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).

The prognostic significance of specific IgG antibodies in insect sting allergy.

Specific IgG antibodies against bee venom and bee venom components were studied in the serum of 40 bee-sting patients, 60 bee keepers, and 31 control subjects. The highest titres were observed in successfully hypothesized patients and in bee keepers. Subclass-typing in bee-sting allergic patients showed the highest antibody levels in subclass IgG2, followed by IgG3, IgG 4 and IgG1.

Immunological studies on bee-keepers: specific IgG and subclass typing IgG against bee venom and bee venom components.

Specific IgE antibodies against bee venom and its components were studied in 23 bee-keepers. The highest IgG serum levels were observed for whole bee venom followed by phospholipase A. The serum levels of specific IgG antibodies against melittin and MCD-peptide were lower, the lowest serum levels being observed for apamin.

[Recovery of hypothalamic-pituitary-adrenocortical axis after high-dose dexamethasone treatment (author's transl)].

High doses of dexamethasone (Fortecortin) for the prophylaxis of cerebral oedema were administered to 19 children aged 3 to 14 years because of head injury of varying degrees of severity. In order to assess possible suppression of the hypothalamic-pituitary-adrenocortical activity an insulin tolerance test and an intravenous tetracosactid (Synacthen) test was performed 1 to 26 weeks after the last dexamethasone dose. There was no evidence from the insulin tolerance test of inadequate cortisol excretion.

[The albumin-IgG ratio in the CSF in inflammatory diseases of the CNS (author's transl)].

Levels of albumin and IgG in the cerebrospinal fluid were determined in 69 children with infectious diseases of the central of the central nervous system in the years 1975 to 1978. The cerebrospinal fluid in 63 children without infectious diseases of the central nervous system, serving as a control, showed a close correlation between albumin and IgG levels. In the acute stages of serous and purulent meningitis (meningococci, streptococci, and hemophilus influenzae) however, IgG levels in the cerebrospinal fluid were overproportionately high.

[Suggestion for treatment of primarily inoperable omphalocele (author's transl)].

Bending primarily inoperable omphalocele can be avoided by simply using Stülpa tube-dressing together with a spongering. Epithelising under the employment of tannin or Betaisodona becomes much easier if this method is used.

[Hyposensitisation treatment with pure bee venom (author's transl)].

Hyposensitisation with pure bee venom was undertaken in eleven persons with proven allergy to bee stings. Rapid hyposensitisation led to side effects in seven of them during dosage increase. Serial determination of total IgA, IgG, IgM and alpha1-antitrypsin revealed no changes, while allergen-specific IgG and IgE and total IgE increased maximally after 30 days.

[Serum IgE in skin diseases of children (author's transl)].

Using the paper-radioimmunosorbens test (PRIST) normal values were obtained from 200 non-allergic children. The IgE level was aged-dependent, in part with significant differences between various age groups. These normal values were compared with those in children with seborrhoeic dermitis, constitutional neurodermatitis, parasitoses, urticaria, Quincke oedema, Schönlein-Henoch purpura, pityriasis rosea, multiform exudative erythema, erythema nodosum and infantile papular acrodermatitis.

[Liver disease in homozygous alpha1-antitrypsin deficiency (author's transl)].

Among twelve patients with homozygous alpha1-antitrypsin deficiency (Pi-type Z), five cases of infantile liver disease were diagnosed. The course of the disease was extremely variable; only one patient died of liver cirrhosis at the age of fourteen. In four cases the clinical, biochemical and histological (2 cases) findings became normal over a follow-up period of one to fifteen years.

[Prolonged jaundice by heterozygous alpha-1-antitrypsin-deficience? (author's transl)].

Of 28 newborns with prolonged jaundice and lowered or subnormal values of alpha-1-antitrypsin in the serum six patients were found to have a Pi-type MZ, three a Pi-type MS and one patient a Pi-type MP. One of the newborns had Pi-type SZ (double heterozygous), one infant was a homozygous carrier of the Pi-type S. It seems to the possible that one of the reasons for the not yet explained cases of prolonged jaundice of the newborns period can be found in the high frequency of homozygous or heterozygous genotype carriers of Pi-system.

[Allergy to insect stings (author's transl)].

Generalised reactions after an insect bite call for an exact diagnosis: nonspecific general reactions must be distinguished from true allergic reactions. 12 children with allergic reactions to bee and/or wasp stings were investigated. The results of the skin tests and the allergen-specific IgE determinations were compared and found to vary distinctly in some cases.

[T and B lymphocytes before, during, and after cytostatic therapy of acute lymphoblastic leukemia (ALL) in children (author's transl)].

In 56 children with acute lymphoblastic leukemia (ALL) T and B lymphocytes and immunoglobulins (IgG, IgM, and IgA) were studied before, during, and after therapy. At the time of diagnosis T lymphocytes were normal, the number of B lymphocytes was increased, and immunoglobulins usually were normal. Only two of 30 children had abnormally low immunoglobulin levels.

[The quantitative determination of the individual CSF proteins in the diagnosis of inflammatory diseases of the CNS in children (author's transl)].

Albumin, IgG, IgA, IgM, transferrin, and alpha 1-antitrypsin were determined quantitatively in the cerebrospinal fluid (CSF) of 44 healthy children and 37 pediatric patients with central nervous system diseases. Neither IgA nor IgM were found in the CSF of normal children, but they were present in cases of purulent and non-bacterial meningitis. In cases of encephalitis all proteins studied were increased except IgA and IgM, which could not be demonstrated.

[Long term follow up after parenteral specific hyposensitization in children (author's transl)].

The long term follow up of 55 children with allergy treated by specific hyposensitization therapy is reported. Hyposensitization therapy had been terminated from 2--7 years previously. The success of treatment was subjectively judged to be satisfactory of good in 62% of the cases.

[Hypophosphataemia as early sign of septicaemia in childhood (author's transl)].

The early diagnosis of septicaemia in children is difficult because the clinical and laboratory findings can be inconclusive. Results of blood cultures are usually available after two days only. Especially in the neonate, delayed or uncritical use of wide spectrum antibiotics can have negative consequences.