Henoch-Schonlein purpura from vasculitis to intestinal perforation: A case report and literature review

Henoch-Sch?nlein purpura(HSP) is generally a selflimited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Imaging modalities, including ultrasonography and contrastenhanced computed tomography, could not detect intestinal ischemia prior to perforation. In this patient, we also postulated that vasculitis-induced mucosal ischemia was a cause of the ulcer, leading to intestinal perforation, and high-dose corticosteroid could have been a contributing factor since the histopathology revealed depletion of lymphoid follicles. Intestinal perforation in HSP is rare, but life-threatening. Close monitoring and thorough clinical evaluation are essential to detect bowel ischemia before perforation, particularly in HSP patients who have hematochezia, persistent localized abdominal tenderness and guarding. In highly suspicious cases, exploratory laparotomy may be needed for the definite diagnosis and prevention of further complications.

Novel ABCB11 mutations in a Thai infant with progressive familial intrahepatic cholestasis

Progressive familial intrahepatic cholestasis(PFIC) type 2 is caused by mutations in ABCB11,which encodes bile salt export pump(BSEP).We report a Thai female infant who presented with progressive cholestatic jaundice since 1 mo of age,with normal serumγ-glutamyltransferase.Immunohistochemical staining of the liver did not demonstrate BSEP along the canaliculi,while multidrug resistance protein 3 was expressed adequately.Novel mutations in ABCB11,a four-nucleotide deletion in exon 3,c.90_93delGAAA,and a single-nucleotide insertion in exon 5,c.249_250insT, were identified,with confirmation in her parents. These mutations were predicted to lead to synthesis of truncated forms of BSEP.Immunostaining and mutation analysis thus established the diagnosis of PFIC type 2.

Abnormal layering of muscularis propria as a cause of chronic intestinal pseudo-obstruction:A case report and literature review

Visceral myopathy is one of the causes of chronic intestinal pseudo-obstruction. Most cases pathologically reveal degenerative changes of myocytes or muscularis propia atrophy and fibrosis. Abnormal layering of muscularis propria is extremely rare. We report a case of a 9-mo-old Thai male baby who presented with chronic intestinal pseudo-obstruction. Histologic findings showed abnormal layering of small intestinal muscularis propria with an additional oblique layer and aberrant muscularization in serosa. The patient also had a short small bowel without malrotation, brachydactyly,and absence of the 2nd to 4th middle phalanges of both hands. The patient was treated with cisapride and combined parenteral and enteral nutritional support.He had gradual clinical improvement and gained body weight. Subsequently, the parenteral nutrition was discontinued. The previously reported cases are reviewed and discussed.

Epidemiological characteristics of Asian children with inflammatory bowel disease at diagnosis: Insights from an Asian-Pacific multicentre registry network

BACKGROUND There remains a dearth of Asian epidemiological literature for paediatric inflammatory bowel disease(PIBD).AIM To describe the presenting features of PIBD from 7 Asia-Pacific pediatric gastroenterology centers via a central standardised electronic data platform.METHODS Clinical,endoscopic and radiologic data at diagnosis from the registry were extracted between 1^(st) January 1995 to 31^(st) December 2019.Disease phenotypic characteristics were classified as per the Paris classification system.RESULTS There was a distinct rise in new PIBD cases:Nearly half(48.6%)of the cohort was diagnosed in the most recent 5 years(2015-2019).The ratio of Crohn’s disease(CD):Ulcerative colitis(UC):IBDUnclassified was 55.9%:38.3%:5.8%.The mean age was 9.07 years with a high proportion of very early onset IBD(VEO-IBD)(29.3%)and EO-IBD(52.7%).An over-representation of the Indian/South Asian ethnic group was observed which accounted for 37.0% of the overall Singapore/Malaysia subcohort(6.8%-9.0%Indians in census).Indian/South Asian CD patients were also most likely to present with symptomatic perianal disease(P=0.003).CD patients presented with significantly more constitutional symptoms(fever,anorexia,malaise/fatigue and muscle-wasting)than UC and higher inflammatory indices(higher C-reactive protein and lower albumin levels).CONCLUSION We observed a high incidence of VEO-IBD and an over-representation of the Indian ethnicity.South Asian CD patients were more likely to have symptomatic perianal disease.