[Neonatal erythrodermia - early manifestation of Omenn's syndrome]. / Noworodkowa erytrodermia-wczesna manifestacja zespolu omenna.

Ommen's syndrome is a rare, autosomal recessive disease that is characterized by symptoms of a severe combined immune deficiency, severe infections, erythrodermia, hypereosinophilia, hepatosplenomegaly, lymphadenopathy, protracted diarrhoea, hypogammaglobulinemia and elevated serum IgE. The presented case demonstrates diagnostic problems in recognizing Omenn's syndrome. These resulted predominantly from the incomplete clinical picture of the disease in its early phase. Persistent erythrodermia was the basic manifestation of the syndrome in the initial phase. Since the occurrence of the disease is extremely rare and particular symptoms are not pathognomonic, it is their co-occurrence in association with typical immunological disorders that enabled to establish the diagnosis. Through the presentation of the above case history we should like to point to the necessity of considering Omenn's syndrome in differentiating causes of erythrodermia in neonates and infants.

[Mycotic complications of shunt infection in children with primary hydrocephalus]. / Grzybicze powiklania zakazen ukladu zastawkowego u dzieci z wodoglowiem wrodzonym.

Recently, the incidence of fungal infections in children, including children with shunt-dependent hydrocephalus, has increased. The analysis comprised 8 children treated in the III Clinic of Pediatrics of ICZMP during the period of 12 months (12% of all infectious complications of the shunt system). The clinical picture of fungal infection included symptoms of shunt dysfunction: febrile conditions, vomiting, distress and loss of appetite. The most common pathogens isolated from the cerebro-spinal fluid were fungi from the Candida species. Mean value of pleocytosis in the cerebro-spinal fluid was 812 cell/microl, and mean protein concentration was 311 mg/dl. Treatment consisted of monotherapy with Diflucan, monotherapy with Ancotil or combined treatment with Ancotil and Amphotericine B. The drugs were administered intravenously and intraventricularly after removal of the shunt and application of external drainage. Sterility of cerebro-spinal fluid was obtained in the shortest time with the use of Ancotil. Prophylactic application of antifungal drugs decreases the frequency of infections in children with shunt-dependent hydrocephalus.