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J Brodehl

Publications and source records attributed to J Brodehl.

At least 73 records · Page 4Linked to original sources
PubMed1992

[Neonatal variant of Bartter syndrome].

A male preterm infant of 32 weeks of gestation with history of severe polyhydramnios during pregnancy presented soon after birth with polyuria with initial sodium chloride loss subsequently followed by increasing potassium loss. After manifestation of hypokalaemia, hypochloraemia, alkalosis and high urinary prostaglandin concentrations, the diagnosis of the neonatal variant of Bartter's syndrome was made. The treatment consisted of administered of large amounts of fluid with sodium chloride and potassium supplementation and indomethacin (1.5 to 2 mg/kg per day).

Bartter Syndrome↗
PubMed1991

Practical aspects in the use of cyclosporin in paediatric nephrology.

Many factors must be considered for the effective and safe use of cyclosporin A (CsA) in paediatric nephrology. Detailed knowledge of the variable bioavailability, tissue distribution, and metabolism, as well as causes which lead to their alteration are necessary. Factors which affect the activity of the mixed function oxidase system cytochrome P-450 must be considered, i.e. liver dysfunction and many drugs. Precise knowledge of the CsA determination method and the spectrum of metabolites is essential. In children with renal transplants, a body surface area-related dose will better meet the dose requirements than a body weight related-dose. For drug level monitoring whole blood rather than plasma should be used, and the parent drug level should be the main determinant; elevated metabolite levels may be important in suspected nephrotoxicity or liver dysfunction. Pharmacokinetic profiles are necessary to discover absorption problems or increased CsA clearance rates which necessitate shorter dosing intervals. In children with steroid-dependent minimal change nephrotic syndrome, remission without steroids is maintained as long as CsA is given. The appropriate starting dosage is 150 mg/m2 per day; trough level monitoring is mandatory to prevent nephrotoxicity and to confirm adequate immunosuppressive drug levels which should be 80-160 ng/ml (parent drug level). Although the benefit of CsA has been reported in some cases of lupus erythematosus, its use should be restricted to severe cases only until its efficacy and safety has been confirmed in controlled trials.

Child↗
PubMed1991

Renal transplantation in 22 children with nephropathic cystinosis.

In 1989, 22 children (11 boys, 11 girls aged 8-23 years) with nephropathic cystinosis, who had received a total of 28 renal allografts over the previous 14 years, were reviewed. Nineteen were alive, of whom 17 had functioning grafts 5 months to 13 years after transplantation. The mean serum creatinine level in these 17 was 135 mumol/l. Patient and graft survival did not differ from non-cystinotic children. Persistent hypothyroidism was found in 3 patients, transient diabetes mellitus in 1, severely disturbed vision in 1 and brain atrophy in 11. Arterial hypertension was present in 16 patients. Growth retardation was universal, although in 4 patients on cyclosporin A post-transplant catch-up growth occurred. Five patients over 15 years completed puberty. Readjustment in terms of school performance was good but was less good for psychosocial development. None of the patients had ever been treated with cystine-depleting agents; the data will therefore provide a historical control group with which to compare the results from a group treated with these agents.

Adolescent↗
PubMed1991

Growth after renal transplantation: an update.

Several factors influencing post-transplant growth were analysed in a total of 163 children receiving transplants at the Medical School Hannover. Statural height at the time of transplantation depended on the length of the pre-transplant period of chronic renal failure, and was more retarded in children with congenital renal diseases than in those with acquired diseases. The retardation of bone age correlated significantly with the degree of growth retardation. The immunosuppressive regimen of cyclosporine A (CyA) and low-dose prednisolone was followed by significantly better growth rates than azathioprine (Aza) plus high-dose prednisolone. In 22 prepubertal children receiving CyA, poor graft function with a glomerular filtration rate below 40 ml/min per 1.73 m2 inhibited catch-up growth. The final height of 20 grown-up transplant recipients was found to be in the lower range of normal. A comparison of conventional and CyA treatment showed that adult height in the CyA group was higher than in the Aza group due to a significantly higher growth velocity.

Adolescent↗
PubMed1991

Treatment of inherited metabolic disorders by liver transplantation.

Among the worldwide accepted indications for liver transplantation, inherited metabolic disorders play an increasing role. In some paediatric centres this indication runs second after extrahepatic biliary atresia. The aim of liver transplantation in inherited metabolic disorders is twofold: the first is to save a patient's life, the second is to accomplish phenotypic and functional cure of his disease. These aims may be achieved in disorders presenting with cirrhosis, hepatoma, life-threatening progression or failure of other organs with preserved liver function. The timing of liver transplantation has become easier with development of surgical techniques of reduced-size donor livers. These techniques enable the performance of liver transplantation with ABO blood group compatible organs of almost any size if indicated either by deterioration of liver function or impending complications such as hepatoma or life-threatening progression. In comparison with other indications such as extrahepatic biliary atresia, postnecrotic liver cirrhosis or acute liver failure, the results of transplantation in patients with inherited metabolic disorders seem to be better, reaching up to 78-95% actuarial 1-year survival rates. However, lifelong immunosuppressive therapy is necessary. This seems to be acceptable even in disorders with only partial liver function defects.

Humans↗
PubMed1991

The treatment of minimal change nephrotic syndrome: lessons learned from multicentre co-operative studies.

Minimal change nephrotic syndrome (MCNS) is the most frequent type of nephrotic syndrome in childhood. Treatment aims to induce remission, prevent relapses and avoid side-effects. MCNS is responsive to immunosuppressive therapy with glucocorticosteroids, alkylating drugs and cyclosporine A. From results of multicentre controlled studies it became evident that the initial treatment with prednisone should be intensive, i.e. for 12 weeks, in order to reduce the risk of subsequent relapses. Treatment of relapses should be standardized in order to categorize the patient's disease for further treatment. In case "frequent relapsers" develop signs of steroid-toxicity, they should be treated with alkylating drugs: frequent relapsers without steroid dependency with cyclophosphamide or chlorambucil for 8 weeks, steroid-dependent cases with cyclophosphamide for 12 weeks. If frequent relapses re-occur after cytotoxic drug therapy, treatment with cyclosporine A should be considered. Starting dose is 100-150 mg/m2 per day and needs to be adjusted to the whole blood trough level. Treatment with cyclosporine A must be strictly controlled in order to avoid chronic nephrotoxicity. Cyclosporine A does not cure MCNS. It is only effective as long as it is administered; therefore long-lasting treatment is necessary in most cases.

Alkylating Agents↗
PubMed1991

Assessment of pretransplant prognosis in patients with cirrhosis.

The objective of this prospective study was to assess the prognostic value of dynamic liver function tests and traditional methods of evaluating liver function in potential candidates for hepatic transplantation. Patients who underwent orthotopic liver transplantation within the follow-up period of 120 days were excluded. The study included 107 adult and 57 pediatric patients with cirrhosis. Postnecrotic cirrhosis was present in 107 and biliary cirrhosis in 57 of 164 patients. During the follow-up period, 26 of 164 patients died of their liver disease. At the time of inclusion, we recorded monoethylglycinexylidide (MEGX) formation from lidocaine, indocyanine green (ICG) half-life, bilirubin and albumin serum concentration, activity of cholinesterase and alkaline phosphatase, prothrombin time, the clinical complication of ascites, and--in adults--the Pugh score also. These variables were subjected as covariates to a survival analysis (Cox proportional hazards regression model) using separately the data from adults, pediatric patients, all patients with postnecrotic cirrhosis, and all patients with biliary cirrhosis. In all of these four subgroups there was a significant relationship between MEGX and ICG test results and the 120-day survival. In the stepwise analysis, none of the remaining parameters contributed to a further relevant improvement of our predictive ability when added to the values of ICG and MEGX. Our results suggest that the ICG and the MEGX test are superior to conventional liver function tests and the Pugh score in assessing short-term prognosis in cirrhotics independently from the etiology of the underlying liver disease. These findings may have important implications for determining the optimum timing of transplantation.

Adolescent↗
PubMed1991

Conventional therapy for idiopathic nephrotic syndrome in children.

In children, the most frequent type of idiopathic nephrotic syndrome is the minimal-change nephrotic syndrome (MCNS). Its treatment is aimed at inducing remission, preventing relapses and avoiding side-effects. Minimal-change disease is responsive to immunosuppressive therapy. The conventional treatment consists of glucocorticosteroids and the most widely used derivative is prednisone. Initial treatment should be intensive, for example, prednisone for 12 weeks, in order to reduce the risk of subsequent relapse. Treatment of relapses should be standardized in order to categorize the patient's disease for further treatment. If a frequent relapser develops signs of steroid toxicity, alkylating drugs should be prescribed: cyclophosphamide or chlorambucil for eight weeks for frequent relapsers who are not steroid-dependent; cyclophosphamide for 12 weeks for those who are. In steroid-resistant cases, a renal biopsy is indicated and treatment should be administered according to histological changes.

Child↗
PubMed1991

[Biotinidase deficiency. Results of neonatal screening 1985-1989 in Lower Saxony].

During a five-year-period (1985-1989) 420,000 newborns in Lower Saxony, FRG, were screened for biotinidase deficiency using biotinyl-para-amino-benzoic-acid as substrate. Three newborns with profound biotinidase deficiency (activity 1.1%, 2.1%, 2.3% of mean normal activity level) were detected. Nine newborns had partial biotinidase deficiency (activity 17-26% of mean normal activity level), thus giving an incidence of 1:140,000 with profound, and 1:46,667 with partial biotinidase deficiency, respectively. The infants with profound biotinidase deficiency are treated with biotin (2 x 5 mg/day) from the 3rd, 6th and 8th week of life and have developed normally so far. The children with partial biotinidase deficiency are not treated but followed up closely. The necessity of newborn screening for biotinidase deficiency is stressed.

Amidohydrolases↗
PubMed1991

Diagnostic and genetic studies in 43 patients with classic cystinuria.

We present results for laboratory screening and diagnostic tests--cyanide-nitroprusside test, semi-quantitative thin-layer chromatography, and quantitative amino acid column chromatography--of 43 patients with classic cystinuria. We report the efficaciousness of the cyanide-nitroprusside test and of thin-layer chromatography, as compared with quantitative amino acid chromatography, for detecting heterozygotes for type II or III cystinuria. The quantitative results for aminoaciduria in 57 blood relatives in 23 families were used to categorize the index patients with classic cystinuria. By column chromatography the ranges of excretion rates (mumol/24 h per 1.73 m2 body surface area) of diagnostic amino acids in the index patients were as follows: cystine 556-54044 (normal 20-128), arginine 131-11543 (10-80), lysine 768-21848 (51-514), ornithine 185-5685 (0-80). Also by column chromatography the median values for arginine and ornithine excretion in cystine-lysinuric heterozygotes (among the 57 blood relatives) were significantly higher (P less than 0.01) than in controls but never approached the values for homozygotes. The cyanide-nitroprusside test results were positive in urine samples of 41 of 43 index patients and in 16 (51.6%) of the urine samples of 31 obligate heterozygotes with column chromatographically proven cystine-lysinuria. Thin-layer chromatography detected all of the homozygotes, all the compound heterozygotes, and 54.8% of the carriers. According to the type of aminoaciduria in their relatives, 11 patients with classic cystinuria could be classified as having classic cystinuria type I, 11 as having type II or III, and three as being compound heterozygotes. We discuss the implications of these results for correct diagnoses and for genetic studies in classic cystinuria.

Amino Acids↗
PubMed1990

Evidence suggesting hyperoxaluria as a cause of nephrocalcinosis in phosphate-treated hypophosphataemic rickets.

Urinary excretion of oxalate and phosphate was measured in twelve vitamin-D-treated, phosphate-supplemented patients with X-linked hypophosphataemia (XLH; four children, eight adolescents and adults) to investigate possible causative factors of nephrocalcinosis other than calcium. Oxalate excretion correlated highly with urinary phosphate excretion and with intake of phosphate supplements corrected for body surface area. Young children received the highest relative doses of phosphate (range 2.27-10.8 g/1.73 m2 daily) and their urinary oxalate excretion was very high (0.94-3.38 mmol/1.73 m2 daily). The urinary oxalate excretion of untreated adults with XLH was within normal limits. Six patients had evidence of nephrocalcinosis on ultrasound. The high urinary oxalate excretion in phosphate-supplemented XLH may be seen as a special type of enteric hyperoxaluria, in which the conditions of calcium-oxalate crystal precipitation could be reached even at normal levels of urinary calcium excretion. Urinary excretion of both calcium and oxalate should therefore be monitored during treatment in young XLH patients.

Adolescent↗
PubMed1990

Adult height achieved in children after kidney transplantation.

We evaluated posttransplantation growth, bone maturation, and adult height in 20 adolescents who had received kidney transplants at the age of 10.5 to 17 years. Nine patients (five male, four female) were treated with cyclosporine and low-dose prednisolone, and 11 children (six male, five female) were treated with azathioprine and high-dose prednisolone. The cumulative dose of steroids after transplantation was significantly lower in the cyclosporine-treated group. Bone age, according to the radius-ulna-short bones method of Tanner and Whitehouse, was almost the same in both groups at the time of transplantation (15.0 and 14.6 years for male subjects, 13.3 and 13.1 years for female subjects). Predicted adult height (Tanner-Whitehouse Mark II-method of Tanner et al) and target height were estimated at transplantation. Adult height was defined as achieved when bone age in male subjects had reached 18 years and, in female subjects, 16 years. Bone maturation of the cyclosporine-treated patients occurred at a normal rate (0.92 bone-age years per chronologic year), whereas the azathioprine-treated group exhibited a significantly slower rate (0.56 bone-age years per chronologic year). The growth rate per year for the cyclosporine-treated group was more than double that of the azathioprine-treated group (3.0 cm vs 1.4 cm). The adult height of the cyclosporine-treated group exceeded the predicted adult height by a mean of 1.3 cm, but the azathioprine-treated group missed it by 3.9 cm. Target heights could not be achieved in any group. Kidney function was significantly lower in the cyclosporine- vs the azathioprine-treated group, but no patients suffered from severe renal insufficiency. We conclude that cyclosporine and low-dose prednisolone are associated with normal bone maturation and a better prognosis for final height in children with renal transplants.

Adolescent↗
PubMed1990

Efficacy of calcium carbonate and low-dose vitamin D/1,25(OH)2D3 in reducing the risk of developing renal osteodystrophy in children on continuous ambulatory peritoneal dialysis.

Eight children with terminal renal insufficiency on continuous ambulatory peritoneal dialysis were followed for 12 months to evaluate laboratory parameters of mineral ion and bone metabolism. Calcium carbonate (range 47-295 mg/kg body weight per day) was given in combination with low doses of either vitamin D or 1,25(OH2D3. Blood urea nitrogen and serum phosphate concentrations remained well controlled throughout the observation period. A significant increase in serum calcium levels from 2.35 +/- 0.18 to 2.61 +/- 0.22 mmol/l (mean +/- SD) was observed during the first 6 months. Alkaline phosphatase activity and mid-C-regional parathyroid hormone, both indirect parameters of bone metabolism, revealed no evidence of severe secondary hyperparathyroidism. Our data indicate that calcium carbonate may be sufficient to induce relative hypercalcaemia in uraemic children, and thus reduce the risk of developing renal osteodystrophy. Unwanted side-effects of vitamin D preparations, i.e. increased intestinal phosphate absorption and hypercalcaemia after successful renal transplantation, may thus be avoided.

Administration, Oral↗
PubMed1990

Association of spondylo-epiphyseal dysplasia with nephrotic syndrome.

The association of a spondylo-epiphyseal dysplasia and growth failure with the nephrotic syndrome was found in three boys. Renal biopsy performed on two revealed focal and segmental glomerulosclerosis. The nephrotic syndrome occurred at the age of 3-7 years, leading to end-stage renal failure in all patients. Growth failure persisted after successful renal transplantation. This association may represent a distinct disease entity.

Child↗