Muscle electrolytes and fluid compartments in six children with Bartter's syndrome.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to M Broyer.
Explore the source record for details and available documents.
The insulin and growth hormone responses to arginine and the growth hormone response to insulin were studied in 10 children undergoing chronic hemodialysis 1) under usual dietary prescription and 2) after 7 days of dietary manipulation (DM) decreasing mean carbohydrate intake from 48 to 36% and increasing lipid intake from 42 to 54% (percent of total energy) with the polyunsaturated/saturated fatty acids ratio being increased from 0.2 to 2. Mean fasting insulin and growth hormone were significantly decreased after DM: 10.3 +/- 3 muunits/ml and 19.9 +/- 3.5 ng/ml before and 4.3 +/- 0.8 muunits/ml and 9.3 +/- 2.4 ng/ml after DM. The mean arginine-induced insulin peak and the growth hormone peaks after arginine and insulin remained very high after DM. There was no decrease of mean plasma triglycerides: 214 +/- 30 mg/dl before and 237 +/- 26 mg/dl after DM. However, two children who had the greatest decrease in carbohydrate intake exhibited a decrease of triglycerides and of arginine-induced insulin secretion. The percent variation of insulin area after DM was significantly correlated with the percent variation of plasma triglycerides.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The prognostic significance of the finding of diffuse mesangial proliferation (DMP) in patients presenting with idiopathic nephrotic syndrome (INS) has not been well established. The clinical course, therapeutic response and final outcome of 38 patients in whom renal biopsy showed DMP are reported. They have been subdivided into 2 groups according to the absence (18 patients: group I) or presence (20 patients: group II) of superimposed lesions of focal and segmental sclerosis and/or hyalinosis (FSS/H). Clinical presentation was similar in both groups although patients in group I were less severely affected. Non of the patients of group II responded to corticosteroids, whereas in group I 2/16 responded and 2 infants remitted without treatment. At the latest assessment, 5/18 patients in group I and 10/20 in group II had progressed to terminal renal failure or had impaired renal function. Five patients in group I and 3 in group II were in clinical remission. Eight of 11 repeat biopsies performed in patients of group I showed the development of FSS/H. Thus patients with DMP seem prone to develop lesions of FSS/H. Their course if often worse than that of minimal change with FSS/H since 7 of the 10 patients who developed renal failure did so within 3 years of onset. The finding of DMP in a patient with idiopathic nephrotic syndrome is usually but not invariably an ominous feature.
Explore the source record for details and available documents.
The 24 hour clearance of endogenous creatinine was compared with that of inulin in 112 children aged between 15 months and 17 years. The children had a variety of kidney disorders with renal function than ranged from normal to terminal renal failure. When the inulin clearance was less than 10 ml/min/1.73 m2 it was on average 75% of the value of the creatinine clearance. Between 10 and 60 ml/min/1.73 m2 the relationship of the inulin clearance to that of creatinine was similar (77%) but above 60 ml/min/1.73 m2 the ratio of the inulin clearance to that of creatinine was 1.05 with a wide scatter. With increasing degrees of renal failure the plasma creatinine increased less in the children under 2 years old than in those over this age so it is poor index of glomerular function in infants. In renal failure the urinary excretion of creatinine in 24 hours was reduced. When the clearance was less than 10 ml/min/1.73 m2, the urinary creatinine excretion for children under the age of 2 was 58% of the normal for the age and for children over 2, it was 86% of the expected mean.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Thyroid function was assessed in 15 children with cystinosis and it was abnormal in 12. Three of these children were clearly hypothyroid and in the other 9 there were abnormalities of TSH or in the response to TRF. Compensated hypothyroidism is frequent in cystinosis and may be one factor in the severe growth retardation in this condition. The preliminary results of replacement therapy support this hypothesis.
A 4-month old child presented with facial malformations and severe hypernatremia. Hypernatremia was secondary to diabetes insipidus due to a disorder of ADH secretion, associated with cerebral malformations. Clofibrate treatment was ineffective. However, after the patient was treated by a low osmotic residue diet, an increased water-intake and hydrochlorothiazide, natremia became normal and growth resumed.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
27 children, aged 7 months to 15 years, with terminal renal failure and no available vascular access, were treated with chronic peritoneal dialysis for 3 weeks to 9 months (mean 3 months). An indwelling silicon catheter fitted with a subcutaneous dacron felt cuff was used; the average catheter life time was 10 weeks (3 to 25 weeks). Control of uremia was satisfactory with mean serum urea decreasing from 2 to 1 g/l and creatinine from 130 mg/l to 60 mg/l after 48 hours of dialysis. No uremic complications occured. Total serum protein remained stable: mean: 62 g/l prior to treatment and 60 g/l after the treatment period. Hematocrit was higher than in hemodialysed children (17% versus 15%). Three children were directly transplanted without difficulty. However, some complications did occur. There were 27 episodes of catheter obstruction leading to 12 surgical interventions. 18 episodes of peritonitis (5% of total dialyses) occured in 12 patients, and two were lethal. The frequency of complications prohibits a recommendation of chronic peritoneal dialysis over hemodialysis in children; this technique however remains very helpful in those situations where vascular access is difficult.
Serum antibodies (AB) have been measured before and after vaccination in haemodialysed children. The main finding was a normal increase in AB titres following adsorbed vaccines (diphtheria, tetanus, pertussis and poliomyelitis) demonstrating a normal humoral immune ability. By contrast, a poor response to live attenuated viruses (poliomyelitis and measles) was observed. Two additional findings were an inhibitor effect of tetanus toxoid in mice by uraemic serum, and a constant negative Schick test indicative of suppressed non-specific skin reactivity.