Patchy dermal hypoplasia as a characteristic feature of Proteus syndrome.

Background: The diagnostic criteria of Proteus syndrome include various lesions of localized overgrowth such as digital gigantism, hemihyperplasia with unilateral macrocephaly, epidermal nevus, and mesodermal hamartomas such as lipoma, lymphangioma, hemangioma, or fibroma. Hyperplasia of the plantar dermal tissue may result in a characteristic cerebriform appearance. However, hypoplastic lesions involving various tissues such as subcutaneous fat or muscles also may be observed in this syndrome.

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine.

We report a 3-year-old girl presenting with bilateral cervical lymph node enlargement persisting for > 3 months. Leukocytosis, elevated erythrocyte sedimentation rate, a marked hypergammaglobulinemia, and a moderate hepatosplenomegaly were also found. The diagnosis of sinushistiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, was established histologically by the demonstration of characteristic sinushistiocytosis with lymphocytophagocytosis.

[Acute pancreatitis as an initial manifestation of hypercalcemia in primary hyperparathyroidism in childhood].

A 12-year-old girl was admitted to our hospital with signs of an acute abdomen with paralytic ileus. The previous and family history were without abnormalities. Abdominal pain and vomiting had started two days earlier.

[Neonatal septicemia and bacterial neonatal infection. Manifestation and course in early antibiotic therapy].

The authors studied in the Paediatric Department of the Leverkusen Municipal Hospital retrospectively the influence of an early dose of antibiotics (broad-spectrum penicillin combined with an aminoglycoside) in respect of the manifestation of bacterial newborn infections in infants with an increased risk of infection, during a period of 3 years (1981-1983). Among the infants born in the Gynaecological Department of the Leverkusen Municipal Hospital (exact data were available on the number of births and referrals) the incidence of bacterial newborn infections was 3.3% referred to the total number of deliveries (n = 3598); 0.

[Lung functions in children with marked alpha 1-antitrypsin deficiency (Pi-Z type) (author's transl)].

Various lung volumes and flows were measured in five children (aged 9-11 years), a male adolescent and one adult, all carriers of the homozygotic form of alpha 1-antitrypsin deficiency (Pi-Z type). In five patients lung function tests demonstrated overdistention, in three decreased CO diffusion capacity, as an expression of pulmonary emphysema at an early stage. Airway obstruction was not demonstrable in any of the patients (normal values for FEV 1, Tiffeneau test, peak flow and V25, V50 and V75).