Basidiobolomycosis is a rare fungal disease caused by Basidiobolus ranarum. Involvement of the gastrointestinal tract is unusual and poses both a diagnostic and therapeutic challenge, as clinical signs are non-specific and predisposing risk factors are lacking. It can mimick inflammatory bowel disease, primary immunodeficiency, or a malignancy and should be considered in patients who do not respond to standard therapy. We present the case of a 22 months old boy with confirmed colonic Basidiobolomycosis, who presented with severe eosinophilic inflammation of the gastrointestinal tract. Panfungal PCR performed on DNA extracted directly from a tissue sample confirmed the presence of Basidiobolus. He made a full recovery with a combination of surgery and prolonged targeted antifungal medication.

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Objectives and Study: Congenital chloride diarrhea (CCD) is a rare, autosomal recessive disorder caused by mutations in the SLC26A3 gene encoding a transmembrane chloride/bicarbonate ion exchanger mainly expressed in the apical brush border of the ileal and colonic epithelium. Lifelong, secretory, chloride-rich diarrhea and hypochloremic, hypokalemic metabolic alkalosis are characteristic. Histological evidence of bowel inflammation is not typically described in CCD and has only been reported in a few patients. Methods: We report four cases of CCD who received adequate resuscitation with appropriate replacement of their fecal salt and water losses. Three had associated inflammatory bowel changes at endoscopy. The index case of CCD who developed frankly bloodstained diarrhea aged 7 months was found to have histologically confirmed colitis at endoscopy. An electronic search of the hospital database to identify all patients with confirmed CCD was performed. A further three children underwent de novo diagnostic evaluation and treatment. A retrospective case note review was undertaken to determine the incidence and subtype of inflammatory bowel disease (IBD) by clinical, endoscopic, and histological means. Results: Four children with genetically confirmed CCD were identified, two being female. The first girl had a granulomatous colitis with ulceration. She went into remission with a combination of steroids and azathioprine. Immunosuppression was subsequently discontinued without a further flare of colitis. A second girl was found to have patchy inflammatory changes in the small bowel and focal active colitis. A third patient, a boy, demonstrated mild inflammatory changes in the small bowel with apoptotic debris and mild inflammation in the colon. A fourth patient did not develop intestinal inflammation. Conclusion: Our case series highlights the potential association of CCD with panenteric inflammation. While our cohort was small, CCD is rare and three out of four children referred to our tertiary referral center were affected. While early diagnosis and adequate salt replacement therapy are crucial in CCD management, the clinician should also be aware of bowel inflammation as a potential cause of failure of CCD therapy to control bowel symptomatology. Further insight is needed to understand the underlying patho-mechanism giving rise to bowel inflammation in this group.

Background Wireless Video Capsule Endoscopy (VCE), Magnetic resonance enterography (MRE) and Small Bowel ultrasound (SBUS) are well-established diagnostic tools, used in the evaluation of small bowel disease in paediatric inflammatory bowel disease (IBD) patients.The aim of this study was to compare VCE findings with those of MRE and SBUS and evaluate discrepancies between them. Methods VCE examinations were conducted in histologically confirmed paediatric IBD patients in a period of 19 months (March 2018 – November 2019) in a tertiary center. The VCE findings were retrospectively compared to relevant findings on MRE and SBUS, collected from electronic data records. Results 34 patients were included in the study (16 males,18 females) with an age range at the moment of diagnostic assessment between 4–17 years (median 12 years). 21 patients were diagnosed with Crohn disease (CD), 9 patients with Ulcerative Colitis (UC) and 4 patients with IBD Unclassified (IBD-U). 8/34 (23.5%) patients were found to have all three diagnostic investigations, 21/34 (61.8%) had MRE and 21/34 (61.8%) had SBUS. Concordance between the three modalities were seen in 5/8 (62.5%) patients. Of these, 3/5 (60%) had no small bowel disease identified on VCE, MRE and SBUS, while in 2/5 patients (40%) the same distribution of small bowel disease was identified using all three diagnostic modalities. When comparing VCE to MRE, differing distributions of disease were seen in 10/21 patients (47.6%). 6/10 (60%) had terminal ileitis, 2/10 (20%) had ceacal disease and 3/10 (30%) jejunal disease noted on MRE, but with no disease identified on VCE. 1/10 patient (10%) had a normal MRE but duodenal and proximal jejunal ulceration was reported on VCE. VCE and SBUS had a higher concordance (71.4%) in identifying disease distribution. In 6/21 patients (28.5%) there was a discrepancy in the findings reported on SBUS when compared to VCE.Of these, 4/6 (66.7%) had a normal SBUS but small bowel disease (jejunal and ileal disease) on VCE.1/6 patient (16.7%) had caecal inflammation and 1/6 (16.7%) had active terminal ileitis on SBUS, which was not reported on their VCE. Conclusion The identification of small bowel disease is essential in the diagnosis and subsequent management of paediatric IBD. VCE, MRE and SBUS are established modalities to detect intestinal disease. This study demonstrates that performing all three diagnostic modalities may have important practical significance and increase diagnostic yield in detecting small bowel inflammatory bowel disease.

manifestations of infection such as fever, rigors, and/or hypotension and a positive blood cultures obtained via CVC in the absence of other potential sources of infection. CVC were removed if severe, potentially life-threatening symptoms occurred. The incidence of CRBSI was measured as number of catheter-related episodes per 1000/catheter days. Results A total of 58 children (26 male, aged 7.2±4.6 years) were reviewed. The indications for PN were motility disorder in 44.8%, short bowel syndrome in 36.2% and enteropathy in 19%. The catheters used were single-lumen tunneled Hickmann (82/108), double-lumen (26/108), peripheral inserted central catheter (2/108) and Broviac (1/108). Thirty-one of 58 (53.4%; 15 M, aged 5.8±4.3 years) children developed 108 CRBSIs over the study period. The median (range) number of CRBSI episodes per patient was 1 (0-14). The overall catheter days was 58414 and the CRBSI rate was 1.85/1000 catheter days. Only 23 (21.3%) catheters were removed because of lifethreatening symptoms and 85 (78.7%) of catheters were salvaged and retained despite CRBSI. By organism, 38% were gram positive, 34.2% gram negative, 21.2% polymicrobial and 6.5% fungal CRBSI. The most frequent gram positive and negative organism was Staphylococcus aureus (31.7%) and Klebsiella species (43.2%) respectively. Catheter infected with gram positive bacteria showed the highest rate of CVC salvage (gram positive 92.7%, 78.2% polymicrobial, 67.6% gram negative, 57.1% fungal infection; P<0.05).The CRBSI rate for double-lumen catheters was significantly greater than single-lumen catheters (24.1% vs 4.8%; P<0.0001). Patients with a double-lumen CVC were found to be at increased risk for CRBSI development (HR 2.51;]; P <0.01). Conclusion CVC is possible in more than three-quarters of CRBSIs in children on long-term home PN for IF. Successful salvage may depend on the species isolated. CRBSIs caused by gram positive bacteria, the most bacteria causing CRBSI, had a CVC salvage rate approaching 93%. Effective antibiotic treatment without removal of the CVC should be considered as first line treatment. A single-lumen CVC should be the catheter of first choice. Further studies to identify predictive factors of catheter removal after CRBSI are required.