JPGN Journal Club: May 2025

Hello! The JPGN Journal Club, led by Dr. Jake Mann, is back — in your speakers, your earbuds, or maybe even over the airport tannoy… well, probably not the last one. Anyway, it's good to be back in touch.

Please visit https://www.espghan.org/knowledge-center to explore current offerings, and don't forget ESPGHAN’s annual meeting, taking place May 14–17 in Helsinki.

This session’s discussion papers:

1.From Journal of Pediatric Gastroenterology and Nutrition (JPGN):Ritchie et al. (Aberdeen, UK, and Christchurch, NZ) –"Role of Noncontrast-Enhanced Abdominal Ultrasound in the Diagnostic Assessment of Pediatric Inflammatory Bowel Disease"

A retrospective review of 47 children in New Zealand with inflammatory bowel disease (IBD).

Examined the contributions of endoscopy, biopsy, magnetic resonance enterography (MRE), and noncontrast sonography (NCS) in diagnosing small-bowel involvement.

Findings:

14 children had no small-bowel disease.

Of the 33 with small-bowel involvement:

19 had findings on NCS.

In 7 cases, NCS detected disease that endoscopy, biopsy, and MRE missed.

In 12 cases, NCS confirmed disease found by other modalities and found additional sites of involvement.

In 14 cases, NCS failed to detect small-bowel disease that other methods identified.

Conclusion:NCS alone missed about two-fifths of cases but expanded diagnosis in about one-fifth.

2. From Nature:Rosenberger et al. (Czechia, Denmark, Germany) –"Deep Visual Proteomics Maps Proteotoxicity in a Genetic Liver Disease"

Focused on alpha-1-antitrypsin storage disorder (A1ASD), where mutations in SERPINA1 cause alpha-1-antitrypsin (A1A) to accumulate in liver cells.

Mechanisms of injury ("proteotoxicity") were unclear.

The study used staining, microdissection, and mass spectrometry to analyze individual hepatocytes.

Key findings:

Upregulation of the unfolded protein response.

Downregulation of hepatocellular synthesis and secretion processes.

Strong activation of peroxisomes in samples from individuals with less fibrosis; this activation declined in cases with more fibrosis.

Implication:The authors speculate that peroxisome activators might help modulate disease progression.

Limitations:The study focused on adult samples (ages 40–80), leaving unanswered how A1ASD leads to cholestasis in some infants but not others.

References

Ritchie K, et al. Role of Noncontrast-Enhanced Abdominal Ultrasound in the Diagnostic Assessment of Pediatric Inflammatory Bowel Disease. JPGN, 2025 Apr 9.PMID: 40201985 | DOI: 10.1002/jpn3.70044

Rosenberger FA, et al. Deep Visual Proteomics Maps Proteotoxicity in a Genetic Liver Disease. Nature, 2025 Apr 16.PMID: 40240610 | DOI: 10.1038/s41586-025-08885-4

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