[A clinical analysis of internal or external diversion in biliary atresia]. / Análisis clínico de la derivación interna o externa en la atresia biliar.

Cholangitis has been the most common postoperative complication of Kasai's operation for biliary atresia. A host of ingenious surgical procedures have been used to prevent this complication, some including exteriorization of the bilioenteric conduit. The purpose of this report was to investigate the role of the derivation on the incidence of cholangitis, liver function and the survival of patients with biliary atresia treated with Kasai's operation. We have analyzed the clinical data of thirty-six patients with biliary atresia treated with Kasai's portoenterostomy during a seven year period (1987-1993). The patients were divided into two groups: Group I, patients treated with portoenterostomy and exteriorization of the bilioenteric conduit (n = 18) and Group II, patients treated with portoenterostomy without enterostomy. Our results suggest that the use of the exteriorization of the bilioenteric conduit was not shown to be effective in the prevention of episodes of cholangitis. The survival for both groups was not statistically significant and there was no increase in morbidity after the postoperative period or during the liver transplant.

[Experience with the impression cytology test for detecting vitamin A deficiency in children]. / Nuestra experiencia con el test de impresión citológica para detectar deficiencia de vitamina A en niños.

Impression cytology has been suggested as a relatively simple, cheap, practical and non invasive technique for documenting physiologically significant vitamin A deficiency. The vitamin A status of 41 patients was evaluated by serum retinol concentration by high power liquid chromatography (HPLC) and conjunctival impression cytology was performed on each child. All children with normal vitamin A status had normal conjunctival impression cytology. The sensibility of this method was 100%. However, in the 8 vitamin A deficient patients by impression cytology, only 6 of them showed low serum vitamin A levels. Impression cytology appears to detect preclinical vitamin A deficiency that this technique may by of potential usefulness as a screening tool for preclinical vitamin A deficiency populations.

[The current state of Niemann-Pick disease: evaluation of six cases]. / Estado actual de la enfermedad de Niemann-Pick: valoración de seis casos.

Six pediatric patients with Niemann-Pick disease are reported. They have been studied at Hepatology Unit HI "La Paz" (Madrid) in the period of time between 1975-1988. They are one case of type A, one case of type B and four cases of type C. This group of pediatric patients serve us to make a revision of the disease attending to clinical and biochemical classification aspects, diagnosis and treatment. We insist on two aspects: 1) greater importance of enzymatic diagnose for the "Niemann-Pick complex", in view of the overlapping of clinical symptoms, 2) bone-marrow transplantation as therapeutic alternative and its indications.

[Indications and selection of candidates for liver transplants in children]. / Indicaciones y selección de candidatos a trasplante hepático en niños.

Liver transplantation in children offers a therapeutic alternative for severe liver disease. Post-transplantation five-year survival reaches 76%. At the moment this technique must be indicated as a controversial subject, which requires considering both the severity of liver disease and scarcity of donors, with unpredictable waiting time. We report our experience after the study of 57 children selected for liver transplant. Their diagnosis, clinical and functional stage, situations considered as contraindications, and pre-transplantation management aimed at supporting candidates during waiting time and to diminish avoidable risks after surgery, is described. During waiting time, 15.9% of the patients died. Post-transplantation actuarial survival in our center is 63.6% (January 1986 to November 1988). Survival has markedly improved, reaching 81%, in the last 23 months in relation to a prophylactic approach of cytomegalovirus disease and performance of retransplantation in cases of severe graft dysfunction.

[Viral infection and obstructive cholangiography in children]. / Infección vírica y colangiopatía obstructiva infantil.

In the last years it has been suggested that idiopathic obstructive cholangiopathy is a pathologic process with evolutive studies: extrahepatic biliary atresia and neonatal hepatitis. The possibility of a viral agent as the inciting factor has been investigated. This report shows four male and one female with confirmed idiopathic obstructive cholangiopathy with different clinical picture. There was evidence of cytomegalovirus infection in two patients, rubella infection in one patient, hepatitis B virus infection in one patient and Epstein-Barr virus infection in one patient. While other causes of neonatal cholestasis were excluded, we suggest that those viruses has an etiologic role in the initial injury and the progression of the disease.

[Alpha 1-antitrypsin deficiency with histologic expression of metabolic disease of carbohydrates]. / Déficit de alpha-1-antitripsina con expresividad histológica de enfermedad metabólica de hidratos de carbono.

A two months old male affected by alpha-1-AT PiZZ deficiency with severe transient neonatal cholestasis is presented. Two hepatic biopsies were practiced in neonatal period. There was no evidence of PAS positive globules, but an intense univacuolar steatosis and a rossetoid transformation of hepatocytes were observed. Both findings are identical to those found in the histopathologic study of the liver in certain metabolic diseases such as fructosemia and galactosemia. A third biopsy practiced at an age of two years confirmed diagnosis of alpha-1-AT deficit since presence of PAS positive globules was established. It must be pointed out that histopathological findings show great variability among different patients with alpha-1-AT deficit in the neonatal period, as well as the infrequent presence of PAS positive globules in hepatic biopsies of those c during the first months of life.

[Chronic hepatitis caused by B virus and glomerulonephritis in children]. / Hepatitis crónica por virus B y glomerulonefritis en niños.

Eight children with chronic HBV hepatitis and glomerular disease were studied. There were seven males and one female, with ages between one and nine years old. Three had CPH and five CAH. All patients with CPH had membranous nephropathy, and those with CAH three had MNG. one membranoproliferative glomerulonephritis and one mesangial glomerulonephritis. Only six patients had moderate liver enlargement. In all, the diagnosis of HBV infection was performed by investigation of the etiology of their nephropathy. Liver function test showed signs of moderate necrobiosis with impaired biliary acid metabolism. All were HBsAg (+), antiHBc (+), six were antiHBe (+) and two were HBeAg (+). In one case renal deposit of HBsAg was found. Due to the frequent association of HBsAg and glomerulonephritis (GN) in children, an investigation of liver function is highly recommended in those patients with GN a HBV infection.