Development of Primary Sclerosing Cholangitis in Pediatric Patients with Inflammatory Bowel Disease

A recent study (A Chandrakumar et al. J Pediatr 2019; 215: 144-51) followed 190 children with inflammatory bowel disease from 2011 to 2018 in a longitudinal population-based cohort in Manitoba and examined the development of primary sclerosing cholangitis (PSC).  The diagnosis of PSC was made on discretion of the treating physician; thus, only a subset of patients underwent extensive evaluations for PSC.

Key findings:

  • 9 developed PSC-UC (9/95) and overall 11 developed PSC-IBD (11/190)
  • Among children with PSC-UC, 8 had high GGT (>50) at baseline and only 1 had a normal GGT at baseline.
  • All UC patients who developed PSC were diagnosed withing 6 months of their UC diagnosis.
  • At baseline, 22 patients with UC had an elevated GGT and 73 had a normal GGT.  Thus, about one-third of patients with an elevated GGT developed PSC (possibly more as all patients were not subjected to extensive testing)

My view: This study reinforces two concepts: 1) GGT is valuable as a screening test 2) PSC (often asymptomatic) is fairly common in UC and needs to be considered especially in the first year of diagnosis.  What this study does not do is help us figure out what should be done about children with asymptomatic PSC as there are no proven therapies.

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Do You Need Separate Cookware for Celiac Disease?

Maybe.  A recent abstract at 2019 NASPGHAN meeting addressing this issue was highlighted in Gastroenterology and Endoscopy News.

Link: Recommendations for Children With Celiac Disease Need Update

An excerpt:

In two related experiments, researchers from the celiac disease program at Children’s National Medical Center in Washington D.C., looked at whether, and how much, gluten could be transferred from contaminated cafeteria foods and school supplies to children’s hands, work tables and gluten-free food (abstract 656). The researchers also analyzed how effective different washing methods were at removing gluten contamination…

Ms. Weisbrod said she and her colleagues were surprised that using a shared toaster for both gluten-free and gluten-containing bread transferred minimal gluten (<5 parts per million [ppm] in most samples), as did playing with Play-Doh (median, 1.25 ppm). Both exposures were well below the 20-ppm threshold the FDA uses to consider an item gluten-free.

My take: The NASPGHAN meeting also featured a lecture by Alessio Fasano indicating that ~30%of patients with celiac disease had persistent disease due to poor adherence with a gluten-free diet and about 10% of patients with celiac disease are exquisitely sensitive to gluten.  So, while this small study indicates that gluten exposure may be lower than gluten threshold in many cases when sharing toasters, etc, I think more attention should be directed at strict gluten avoidance rather than trying to discern if some level of cross contamination may be acceptable.

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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

P’tit Train du Nord Linear Park (near Montreal) -124 Miles

 

Bad Fatty Liver Disease Can Get Worse Quickly

A recent study (AJ Sanyal et al. Hepatology 2019; 70: 1913-27) used prospectively collected data from two large randomized, placebo-controlled phase 2b studies of simtuzumab in patients with either bridging fibrosis (n=217) or compensated cirrhosis (n=258) due to nonalcoholic fatty liver disease (NAFLD). The age range of participants were 48-61 years with median ages of 55 years and 57 years for the two cohorts.  All patients had liver biopsies at screening and at weeks 48 and 96.

Key findings:

  • Progression to cirrhosis occurred in 22% (48/217) of patients with bridging fibrosis (F3) over a median of 29 months
  • Liver-related adverse clinical events (eg. ascites, variceal bleeding, encephalopathy, MELD score ≥15, liver transplantation or death) occurred in 19% (50/258) with compensated cirrhosis over a median of 29 months. Only 1 patient in this cohort died.
  • Higher  baseline hepatic fibrosis or serum markers of fibrosis were associated with disease progression in patients with F3 disease

My take: Among those with advanced liver disease, this study indicates that disease progression/deterioration is rather rapid in about 20%.

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A Little More Data on Antibiotic Cocktail for Pediatric Acute Severe Ulcerative Colitis

A recent prospective study (D Turner et al. Inflammatory Bowel Diseases, izz298, https://doi.org/10.1093/ibd/izz298) with 28 children found improvement in 5-day PUCAI scores in patients who received quadruple antibiotics in combination with IV corticosteroids compared to those who received IV corticosteroids alone.

Link: Antibiotic Cocktail for Pediatric Acute Severe Colitis and the Microbiome: The PRASCO Randomized Controlled Trial

Methods:

Hospitalized children with ASC (pediatric ulcerative colitis activity index [PUCAI] ≥65) were randomized into 2 arms: the first received antibiotics in addition to IVCS (amoxicillin, vancomycin, metronidazole, doxycycline/ciprofloxacin [IVCS+AB]), whereas the other received only IVCS for 14 days. The primary outcome was disease activity (PUCAI) at day 5. Microbiome was analyzed using 16S rRNA gene and metagenome.My t

Results

Twenty-eight children were included: 16 in the AB + IVCS arm and 12 in the IVCS arm (mean age 13.9 ± 4.1 years and 23 [82%] with extensive colitis). The mean day-5 PUCAI was 25 ± 16.7 vs 40.4 ± 20.4, respectively (P = 0.037). Only 3 and 2 children, respectively, required colectomy during 1-year follow-up (P = 0.89). Microbiome data at time of admission were analyzed for 25 children, of whom 17 (68%) had a predominant bacterial species (>33% abundance); response was not associated with the specific species, whereas decreased microbiome

My take: Combination antibiotic therapy appears to improve disease activity in children with acute severe ulcerative colitis.  More and larger studies are needed to determine whether this is associated with improved long-term outcomes as well as which antimicrobials are optimal.

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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

Predicting Survival Without Disability Among Preterm Infants

A recent article (J Bourke et al. .J Pediatr 2019; 215: 90-7) made me wonder if my outlook on disability-free survival of preterm infants has been skewed by the population that I encounter.  That is, the outcomes from this large Australia study were better than I would have guessed.

This retrospective cohort study identified 720.091 live births from 1983-2010; in this group, 12,083 were diagnosed with a disability and 5,662 died. The authors sought to determine rates of intellectual disability or autism as identified by the IDEA (Intellectual Disability Exploring Answers) database.  Because this is a retrospective study, it did not capture milder and more common neurodevelopmental disorders like attention deficit hyperactivity disorder.

Key findings:

The probability of disability-free survival to 25 years was the following:

  • 4.1% for those born at 22 weeks gestation
  • 19.7% for those born at 23 weeks gestation
  • 42.4% for those born at 24 weeks gestation
  • 53.0% for those born at 25 weeks gestation
  • 78.3% for those born at 28 weeks gestation
  • 97.2% for those born full term (39-41 weeks)

Risk factors for lower rates of disability-free survival:

  • Aboriginal population (instead of Caucasian), low Apgar score, male sex, low socioeconomic status, and remote region of residence

My take: This data shows the marked improvement in outcomes with longer gestation age.