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.

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.

A 1-month-old boy with shortness of extremities on prenatal US was referred to our department with a provisional diagnosis of achondroplasia. His height was normal but he had short extremities and platyspondyly, premature carpal epiphyses on both hands, and short tubular bones with irregular metaphyses on radiographs. Re-evaluation of the patient at the age of 1 year revealed very short height and premature calcification of the costal cartilages and epiphyses. Spondylometaepiphyseal dysplasia (SMED), short limb-abnormal calcification type was diagnosed. This condition is a very rare autosomal recessively inherited disorder, and most of the patients die in early childhood due to neurological involvement. At the age of 2 years and 5 months, a CT scan showed narrowing of the cervical spinal canal. One month later he died suddenly because of spinal cord injury. In conclusion early diagnosis is very important because the recurrence risk is high and patients may die due to early neurological complications. The time of onset of abnormal calcifications, a diagnostic finding of the disease, is at the age of around 1 year in most patients. When abnormal calcifications are not yet present, but radiological changes associated with SMED are present, this rare disease must be considered.

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.