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Biomedical subjects

B Francois

Publications and source records attributed to B Francois.

34 records · Page 2Linked to original sources
PubMed1989

Aetiology of membranous glomerulonephritis: a prospective study of 82 adult patients.

Eighty-two consecutive Caucasian adults (52 males, 30 females, aged 17-86 years) with membranous glomerulonephritis were prospectively evaluated for possible aetiological factors 1-4 weeks after renal biopsy. Presumed causes were identified in 17 patients (21%) as follows: drugs in five (D-penicillamine 3, captopril 1, fenoprofen 1); malignancy in four; chronic thyroiditis in three; systemic lupus erythematosus (SLE) in two; secondary syphilis in one; hepatitis B virus (HBV) infection in one and non-insulin-dependent diabetes mellitus in one patient. Except for age (patients with secondary membranous glomerulonephritis were older), clinical presentation and histological stage distribution did not differ between the secondary and the primary groups. Ten out of the 17 patients with secondary membranous glomerulonephritis (59%) achieved complete clinical remission within 12 months. The incidence of associated conditions in adults with membranous glomerulonephritis in this study corresponds with that reported in the few previous series. Although membranous glomerulonephritis is deemed to be idiopathic in most cases, it seems warranted to search for medication, malignancy, SLE, HBV infection, syphilis and thyroiditis as possible aetiological factors. Further evaluation should be orientated by the clinical context. An improved outcome of membranous glomerulonephritis may be expected insofar as the underlying condition is controlled.

Adolescent↗
PubMed1983

Lactic acidosis, neurological deterioration and compromised cellular pyruvate oxidation due to a defect in the reoxidation of cytoplasmically generated NADH.

Two patients, one dying at 25 days and one at 20 months had 'chronic' lactic acidaemia with a high lactate to pyruvate ratio. Both showed EEG abnormalities and seizure activity and both died of respiratory failure. Investigation of cultured skin fibroblasts from these patients revealed normal pyruvate dehydrogenase and pyruvate carboxylase activities but the cells showed a decreased ability to oxidase pyruvate which was returned to normal on the addition of methylene blue. Subsequent investigations revealed that the mitochondria from the patients' cells could oxidase pyruvate normally but that the cells had an abnormal NAD to NADH ratio under standard conditions of incubation. It was concluded that both children had a redox disequilibrium in the cytoplasmic compartment due to a problem in transporting reducing equivalents from the cytoplasmic to the mitochondrial compartments.

Acidosis↗
PubMed1983

Pharmacokinetics of disopyramide in patients with chronic renal failure.

The pharmacokinetics study of a single oral dose of 200 mg of disopyramide was performed in 22 normal control subjects and 33 patients with chronic renal failure (CRF). The latter were subdivided into 3 groups of 11 patients each as a function of the gravity of renal insufficiency. With the exception of maximum concentration (C max), which was only slightly modified, and of the apparent distribution volume which remained unchanged, all the other pharmacokinetic blood parameters (t max, concentration at 24th hour, elimination constant (ke h-1), elimination half-life, area under the curve and plasma clearance) were significantly modified in the CRF group; in particular, the elimination half-life was significantly increased (for 22 cases of CRF with mean plasma creatinine greater than 250 microM at 16.3 hours compared to 8.0 hours in controls). The urinary elimination of disopyramide was studied in 14 renal insufficiency patients and in 6 controls. The decreased rate of urinary excretion of disopyramide and its monodealkylated derivative (NMD), during the first 24 hours, was directly related to the severity of renal insufficiency. The ratio of urinary NMD/(disopyramide + NMD) was unchanged in CRF patients as compared to the controls. The results suggest that the dosage of disopyramide should be decreased when plasma creatinine values are greater than 250 microM, and creatinine clearance is less than 30 ml/min. The dose for a 70 kg subject would be 100 mg, administered every 12 hours.

Adolescent↗
PubMed1982

Ornithine transcarbamylase deficiencies in human males. Kinetic and immunochemical classification.

Enzymatic activities and kinetics of liver ornithine transcarbamylase (carbamoylphosphate:L-ornithine carbamoyltransferase) were studied in 16 human males with ornithine transcarbamylase mutations. In the same liver fragments, cross-reactive material was measured with specific anti-ornithine transcarbamylase antibody. These studies allowed us to describe five groups of mutations. Two of them were similar on the basis of their enzymatic properties and cross-reactive material amounts to two mouse ornithine transcarbamylase mutations:sparse-fur (spf) and sparse-fur with abnormal skin and hair (spf-ash).

Ammonia↗
PubMed1979

Screening for neonatal hypothyroidism by thyroxine and thyrotrophin radioimmunoassays using dried blood samples on filter paper.

A routine and automatized methodology for thyroxine (T4) and thyrotrophin (TSH) radioimmunoassay (RIA) using dried blood samples on filter paper is described. T4-RIA was performed on one single dot (5 mm diameter equivalent to 4 microliters of serum) while two dots were necessary for TSH-RIA. Reference filter papers were introduced in each assay for quality control. In a preliminary study on 4,155 neonates, samples generally obtained between the 5th--7th day gave a mean 'dot-T4' of 97.95 +/- 36.04 nmol/l and a mean 'dot-TSH' of 10.19 mU/l +/- 8.25, corresponding to 2.47 mU/l of serum. Within an 18-month period (November 1976-April 1978), a total of 16.522 neonates have been screened allowing detection of three cases of congenital hypothyroidism (incidence 1 : 5507), two cases of congenitally low TBG and thirty-three cases of transient hypothyroidism.

Female↗
PubMed1976

Peritoneal dialysis and exchange transfusion in a neonate with argininosuccinic aciduria.

Peritoneal dialysis rapidly reduced blood ammonia concentration in this child with arginino-succinic acid-lyase deficiency, whereas exchange transfusion did not. Yet this reduction in plasma ammonia level did not produce clinical improvement. We speculate that the effects of ammonia intoxication on the highly susceptible neonatal metabolism are due to an accumulation of toxic products and to an altered energy metabolism. Both aspects must be considered in any attempt to treat congenital hyperammonaemia.

Ammonia↗