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. RESULTS: The time of diagnosis of shunt nephritis ranged from 0.3 to 4.5 years after the last shunt operation. Diagnosis was delayed up to 1.5 years after the first clinical manifestations. All patients had signs of infection, i.e. recurrent fever, hepatosplenomegaly, anaemia, and cerebral symptoms. Renal manifestations consisted of haematuria (macroscopic in 3 patients), proteinuria (heavy in 5), renal insufficiency (4) and hypertension (2). Decreased C3 levels, cryoglobulins, and antinuclear factors were frequent. Cultures of blood and cerebrospinal fluid provided growth mainly of S. epidermidis. Renal biopsy revealed endocapillary GN (1), membranoproliferative GN (1) and endocapillary/extracapillary GN with crescents (2). All patients received antibiotics i.v. Complete recovery was observed in three of four patients in whom the shunt was totally removed, supported by transient external drainage of cerebrospinal fluid, and followed by placement of a ventriculoperitoneal shunt. One child with delayed diagnosis, presenting with a serum creatinine of 3.2 mg/dl, hypertension, and severe scarring on renal biopsy, rapidly progressed to irreversible ESRD within 5 months. Two patients without and only partial removal of the shunt died subsequently from sepsis. CONCLUSIONS: The renal outcome of shunt nephritis is good if early diagnosis and treatment is provided including i.v. antibiotics and total removal of the infected shunt. The possible progression to ESRD requires frequent nephrological monitoring of patients with ventriculoatrial shunts.

[Life threatening salicylate poisoning caused by percutaneous absorption in severe ichthyosis vulgaris]. / Lebensbedrohliche Salicylatintoxikation durch perkutane Resorption bei einer schweren 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. The patient was referred to hospital by the family doctor: he was in a deep somnolent state, apparently caused by hyperventilation following wheezing, vomiting, tinnitus and vertigo. Salicylate intoxication was suspected because of metabolic acidosis, an anion gap and respiratory overcompensation. The diagnosis was confirmed by a serum salicylate level of 985 micrograms/ml (therapeutic level 150-300 micrograms/ml). Following forced diuresis and alkalization with sodium bicarbonate, haemodialysis was unnecessary. As the salicylate level declined to values within the therapeutic range, the patient started to recover consciousness, waking on the 4th day. By day 6 there were still obvious neurological deficiencies. Fecal incontinence, bilateral ptosis and intermittent diverging strabismus on the right persisted for some weeks. It was 6 months before complete neurological resolution was achieved. The pathogenesis of salicylate toxicity and the need for safer therapies for ichthyosis vulgaris are discussed.

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. 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.

[Microbiologic-immunologic laboratory diagnosis in suspected meningitis/encephalitis]. / Mikrobiologisch-immunologische Labordiagnostik bei Verdacht auf Meningitis/Enzephalitis.

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.

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]. / Sonographisch geführte Vorbereitung zur Dünndarmbiopsie mit der Watson-Kapsel--signifikant verringerte Strahlenbelastung.

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]. / Familiäres Mittelmeerfieber.

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]. / Die perioperative Therapie beim kindlichen Phäochromozytom.

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.

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. 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.

[Clinical features, pathogenesis, and therapy of hereditary angioneurotic edema (author's transl)]. / Klinik, Pathogenese und Therapie des hereditären angioneurotischen Odems.

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. Two thirds of the patients are already affected in infancy with peripheral edema, facial edema, abdominal symptoms and/or edema of the glottis occuring spontaneously or secondary to trauma. Recently the prognosis has become better due to early diagnosis and the possibility of substitution therapy with C1 esterase inhibitor. A long-term prophylaxis may be achieved with synthetic androgens.

["Abnormal reaction" or cultural misunderstandings? A contribution to the improvement of medical care to children of migrant labourers in the FRG (author's transl)]. / Abnorme Reaktionen oder kulturelle Missverständnisse? - Ein Beitrag zur Verbesserung der medizinischen Versorgung der Gastarbeiterkinder in der Bundesrepublik.

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.

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. The method is less traumatic than operative revascularization and may be an alternative procedure to vascular surgery in the treatment of renovascular hypertension in childhood.