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Biomedical subjects

M Broyer

Publications and source records attributed to M Broyer.

At least 19 recordsLinked to original sources
PubMed1992

[Reflux nephropathy].

Reflux nephropathy is characterized by the presence at radiological examination of one or several segmental scars in the renal parenchyma, associated with vesico-ureteral reflux. Histology displays a variety of lesions, among which chronic and atrophic pyelonephritis, segmental hypoplasia and renal dysplasia can be individualized. Most of these renal lesions can be prevented by early detection of the reflux which encourages the development and recurrence of urinary tract infection and its diffusion to the upper urinary tract and the kidneys. The finding of a vesico-ureteral reflux with renal lesions, even after spontaneous or surgical regression of this reflux, requires prolonged surveillance in view of the long-term risk of arterial hypertension and renal failure.

Child↗
PubMed1992

Alport syndrome: a genetic study of 31 families.

Thirty one families with Alport syndrome including 3 families with associated syndromes were studied. The location of the COL4A5 gene, responsible for the Alport syndrome, was determined by linkage analysis with eight probes of the Xq arm and by a radiation hybrid panel. Concordant data indicated the localization of the Alport gene between DXS17 and DXS11. Four deletions and one single base mutation of the COL4A5 gene were detected. Homogeneity tests failed to show any evidence of genetic heterogeneity superimposed on clinical heterogeneity for ophthalmic signs and end-stage renal disease age.

Adolescent↗
PubMed1992

Growth rate in children receiving alternate-day corticosteroid treatment after kidney transplantation.

A controlled study of growth in children receiving alternate-day versus daily corticosteroid regimens was performed with 60 children and adolescents with kidney grafts. The study started 14 to 27 months after transplantation in patients with normal graft function, after a graft biopsy. Thirty-five patients were available for the growth study; 17 were randomly allocated to receive alternate-day therapy and 18 remained on a daily regimen. The cumulative dose was similar in the two groups, as were bone age and renal function. After 1 year of follow-up, the mean statural growth, expressed as change in SD score, was significantly better in those on the alternate-day regimen (+0.49 +/- 0.42 SD/yr) than in those on the daily regimen (-0.12 +/- 0.53 SD/yr; p less than 0.005). The difference was also significant when prepubertal and pubertal children were analyzed separately. During the second year of the study most children who were receiving daily treatment were given alternate-day therapy; their mean growth velocity increased to +0.29 +/- 0.35 SD/yr (p less than 0.05 vs the first year); children who had been on the alternate-day regimen since the outset of the study continued to have similar positive SD scores (0.52 +/- 0.37 SD/yr). Renal function remained stable throughout the study regardless of corticosteroid regimen, except in the case of one patient undergoing daily therapy who had a rejection crisis. We conclude that in children with a kidney graft a given cumulative dose of corticosteroid has a significantly lesser inhibitory effect on growth velocity when given on alternate days.

Adolescent↗
PubMed1992

Primary distal tubular acidosis in childhood: clinical study and long-term follow-up of 28 patients.

The long-term follow-up of 28 patients with congenital primary tubular acidosis is described. Ten patients had affected siblings but no history of similar symptoms in the preceding generation. Deafness was associated in 14 patients and developed before 12 years of age. Deafness was present in all familial cases, and patients without deafness showed no familial incidence, suggesting the existence of two different entities. All patients had growth retardation, which was more severe in the older patients and was always markedly improved by alkaline therapy. Rickets was found in some patients but seemed related to vitamin D deficiency. Catch-up growth was limited to the first 2 years of therapy in patients treated before 2 years of age, but sometimes lasted longer in older patients. Of the 12 patients who reached adulthood, those without rickets achieved a normal height but the others did not. We believe that therapy should be continued throughout life because of the risk of nephrocalcinosis.

Acidosis, Renal Tubular↗
PubMed1992

Rehabilitation of young adults during renal replacement therapy in Europe. 1. The presence of disabilities.

The aim of this study was to analyse rehabilitation during RRT in 617 young adults from different European countries who started dialysis or transplantation before the age of 15 years. The data were derived from the EDTA Registry patient data files and a special questionnaire that was sent to centres reporting to the EDTA Registry. The duration of RRT was more than 10 years in 63% of patients. Fifty-four percent were living with a functioning graft and 46% were on dialysis. The prevalence and severity of motor, hearing, sight, and mental disabilities were analysed retrospectively. They were found to vary according to primary renal disease and method of treatment. One-third of patients had one or more disabilities at the start of RRT. Although disability status had changed in many patients by 31 December 1986, some disability remained in one-third of the patients available for study. Disabilities were recorded as mild in the majority of patients. Both improvement and worsening of motor and mental disability occurred more often than changes of hearing capacity and sight. It is concluded that prevention and treatment of disabilities need special attention in children and young adults on RRT in order to improve rehabilitation.

Adolescent↗
PubMed1992

Rehabilitation of young adults during renal replacement therapy in Europe. 2. Schooling, employment, and social situation.

The educational status, employment rate and social situation were studied in 617 patients between 21 and 35 years of age who started renal replacement therapy (RRT) as children. The data were derived from a special questionnaire concerning disability and rehabilitation sent to dialysis and transplant centres reporting to the EDTA Registry. Fifty-six percent of patients completed secondary school and one in three went on to vocational training. Eleven percent of patients attended university, and 16% were reported to have gone to a special school for the handicapped. Up to one-third of patients who attended different school types failed to complete their education. There were notable geographical differences in schooling and in employment. Fifty-six percent of all patients were employed. Lack of schooling was considered to be a major reason for unemployment. Sixty-one percent of patients with disabilities and 34% without disabilities were receiving invalidity payments. The place of residence of these patients aged 21-35 was usually the parental home. Compared to the general population of similar age, only a few patients were married (13.5% of the total study group) and 8% had children. In summary, the present report shows that the major factors influencing rehabilitation on RRT are the presence of disabilities, the method of treatment, geographical factors, duration of RRT, and the underlying primary renal disease.

Adolescent↗
PubMed1992

Successful pregnancies in women on renal replacement therapy: report from the EDTA Registry.

This study reports the geographical incidence of successful pregnancies in women on renal replacement therapy (RRT) and related information on gestation and clinical status of newborns. The impact of successful pregnancy on graft function was assessed by means of a retrospective case-control study. Since 1977 special questionnaires have been sent to each dialysis and transplant centre which reported babies born to mothers on RRT on the yearly centre questionnaire. After 10 years of data collection, a total of 490 pregnancies and 500 babies were available for analysis. A percentage of 88.4 of the babies were born to mothers with a functioning graft, 11.2% to mothers on chronic haemodialysis, and the remaining 0.4% to mothers on CAPD. Almost 50% of all successful pregnancies were reported from the UK. The number of successful pregnancies increased steadily and in parallel with the increasing number of females of childbearing age with a functioning renal transplant. The majority of mothers delivered at age 24-32. For transplanted mothers delivery occurred most commonly during the 3rd and 4th year after successful transplantation. In approximately 85% of cases the duration of pregnancy was shorter than the lower 10th percentile of normal. Birthweight was reduced in accordance with gestational age. Newborn mortality was 1.8%. Fifty-three mothers with a successful pregnancy in 1984-1987 were computer matched with controls according to a number of criteria. The serum creatinine concentration recorded in coded form at the end of each year on the individual EDTA patient questionnaire was used to assess changes in graft function.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1992

Recurrence of primary renal disease on kidney graft: a European pediatric experience.

Of the 4,776 first grafts recorded in the pediatric European Dialysis and Transplant Association (EDTA) registry, 2,113 were reported to have failed and 5.6% of graft failures were related to a recurrence of primary renal disease. Nephrotic syndrome with focal and segmental glomerulosclerosis was the main renal disease prone to recur because recurrence represented 20% of the causes of graft failure in these patients; an even higher proportion was reported in a single-center experience in Europe. Other glomerulonephropathies, such as membranoproliferative glomerulonephritis and Berger's disease, were also reported to be the cause of graft failure by means of recurrence in a proportion similar to focal and segmental glomerulosclerosis. The usual recurrence of primary oxaluria was the cause of close to 50% of graft failure in this disease. Finally, hemolytic uremic syndrome recurred rarely with the graft in the EDTA registry, which is the opposite of what was reported in the United States.

Adolescent↗
PubMed1992

The short-term effect of testosterone on growth in boys on hemodialysis.

In view of a possible resistance to hormones as a cause of growth failure in renal insufficient children, a short-term study was performed to assess the effect of low-dose testosterone administration in boys with end-stage renal failure. Eight boys with a mean age of 11.4 +/- 1.2 years and a mean height deviation of -3.9 SD according to French standards received 50 mg/m2/day of a testosterone or placebo gel in a double-blind crossover fashion, each for a period of four weeks. Growth velocity was assessed by knemometry. The mean plasma testosterone levels rose from 0.16 +/- 0.06 ng/ml to 1.98 +/- 0.35 ng/ml during the testosterone treatment (p less than 0.01), whereas it remained low during the placebo treatment: 0.23 +/- 0.09 ng/ml versus 0.19 +/- 0.06 ng/ml before. The mean growth velocity rose from 0.27 +/- 0.07 mm/week during the control period to 0.50 +/- 0.03 mm/week during the testosterone treatment (p less than 0.05). Two groups of boys could be distinguished: the four boys of the first group whose initial growth velocity was below the mean growth velocity of a healthy control group, showed a significant improvements during the testosterone treatment (p less than 0.01). The growth velocity of the four boys of the second group whose initial growth velocity was normal did not improve during the testosterone treatment. These facts suggest that low dose testosterone can stimulate short-term growth of boys on regular hemodialysis when the basal growth velocity is low.

Administration, Cutaneous↗
PubMed1991

Growth after renal transplantation: correlation with immunosuppressive therapy.

The growth data for children transplanted between 1973 and 1987 were analysed according to their immunosuppressive regimen. All patients treated before 1985 received conventional treatment (prednisone, azathioprine); 37% of the prepubertal children with a follow-up of longer than 2 years showed catch-up growth, and 30% of the pubertal children exhibited a normal adolescent growth spurt. Reduced renal function and corticosteroid treatment are the two main causes of growth delay. The children transplanted between January 1985 and September 1987 were given either triple therapy [cyclosporine (CsA), prednisone, azathioprine] or conventional treatment after randomisation. Growth data were significantly better with CsA. The mean height gain for prepubertal children was +0.24 SD/year on triple therapy and +0.14 SD/year on conventional therapy during the 1st year after transplantation; and 0.4 SD/year and 0 SD/year during the 2nd year (P less than 0.05). The mean height gain for pubertal children was 5.6 cm/year on triple therapy and 3.6 cm/year on conventional therapy (P less than 0.005). The patients on triple therapy also received a significantly lower cumulative dose of prednisone. Some selected patients on triple therapy were taken off prednisone 12 months after transplantation. All patients showed catch-up growth (+0.83 SD/year in prepubertal children, 7.2 cm/year in pubertal children). In conclusion, protocols including CsA and the lowest cumulative dose of steroid (with alternate-day or even steroid withdrawal) allow the best restoration of growth.

Adolescent↗
PubMed1991

Growth, free plasma and muscle amino-acids in uraemic rats fed various low-protein diets.

The nutritional effects of low-protein diets are difficult to assess in humans. Normal and uraemic growing rats were therefore fed: a moderately low-protein (12%) reference diet (diet R), two 5% casein diets, one supplemented with essential amino acids (AA) (diet A) and the other with their keto acids (diet K), and a 7% casein diet isonitrogenous with diet K (diet L). Appetite and growth of both uraemic and control rats were identical on diets R and A and were reduced on diets K and L. Stunting was prominent in rats fed diet L and more severe than in those on diet K. Diet K induced marked anorexia in controls. This effect was smaller in uraemic rats, which were all anorectic, regardless of the diet. Plasma essential AA were similar in rats on diets R and A but low in control rats fed diets L and K. In particular, diet K did not improve the branched-chain AA levels although it produced better growth than diet L. Plasma and muscle threonine were surprisingly elevated in rats on the semi-synthetic diets A and K, despite identical or lower consumptions. Regardless of the diet, uraemia resulted in unchanged or increased plasma essential AA, despite reduced appetite and stunting. Uraemia caused a marked rise in some non-essential AA. Muscle essential AA, except for threonine, were essentially unaltered and did not correlate with growth or uraemia.

Amino Acids, Essential↗
PubMed1991

Plasma growth hormone-binding activity is low in uraemic children.

Plasma growth hormone-binding protein (GH-BP) activity was evaluated in two groups of prepubertal children with chronic renal failure (CRF) who had been treated with recombinant human GH (rhGH). Group 1 consisted of eight children (mean chronological age 10.8 years) with advanced renal failure; group 2 consisted of nine children (mean chronological age 6 years) presenting with end-stage renal disease, who were on dialysis. Before treatment the specific binding of (125I)hGH to high-affinity GH-BP was low in the two groups (group 1, 17.3 +/- 1.6% of radioactivity; group 2, 14.2 +/- 1.4%) compared with the mean value obtained in normal prepubertal children (24.8 +/- 1.7%). No significant changes in GH-BP activity were found during the 1st year of GH therapy, although growth velocity and plasma levels of insulin-like growth factor-I increased significantly in both groups. The low GH-binding activity found in children with CRF supports the state of GH resistance. The reason for the absence of a GH-BP response to GH therapy has to be clarified.

Carrier Proteins↗
PubMed1991

Thrombosis in systemic lupus erythematosus: a French collaborative study.

A retrospective study was undertaken of 120 children with systemic lupus erythematosus (SLE) seen in Paris and its immediate suburbs who fulfilled at least four of the American College of Rheumatology diagnostic criteria for SLE, and in whom the disease was diagnosed before the age of 16 and between January 1975 and December 1987. Eleven of these children (eight girls and three boys) all more than 10 years of age (mean follow up 8.1 years; range 3-13) had thrombotic episodes (9%). Thrombosis was one of the presenting signs in seven patients; in five it was associated with typical symptoms of SLE, and in the other two the thrombotic episode was isolated and diagnosis of SLE was delayed one and three years. Of a total of 16 thrombotic episodes (six of which were recurrent), 14 involved the leg veins, and in four there was associated pulmonary embolism. There were two episodes that affected cerebral arteries. The American College of Rheumatology diagnostic criteria for SLE as well as the incidence of lupus anticoagulant, positive direct Coombs test, and vasculitis in this group of patients was compared with the incidence in patients with SLE but no thrombosis. Only lupus anticoagulant was significantly associated with thrombotic episodes: eight of 11 (73%) of patients with SLE and thrombotic (arterial or venous) episodes had lupus anticoagulant compared with only 10 of 74 patients (14%) with no history of thrombotic events in the same age group.

Adolescent↗
PubMed1991

[Prevention of vascular thromboses after renal transplantation using low molecular weight heparin].

Vascular thrombosis is one of the main causes of early transplant failure in pediatric patients. This paper reports the results of an open trial of the low molecular weight heparin (Enoxaparine) used to prevent renal graft thrombosis in pediatric recipients with risk factors including donor or recipient age under 5 years, multiple arteries supplying the transplant, and positive history for recurrent thrombosis. During 1989, 42 of 67 children given a renal transplant were prophylactically treated with Enoxaparin. Only one transplant was lost to thrombosis among treated patients (1.5%), versus 9 transplants among 73 (12%) children who received their kidney in 1988 without prophylactic Enoxaparin. Risk factors were comparable in both groups of recipients. Enoxaparine therapy was associated with an increased rate of bleeding (12/42) without severe consequences. In conclusion, Enoxaparin is effective in preventing renal graft thrombosis. Availability of this prophylactic therapy makes it possible to use transplants removed from the youngest donors considered as inadequate by some groups.

Age Factors↗
PubMed1991

[Hepatic and renal transplantation in the treatment of type I hyperoxaluria].

Hyperoxaluria type I (HPI) is a metabolic disorder secondary to liver alanine glyoxylate aminotransferase deficiency. Renal failure occurs due to the excessive production and precipitation of oxalate in the kidney. Combined liver-renal transplantation is the correct treatment for this condition when end-stage renal failure occurs as with renal transplantation alone the risk of recurrence of the same pathology in the transplanted kidney would be high. We report the case of a 4 year-old child with HPI suffering from terminal renal failure in whom a hepato-renal transplantation was performed: six months later, creatinine clearance was 62 ml/min/1.73 m2 and liver function tests were normal.

Actuarial Analysis↗
PubMed1991

[Intra-leukocyte cystine in cystinosis treated with cysteamine].

Data on cystine leukocyte content are analyzed in cystinotic patients receiving cysteamine as a depleting agent of cellular cystine. During 2 years, 63 measurements of cystine leucocyte content were performed in 15 cystinotic patients, aged 20 months to 22 years, taking daily 40 to 65 mg/kg of cysteamine. An original method was used to measure leukocyte cystine: a binding protein assay with a specific cystine-binding protein from E. coli. Results were taken individually because of various clinical situations in patients. In 8 patients taking cysteamine regularly, 6 hours after a dose, cystine leukocyte content was between 1 and 2 nmol 1/2 cystine/mg protein, about 10 times less than basal values without treatment and 5 to 10 times more than control subjects. In less compliant patients, cystine leukocyte content was close to basal values without treatment (3 to 25 nmol/mg). Some variability was observed between individuals receiving cysteamine: pharmacokinetic parameters may need further investigation. This sensitive and specific assay helped in controlling compliance and adjusting dosage regimen in each patient. The aim was to maintain a minimum effective dosage in order to avoid toxic side effects of cysteamine.

Adolescent↗