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Biomedical subjects

J Brodehl

Publications and source records attributed to J Brodehl.

At least 145 records · Page 8Linked to original sources

Acute interstitial nephritis in childhood.

Three children aged 11 to 14 years with acute interstitial nephritis (AIN) are presented. In one patient AIN developed following antibiotic treatment with trimethoprim/sulfamethoxazole (TMP/SMX). In two patients no infection, drug, or toxin could be implicated. Severe polyuric renal failure without elevation of blood pressure was the predominant clinical feature. Uveitis occurred either simultaneously with the nephritic symptoms or several weeks after complete recovery of renal function. Renal functions were characteristically altered and led to suspicion of AIN even prior to renal biopsy. Renal plasma flow was relatively more reduced than glomerular filtration rate (GFR) with an accordingly increased filtration fraction. Quantitative evaluation of selective tubular functions revealed significant transport deficiencies for glucose, amino acids, inorganic phosphate and low molecular weight proteins. In two patients GFR increased rapidly following initiation of steroid treatment and tubular symptoms simultaneously disappeared. In one patient spontaneous remission occurred. We conclude that--in contrast to adults--the prognosis of AIN in childhood is favorable. Although general clinical features are rather nonspecific, symptoms of decreased tubular reabsorption ability provide a good indication of the diagnosis and may contribute to enhanced recognition of this disease.

Acute Kidney Injury↗

Dosage of Cyclosporin A in children with renal transplants.

Results of Cyclosporin A (CyA) treatment following kidney transplantation in 16 children are reported. CyA was used in combination with low-dose prednisolone. The dosage of CyA was related to body surface area, starting with 500 mg/m2 daily and was reduced weekly by 50 mg/m2 until the maintenance dose of 300 mg/m2 was reached at the end of the fifth week. The dosage was controlled and adjusted by monitoring the CyA blood concentrations. In comparison with adults, children required higher CyA doses related to body weight to maintain the desired trough blood level range (200-750 ng/ml). In 16 children treated with CyA the graft function rate at three months was 100% and at six months 90%, because one patient died of septicemia. 10 patients experienced 20 reversible rejection episodes. Infectious complications and side-effects were similar to those observed in adults. Almost half of the patients exhibited transient nephrotoxicity which reversed after dose reduction. It is concluded that CyA treatment in a body surface area related dosage and in combination with blood level monitoring offers a successful way for kidney transplantation in childhood.

Adolescent↗

Liver transplantation in children.

Liver transplantation in children is still rarely performed although the prognosis for children with benign and final-stage liver disease and individual cases with unresectable hepatomas is acceptable. This report is based on the cases of eight children who underwent liver transplantation, in five cases for end-stage cirrhosis and in three cases for hepatoma. There was early mortality in only one case while in all the others development and rehabilitation were remarkable. For patients with malignancies, tumour recurrence is a limiting factor. Transplantation in biliary atresia is complicated when carried out at late stages of the disease. In cases where the time of indication is sufficiently early, a long waiting period for a suitable donor organ must be expected, during which several potential candidates may succumb to the underlying disease. Increasing experience and improved immunosuppressive therapy signify that liver transplantation can be considered more often in end-stage liver diseases.

Age Factors↗

Acute rejection episodes after renal transplantation in children.

46 renal transplants performed in children at the Medizinische Hochschule Hannover between 1975 and 1980 are evaluated for the occurrence of acute rejection episodes. 33 patients received cadaveric donor grafts (CAD) and 13 living donor grafts (LD). Immunosuppression was carried out with prednisone and azathioprine. 14 patients were treated additionally with antilymphocyte globulin (ALG). A total of 68 acute rejection episodes occurred, 38% of them within the first week of transplantation, and the latest 3 years after transplantation. The most important signs of acute rejection were a rise in serum creatinine concentration, a decrease in urine output and fever. Patients with living donor grafts and full-house matched kidneys had fewer reversible and irreversible rejection episodes than did patients with grafts from cadaveric donors and with grafts with 1-4 mismatches. The value of ALG treatment is doubtful: only 1 out of 14 patients who received ALG treatment experienced no rejection episodes compared to 12 out of 33 patients who did not have ALG treatment. 2.4 rejection episodes/patient occurred in patients who had cadaver grafts and had received ALG compared to 1.17 episodes/patient in similar patients who had not received ALG. Irreversible rejection episodes occurred in 4 out of 9 ALG-treated and in 3 out of 23 non-ALG-treated recipients of cadaver grafts.

Acute Disease↗

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.

Adolescent↗

Psychosocial and intellectual development in 12 patients with infantile nephropathic cystinosis.

The psychosocial and intellectual development of 12 children with infantile nephropathic cystinosis was investigated longitudinally by use of biographical data, long-term behavioral observations and psychological assessment. Of the 12 patients, eleven suffered terminal renal failure and 7 of these were followed up after renal transplantation. In spite of the severe illness and the resulting unusual life conditions the patients showed normal intellectual capacity and most of them average school performance. The patients were socially adapted, their behavior was predominantly cooperative, shy and reserved, but depressive at times. After transplantation the children became more active and outgoing. However, growing discrepancies between their physical development and their emotional and social age may lead to adaptational problems at adolescence which may require psychological counselling.

Adolescent↗

Postnatal development of tubular phosphate reabsorption.

In 51 infants (0.5-12 months) and 143 children (1-15 years), the postnatal development of renal phosphate handling could be studied by short term clearance investigations. The infants demonstrated significantly higher values of plasma phosphate (Pp), urinary phosphate excretion and endogenous phosphate clearance than the children. Net tubular phosphate reabsorption (Tp) was low infancy due to low glomerular filtration rate (CIn). The fractional phosphate reabsorption (Tp/CIn), however, was significantly higher in infancy than in childhood. There was a close correlation between fractional phosphate reabsorption and plasma phosphate for both children and infants. When the regression lines of Pp to Tp/CIn were analyzed separately for children and infants, a parallel shift was recognized, which means that at each level of Tp/CIn infants had higher plasma phosphate concentrations than children. Evaluation of available data suggests that the shift was very probably related to low CIn in the young infants which may lead to further retention of phosphate.

Absorption↗

Evidence for cerebral involvement in nephropathic cystinosis.

Cranial computerized tomography (CCT) of 3 children with nephropathic cystinosis and chronic renal failure (CRF) revealed a hydrocephalus internus and externus. In two boys the findings consisted of bilateral dilatation of the ventricular system and of the subarachnoid space; in one boy the alterations were mainly unilateral. The children had repeated convolsions which could not be explained by deterioration of renal function. Their neurological condition was otherwise normal. In six non-cystinotic patients with chronic renal failure, CCT showed normal anatomical structures. The possibility is discussed that the hitherto unknown pathogenetic mechanism of cystinosis leads to diffuse cerebral atrophy, resulting in internal and external hydrocephalus.

Atrophy↗

[Options in dietary treatment by oral and parenteral balanced nutrition (author's transl)].

Balanced nutrition is an important tool in the treatment of several pediatric disorders. Its application is based on the knowledge of normal metabolism and of the pathophysiology in metabolic disorders. The principles of oral balanced dietary treatment are discussed in relation to phenylketonuria and renal insufficiency. In case of unability to ingest foods by the oral-enteral route parenteral nutrition becomes necessary. The indications, principles and hazards of parenteral nutrition are discussed, especially the type of amino acid solution required. It is pointed out that xylit and sorbitol containing solutions should be abandoned completely since there are no indications for its use but potential hazards by toxic side effects.

Amino Acids↗

[Psychosocial intellectual development of children with infantile cystinosis and cerebral atrophy (author's transl)].

The psychosocial and intellectual development of 4 boys with nephropathic cystinosis and brain atrophy documented by cranial computerized tomography was investigated by use of biographical data and psychological tests (HAWIK, Deutscher Rechtschreibtest). Inspite of the brain atrophy the patients showed low-normal intellectual capacities and mainly average school performance. There were no psychosocial abnormalities correlated to the primary metabolic disease. However, renal dwarfism led to mascotism requiring psychotherapy.

Atrophy↗

[The treatment of acute dehydration (author's transl)].

In infants and children acute dehydration is mainly caused by gastroenteritis with vomiting and diarrhoea, and by feeding failures. Since in the German speaking literature very different therapeutic regimens for rehydration are discussed the principles of oral and parenteral treatment are described. The rational therapy is based on the knowledge of physiology and pathophysiology of water and electrolyte metabolism. Therefore a few basic aspects are described, i.e. compartments of body fluids, turnover rates, the balance, types of dehydration, concentration of electrolytes in body fluids and their relevance to dehydration. Special problems exist in the treatment of hypertonic dehydration. In order to avoid cerebral edema the rehydration should not be attempted quickly with very hypotonic salt solutions, but should be performed with a 1/2--1/3 isotonic Ringer-lactate solution and early begin of potassium substitution over a period of 48 h.

Acute Disease↗

Renal transport of amino acids in children with oculocerebrorenal syndrome.

Inulin and amino acid clearance studies were made in 6 boys with oculocerebrorenal syndrome (Lowe) aged 11 months to 5 years. Glomerular filtration rate ranged between 50 and 112 ml/min/1.73 m2. Clearance values of all amino acids were increased. Tubular reabsorption of cystine, ornithine, lysine and arginine was relatively more reduced than that of the other amino acids. In Lowe's syndrome the transport system of the basic amino acids and cystine appears to be more impaired than that of other amino acids.

Amino Acids↗