Résumé
We report a 5-year-old girl with congenital hepatic fibrosis who presented with clubbing and cyanosis. Partial pressure of oxygen was 40 mmHg with oxy-gen saturation of 70% on room air, which improved to 128 mmHg and 92% on inhalation of 100% oxygen. Macroaggregated albumin scan showed 58% shunting to the brain, suggestive of severe hepatopulmonary syndrome. Echocardiogram and pulmonary angiogram ruled out pulmonary hypertension. Four weeks after living-related liver transplantation, she had normal blood gases and reduction in shunting to 7% on macroaggregated albumin scan.
Sujets)
Enfant d'âge préscolaire , Femelle , Études de suivi , Syndrome hépatopulmonaire/complications , Humains , Cirrhose du foie/congénital , Transplantation hépatique , Donneur vivant , Agrégat d'albumine marquée au technétium (99mTc)/diagnostic , Résultat thérapeutiqueRésumé
A clinicopathological analysis of 18 cases of primary intracranial malignant lymphoma encountered in the period 1980 to 1994 is presented. The total SOL's in this period was 924, the incidence of malignant lymphoma working out to 2%. The patients age ranged from 14 to 72 years with maximum prevalence in the sixth and seventh decade. The male to female ratio was 2:1 and the patients presented with evidence of CNS deficit, change in mental status or raised ICI. 15 cases had a single site of involvement while 3 patients had multicentric lesions. As far as could be determined none of the cases had evidence of immunesuppression and test for HIV carried out in five cases was negative. All the tumors were subjected to light microscopy and the diagnosis was confirmed either by ultrastructural examination or immunohistochemistry. 13 tumors were large cell high grade lymphomas while the rest revealed smaller cells. Eleven patients received post operative radiotherapy and two were given post operative chemotherapy. The outcome of the patients was poor in most cases, fifteen out of eighteen died within two years.