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Biomedical subjects

M Broyer

Publications and source records attributed to M Broyer.

431 records · Page 24Linked to original sources
PubMedDate not supplied

[Hereditary chorioretinal degeneration and nephronophthisis. The role of Senior-Löken syndrome].

Systematic clinical ocular examinations completed by electrophysiological studies were performed on fifty-five children with nephronophthisis. Twenty children, all under ten years of age, had tapetoretinal degeneration, either pure in fourteen cases (Senior-Löken syndrome), either associated with extra-oculorenal abnormalities in six cases (bones, liver, neurology). Twelve older children had a normal examination. Twenty-three children had a normal clinical examination, but non evolutive alterations of ERG.

Adolescent↗
PubMedDate not supplied

[Parathyroidectomy in children with renal failure. Retrospective study of 17 cases].

Parathyroidectomy (PTX) is rarely needed in children with end-stage renal disease and the new forms of vitamin D decrease its frequency. We report 17 cases of patients (14 with dialysis and 3 with functional renal graft) suffering from renal insufficiency in the pediatric age group who required a PTX. The surgical indications and techniques (partial PTX, total PTX with or without graft) varied with time and circumstances. After partial PTX, 3/10 patients were reoperated on. After PTX + graft one patient needed a second graft and another a graft curettage. Whatever the method, we noted normalization of clinical and radiological signs and a decrease in biological signs. The growth rate improved in most children during puberty, but outside of this period, no effect was noted. There is a definite indication for a PTX in cases with tertiary hyperparathyroidism. The indication seems questionable in cases with secondary hyperparathyroidism which can now be effectively treated with 1 alpha-hydroxylated forms of vitamin D. When necessary, total PTX with or without graft should be performed.

Adolescent↗
PubMedDate not supplied

[Pharmacokinetics of prednisone after oral administration in children with renal grafts. Changes induced by phenobarbital and renal insufficiency].

A study of blood and urinary clearance of prednisone after ingestion of a 25 mg/m2 body surface test-dose, at 8 AM, was undertaken in 20 children treated for 18 months with prednisone after renal transplant. Results show important variability between patients: the elimination half life was 2.70 +/- 0.78 hr.; Tmax time to reach Cmax was 2.10 +/- 1.08 hr.; Maximal concentration (Cmax) was 474 +/- 153 ng/ml. With respect to the dose of steroid administered, the urinary excretion of corticosteroids: 17-hydroxycorticosteroids was 12.9 +/- 7.4% and that of unchanged prednisolone 2.8 +/- 3.1%. This level was essentially achieved in the 6 first hours: 55.4 +/- 16.2% for 17-OH steroids and 87.2 +/- 14.3% for prednisolone. Two points emerge from this study: (a) Renal failure slows urinary excretion of prednisone and its metabolites, making a reduction in the doses of corticosteroids necessary at certain doses. (b) The association prednisone-phenobarbital changes the blood kinetics (excretion is faster) without a clear change in 17-OH steroid and prednisolone urinary excretion. It is associated with a decrease in graft tolerance. The kinetic changes do not seem to be the only factors implicated in the decreased therapeutic response.

Administration, Oral↗
PubMedDate not supplied

Nitrogen mustard therapy in idiopathic nephrotic syndrome of childhood.

The effects of intravenous Nitrogen Mustard (NM), given with a total cumulative dose of 0.8 mg/kg BW, were studied in 60 children with idiopathic nephrotic syndrome (INS). Thirty patients were frequent relapser with corticosteroid dependence. In this group the actuarial remission rate was 43, 30 and 15% after 1, 2 and 3 years respectively. The relapse rate per year was decreased from 2.76 +/- 1.56 to 0.88 +/- 0.79 after NM. Ten of the 17 patients showing a partial response to steroids went into complete remission after NM but 6 of them relapsed. Of the 13 patients who did not respond to steroids only 3 went into remission for a short time. Vomiting and leukopenia were noted in one third of cases. The data indicate that NM may improve the course of corticosteroid dependent INS, being less effective in partial responders and probably without effect in non responders.

Adolescent↗
PubMedDate not supplied

Modification of peritoneal ultrafiltration capacity in children undergoing peritoneal dialysis.

Eleven children (7 girls and 4 boys) 2 1/2 to 17 years and 8 months of age were treated with CAPD for periods ranging from 6 to 31 months. All children were treated with commercially available dialysate solutions containing lactate. Peritoneal ultrafiltration capacity (PUFC) decreased progressively in all children without accompanying decrease in peritoneal urea and creatinine clearances. Five children developed membrane failure with negative ultrafiltration. One episode of peritonitis occurred in one of these 5 children and in 4 of them only 1.5% glucose solutions had been used. After an initial period (ranging from 14 to 31 months) of CAPD, 2 children were treated with Intermittent Ambulatory Peritoneal Dialysis (IAPD) and two others with Intermittent Cycling Peritoneal Dialysis (ICPD). In these 4 children, PUFC increased within one month from -3.75 ml/kg/day to + 5 ml/kg/day. By providing a shorter dwell time, IAPD and ICPD may allow a reduction in net inward transport of glucose, the maintenance of osmolar gradient and preservation of ultrafiltration capacity. Furthermore, periods of rest may allow some recovery from the progressive deterioration of the peritoneum resulting from long-term irrigation of the peritoneal cavity. These results indicate that IAPD and CPD may be superior to CAPD to maintain the ultrafiltration capacity of the peritoneum.

Adolescent↗
PubMedDate not supplied

Diffuse arterial calcified elastopathy--a new cause of renovascular hypertension in children.

Diffuse arterial calcified elastopathy was observed in 6 pediatric patients presenting with severe renovascular hypertension. Renal ultrasonography showed a characteristic pattern, the dotted corticomedullary junction, related to the increased echogenicity of the interlobar and/or arcuate arteries. Superficial temporal artery biopsy demonstrated the presence and the extension of the calcifying process involving the elastic layers of the muscular arteries. This clinicopathological syndrome may be heterogeneous from an etiological point of view: etiologic investigations led to the diagnosis of pseudoxanthoma elasticum in 2 patients; no etiologic cause could be found in the others.

Arterial Occlusive Diseases↗
PubMedDate not supplied

[Association of early-onset nephrotic syndrome and microcephaly. Apropos of 4 cases in 2 families].

The authors report 4 cases in 2 different families of a syndrome characterized by nephrotic syndrome of early onset (during the first 2 years of life) and microcephaly. Such an association was previously reported in 5 cases. In 4 it was familial. The study of families suggests an autosomal recessive transmission. Microcephaly was associated with psychomotor retardation, sometimes dysmorphic facies and various neurologic abnormalities. The nephrotic syndrome was characterized by its early onset and prognostic severity. However, the renal histologic lesions were heterogeneous: either minimal glomerular changes with focal and segmental hyalinosis or mesangial sclerosis, or, so-called "microcystic dysplasia". This heterogeneity does not suggest a single genetically determined disorder.

Age Factors↗
PubMedDate not supplied

[Prognosis of nephrosis].

Renal biopsy, the introduction of immunohistologic methods and electron microscopy have allowed the differentiation of clinicopathologic entities associated to nephrotic syndrome. Two main categories must be differentiated: in the first, diffuse lesions of the glomerulus, including those secondary to specific diseases the same as those that are apparently primary, are responsible for the increased permeability of glomerular capillaries. Any one of the following clinical signs suggests this category: acute onset with nephritic syndrome, moderate nephritic syndrome, moderate nephrotic syndrome, gross hematuria, persistent hypertension and/or renal failure, poor selectivity of proteinuria and drop in complement serum levels (C3). In the second category, known as idiopathic nephrotic syndrome, the mechanism of disorder of the glomerular capillary is unknown and the nephrotic syndrome is more marked. In most cases with idiopathic nephrotic syndrome, minimal glomerular lesions (MGL) are present. The clinicopathologic correlation among these three types of lesions shows that the type with MGL is characterized by selective proteinuria, absence of hematuria, good response to corticosteroids and good outlook; whereas in types with diffuse mesangial proliferation (DMP) and segmentary sclerosis, proteinuria is frequently non selective, hematuria shows in 50 to 75% of the patients; prognosis is poor. However, MGL, DMP and focal segmentary glomerular sclerosis are not different entities, but represent variants of the same disease. Considering that corticosensitive nephrosis to this moment is the most common cause of the nephrotic syndrome, especially in children under 8 years, renal biopsy should be done only under two circumstances: a) when the clinical symptoms suggest diffuse glomerular lesions and b), when resistance to corticosteroids becomes evident.

Adrenal Cortex Hormones↗
PubMedDate not supplied

[Partial lipodystrophy, hypocomplementemia and glomerulonephritis].

Renal involvement is found in 20 to 50% of cases of Partial lipodystrophy (PLD). We report 8 cases of PLD of which 6 had a glomerular nephropathy and 2 had no renal disease but all had persistent hypocomplementemia and 5 had circulating nephritic factor (C3NeF). The analysis of these cases and of all the cases reported in the literature shows the specificity of the glomerular involvement. Membranoproliferative glomerulonephritis (MPGN) with dense intramembranous deposits is a constant finding in PLD with renal involvement. This variety of MPGN is well known for being associated with persistent hypocomplementemia. However, the presence of hypocomplementemia and C3NeF in patients with PLD but without nephritis raises the question of the interrelationship between alternative pathway complement activation and the development of MPGN with or without lipodystrophy. There is no valid explanation in the present state of knowledge for the association of partial lipodystrophy hypocomplementemia, and MPGN. From the answer to this problem should emerge a better understanding of the role of complement in renal disease and in particular in the unusual form of glomerular injury seen in MPGN.

Adolescent↗