Multicenter registry of pediatric inflammatory bowel disease from a developing country.

BACKGROUND: Despite the rising incidence of pediatric inflammatory bowel disease (PIBD) globally, multicenter collaborative studies of PIBD children among developing countries remain sparse. We therefore aimed to define the initial presentation and short-term outcomes of Thai children with PIBD from a multicenter registry. METHODS: Four teaching hospitals participated in this study. A diagnosis of PIBD requires gastrointestinal endoscopy and histopathology in children aged < 19 years. Besides demographics, we collected clinical information and treatment with the data at 1-year follow up. RESULTS: We included 35 Crohn's disease (CD), one IBD-unclassified, and 36 ulcerative colitis (UC) children (total n = 72 with 60.6% males). The mean age at diagnosis was 7.9 years (SD 4.1) with 38% being very early onset IBD (VEO-IBD). When compared with UC, the CD children were more likely to exhibit fever (42.3 vs. 13.9%), weight loss/failure to thrive (68.6 vs. 33.3%), and hypoalbuminemia (62.9 vs. 36.1%) but less likely to have bloody stools (51.4 vs. 91.7%) (all P < 0.05). No significant differences in demographics, clinical data and medications used with regards to VEO-IBD status. At 1 year after diagnosis (n = 62), 30.7% failed to enter clinical remission and 43.7% remained on systemic corticosteroids. Diarrhea (OR 9.32) and weight issues (OR 4.92) at presentation were independent predictors of failure to enter clinical remission; and females (OR 3.08) and CD (vs. UC) (OR 3.03) were predictors of corticosteroids use at 1-year follow-up. CONCLUSIONS: A high proportion of VEOIBD is noted, and CD was more likely to present with significant inflammatory burden. Diarrhea and weight issues at presentation were independent predictors of failure to enter clinical remission; and females and CD (vs. UC) were predictors of corticosteroids use at 1-year follow-up.

Approach to Congenital Diarrhea and Enteropathies (CODEs).

Congenital diarrhea and enteropathies (CODEs) constitute a group of rare genetic disorders characterized by severe diarrhea and malabsorption in the neonatal period or early infancy. Timely diagnosis and treatment is essential to prevent life-threatening complications, including dehydration, electrolyte imbalance, and malnutrition. This review offers a simplified approach to the diagnosis of CODEs, with a specific focus on microvillus inclusion disease (MVID), congenital tufting enteropathy (CTE), congenital chloride diarrhea (CLD), and congenital sodium diarrhea (CSD). Patients with CODEs typically present with severe watery or occasionally bloody diarrhea, steatorrhea, dehydration, poor growth, and developmental delay. Therefore, it is crucial to thoroughly evaluate infants with diarrhea to rule out infectious, allergic, or anatomical causes before considering CODEs as the underlying etiology. Diagnostic investigations for CODEs encompass various modalities, including stool tests, blood tests, immunological studies, endoscopy and biopsies for histology and electron microscopy, and next-generation sequencing (NGS). NGS plays a pivotal role in identifying the genetic mutations responsible for CODEs. Treatment options for CODEs are limited, often relying on total parenteral nutrition for hydration and nutritional support. In severe cases, intestinal transplantation may be considered. The long-term prognosis varies among specific CODEs, with some patients experiencing ongoing intestinal failure and associated complications. In conclusion, the early recognition and accurate diagnosis of CODEs are of paramount importance for implementing appropriate management strategies. Further research and advancements in genetic testing hold promise for enhancing diagnostic accuracy and exploring potential targeted therapies for these rare genetic disorders.

Pancreaticopleural fistula in a child with chronic pancreatitis harboring compound SPINK1 variants.

BACKGROUND: Pancreaticopleural fistula (PPF) is a rare complication of chronic pancreatitis (CP) that requires a high index of clinical suspicion in the patient who presents with a pleural effusion. Visualizing the fistula tract from the pancreatic duct to the pleural space by radiological imaging provides confirmation of this complication. CASE PRESENTATION: A 9-year-old boy who presented with massive right pleural effusion secondary to PPF, a complication of CP from a genetic mutation involving two mutations of SPINK1. We successfully managed the case with by endoscopic pancreatic duct stent placement after failure of conservative treatment approaches. CONCLUSIONS: PPF is a rare but serious complication of CP in all ages. The diagnosis of PPF in children requires a high index of clinical suspicion and should be considered in the differential diagnosis of massive pleural effusion where pancreatic pathology is present. A high level of pleural fluid amylase and the results from radiological imaging when the patients have symptoms play essential roles in the diagnosis of PPF. Currently, Magnetic resonance cholangiopancreatigraphy (MRCP) is the imaging modality of choice. Endoscopic therapy and surgery are treatment options for patients who do not respond to conservative therapy.

Clinical Characteristics of Allergy to Hymenoptera Stings.

OBJECTIVE: The purpose of this study was to evaluate the clinical characteristics of allergy to stings from the Hymenoptera order of insects in a hospital in Thailand. METHODS: A descriptive retrospective analytical study was carried out in inpatients and outpatients suffering from Hymenoptera stings from 2009 to 2013 in Siriraj Hospital. RESULTS: Medical records of 386 patients with an allergy to Hymenoptera stings were evaluated. Anaphylaxis was more common in patients younger than 15 years, who comprised 22.3% of patients in this study. The most common sting type was from wasps (58.3%). Eighty-five percent of patients sought medical treatment less than 24 hours after being stung. A systemic reaction and anaphylaxis were found in 9.9% and 4.4% of subjects, respectively. In 17 patients with anaphylaxis, only 11 patients (64.7%) received an epinephrine (adrenaline) injection as treatment, and only 8 (47.1%) received epinephrine autoinjectors or epinephrine-prefilled syringes to prevent a possible subsequent severe reaction. Significantly more patients younger than 15 years received epinephrine for prevention of an allergic reaction than did those older than 15 years (87.5% vs 11.7%, P < 0.001). Antibiotics were given to 43.0% of patients. CONCLUSIONS: Anaphylaxis from Hymenoptera stings was more common in children than in adults. Only half of the patients visited the emergency room within 1 hour of being stung. Overuse of antibiotics and underuse of epinephrine were found. More information about Hymenoptera stings should be provided to the public, and the use of epinephrine should be encouraged in the case of severe reactions and anaphylaxis.

A rare cause of multiple small bowel ulcers and strictures in a 10-year-old child.

Enteritis and small bowel ulcers can be caused by inflammatory bowel disease, drug-induced enteritis, cytomegalovirus, tuberculosis, or intestinal lymphoma. Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is an uncommon idiopathic cause of ulceration and stricture of the small bowel. CMUSE can occur in adults, but only few pediatric cases have been reported. Inflammatory bowel disease and other causes should be carefully sought first before the diagnosis of CMUSE can be made. Previous reports demonstrated that surgical intervention may be necessary for both diagnostic and therapeutic purposes. With regard to the management, systemic corticosteroids may help, and surgery plays a role in patients present with signs of intestinal obstruction. We report a young girl who presented with a prolonged history of refractory iron deficiency anemia with protein-losing enteropathy without other obvious gastrointestinal symptoms. She underwent several laboratory and endoscopic investigations as well as histopathology of the resected full-thickness small bowel area before a proposed diagnosis of CMUSE was made. A trial of immunosuppression (both prednisolone and azathioprine) was initiated that provided a relatively satisfactory result.