PubMed Health⌕ Search

Biomedical subjects

P Landais

Publications and source records attributed to P Landais.

At least 109 records · Page 6Linked to original sources
PubMed1989

Childhood manifestation of autosomal dominant polycystic kidney disease: no evidence for genetic heterogeneity.

Autosomal dominant polycystic kidney disease (ADPKD) usually becomes symptomatic between the third and fifth decades. We studied ten families segregating for ADPKD in which children were observed with typical manifestations of the disease at birth or in early childhood. In these families, linkage analysis was carried out with a cloned DNA sequence from the alpha-globin locus known to be closely linked to the disease gene in adult onset ADPKD. In the families studied here, close linkage (theta max = 0.09 at zmax = 2.32) was also observed between the marker and disease loci. These results provide no evidence for genetic heterogeneity of ADPKD in families with early and adult onset.

Child↗
PubMed1989

Renal prognosis in Alport's and related syndromes: influence of the mode of inheritance.

Progressive hereditary nephritis is subdivided into Alport's syndrome (with extrarenal involvement) and hereditary nephritis without deafness. Three modes of inheritance have been described: X-linked dominant, autosomal dominant, and autosomal recessive. We reviewed the mode of inheritance in 48 kindred with hereditary nephritis (41 with Alport's syndrome and 7 with hereditary nephritis without deafness). It was presumed X-linked dominant in 34 Alport's syndrome and 6 hereditary nephritis without deafness, autosomal dominant in five hereditary nephritis and one hereditary nephritis without deafness, and autosomal recessive in two Alport's syndrome. We studied the cumulative renal survival of 149 patients, 128 (76 males, 52 females) with Alport's syndrome and 21 (10 males, and 11 females) with hereditary nephritis without deafness. Major prognostic factors were the patient's sex (median renal survival in males and females being respectively 32 versus 61 years in Alport's syndrome and 34 versus 57 years in hereditary nephritis without deafness), and the mode of inheritance (median renal survival in males being 25 years in X-linked dominant Alport's syndrome versus 51 years in autosomal dominant Alport's syndrome). The presence of hearing loss in the kindred or in the patient himself did not appear as a significant prognostic factor. We conclude that Alport's syndrome and hereditary nephritis without deafness are predominantly X-linked dominant diseases with the same renal outcome, and that in Alport's syndrome the patient's sex and the mode of inheritance are two independent prognostic factors.

Adult↗
PubMed1989

Diet-induced hyperlipoproteinemia and lipid accumulation within spontaneous arterial lesions in the rat.

The extent of diet-induced lipid accumulation evaluated histologically within rat caudal artery lesions, which represent sites of spontaneous intimal injury, was studied in relation to plasma cholesterol and lipoprotein changes in two consecutive experiments: 1) Rats were fed diets enriched with various amounts of cholesterol and saturated lipids from 5 to 12 weeks of age. A large range of values of cholesterolemia was observed at the time of sacrifice, and the more the diet was enriched in cholesterol and saturated lipids, the more the mean of the arterial score, which expressed the extent of lipid deposits within caudal artery lesions, was elevated. Among the biochemical parameters studied, in decreasing order, total cholesterol, LDLchol and VLDLchol were determinant for the arterial score. 2) Rats were fed a diet containing a moderate amount of lipids and the cholesterol concentration in the 3 classical plasma lipoprotein fractions was determined at 1.5, 3 and 6.5 weeks after starting the diet. In this experiment, statistical analysis showed that the degree of lipid accumulation within caudal artery lesions correlated only with the values of VLDLchol determined early (1.5 week) after starting the diet and not with those at the time of sacrifice. However, VLDLchol values correlated with those of LDL and total plasma cholesterol. The data from these two series of experiments suggest that in diet-induced hypercholesterolemia, the intensity of lipid deposits within areas of the arterial wall where spontaneous injury had occurred was related to the values of total plasma cholesterol as reflected both by VLDLchol and LDLchol. An independant effect of one of these lipoproteins cannot be demonstrated since they are correlated with one another.

Animals↗
PubMed1988

[Home parenteral nutrition for adults. Results of a multicenter survey in France].

Home parenteral nutrition (HPN) is a growing therapy in North America and Europe. This first multicenter retrospective study of HPN in France has collected data on 81 patients sent home before December 31st, 1985. Intestinal failure, secondary to short bowel syndrome, small bowel stenosis or fistula, was the main indication for HPN. In 95 p. 100 of the cases, the clinical nutritional status during HPN was either normal or subnormal. The annual incidence of catheter change for technical complication was 0.78 and the mortality rate was 1.2 p. 100. Social rehabilitation was recovered during HPN in 60 p. 100 of patients. Thirty percent of the patients died of their primary disease during HPN but 43 p. 100 were off treatment, and 27 p. 100 were on HPN at the end of the study. The cost of HPN was reduced by 64 p. 100 in comparison with the cost of parenteral nutrition carried out in hospital.

Adult↗
PubMed1988

Factors associated with early remission of type I diabetes in children treated with cyclosporine.

To improve criteria for entry into future trials of immunosuppression, we enrolled 40 children with recent-onset Type I insulin-dependent diabetes in a pilot trial of cyclosporine. Twenty-seven patients were able to discontinue insulin therapy 48 +/- 5 days after the start of immunosuppression. At four months, their fasting and postprandial blood glucose concentrations averaged 110 and 160 mg per deciliter (6.1 and 8.9 mmol per liter) with a mean hemoglobin A1c level of 6.15 percent. Seventy-five percent of these patients with early remission still did not need insulin at 12 months, and their glycemic control was similar to that at 4 months. The major differences between the 27 patients with remission and the 13 without remission were the duration of symptoms before diagnosis (26.8 vs. 48.0 days, P less than 0.01), the degree of weight loss (3.2 vs. 10 percent of body weight, P less than 0.001), the initial hemoglobin A1c level (10.7 vs. 13.2 percent, P less than 0.001), and the frequency of ketoacidosis (11 vs. 61.5 percent, P less than 0.001). The lesser degree of weight loss was the strongest independent predictor of remission. The response of C-peptide to intravenous glucagon (0.50 vs. 0.17 pmol per milliliter, P less than 0.05) was also an independent predictor. No differences were observed between the two groups of patients in age, sex, HLA phenotype, autoantibodies to insulin or islet-cell antigens, or doses or trough levels of cyclosporine. Only minimal manifestations of toxicity were detected over the period of observation. We conclude that early treatment with cyclosporine in children with recent-onset Type I diabetes can induce remission from insulin dependence, with half the patients not requiring insulin after a full year.

Adolescent↗
PubMed1988

Treatment of the childhood haemolytic uraemic syndrome with plasma. A multicentre randomized controlled trial. The French Society of Paediatric Nephrology.

Seventy-nine children with haemolytic uraemic syndrome (mean age 28 months) were randomly assigned either to a group receiving plasma infusions (plasma group, n = 39) or to a group treated conservatively (control group, n = 40). The duration of haemolysis, thrombocytopenia and anuria was similar in the two groups. Serum creatinine levels were similar in the two groups at the 1-month follow-up but were higher in the control group at 3 months (plasma group 49 +/- 14, control group 66 +/- 28 mumol/l; P less than 0.02) and at 6 months (plasma group 48 +/- 13, control group 63 +/- 21 mumol/l; P less than 0.005). The prevalence of proteinuria was also higher in the control group at the 6-month follow-up (plasma group 17%, control group 46%; P less than 0.02). However, differences were no longer significant after 1 year. Renal tissue was examined in 54 cases (plasma group, n = 27; control group, n = 27). Diffuse cortical necrosis was present in 7 cases in the control group but was absent in the plasma group (P less than 0.02). Taking into consideration the higher serum creatinine levels, the higher prevalence of proteinuria during the first 6 months of follow-up in the control group and the presence of diffuse cortical necrosis in this group compared with the plasma group, we conclude that plasma infusions should be regarded as beneficial. Further study is needed to determine which plasma fraction is involved.

Adolescent↗
PubMed1986

Bone-marrow transplantation for immunodeficiencies and osteopetrosis: European survey, 1968-1985.

In this retrospective analysis of allogeneic bone-marrow transplantation (BMT) carried out between 1969 and 1985 at fourteen European centres in 162 patients with sixteen different types of inherited immunodeficiencies and osteopetrosis, the overall survival with functional grafts was 51.7% (85 patients), with a minimum follow-up of 5 months. In patients with severe combined immunodeficiency HLA-matched (n = 41) and T-cell-depleted HLA-mismatched BMT (n = 46) resulted in 68% and 57% disease-free survival, respectively; after HLA-mismatched transplants, older age (greater than 6 months) and adenosine-deaminase deficiency resulted in poorer survival. Eight other lethal immunodeficiencies, including profound T-cell deficiencies, Wiskott-Aldrich syndrome, Kostmann syndrome, LFA-1/CR 3/p150,95 deficiency, and Chediak-Higashi syndrome as well as malignant osteopetrosis, have been successfully treated by BMT. In this group, survival with functional graft was 47% with HLA-matched and 29% with T-cell-depleted HLA-mismatched BMT. Engraftment failure was the major complication in this group. Poorer prognosis was associated with older patients, profound T-cell deficiencies, and the degree of HLA incompatibility.

Adolescent↗
PubMed1986

Analgesic and non-steroidal anti-inflammatory drug-associated acute renal failure: a prospective collaborative study.

During a one-year period analgesic and non-steroidal anti-inflammatory drug-(NSAID) associated acute renal failure (ARF) was recorded in 147 of 398 patients registered in 58 nephrology units. This figure represented 36.9% of drug-associated ARF, and 6.8% of total patients with ARF hospitalized during the same period. Drugs involved were primarily glafenin (79), NSAID (62), paracetamol (5) and phenacetin (1 case). Hypersensitivity reactions were documented in 32 patients. Acute tubular necrosis was found in 20, and interstitial nephritis (AIN) in 9 of 34 biopsied patients. All patients in the glafenin group and 71.4% in the NSAID group recovered fully or regained previous renal function (p less than 0.01). Permanent renal damage (9.5% of total cases) was more frequent in patients with AIN than in those with other types of ARF (p less than 0.001). Preventive measures should be especially directed to older patients receiving NSAID, by avoiding the combined use of drugs potentiating their action and by correcting any predisposing factor to ARF.

Acetaminophen↗
PubMed1986

[Acute renal failure associated with drugs or iodinated contrast media. Results of a cooperative multicentric study by the Nephrology Society].

During a one-year period, drug-associated acute renal failure (ARF) was prospectively recorded in 398 patients, registered in 58 french nephrology Units. Drugs involved were primarily antibiotics, mainly aminoglycosides, glafenine, non-steroidal antiinflammatory drugs and contrast media. Hypersensitivity reactions were reported in 69 patients. Renal biopsy, performed in 81 instances, showed acute tubular necrosis in 42 and acute interstitial nephritis in 20 patients. Hypotension, sodium depletion and/or cardiac failure were predisposing factors in 198 cases. Fifty patients died, 251 recovered fully or regained previous renal function, and in 93 permanent renal damage remained. Advanced age, oliguria, severe ARF, and preexisting cardiac, hepatic or renal insufficiency were poor prognostic factors. Prevention of drug-associated ARF should be directed to high-risk patients, particularly those receiving aminoglycosides and contrast media.

Acute Kidney Injury↗
PubMed1985

Biological measurements of Crohn's disease activity--a reassessment.

Fifty-eight patients with Crohn's disease were studied in order to compare disease activity as measured by a clinical scoring system, with nutritional status, the laboratory measurements reflecting nutritional status, and the serum levels of acute phase proteins, using principal component analysis and stepwise multiple regression analysis. We confirmed that orosomucoid is the laboratory measurement which correlates best with clinical assessment of disease activity, followed by ESR and C-reactive protein. Serum albumin correlated poorly with clinical disease activity, although body weight showed an inverse correlation. Serum fibronectin, a protein involved in the production of fibrous tissue, did not detect the presence of fibrous strictures in the intestine.

Acute-Phase Proteins↗
PubMed1984

Cholangiocellular carcinoma in polycystic kidney and liver disease.

Cholangiocellular carcinoma developed in two uremic patients with polycystic kidney and liver disease, who had been treated with intermittent hemodialysis for one and nine years. In one case, in situ transformation of the liver cyst epithelium into cholangiocellular carcinoma could be demonstrated. The incidence of cholangiocellular carcinoma in patients undergoing long-term dialysis for polycystic kidney and liver disease, however, has yet to be determined.

Adenoma, Bile Duct↗
PubMed1983

Assessment of appropriate laboratory measurements to reflect the degree of activity of ulcerative colitis.

Eleven laboratory parameters were compared with the clinical grading of disease activity in 44 patients with ulcerative colitis, using multiple stepwise regression analysis to identify which measurements significantly reflect the severity of the disease. Only 2 parameters, serum orosomucoid and haematocrit showed significant correlation with clinical status. It is suggested that in the method of assessing disease activity proposed by Truelove and Witts the haemoglobin, which in this study was found to have no value, could be replaced by an equally simple test, the haematocrit. Comparisons are made between the relative usefulness of these parameters in ulcerative colitis and Crohn's disease.

Adult↗