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Biomedical subjects

J Brodehl

Publications and source records attributed to J Brodehl.

At least 55 records · Page 3Linked to original sources
PubMed1993

Twenty-four hour blood pressure monitoring in healthy children.

Blood pressure and heart rate were measured every 20 min during the day and every 30 min during the night in 105 children (51 girls and 54 boys, aged 6-10 years) with a portable automated blood pressure monitor using an oscillometric principle of measurement. The monitor was well accepted by most of the children and the rate of invalid measurements was only 13%. Mean systolic and diastolic blood pressure was 114 +/- 7/72 +/- 5 by day and 99 +/- 7/56 +/- 6 by night. The corresponding heart rates were 93 +/- 8 and 72 +/- 9 beats/min. No significant differences were found between boys and girls. At night, systolic blood pressure dropped by 13% +/- 4%, the diastolic value by 22% +/- 7% and heart rate fell by 22% +/- 6%. Mean systolic and diastolic blood pressure measurements correlated positively with the subject's height, whereas no correlation was found with age.

Age Factors↗
PubMed1993

Growth and pubertal development in nephropathic cystinosis.

In a retrospective investigation growth and pubertal development were evaluated in 30 patients with nephropathic cystinosis. Growth was investigated during the stage of chronic renal insufficiency as well as after successful kidney transplantation and growth rates were related to kidney function. Pubertal development was evaluated in 17 patients between 12 and 25 years of age. Prepubertal growth rates were stable in a range between -2 and -3 height velocity SDS as long as glomerular filtration rate was above 20ml/min per 1.73m2. A decrease in glomerular filtration rate below this threshold was followed by further decrease in height velocity. After kidney transplantation a significant catch-up growth was seen if immunosuppression was performed with cyclosporine A and low dose prednisolone. This did not occur if conventional therapy with azathioprine and high-dose prednisolone was used. Onset of puberty was delayed in all patients. Gonadotropin and oestradiol levels in female patients showed normal fluctuations according to ovulatory cycles. In male patients after puberty there was an increase in gonadotropin levels above the normal range for adult men while testosterone levels remained in the low normal range. These results indicate that adult men with nephropathic cystinosis may develop hypergonadotropic hypogonadism.

Adolescent↗
PubMed1993

Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children. Arbeitsgemeinschaft für Pädiatrische Nephrologie.

Two regimens of steroid treatment for the initial attack of idiopathic nephrotic syndrome (NS) in children were compared in a controlled prospective multi-centre study. Long prednisone therapy consisted of 60 mg/m2 per 24 h for 6 weeks, followed by alternate day 40 mg/m2 per 48 h for 6 weeks. The standard prednisone therapy was 60 mg/m2 per 24 h for 4 weeks, followed by 40 mg/m2 per 48 h for 4 weeks. A total of 71 children with an initial attack of idiopathic NS were allocated at random to the two groups. The cumulative rate of patients with sustained remissions after 2 years was significantly higher after the long course than after the standard treatment (49% vs 19%, P = 0.0079). The mean relapse rate per patient at intervals of 3, 6 and 12 months was lower in the long-course prednisone group than in the standard prednisone group, and the proportion of children with frequent relapses during any subsequent 6 months period was lower in the long-course group than in the standard group (29% vs 57%, P = 0.03). Mild side-effects of corticosteroid therapy were observed more frequently after long-course prednisone treatment. It is concluded that long-course prednisone therapy of the initial attack of steroid responsive NS is preferable to the standard regimen because it reduces the rate of subsequent relapses without increasing the risk for severe steroidal side-effects.

Adolescent↗
PubMed1993

Long-term outcome in 134 patients with galactosaemia.

In a retrospective study 134 galactosaemic patients, born between 1955 and 1989 in the Federal Republic of Germany were traced and their long-term outcome evaluated. We investigated 83 galactosaemic patients (78 homozygotes, 5 compound heterozygotes) by clinical, psychometric and laboratory testing; 31 patients were evaluated by medical history, the remaining 20 patients had died due to sequelae of the underlying disease. In 48 out of 78 classical galactosaemia patients galactose-free therapy had been started before the 15th day, in 19 between days 15 and 56 and in 11 patients after the 56th day. Physical findings revealed that puberty was delayed in 1 out of 18 males and 6 out of 11 females. Neurological abnormalities included ataxia (n = 6), intention tremor (n = 11) and microcephaly (n = 10). Speech abnormalities were found in 43 out of 66 patients over 3 years of age and disturbance of visual perception and/or arithmetic deficits in 29. Intelligence declined with age, i.e., a DQ or IQ less than 85 was found in 4 out of 34 patients less than 6 years of age (12%), in 10 out of 18 between 7 and 12 years (56%) and in 20 out of 24 older than 12 years (83%). Metabolite patterns (RBC galactose-1-phosphate and UDP-galactose, plasma and urinary galactitol) did not correlate with DQ or IQ. Dietary compliance was good in almost all patients. Compound heterozygotes (n = 5) had normal mental and growth development and all laboratory parameters were in the normal range.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

The plasma amino acid profile and its relationships to standard quantities of liver function in infants and children with extrahepatic biliary atresia and preterminal liver cirrhosis.

The absolute and relative concentrations of 16 plasma amino acids in 48 mostly dystrophic infants and children (median of age 1 1/2 years) with extrahepatic biliary atresia and mainly stable preterminal cirrhosis were compared with those of controls. Patient plasma amino acid data were analysed statistically for diagnostic usefulness and correlated with standard biochemical quantities of liver function and of liver perfusion. In the patients the total amounts of non-essential and essential amino acids were reduced by 19% and with the same significance (p < 0.0005). Plasma tyrosine was increased (+40%), while taurine (-44%) and branched chain amino acids (+28.8% to -34.7%) were decreased. Methionine values varied widely. In the molar fractional plasma amino acid profile, only alanine, valine, and leucine were decreased, while threonine, methionine, tyrosine, phenylalanine, ornithine, and serine were increased. Discriminate function analysis showed that the plasma amino acid data discriminated 93.8% of the patients from controls. The concentrations of some amino acids in plasma seemed to have been influenced by protein-calorie deficiency in the patients. The valine/tyrosine ratio and the Fischer index (ratio branched chain/aromatic amino acids) were significantly reduced in the patients versus controls (1.54 +/- 0.55 vs 3.08 +/- 0.55 and 1.66 +/- 0.39 vs 3.00 +/- 0.48). A number of significant correlations (range of r: 0.37-0.59, p < 0.05, 30-48 data pairs) were calculated between plasma amino acid data and several standard biochemical quantities of liver function. The statistical analyses also showed that the Fischer index began to decrease gradually and linearly early in the progression of liver failure.(ABSTRACT TRUNCATED AT 250 WORDS)

Amino Acids↗
PubMed1993

[Physical and mental development of children with congenital hypothyroidism].

69 children with congenital hypothyroidism, who were detected by neonatal screening in Lower Saxony, were reevaluated 1-12 years after diagnosis. They had been treated either by regional children's hospitals, local pediatricians or general practitioners. Substitution of thyroid hormone had started for the majority between day 7 and 14, for 17%, however, only later. Symptoms suggestive of hypothyroidism at birth were observed in 35 children, but only 3 cases were correctly diagnosed before the result of the screening was known. Further diagnostic tests to elucidate the cause of congenital hypothyroidism had been performed in 33 children. In 36 cases the etiology remained undiagnosed at the evaluation. Hormone therapy had been administered continuously in all cases. The somatic development of all children was normal. Bone age at the time of diagnosis was retarded in more than 50%, later it became normal in most cases. The psychomotor and intellectual development was satisfactory as assessed by psychometric tests. The mean value of the Intelligence Quotients in the children older than six years was 96.3. 5 children of this age group had an IQ below 85 and only one child had an IQ of more than 115. In the younger group the results were similar, but children younger than 4 years showed deficiencies in speech development. In summary, the somatic development of the re-examined children with congenital hypothyroidism was normal, but the psychomotor and intellectual development was only subnormal in some cases. It is therefore suggested that children with congenital hypothyroidism should be closely followed by experienced pediatricians, especially in the early years of life.

Age Determination by Skeleton↗
PubMed1992

Paediatric aspects of renal transplantation: experience of a single centre.

From 1970 to 1991 a total of 244 renal transplantations were performed in 203 children at the Medical School in Hannover. The mean patient age was 10.4 years with a range between 11 months and 16.9 years. Fifty-nine children received a living donor graft from one parent and 144 received cadaveric grafts. Forty-two children were transplanted without prior dialysis treatment. After 20 years the overall survival rates were 86% for the patients and 39% for the first grafts. Grafts from donors below 5 years of age had a less favourable survival (44% after 5 years). Pre-emptive transplantation yielded comparable results with the benefit of a shorter period of uraemia. Hypertension developed in 80% of transplanted patients. Only children with living related donor grafts had significantly less hypertensive problems independent of the immunosuppressive regimen. Post-transplantational growth improved under cyclosporin. Children with nephropathic cystinosis also showed catch up growth after transplantation under cyclosporin. The long-term outcome and rehabilitation of grown-up recipients were encouraging.

Adolescent↗
PubMed1992

Pre- and post-transplant assessment of liver function in paediatric liver transplantation.

The pre-operative risk of paediatric liver transplantation candidates (n = 41) was assessed in a prospective study by means of clinical symptoms, conventional static and liver blood flow dependent dynamic liver function tests. Nine patients died during the 365-day waiting period. The data were subjected as covariates to a survival analysis in the Cox proportional hazards model. There was a significant relationship between the results of mono-ethylglycinexylidide (MEGX) formation and ICG test and the 365-day survival rate. In the stepwise analysis, none of the remaining parameters improved the predictive ability when added to the dynamic liver function test results. The assessment of post-transplantation liver function was studied in 27 patients during the first 28 postoperative-day period. In addition, liver function was studied in a cross-sectional study 1-7 years after successful liver transplantation in children with complete or partial rehabilitation. In the early postoperative period severe organ damage was indicated by both static and dynamic liver function tests. In the later course after transplantation no deterioration of liver function measured with MEGX formation was to be observed. These findings demonstrate the usefulness of dynamic liver function tests in the pre- and post-transplant assessment of liver function.

Child↗
PubMed1992

Long-term results of cyclosporin A therapy in children.

In order to assess the long-term effectiveness and tolerability of cyclosporin A (CsA) treatment in children with renal transplantation (Tx), the follow-ups of 32 children (17 boys, 15 girls; age at Tx = 12.2 yr; range, 5.1 to 16.9) with a functioning graft of greater than or equal to 5 yr and continuous treatment with CsA and low-dose prednisolone are analyzed retrospectively. For comparison, data of 34 children (19 boys, 15 girls; age at Tx, 11.0; range, 3.2 to 17.1) are collected who had received a graft before the introduction of CsA, had at least 5 yr of graft function, and were continuously treated with azathioprine (AZA) plus high-dose prednisolone. The mean observation period in the CsA group was 6.5 (range, 5.0 to 8.0) yr, and in the AZA group was 10.4 yr (range, 5.7 to 15.8). CsA dosage remained unchanged in the range of 200 mg/m2/day; CsA whole blood trough level was 120 to 130 ng/mL throughout the years. One patient died in each group. Four more grafts were lost in the CsA group by chronic rejection, which was associated with noncompliance in three, and two grafts were lost in the AZA group by chronic rejection. Late acute reversible rejection episodes occurred more frequent in the CsA (six) than in the AZA group (two). The overall survival rates for patients and grafts were significantly better with CsA. The graft function in CsA-treated recipients was significantly lower than that in AZA patients, but there was no progressive loss over the years.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Continuous peritoneal dialysis in children.

During the period from June 1985 to December 1991, 48 children were treated with continuous peritoneal dialysis (CPD) in our centre because of acute renal failure. The median age was 1.8 years (range 0.01-17.1). The most common diagnoses were: hemolytic uremic syndrome (n = 22), anuria after cardiac surgery (n = 7), and septicemia with multiorgan failure (n = 7). Kidney function recovered in 35 (73%); 13 (27%) died of their original disease. One further patient with HUS recovered from dialysis but died of cerebral complications shortly afterwards. One patient remained anuric and requires renal replacement therapy. Hyperkalemia, when present initially, and uremia could be controlled adequately in all cases. However, ultrafiltration posed problems when cardiac output was low. Peritonitis occurred in 11 patients; in 8 children the Tenckhoff catheter had to be revised because of leakage (5), flow problems (2), or bowel perforation (1). CPD proved to be an excellent method to treat acute renal failure in children of all age groups. The rate of complications was acceptable.

Acute Kidney Injury↗
PubMed1992

[Has post-streptococcal glomerulonephritis disappeared?].

We report on 21 children with poststreptococcal glomerulonephritis (PSGN). The diagnosis was based on clinical and laboratory criteria. 19/21 had a clinically apparent throat infection initially, but only 6 received antibiotic treatment. Initial symptoms were: macrohematuria (20/21), edema (10/21), arterial hypertension (6/21), reduced creatinine-clearance (11/21), and proteinuria (18/21). No Patient was dialyzed. After an interval of 0.3 to 25 months 19 children are asymptomatic. All patients have normal creatinine-clearances; none is hypertensive. The present data show that PSGN has not disappeared in Mid-Europe. Remarkable acute disturbances occurred, which healed in almost all cases. The importance of accurate bacteriologic diagnosis and adequate therapy is emphasized.

Acute Kidney Injury↗
PubMed1992

[Clinical spectrum of nephrotic syndrome].

AIM OF STUDY: A wide spectrum of glomerular diseases manifests as a nephritic syndrome with haematuria, proteinuria, hypertension, edema, and impaired renal function. Little is known about the presentation of each symptom and the distribution of the underlying glomerular diseases. METHODS: In order to delineate the clinical spectrum of glomerular disease presenting as a nephritic syndrome, we examined the records of 192 patients who had been followed in our paediatric nephrological outpatient clinic between 1973 and 1988 for a nephritic syndrome. RESULTS: Oligosymptomatic courses with microhaematuria and proteinuria predominated. The mean age at presentation was 8.1 years. In a broad spectrum of 29 diagnoses, postinfectious glomerulonephritis and Henoch-Schönlein nephritis were the most prevalent. End-stage renal failure developed in 16% of the patients. Prognosis was poor in cases of rapidly progressive glomerulonephritis, focal segmental glomerulosclerosis, Henoch-Schönlein nephritis and in autoimmune disease, with end-stage renal failure developing in more than 20% of each of these groups. CONCLUSIONS: The nephritic syndrome often manifests oligosymptomatically. An intensive nephrological workup is indicated in order to early diagnose serious--and potentially treatable--glomerular diseases.

Adolescent↗
PubMed1992

[Unilateral renal artery stenosis. Color-coded Doppler sonography and captopril scintigraphy in a 13-year-old patient].

Modern noninvasive techniques, such as doppler sonography or color-encoded doppler sonography, have only rarely been used for diagnosis of renovascular hypertension in children. In the following case report, we describe the successful diagnosis of renovascular hypertension in a 13-year-old girl by using color-encoded doppler sonography and Captopril renal scintigraphy. The patient was admitted with hypertension of 180/130 mmHg. Laboratory findings showed elevated plasma renin and aldosterone concentrations. No abnormalities were found by abdominal sonography, isotope renography, intravenous pyelography, or in venous digital subtraction angiography. However, subsequent color-encoded doppler sonography clearly showed evidence of an artery stenosis of the right kidney. Furthermore, isotope renography one hour after oral administration of captopril revealed an almost complete loss of glomerular filtration rate of the right kidney. Based on these findings, arterial digital subtraction angiography, including transluminal angioplasty, was performed. During this treatment procedure, the right renal artery stenosis could be confirmed and was subsequently dilated without complication. In the following twelve months the patient remained normotensive and required no further antihypertensive drug treatment.

Angiography, Digital Subtraction↗