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

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

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).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.

Out of Pocket Maximum (Sad Humor)

Dr. Glaucomflecken Twitter Link (w/o login): Out of Pocket Maximum (1:13 min)

There are a bunch of new videos (one for everyday in September) similar to this on related topics -search 30 Days of US Healthcare. Here’s another one: 30 Days of US Healthcare: Surprise Billing (YouTube)

My take: As usual, Dr. Glaucomflecken humor sheds light on the faults in our nation’s health care insurance coverage.

The Quality of Evidence for Dietary Treatments in Inflammatory Bowel Disease

BN Limketkai et al. Clin Gastroenterol Hepatol 2023; 21: 2508-2525. Open Access! Dietary Interventions for the Treatment of Inflammatory Bowel Diseases: An Updated Systematic Review and Meta-analysis

This was a systematic review of prospective controlled trials (n=27) of solid food diets for the induction or maintenance of remission in IBD.

Key findings:

  • For induction of remission in Crohn’s disease (CD), the Mediterranean diet was similar to the Specific Carbohydrate Diet (low certainty of evidence), and partial enteral nutrition (PEN) was similar to exclusive enteral nutrition (very low certainty of evidence).
  • PEN reduced risk of relapse (very low certainty of evidence), whereas reduction of red meat or refined carbohydrates did not (low certainty of evidence).
  • For ulcerative colitis, diets were similar to controls (very low and low certainty of evidence).

My take: Most of the dietary treatments for IBD have low to very low certainty of evidence regarding their effectiveness. Dietary changes are very likely to be helpful but more studies with rigorous endpoints are still needed.

Related blog posts:

Gastroparesis is Frequently Misdiagnosed

D Cangemi, L Stephens, BE Lacy. Clin Gastroenterol Hepatol 2023; 2670-2672. Misdiagnosis of Gastroparesis is Common: A Retrospective Review of Patients Referred to a Tertiary Gastroenterology Practice

In this retrospective study with adult patients (n=339) referred specifically to a tertiary center for evaluation of gastroparesis (GP) from 2019-2021, the key findings:

  • Nausea was most common symptom (in 89%), followed by abdominal pain (76%, constipation (71%), vomiting (66%), bloating (38%) and early satiety (35%)
  • 196 (58%) had undergone a prior gastric emptying study; though only 23 (7%) had ingested radiolabeled eggs as the test meal.
  • 66 (19.5%) ultimately received a diagnosis of GP; 80.5% received alterative diagnosis including functional dyspepsia in 44.5%. In those with GP, diabetes was more common (40% vs. 17%, P=.017).
  • GP patients more often had retained food in the stomach during EGD (23% vs. 11%, P=.013)

My take: In adults (& probably in children), most individuals labelled as having gastroparesis actually have an alternative explanation, usually functional dyspepsia. The symptoms are indistinguishable and improperly performed scintigraphy contributes to confusion.

Related blog posts:

Nelson Rocks, Circleville, WV

Getting over the Stigma of Medicines for Anxiety/Depression and Obesity

This is a terrific personal account of starting medications for anxiety/depression and obesity.

NY Times: Aaron Carroll 9/9/23, What Obesity Drugs and Antidepressants Have in Common

An excerpt:

Until a few years ago, I had controlled my depression and anxiety through decades of counseling. I was reluctant to try medications because the medical understanding of them seemed vague…We also can’t explain why some people benefit from S.S.R.I.s and others do not. Because of this, many people still believe those who take them don’t really need them. I also believed that, if I was strong enough, I didn’t need medication…

I was wrong to doubt. It’s had a remarkable effect on my mood, and almost everyone around me noticed the difference. I was more optimistic, friendlier and more engaging. I was forced to reconsider why I had avoided taking the medication for so long. I think it’s because — even though I realize this isn’t true — taking it felt like an admission of failure…

I’ve recently faced a similar scenario with new drugs for obesity. I’ve struggled with my weight for most of my life. I’ve always been overweight, and in the last few years, I’ve slipped into obesity, according to my body mass index. I exercise regularly and carry the weight well, but it bothers me immensely. It especially troubles me because I have a fair amount of self-discipline and eat quite healthfully

Despite all the advances in science, we don’t know why some people, even when they try desperately, can’t seem to lose weight. Because of that, we often assume it must be a lack of willpower…

These drugs are expensive, but I was determined to see what would happen if I took one. It is hard to explain what life is like on this medication to people who don’t have trouble controlling their weight. I’m not hungry all the time. I’m not thinking about food incessantly. I’m not obsessing about what I wish I could eat and what I can’t. My mental health, and even my temperament, improved so much that my whole family rejoiced…

Before writing this essay, I had told just a few people I’m on the drug. I think it’s because, on some level, I still feel shame. I felt the same when I finally started taking an antidepressant…

Medical treatments should not be dismissed just because we don’t fully grasp their mechanisms; people who use them are not cheating.

Related blog posts:

Photos from Washington DC

Timing of Cleft Palate Surgery

C Gamble et al. NEJM 2023; 389:795-807. Timing of Primary Surgery for Cleft Palate

Conclusion from study authors: Medically fit infants who underwent primary surgery for isolated cleft palate in adequately resourced settings at 6 months of age were less likely to have velopharyngeal insufficiency at the age of 5 years than those who had surgery at 12 months of age.

The associated editorial (R Tse, O Jackson, N Engl J Med 2023; 389:857-858. Mind the Gap) notes that “over two thirds of infants who were screened were excluded because their cleft was part of a syndrome, they were deemed to be medically unfit for early surgery, or they had a cleft that was too wide.” Also, “the incidence of additional surgery to treat velopharyngeal insufficiency was greater in the 6-month group than in the 12-month group (30 procedures in 27 children vs. 17 procedures in 16 children), and speech assessments performed after the secondary procedures were used in the analysis. Thus, the trial evaluated the mixed effects of primary surgery and secondary surgery in some patients.” Finally, “the considerations with regard to early surgery include the greater technical complexity of the procedure and the greater risks associated with anesthesia (airway complications and potential neurodevelopmental sequelae). Early surgery may also contribute to midfacial growth restriction, which becomes apparent only later, in adolescence, and may require complex corrective jaw surgery.”

My take: Early cleft palate surgery is not best for all infants with this defect.

Related blog post: Skinny Babies with Cleft Lips and/or Cleft Palates

Risk Factors for Inflammatory Bowel Disease: Antibiotics (Part 2)

AB Jawad et al. JPGN 2023; 77: 366-372.Early Life Oral Antibiotics Are Associated With Pediatric-Onset Inflammatory Bowel Disease-A Nationwide Study

Key findings:

  • Oral antibiotic exposure during the first 5 years of life was associated with a higher risk of developing pIBD (HR = 1.33,  P <0.0001). The risk was also increased if patients had ≥4 antibiotic prescriptions compared to no antibiotics (HR = 1.33, P <0.0001).
  • Broad-spectrum antibiotics increased the risk of pIBD compared to narrow-spectrum antibiotics (HR = 1.29, P < 0.0001).
  • When stratified by IBD subtypes, only Crohn disease was significantly associated with exposure to antibiotics (HR = 1.37, P = 0.002).

My take: This study indicates that antibiotics (and/or serious infections) are associated with an increased the risk of pediatric Crohn’s disease but the absolute risk is very low. We still have a lot to learn about how environmental exposures, including diet, infections, antibiotics, and pollution, contribute to the increasing prevalence of inflammatory bowel disease.

Related blog posts:

Risk Factors for Inflammatory Bowel Disease: Ultra-Processed Food (Part 1)

N Narula et al. Clin Gastroenterol Hepatol 2023; 21: 2483-2495. Open Access! Food Processing and Risk of Inflammatory Bowel Disease: A Systematic Review and Meta-Analysis

JA Fitzpatrick et al. Clin Gastroenterol Hepatol 2023; 21: 2478-2480 (editorial). Open Access! Ultra-processed Foods and Risk of Crohn’s Disease: How Much is Too Much?

Figure 1 from editorial: The NOVA classification of food

A total of 1,068,425 participants were included (13,594,422 person-years) among 5 cohort studies published between 2020 and 2022. The average age of participants ranged from 43 to 56 years. Key findings:

  • Crohn’s disease:  During follow-up, 916 participants developed CD, and 1934 developed UC. There was an increased risk for development of CD for participants with higher consumption of ultra-processed foods compared with those with lower consumption (HR, 1.71; 95% CI, 1.37–2.14; I2 = 0%) and a lower risk of CD for participants with higher consumption of unprocessed/minimally processed foods compared with those with lower consumption (HR, 0.71; 95% CI, 0.53–0.94; I2 = 11%). 
  • Ulcerative colitis: There was no significant association between risk of UC and ultra-processed foods (HR, 1.17; 95% CI, 0.86–1.61; I2 = 74%) or unprocessed/minimally processed foods (HR, 0.84; 95% CI, 0.68–1.02; I2 = 0%).

The associated editorial by Fitzpatrick et al, notes that “there are plausible mechanisms that explain the associations of higher UPFs and development of CD, such as: (1) displacing the intake of minimally processed foods and subsequently reducing exposure to beneficial micronutrients, antioxidants, and phytochemicals; (2) driving overconsumption of total calories7; and (3) increasing exposure to non-nutritive food substances that have been implicated in the development of CD in pre-clinical studies…The notion is that a lower UPF intake is better, but a cutoff value remains elusive.”8

My take (borrowed from editorial): “the population studies have indicated that the extremes of UPF intake are related to risk of CD and that such associations are underpinned by plausible biological mechanisms, suggesting causality.”

Related blog posts:

How to Handle Gastric Intestinal Metaplasia

I Mansuri et al. JPGN 2023; 77: 332-338. Gastric Intestinal Metaplasia in Children: Natural History and Clinicopathological Correlation

This retrospective single-center study (2013-2019) identified 38 patients with gastric intestinal metaplasia (GM) with a mean age of 12.5 years.

Background: “Gastric intestinal metaplasia (GIM) is defined as the replacement of the normal gastric epithelium by intestinal-type epithelium. GIM is considered a preneoplastic lesion for gastric adenocarcinoma in adults and is found in 25% of Helicobacter pylori ( H pylori ) exposed adults.”

Key findings:

  • The prevalence was 0.53% based on 7104 patients who were 18 or younger who underwent EGDs
  • 2 cases of H pylori were identified; chronic gastritis was noted in 47%
  • Only a third of patients had f/u EGDs; none of these had progression to dysplasia. In fact, GIM was NOT identified in the majority at followup

In their discussion, the authors note that the AGA’s clinical practice guidelines for GIM in adults provides the following recommendation: “Routine surveillance endoscopy in patients with incidental detection of gastric intestinal metaplasia (GIM) is discouraged.”

Also, it is worthwhile to consider that many cases of GIM are likely overlooked given the often patchy distribution.

My take (borrowed from authors): GIM appears to be an incidental gastric biopsy finding of uncertain significance. Unless there are high risk factors for gastric cancer, routine followup is unlikely to be beneficial.

Pictures from Villa Ephrussi de Rothschild:

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