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Biomedical subjects

M Broyer

Publications and source records attributed to M Broyer.

At least 271 records · Page 15Linked to original sources

[Results of 310 cadaver kidney transplants in children and adolescents].

This study reports the results of 310 cadaver kidney transplantations in 295 children and adolescents performed from 1973 to 1983. The actuarial survival of patients was 97% at one year and 92% at 5 years; that of grafts was 79% at 1 year and 65% at 5 years, these rates having improved during the last years. Results were similar and even better for the 18 second transplantations. Among the causes of failure, rejection comes first (65%), then thromboses of renal artery (13%) and relapses of oxalosis or steroid resistant nephrosis (12%). Patients with cytotoxic antibodies have a less good survival of grafts, especially after 5 years. HLA A and B compatibility is a factor of success. Among complications hypertension is frequent, 53% of patients receiving antihypertensive treatment after 1 year. It is sometimes severe and was responsible for death in 5 cases. Growth was variable after transplantation: 25% of children before puberty had a catch-up curve, 25% had an unchanged growth and 50% an increased retardation. The average standard deviation was near zero but it was -0.49 SD/year in patients with creatinine level greater than or equal to 150 mumol and +0.24 SD in children under alternate day steroid therapy. Rehabilitation was excellent, less than 3% of patients not being engaged in any activity 1 year after transplantation.

Adolescent↗

[Diffuse arterial calcified elastopathy].

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 etiology could be found in the others.

Arteries↗

Treatment of idiopathic nephrotic syndrome with levamisole.

Thirty children with frequently relapsing idiopathic nephrotic syndrome (INS) were treated with levamisole (2.5 mg/kg BW) twice a week for a mean period of 9.9 months. A beneficial effect was observed in 16 children in whom corticosteroids could be significantly decreased without relapse. Levamisole was ineffective in 14 patients. There was no difference between the two groups in the duration of INS, the number of relapses and the duration of treatment with levamisole. The mean age at onset of INS was higher in the group of patients where levamisole was effective (5.8 years versus 2.8 years). In 7 patients who responded to levamisole neutrophils decreased below 4 X 10(9)/l. Transient granulocytopenia was observed in 3. It is concluded that levamisole may be effective in frequently relapsing INS with minimal side effects.

Chemotaxis↗

Plasma lipids, lipoproteins and apolipoproteins AI, AII, and B in renal transplanted children: what risk for accelerated atherosclerosis?

The aim of the study was to investigate the atherosclerosis risk factors related to hyperlipidemia in renal transplanted children. Plasma cholesterol, triglycerides, apolipoproteins (Apo) AI, AII and B, and the major lipoprotein classes separated by gradient ultracentrifugation were compared in 30 renal transplanted patients and 14 healthy children. Hyperlipidemia was present in 66% of the transplanted children. 'Positive' risk factors for atherosclerosis (high plasma cholesterol and Apo B) were present in hypercholesterolemic and combined hyperlipidemic subgroups. All transplanted children, whether normo- or hyperlipidemic, presented essentially 'negative' risk factors for atherosclerosis, i.e. significantly higher levels of Apo AI and AII in plasma and in high-density lipoprotein HDL2 and higher Apo AI/Apo B and/or Apo AII/B ratios. Repeated evaluations (over a 12-month period) in transplanted children indicated relatively frequent individual changes in the lipid pattern, but not in Apo AI and AII content. These results suggest that the risks for accelerated atherosclerosis related to hyperlipidemia may be considered as moderate in transplanted children.

Adolescent↗

Inactive renin in infants and children: evidence for its physiological response to orthostasis in children.

The aim of this work was to investigate the presence of inactive renin (IR) in plasma of normal infants and children and nephrectomized children and to study the plasma IR response to stimulation of the renin-angiotensin system (orthostasis) in children. The study was performed in 10 normal infants (2 days to 1 yr old), 28 normal children (1-15 yr old), 8 nephrectomized children (8-14 yr old), and 7 normal adults (20-40 yr old). IR was calculated as the difference in renin activity in trypsin-treated (1500 micrograms/ml) plasma, e.g. total renin (TR), and in untreated plasma, e.g. active renin (AR). IR was not detectable in most infants in the supine position, but their AR values were high (8.8-30 ng/ml X h). Moreover, in some of these infants, trypsin appeared to degrade renin activity, since TR values were lower than AR values. IR was detectable in 3 infants and 27 children, but their AR values were in a lower range (0.3-10 ng/ml X h). Trypsin degradation of renin activity was not found in either children or adults. With increasing age (2 days to 40 yr), AR decreased while IR and the IR to TR ratio increased significantly (P less than 0.001). A significant (P less than 0.001) inverse relationship was found between the IR and AR values of subjects 2 days to 40 yr old. IR was detectable in all nephrectomized children and represented 25% of normal values, while AR was undetectable (less than 0.1 ng/ml X h). In children in the upright position, IR decreased and AR increased significantly (P less than 0.001) in a reciprocal manner. TR did not change. These data suggest 1) that trypsin degradation of renin activity and absence of trypsin-activated IR are specific to infants with high AR levels, and 2) that IR might be activated in vivo into AR, especially after changes in position in children. IR could be a prorenin playing a physiological role in children.

Adolescent↗

[Treatment of childhood hemolytic-uremic syndrome with urokinase. Cooperative controlled trial].

The results of a controlled therapeutic trial comparing 2 groups of patients presenting with hemolytic-uremic syndrome (HUS) are reported. Group A (15 children) was given urokinase (UK) and heparin; group B (18 children) received no treatment. Ages of patients, the delay before admission, the severity of anemia, thrombocytopenia and initial renal failure were similar in both groups. UK was responsible for bleedings in 12 children, minimal in 8, severe in 4. No child died in group A, 3 children died in group B (n.s.). Durations of hemolysis, thrombocytopenia and anuria were similar in both groups. Long-term evolutions of renal function and arterial pressure were comparable in both groups. Needle kidney biopsy (26 cases) showed cortical necrosis in 3 children of group A and in 2 of group B, and glomerular thrombotic microangiopathy in 10 children of group A and in 11 of group B. The average ratio of injured glomeruli was 40 (19 to 80) in group A, and 38 (21 to 75) in group B. Two children in group A and 3 children in group B presented with 50 to 80% of glomerular lesions. This trial suggests that UK is of no significant value in the treatment of HUS.

Adolescent↗

[Beta 2 microglobulin. Index of glomerular filtration in children].

121 assays for plasma Beta 2 microglobulin (B2M) levels were carried out with an immuno-enzymatic technique in 94 children whose ages ranged from 13 months to 18 years and whose renal functions showed various levels of renal impairment. In the 37 children with normal glomerular filtration rate, plasma B2M level was 1.58 +/- 0.48 mg/l (mean +/- 1 SD) and no significant differences were found according to sex, ages or heights. In the 29 children with glomerular filtration rate (GFR) ranging from 20 to 100 ml/min/1.73 m2, there was a significant correlation between B2M and the inulin clearance, plasma creatinine level and creatinine clearance (p less than 0.001). In 28 patients presenting with terminal renal failure, plasma B2M levels were significantly higher in children undergoing hemodialysis than in those under chronic peritoneal dialysis. These results indicate that B2M levels are as good an index of glomerular filtration rate as serum creatinine whereas its assay uses a long and difficult technique.

Adolescent↗

[Diabetes induced by corticoids in 6 children after renal transplantation].

We studied the occurrence of diabetes mellitus in 6 children receiving corticosteroid therapy after renal transplantation. The first hyperglycemic episode occurred in all cases before the fortieth day of treatment but other episodes were observed thereafter. All children were glycosuric, without ketonuria. The diabetes has always been transient, and easily managed with insulin treatment and usual diabetic diet. A glucose tolerance test was performed 3 to 6 months after these episodes; glycemic response to glucose was abnormal in 2 of 6 children; in all cases, the insulin response to the glucose load was inadequate. In 2 children, the fasting blood glucose is still abnormal after a follow-up of 3 years. The other patients have recovered despite sustained corticotherapy. No specific background (genetics, HLA groups) or specific circumstances of treatment were identified. Therefore, we recommend to follow closely glycemia in children after renal transplantation, with a daily glycemic determination especially during the first 3 weeks.

Adolescent↗

[Captopril treatment of arterial hypertension in children after renal transplantation].

52 children with renal allograft received captopril during 56 periods of treatment. High blood pressure (BP) was due to renal artery stenosis in 22 patients, to diffuse vascular lesions (mainly due to chronic rejection) in 13, to high-dose corticosteroid treatment in the early phase in 11, and to various or unknown causes in 10 patients. In the last 3 groups (including 34 cases) captopril use did not induce any marked drawback. In the absence of overload, a good control of BP was obtained with a mean dosage of 2.2 mg/kg (0.7----5 mg/kg). A mild, transient renal failure (RF) was observed in 6 patients, who were given diuretics without any need. The 22 patients with renal artery stenosis received captopril during 26 periods of treatment. The mean dosage was 1.6 mg/kg (0.3-4.4). The result was excellent in 12 cases (normal BP without any RF), less good in 8 cases (moderate RF +/- borderline BP) and poor (acute RF) in 6. Sodium depletion, due to diuretics in 9, was present in all 14 cases with RF and in 10 of them captopril could be continued or reintroduced without any reappearance of RF after the correction of salt depletion. We conclude that sodium depletion is the main cause of renal failure in transplanted patients receiving captopril and that avoidance of diuretics largely diminishes the risk of RF.

Acute Kidney Injury↗

[Prospective study of cytomegalovirus infections in 211 children with kidney transplants].

A prospective serologic study was conducted to determine the incidence of primary Cytomegalovirus (CMV) infection after a renal transplant and to evaluate the consequences on the graft. Serologic tests using the indirect hemagglutination technique with CMV-sensitized erythrocytes were performed serially over 6 years in 370 children initially awaiting renal transplantation. 211 children received a graft during the study period. Transplantation was followed by primary CMV infection in 48 (35.3%) of the 136 children with negative serologic tests at the time of the procedure. The percentage of graft failures is identical in the 75 children with positive tests before transplantation and in the 88 children with negative tests before and after transplantation (24 and 23% respectively). Conversely, only 6 (12.5%) graft failures were recorded among the 48 children who experienced primary CMV infection after transplantation. It can be speculated that these results are due to the immunosuppressive effect of CMV infection.

Adolescent↗

Erythrocyte Na+ and K+ transport systems in children with Bartter syndrome: increase in passive sodium permeability.

Na+ and K+ intracellular content was studied in five children with Bartter syndrome and their age and race-paired controls. Na+ and K+ pump (ouabain sensitive) fluxes, Na+-K+ co-transport (furosemide sensitive), and rate constants of passive Na+ and K+ permeability were determined in each patient and control and also in six parents. The results show that in Bartter syndrome, there is a significant increase in the rate constant of passive Na+ permeability without any change in passive K+ permeability. This increase in the rate constant of passive permeability might explain at least partially the increased intracellular Na+ concentration also found in these patients. Moreover, the maximal rate of ouabain sensitive Na+ efflux was increased slightly, and co-transport fluxes were variable. Parents of patients had normal erythrocyte fluxes.

Adolescent↗

Rickets and alopecia with resistance to 1,25-dihydroxyvitamin D: two different clinical courses with two different cellular defects.

UNLABELLED: Two unrelated patients, aged 22 months and 31 months, with alopecia and rickets resistant to 1,25-dihydroxyvitamin D (1,25-(OH)2D] (vitamin D-dependency type II) presented with similar biochemical and radiologic features. They were treated with large doses of vitamin D3 derivatives [25-hydroxyvitamin D3 (25-(OH)D3), 1,25-(OH)2D3, and 1 alpha-hydroxyvitamin D3] for 28 months and 6 yr, respectively. In both patients, serum 1,25-(OH)2D levels remained high (approximately 10- to 100-fold normal) during the different therapeutic regimens. Circulating 1,25-(OH)2D and 24,25-dihydroxyvitamin D levels at various stages of the disease suggested in these children disturbances in the regulation of 25-hydroxyvitamin D (25(OH)D) 1 alpha- and 24-hydroxylase systems. In one child, all therapeutic trials were unsuccessful. Studies of her cultured skin fibroblasts showed low capacity (10% normal) for saturable (presumably receptor mediated) nuclear uptake of tritiated 1,25-(OH)2D3; the uptake process of nucleus associated 1,25-(OH)2D3 was normal in apparent affinity for 1,25-(OH)2D3 and in sedimentation velocity of nucleus-associated hormone. In the second child, correction of biochemical abnormalities, healing of rickets, and catch-up growth were obtained during similar therapeutic trials up to the age of 6 yr when a relapse occurred. This relapse has persisted for 2 yr in spite of similar or higher circulating concentrations of 25-(OH)D and 1,25-(OH)2D than those obtained previously when she was responsive to therapy. In her cultured skin fibroblasts, saturable high affinity nuclear uptake of 1,25(OH)2D was unmeasurable. IN CONCLUSION: 1) distinct patterns of clinical response can occur in patients with the syndrome of vitamin D-dependency type II, and can be associated with differing abnormalities in interaction of 1,25-(OH)2D3 with cultured skin fibroblasts; 2) aggravation of the resistance to 1,25-(OH)2D3 may occur during long term therapy in some patients.

24,25-Dihydroxyvitamin D 3↗