[Alagille syndrome and isolated persistent ductus arteriosus]. / Síndrome de Alagille y ductus arterioso persistente aislado.

The clinical case of a 21-year-old patient diagnosed with a variant of intrahepatic biliary ductopenia or Alagille syndrome, a dominant inherited disorder associated with a chronic cholestatic syndrome and abnormalities in different organs and systems. The present article discusses the main clinical abnormalities found in this syndrome and we describe a persistent arterial ductus as an isolated cardiovascular manifestation not previously reported in association to this syndrome.

[Leukocytoclastic vasculitis as the form of presentation of hepatocarcinoma]. / Vasculitis leucocitoclástica como forma de presentación de un hepatocarcinoma.

The clinical case of a 45-years old patient with previous diagnosis of alcoholic cirrhosis who was admitted to study the appearance of a picture consisting in erythematoviolescent lesions in the lower limbs and advanced renal failure is presented. Anatomopathologic study of the cutaneous lesions suggested the diagnosis of leukocytoclastic vasculitis. This fact was of special clinical interest on having taken into consideration the cytologic demonstration of hepatocarcinoma superimposed to the established chronic liver disease of the patient. The rarity of leukocytoclastic vasculitis as the initial clinical manifestation of a hepatocarcinoma versus the most common forms of presentation is herein discussed.

Free protein S deficiency in hemodialysis patients due to vascular calcifications?

Vascular calcifications are frequent in hemodialysis patients. Its incidence ranges from 25 to 67% depending of different series. Thirty hemodialysis patients were selected from a dialysis population of 150 patients. These 30 patients were divided into two groups: group I included 15 hemodialysis patients with severe secondary hyperparathyroidism and severe, roentgenographically visible diffuse vascular calcifications, and group II included 15 other hemodialysis patients with moderate hyperparathyroidism without radiographic evidence of arterial calcifications. The control group comprised 20 normal volunteers. In all patients, measurements of protein C activity, free protein S and intact parathyroid hormone (PTH) were performed. Statistical analysis showed that free protein S in the patients of group I had a tendency to be lower than in the patients of group II (p < 0.01) and the control group (p < 0.001). We did not find significant differences in free protein S between group II and control group patients nor a significant correlation between intact PTH and free protein S in groups I and II. Protein C activity was found to be in the normal range in both groups. Free protein S deficiency in patients of group I would suggest a synthesis defect by impaired endothelial cells-due to vascular calcifications (?). Free protein S deficiency could increase the risk of thrombotic complications in these patients.