Category Archives: Pediatric Gastroenterology Intestinal Disorder

Disparities Are Abundant in Pediatrics -4 Studies on IBD, SUID, Specialty Referrals and in the NICU

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Lately, I have been struck by the increasing volumes of research on disparities in medicine. A recent issue from the Journal of Pediatrics had at least 4 articles touching on this subject. Most of these articles view these disparities as being due to structural racism.

  1. J Smith et al. J Pediatr 2023; 260: 113522. Racial Disparities in Pediatric Inflammatory Bowel Disease Care: Differences in Outcomes and Health Service Utilization Between Black and White Children

In this article, the authors show that among 519 children with newly diagnosed IBD, black patients were less likely to achieve CSFR (corticosteroid-free remission) 1-year post diagnosis (OR: 0.52, 95% CI:0.3-0.9) and less likely to achieve sustained CSFR (OR: 0.48, 95% CI: 0.25-0.92). This was despite a similar phenotype. Black patients had much higher rate of public insurance (58% vs 30%) and were less likely to be seen for routine follow-up visits.

While the authors attribute the response to therapy as likely to be related mainly to social determinants of health, there may be biologic factors at work as well. In a recent study (R Greywoode et al. Inflamm Bowel Dis 2023; 29: 843-849. Open Access! Racial Difference in Efficacy of Golimumab in Ulcerative Colitis), there were disparate racial response rate differences among patients receiving the same therapy.

2. AP Srinivasan et al. J Pediatr 2023; 260: 113485. Open access! Disparities in Pediatric Specialty Referral Scheduling and Completion

In this retrospective review of 38,334 specialty referrals (2019-2021), of all referrals, 62% were scheduled and 54% were completed. Referral completion rates were lower for patients with Black race (45%), Native Hawaiian/Pacific Islander race (48%), Spanish language (49%), and public insurance (47%). Since the report focused on primary clinics within 5 miles of the hospital, the authors indicate that the lower referral completion is due to nongeographic structural barriers, including problems with arranging visits as some families have less flexible jobs. The authors conclude that the fact that specialty care is inaccessible is due to ” the effects of racism and discrimination on the scheduling process.” The authors recommend that the referral workflows should not “place the administrative onus of scheduling exclusively on families.”

3. SS Hwang et al. J Pediatr 2023; 260: 113498. Racial and Ethnic Disparities in Sudden Unexpected Infant Death Among US Infants Born Preterm

This was a retrospective cohort analysis of linked birth and death certificates from 50 states from 2005 to 2014 to investigate among US infants born at <37 weeks gestation (a) racial and ethnic disparities in sudden unexpected infant death (SUID).  Key findings:

  • Among 4,086,504 preterm infants born during the study period, 8096 infants (0.2% or 2.0 per 1000 live births) experienced SUID. State variation in SUID ranged from the lowest rate of 0.82 per 1000 live births in Vermont to the highest rate of 3.87 per 1000 live births in Mississippi
  • In the adjusted analysis (for sociodemographic and clinical factors), compared with Non-Hispanic white infants, Non-Hispanic black infants and Alaska Native/American Indian preterm infants had greater odds of SUID (aOR, 1.5)and aOR, 1.44) 
  • The authors attribute the adjusted differences to “structural racism [that] creates and perpetuates health inequities.”

4. KL Karvonen et al. J Pediatr 2023; 260: 113499. Open access! Structural Racism Operationalized via Adverse Social Events in a Single-Center Neonatal Intensive Care Unit

This was a retrospective cohort study of 3290 infants hospitalized in a single center NICU between 2017 and 2019 in the Racial and Ethnic Justice in Outcomes in Neonatal Intensive Care (REJOICE) study. Key findings:

  • 205 families (6.2%) that experienced an adverse social event. Black families were more likely to have experienced a CPS referral and a urine toxicology screen (OR, 3.6). American Indian and Alaskan Native families were also more likely to experience CPS referrals and urine toxicology screens (OR, 15.8 and OR, 7.6)
  • Black families were more likely to experience behavioral contracts and security emergency response calls

My take: These articles offer more proof that racial disparities are highly prevalent in healthcare. While we should strive to help improve access/equitable care in medicine, the approach needs to start well before the clinic/hospital. To make the greatest impact, policies are needed to address education outcomes and poverty (eg. expanded child income tax credit) which overall impact health more than anything that happens in our clinics.

Related blog posts:

On Disparities:

On SUID:

  • Safe Sleep Recommendations
  • Safe Sleep A terrific website that focuses on this crucial issue: Charlieskids.org; it has videos, do’s and don’ts as well as a link to Cribs for Kids (discounted safe crib website). In addition, this website has a book called “Sleep Baby Safe and Snug” which incorporates updated recommendations on safe sleep practices.
  • The High Toll of Sudden Infant Death From 2013-2015, there was an average of 3523 US infants each year who died from SUID (sudden unexpected infant death), peaking at 1-2 months of life.  More black infants died of SUID in the first year than black children who died from firearm homicides in all of childhood through age 19 years. SUID deaths from 2013-2015 (10,568) was similar to the total number of motor vehicle-traffic deaths in all of childhood (10,714) and greater than the total number of any of the other causes.
  • Are We Making Progress on Infant Sleep-Related Deaths? (not anymore) 
  • Safe Sleep (AAP 2017) 
Eggplant

Briefly noted: Necrotizing Enterocolitis After Onasemnogen Abeparvovec for Spinal Muscular Atrophy

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J Gaillard et al. J Pediatr 2023; 260: 113493. Necrotizing Enterocolitis following Onasemnogene Abeparvovec for Spinal Muscular Atrophy: A Case Series

Background: Onasemnogene abeparvovec treats spinal muscular atrophy by delivering a functional SMN1 gene.

Key finding: This case report documents two cases of medically-treated necrotizing enterocolitis (NEC) that developed shortly after Onasemnogen Abeparvovec which was administered at ~3 weeks of life in two full term infants (born at 40 and 41 weeks).

My take: Consider the diagnosis of NEC in full term infants with SMA who have received gene therapy.

Related blog posts:

Views from Bike Trail near Ospedaletti, Italy
While grabbing a snack & taking a break from our bike ride, we saw all of these wind surfers. The wind on this day was crazy and these surfers looked like they were going 30-40 mph

How Much Harder is a Colonoscopy in Children Less Than 6 Years of Age

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R Bolia et al. JPGN 2023; 77: 396-400. Characterization of Colonoscopies in Preschool Children

In this retrospective review, among 1671 colonoscopies (2014-2020), 13% (n=219) were in children less than 6 year of age (Median 3.9 yrs). Key findings:

  • Most common indications in preschoolers were rectal bleeding 35% (n = 78), inflammatory bowel disease 24% (n = 53), diarrhea 13% (n = 30), iron-deficiency anemia 11% (n = 25), and abdominal pain 7% (n = 16).
  • Ileal intubation rate (IIR) and cecal intubation rate (CIR) were lower in preschoolers (2 to <6 yrs) compared to older children, 81% vs 92% (P = 0.0001), and 93% vs 96.4% (P = 0.02), respectively, and even lower in those aged <2 years, 48.1% IIR (P = 0.0001) and 85.1% CIR. 
  • Diagnostic yield was highest for rectal bleeding at 41% (32/78) including juvenile polyps in 27. The diagnostic yield was 37% for those with diarrhea (12/30) and 36% (9/25) for those with iron deficiency anemia. Overall, diagnostic yield was 40% (87/219)
  • 10 patients (5% of total and 11.5% of those with abnormalities) had findings limited to right colon and/or ileum; thus, incomplete evaluation would have missed these findings.

The authors suggest modifying the PEnQuIN goal of IIR >/= 85% in young children. However, this is unnecessary as most endoscopists are not separating their cases by age.

My take: This study shows that colonoscopy is often more difficult to complete in younger children. Achieving high IIR improves the yield of colonoscopy. Overall, the findings in this report mirror our experience in which colonoscopy had a diagnostic yield of 42% (in non-folllowup colonoscopies) and findings isolated to ileum were noted in 6% (and additional 4% with grossly normal/abnormal histology).

Related blog posts:

Whispering Spruce Trail, Davis WV
Nelson Rocks, Circleville, WV

Changing Approach to Refeeding Syndrome in Children/Adolescents with Eating Disorders

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VB Stoody et al. J Pediatr 2023; 260: 113482. Advancements in Inpatient Medical Management of Malnutrition in Children and Adolescents with Restrictive Eating Disorders

This article is a good review detailing the management of restrictive eating disorders, including anorexia nervosa, atypical anorexia nervosa (weight in normal range despite significant loss) and avoidant/restrictive food intake disorder. The tables provide diagnostic criteria, medical complications, and clinical factors supporting admission.

The authors also delve into the topic of refeeding syndrome. Key points:

  • “Contrary to prior belief, HCR [high calorie restriction] does not carry a significant risk of RS [refeeding syndrome] when close electrolyte surveillance and supplementations are implemented. Furthermore, LCR is associated with poor inpatient weight gain…and longer hospital admissions.”
  • “The evidence in our progress report supports an HCR strategy of initiating inpatient refeeding at 2000-2400 kcal/day…and increasing by 200 kcal/day in patients with an admission BMI of >60% mBMI.”
  • The authors recommend daily electrolyte assessment for first 7 days, followed by every other day for duration of admission.
  • Daily caloric requirements “often exceed 3000 kcal/day in the initial weeks of refeeding”

My take: While the authors focus on restrictive eating disorders, their approach to refeeding implies consideration in other disorders associated with moderate malnutrition.

Related blog posts:

Saw this dog at a bar along the Hollywood, FL broadwalk

Dog walks into a bar jokes:

A dog walks into the bar, jumps up on the stool and says to the bartender, “Hey barkeep, it’s my birthday today. How ’bout a free drink?” The bartender turns, looks at the dog and nods his head, “Sure pal, toilet’s right down the hall.”

And

A dog walks into a bar. Bartender nods and says, “Hey dog, haven’t seen you in a while, how are things going?” Dog looks at him sadly and replies, “Ruff.”

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.

Neuro-Stim for Refractory Cyclic Vomiting?

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K Karrento et al. JPGN 2023; 77: 347-353. Percutaneous Electrical Nerve Field Stimulation for Drug-Refractory Pediatric Cyclic Vomiting Syndrome

In this prospective study with 30 children with drug-refractory CVS, response was classified as ≥50% improvement in either frequency or duration of attacks at extended follow-up.

Key findings:

  • At follow-up, 80% met criteria for treatment response with a median (IQR) response duration of 113 (61–182) days.
  • At end of therapy, 66% and 55% patients reported global response of at least “moderately better” and “a good deal better,” respectively.
  • There were no serious side effects.

Limitations: episodic nature of CVS, no control group, hx/o strong placebo control group and lack of validated assessment tool

My take: 1. As with the drug therapies with CVS, it is difficult to know how effective PENFS is for CVS. At the same time, its good safety profile makes this therapy an intriguing option in those not responding to more typical treatments. 2. The visual abstract is funny -mainly due to the two laughing kids holding hands. Though it would be even better if the person on the left was puking a lot instead of having a PENFS device.

Related blog posts:

How to Provide More Cost-Effective Celiac Care

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PF Farmer et al. J Pediatr 2023; 259: 113487. Single-Center Analysis of Essential Laboratory Testing in Patients with Newly Diagnosed Celiac Disease

In this study, the authors analyzed laboratory testing results from pediatric patients newly diagnosed with celiac disease (2018-2021) to determine the usefulness of each test derived from recommended guidelines (J Snyder et al. Pediatrics 2016; 138: e20153147). Screening protocols in their center resulted in an estimated cost of approximately $320,000 during the study. Tests at diagnosis included hemoglobin, alanine/aspartate aminotransferase, ferritin, iron, TSH, Free T4, and vitamin D screening. These screening tests were done in ~80% of 468 patients.

Key findings:

  • Ferritin was abnormal in 29%, hemoglobin was abnormal in 12%, and iron was abnormal in 22%. Abnormal ferritin captured all patients in this cohort with an abnormal iron. If ferritin was used as an isolated screen with reflective iron testing, this would have reduced costs by about $12,000
  • AST and ALT were abnormal in 2% and 11% respectively
  • 25-OH Vitamin D was abnormal in 14%. Recent data indicated that low Vit D levels are similar among patients with and without celiac disease (R Ahlawat et al. JPGN 2019; 69: 449-454)
  • TSH and Free T4 were abnormal in 7% and 0.3% respectively. For thyroid disease, TSH and free T4 testing did not lead to any new diagnosis of thyroid disease (7 carried a preexisting diagnosis). There were 19 additional patients with abnormal lab values who had more testing due to initial abnormalities. If TSH alone were used for screening, costs savings would be about $29,000. If no thyroid testing were done, this would have reduced costs by about $40,000.
  • Hepatitis B immunity was NOT present in 69%. However, recent studies have shown similar levels of immunity in those with and without celiac disease. In addition, it is not clear that a low level hepatitis B surface antibody always indicates a lack of immunity. Eliminating hepatitis B screening would have reduced costs by about $63,000.
  • The authors note that the cost savings by adopting their recommendations would have saved about $104,000 (out of $320,000).

My take: This is a very useful study and indicates that curtailing initial testing for celiac disease could reduce costs substantially and without compromising care. This would include not checking a serum iron, a free T4, or hepatitis B studies. The authors note that the value of Vit D testing is also questionable but may be worthwhile due to increased risk of bone disease in individuals with celiac disease.

Related blog posts:

Blackwater Falls near Davis, WV
Seneca Rocks Trail, WV

Nonanaphylactic Alpha-Gal and Chronic Gastrointestinal Symptoms

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D Glynn et al. J Pediatr 2023; 259: 113486. Nonanaphylactic Variant of Alpha-Gal Syndrome as an Etiology for Chronic Gastrointestinal Symptoms in Children

Background: A CDC report showed that between 2010 and 2022, more than 110,000 suspected cases of alpha-gal syndrome were identified. The majority of cases are linked to bites from the lone star tick which affects much of the U.S. (map below) as well as Central and South America, Asia, Africa, Australia and parts of Europe.

Findings: This study reports 3 pediatric patients who presented with only nonanaphylactic symptoms of alpha-gal syndrome. These patients with recurrent gastrointestinal distress and emesis after consuming mammalian meat, even in the absence of an anaphylactic reaction.

The diagnosis in these three patients was established by history, serum alpha-gal immunoglobulin E elevation and response to avoidance of red meat.

My take: Checking a serum Alpha-gal IgE seems like a good idea in some children with unexplained abdominal pain with episodic exacerbation with vomiting, especially if tick exposure. Anecdotally, I have checked this a few times and so far I have not I identified a case. Most cases of Alpha-gal will be associated with urticaria.

Related blog post: Tick Bites Can Lead to Allergy to Red Meat

CDC: Alpha-gal syndrome “Symptoms commonly appear 2-6 hours after eating meat or dairy products, or after exposure to products containing alpha-gal (for example, gelatin-coated medications).”

CDC: Food products that may contain alpha-gal: Mammalian meat (such as beef, pork, lamb, venison, rabbit, etc.) can contain high amounts of alpha-gal. Food products that contain milk and milk products typically contain alpha-gal (though many patients tolerate dairy products)

Foods that do NOT contain alpha-gal (unless cross contamination):

  • Poultry, such as chicken, turkey, duck, or quail
  • Eggs
  • Fish and seafood, such as shrimp
  • Fruits and vegetables
From CDC website: https://www.cdc.gov/ticks/maps/lone_star_tick.html

Case Report: 20 month old with Abrupt Vomiting and Multisystem Disease

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Case history: A well-nourished previously healthy 20 month old was admitted to the hospital with a one week history of frequent vomiting. He had a history of frequent lint ingestion. He had been seen by three different health care providers during the week prior to his admission.

At the time of admission, he had numerous electrolyte derangements (Na 124, K 3.2, CL 76) and acute kidney dysfunction with a BUN of 118 and Creatinine of 3.06. He had severe multisystem disease including severe ventricular dysfunction (BNP 2196). He needed an oscillator ventilator, dialysis and cardiac medications (including epinephrine, and milrinone).

He had an extensive evaluation. After he had stabilized and then markedly improved (12 days after admission), an UGI study demonstrated an obstruction near the 2nd-3rd portion of the duodenum with a dilated proximal duodenum.

UGI study:

Due to the obstruction which was thought to be anatomic, the GI service deferred management to pediatric surgery. The surgical service requested GI inspection with endoscopy immediately prior to surgery. If a bezoar was identified, the surgical plan was for a mini-laparotomy. If not, the surgical plan was for laparoscopy repair.

Endoscopy findings: There was a narrowed opening (different orientation of same narrowing in both pictures) with a string-like material.

Surgical findings: A duodenal web with a piece of lint was identified. The patient had a laparoscopic wedge excision with a transverse closure of the duodenotomy. The lint may have occluded the tiny opening of the web that he had been living with since he was born.

My take: This is the first time I have seen the endoscopic appearance of a duodenal web.

In my view, this was a ‘great case.’ In a commentary by Jerome Groopman (N Engl J Med 2004; 351:2043-2045), his wife noted that a case is “a great case because you not only make the diagnosis — you do something fundamental about it. You can really help.” However, Dr. Groopman stopped using the words, ‘a great case’ after his personal experience when his son was severely ill as an infant with a bowel obstruction due to intussusception: “For me and for Pam, the experience had no resonance of “a great case.” There was no intellectual pleasure in solving a clinical puzzle, no charge of exhilaration from the drama of the operation. Instead, there was terror, raw and palpable, as we realized how close we had come to burying our first son….I still find myself unable, except in retrospect, to retrieve the language of my youth and speak about “a great case.” It is as if medicine at this stage of my life has split into two streams — a current of marvelous biology and an undertow that pulls at the soul.”

Subcutaneous Vedolizumab Now FDA-Approved for Adults with Ulcerative Colitis

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9/27/23 Takeda: U.S. FDA Approves Subcutaneous Administration of Takeda’s ENTYVIO® (vedolizumab) for Maintenance Therapy in Moderately to Severely Active Ulcerative Colitis

” Takeda (TSE:4502/NYSE:TAK) today announced that the U.S. Food and Drug Administration (FDA) has approved a subcutaneous (SC) administration of ENTYVIO® (vedolizumab) for maintenance therapy in adults with moderately to severely active ulcerative colitis (UC) after induction therapy with ENTYVIO intravenous (IV).1 ENTYVIO SC is expected to be available in the U.S. as a single-dose pre-filled pen (ENTYVIO Pen) by the end of October. Additionally, a Biologics License Application for an investigational SC administration of ENTYVIO for the treatment of adults with moderately to severely active Crohn’s disease is currently under review by the FDA.”

From WJ Sandborn et al. Gastroenterol 2020; 158: 562-572. Open Access! Efficacy and Safety of Vedolizumab Subcutaneous Formulation in a Randomized Trial of Patients With Ulcerative Colitis

In the VISIBLE1 Trial, dosing was IV for week 0 and 2, then every other week SC for maintenance.