Search PubMed⌕ Search

Biomedical subjects

J Brodehl

Publications and source records attributed to J Brodehl.

198 records · Page 11Linked to original sources
PubMedDate not supplied

[Long-term course of 6 boys with congenital nephrogenic diabetes insipidus].

From 1975-1986 6 boys with congenital nephrogenic diabetes insipidus were diagnosed at the age of 3 months to 10 years. Symptoms appeared within the first few weeks of life. The diagnosis was confirmed by polyuria, low urinary osmolality (97-225 mosm/kg H20), hypernatraemia (max. 171 mmol/l) and the missing response to vasopressin. The treatment was variable; 4 boys received only hydrochlorothiazide (2-2.5 mg/kg/d) which lead to a reduction of the daily urinary volume of 26-44%. Hyperelectrolytaemia disappeared and a normal thriving could be achieved. Later an additional treatment with indomethacin (2 mg/kg/d) was necessary in 3 boys because of an increase of polyuria; there was a further reduction of the daily urinary volume of 50-60%. The combination of hydrochlorothiazide and indomethacin in the treatment of the congenital nephrogenic diabetes insipidus was well tolerated and seems to be--especially during the first few years of life--a necessary and effective treatment which allows a normal thriving and psychointellectual development.

Adolescent↗
PubMedDate not supplied

[Psychosocial aspects of the treatment of phenylketonuria].

The psychosocial aspects of phenylketonuria (PKU) were investigated retrospectively in the families of 25 children and adolescents with classical PKU. With the help of a semistructurated questionnaire the parents were asked in interviews what impact the birth, the diagnosis of PKU in their child and the treatment itself had on them. 19 of the children had been detected by newborn screening and given early treatment. With one exception they all showed adequate intellectual development. Six other children were diagnosed late and only three of them had been given dietary treatment with some degree of success. Many of the parents reacted with shock and disbelief after being informed that their child was abnormal. In many ways the parents expressed retrospectively the wish that they had been better informed about their childs disease and had more intensive support from the medical professionals. They believed that with these prerequisites they would have been more competent in the case of their children. The data demonstrate the necessity of psychological care for the entire family of the chronically sick child.

Adaptation, Psychological↗
PubMedDate not supplied

The treatment of minimal change nephrotic syndrome (lipoid nephrosis): cooperative studies of the Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN).

Minimal change nephrotic syndrome (MCNS) is the most frequent type of nephrotic syndrome (NS) in childhood. During the last decade only little progress has been made in the understanding of the etiology and pathogenesis of MCNS. However, treatment has been improved pragmatically by controlled cooperative studies, which have lead to an unexpected degree of standardization in pediatric nephrology. The Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN) is conducting its own cooperative studies in close contact with the International Study of Kidney Diseases in Children (ISKDC). In Steroid Study II of APN two regimens of initial prednisone treatment are compared: standard initial versus short initial prednisone therapy. Preliminary results reveal that a complete remission of NS can be obtained with less than half of the prednisone dose usually applied by the standard regimen. The relapse rate after initial treatment, however, seems to be the same in both groups. In the Cytotoxic Drug Study I of APN the effectiveness of chlorambucil (0.15 mg/kg for 8 weeks) or cyclophosphamide (2 mg/kg for 8 weeks) in the treatment of steroid toxic frequent relapsers without steroid dependency (FRNS) and with steroid dependency (SDNS) are compared. In FRNS the treatment is followed by long lasting remissions in most cases, while in SDNS relapses occur very soon again. The latter group, therefore, does not profit from cytotoxic drugs in the dosage used. In a new trial, therefore, it is asked whether patients with SDNS will profit more from prolongation of cyclophosphamide treatment for 12 weeks.

Child↗
PubMedDate not supplied

[Screening for congenital metabolic diseases in mentally retarded patients in 6 psychiatric institutions of Lower Saxony].

The results of screening for inborn errors of metabolism in patients in six institutions for the mentally retarded in Lower Saxony are presented. A battery of standard chemical and chromatographic tests for amino acids, carbohydrates and mucopolysaccharides in urine were employed. Combined gas chromatography-mass spectrometry was used to detect organic aciduria. All together 975 patients - 347 females and 628 males - aged from 5 to 93 years were studied. 78.6% of the patients were over 20 years of age. There were positive findings in 56 cases: phenylketonuria (n = 8), alcaptonuria (1), pentosuria (5), galactosuria (2), tryptophanuria (1), xanthurenic aciduria (3), mucopolysaccharidosis type III A (1), hyperglycinemia with hyperglycinuria (1), renal hyperaminoaciduria (9, lactic aciduria (3), 4-hydroxyphenylaceticaciduria (2), organic aciduria (12) and mellituria (8) of unknown type. The organic acids most often detected in urine were lactic, 4-hydroxyphenylacetic and 3-hydroxybutyric acids. Only 20 of the patients (2.0%) excreted appreciable amounts of benzoic acid in urine. The results are discussed and compared with other investigations.

Adolescent↗
PubMedDate not supplied

[Long-term ursodeoxycholic acid treatment of cholestatic liver diseases in childhood--clinical and biochemical effects].

BACKGROUND: In adults with chronic cholestatic liver disorders, controlled studies have shown a reduction of clinical, biochemical and possibly histological parameters during long-term medication with ursodeoxycholic acid (UDCA). It is not yet clear, however, whether similar effects can be achieved in children. Therefore, we retrospectively evaluated the use of UDCA in typical liver diseases of childhood. METHOD: 20 children were treated for at least 6 months (age at start of therapy 5-87, median 24 months; diagnosis: biliary atresia n = 10, Alagille's syndrome n = 4, intrahepatic biliary hypoplasia n = 3, Byler disease n = 3). Pruritus, liver cell injury, cholestasis, synthetic liver function and weight and height for age before medication with UDCA (7-26, mean 13 mg/kg BW/d) was compared to values after 3, 6, 12, 18 and 24 months of therapy, with special attention towards possible adverse effects. RESULTS: No adverse effects of UDCA necessitating modification of therapy were encountered. During the first year of medication, weight for age improved in 15 patients, but pruritus in only four. During UDCA treatment, GIDH and gamma GT decreased significantly. GOT and GPT declined in the majority of patients. No significant changes of bilirubin and parameters of liver synthesis were seen. CONCLUSION: Long-term medication with UDCA appears to be safe in children. Thus, controlled studies of UDCA medication in children are justified, and are urgently needed to further investigate the prognostic significance of the positive effects of UDCA identified in this retrospective analysis.

Alagille Syndrome↗
PubMedDate not supplied

Four years' experience with cyclosporin A in pediatric kidney transplantation.

From 1982 to 1987 sixty-three children were treated with cyclosporin A and low dose prednisolone after kidney transplantation. Patient survival rate at 4 years after transplantation was 98.3%, survival rate of living related grafts 100% (n = 10), and survival rate of cadaveric grafts 73% (n = 53). Adequate cyclosporin blood levels were achieved in all children with a dosage regimen related to body surface area. Major concerns during the observation period were the loss of glomerular filtration rate from 51.8 to 40.5 ml/min/1.73 m2, a hypertension rate of 77.8%, and hyperuricemia. Cyclosporin A-side effects were mild. Infections occurred in 11.1%. Growth retardation in prepubertal children improved by 0.74 standard deviations of normal height, and in pubertal children by 0.51. We conclude that cyclosporin A treatment in children enables excellent long term graft survival rates with improved growth rehabilitation, however, the prevention of the cyclosporin associated nephrotoxicity and hypertension remains the major problem.

Adolescent↗