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Biomedical subjects

J Brodehl

Publications and source records attributed to J Brodehl.

At least 91 records · Page 5Linked to original sources
PubMed1990

One year's experience with recombinant erythropoietin in children undergoing continuous ambulatory or cycling peritoneal dialysis.

Fourteen patients (aged 5.9-22.1 years) undergoing continuous ambulatory or cycling peritoneal dialysis were treated with recombinant human erythropoietin (rhEPO), which was given intravenously once a week at a dosage of 300 units/kg. The mean haematocrit level increased from 18.5% to 27.5% and the reticulocyte count from 19% to 62% within 1 month. After an average time of 3.1 months rhEPO dosage could be adjusted to 100 units/kg per week to keep the haematocrit level at 30%. Only 1 patient had an exacerbation of hypertension, which required a dosage reduction; other side-effects were not noted.

Adolescent↗
PubMed1990

Primary hyperoxaluria type I.

Primary hyperoxaluria type I is a metabolic disorder caused by the deficiency of the peroxisomal alanine:glyoxylate aminotransferase. The disease is inherited as an autosomal recessive trait. The clinical course is outlined based on data from 330 published cases. Diagnostic cornerstones are clinical parameters, urinary excretion of oxalate and glycolate, and the determination of enzyme activity in liver tissue. Principles of conservative treatment, e.g. volume load and pyridoxine substitution, are described as well as experience with different modes of dialysis and transplantation. Kidney transplantation is associated with a high rate of recurrence of the original disease despite excellent management resulting in many instances in early graft loss. Liver transplantation offers the possibility to correct the metabolic defect and to prevent the progression of crystal deposition in the body.

Age Factors↗
PubMed1990

X linked hypophosphataemia: treatment, height gain, and nephrocalcinosis.

The clinical data of 18 patients with X linked hypophosphataemia were analysed retrospectively. The height data were expressed as SD scores. There was no difference in the final height of patients treated with vitamin D (or 1,25-dihydroxyvitamin D) and phosphate for at least two years (n = 12) and that of 16 hypophosphataemic family members who had never been treated. The mean final SD score (-2.07) of treated patients, however, was significantly higher than the value before treatment (-2.79), which indicated an average absolute height gain of 4-4.5 cm compared with the expected height values. Six of the treated patients developed ultrasonographically detectable nephrocalcinosis with normal renal function. The daily phosphate intake and excretion of patients with nephrocalcinosis was significantly higher than that of patients with normal renal morphology. There was no difference in the doses of vitamin D between the two groups. The average urinary calcium:creatinine ratio of the two groups was similar to and below the hypercalciuric 0.6 mmol:mmol limit. The group with nephrocalcinosis, however, had a higher incidence of hypercalciuric episodes than the group without nephrocalcinosis (12 in 130 observations compared with six in 334 observations, respectively). The benefits and risks of treatment of patients with X linked hypophosphataemia must be further evaluated. The high dose of phosphate seems to be an important factor in the development of nephrocalcinosis in this group of patients.

Adolescent↗
PubMed1990

Hypertension after renal transplantation in patients treated with cyclosporin and azathioprine.

The incidence of hypertension was sought in 102 children who had undergone renal transplantation. Fifty five were being treated with cyclosporin and 47 with azathioprine, and they were followed up for a maximum of five years. After one year 35 of those receiving cyclosporin (64%) and 34 of those receiving azathioprine (72%) were hypertensive; after five years the figures were 5/6 (83%) and 25/35 (71%), respectively. Recipients of grafts from living related donors had a lower incidence of hypertension than recipients of cadaveric grafts. The incidence of hypertension was higher in patients with acquired original kidney disease than in children with congenital or familial diseases. In both groups creatinine clearance and the frequency of acute rejection episodes did not differ between normotensive and hypertensive patients. When the lowest concentrations of cyclosporin in whole blood were more than 400 ng/ml the incidence of hypertension one year after transplantation was higher. The incidence of hypertension after renal transplantation in children is higher than that reported in adults. Acquired original disease, transplantation of cadaveric grafts, and nephrotoxicity of cyclosporin are all contributory factors.

Azathioprine↗
PubMed1990

[Chronic recurrent aphthous stomatitis in a 15-year-old carrier of x-chromosome inherited cytochrome b558-negative septic granulomatosis].

A 15-year-old girl, suffering from recurrent stomatitis since the age of 7 years, turned out to be heterozygous for x-linked cytochrome b558-negative chronic granulomatous disease. Two different populations of neutrophils were found in her peripheral blood: one normal (ca. 44%) and one cytochrome b558-negative (ca. 56%) subpopulation which was unable to produce H2O2. There was no history of chronic granulomatous disease in the maternal family line and the blood from the mother and from the grandmother contained a single normal population of neutrophils only. Therefore a recent mutation in one of the x-chromosomes of maternal or paternal origin is most likely. We wish to emphasize that the possibility of a carrier status for chronic granulomatous disease should be considered if recurrent aphthous stomatitis occurs (especially in context with discoid lupus). An easy flow cytometrical method for H2O2 measurement on the single cell level is advised as a screening test [5]. Genetic counselling is a most important consequence of the correct diagnosis.

Adolescent↗
PubMed1990

[Recurrent post-infectious immune complex glomerulonephritis with persistent activation of the alternative complement pathway].

Recurring upper respiratory tract infections in a previously healthy 4 years old boy led to an acute nephritic syndrome requiring haemodialysis. Renal biopsy showed poststreptococcal like immune complex glomerulonephritis with subepithelial humps, C3 deposition and crescent formation. Renal function improved after methyl-prednisolone pulse therapy. Two relapses occurred in the following six months which responded to immunosuppressive therapy. 21 months following the first presentation the boy has normal glomerular filtration rate but persistent glomerular proteinuria and haematuria. Detailed complement analyses revealed a persistent activation of the alternate pathway.

Child, Preschool↗
PubMed1990

[Multicystic kidney dysplasia].

The clinical course of 48 children (27 boys and 21 girls) with multicystic kidney dysplasia was analysed retrospectively. The patients were seen and treated at the Children's Hospital of Medical School Hannover between 1976 to 1989. There was no familial occurrence of the disease, yet in eight families various other renal diseases could be observed. In 20 patients the diagnosis of multicystic renal dysplasia was presumed by prenatal sonographic examination, in the other patients the diagnosis was established at the age between 1 day to 12 years. The first manifestations were palpable abdominal mass (n = 16), urinary tract infection (n = 4), casually because of a sonographic evaluation for other reasons (n = 4) and vomiting (n = 2). Associated malformations were found in 18 patients: cardiac malformations (n = 6), dysplasia of the other kidney (n = 5), ureter obstruction of the other kidney (n = 3), horseshoe kidney (n = 1) and others (n = 3). There was no hypertension and serum creatinine levels were normal in those children, who did not suffer from associated malformations of the other kidney. The multicystic kidney was removed operatively in 42 patients at the age of 3 days to 9.5 years (median 4 weeks). The prognosis of multicystic kidney dysplasia depends on associated renal and other malformations.

Child↗
PubMed1989

Neonatal pyruvate dehydrogenase deficiency with lipoate responsive lactic acidaemia and hyperammonaemia.

A 2-day-old girl developed a severe lactic acidosis with a normal lactate/pyruvate ratio and hyperammonaemia. Plasma arginine and citrulline levels were below the limit of detection. In muscle total pyruvate dehydrogenase complex (PDHC) and pyruvate decarboxylase (E1) activities were reduced to a fraction of lower control values. The acute neonatal period was bridged with peritoneal dialysis, dichloroacetate therapy, supplements of arginine and branched chain amino acids, a complete vitamin B complex and lipoic acid. Lactate homeostasis responded to pharmacological supplements of lipoic acid. At age 1 year the child was hypotonic, showed severe developmental retardation, optic atrophy and cranial dysmorphism. She died aged 1 year 8 months with signs of respiratory paralysis but with normal lactate levels under assisted breathing. Pathological findings at autopsy were suggestive of Leigh syndrome, interstitial pneumonia and extensive fatty infiltration of hepatocytes. Regression analysis of data from 187 plasma amino acid determinations from the patient over a period of 1 year 8 months revealed a persistent-imbalance involving alanine, glutamic acid, glutamine, proline, citrulline and branched chain amino acids. Aspects of acute and long-term therapy in this patient and some implications of the imbalances in plasma amino acids are discussed.

Acidosis, Lactic↗
PubMed1989

[Cystic kidneys in children].

From 1976-1987 a total of 26 infants and children with polycystic kidney disease were treated at the Children's Hospital of the Medical School Hannover. 13 of them suffered from infantile recessive polycystic kidney disease (IRPKD), and 13 from adult dominant polycystic kidney disease (ADPKD). IRPKD was diagnosed at a median age of 0.33 years (range 1 day-13 years), ADPKD at 6.0 years (3 days-14 years). Of those with IRPKD two infants died from bacterial infection and two others developed terminal renal insufficiency at the age of 8 years, while the others are living and 1-20 years old. All those suffer from severe arterial hypertension and have reduced renal function, but only 5 developed signs of liver fibrosis. Of those with ADPKD one infant died from sepsis and renal insufficiency, while the others are well and now 2-17 years old. Only one child needs an antihypertensive treatment. The most important criteria to differentiate IRKPD and ADKPD in children are the genetic transmission, age of first manifestation, hypertension and renal function. The prognosis is much more severe in IRPKD than in ADPKD, but is not as infaust in IRPKD as often assumed.

Adolescent↗
PubMed1989

[Parenteral rehydration treatment of acute diarrhea].

Parenteral rehydration is mandatory if dehydration is severe, vomiting and anorexia prevails, peristalsis is abolished or consciousness disturbed. It has the aim to prevent a circulatory collapse, to fill up the deficit and maintain the requirement until oral feeding is restarted. The principles of parenteral rehydration did not change during the last 20 years. Initially a rapid infusion of isotonic Ringer's lactate solution is mandatory, which usually is followed by half isotonic Ringer's glucose solution. Hypertonic dehydration should be rehydrated very carefully and slowly. During 1976-1986 212 infants and children with severe dehydration were parenterally rehydrated in the Children's Hospital of Medical School Hannover. Dehydration was isotonic in 65.7%, hypertonic in 20.7%, and hypotonic in 13.6%. The parenteral rehydration lasted from 1 to 7 days and was longer necessary in the hypertonic and hypotonic than isotonic states. 4 infants with hypertonic dehydration showed cerebral complications, and 2 of them died. All other patients recovered quickly without acute sequelae.

Child, Preschool↗
PubMed1988

Cyclosporin A treatment in children with minimal change nephrotic syndrome and focal segmental glomerulosclerosis.

In a pilot study 23 children with nephrotic syndrome were treated with cyclosporin A (Cs) for 6-45 months. 8 children suffered from steroid dependent minimal change nephrotic syndrome (MCNS) and had experienced at least one course with cytotoxic drugs, but had relapsed thereafter. 2 children had diabetes mellitus type I with nephrotic syndrome and 13 children had steroid resistant focal segmental glomerulosclerosis (FSGS). Cs was started with 100 mg/m2/day in two doses and increased stepwise to obtain a Cs whole blood trough level of 200-400 ng/ml. In steroid dependent MCNS treatment with Cs reduced relapse rate significantly, and prednisone therapy could be stopped completely. After discontinuation of Cs, relapses reoccurred as frequently as before. Renal function remained unimpaired despite repeated Cs treatment courses up to 38 months. In cases of nephrotic syndrome with diabetes type I Cs treatment led to complete remission without changing the insulin requirement. However, after discontinuation of Cs relapses reoccurred. In steroid resistant FSGS 6 children benefited from Cs treatment: 4 went into complete remission, 2 into partial remission. The 2 children with complete remission relapsed but remained Cs responsive. The remaining 7 children with FSGS did not respond to Cs but continued the course of their disease, with two patients rapidly progressing to terminal renal failure. Side-effects of Cs treatment were mild. It is concluded that Cs is an effective agent in steroid dependent MCNS and can be used as an alternative drug in specific cases like steroid toxicity or diabetes mellitus. In steroid resistant FSGS a trial with Cs seems to be warranted since some cases do respond favorably. To avoid nephrotoxicity treatment with Cs should always be monitored closely by determination of blood levels and renal function.

Biopsy↗
PubMed1988

Renal handling of magnesium in transplanted children under cyclosporin A treatment.

We investigated the renal handling of magnesium in 12 transplanted children under cyclosporin A treatment during the early period after transplantation. We also studied 30 children treated with cyclosporin A 2 years after renal transplantation and compared the results with those of 22 children treated with azathioprine and prednisolone 2-4 years after transplantation. Twenty-two children with chronic renal failure and 10 healthy children served as controls. During the 1st week after transplantation, the mean serum magnesium level dropped to 0.54 +/- 0.14 mmol/l and was accompanied by a high fractional magnesium clearance. During this period two patients showed generalized convulsions. After magnesium substitution, serum levels increased gradually and normalized 4 months later. Fractional magnesium clearance remained slightly elevated due to lower glomerular filtration rates. One to four years after transplantation there were no statistically significant differences in serum levels, clearance, excretion and fractional clearance of magnesium between patients treated with cyclosporin A and those receiving azathioprine. There was a linear regression between magnesium clearance and creatinine clearance and an inverse exponential correlation between fractional magnesium clearance and creatinine clearance. We conclude that cyclosporin A treatment after transplantation has no major effect on the renal handling of magnesium in kidney transplants. The observed changes in serum magnesium levels and fractional clearance are probably due to tubular cell damage early after transplantation and to high doses of furosemide.

Adolescent↗