The clinical spectrum of shunt nephritis.

Background: Shunt nephritis is an immune-complex-mediated glomerulonephritis (GN) associated with chronically infected ventriculoatrial shunts inserted for treatment of hydrocephalus.

Methods: Six patients aged 5-22 years with shunt nephritis are reported who have been observed between 1971 and 1994. The clinical course and long-term outcome are analysed in relation to the time of diagnosis and renal histopathology.

[Life threatening salicylate poisoning caused by percutaneous absorption in severe ichthyosis vulgaris].

In a 7-year-old boy, ichthyosis vulgaris was treated with a 10% ointment for application over a large area of the body surface. In this way, the child received 400 g salicylic acid (0.6 g/kg body weight per day) percutaneously over a period of 4 weeks.

Therapy for systemic juvenile rheumatoid arthritis with gamma-interferon: a pilot study of nine patients.

Nine severely ill patients with a confirmed diagnosis of systemic juvenile rheumatoid arthritis were treated with recombinant gamma-interferon (gamma-IFN) in addition to the therapy they were previously receiving for their disease. Improvements in clinical symptoms were noted in 7 of the patients, and median laboratory values also showed a marked improvement after gamma-IFN treatment. A relapse occurred in 1 patient.

[Microbiologic-immunologic laboratory diagnosis in suspected meningitis/encephalitis].

The diagnosis on the exclusion of infectious diseases of the central nervous system, especially of bacterial infections still is one of the most important issues in clinical microbiology. In bacterial meningitis, where lethal courses as well as severe sequelae are still frequent, there should be a rapid diagnosis not only with microscopy but also with Limulus test and antigen detection tests because a specific therapy should be initiated as soon as possible. But also viral infections caused by varicella or herpes virus are increasingly susceptible to chemotherapy.

Congenital cystic adenomatoid malformation of the lung.

The clinical and pathologic-anatomical picture of this rare lung malformation is described with reference to three cases from three different paediatric surgical hospitals. Methods of differential diagnosis against lung sequestration, pulmonary cysts, lobar emphysema, diaphragmatic hernia and pneumothorax are indicated. A definitive diagnosis and classification can be made only by means of histomorphological examinations.

[Sonographically controlled preparation for biopsy of the small intestine with the Watson capsule--significantly decreased radiation burden].

Diagnostic ultrasound showed that the passing of the Watson capsule through the pylorus can be proved demonstrating its catheter within the pylorus. Only the biopsy itself has to be done under radiologic control which results in decreasing X-ray time from sometimes six minutes to a few seconds, usually less than one minute.

[Familial Mediterranean fever].

After 10 years of disease a Turkish boy and his sister were diagnosed to suffer from familial Mediterranean fever. Because an elder brother showed the symptoms of recurrent attacks of fever, abdominal pain, arthralgias and nephrotic syndrome due to amyloidosis. When these symptoms occur in residents of the Mediterranean area, the diagnosis "Familial Mediterranean Fever" has to be taken into account.

[Perioperative therapy in juvenile pheochromocytoma].

The article reports on the paediatric-anaesthesiological treatment of 6 phaeochromocytomas in 5 children who were 8 to 16 years of age. Therapeutic recommendations for the perioperative medication of infantile phaeochromocytoma patients are involved. The therapeutic aim of this study was the management of the effects of phaeochromocytoma before and after extirpation of the tumour, the effect of the phaeochromocytoma being of an alpha-adrenergic and beta-adrenergic cardiovascular nature and transmitted by catecholamines.

Survival time in cystinosis. A collaborative study.

In a retrospective study the overall survival time of 205 cystinotic patients of six countries was determined. The median survival time was 8.5 years.

[Clinical features, pathogenesis, and therapy of hereditary angioneurotic edema (author's transl)].

The hereditary angioneurotic edema is probably a more frequent disease than presumed until now. It is inherited as an autosomal dominant trait, and is due to diminished or functionally deficient C1 esterase inhibitor. Family history is negative in cases of spontaneous mutations.

["Abnormal reaction" or cultural misunderstandings? A contribution to the improvement of medical care to children of migrant labourers in the FRG (author's transl)].

The overproportional rate of medical treatment of foreign children in the private practice of paediatricians and in the paediatric hospitals imposes very often special difficulties on our medical care system. These difficulties do not only result from the language barrier but also from the vast difference between the illness concepts of our medical system which bases in natural science, and the traditional concepts of the prescientific medical layman system of the foreign patients. Because of the doctors ignorance in these different cultural forms of understanding, feeling and expression of illness, as well as in the specific attitudes to the body, shown by members--specially women and girls--of the South European an Asia Minor societies, it leads often to deep misunderstandings in the doctor-patient-relation and therefore to false diagnosis and wrong treatment.

Percutaneous treatment of renovascular hypertension.

Percutaneous transluminal dilatation of a right sided fibrous renal artery stenosis was performed in a 5-year-old boy with severe hypertension. Biochemical and hemodynamic activity of the renal artery stenosis was demonstrated by measurement of renal venous renin concentration and of pre- and poststenotic blood pressure. Hypertension disappeared within 3 weeks and the renin values became normal.

[Localisation of an extra-adrenal phaeochromocytoma with stenosis of the renal artery (author's transl)].

Compression of the left renal artery occurred in an eleven-year old girl with arterial hypertension, the compression being caused by an extra-adrenal phaeochromocytoma. Localisation of the arteriographically not identifiable phaeochromocytoma was achieved by examining the blood in stages for the presence of catecholamines from the vena cava. This examination revealed enhanced norepinephrine levels in the left renal vein.

[Kidney transplantation in children].

Ten years' experience of renal transplantation in 40 children (aged 5--18 years) is reviewed. Including 3 second transplantations 40 cadaver grafts and 3 living donor grafts were transplanted. Mean graft survival was found to be 19 months, the longest being 8 years.

[Henoch-Schoenlein purpura (author's transl)].

Routine EEG investigations and observance of discrete neurological and psychological symptoms in 13 children in the acute phase of Henoch-Schoenlein purpura showed that involvement of the central nervous system in this disease is the rule rather than the exception. Capillary resistance was reduced in 51 out of 76 investigated children. On the other hand a reduction in factor XIII activity was much less commonly found (n =6).

[Hereditary angioneurotic edema. Clinical aspects, pathogenesis and therapy].

A family with hereditary angioedema is presented. In 4 members of the family the diagnosis was confirmed by decreased C1 esterase inhibitor levels. Clinical course and pathogenesis are described briefly.