Hepatobiliary Complications in Children with Sickle Cell Disease: A Retrospective Review of Medical Records from 616 Patients.

Hepatobiliary complications in children with sickle cell disease (SCD) are rarely reported but can be life-threatening. We retrospectively assessed their prevalence in a cohort of 616 children followed in a French university-hospital SCD reference center. Eligibility criteria were the following: age <18 years, seen at least twice with an interval of more than 6 months from January 2008 to December 2017, with all genotypes of SCD. Patients with hepatobiliary complications were identified via the local data warehouse and medical files were thoroughly reviewed. At least one hepatobiliary complication was reported in 37% of the children. The most frequent was cholelithiasis, in 25% of cases, which led to systematic screening and elective cholecystectomy in the case of gallstones. Overall, 6% of the children experienced acute sickle cell hepatic crisis, sickle cell intra-hepatic cholestasis, or acute hepatic sequestration, with severity ranging from mild liver pain and increased jaundice to multiple organ failure and death. Emergency treatment was exchange transfusion, which led to normalization of liver tests in most cases. Five children had chronic cholangiopathy, associated with auto-immune hepatitis in two cases. One needed liver transplantation, having a good outcome but with many complications. Transfusion iron load and infectious hepatitis cases were mild. Hepatotoxicity of an iron chelator was suspected to contribute to abnormal liver test results in five patients. We propose recommendations to prevent, explore, and treat hepatobiliary complications in SCD children. We underline the need for emergency exchange transfusion when acute liver failure develops and warn against liver biopsy and transplantation in this condition.

Endoscopic ultrasound in pediatric population: a comprehensive review of the literature.

BACKGROUND AND AIM: Endoscopic ultrasonography (EUS) with or without fine needle aspiration/biopsy (FNA/B) is a well-established diagnostic tool in adults for the evaluation and management of gastrointestinal (GI) tract disorders. Its use in children is still limited as well as literature in pediatric age is limited, although the application of EUS is now increasing. The present article aims to review the current literature about EUS indication, accuracy and safety in pediatric age. METHODS: Electronic literature searches were conducted using Pubmed, Medline, Embase, and the Cochrane Central Register of Controlled Trials using the word pediatric endoscopic ultrasound, pediatric pancreaticobiliary AND/OR EUS, pediatric EUS technique. Main patients and procedures characteristics were analyzed. The primary endpoint was the indication of EUS. Secondary endpoints were the accuracy of the technique and the incidence of complications. RESULTS: Data were extracted from 19 articles. A total of 571 patients were investigated, with a median age of 12,7 years. A total of 634 EUS procedures were performed. The majority of EUS procedures investigated the pancreaticobiliary tract (77,7%). Most studies showed a high positive impact on management with a median value of 81,7%. No major complications were reported. Five studies reported minor complications with a median value of 2%. CONCLUSIONS: EUS is safe and has a significant role in the diagnosis of pancreaticobiliary and GI diseases even in children, with a high therapeutic success. An increasing EUS utilization by pediatric gastroenterologists is expected and offering dedicated EUS training to some selected pediatric gastroenterologists might be indicated.

Endoscopic dilation in pediatric esophageal strictures: a literature review.

BACKGROUND: Esophageal strictures in pediatric age are a quite common condition due to different etiologies. Esophageal strictures can be divided in congenital, acquired and functional. Clinical manifestations are similar and when symptoms arise, endoscopic dilation is the treatment of choice. Our aim was to consider the efficacy of this technique in pediatric population, through a wide review of the literature. METHOD: A search on PubMed/Medline was performed using "esophageal strictures", "endoscopic dilations" and "children" as key words. Medline, Scopus, PubMed publisher and Google Scholar were searched as well. As inclusion criteria, we selected clinical studies describing dilations applied to all type of esophageal strictures in children. Papers referred to single etiology strictures dilations or to adult population only were excluded, as well as literature-review articles. RESULTS: We found 17 studies from 1989 to 2018. Overall, 738 patients in pediatric age underwent dilation for esophageal strictures with fixed diameter push-type dilators (bougie dilators) and/or radial expanding balloon dilators. Severe complications were observed in 33/738 patients (4,5%) and perforation was the most frequent (29/33). Conversion to surgery occurred only in 16 patients (2,2%). CONCLUSIONS: Endoscopic dilation is the first-choice treatment of esophageal strictures, it can be considered a safe procedure in pediatric age. Both, fixed diameter push-type dilators and radial expanding balloon dilators, showed positive outcomes in term of clinical results and cases converted to surgery. However, it's essential to perform these procedure in specialized Centers by an experienced team, in order to reduce complications.