Search PubMed⌕ Search

Biomedical subjects

M Broyer

Publications and source records attributed to M Broyer.

At least 235 records · Page 13Linked to original sources
PubMed1987

Ocular changes in long-term evolution of infantile cystinosis.

Infantile cystinosis is an autosomal recessive lysosomal disorder of aminoacid metabolism leading to a storage of cystine crystals in the cells of many tissues, but mainly in the kidney and the eye. The ocular symptoms and long term evolution were studied in a series of 25 patients at the Enfants Malades Hospital. The follow-up over 26 years demonstrated that infantile cystinosis affected mainly corneal and retinal epithelium just as it affected the kidney epithelium. Corneal involvement was a constant finding after one year of age. It induced photophobia, which appeared between three and four years when a superficial punctate keratopathy appeared. A characteristic retinopathy was observed as early as three years of age. It was constantly present at seven years of age and caused loss of vision. When ERG results were compared to the most recent measurement of visual acuity, a correlation was observed between retinopathy and visual defect. For this reason, no corneal grafts were performed. The use of topic cysteamine appeared promising but no definitive conclusions could be made.

Adolescent↗
PubMed1987

[Urinary diversions and intestinal grafts in renal transplantation in children. Report of 8 cases].

In a series of 400 transplantations, 8 children presented a severe lesion of the lower urinary tract requiring an intestinal graft to divert the urine or to replace the useless bladder: 4 cases of posterior urethral valves and 4 cases of neurogenic bladder. Four permanent urinary diversions and 4 bladder enlargements, including 3 temporary diversions, were performed. The sigmoid colon was used in 5 cases and the ileum was used in 3 cases. The preparation of the intestinal graft was always performed prior to the transplantation. The uretero-intestinal anastomosis included an antireflux device in 3 of the 8 cases. There were no deaths in this series. Three occlusions and 2 lymphoceles occurred in the immediate post-transplantation period, but were corrected by re-operation. Two cases of calculi occurred in the long term, one of which required operation. Moderate stenosis of the renal artery in one case resulted in systemic hypertension which was well controlled by medical treatment. An episode of acute pyelonephritis resolved rapidly with antibiotic treatment. Two cases of acidosis were corrected by salt supplementation. There was one case of early graft rejection on the 15th day and a delayed rejection after 18 months, leading to removal of the transplant. At the present time, 6 renal grafts function normally (75%) with a follow-up of 1 to 8 years.

Adolescent↗
PubMed1986

Role of amount and nature of carbohydrates in the course of experimental renal failure.

The renal effects of carbohydrates (CHO) were studied in two experiments. 1) The effects of CHO-energy restriction was evaluated by comparing uremic growing rats (initial weight: 80 g) fed "ad lib" (L rats) or CHO-restricted (starch and glucose) but receiving identical amounts of all other nutrients (R rats). R rats showed reduced growth, slower increase in plasma creatinine, lower mortality rate, and less histological renal damage than L rats. 2) Two types of CHO restriction, low glucose (R1 rats) or low starch (R2 rats) were compared to "ad lib" feeding (L1 rats) in adult rats (initial weight: 130 g). Growth was identically reduced in R1 and R2 rats. Mean plasma creatinine levels at week four was lower in R1 than in L1 rats. The overall rate mortality was higher for L1 and R2 than in R1 rats (79%, 81%, 53%) but included deaths from other causes than renal failure. Actuarial survival excluding these deaths was 27%, 83% and 10% in L1, R1 and R2 rats, respectively. Diffuse renal lesions were found in 25 of 30 L1, 5 of 15 R1, and 12 of 15 R2 rats (R1 vs. R1 and R2, P less than 0.01). The results show that CHO restriction may preserve the renal parenchyma, and suggest that restriction of "simple" rather than "complex" CHO restriction may be beneficial, a finding which could be of clinical importance if confirmed by further investigations.

Animals↗
PubMed1986

Efficiency of substitution of 2-ketoisocaproic acid and 2-ketoisovaleric acid in the diet of normal and uremic growing rats.

Effects of various intakes of the ketoanalogues of leucine (KICA) and valine (KIVA) on growth, nitrogen, and urea excretion were examined and compared to those of an optimal intake (A) of the corresponding amino acids. Diet KICA and KIVA contents varied from 1 to 4 times A. In controls, growth was significantly reduced with equimolar substitution, corrected with twice A, and unchanged at higher levels. Doubling KICA corrected growth except with substantial anorexia. In uremic rats fed KIVA, growth was corrected at twice A. Low-KICA diets reduced plasma-leucine level; higher KICA diets normalized plasma leucine and revealed branched-chain amino acid (BCAA) antagonism. Changes in 2-ketoacids were unrelated to those of BCAA. In uremia, KICA decreased plasma and urinary urea without changing nitrogen retention. Ketoacid substitution for amino acids was 50% efficient in normal rats and not altered by uremia. BCKAs, specifically KICA, could modify urea metabolism.

Animals↗
PubMed1986

Cerebral atrophy and nephropathic cystinosis.

The management of end stage renal failure in cystinotic children is correlated with a longer survival, sometimes complicated with neurological abnormalities. Cranial computed tomography was performed in 10 patients and showed a significant atrophy; the pathogenesis of this damage remains unclear.

Adolescent↗
PubMed1986

Pulmonary calcifications in children on dialysis.

Pulmonary calcifications are known to occur in patients with chronic renal failure. Recently, scintigrams with bone-seeking radionuclides have been used to detect subclinical pulmonary calcium deposits. We studied 18 children on maintenance dialysis without evidence of pulmonary calcification on chest X-ray. Four children (22.2%) had a positive technetium 99m hydroxymethylene diphosphate scan (group 1), and 14 children had a negative scan (group 2). Mean serum aluminum levels were 2.68 +/- 0.30 mumol/l (mean +/- SD) in group 1 as compared to 1.66 +/- 0.72 in group 2 (p less than 0.01). No significant difference was found between the groups with respect to serum levels of calcium, phosphorus, bicarbonate, magnesium and the calcium-phosphorus product as well as parathyroid hormone and vitamin D levels. The patients with pulmonary calcifications were on dialysis a significantly longer time than those of group 2 (62 +/- 15 versus 35.7 +/- 23 months; p less than 0.01). These data show that pulmonary calcification occurs with high frequency in children undergoing long-term dialysis. They seem to be related to high serum aluminum levels. We propose that pulmonary scintigrams with bone-seeking radionuclides be used routinely for the diagnosis and follow-up of uremic pulmonary calcification.

Adolescent↗
PubMed1986

[Radioisotopic quantification of kidney function using Tc-99m-DMSA. Comparison with creatinine clearance in children with a single kidney].

To assess the accuracy of renal function quantification with Tc 99m-DMSA in children, we compared DMSA renal uptake and creatinine clearance in 16 cases of children with single kidney. The age of the patients ranged from two month to fourteen years. DMSA renal uptake was measured 7 hours after injection and was normalized in percent of the injected activity. A significant correlation was found between creatinine clearance and DMSA uptake (Pearson's r = 0.866, p less than 0.01). Normal creatinine clearance in children (80 to 120 ml/min-1 X 1.73 m-2) allowed determination of normal renal uptake (36 to 60%). This study indicates that in cases of asymmetrical renal impairment renal uptake reflects split renal creatinine clearance. Since the former is much easier to measure, DMSA should play an important role in the evaluation of differential renal function.

Adolescent↗