Congenital syphilis presenting with nephrotic syndrome and leucocytoclastic vasculitis.

Nephrotic syndrome is a well-recognized entity in congenital syphilis but leucocytoclastic vasculitis has not yet been described. We report a case of nephrotic syndrome and leucocytoclastic vasculitis associated with congenital syphilis in a 45-day-old male infant who did well after treatment with intravenous penicillin.

Haemoperfusion in Amanita phalloides poisoning.

Amanita phalloides is responsible for about 90 per cent of all fatal cases of mushroom intoxication. The amatoxins, the main toxic component of these fungi, are responsible for gastro-intestinal symptoms as well as hepatic and renal failure. Three brothers with Amanita phalloides poisoning were admitted with gastro-intestinal symptoms beginning 12 h after ingestion. Jaundice, hepatomegaly and neurological symptoms were not present, but liver enzymes were moderately increased. Alfa-amanitin was detected in sera of all patients. All patients underwent charcoal hemoperfusion and two of them had additional hemodialysis along with conservative therapy. Liver enzymes that showed a marked increase on the second day of therapy decreased to normal levels on the 28th day. All of our patients survived. This life saving role of early haemoperfusion in Amanita phalloides poisoning is emphasized.

Prognostic factors in meningococcal disease and a new scoring system.

One-hundred-and-forty patients diagnosed as having meningococcal disease have been investigated retrospectively with respect to prognostic factors. The overall mortality was 8.6%, the mortality rate of the infants under 6 months of age being higher than that of the other groups. In cases where there was no meningitis or leucocytosis, the presence of hypotension, disturbed consciousness and diffuse petechiae increased the mortality rate significantly. High fever did not have any effect on mortality. We propose a new practical and reliable scoring system for meningococcal disease for determining the influence of prognostic factors on mortality.

The role of high-dose intravenous methylprednisolone in the treatment of varicella associated thrombocytopenic purpura.

Two children with varicella associated thrombocytopenia are presented. Case 1, evidencing no mucosal or parenchymal bleeding was administered prednisone 2 mg/kg/day therapy after having been put under observation for a period of one week without medical treatment. There was an increase in the platelet count during the period of therapy culminating in a return to normal on the eighth day. Case 2, who was in poor clinical condition at presentation, was treated with high-dose intravenous methylprednisolone (HIVMP) 30 mg/kg/day, resulting in the platelet count returning to a normal level (180,000/mm3) on the second day of therapy. HIVMP therapy is recommended in the treatment of life-threatening cases of thrombocytopenia because of its rapid action, limited side-effects, and its low cost.

Pulmonary hypertension due to chronic upper airway obstruction: a clinical review and report of four cases.

Partial airway obstruction due to the enlargement of the tonsils and adenoids is a well recognized clinical entity, but cardiorespiratory changes due to chronic obstruction have infrequently been reported. Four children with severe nasopharyngeal obstruction due to tonsil and adenoid hypertrophy, who developed pulmonary hypertension and cardiac failure, were studied. Relief of upper airway obstruction by adenotonsillectomy resulted in a regression of the presenting signs and symptoms.