Technical aspects and outcomes of living donor liver transplantation for pediatric patients with situs inversus.

The vascular anomalies encountered in patients with biliary atresia associated with polysplenia syndrome and situs inversus (SI) demand technical refinements when liver transplantation is being performed. The available data describing the technique used in living donor liver transplantation (LDLT) in this population are limited; the short vascular stumps of the donor's liver can impart additional technical difficulties during vascular reconstruction. Here we describe our experience with 9 children with biliary atresia and SI who underwent LDLT. In our series, the retrohepatic vena cava was absent for 7 patients, 7 had a preduodenal portal vein (PV), and 4 had a variant arterial anatomy. The donor's left hepatic vein was anastomosed to the confluence of the recipient's 3 hepatic veins in 7 patients. Vascular grafts were used for PV reconstruction in 3 cases. A left lateral segment graft was used in all but 1 patient who needed a graft reduction. All grafts were placed in the upper left abdomen. There were no vascular complications after transplantation. All patients were alive and well at a median follow-up of 55 months. In conclusion, LDLT can be successfully performed in pediatric patients with SI. Complex vascular anomalies associated with the use of partial liver grafts obtained from living donors are not associated with an increased occurrence of vascular complications.

Liver transplantation after stage II palliation for hypoplastic left heart syndrome.

The association of biliary atresia (BA) with congenital heart diseases has been extensively described, and there are a number of reports on the outcomes of patients in this group who undergo liver transplantation (LT). The intraoperative management and the timing of LT for patients with end-stage liver disease are matters of debate, especially when complex heart diseases are involved. This report describes the outcome after LT for a pediatric recipient with BA and hypoplastic left heart syndrome. The patient underwent Norwood-Sano and Glenn procedures for heart palliation before LT. He was cyanotic, was severely malnourished, and had complications secondary to chronic liver failure. At the time of transplantation, the child was 16 months old and weighed 5175 g. Despite the critical clinical scenario and the long hospitalization period, there were no cardiac, vascular, or biliary complications after LT. At the age of 48 months, the patient was awaiting the final cardiac repair. In conclusion, the presence of complex cardiac malformations may not be a contraindication to LT. An experienced surgical team and a multidisciplinary approach are key to a successful outcome.

Four hundred thirty consecutive pediatric living donor liver transplants: variables associated with posttransplant patient and graft survival.

The availability of living donors allows transplant teams to indicate living donor liver transplantation (LDLT) early in the course of liver disease before the occurrence of life-threatening complications. Late referral to transplant centers is still a problem and can compromise the success of the procedure. The aim of this study was to examine the perioperative factors associated with patient and graft survival for 430 consecutive pediatric LDLT procedures at Sirio-Libanes Hospital/A. C. Camargo Hospital (São Paulo, Brazil) between October 1995 and April 2011. The studied pretransplant variables included the following: recipient age and body weight, Pediatric End-Stage Liver Disease score, z score for height/age, bilirubin, albumin, international normalized ratio, hemoglobin, sodium, presence of ascites, and previous surgery. The analyzed technical aspects included the graft-to-recipient weight ratio and the use of vascular grafts for portal vein reconstruction. In addition, the occurrence of hepatic artery thrombosis (HAT), portal vein thrombosis (PVT), and biliary complications was also analyzed. The liver grafts included 348 left lateral segments, 5 monosegments, 51 left lobes, and 9 right lobes. In a univariate analysis, an age < 12 months, a low body weight (≤10 kg), malnutrition, hyperbilirubinemia, and HAT were associated with decreased patient and graft survival after LDLT. In a multivariate analysis, a body weight ≤ 10 kg and HAT were significantly associated with decreased patient and graft survival. The use of vascular grafts significantly increased the occurrence of PVT. In conclusion, a low body weight (≤10 kg) and the occurrence of HAT independently determined worse patient and graft survival in this large cohort of pediatric LDLT patients.

Spontaneous intratumoral bleeding and rupture of giant gastric stromal tumor (> 30 cm) in a young patient.

BACKGROUND: Few cases of GIST bigger than 15 cm have been reported in medical literature, all primarily in elderly patients. We report an unusual case, in which a giant gastric GIST - in a young patient - presented as spontaneous intratumoral bleeding followed by intraluminal rupture. CASE PRESENTATION: A 37-year-old man was admitted with an acute onset of abdominal pain. CT showed a 32 x 25 cm mass with some cystic lesions and areas of calcification. Twelve hours after admission the patient presented with an episode of upper GI bleeding, and a significant decrease of tumor size and hemoglobin level. An upper endoscopy showed a large bulge in the posterior aspect of the gastric wall, and a small ulcer with continuous bleeding coming from a central orifice. A subtotal gastrectomy was carried out. Pathological examination showed a giant gastric GIST measuring 32 x 25 x 21 cm and weighing 3.750 g. Immunohistochemical staining demonstrated positive reactivity to C-kit protein, CD34, and alpha-smooth muscle actin; but negative reactivity to S-100 protein. CONCLUSION: Intratumoral bleeding is a very rare presentation of GIST; preoperative diagnosis is always made difficult by the absence of pathognomonic signs or symptoms. Emergency local excision with negative margins associated with adjuvant therapy with imatinib mesylate remains the main modality of treatment for high risk GISTs.

Duodenal obstruction - an unusual presentation of Strongyloides stercoralis enteritis: a case report.

BACKGROUND: Intestinal obstruction is a poorly recognized and probably underreported complication of strongyloidiasis. We present herein an unusual case, of complete duodenal obstruction caused by S. stercoralis. METHODS: A systematic review of the literature examining the clinical course, diagnostic methods, and outcome of this rare complication of strongyloidiasis was performed. RESULTS: A 42-year-old woman presented with a 5-month history of abdominal pain, vomit, and weight loss. An abdominal CT scan showed an obstruction of the third part of the duodenum. Segmental intestinal resection was carried out and histopathology examination revealed heavy Strongyloides stercoralis infestation. Duodenal obstruction is a rare complication of S. stercoralis infection, with only 8 cases described in the literature since 1970. Most of the patients are males, middle-aged, and the diagnosis was made by duodenal aspirate/biopsy, or analysis of surgical specimen. CONCLUSIONS: Duodenal obstruction is an unusual, but potential fatal, complication of S. stercoralis infection. The large spectrum of clinical manifestation and lack of classic clinical syndrome make the final diagnosis of strongyloidiasis extremely difficult. A high index of suspicion, mainly in patients from endemic areas, is needed for correct and early diagnosis of this uncommon presentation of Strogyloides stercoralis enteritis.

Análise da preservação do miocardio após administração de N-2 meercaptopropionilglicina / Analysis of myocardiocardial preservation after B2-mercaptopropionylglicin administration in an experimental model of ischemy and reperfusion in dogs

As doenças cardiovasculares representam a principal causa de morbi-mortalidade da atualidade, sendo a doença arterial coronariana seu maior expoente...

Analise do Timi Risk score em pacientes com infarto agudo do miocardio com supradesenvolvimento do segmento ST apos 6 meses / The Timi Risk score analysis in ST-elevation myocardial infarction patients after 6 months

O escore de Timi Risk (TR) para infarto agudo do miocardio com supradesnivelamento do segmento ST (IAM) foi...