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L Zancan

Publications and source records attributed to L Zancan.

66 records · Page 4Linked to original sources

Antiphospholipid syndrome in a child with trisomy 21: the relationship between anticardiolipin G antibodies and the von Willebrand factor.

A young child with trisomy 21 developed severe multiple arterial thromboses in a three-phase clinical course which proved fatal. The episodes were characterized by high levels of both IgG anticardiolipin antibodies and the von Willebrand factor. The data suggest that there is a relationship between these two elements and the clinical events which followed.

Antibodies, Anticardiolipin↗

[Long-term prognosis of patients with extrahepatic biliary atresia successfully treated with surgery. Our experience].

The long-term prognosis of extrahepatic biliary atresia after surgical restoration of bile flow is still controversial. An ongoing process of cirrhosis and the development of portal hypertension continue to create frequent and frustrating management problems. Clinical features, hepatic function, echotomography aspect, calcium-phosphorus metabolism and serum levels of 25-OH-D-3 were evaluated in 12 anicteric patients with extrahepatic biliary atresia successfully treated in a period from 1974 through 1987. Seven of these children had a total of 21 episodes of cholangitis. In five patients liver biopsy, obtained at the time of the external diversion closure, showed a biliary cirrhosis. Growth, development and hepatic function were normal in all children studied; one patient had esophageal varices. The serum levels of 25-OH-D3 in patients without oral supplementation of vitamin D are lower than normal. This deficit can be corrected by oral administration of vitamin D. Our study revealed that the children with successful portoenterostomy appeared to thrive normally and that they tolerated the relatively mild liver damage. We believe that Kasai operation should be done in all patients with extrahepatic biliary atresia and that the liver transplantation is to be reserved only in those with unsuccessful Kasai. In our experience external diversion was not useful to prevent cholangitis and moreover it complicates the hepatectomy in case of transplantation.

Biliary Atresia↗

[Membranous nephropathy associated with chronic hepatitis caused by B virus].

We report 6 cases with membranous glomerulonephritis associated with chronic hepatitis B virus infection, who came under our observation for microscopic haematuria in 2 cases, haematuria and proteinuria in 1 case, and nephrotic syndrome in the others. At the examination all 6 children were found to have hepatomegaly with raised serum transaminase activity. All the patients were positive in the serum for HBsAg and anti-HBc, 3 were HBeAg and anti-HBe negative. Liver biopsy showed features of chronic hepatitis with moderate signs of activity. Renal biopsy was consistent with membranous glomerulonephritis in all patients. With a fluorescent antibody technic HBeAg was found to be deposited in diffuse granular fashion, along glomerular capillary walls, together with IgG, in 2 out of 3 cases stained, but no deposition of HBsAg was detected in all the patients. Steroid therapy was started from 18 to 33 months. Urine analysis became negative in 4 cases and persisted normal in the follow-up. One patient with haematuria developed nephrotic syndrome and one died from miliary tuberculosis. Our findings suggest that the clinical outcome is favourable in children with membranous glomerulonephritis and chronic active hepatitis and that the pathogenesis probably is not unique.

Child↗

[Gastric trichobezoar: a case report (author's transl)].

We describe the case of an 11 years old girl admitted to investigate an abdominal mass. The patient presented with abdominal pain, halitosis, exhaustion, started about 1 year prior to admission. An upper gastrointestinal tract with barium and a gastric endoscopy showed a massive trichobezoar which was then surgically removed. A detailed medical history revealed a prolonged habit of trichophagia.

Bezoars↗

[Abdominal pain syndrome recurring after 40 years: critical revision].

Recurrent abdominal pain (RAP) syndrome is described by Apley 40 years ago. The definition of condition, still generally accepted, is at least three episodes of abdominal pain over a period of three months, with pain of intensity which affects the behaviour of the child. The prevalence of condition among school children is 10-15%. Apley's classic studies demonstrated organic disease in only 10% of the children. Apley's conclusions have dominated pediatric writing through present era. In recent years, however, a number of reports have appeared in the medical literature that have suggested that careful investigation of children with RAP may reveal previously unsuspected functional or morphologic abnormalities of the gastrointestinal tract. These have included reports of peptic disease and Helicobacter Pylori infection, abnormal antro-duodenal motility, lactase malabsorption, gastro-esophageal reflux. Nevertheless these abnormalities cannot be correlated always with specific complaints. Therefore pathogenetic background is not clarified. Despite greater understanding of these disorders the enigme remains. There is a need for controlled studies in non selected patients.

Abdominal Pain↗

[Cystic dilatation of the choledochus: the diagnostic problems].

Choledochal cyst is a rare pathology in western countries and the typical signs and symptoms are not always present at the onset. The diagnosis is often difficult and it needs a long time to be clear. In this paper we review our recent experience with this pathology (8 patients) and particularly we point out the problems of the delate in the diagnosis and the best choice to study these patients.

Choledochal Cyst↗