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Biomedical subjects

F Bouissou

Publications and source records attributed to F Bouissou.

At least 37 records · Page 2Linked to original sources

HLA class II associations with idiopathic nephrotic syndrome in children.

The occasional familial occurrence of idiopathic nephrotic syndrome (NS) points to a genetic predisposition. Reports on associations with certain HLA class II antigens support this hypothesis. In order to define the immunogenetic background of NS more precisely, HLA class II allele frequencies in 161 children with NS were studied by restriction fragment length polymorphism (RFLP) typing. The patient cohorts consisted of 87 children from Southwest-France and 74 from Southwest-Germany. The control group consisted of 118 French and 101 German unrelated individuals from the same geographical areas. HLA alleles were defined in patients with steroid-sensitive (SS) and steroid-resistant (SR) NS and in controls. RFLP typing revealed that the previously reported association between SSNS and HLA-DR7 is confined to the RFLP split 7.1 (DRB1*07) with a combined relative risk (RRcomb) of 6.2. HLA-DQB typing showed an increased frequency of the allele DQB2b (DQB1*0201) (RRcomb = 7.8). HLA-DQA typing showed an association of SSNS with DQA3 (DQA1*0201,0301,0302) (RRcomb = 4.1). The highest RR (16.5) for SSNS was found in German patients who carried the two DRB1 specificities 17.1 (DRB1*0301) and 7.1 (DRB1*07). All associations were stronger in SS patients with frequent relapses or steroid dependency than in non- or infrequent relapsers. SR patients exhibited no significant associations with HLA class II alleles.

Alleles↗

[Fetal and neonatal effects of maternal treatment with angiotensin converting enzyme inhibitor].

BACKGROUND. Exposure of pregnant women to angiotensin converting enzyme inhibitor may have side effects on the fetus or newborn, mainly oligoamnios and impaired renal function. CASE REPORT N zero 1. A 34 year-old woman was given enalapril from the onset of her pregnancy because of hypertension from the age of 18 years. Oligoamnios was diagnosed in the fetus on gestational week 28; enalapril was then replaced by nifedipine but this drug was badly tolerated so that the woman was again given enalapril 8 days later. The baby (1700 g) was born by cesarean section at gestational week 34 because of acute distress syndrome; he developed hypotension, anuria, generalized oedema and was placed in intensive care. Treatment included ventilation, sympathomimetic agents, and diuretics. An exchange-transfusion followed by peritoneal dialysis was performed a few hours later. Renal function returned to normal between the 3rd and 5th day. Unilateral kidney hypoplasia was diagnosed at the age of 2 years. CASE N zero 2. A 24 year-old woman was given enalapril at the third trimester of a twin pregnancy. Delivery was full term at 37 weeks. The first baby, a boy weighing 2610 g, suffered from hypoglycemia and vomiting followed by hypotension and oliguria that required exchange-transfusion and repeated peritoneal dialysis. This boy has developed moderate chronic renal failure and hypertension. The second baby, a girl weighing 2,165 g, suffered from respiratory distress syndrome followed by hypotension and oliguria, but her renal function returned to normal within a few days. CONCLUSIONS. The use of angiotension converting enzyme inhibitor by pregnant women places the fetus at severe risk: treatment with this type of drug should be stopped as soon as pregnancy is confirmed.

Acute Kidney Injury↗

[Technique of renal puncture biopsy in children].

Ultrasound guidance is usual and has improved the morbidity and the efficacy of percutaneous renal biopsy in children. New needles and gun device are now available and make the biopsy safer. These improvements should not eliminate the contra-indications.

Biopsy, Needle↗

Plasma exchange therapy in pediatric patients. Plasma filtration with a unipuncture technique.

Therapeutic apheresis in children is limited by technical considerations, such as circuit blood volume, venous access and hemodynamic instability. Here we report our experience with 310 plasma-filtration sessions in 44 children (aged 9 months-17 years). We used a miniaturized circuit with a unipuncture technique and a "balancing system" which assured a permanent fluid equilibrium. This procedure is well adapted to children and always made pediatric plasma exchange therapy easier and safer. The more common indications remain Guillain-Barré syndrome and acute severe glomerular diseases.

Adolescent↗

[Results of the evaluation of platelet sodium-proton exchange in the young hypertensive subject].

In order to assess the relationships between increased cellular sodium-proton (Na+/H+) exchange and cardiovascular abnormalities in essential hypertension (EH), 21 young subjects as part of an ongoing longitudinal study were tested for the platelets Na+/H+ exchange using the amiloride sensitive sodium dependent component of platelet volume change under cytoplasmic acidification induced by a sodium propionate medium; cell volumes were determined by electronic cell sizing (Livne et al., Lancet 1987; i: 533-6). 24 normal subjects with normotension and without familial history of hypertension were taken as controls. Data of ambulatory blood pressure recording (ABPR) defined 2 groups according to the presence of normotension (group I, n = 10), or of hypertension (group II, n = 11): established (n = 2) or borderline (n = 9) hypertension. Hypertensive subjects (group II) had increased values of Na+/H+ exchange (k coefficient, mean (SEM): 0.287 (0.07) vs 0.228 (0.05) in control group (p less than 0.01). Na+/H+ rates were significantly related to ABPR data (r = 0.46, p less than 0.02 with diastolic charge during ABPR), but not to left ventricular mass index in g/m2 by echocardiography. Increased rates of platelets Na+/H+ exchange which were related to diastolic blood pressure levels by ABPR, and perhaps to the level of peripheric vascular resistances, may play a significant role in the development of EH in the early stages.

Adolescent↗

Pharmacokinetics of prednisolone in children with the nephrotic syndrome.

The aim of this study was to establish whether the criteria for the clinical effectiveness of steroids are correlated with the pharmacokinetics of prednisolone in children treated with prednisone during an attack of idiopathic nephrotic syndrome (INS). Thirteen patients with nephrosis were included. Prednisolone, prednisone and cortisol levels were measured using a specific high-performance liquid chromatography assay after an oral dose of 1 mg/kg body weight of prednisone taken at the onset of the disease. All the pharmacokinetic parameters, including the conversion of prednisone to prednisolone were similar to the data already published in children with INS. No correlation was found between the values of pharmacokinetic parameters and criteria of clinical effectiveness. Hypo-albuminaemia was significantly correlated with the area under the plasma-concentration curve but not with the elimination half-life of prednisolone. Moreover, the prednisolone elimination half-life correlated with the urinary excretion of 17-hydroxycorticosteroids achieved in the first 6 h. The present study suggests that routine measurements of prednisolone kinetics do not help when assessing the treatment of children with INS.

17-Hydroxycorticosteroids↗

[Quantitative renal scintigraphy with technetium-labelled dimercaptosuccinic acid in children].

Separate measurement of renal function is essential in children with urinary tract disease. This can be done satisfactorily by means of kidney scintigraphy with technetium 99m-labelled dimercaptosuccinic acid. A retrospective study of the records of 415 children, which comprised renal function tests, radiology and scintigraphy, confirmed the value of the radioisotope technique. Graphs of height, kidney uptake and kidney uptake density in relation to age were established, showing highly significant correlations with renal function tests and intravenous urography results, but many dissociations were encountered. Separate renal function evaluation by radioisotope scintigraphy seems to be more reliable than evaluation extrapolated from measurement of the cortical areas at intravenous urography. In vesico-ureteral reflux scintigraphy makes it possible to detect reflux nephropathy at an earlier stage than with other methods.

Age Factors↗

Long term therapy by captopril in children with renal hypertension.

We report on 29 treatment courses in 25 children aged 1.5-18 years who received captopril because of severe renal hypertension. The mean initial dosage was 1.3 (0.5 to 3) mg/kg/day and the mean sustaining dose 2.2 mg/kg/day. The treatment was followed for 2-40 (mean 15) months. We observed a lowering of both systolic and diastolic blood pressure (BP) from 26% at the 2nd day to 65% at the 6th month. The isolated use of captopril was ineffective in 13% of cases, but blood pressure dropped in all of them after addition of beta blocking agents. No clear-cut relation was found between response of blood pressure and etiology, degree of HT or plasma renin activity (PRA). No side effects occurred during treatment except for one case of reversible acute renal failure in a transplanted patient with renal artery stenosis.

Acute Kidney Injury↗

[Partial somatotropin deficiency in a case of chronic renal insufficiency with renal transplantation. Efficacy of treatment with human growth hormone].

The authors report a case of chronic renal failure with a -4 SD growth retardation. The patient underwent dialysis and then transplantation. Investigations to determine somatotropin deficiency showed dissociated results of 4 stimulation tests: 2 normal responses and 2 low responses. Treatment with human growth hormone (10 mg/kg/year) induced a dramatic acceleration of the growth velocity, which increased from 2 to 7 cm/year. After 18 months, the gain in height was 10 cm. Data submitted by several authors on Cushing's disease and the interrelations cortisone/somatotropin secretion suggest that continuous and/or alternate corticotherapy may be the cause of this partial somatotropin deficiency.

Adolescent↗

[HLA and Bf in idiopathic nephrotic syndrome in children: differences between corticosensitive and corticoresistant forms].

An association between HLA-DR7 and the steroid sensitive idiopathic nephrotic syndrome in the children has already been reported. Immunogenetic data in the less frequent steroid resistant form of this disease have never been published. In this study, we analyse HLA-A, B and DR typing in 99 cases of nephrotic children divided in 72 with the steroid sensitive (SS) form and 27 with the steroid resistant (SR) syndrome, in comparison with those of 207 healthy controls; Bf allotypes were determined in 53 of the patients. The results show the increased frequency of DR7 in the SS syndrome (75% vs 30%, RR = 6.9, pc less than 10(-6), while the SR one is more associated to DR3 (52% vs 27%, RR = 3, p less than 0.004). In the SS patients, atopy is associated to DR7 (p less than 0.001), which is not the case in the SR group. Furthermore, a high relative risk is associated to the phenotype DR3/DR7 (30% vs 4%; RR = 9.3; pc less than 0.0004), for the SR disease; besides, this phenotype is associated to an early onset of the disease and to lesions of focal sclerosis. Thus a heterozygous effect in the SR form of idiopathic nephrotic syndrome of children has been demonstrated; the steroid sensitive and the steroid resistant forms of the disease seem to have different immunogenetic components.

Adrenal Cortex Hormones↗

[Pyeloureteral junction syndrome in children. Apropos of 178 cases].

The authors review 178 cases of anomalies of the pyelo-ureteric junction, with a total of 189 anomalies. Almost one half of the cases were less than 5 years old (47.2%) and about 1/4 were less than 1 year old (24.7%). In girls, the incident which reveals the diagnosis is most often an infection and in boys, it is pain or haematuria. 26% of the cases had an associated urological lesion (23 cases of vesico-ureteric reflux, 8 cases of renal stones, 5 cases of mega-ureter and 4 cases of horseshoe kidney). 133 children were operated, essentially by resection of the pyelo-ureteric junction with an upstream by-pass. From the 126 cases with sufficient follow-up, 48 (38.1%) obtained great improvement, 63 (50%) a moderate improvement and 15 (11.9%), a stabilisation of the dilatation. In all cases except 3, the pyelo-ureteric junction was perfectly catheterizable. The histological lesions of the junction were variable and difficult to systematise, but usually extended beyond the junction to involve a large part of the pelvis. The variability of the caliceal dilatation makes any attempt at classification difficult. The authors recommend cystography in every case, as associated reflux was discovered in 16% of cases. Retrograde uretero-pyelography is practically no longer indicated. The isotope kidney scan with DTPA technetium, with a hyper-diuresis test, is often used, as it gives an idea about the clearance of filtration and about the degree of obstruction. Quantitative DMSA kidney scan can reveal the effects on the renal parenchyma. Conservation of the isthmus of a horseshoe kidney never interferes with the uretero-pyelic re-implantation. After correction of the anomaly of the uretero-pyelic junction, 5 cases of unilateral reflux out of 8 and 3 cases of bilateral reflux out of 10 resolved spontaneously.

Adolescent↗

HLA-DR7 in children with idiopathic nephrotic syndrome. Correlation with atopy.

Idiopathic nephrotic syndrome (INS) of childhood is likely to be underlain by an immunopathological mechanism; we investigated the presence of immunogenetic HLA markers in this disease. Fifty-four unrelated INS-affected children, among them 20 with an allergic status, were studied for 33 HLA-A,B and 6 HLA-DR antigens. The results were compared to those obtained in 49 children with glomerulonephritis, 28 children with atopy but without nephropathy, and 91 healthy blood donors. The HLA-A and B antigen frequencies were not significantly different from normal frequencies. The incidence of HLA-DR7 was significantly increased in INS-affected patients as compared to the other groups (66.7% in patients vs 31.1% in healthy controls; corrected P value less than 0.001; relative risk = 4.4), and more so in those with atopy than in those without atopy (90% vs 46%; P = 0.002). The frequency of this antigen is not increased in atopic non-nephrotic children. No relationship between HLA-DR7, clinical outcome and steroid-responsiveness was found. We suggest that the pathogenesis of INS could be influenced by an HLA-linked immune response gene, especially in its atopy associated form.

Child↗

Comparison of progressive antithrombin activity and the concentration of three thrombin inhibitors in nephrotic syndrome.

In order to compare the plasmatic progressive antithrombin activity to the concentration of three thrombin inhibitors, antithrombin III (AT III), alpha 2 macroglobulin (alpha 2 M), alpha 1 anti-trypsin (alpha 1, AT) in nephrotic syndrome, a prospective study was carried out on a group of 28 children affected with the disease. A dramatic reduction of the level of AT III and of alpha 1 AT, two inhibitors of molecular weight close to that of albumin, was observed. The decreased level of AT III was counterbalanced by an increase in alpha 2 M. This phenomenon accounts for the increased progressive antithrombin activity observed in all the affected children. It is suggested that the above compensatory mechanism explains the absence of thrombotic accidents in this series and that the benefit of heparin therapy is doubtful in these conditions.

Adolescent↗