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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. This should be demonstrated in one case.

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.

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.

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. The indication for the examination of cerebrospinal fluid, the minimal volume for exhaustive laboratory tests and the possibilities of a stepwise diagnostic procedure are given with reference data from literature for the various techniques. In our experience the synopsis of laboratory results and clinical symptoms yielded in 75% of all cases the exclusion of an infectious etiology of the disease. In 17% a bacterial meningitis or the infection of a hydrocephalus shunt could be diagnosed. Viral infections could be proven in 4% either by antibody or by antigen detection. Only in 1% of all patients the clinical symptoms and the laboratory parameters remained unclear.

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. The median time for 'renal death' (age at death due to uraemia or age at starting renal replacement therapy) was 9.2 years. The youngest patient dying of renal death was 5.2 years. No sex difference in survival time was noticed. Furthermore no difference in survival time was noted between the different countries. The analysis of the overall survival curve indicates no clear differences between the infantile and adolescent types of cystinosis.

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. The results of this study should stimulate further research on the use of gamma-IFN in systemic juvenile rheumatoid arthritis, particularly in determining the appropriate effective dosage.

In the course of Candida albicans pyelonephritis an infant devleoped acute renal failure as a result of bezoar production with bilateral obstruction of the ureters. As on the one hand "obstructive" Candida pyelonephritis is not a well-recognised cause of renal failure, and on the other a noticeable increase in the number of cases of serious Candida infection with pyelonephritis can be observed during antibiotic and immmunosuppressive therapy, it seems worthwhile to report this case.

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). Immune complex determination in 28 children, together with antibody studies, showed that in 13 of them the Henoch-Schoenlein purpura was triggered off by an influenza-A-virus infection of the upper respiratory tract. Two thirds of the patients had markedly raised levels of total serum complement which fell weeks within several.

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. New ways of treatment are discussed, especially injection of purified C1 esterase inhibitor and stimulation of C1 esterase inhibitor synthesis by oral therapy with attenuated androgens.

A child aged 4 years was stung by more than 250 wasps. There was no anaphylactic shock. There rather were increasingly heavy ictero-hemorrhagic symptoms with simultaneous insufficient kidney function. On the second day of the disease disorders of the CNS started, as well as circulation insufficiency and disorder of the heart function. According to the signs there was no intravasal coagulation or hemolysis. Heavy doses of corticoids were successless, just as a peritoneal dialysis starting on the 3rd day of the disease. The child died 5 and 1/2 day after the wasps' attack. The pathophysiology and the possible therapeutic consequences of the case as well as rapid protective actions in such a case of insect attack are being discussed.

Dimer and polymer albumin was detected in the urine of a proportion of pantients with a nephrotic syndrome. Most of it was present as S-S bonded dimer and polymer; co-polymers, however, with IgG and alpha (1) anti-trypsin could be demonstrated. It is suggested that albumin polymerizes after it has passed the glomerular membrane. Albumin dimer was associated mainly with minimal change disease and early membranous glomerulopathy in patients, who in general responded well to therapy.

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. Preoperative stabilization of blood pressure by means of the alpha-blocker phenoxybenzamine and a subsequent intraoperative, controlled reduction of blood pressure by means of sodium nitroprusside were found to be an effective, safe and easily appreciated therapeutic concept for the perioperative care of paediatric phaeochromocytoma patients. Considerable individual differences in dose an duration of the necessary preoperative phenoxybenzamine administration rendered ward control of therapy recommendable. The risk of complete alpha-sympathicolysis by additive drug effects during premedication and induction of anaesthesia, had to be taken into consideration for conducting phenoxybenzamine therapy. Additional administration of the beta-blocker pindolol successfully controlled the intraoperatively manifested tachycardial heart rhythm phases without provoking any complicating arrhythmias. During the entire perioperative treatment of the patients it is mandatory to ensure sufficient substitution of intravascular volume to prevent hypotensive complications. Our patients did not need any cardiac and sympathicomimetic drugs as postoperative administration. None of the patients had any perioperative complications worth mentioning.

A very rare case of female epispadia without bladder extrophy is presented. The main symptoms with this malformation is incontinence and the presence of a bifid clitoris. The therapy of choice is the operative reconstruction of the urethra and reforming the bladder neck. This should not be carried out before the age of three years since the post operative bladder continence training is important for the success. Lasting urinary continence can only be expected in less than 50% of the cases.

Unlabelled: Case report on a boy aged 4. The whole of the right kidney showed changes of xanthogranulomatous pyelonephritis., Histology: dense granulomatous infiltrations of xanthome cells, epitheloid and plurinuclear giant-cells, lymphocytes and plasma cells. Diagnostic proof was based on the xanthome cells, large histocells with partially pycnotic nucleus and wide margin of cytoplasm. This condition has, so far, been seen only very rarely in children.

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. Today the following possibilities are utilisable for localising a phaeochromocytoma: diagnosis via ultrasound, axial computer tomography, stepwise withdrawal of blood from the tributaries to the vena cava superior and inferior for determining the catecholamine content. The indication for performing arteriography after preliminary treatment of the patient with alpha-receptor blockers, has become an exception.

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. At the time of last examination 24 children were alive wtith a functioning graft (mean serum creatinine level 1.5 mg/dl). After graft failure 9 children were again on intermittent hemodialysis. 7 children had died mainly because of infections or cardiovascular complications. Cumulative survival rate of patients and cadaver grafts after 2 years was 84 and 53% respectively. Growth velocity was variable but improved in the majority of patients compared to the previous dialysis period. Full social rehabilitation could be achieved in every case. Main complications were acute rejections (irreversible in 7 of 67), chronic rejections (irreversible in 6 of 10), side effects of immunosuppressive drugs (infections; hematologic, metabolic, and bone disorders), hypertension (60%), recurrence of primary renal disease (in 5 of 9 patients with focal segmental glomerulosclerosis) and surgical complications (28%). Therapeutic guide-lines are given emphasizing the role of specialized children's centres and the cooperation between pediatric nephrologists, immunologists, urologists and psychologists including the time before, during and after transplantation.

Two apparently unrelated families with a history of leukodystrophy associated with adrenal insufficiency are presented. Only about 20 cases of this syndrome have been reported until now. It was first described by Siemerling and Creutzfeldt; therefore we propose the designation Siemerling-Creutzfeldt disease. Our pedigrees include 15 additional cases and prove that this disease is inherited as an X-linked or as an autosomal dominant trait with male sex limitation. Within these families, the interindividual variability of clinical signs is remarkable. Patients can survive into the fifth decade, and one has reproduced. Attempts to identify heterozygotes on the basis of endocrinologic investigations were unsuccessful.