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2025 Pediatric Cyclic Vomiting Syndrome Guidelines

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K Karrento et al. J Pediatr Gastroenterol Nutr. 2025;80:1028–1061. Open Access! North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition 2025 guidelines for management of cyclic vomiting syndrome in children

This is an excellent CVS guideline (36 pages). There is a lot of information and advice that is not easily summarized. Some important points:

  • Epidemiology: “The prevalence of pediatric CVS is estimated between 1.9% and 2.3% with an incidence of 3.2 per 100,000 children/year.1–3, 16 CVS peaks among school-aged children and often evolves into migraine headaches in adolescent years…17 56% of children experience resolution of CVS during a median follow-up of 29 months (range 6 months to 7 years)…A long-term follow-up study demonstrated progression to migraine in 26% of those with pediatric CVS.1“
  • Autonomic dysfunction: “An underlying autonomic dysregulation is also supported by clinical features during attacks (diaphoresis, listlessness, palpitations, and peripheral vasoconstriction), and a study shows that 40% of pediatric patients with CVS develop chronic dysautonomia during adolescence.18“
  • Cannabinoid hyperemesis syndrome: “CHS is considered a probable subtype of CVS that presents after prolonged and excessive cannabis use…26 Topical capsaicin, benzodiazepines, and droperidol or haloperidol have all been proposed as possible treatments for acute CHS episodes…50 Adult guidelines recommend that CHS patients be offered the same therapies as CVS patients…Complete cannabis cessation is the only known effective long-term treatment for CHS.”
  • Sato-variant: “This subtype manifests elevated levels of adrenocorticotropin hormone, cortisol, antidiuretic hormone, catecholamines, and prostaglandin E2, consequently presenting with hypertension and profound lethargy.25 While there is no published data for guidance, electrolyte monitoring is warranted, and episodic hypertension is generally managed by short-acting agents such as lisinopril or labetalol.”
  • L-carnitine: “The panel did not find evidence of efficacy other than when used in combination with coenzyme Q10 and cautioned against use based on concerns for atherosclerosis in animals.”
  • Propranolol: “The panel cautioned for use in patients with reactive airway disease…Retrospective studies showed high long-term efficacy of propranolol (57%–81%) when used as a first-line agent for pediatric CVS.155, 156 Two prospective, observational studies in pediatric CVS showed a high response rate to propranolol (77%–93%).157, 158 A larger (n = 81) randomized (uncontrolled and unblinded) trial demonstrated long-term effects of propranolol 1 mg/kg/day on both frequency and severity of CVS attacks with a 92% response rate and superiority over amitriptyline (53% response rate).159“
  • Cyproheptadine: “Using criteria of ≥50% improvement in outcomes of interest (episode frequency and duration), 55%–75% (retrospective to randomized) met this threshold. In pediatric migraine, 83% had a positive response.”
  • Aprepitant: “The use of aprepitant two or three times per week for prophylaxis resulted in significant improvement in several essential outcomes, including episode frequency, duration, intensity, symptom-free periods, hospitalization rates, and school attendance.69, 169 At the 12-month follow-up, 82% of children [n=95] achieved either partial or complete treatment response.”
  • Tricyclic Antidepressants (TCAs): “The panel suggests that this medication be reserved for those with more frequent and severe disease who have not responded to therapies with more favorable side effect profiles. Caution for possible behavioral changes, including suicidality, is indicated in all children and adolescents….Using the common criteria of ≥50% improvement as definition of response (complete or partial), 57% of pediatric and 81% of adult CVS patients responded.”
  • Anticonvulsants: “The guideline panel suggests not using anticonvulsants (e.g., topiramate or valproate) for preventing CVS episodes in children and adolescents, except for refractory CVS.”

My take: While data for CVS remains limited, these guidelines are likely to influence how CVS is managed in children.

Related blog posts:

Disclaimer: This blog, gutsandgrowth, assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  Because of rapid advances in the medical sciences, the gutsandgrowth blog cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition

Dr. William Balistreri: Whatever Happened to Neonatal Hepatitis (Part 2)

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Recently Dr. Balistreri gave our group an excellent lecture. I have taken some notes and shared some slides. There may be inadvertent omissions and mistakes in my notes.

Key Points:

  • Producing enough bile acids and recycling bile acids in enterohepatic circulation is crucial for bile acid flow. In addition, there are ‘good’ bile acids like cholic acid that have trophic properties and ‘bad’ bile acids like lithocholic acid that cause liver toxicity
  • In addition to defects in the metabolic pathway of bile acids, discoveries identified defects in the membrane transporters (eg. FIC1, BSEP, MDR3), trafficking proteins (eg. MYO5B, VPS33B), nuclear control receptors (eg. FXR), and tight junction proteins (eg. TJP2). Tight junction protein defects are associated with bile leakage from bile canaliculus
  • Alagille syndrome, a disorder of embryogenesis, related to JAG1-NOTCH2 signaling pathways affects organs throughout the body
  • Many of these genetic mutations are now being identified in adults with unexplained liver diseases (eg. intrahepatic cholestasis of pregnancy and cryptogenic cirrhosis)
  • Cholestasis panels and whole exome sequencing are important tools
  • Ileal bile acid transporter (IBAT) inhibitors have emerged as important therapies for conditions like Alagille which were previously treated with biliary diversion

Cholestasis Evaluation:

See blog post: Identifying Biliary Atresia in Infants: New Guidelines

Baby with Carbamoyl-phosphate synthetase 1 (CPS1) deficiency (urea cycle defect)

My take: This lecture really shows how the field of pediatric liver disease has been a puzzle. Now one can see how almost all of the pieces of the puzzle work together.

Related blog posts:

Dr. William Balistreri: Whatever Happened to Neonatal Hepatitis (Part 1)

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Recently Dr. Balistreri gave our group an excellent lecture. I have taken some notes and shared some slides. There may be inadvertent omissions and mistakes in my notes.

In my view, Dr.Balistreri’s contributions to our field of pediatric gastroenterology, hepatology and nutrition are unsurpassed by any other individual. This is due to his leadership roles (division director, president of AASLD and NASPGHAN), his editor roles (Journal of Pediatrics, and JPGN), his extensive publications/research including sentinel discoveries in bile acid pathophysiology and treatment, and through education (lectures and mentorship).

Key points:

  • Fifty years ago, ~65% of neonatal cholestasis cases were poorly understood and lumped together under the heading of “Idiopathic Neonatal Hepatitis” (INH). The discovery of Alpha-One Antitrypsin (A1AT) Deficiency was instrumental, indicating that there were specific medical diseases mislabeled as INH
  • A1AT deficiency proved that many cases were NOT “idiopathic,” NOT necessarily “neonatal,” and NOT “hepatitis”
  • Currently, less than 10% of infants with neonatal cholestasis are unspecified. Dr. Balistreri’s goal has been to make sure every patient receives a precise diagnosis
  • Now more than 90 genetic conditions have been recognized as causing neonatal cholestasis
  • The journey of unraveling the reasons for neonatal cholestasis includes the referral of two infants from Atlanta by Dr. Saripkin to Cincinnati. These infants who had an older sibling who had died at 4 months of age were determined to have an inborn error of bile acid metabolism. Subsequently, they were treated successfully with cholic acid which suppressed production of toxic precursors via feedback inhibition
  • There are 11 steps in the conversion of cholesterol to bile acids; thus, it was hypothesized and later proven that there would be many other inborn errors of bile acid metabolism

Related blog posts:

Treatment Guidelines for Pediatric Irritable Bowel Syndrome

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J Green et al. JPGN 2025; Open Access! ESPGHAN/NASPGHAN guidelines for treatment of irritable bowel syndrome and functional abdominal pain-not otherwise specified in children aged 4–18 years

Overall, this article notes that the evidence based for most treatments for pediatric irritable bowel (IBS) is often lacking.

Specific recommendations:

Executive summary of Best Practice Statements‐ The Guideline Development Group (GDG) notes that a crucial emphasis should be placed on education regarding the abdominal pain‐related disorders of gut–brain interaction.

My take: This is a helpful guideline and likely to influence practice.

Related blog posts:

Understanding the Prevalence and Burden of Pediatric Inflammatory Bowel Disease in U.S.

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 From editorial (which is more expansive than the study):

Kappelman et al4 report the US prevalence of pediatric-onset IBD (diagnosed before the age of 20 years by a physician) as well as rates of disease based on race and ethnic background. To ensure that a representative population was captured, they combined multiple health administrative databases…

The authors report that the US currently has a pediatric IBD prevalence of 125 per 100,000 population, increased from 110 per 100,000 in 2011. This is higher than previously reported in Canada (82 per 100,000 in 2023)6 and Sweden (75 per 100,000 in 2010).7 These differences may be due to the older age cutoff used in the US data, <20 years vs <18 years in the Canadian and Swedish studies. However, misclassification bias may also play a role...

Nevertheless, understanding the approximate prevalence of pediatric IBD in the US allows for adequate human and financial resource planning for this important population of children with an impactful chronic disease. The high prevalence should raise concerns among health care practitioners and policy makers that we have under-resourced IBD care in children, especially considering the high rate of use of biologics and the growing direct health costs incurred in the treatment of this population.11

The burden of IBD in pediatrics goes beyond that of the child. Compared with adult IBD, it disproportionately affects caregivers and families (owing to missed work for appointments, hospitalizations, and home care), mental health of both the patient and the parents, and the health system...

They report that pediatric IBD is more frequent among White children and adolescents (145 per 100,000) compared with Black (91 per 100,000) and Hispanic (88 per 100,000) children, whereas children of Asian origin have markedly lower rates (52 per 100,000).“

My take: The updated prevalence data helps understand the increasing frequency of pediatric IBD. The associated commentary reminds us of the broader burden the disease has for families and for our communities.

Related blog posts:

Constipation Preceding a New Diagnosis of Inflammatory Bowel Disease

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S Cenni et al. J Pediatr Gastroenterol Nutr. 2025;80:799–806. The prevalence of constipation in children with new diagnosis of inflammatory bowel disease: A retrospective study

This was a cross-sectional observational study in pediatric IBD-patients (n=238) with 104 (43.6%) with Crohn disease (CD), 130 (54.6%) with ulcerative colitis (UC) and 4 (1.6%). Only patients who filled out the Rome IV questionnaire for FC, through dedicated symptom recall at the next clinic appointment or telephone recall, were finally enrolled in the study for subsequent analysis.

Key findings:

  • Forty-seven out of 238 (19.7%) had a functional constipation history before the IBD diagnosis. In the CD children the prevalence of constipation before the IBD diagnosis was 19/104 (18.2%) and in the UC patients was 28/130 (21.5%).
  • The difference in terms of endoscopic localization was statistically significant in UC patients presenting FC (p = 0.026) with a prevalence of proctitis and left side colitis (30% and 15%, respectively)
  • There was a delay in the diagnosis of patients with preceding constipation

Discussion Points:

  • The main limitations of the present study are certainly related to the retrospective nature and, therefore, the possibility of recall biases must be taken into account.
  • Rectal bleeding that persists despite stool softener therapy should be investigated

My take: While this study shows that constipation is fairly common prior to a diagnosis of IBD, many times a parent is told that their child is constipated on the basis of an xray or simply because the child complained of stomach pain. This likely increases the risk of recall bias. My guess is that a prospective study involving careful questioning at the time of the initial colonoscopy would yield a lower number of children who had constipation at the time of diagnosis.

Related blog posts:

Set of Shucked! at The Fox Theater. Really enjoyed this ‘corny’ musical.

Excellent Review of H Pylori in Children Plus One on Healthy Aging

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Congratulations to my partner Ben Gold and coauthors on their recent publication –an excellent review of H pylori infection in children:

DL Mebuge, RJ Noel, BD Gold. Current Treatment Options in Pediatrics 2025; 11:13. doi.org/10/1007/s40746-025-00328-6. Open Access: Helicobacter pylori in Children: An Individualized Approach to a Worldwide Disease

Related blog posts:

For those interested in healthy aging/healthspan, Eric Topol had a recent interview with Katie Couric (54 minutes): On the State of US Life Science and Extending Healthspan

MMR Vaccination Safety in Immunocompromised Kids with IBD and Liver Transplant

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A Keutler et al. Vaccine 2025; 59: 127288. Open Access! Safety and immunogenicity of the measles-mumps-rubella vaccine in immunocompromised children with inflammatory bowel disease, or after liver transplantation: An observational study

Background: “Measles is a highly contagious disease and, despite the availability of a safe and effective vaccine, remains still an important cause of childhood death worldwide [1,2]. The risk of severe illness in measles-naive individuals is particularly high in immunocompromised patients with inflammatory bowel disease (IBD) or after liver transplantation (LT) [3]…Ideally, vaccination with live attenuated vaccines (LAVVs) should be completed four weeks before organ transplantation or the initiation of immunosuppressive therapy (IST) to allow for the live vaccine’s incubation period and minimize the risk of vaccine-associated disease…LAVVs are considered contraindicated during IST due to safety concerns and limited experience.”

Methods: “In this prospective multicenter observational study (DRKS00014569) 22 children and adolescents with incomplete MMR vaccination status were identified… with stable immunosuppressive therapy in the last three months with no evidence of underlying disease activity…Sixteen patients were vaccinated against MMR, eleven after liver transplantation and five with inflammatory bowel disease. At the time of vaccination, four patients were receiving moderate (e.g., tacrolimus drug level below 5 ng/ml), eleven were receiving high-intensity immunosuppression (e.g. anti-tumor-necrosis factor agents, mycophenolate mofetil) and one child had previously discontinued immunosuppressive treatment.”

Immediately prior to the references, the authors provide a downloadable document detailing how they chose to categorize the degree of immunosuppression and their precise protocol, including immunologic pretesting and drug contraindications as noted below.

Key findings:

  • There were no serious adverse events or complications related to the vaccination
  • In children receiving immunosuppressive medications, the seroconversion rate for measles after the first MMR vaccination was 73.3 % (11/15) and after the second vaccination 80 % (12/15)

My take: In carefully-selected immunocompromised pediatric patients, the MMR vaccine may be safe. However, given the small numbers receiving vaccination in this study, the absolute safety is unclear. Even infrequent adverse effects would be problematic. This study’s protocol could be helpful for those considering vaccination in immunocompromised populations with a measles epidemic. For now, the most important approach is improving vaccination rates in those (especially family members) without contraindications.

Related blog posts:

Disclaimer: This blog, gutsandgrowth, assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  Because of rapid advances in the medical sciences, the gutsandgrowth blog cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

Personal Quest Update: The Beginning of the End for the Penny & World Button Battery Day

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As I noted in a blog in 2012, I had advocated for removal of the penny due to frequent ingestions (starting more than 20 years ago) (link: “Watch for change in the stools”). Now it appears I will finally get my wish.

RP Jones, Reuters, 5/22/25: US Treasury unveils plan to end production of penny coin

An excerpt:

In recent weeks, Republican and Democratic lawmakers introduced separate bills in the House of Representatives and the Senate calling for an end to penny production. The change means that businesses will have to start rounding the prices of cash transactions up or down to the nearest five-cent nickel as the number of pennies in circulation dwindles.

The Treasury’s penny phase-out plan was first reported by the Wall Street Journal…The cost of producing the penny has risen from 1.3 cents per coin to 3.69 cents over the past 10 years, according to the Treasury. It said stopping production will lead to immediate annual savings of $56 million. The penny was first issued by the government in 1793

Related article:

K Bucholtz, Statista 6/11/20: The Most Commonly Swallowed Objects in America

The following statistic will delight fans of obscure facts and overbearing bureaucracy: According to the U.S. Consumer Product Safety Commission, more than 850,000 accidental ingestions of everyday objects were recorded in the U.S. between 2010 and 2019. ..Coins topped the list of accidental ingestions, with around 33,000 being recorded each year (more might go unreported).

*According to the U.S. Consumer Product Safety Commission, 

My take: While this is a step in the right direction, there are still 114 billion pennies currently in circulation in the U.S. according to the Reuters article. Perhaps, other coins will be ingested less in the coming years too as so many business transactions are via smartphones and credit cards.

Button batteries are a much more serious ingestion than a coin. For World Button Battery Day, here’s a link to a 3 minute video from FISPGHAN. FISPGHAN Button Battery Video

The video provides a simple explanation of the problem though at times it is not certain who the target audience is. Particularly the first part feels like a video for kids rather than for parents. Thanks to Ben Gold for sharing this link.

Aso, Link: FISPGHAN Button Battery Website

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