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Biomedical subjects

M Broyer

Publications and source records attributed to M Broyer.

At least 253 records · Page 14Linked to original sources
PubMed1986

[Use of the intestinal graft in renal transplantation in children and adults].

Intestinal grafts as a means to external shunting of urine during renal transplant operation were described by W.D. Kelly as early as 1966. Since then 80 cases have been reported in the Anglo-Saxon literature. A total of 68 well-documented cases showed functional kidneys in 52%, complications in 42% and a 13% mortality rate. Between 1973 and 1985, of 400 renal transplant operations in children, an intestinal graft was used in 8 cases (2%) to provide 4 definitive external diversions and 4 enlargements or replacements of bladder. Indications for use were neurological bladder and posterior urethra valves. In all cases the graft was prepared before transplant operation. Enlargement of bladder requires good cervico-urethral function determined by previous study of a generally nonfunctioning bladder distal to an cutaneous ureterostomy. To avoid post-transplant urological effects the graft for enlargement or replacement is opened temporarily on to skin and closed several months after grafting. Follow up for 2 to 8 years showed 6 kidneys functioning normally, and 3 enlarged or replaced bladders out of 4 currently closed functioning satisfactorily. The 2 lost kidneys were rejected 2 weeks and 2 years respectively after the graft operation. There was no mortality or urological complication. The only surgical complications related to the intestinal graft were 3 early-onset occlusions treated successfully. Calculi formed in 2 cases, one being eliminated spontaneously at an early stage and the other, of late onset, requiring two operations. Metabolic or infectious complications were benign.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1986

[Antenatal diagnosis of Lowe's syndrome based on histologic tests of the fetal eyes].

Parents who had previously given birth to a male with Lowe's syndrome were watched during a second pregnancy and a prenatal sex diagnosis was performed by amniocentesis. The fetus was a male and in proportion to the high rate of risk (50%), parents require to stop this second pregnancy. Instead of later renal failure and, of course, mental retardation, it was the histological features of the fetus eyes which permit to diagnose and exhibit both congenital cataract and irido-corneal angle dysgenesis.

Eye Diseases↗
PubMed1986

[Charcot-Marie-Tooth disease and glomerular nephropathy].

Anatomoclinical profile and follow-up of a glomerular nephropathy in a prepubertal girl with Charcot-Marie-Tooth disease is reported; striking common pattern features with the few cases previously published of such an association strongly suggest that renal involvement is possible in sensorineural neuropathies and may be one major factor in the overall prognosis of the disease.

Charcot-Marie-Tooth Disease↗
PubMed1985

[Intrafamilial renal transplantation in children after donor blood transfusions].

This article represents an update of our experience with deliberate donor specific blood transfusions (D.S.T.) prior to living-related renal transplantation in children. Eighteen recipients with a negative cross- match to donor T and B cells entered the D.S.T. protocol without regard to the results of the mixed lymphocyte cultures and third party transfusion. The D.S.T. procedure involves the administration of fresh packed cells on 2 or 3 separate occasions at 4 weeks intervals. The potential recipient's sensitization is closely monitored against donor's isolated T and B lymphocytes and against a random panel. This immunological monitoring is done 10 to 20 days after each D.S.T. and just prior to grafting. A negative T and B cross-match at 37 degrees C is a pre-requisite for transplantation. Sixteen patients have been transplanted. All kidneys are functioning with follow-ups ranging from 6 to 48 months; 10 patients have normal renal function, 2 of them experienced reversible acute rejection and 6 have chronic rejection. The benefits derived from pretransplant D.S.T. in parental renal transplantation appear to be substantial, although the nature of the various immunological mechanisms remains unclear.

Adolescent↗
PubMed1985

Elevated plasma 1,25-dihydroxyvitamin D concentrations in infants with hypercalcemia and an elfin facies.

We measured plasma concentrations of 1,25-dihydroxyvitamin D (1,25-(OH)2D) in the course of a 6-to-37-month survey of four children with hypercalcemia and an elfin facies (Williams syndrome). Levels of 1,25-(OH)2D were elevated (160 to 470 pg per milliliter) during the hypercalcemic phase of the disease, when the children were five to nine months old, and they decreased thereafter. Plasma 1,25 (OH)2D levels were higher than those found in three children (16 to 60 months old) with the elfin facies syndrome and no hypercalcemia (42 to 71 pg per milliliter) and eight children (1 to 36 months old) with hypercalcemia and no dysmorphy (12 to 140 pg per milliliter), including two children with vitamin D intoxication. Hypercalcemia in the three children with elfin facies was controlled by a low-calcium diet. Serum calcium levels fell to the normal range, and plasma 1,25-(OH)2D levels were normal for age (18 to 105 pg per milliliter) at 14 to 47 months of age, even after appropriate therapy had been discontinued. These observations suggest that hypercalcemia may be the consequence of abnormal synthesis or degradation of 1,25-(OH)2D in children with the elfin facies syndrome.

Calcitriol↗
PubMed1985

Genetic heterogeneity of Alport syndrome.

Forty-one families have been studied with stringent diagnostic criteria of Alport syndrome: proven renal disease with hematuria affecting at least two relatives, neural hearing loss in at least one affected individual, and evolution to renal failure in at least one affected individual. The proportion of affected offsprings of affected females does not significantly differ from the ratio expected for a dominant trait. The descendance of affected males shows a lack of affected males. In four families, with parental consanguinity and nonaffected parents, the findings agree with an autosomal recessive inheritance. Study of quantitative traits such as death or renal death among brothers, uncle-nephew pairs and whole families shows evident intra-familial resemblances. We conclude that Alport syndrome seems to be a heterogeneous state composed of a number of genetically distinct syndromes, with an autosomal dominant, an X-linked dominant, and an autosomal recessive form.

Consanguinity↗
PubMed1985

Quantitation of renal function with 99Tcm-DMSA. A comparison with creatinine clearance in children with single kidney.

To study the accuracy of renal function quantification with 99Tcm-DMSA we compared DMSA renal uptake and creatinine clearance in 16 cases of children with single kidney. The age of the patients ranged from two months to fourteen years. Creatinine clearance was normalized to 1.73 m2. DMSA uptake was measured 7 h after intravenous injection. Background subtraction was used and soft tissue attenuation was taken into account. The uptake was normalized in percentage of the injected activity. A significant correlation was found between creatinine clearance and DMSA uptake (rt = 0.866, p less than 0.01). Normal creatinine clearance range in children (80 to 120 ml min-1/1.73 m2) allowed determination of normal uptake range (36 to 60%). This study indicates that in case of asymmetrical renal impairment renal uptake will reflect 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↗
PubMed1985

The nephropathy associated with male pseudohermaphroditism and Wilms' tumor (Drash syndrome): a distinctive glomerular lesion--report of 10 cases.

We report on 10 children, less than 2 years of age, who presented with a genuine type of glomerulopathy: diffuse mesangial sclerosis. In 5, the nephropathy was associated with male pseudohermaphroditism (MPH) and Wilms' tumor (WT); in 3 with MPH and in 2 with WT. The nephropathy was characterized by its very early onset, between the age of 2 weeks and 18 months. Eight patients presented with a nephrotic syndrome with (7 cases) or without (1 case) hypertension. All, but one, who is in advanced RF at 11 years of age, progressed to chronic or end-stage renal failure (ESRF) within a few months to 2 years from the onset. One additional child presented with advanced renal failure at the age of 8 months and the last one, who was hypertensive, developed an anuria related to thrombosis of renal veins at 1 year of age. Drash syndrome is characterized by the association of a "nephron disorder" with MPH and WT. We propose, on the basis of our histological findings, to extend the concept of Drash syndrome to patients who, in addition to the nephropathy, have either WT or MPH and to consider the distinctive glomerular lesions presented by all these patients as their common denominator. The pathogenesis of this glomerulopathy is obscure. Its early onset, its association with a dysembryoplastic tumor and/or with gonadal dysgenesis both suggest an antenatal dysgenetic process.

Disorders of Sex Development↗
PubMed1985

Varicella vaccine in children with chronic renal insufficiency.

This study reports the antibody response and clinical follow-up of uraemic children awaiting kidney transplantation after administration of the Oka-strain varicella vaccine (Varilirix). Seroconversion was observed in 20 out of 23 patients found to be seronegative when tested by the fluorescent antibody to membrane antigen technique, and an antibody booster response was observed in 41 out of 47 seropositive patients. Mild clinical varicella occurred in 5 vaccinated patients and herpes zoster in 3 initially seropositive ones. Nevertheless, a dramatic decrease in the incidence of both varicella and herpes zoster was observed in a series of 330 consecutive transplantations after the introduction of the varicella vaccine.

Adolescent↗
PubMed1985

[Circulating metabolites of vitamin D in 14 children with hypercalcemia].

Circulating vitamin D metabolite concentrations, i.e. 25-(OH)D, 24,25-(OH)2D, 1,25-(OH)2D have been assayed in 14 hypercalcemic children. Results are as follows: a) Children with vitamin D intoxication (n = 2) had elevated serum 25-(OH)D and 24,25-(OH)2D concentrations but their 1,25-(OH)2D concentrations were similar to those found in normocalcemic children (10-110 pg/ml); b) Children with familial idiopathic hypercalcemia and hypocalciuria (n = 5), children with hypercalcemia and either Bartter's syndrome (n = 1), hemangiomatosis (n = 1), osteopetrosis after medullary graft (n = 1), also had 1,25-(OH)2D concentrations in the normal range; c) In contrast, 1,25-(OH)2D were elevated (160-470 pg/ml) in the four children with severe idiopathic hypercalcemia and elfin facies.

24,25-Dihydroxyvitamin D 3↗
PubMed1985

Berger's disease in children. Natural history and outcome.

The clinical course and outcome of 91 children less than 15 years of age at onset and followed for at least 1 year have been retrospectively analyzed. The course has been characterized by recurrent macroscopic hematuria in 74 patients, by proteinuria-microscopic hematuria and a single episode of macroscopic hematuria occurring either at onset or a few months later in 8, by proteinuria-microscopic hematuria in 7, and by proteinuria only in 1. Lastly, one patient showed rapidly progressive renal failure. Four groups were identified by light microscopy: minimal glomerular changes (26), focal and segmental glomerulonephritis (41), pure mesangial proliferation (3) and proliferative glomerulonephritis with crescents (21). A good correlation was found between the glomerular lesions observed by light microscopy and the outcome. In this series we have not observed a dramatic clinical deterioration suggesting a transformation from one histologic type to another, as reported by others. None of the 70 patients belonging to the first three groups has impaired renal function but two with focal and segmental glomerulonephritis have developed hypertension. Although the clinical course is benign, many patients have, at the last observation, an abnormal urinalysis characterized by microscopic hematuria and/or mild proteinuria; the proteinuria is over 1 g/24 h in six patients with focal and segmental glomerulonephritis. Ten patients remained in clinical remission for several years, but mesangial IgA deposits were still present in the only patient who had a repeat biopsy while in remission. In contrast, none of the patients with proliferative glomerulonephritis with crescents has had a prolonged remission. Six patients developed terminal renal failure 0.7, 0.11, 2, 4, 8 and 10 years after onset. Two additional patients are in moderate chronic renal failure with hypertension 10 and 12 years after onset. Most children show a persistent nephropathy, (in five proteinuria is over 1 g/24 h), and two of them have developed hypertension. Therapeutic trials using drugs with side-effects should, therefore, be used only in this group of patients.

Adolescent↗