Background: Malnutrition has a high incidence among pediatric surgical patients and contributes to increased risks of postoperative complications and extended hospital stays.Purpose: The present study aimed to determine the influence of preoperative nutritional status on the postoperative outcomes of pediatric patients who underwent elective gastrointestinal (GI) surgery.Methods: This prospective observational study was conducted at Cairo University Specialized Pediatric Hospital. According to the designated inclusion criteria, 75 surgical cases of both sexes were included. A structured questionnaire was developed and administered. This questionnaire included 3 main sections: demographic data and nutritional status parameters at admission and discharge. Pre- and postoperative nutritional statuses were compared.Results: According to both the subjective global nutritional assessment and STRONGKIDS score Questioner, more than 60% of patients in the upper GI patient group were at risk of malnutrition. Wasting status was most common in the upper GI patient group (67%; vs. 39.1% in the lower GI group). Underweight status was more common in the hepatobiliary and upper GI patient groups (nearly 50% for each group) than in the lower GI group (30.4%). On the other hand, stunted patients had a higher incidence of complications and a prolonged hospital stay (P=0.003 and P=0.037, respectively), while underweight lower GI patients experienced a prolonged hospital stay (P=0.02). A higher proportion of patients with preoperative anemia than those without preoperative anemia required a blood transfusion (P=0.003).Conclusion: Nutritional assessment is a crucial component of pediatric surgical patient management. Both underweight and wasting statuses were more common among hepatobiliary and upper GI patients. Postoperative complications and a long hospital stay were more common among stunted patients.

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Background The liver biopsy is an essential element of evaluating progression of liver disease in children with Progressive Familial Intrahepatic Cholestasis (PFIC) and Allagille Syndrome (AGS). Several noninvasive techniques, including radiological imaging and blood biomarkers assay, can be used to evaluate liver stiffness. Objectives To identify whether liver Transient elastography (FibroScan) and AST/PLT Ratio Index (APRI) could be reliable tools to assess the degree of fibrosis prior to partial biliary diversion (PBD). Methods A prospective cohort in which all patients with PFIC and AGS who underwent PBD from July 2019 to July 2021 were included. Preoperative liver functions, pelvic-abdominal ultrasonography and FibroScan assessments were performed while intraoperative liver biopsy was obtained. Results Eight patients with chronic cholestatic liver disease who were candidates for PBD due to intractable pruritus were enrolled, including PFIC (n = 6; 75%), and AGS (n = 2; 25%). The liver FibroScan results were similar to the liver biopsy histopathological assessment in 87.5% of cases. APRI ranged from 0.1 to 3.2 (median = 1.2). In four cases (50%), APRI was consistent with histological evaluation of liver samples. The FibroScan results were in concordance with APRI results in three patients (37.5%). Conclusion The current cohort demonstrated that fibroScan was consistent with histopathology in 87.5% of patients, highlighting its value in determining the degree of liver fibrosis prior to surgery, whereas the APRI was only consistent with histopathology in half of cases.

Purpose: Severe pruritus caused by progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AGS) is refractory to medical treatment. Surgical interruption of the enterohepatic circulation is considered the mainstay of alleviating distressing symptoms and delaying cirrhosis. This study aims to evaluate the short-term effect of partial external biliary diversion (PEBD) on pruritus, liver disease progression, patient’s growth, and quality of life.Methods: This prospective cohort study enrolled children with PFIC and AGS from July 2019 to July 2021, whose guardians consented to the PEBD procedure. A standard surgical approach was performed by a single surgeon. Outcomes were measured subjectively and objectively pre- and post-procedure using the pruritus 5-D itching score, Paediatric Quality of Life Inventory scale (PedsQL), growth parameters, bile acids level, and liver function tests. Patients’ follow-up period ranged from 6 to 12 months.Results: Seven patients had PEBD procedure; five with PFIC and two with AGS. A significant improvement was detected in the 5-D itching score (p-value < 0.001), PedsQL (p-value < 0.001), and bile acids level (p-value 0.013). The preexisting growth failure was ameliorated. The downward trend in the bilirubin level was not significant. No influential difference in the other liver function tests occurred. No intra-operative complications encountered. Only one case had a post-operative stoma prolapse which was managed surgically.Conclusion: PEBD procedure could be considered as an effective and safe treatment options for intractable pruritus in patients with PFIC or AGS, providing preserved synthetic liver functions.Research Ethics committee approval: MD110-2019 on 28/12/2019