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Biomedical subjects

I Durieu

Publications and source records attributed to I Durieu.

At least 37 records · Page 2Linked to original sources
PubMed2000

[Cystic fibrosis. Fertility and reproduction].

Fertility and reproduction are now frequent questions for cystic fibrosis adult patients. Females are sometimes subfertile because of abnormal cervical mucus viscosity or anovulatory cycles. After genetic counseling and evaluation of respiratory prognosis, assisted reproduction can be proposed. Fertility is frequently normal and contraception should be proposed. Oral contraception appears to be safe. Pregnancy in cystic fibrosis women should be undertaken after genetic counseling and clinical evaluation; the prognosis for the mother is not modified for patients with stable pulmonary status and FEV1 up to 30% of predicted with correct nutritional status. Preterm delivery is frequent. Usual therapeutics should be continued unless ursodeoxycholic acid or nonsteroidal antiinflammatory drugs. Usual antipseudomonas antibiotics can be used; pulmonary exacerbations have to be treated early. Almost all cystic fibrosis men are infertile because of congenital bilateral absence of vas deferens (CBAVD). CBAVD can revealed incomplete cystic fibrosis phenotypes associated with specific genotypes. After genetic counseling, intracytoplasmic injection after epididymal puncture can be proposed. In all cases, it is important to evaluate the life prognosis, before pregnancy or paternity and to propose genetic counseling, essentially depending on the genotype of the spouse.

Adult↗
PubMed1999

[Hypokalemic quadriplegia with distal tubular acidosis revealing a a case of primary Gougerot-Sjögren syndrome].

BACKGROUND: Primary Sjögren's syndrome is associated in 6 to 25% of cases with kidney involvement Most often it is a chronic interstitial nephritis, generally asymptomatic but sometimes responsible for distal tubular acidosis which can be complicated by severe hypokalemia. CASE REPORT: A 48-year-old woman had hypokalemic quadriplegia, distal renal tubular acidosis and chronic interstitial nephritis at kidney biopsy. Primary Sjögren's syndrome was diagnosed. DISCUSSION: We discuss briefly the clinical, biological and immunological features of 18 similar cases reported in the literature.

Acidosis, Renal Tubular↗
PubMed1999

[Agenesis of the vas deferens in male infertility. A tentative classification based on 39 cases].

OBJECTIVES: We attempted to establish a classification of vas deferens agenesia observed in male infertility to better ascertain the pathophysiology involved and help guide genetic counselling. PATIENTS AND METHODS: Among 387 men consulting for infertility, agenesia of the vas deferens was confirmed by transrectal ultrasonography in 39. A search for 13 different cystic fibrosis mutations was performed in subjects without associated renal agenesia. RESULTS: Among the 39 cases, we identified 4 ultrasonographic categories: unique bilateral agenesia (25 cases), bilateral agenesia associated with renal agenesia (1 case), unique unilateral agenesia (6 cases) and unilateral agenesia with renal agenesia (7 cases). Presence or absence of a seminal vesicle was variable. A cystic fibrosis mutation was observed in 64% of the bilateral cases and in none of the unilateral cases. The delta F 508 mutation accounted for 62% of the mutations. Phenotypically, there was not distinction between patients with and without a mutation. In cases of unilateral agenesia of the vas deferens, azoospermia was more frequent in unique forms than in forms with associated renal agenesia. CONCLUSION: Presence or absence of a mutation does not affect the pathophysiology of vas deferens agenesia in cases without associated renal agenesia. Genetic counselling should however take into account the presence of an associated mutation. Vas deferens agenesia associated with renal agenesia is not associated with cystic fibrosis mutations and results from a different pathogenic mechanism.

Adult↗
PubMed1999

Laboratory data in healthy volunteers: reference values, reference changes, screening and laboratory adverse event limits in Phase I clinical trials.

OBJECTIVE: Laboratory data are key evaluation procedures for Phase I clinical pharmacology for two reasons. Firstly, laboratory data are used within the screening process to exclude subjects with asymptomatic diseases, which could result in increased danger to themselves or confuse interpretation of the study results. Secondly, during study implementation, safety evaluation and in particular maximum tolerated dose determination have to be done by a case-by-case analysis, sometimes using laboratory adverse events (LAEs). Thus, relevant limits are needed to discriminate between a usual common variation and a significant abnormality, which is considered to be a LAE. This report presents laboratory data distribution, reference values and reference changes and, based on previously published new methods, suggests inclusion limits at screening and laboratory adverse event limits for analysis during study implementation. SUBJECTS AND METHODS: Nine hundred and twenty-seven young healthy male volunteers were recruited in one centre (Association de Recherche Thérapeutique). A standard screening process was carried out. Protocols were approved by the local ethics committee. Blood sampling was performed in the same conditions. Reference values (at screening and at baseline) were determined by a non-parametric procedure selecting 2.5% and 97.5% of the distribution of data. Reference changes were also defined as the 2.5-97.5% interval of distribution of the variations between the end of treatment and baseline. Inclusion limit and LAE limit methods of determination used had been specified in previous articles. RESULTS: Detailed results of laboratory data distribution, reference values at screening and at baseline, reference changes, inclusion limits and LAE limits are presented in tables with number of subjects, mean, median, standard deviation, minimal and maximal values and the 2.5-97.5% interval for each laboratory parameter. CONCLUSION: The key aims of this paper are to provide clinical pharmacologists with data, reference values or changes obtained in the real conditions of Phase I study implementation, and to propose relevant limits, either for screening as inclusion limits, or during studies as LAE limits. Thus, these data, reference values and specific limits improve the capacity to screen healthy volunteers and to analyse LAEs during Phase I studies.

Adult↗
PubMed1999

Sclerosing cholangitis in adults with cystic fibrosis: a magnetic resonance cholangiographic prospective study.

BACKGROUND/AIMS: Liver disease is a leading cause of morbidity in adult patients with cystic fibrosis. Diagnosis of limited liver involvement in asymptomatic patients is important since a safe and effective treatment with ursodeoxycholic acid can be used. We carried out a prospective open study to describe the intrahepatic biliary lesions using magnetic resonance cholangiography. METHODS: Twenty-seven adult patients with cystic fibrosis were prospectively enrolled, whatever their hepatobiliary status. All patients underwent liver function tests, ultrasonography and magnetic resonance cholangiography. Magnetic resonance cholangiograms were acquired on a Philips 1.5 Tesla unit using a 3D TSE MR sequence. Acquisition parameters (120 slices, 1.6 mm thickness, interslice overlap 0.8 mm) were followed by MIP reconstruction in two orthogonal planes. Magnetic resonance cholangiography images were assessed for the presence of stenosis, dilatations and rigidity corresponding to current criteria of cholangitis. Among the 27 cystic fibrosis patients, 18 (Group I) fulfilled none of the clinical, biological or ultrasonographic criteria of liver disease; the remaining nine (Group II) fulfilled the criteria for liver disease. In every patient, current causes of secondary sclerosing cholangitis had been excluded. RESULTS: All the Group II patients had abnormal magnetic resonance cholangiograms with features resembling those of primary sclerosing cholangitis in five, and simple biliary lesions in four. Nine Group I patients had abnormal magnetic resonance cholangiograms with primary sclerosing cholangitis-like lesions in five and simple biliary lesions in four. Magnetic resonance cholangiography anomalies were always dilatations, either isolated or associated with strictures and rigidity, both resembling those seen in cholangitis. They were seen in all the patients with known liver disease and in half the patients without evidence of liver disease. CONCLUSION: This study confirms the high frequency of intrahepatic biliary abnormalities in CF patients, which is probably underestimated by clinical, biological and ultrasonographic evaluation. The magnetic resonance cholangiography technique could be useful to detect early intrahepatic biliary tract involvement in cystic fibrosis patients.

Adult↗
PubMed1999

[Primary sarcoma of the pulmonary artery. Case report].

INTRODUCTION: Sarcoma of the common pulmonary artery is a rare malignant tumor that can mimic pulmonary embolism. EXEGESIS: We report a case of a pulmonary artery sarcoma that occurred in a 60-year-old woman and had an unusual (4-year duration) evolution. CONCLUSION: Early diagnosis with adequate surgical procedures (complete resection) leads to better prognosis.

Angiography↗
PubMed1999

[Constrictive bronchiolitis obliterans. Apropos of a case].

INTRODUCTION: Constrictive bronchiolitis obliterans is defined histologically as obliteration of the lumina of bronchioles by inflammatory tissue elements and progressive destruction of bronchioles, eventually replaced by fibrotic tissue. Most of the cases, which are not associated with either solid organ or bone marrow transplantation, occur in the development of rheumatoid arthritis. EXEGESIS: We present the case of a 73-year-old woman who developed severe constrictive bronchiolitis obliterans without readily identifiable cause, rapidly fatal despite corticosteroid therapy. The patient had antinuclear antibodies (titer 1/1024) and antiphospholipid antibodies, but there was no anti-DNA, anti-ENA or anti-beta 2GPI antibodies. Tests for rheumatoid factor were negative. Cryoglobulinemia was present (monoclonal IgM Kappa). CONCLUSION: In this case, constrictive bronchiolitis obliterans was associated with serologic abnormalities and cryoglobulinemia. However, were was no clinical evidence of connective tissue disease.

Aged↗
PubMed1999

Acute posterior multifocal placoid pigment epitheliopathy associated with Wegener's granulomatosis.

PURPOSE: To report acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in the course of systemic Wegener's granulomatosis. METHODS: A complete ophthalmologic evaluation was undertaken, including fluorescein and indocyanine green angiography. RESULTS: Two patients diagnosed with Wegener's granulomatosis developed multiple white-yellow subretinal areas with smooth margins typical of APMPPE. Case 1 presented with bilateral choroidal involvement and a granulomatous anterior uveitis. In Case 2, placoid lesions were diagnosed 2 months after onset of systemic Wegener's granulomatosis. In both cases, fluorescein angiography showed an early hypofluorescence and a late staining of the lesions. Indocyanine green angiography revealed early and late hypofluorescence corresponding to the areas seen clinically. Wegener's granulomatosis was diagnosed histologically in both cases. After combined steroid and cyclophosphamide therapy, the lesions healed, and after a follow-up period of 1.5 and 4 years, respectively, funduscopic and angiographic examinations showed cicatricial lesions. CONCLUSION: Acute posterior multifocal placoid pigment epitheliopathy is a rare manifestation of Wegener's granulomatosis and should be considered a possible systemic involvement of the disease. A prompt, thorough investigation should be undertaken for an underlying systemic disorder.

Acute Disease↗
PubMed1999

Fas and Fas ligand expression in cystic fibrosis airway epithelium.

BACKGROUND: Cystic fibrosis (CF) is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and defective expression of CFTR protein in epithelial cells. The main cause of mortality in CF is linked to chronic inflammatory and infectious airway processes. Recent studies have suggested perturbations in the apoptotic process in CF cell lines and enterocytes. A study was undertaken to investigate the expression of Fas and Fas ligand (FasL) in CF bronchial epithelium and CF tracheal cell lines. METHODS: Immunohistochemical staining for Fas (alkaline phosphatase anti-alkaline phosphatase) and FasL (immunoperoxidase) was performed in eight CF bronchial epithelial samples and four controls and immunohistochemical DNA fragmentation (TUNEL) was carried out in four CF patients and four controls. Immunofluorescence staining and flow cytometric analysis of Fas and FasL expression was performed in two human tracheal epithelial cell lines (HTEC) with normal and CF genotype. The dosage of serum soluble FasL was examined in 21 patients with CF and 14 healthy volunteers. RESULTS: FasL expression was markedly increased in patients with CF in both the ciliated and submucosal glandular bronchial epithelium compared with controls; Fas was similarly expressed in bronchial samples from controls and CF patients in both the ciliated epithelium and submucosal glands. High levels of DNA fragmentation were observed in CF but with some epithelial cell alterations. Serum concentrations of soluble FasL were frequently undetectable in patients with CF. In vitro, HTEC expressed Fas and FasL in both genotypes. A higher mean fluorescence intensity for FasL expression was noted in CF genotype HTEC with median (range) for six experiments of 74 (25-101) for CF cells and 42 (21-70) for non-CF cells. CONCLUSION: Fas/FasL interaction is probably implicated in the human CF airway apoptotic pathway. The mechanisms of induction of FasL expression and its role in inducing tissue damage or remodelling or in controlling local inflammatory cell apoptosis remain to be determined.

Adolescent↗
PubMed1999

Confusional state in stroke: relation to preexisting dementia, patient characteristics, and outcome.

BACKGROUND AND PURPOSE: Acute confusional state (ACS) is frequent in hospitalized stroke patients. We previously showed that 16% of patients admitted for a stroke have preexisting dementia. The extent to which preexisting cognitive decline is associated with a risk of ACS at the acute stage of stroke remains to be systematically examined. The aim of this study was to evaluate the prevalence of ACS in acute stroke patients, to study the influence of preexisting cognitive decline and other patient characteristics, and to evaluate the influence of ACS on outcome. METHODS: We diagnosed ACS using DSM-IV criteria and the Delirium Rating Scale with a cutoff of 10 in 202 consecutive stroke patients aged 40 years or older (median age, 75 years; range, 42 to 101 years). Cognitive functioning before stroke was assessed with the Informant Questionnaire on Cognitive Decline in the Elderly. RESULTS: Forty-nine stroke patients (24.3%; 95% CI, 18.3% to 30.2%) had an ACS during hospitalization. Using logistic regression analysis, we found preexisting cognitive decline (P=0.006) and metabolic or infectious disorders (P=0.008) to be independent predictors of ACS. Functional, but not vital, prognosis was worse in patients with ACS at discharge and 6 months after stroke. CONCLUSIONS: ACS occurs in one fourth of stroke patients older than 40 years. Its occurrence requires inquiry for a preexisting cognitive decline, which usually remains unrecognized in the absence of a systematic evaluation.

Acute Disease↗