Hyperinsulinemic hypoglycemia in adolescents: case report and systematic review.

BACKGROUND: Hyperinsulinemic hypoglycemia is the most common cause of severe and persistent hypoglycemia in neonates and children. It is a heterogeneous condition with dysregulated insulin secretion, which persists in the presence of low blood glucose levels. CASE PRESENTATION: We report a case of a 15 year-old male with hyperinsulinemic hypoglycemia, who underwent a subtotal pancreatectomy after inadequate response to medical therapy. Pathological examination was positive for nesidioblastosis (diffuse ß-cell hyperplasia by H-E and immunohistochemical techniques). The patient's blood glucose levels normalized after surgery and he remains asymptomatic after 1 year of follow-up. The systematic review allowed us to identify 41 adolescents from a total of 205 cases reported in 22 manuscripts, from a total of 454 found in the original search done in PubMed and Lilacs. CONCLUSIONS: Although very well reported in children, hyperinsulinemic hypoglycemia can occur in adolescents or young adults, as it happens in our reported case. These patients can be seen, treated and reported by pediatricians or adult teams either way due to the wide age range used to define adolescence. Most of them do not respond to medical treatment, and subtotal distal pancreatectomy has become the elected procedure with excellent long-term response in the vast majority.

Right Extended Split Liver Transplantation Compared With Whole Liver Transplantation: Lessons Learned at a Single Center in Latin America-Results From a Match Case-Control Study.

BACKGROUND: Despite the progressively increasing gap between patients waiting for liver transplant under the Model for End-stage Liver Disease MELD system and the availability of deceased donor organs, the use of right extended split liver grafts (RESLG) has not been accepted by all centers. In this study, we compared the results obtained using RESLG vs a group of matched whole liver graft (WLG) recipients at a single center in Latin America. METHODS: A single-center retrospective review performed between August 2009 and December 2015. RESULTS: Fifteen RESLGs were implanted to recipients between 13 and 70 years of age; 80% were performed ex situ. The "biological MELD" score for the RESLG group was 17.5 ± 5.6, and it was 12.8 ± 4.5 for the WLG group (P = .01). Cold ischemia times were significantly longer in RESLG recipients compared with WLG recipients (528 minutes vs 420 minutes; P < .01). No significant differences were found in biliary (leak or strictures P = .40) and arterial complications (hepatic artery thrombosis, P = .06). RESLG patients benefited from a considerable reduction on their waiting time in list. The 1-, 3-, and 5-year patient survival rates were 93%, 93%, and 93% respectively, for RESLG recipients vs 100%, 95.7%, and 86.1%, respectively, for WLG recipients. The 1-, 3-, and 5-year graft survival rates were 79.4%, 79.4%, and 79.4% for RESLG recipients and 89.7%, 89.7%, and 89.7% for WLG recipients, respectively. No statistical differences were observed. CONCLUSION: RESLG allows expeditious transplantation for low MELD recipients. Its use should be expanded in Latin America and worldwide as a valid alternative to increase the donor pool as it has been used in other regions.

Intrapancreatic common hepatic artery arising from the superior mesenteric artery, a challenging anatomic variation in a multiorgan harvesting.

INTRODUCTION: Understanding abdominal vascular anatomy is crucial for multiorgan recovery. In this case report, we have described a common hepatic artery that arises from the superior mesenteric artery but follows an intrapancreatic course. METHODS: The donor was ideal for multiorgan recovery and the recipient was a 29-year-old woman awaiting a second transplant owing to primary nonfunction of her first engrafted organ. The indication for transplantation was secondary biliary cirrhosis. A type I diabetic recipient on dialysis therapy was awaiting the kidney and pancreas. RESULTS: The urgent condition of our liver recipient combined with the anatomical finding prioritized liver procurement, therefore the pancreas was discarded. CONCLUSIONS: The recognition of all anatomic variations will allow us to improve the use of the scarce resource of deceased donor organs.

Neo-suprahepatic cava: a case report of a modified technique for domino liver transplantation.

Domino liver transplantation, introduced in 1997, originally consisted of a graft from a patient with familial amyloidotic polyneuropathy used as a donor for a compatible recipient, thus increasing the pool of hepatic grafts for liver transplantation. The aim of this report was to present a modification on the technique for outflow reconstruction in domino liver transplantation first proposed by Liu et al and Cescon et al. In this description we proposed a new technique that differs from the one mentioned above by performing a neo-suprahepatic cava, constructed using only an iliac vein graft, facilitating the anastomosis as if it was a regular cadaveric liver transplant.

Trasplante hepático en pacientes con trombosis portal: manejo intraoperatorio y resultados / Hepàtico transplant in patients with thrombosis vestibule: intraoperating handling and results

Antedecentes: La trombosis de la vena porta (TVP) es una complicación del paciente cirrótico que previamente era considerada una contraindicación para el trasplante hepático. Objetivos: Describir los resultados y evolución alejada de una serie consecutiva de trasplantes hepáticos realizados en pacientes portadores de TVP y analizarlos comparativamente con pacientes trasplantados sin TVP. Lugar de aplicación: Programa de trasplante hepático de una hospital público. Diseño: Retrospectivo, longitudinal, descriptivo. Material y Método: Entre julio de 1995 y junio del 2006, se realizaron 26 trasplantes hepáticos en pacientes con TVP (8,7%). Se analizaron factores de riesgo para TVP, variables del trasplante y del postrasplante. Se realizó un análisis comparativo con 273 pacientes trasplantados sin TVP. Resultados: 53,8% varones, edad 40,7 años. La TVP fue un hallazgo intraoperatorio en el 65%. Etiologías: cirrosis postnecróticas 73%, hepatopatías colestáticas 23% y fibrosis hepática congénita 4%. El 61,5% Child-Pugh C. Se realizó trombectomia en 21 pacientes con TVP Grados I, II y IV e injerto mesentérico portal extra-anatómico en 5 pacientes con TVP Grado III. La morbilidad fue del 57,7% la recurrencia de la TVP de 7,7% y la mortalidad durante la internación 26,9%. El trasplante en TVP presentó un incremento en el requerimiento de hemoderivados y en el índice de reoperaciones. La supervivencia al año fue 59,6% 75,2% para el Grado I y 44,8% para el Grado 2, 3 y 4. Conclusiones: La TVP no es contraindicación para el trasplante, su variedad más frecuente es el grado 1 y la técnica más empleada es trombectomía. El trasplante en pacientes con TVP demostró mayor requerimiento de hemoderivados, incidencia de complicaciones y de retrombosis portal y se asoció a una menor supervivencia en TVP grados 2, 3 y 4.

Liver transplantation in adult patients with portal vein thrombosis: risk factors, management and outcome.

BACKGROUND: Portal vein thrombosis (PVT) is a well recognized complication of patients with end-stage cirrhosis and its incidence ranges from 2 to 26%. The aim of this study was to analyze the results and long-term follow-up of a consecutive series of liver transplants performed in patients with PVT and compare them with patients transplanted without PVT. PATIENTS AND METHODS: Between July 1995 and June 2006, 26 liver transplants were performed in patients with PVT (8.7%). Risk factors and variables associated with the transplant and the post-transplant period were analyzed. A comparative analysis with 273 patients transplanted without PVT was performed. RESULTS: The patients comprised 53.8% males, average age 40, 7 years. PVT was detected during surgery in 65%. Indications for transplantation were: post-necrotic cirrhosis 73%, cholestatic liver diseases 23%, and congenital liver fibrosis 4%. Child-Pugh C: 61.5%. Techniques were trombectomy in 21 patients with PVT grades I, II, IV, and extra-anatomical mesenteric graft in 5 with grade III. Morbidity was 57.7%, recurrence of PVT was 7.7%, and in-hospital mortality was 26.9%. Greater operative time, transfusion requirements, and re-operations were found in PVT patients. One-year survival was 59.6%: 75.2% for grade 1 and 44.8% for grades 2, 3, and 4. DISCUSSION: The study demonstrated a PVT prevalence of 8.7%, a higher incidence of partial thrombosis (grade 1), and successful management of PVT grade 4 with thrombectomy. Liver transplant in PVT patients was associated with an increased operative time, transfusion requirements, re-interventions, and lower survival rate according to PVT extension.