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Biomedical subjects

M Broyer

Publications and source records attributed to M Broyer.

At least 163 records · Page 9Linked to original sources
PubMed1991

Thrombosis in systemic lupus erythematosus: a French collaborative study.

A retrospective study was undertaken of 120 children with systemic lupus erythematosus (SLE) seen in Paris and its immediate suburbs who fulfilled at least four of the American College of Rheumatology diagnostic criteria for SLE, and in whom the disease was diagnosed before the age of 16 and between January 1975 and December 1987. Eleven of these children (eight girls and three boys) all more than 10 years of age (mean follow up 8.1 years; range 3-13) had thrombotic episodes (9%). Thrombosis was one of the presenting signs in seven patients; in five it was associated with typical symptoms of SLE, and in the other two the thrombotic episode was isolated and diagnosis of SLE was delayed one and three years. Of a total of 16 thrombotic episodes (six of which were recurrent), 14 involved the leg veins, and in four there was associated pulmonary embolism. There were two episodes that affected cerebral arteries. The American College of Rheumatology diagnostic criteria for SLE as well as the incidence of lupus anticoagulant, positive direct Coombs test, and vasculitis in this group of patients was compared with the incidence in patients with SLE but no thrombosis. Only lupus anticoagulant was significantly associated with thrombotic episodes: eight of 11 (73%) of patients with SLE and thrombotic (arterial or venous) episodes had lupus anticoagulant compared with only 10 of 74 patients (14%) with no history of thrombotic events in the same age group.

Adolescent↗
PubMed1991

[Prevention of vascular thromboses after renal transplantation using low molecular weight heparin].

Vascular thrombosis is one of the main causes of early transplant failure in pediatric patients. This paper reports the results of an open trial of the low molecular weight heparin (Enoxaparine) used to prevent renal graft thrombosis in pediatric recipients with risk factors including donor or recipient age under 5 years, multiple arteries supplying the transplant, and positive history for recurrent thrombosis. During 1989, 42 of 67 children given a renal transplant were prophylactically treated with Enoxaparin. Only one transplant was lost to thrombosis among treated patients (1.5%), versus 9 transplants among 73 (12%) children who received their kidney in 1988 without prophylactic Enoxaparin. Risk factors were comparable in both groups of recipients. Enoxaparine therapy was associated with an increased rate of bleeding (12/42) without severe consequences. In conclusion, Enoxaparin is effective in preventing renal graft thrombosis. Availability of this prophylactic therapy makes it possible to use transplants removed from the youngest donors considered as inadequate by some groups.

Age Factors↗
PubMed1991

[Hepatic and renal transplantation in the treatment of type I hyperoxaluria].

Hyperoxaluria type I (HPI) is a metabolic disorder secondary to liver alanine glyoxylate aminotransferase deficiency. Renal failure occurs due to the excessive production and precipitation of oxalate in the kidney. Combined liver-renal transplantation is the correct treatment for this condition when end-stage renal failure occurs as with renal transplantation alone the risk of recurrence of the same pathology in the transplanted kidney would be high. We report the case of a 4 year-old child with HPI suffering from terminal renal failure in whom a hepato-renal transplantation was performed: six months later, creatinine clearance was 62 ml/min/1.73 m2 and liver function tests were normal.

Actuarial Analysis↗
PubMed1991

[Intra-leukocyte cystine in cystinosis treated with cysteamine].

Data on cystine leukocyte content are analyzed in cystinotic patients receiving cysteamine as a depleting agent of cellular cystine. During 2 years, 63 measurements of cystine leucocyte content were performed in 15 cystinotic patients, aged 20 months to 22 years, taking daily 40 to 65 mg/kg of cysteamine. An original method was used to measure leukocyte cystine: a binding protein assay with a specific cystine-binding protein from E. coli. Results were taken individually because of various clinical situations in patients. In 8 patients taking cysteamine regularly, 6 hours after a dose, cystine leukocyte content was between 1 and 2 nmol 1/2 cystine/mg protein, about 10 times less than basal values without treatment and 5 to 10 times more than control subjects. In less compliant patients, cystine leukocyte content was close to basal values without treatment (3 to 25 nmol/mg). Some variability was observed between individuals receiving cysteamine: pharmacokinetic parameters may need further investigation. This sensitive and specific assay helped in controlling compliance and adjusting dosage regimen in each patient. The aim was to maintain a minimum effective dosage in order to avoid toxic side effects of cysteamine.

Adolescent↗
PubMed1991

[Atrial natriuretic factor in hemodialyzed children].

Indirect evidence suggest that volume overload is the major determinant of plasma atrial natriuretic factor (ANF) elevation in hemodialysis patients. Correlations between plasma ANF levels and extracellular volume (ECV) were investigated by simultaneously measuring both parameters in 30 pediatric hemodialysis patients (aged 1 to 17.5 years; 18 M, 12 F) 24 hours after a dialysis session. Plasma ANF was determined using a commercially available RIA (Amersham) after plasma extraction (SEP-Pack C18); ECV was estimated by determining the volume of distribution of inulin and expressing the result as the % of body weight. In hemodialysis children, ANF levels ranged from 43 to 427 pg/ml (versus 30-70 pmoles/ml in age-matched controls) and EVC ranged from 17 to 33% BW. A significant positive correlation was found between plasma ANF levels and ECV (r = 0.66; p less than 0.001). Patients who exhibited falls in blood pressure during the dialysis session had lower mean ANF and ECV values (133 +/- 90 pg/ml and 23 +/- 3% BW, respectively) than those who did not (211 +/- 123 pg/ml and 26 +/- 4% BW, respectively). Conversely, patients who needed chronic antihypertensive therapy had higher mean plasma ANF and ECV values (204 +/- 122 pg/ml and 26 +/- 4% BW, respectively) than those who did not (149 +/- 100 pg/ml and 23. 5 +/- 4.5% BW, respectively). In a small subgroup of patients who had repeated determinations, individual plasma ANF and ECV changes were closely matched and both parameters were well correlated.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

[Do posterior urethral valves alter the results of renal transplantation in children?].

The authors conducted a retrospective study based on a series of 715 renal transplantations in children performed between January 1973 and December 1989 at the Hôpital Necker-Enfants Malades in order to determine whether the long-term result of renal transplantation was as good in children with posterior urethral valves (PUV) as in children with a normal lower urinary tract. Group 1 consisted of 50 renal transplantations performed in 41 children in whom the primary urological disease was PUV. The bladder was able to be used without modification in 36 cases and had to be enlarged in 5 cases. A permanent cutaneous urinary diversion was not required in any of these transplanted patients. Group 2 consisted of 665 renal transplantations performed in children without PUV. There was no significant difference between the two groups in terms of the distribution of cadaver kidney and living related donor transplantations. Immunosuppressive treatment consisted of various combinations of prednisone, azathioprine, ALS for the earlier patients in the series, OKT 3 and cyclosporin for the more recent patients. In group 1, we observed a urological complication rate of 20% and a vascular complication rate of 14%, while, in group 2, the urological complication rate was 16.9% and the vascular complication rate was 9.5%. The actuarial 5-year and 10-year graft survival rates were 63% and 49% in group 1 and 63% and 49% in group 2, respectively. The actuarial graft survival rates were therefore identical in the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗
PubMed1991

Treatment of idiopathic nephrotic syndrome with cyclosporin A in children.

Cyclosporin A (CyA, Sandimmun) was given to 71 children with idiopathic nephrotic syndrome, 45 of whom were steroid-dependent with signs of steroid toxicity, and 23 who were steroid-resistant. Cyclosporin A was effective in 80% of the steroid-dependent patients, allowing cessation of corticosteroid treatment. However, most of these patients relapsed when CyA was tapered or withdrawn. In these patients, CyA may be required for long periods of time. Conversely, CyA was less effective in steroid-resistant patients; only 7% achieved remission with CyA alone. Cyclosporin A in association with prednisone may be an effective alternative as 8 out of 14 patients entered remission with this treatment combination. Serial renal biopsies were performed in 43 patients to evaluate the potential nephrotoxicity of the treatment. Eighteen patients developed significant tubulointerstitial lesions which were attributable to CyA nephrotoxicity. The risk of developing chronic nephrotoxicity appears to be higher in steroid-resistant patients, and was not related to the duration of treatment. Cyclosporin A nephrotoxicity can develop in patients with normal renal function.

Child↗
PubMed1991

[Alternate day corticosteroid therapy and growth in renal transplant children].

Corticosteroid therapy is probably the main factor inducing stunted growth in pediatric renal transplant recipients. Results of a randomized study in 35 children who received their kidney between 1981 and 1984 are reported. All patients had normal renal function and were taking azathioprine and prednisone to ensure immunosuppression. Eighteen months after transplantation, patients with normal renal biopsy results were randomized to receive further daily corticosteroid therapy (group A) for an additional year or to alternate-day corticosteroid therapy (group B). Chronologic age, bone age, renal function, and previous growth retardation were strictly comparable, in the two groups. During the first year, only prepubertal children in group B exhibited catch-up growth. In children undergoing puberty, annual statural gain was greater in group B (5.6 cm versus 3.2 cm in group A: p less than 0.001). Group A children were switched to alternate-day corticosteroid therapy one year after initiation of the study and exhibited improved growth after this change. No patient had renal function deterioration under alternate-day corticosteroid therapy, throughout the study period. Alternate-day prednisone should be offered to pediatric renal transplant recipients with satisfactory renal function as a mean for protecting growth potential.

Adolescent↗
PubMed1991

[Gonad function and sexual activity in male renal transplant patients].

Gonad function and sexual activity were evaluated in 44 young male adults who received a kidney shortly before or during adolescence. All patients were growth-retarded (-2.2 SD). Volume of the testes was decreased less than 12 ml, i.e, less than 10th centile) in 47% of patients, whereas hormone levels (testosterone, FSH, LH, and prolactin) were within the normal range. Oligospermia or azoospermia was found in 10 of 11 tested patients. Testicular function and sperm count were negatively correlated with renal function. Age at first ejaculation was increased and 57% of patients had never had sexual intercourse, although all patients reported normal sexual drive and normal erections.

Adolescent↗
PubMed1991

[Clinical evaluation of 70 pediatric renal transplants after 10 to 17 years].

Among 149 kidneys transplanted in 141 children and teenagers aged 4 1/2 to 20 years, from November 1972 through December 1979, 70 (47%) were still functioning after ten years (67/145 cadaver donor grafts and 3/4 living related donor grafts). Medical and social status at the last follow-up visit (10 to 16 1/2 years after transplantation; m = 12 years) was analyzed. Patients were divided into five groups on the basis of glomerular filtration rate (GFR; ml/min/1.73 m2) and blood pressure (BP): 1) GFR greater than 80 ml and normal BP: 23 patients (33%); 2) GFR in the 60-80 ml range and/or high BP: 24 patients (34%/3) GFR in the 40-60 range: 6 patients; 4) GFR in the 15-40 range: 7 patients; and 5) hemodialysis restarted 10 1/2 to 13 years after transplantation. Mean adult stature was 155.7 +/- 10.4 cm in males and 149.8 +/- 10 cm in females. Osteoporosis was found in 88% of patients who underwent bone density quantitation. Twenty-four per cent of patients had aseptic osteonecrosis with variable degrees of impairment as a result. Chronic HBsAg carrier status was found in 37% of patients and was accompanied with persistent cytolysis in half the cases. Only one malignancy was seen (carcinoma of the urinary bladder in a child under cyclophosphamide). Six deaths were recorded between 10 and 13 years after transplantation; causes included septicemia (2 cases), cancer (1 case), hepatitis B (1 case), cerebral cystinosis (1 case), and unexplained sudden death (1 case).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

Renal functional reserve in children with reduced renal mass: study by two dietary periods.

It has been suggested that the "renal functional reserve" (RFR) defined by the rise in glomerular filtration rate (GFR) after a protein load could disappear in patients with severe nephron loss but with a normal GFR. This study compared, in 17 children, inulin clearance (Cin) measured by the plasma inulin plateau at the end of two 14-day randomized periods differing in protein intake: 100% (low protein, LP), or 200% (high protein, HP) of recommended dietary allowances (RDA). Diets were aimed at maintaining food habits and energy intake. Compliance was assessed by records of the last 3-4 days, an interview with the dietician and by urinary nitrogen measurements. Mean actual protein intake was 109% (56%-139%) RDA for the LP period and 220% (163%-319%) RDA for the HP period. Cin did not change in 14 children with GFR below (n = 7) or within (n = 7) the normal range. Cin was higher in the HP period than in the LP period (+32, 50, 63%) in 3 children who had a 50% (single kidneys) or a 25% (sclerosed glomeruli) nephron loss. Non-responding children had a GFR below 105 ml/min per 1.73 m2. Nephron loss (70% sclerosed glomeruli) was estimated in only 1 child with no RFR. The results suggest that GFR measurement after prolonged dietary stimulation could help in evaluating the severity of nephron loss in children with normal or borderline GFR. The prognostic value of this test has to be confirmed by long-term follow-up.

Adolescent↗
PubMed1990

Microsurgical creation and follow-up of arteriovenous fistulae for chronic haemodialysis in children.

Three hundred and eighty children underwent 434 angioaccesses. Of these angioaccesses, 113 were constructed in 74 children weighing under 10 kg. Most accesses (n = 340) were distal arteriovenous fistulae (AVF). After microsurgery there was a 96% immediate patency. Seventy percent of AVF, excluding distal ulnarbasilic AVF, were functional, sometimes after secondary superficialization of the vein. Eighty-five per cent of the distal radial-cephalic AVF are still patent after 2 years, 60% are still patent after 4 years. These radial-cephalic AVF required 65 repeat anastomoses, and 12 ligations of the proximal-radial artery in order to reduce to 50% the high blood flow (pre-reduction average index = 900 ml/min per m2. The patency rate of arteriovenous-bridge grafts was not encouraging. The severity of stenoses in the proximal-venous trunks, sometimes related to previous catheterization, is emphasized. Advantages of the radial-cephalic wrist AVF in children are highlighted.

Adolescent↗
PubMed1990

Renal amyloidosis in juvenile chronic arthritis: evolution after chlorambucil treatment.

Over a 22-year period, eight patients affected with severe systemic or polyarticular juvenile chronic arthritis (JCA) developed systemic amyloidosis with nephrotic syndrome. They were treated with chlorambucil over 5-192 months (mean = 44 months). With treatment, an abrupt decrease in the severity of JCA was observed in six patients but two patients were chlorambucil resistant. After a mean follow-up period of 10 years from onset of renal symptoms, one chlorambucil-resistant patient died of end-stage renal failure; two patients have a persistent nephrotic syndrome; and five patients are free from proteinuria, of whom one has developed hypertension. A good correlation was observed between the response of the rheumatic disease to chlorambucil treatment and the clinical course of renal symptoms. Fourteen renal biopsies were performed in these eight patients. In all, amyloid deposits were of the AA type, which persisted on repeat biopsies. In addition, 15%-60% of glomeruli had become globally sclerotic by the second or third biopsies. At the ultrastructural level, modifications in the structure of amyloid deposits and reparative changes of the glomeruli, characterized by partial restoration of glomerular architecture, were observed in three patients with a favourable clinical course.

Amyloidosis↗
PubMed1990

Optimal dietary substitution of racemic ketoanalogues for isoleucine in growing normal and uremic rats.

Dietary ketoanalogues (KAs) were shown to replace their essential amino acids with a 50% efficiency for valine and leucine. We determined the optimal concentration of the racemic KA of isoleucine (KMVA) in uremic and control rats: nutrition responses were compared between a diet containing optimal isoleucine concentration and diets containing various KMVA concentrations. Isomolar replacement of isoleucine produced anorexia, stunting, and poor nitrogen balance. Doubling KMVA partially improved these indices. Tripling KMVA lessened urea production and improved growth up to that obtained with the isoleucine diet in uremic but not in control rats (20% lower). A further KMVA increase produced no further benefit. Among plasma branched-chain amino acids, only alloisoleucine was affected; it increased with increasing KMVA concentration, being maximum after tripling KMVA. Racemic KMVA could replace isoleucine with a 35% efficiency but supported no growth acceleration in uremic rats and no maximal growth in control rats. Plasma alloisoleucine rose without adverse nutrition effects.

Amino Acids, Essential↗
PubMed1990

Adverse effects of sucrose-rich diets on uraemic rats.

The nature of carbohydrate may affect the tolerance and progression of uraemia. The effects of three diets differing only in their carbohydrate source: namely corn starch (C), glucose (G) or sucrose (S) were examined. Study 1 examined the effects of the three carbohydrate diets on unilaterally nephrectomised control rats and severely uraemic rats. The three carbohydrates produced similar nutritional effects in uninephrectomised rats, whereas sucrose rapidly induced anorexia, stunting and slightly accelerated renal damage in uraemia. Study 2 examined the long-term effects of the three carbohydrates in moderate uraemia under conditions of high and identical carbohydrate intakes. Hyperphagic Zucker uraemic rats (F rats) received a daily allotment of each diet plus pure carbohydrate. Lean uraemic rats (L rats) received the same dietary allotment without the carbohydrate supplement. The F rats fed sucrose showed greater morbidity and mortality but little renal deterioration. Their plasma triglycerides increased dramatically. The L rats fed sucrose had the greatest urinary protein, the least creatinine clearance and the most severe renal damage. Thus, sucrose-rich but not glucose-rich diets have two adverse effects in uraemia: a deterioration in nutritional status, perhaps related to abnormal fructose utilisation, and a long-term effect on the kidney, resulting in accelerated renal deterioration.

Animals↗