[Follow-up of pediatric patients evaluated for liver transplantation]. / Evolução dos pacientes pediátricos avaliados para transplante hepático.

OBJECTIVE: To analyze the evolution of pediatric patients chosen for hepatic transplantation. METHODS: A review was made of the clinical charts of the first 65 children and adolescents with chronic liver disease, aged 5 months to 19 years (X = 6.8%), chosen for liver transplantation during the period of August 1994 to March 1996. Data refer to the patients' demographic characteristics, etiology of their liver disease, their psychosocial situation and of their parents, and their clinical and laboratorial evaluation. According to the severity of the disease, patients were classified as active (waiting for a donor), in evaluation, inactive (compensated liver disease), and excluded for psychosocial or medical conditions, or because of bad indication. RESULTS: Eight patients (12%) received transplantation, and one of them died. Seven (11%) died when in evaluation or waiting for a donor. Ten patients (15%) were excluded from the waiting list: 6 for social problems, and 4 for medical problems. No patient was excluded for bad indication. Six patients are in the active list, waiting for donor. The other 23 patients (35%) are in evaluation, and 11 (17%) are classified as inactive in the waiting list. CONCLUSIONS: Eleven patients (17%) were not operated on due to the advanced stage of the liver disease. We emphasize the necessity of organ donation, and the early contact of the patients with a reference center.

[The Rapunzel syndrome - a case report: trichobezoar as a cause of intestinal perforation] / Síndrome de Rapunzel - relato de caso: tricobezoar como causa de perfuração intestinal.

OBJECTIVE: To report a rare case of a patient with gastric trichobezoar extended through the small bowel, proper to the Rapunzel syndrome. CASE REPORT: Girl at age 7 with history and exams suggestive of peritonitis whose laparotomy demonstrated trichobezoar and jejunal perforation. She also presented alopecia and psychological disorder which were not totally eliminated despite the support given. CONCLUSION: A multidisciplinary view of the patient would have increased the suspicion rate for the pathology and would provide early diagnosis and treatment before complication was evident.

Transplante renal en niños: estudio analitico de 25 casos / Renal transplantation: analysis of 25 cases

Se analiza un grupo de 25 pacientes sometidos a trasplante renal, con una edad media de 11=3 años, 9 niñas y 16 varones. Se utilizaron 22 injertos de dador vivo y 3 de cadaver. El peso corporal era menor de 25 kg. En el 60 por ciento de la poblacion y todos tenian deficit pondoestatural. Las uropatias (12/25) y las glomerulopatias (9/25), fueron las enfermedades de base mas frecuentes que llevaron a estos pacientes a la insuficiencia renal. La tasa de mortalidad fue del 12 por ciento (3 casos). Las complicaciones postoperatorias y la tasa de mortalidad fueron mayores para los injertos cadavericos, pero sin significancia estadistica por el numero de casos. Los reimplantes ureterales se realizaron con tecnica de Politano-Leadbetter en 22 y con tecnica de Gregoire en 3. Las complicaciones precoces mas frecuentes fueron la discracia sanguinea (4/25) y el rechazo agudo (5/25). Las tardias fueron la infeccion urinaria (4/25) y el rechazo cronico (3/25). Catorce injertos presentan un clearence > de 80 ml/min y 2 entre 40-45 ml/min. La sobrevida actuarial del injerto fue de 64 por ciento, siendo el rechazo la principal causa de fracaso. Cuatro pacientes tenian reconstrucciones del tracto urinario a traves de un reservorio continente y mecanismo de cateterismo limpio por la tecnica de Mitrofanoff. Se concluye que el trasplante renal es una opcion terapeutica para niños con enfermedades renales, que tiene baja mortalidad y complicaciones locales del facil manejo

Anomalia anorretal baixa (anus coberto completo) com fistula anorreto-uretral (tipo-N). Relato de um caso. / Low anorectal malformation (anus completely covered) with anorectal urethral fistula (N-type). Case report

Os autores descrevem um caso de anomalia anorretal baixa (anus coberto completo) com fistula anorreto-uretral (tipo-N), ocorrido em RN nascido a termo e do sexo masculino. No presente relato, referem a ausencia de anomalias associadas e o bom resultado obtido com o tratamento cirurgico definitivo - anoplastia perineal e ligadura da fistula reto-uretral