Cyclic Vomiting Syndrome: A Functional Disorder

Kaul, Ajay,Kaul, Kanwar K. The Korean Society of Pediatric Gastroenterology 2015 Pediatric gastroenterology, hepatology & nutrition Vol.18 No.4

Cyclic vomiting syndrome (CVS) is a functional disorder characterized by stereotypical episodes of intense vomiting separated by weeks to months. Although it can occur at any age, the most common age at presentation is 3-7 years. There is no gender predominance. The precise pathophysiology of CVS is not known but a strong association with migraine headaches, in the patient as well as the mother indicates that it may represent a mitochondriopathy. Studies have also suggested the role of an underlying autonomic neuropathy involving the sympathetic nervous system in its pathogenesis. CVS has known triggers in many individuals and avoiding these triggers can help prevent the onset of the episodes. It typically presents in four phases: a prodrome, vomiting phase, recovery phase and an asymptomatic phase until the next episode. Complications such as dehydration and hematemesis from Mallory Wise tear of the esophageal mucosa may occur in more severe cases. Blood and urine tests and abdominal imaging may be indicated depending upon the severity of symptoms. Brain magnetic resonance imaging and upper gastrointestinal endoscopy may also be indicated in certain circumstances. Management of an episode after it has started ('abortive treatment') includes keeping the patient in a dark and quiet room, intravenous hydration, ondansetron, sumatriptan, clonidine, and benzodiazepines. Prophylactic treatment includes cyproheptadine, propranolol and amitriptyline. No mortality has been reported as a direct result of CVS and many children outgrow it over time. A subset may develop other functional disorders like irritable bowel syndrome and migraine headaches.

Cyclic Vomiting Syndrome: A Functional Disorder

Ajay Kaul,Kanwar K. Kaul 대한소아소화기영양학회 2015 Pediatric gastroenterology, hepatology & nutrition Vol.18 No.4

Cyclic vomiting syndrome (CVS) is a functional disorder characterized by stereotypical episodes of intense vomiting separated by weeks to months. Although it can occur at any age, the most common age at presentation is 3-7 years. There is no gender predominance. The precise pathophysiology of CVS is not known but a strong association with migraine headaches, in the patient as well as the mother indicates that it may represent a mitochondriopathy. Studies have also suggested the role of an underlying autonomic neuropathy involving the sympathetic nervous system in its pathogenesis. CVS has known triggers in many individuals and avoiding these triggers can help prevent the onset of the episodes. It typically presents in four phases: a prodrome, vomiting phase, recovery phase and an asympto-matic phase until the next episode. Complications such as dehydration and hematemesis from Mallory Wise tear of the esophageal mucosa may occur in more severe cases. Blood and urine tests and abdominal imaging may be indicated depending upon the severity of symptoms. Brain magnetic resonance imaging and upper gastrointestinal endoscopy may also be indicated in certain circumstances. Management of an episode after it has started (‘abortivetreatment’) includes keeping the patient in a dark and quiet room, intravenous hydration, ondansetron, sumatriptan, clonidine, and benzodiazepines. Prophylactic treatment includes cyproheptadine, propranolol and amitriptyline. No mortality has been reported as a direct result of CVS and many children outgrow it over time. A subset may develop other functional disorders like irritable bowel syndrome and migraine headaches.

Interpretation of Impedance Data on High-resolution Impedance Manometry Studies—A Worldwide Survey

Lev Dorfman,Sherief Mansi,,Khalil El-Chammas,Chunyan Liu,Ajay Kaul 대한소화기 기능성질환∙운동학회 2024 Journal of Neurogastroenterology and Motility (JNM Vol.30 No.1

Background/AimsEsophageal manometry is the gold standard for esophageal motility evaluation. High-resolution esophageal manometry with impedance (HRIM) allows concurrent assessment of bolus transit and manometry. Inconsistencies between concomitant impedance and manometry data pose a clinical dilemma and has not yet been addressed. We aim to assess interpretation trends of HRIM data among gastroenterologists worldwide. MethodsA cross-sectional study using an anonymous survey was conducted among gastroenterologists worldwide. Statistical analysis was performed to compare responses between providers. ResultsWe received responses from 107 gastroenterologists (26 countries). Most were adult providers (69, 64.5%), and most (77, 72.0%) had > 5 years of experience. Impedance was found to be helpful by 83 (77.6%) participants, but over 30% reported inconsistencies between impedance and manometry data. With incomplete bolus clearance and normal manometry 41 (38.7%) recommended observation, 41 (38.7%) recommended 24-hours pH-impedance, and 16 (15.1%) recommended prokinetics. With abnormal manometry and complete bolus clearance, 60 (57.1%) recommended observation while 18 (17.1%) recommended 24-hours pH impedance and 15 (14.3%) recommended prokinetics. A significant difference was found between providers from different continents in treating cases with discrepancy between impedance and manometry findings (P < 0.001). No significant differences were seen in responses between adult versus pediatric providers and between providers with different years of experience. ConclusionsThere is no consensus on interpreting HRIM data. Providers’ approaches to studies with inconsistencies between manometry and impedance data vary. There is an unmet need for guidelines on interpreting impedance data in HRIM studies.

Not All Children with Cystic Fibrosis Have Abnormal Esophageal Neutralization during Chemical Clearance of Acid Reflux

Woodley, Frederick W.,Moore-Clingenpeel, Melissa,Machado, Rodrigo Strehl,Nemastil, Christopher J.,Jadcherla, Sudarshan R.,Hayes, Don Jr,Kopp, Benjamin T.,Kaul, Ajay,Di Lorenzo, Carlo,Mousa, Hayat The Korean Society of Pediatric Gastroenterology 2017 Pediatric gastroenterology, hepatology & nutrition Vol.20 No.3

Purpose: Acid neutralization during chemical clearance is significantly prolonged in children with cystic fibrosis, compared to symptomatic children without cystic fibrosis. The absence of available reference values impeded identification of abnormal findings within individual patients with and without cystic fibrosis. The present study aimed to test the hypothesis that significantly more children with cystic fibrosis have acid neutralization durations during chemical clearance that fall outside the physiological range. Methods: Published reference value for acid neutralization duration during chemical clearance (determined using combined impedance/pH monitoring) was used to assess esophageal acid neutralization efficiency during chemical clearance in 16 children with cystic fibrosis (3 to < 18 years) and 16 age-matched children without cystic fibrosis. Results: Duration of acid neutralization during chemical clearance exceeded the upper end of the physiological range in 9 of 16 (56.3%) children with and in 3 of 16 (18.8%) children without cystic fibrosis (p=0.0412). The likelihood ratio for duration indicated that children with cystic fibrosis are 2.1-times more likely to have abnormal acid neutralization during chemical clearance, and children with abnormal acid neutralization during chemical clearance are 1.5-times more likely to have cystic fibrosis. Conclusion: Significantly more (but not all) children with cystic fibrosis have abnormally prolonged esophageal clearance of acid. Children with cystic fibrosis are more likely to have abnormal acid neutralization during chemical clearance. Additional studies involving larger sample sizes are needed to address the importance of genotype, esophageal motility, composition and volume of saliva, and gastric acidity on acid neutralization efficiency in cystic fibrosis children.

Not All Children with Cystic Fibrosis Have Abnormal Esophageal Neutralization during Chemical Clearance of Acid Reflux

Frederick W. Woodley,Melissa Moore-Clingenpeel,Rodrigo Strehl Machado,Christopher J. Nemastil,Sudarshan R. Jadcherla,Don Hayes Jr,Benjamin T. Kopp,Ajay Kaul,Carlo Di Lorenzo,Hayat Mousa 대한소아소화기영양학회 2017 Pediatric gastroenterology, hepatology & nutrition Vol.20 No.3

Purpose: Acid neutralization during chemical clearance is significantly prolonged in children with cystic fibrosis, com-pared to symptomatic children without cystic fibrosis. The absence of available reference values impeded identi-fication of abnormal findings within individual patients with and without cystic fibrosis. The present study aimed to test the hypothesis that significantly more children with cystic fibrosis have acid neutralization durations during chem-ical clearance that fall outside the physiological range. Methods: Published reference value for acid neutralization duration during chemical clearance (determined using combined impedance/pH monitoring) was used to assess esophageal acid neutralization efficiency during chemical clearance in 16 children with cystic fibrosis (3 to <18 years) and 16 age-matched children without cystic fibrosis.Results: Duration of acid neutralization during chemical clearance exceeded the upper end of the physiological range in 9 of 16 (56.3%) children with and in 3 of 16 (18.8%) children without cystic fibrosis (p=0.0412). The likelihood ratio for duration indicated that children with cystic fibrosis are 2.1-times more likely to have abnormal acid neutraliza-tion during chemical clearance, and children with abnormal acid neutralization during chemical clearance are 1.5-times more likely to have cystic fibrosis. Conclusion: Significantly more (but not all) children with cystic fibrosis have abnormally prolonged esophageal clear-ance of acid. Children with cystic fibrosis are more likely to have abnormal acid neutralization during chemical clearance. Additional studies involving larger sample sizes are needed to address the importance of genotype, esophageal motility, composition and volume of saliva, and gastric acidity on acid neutralization efficiency in cystic fibrosis children.