, rehydrate with initial intravenous fluid bolus of 10 mL/kg normal 0.9% saline and repeat as clinically necessary
Infuse 10% dextrose 0.45% (half normal) saline solution with potassium chloride as appropriate at 1.5 times maintenance rates
Intravenous ondansetron 0.3 mg/kg/dose every 6 hours x 24 hours (maximum 20 mg per dose)
Intravenous lorazepam 0.05 mg/kg/dose every 6 hours x 24 hours (maximum 2 mg per dose)
If child has moderate to severe abdominal pain, intravenous ketorolac 0.5 to 1 mg/kg/dose (maximum 30 mg per dose) every 6 hours x 24 hours (avoid in renal insufficiency or prior to hydration if dehydrated)
Admit child if >5 percent dehydrated, no urine output >12 hours, Na+ <130 mEq/L, anion gap >18 mEq/L, or inability to stop emesis
Allow oral fluid intake
Adapted from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
Grade 2C recommendation
A Grade 2C recommendation is a very weak recommendation; other alternatives may be equally reasonable.
Explanation:
A Grade 2 recommendation is a weak recommendation. It means "this is our suggestion, but you may want to think about it." It is unlikely that you should follow the suggested approach in all your patients, and you might reasonably choose an alternative approach. For Grade 2 recommendations, benefits and risks may be finely balanced, or the benefits and risks may be uncertain. In deciding whether to follow a Grade 2 recommendation in an individual patient, you may want to think about your patient's values and preferences or about your patient's risk aversion.
Grade C means the evidence comes from observational studies, unsystematic clinical experience, or from randomized, controlled trials with serious flaws. Any estimate of effect is uncertain.
Recommendation grades
1. Strong recommendation: Benefits clearly outweigh the risks and burdens (or vice versa) for most, if not all, patients
2. Weak recommendation: Benefits and risks closely balanced and/or uncertain
Evidence grades
A. High-quality evidence: Consistent evidence from randomized trials, or overwhelming evidence of some other form
B. Moderate-quality evidence: Evidence from randomized trials with important limitations, or very strong evidence of some other form
C. Low-quality evidence: Evidence from observational studies, unsystematic clinical observations, or from randomized trials with serious flaws
For a complete description of our grading system, please see the UpToDate editorial policy which can be found by clicking "About UpToDate" and then selecting "Policies".
Family support and informational resources
Cyclic Vomiting Syndrome Association (CVSA)
2819 W Highland Blvd, Milwaukee, WI 53208
Phone: 414-342-7880
Fax: 414-342-8980
cvsa@cvsaonline.org, www.cvsaonline.org
Gastroparesis and Dysmotilities Association (GDPA)
5520 Dalhart Hill NW, Calgary, AB, Canada T3A 1S9
Phone: 403-247-3215
www.gpda.net
International Foundation for Functional Gastrointestinal Disorders (IFFGD)
PO Box 170864, Milwaukee, WI 53217
Phone: 414-964-1799
Children: http://www.aboutkidsgi.org
Adults: http://www.iffgd.org
Migraine Awareness Group (MAGNUM)
113 S Saint Asaph, Suite 300, Alexandria, VA 22314
Phone: 703-739-9384
www.migraines.org
National Organization for Rare Disorders
55 Kenosia Ave, PO Box 1968, Danbury, CT 06813-1968
Phone: 203-744-0100, 800-999-6673
TDD number: 203-797-9590
Fax: 203-798-2291
www.nord-rdb.com
United Mitochondrial Disease Foundation
PO Box 1151, Monroeville, PA 15146-1151
Phone: 412-793-8077
Reproduced with permission from: Li, BU, Lefevre, F, Chelimsky, GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2008; 47:379. Copyright ©2008 Lippincott Williams & Wilkins.
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