[Clinical aspects and allergy-immunologic parameters in 40 children 0-7 years of age with atopic dermatitis].

Forty children with atopic dermatitis were evaluated for history, clinical features and allergologic-immunologic parameters. Lichenoid skin lesions were found in 67.5%, follicular lesions in 57.

Variant of hyper-IgE syndrome: the differentiation from atopic dermatitis is important because of treatment and prognosis.

The hyper-IgE syndrome is characterized clinically by recurrent staphylococcal abscesses of the skin, lungs and other sites from infancy. Affected patients also have a pruritic dermatitis that differs in character and distribution from lesions of atopic dermatitis. Most lack other signs of atopic disease, develop persistent pneumatoceles and have osteopenia.

[Dubowitz syndrome: a dysmorphism syndrome with developmental delay, transitory short stature, hyperactive behavior and atopic dermatitis].

The Dubowitz-syndrome, a rare, autosomal-recessive condition, was seen in a 6-year-old female patient. Verbal, fine motor, and social development were severely retarded. Behavioral disturbances, predominantly hyperactivity were apparent.

[Angioedema caused by enalapril (Reniten)].

Two cases of enalapril(Reniten)-induced angioedema are described. In both patients the time lag between the first manifestation of angio-edema and diagnosis was more than one year, during which several bouts of edema occurred. One patient developed life-threatening swelling of the tongue and the larynx followed by asystole and apnea.

[Carbamyl phosphate synthase deficiency: clinical symptoms, diagnosis and dietary-medicamentous treatment in the neonatal period and infancy].

Carbamyl phosphate synthetase (CPS) catalyses the synthesis of carbamyl-phosphate from ammonia and bicarbonate and is the first step in ureagenesis. The infant described in this report suffered from deficiency of this enzyme. The symptoms started on the 2nd day of life with tachycardia, apathy, irritability and metabolic alcalosis, on the 4th day coma and fits occurred due to hyperammonia (ammonia in the blood max 496 mumol/l, normally up to 150 in newborns).