IMPE Abstracts

Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil Congenital hyperinsulinism (CH) causes severe hypoglycemia through inappropriate insulin secretion, with high rates of morbidity and mortality. Conservative treatment may not be sufficient for glycemic control and surgical treatment is necessary. HCRP has a treatment center for Congenital Hyperinsulinism. The purpose of this study was to review the clinical, laboratory and molecular characteristics of patients undergoing surgery for the treatment of CH. A retrospective cross-sectional study of data from medical records was carried out, with the rescue of the following: age at onset, place of origin, type of clinical treatment, short and long-term hypoglycemia resolution, complications, type of surgery performed. From January 2007 to July 2022, 19 patients had pancreatectomy due to no response medical treatment. From these patients, 63% were female. The patients came from almost all states of Brazil, São Paulo, Brasilia, Es Pírito Santo, Goiás, Pernambuco, Minas Gerais and Rio Grande do Sul. Beginning of the symptoms, range from the 2nd hour of life to 28 days of life. In two cases, only a focal lesion was removed. Two cases required second surgery. From the 19 cases operated 6 were by open surgery, one of which was initially performed by videolaparoscopy converted to open due to intraoperative complications. The genetic study found mutations in the ABCC8 and KCNJ11 gene in 36% of the operated patients. During clinical treatment, all patients received EV glucose, 81% used corticosteroids, diazoxide and thiazide was used in 77% of cases, Octreotide in 72%, Nifedipine was used in 27%, Glucagon in 4 patients. Sirulimus was used in 18% of patients. In 91% of cases, gastrostomy or tube feeding was used. The oral route was used in 9% of cases. All patients were cured, with no death record. 18% developed diabetes mellitus just after pancreatectomy and another 36% remained with hypoglycemia that improved in the late postoperative period. 54% of patients developed exocrine pancreatic insufficiency, requiring pancreatic enzyme replacement. 54% of the patients had some degree of development delay. The laboratory and genetic profile showed similar results to other reference centers. Regarding preoperative treatment, glucocorticoid was frequently used. Although diazoxide is not commercialized in our country, it was used, followed by Octreotide. Surgical treatment was successful in 100% of cases. The most common surgery was laparoscopic subtotal pancreatectomy. The most common complications were exocrine pancreatic insufficiency (54%) and delayed neuropsychomotor development (54%).