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Collagenous colitis presenting with skip lesions mimicking Crohn's disease and complicated by intestinal obstruction

Hui CK, Hui NK

J Dig Dis, 2017 Aug;18(8):487-491.
PMID: 28682012 DOI: 10.1111/1751-2980.12507
Fulltext A Prospective Study on the Prevalence, Extent of Disease and Outcome of Eosinophilic Gastroenteritis in Patients Presenting with Lower Abdominal Symptoms

Hui CK, Hui NK

Gut Liver, 2018 May 15;12(3):288-296.
PMID: 29212311 DOI: 10.5009/gnl17056

Background/Aims: The epidemiology of eosinophilic gastroenteritis remains unclear. We aim to determine the prevalence of eosinophilic gastroenteritis in patients with lower abdominal symptoms.
Methods: In a prospective study, colonoscopy was performed on 2,469 consecutive patients. Biopsies were taken from the terminal ileum and ascending, transverse, descending and sigmoid colon in all patients.
Results: Sixty-four of the 2,469 patients (2.6%) had eosinophilic gastroenteritis. Only five of the 64 patients (7.8%) with eosinophilic gastroenteritis had endoscopic mucosal abnormalities during colonoscopy. Six of these 64 patients (9.4%) had severe disease at presentation, and seven of these 64 patients (10.9%) required systemic steroid treatment. An elevated absolute peripheral eosinophil count was independently associated with severe disease at presentation (4/6 [66.7%] vs 3/58 [5.2%], p=0.005; odds ratio [OR], 25.320; 95% confidence interval [CI], 2.628 to 243.910), and severe disease at the time of presentation was independently associated with the use of systemic steroid treatment (6/7 [85.7%] vs 0/57 [0%], p=0.008; OR, 18.021; 95% CI, 2.163 to 150.152).
Conclusions: The prevalence of eosinophilic gastroenteritis is common, and patients usually present normal-appearing mucosa on colonoscopy. Those with severe disease at presentation usually have a raised absolute peripheral eosinophil count and should be commenced on systemic steroids as an initial therapy.
Fulltext IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome

Trautmann A, Vivarelli M, Samuel S, Gipson D, Sinha A, Schaefer F, et al.

Pediatr Nephrol, 2020 Aug;35(8):1529-1561.
PMID: 32382828 DOI: 10.1007/s00467-020-04519-1

Idiopathic nephrotic syndrome newly affects 1-3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4-6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given.

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