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Biomedical subjects

I Durieu

Publications and source records attributed to I Durieu.

At least 55 records · Page 3Linked to original sources

[Fertility and cystic fibrosis. Management at pregnancy].

Life expectancy of patients with cystic fibrosis has regularly increased in recent years, currently exceeding the age of 30 years. The disease now concerns adolescents and young adults more than children. Thus problems linked to fertility have to be considered by physicians caring for adult patients. In males, sterility is quite constant. Changes are related to bilateral agenesis of the efferent ducts leading to excretory azospermia which is one of the major diagnostic criteria leading to the diagnosis of cystic fibrosis in adults. This form of sterility may be relieved by removal of sperm from the epididymis to achieve in vitro fertilization. In the female, fertility is sometimes diminished either by disease-specific abnormalities (tubal epithelium and cervical mucus) or impaired by respiratory failure or nutritional factors. Pregnancy is however possible and is observed in an increasing number of cases. Pregnancy does not appear to alter the general prognosis of the disease as long as cardiorespiratory function is preserved. In all cases, a prognostic evaluation and genetic advice are indispensable.

Adolescent↗

[Association of Biermer's anemia and gastric carcinoid tumors].

BACKGROUND: The occurrence of gastric carcinoid tumors during the cure of pernicious anemia has been reported in the literature. Concerning a new case, we review physiopathological data of this infrequent association. CASE REPORT: We report the case of a 47-year-old women with pernicious anemia in which gastroscopy found two small tumors developed from enterochromaffin-like cells. CONCLUSION: These tumors are usually latent and developed from enterochromaffin-like cells. The pathologic and histologic diagnosis is based on positive chromogranin A immunomarking. The physiopathological mechanism studies are in favour of the predominant part of hypergastrinemia following achlorhydria. Usually, evolution is benign and surgical or endoscopic resection recommended.

Anemia, Pernicious↗

[Necrobiotic xanthogranulomatosis: a cutaneous manifestation of a monoclonal IgM gammopathy].

INTRODUCTION: Necrobiotic xanthogranulomatosis is a rare cutaneous disorder usually associated with monoclonal gammopathy. Most frequently, cutaneous lesions involve the face (periorbital region) and the trunk, and are characterized by indurated xanthomatous plaques and nodules. EXEGESIS: We describe a patient presenting with necrobiotic xanthogranulomatosis that was unusual, as it was associated with IgM kappa monoclonal gammopathy of undetermined clinical significance and because no periorbital involvement was observed. CONCLUSIONS: Necrobiotic xanthogranulomatosis should be distinguished from other cutaneous manifestations associated with plasma cell dyscrasias, such as normolipemic plane xanthoma. It can be associated with IgM gammopathy.

Cryoglobulinemia↗

Medial temporal lobe atrophy in stroke patients: relation to pre-existing dementia.

OBJECTIVE: The links between stroke and Alzheimer's disease seem to be closer than expected by chance. In a previous study it was shown that up to 16% of patients admitted for stroke had pre-existing dementia. Medial temporal lobe atrophy (MTLA) is strongly associated with Alzheimer's disease. The aim of this study was to determine the prevalence of MTLA and its relation with pre-existing dementia. METHOD: The study was conducted on 170 consecutive stroke patients (87 women; median age 75 years; 152 infarcts), who underwent non-contrast CT with temporal lobe oriented 2 mm contiguous slices at admission. A cut off point of 11.5 mm was used to differentiate patients with and without MTLA. Pre-existing dementia was assessed using the informant questionnaire on cognitive decline in the elderly (IQCODE) with a cut off score of 104. RESULTS: Ninety four patients (55.3%) had MTLA, of whom 23 (24.5%) had pre-existing dementia; of 76 patients without MTLA, only four (5.3%) had pre-existing dementia (p=0.0007). The logistic regression analysis with MTLA as dependent variable found the following independent variables: increasing age (p<0.05), and global cerebral atrophy scores (p<0.01). The IQCODE scores just reached significance (p=0.05). CONCLUSION: Stroke patients with MTLA are more likely to have pre-existing dementia; this suggests that Alzheimer's disease might contribute to the dementia syndrome. A longitudinal follow up is now necessary to determine whether stroke patients with MTLA and without pre-existing dementia are at increased risk of Alzheimer's disease over subsequent years.

Adult↗

Subepithelial fibrosis and degradation of the bronchial extracellular matrix in cystic fibrosis.

Cystic fibrosis is a genetic disease caused by mutations of the cystic fibrosis transmembrane conductance regulator gene. Chronic inflammation and proteolysis lead to progressive damage of the bronchial wall. Extracellular matrix determines the structural organization and the mechanical properties of lung airways. It was thus examined in nine patients with cystic fibrosis (six bronchial biopsies and three lobectomies) in order to assess its level of alteration. The submucosal changes in matrix protein distribution were analyzed by immunochemistry and electron microscopy: the subepithelial basal lamina was thinned; an acellular collagen fiber layer composed of interstitial collagens (types I and III) subtended by tenascin and devoid of elastin-associated microfibrils was deposited beneath the basal lamina; this dense fibrous deposit generally formed a thick layer and could extend into the bronchial wall; the bronchial elastic framework lost arborescent distribution and appeared slender, packed, or lacunar; ultrastructural observation gave evidence for elastic and collagenic fiber lysis. Proteolytic activity is probably the major cause of matrix degradation. Fibrosis appears as a repair process rather than as an active fibrogenesis. The reversibility of extracellular matrix alterations is an important challenge and various interventions such as anti-inflammatory treatments can be targeted to halt or reverse this degradation process.

Adolescent↗

[Hydatid cyst of the heart].

BACKGROUND: We report an unusual localization of a hydatid cyst: the septum interventriculare. CASE REPORT: A 60-year-old algerian man with hypertension was treated for cardiac insufficiency with hypereosinophilia. Cardiac echography showed a round tumor in the septum interventriculare. Serologic tests for hydatidosis were positive. Computed tomography and magnetic resonance imaging were consistent with the diagnostic of hydatid cyst. Surgical treatment was rejected because of severe underlying hypertensive cardiopathy. DISCUSSION: Cardiac hydatidosis is uncommon, but may be revealed by cyst rupture. Treatment requires surgery and associated medical management with albendazole requires further evaluation.

Cardiomyopathies↗

[Muscular sarcoidosis: apropos of a case].

We report the case of a 75-year old woman presenting sarcoid myopathy with pseudohypertrophy and skin involvement. Muscular biopsy confirmed the diagnosis of sarcoidosis. Symptomatic muscle involvement in sarcoidosis is rare. Three forms are described: myopathic, myositic and nodular. The relevance of imaging techniques is reviewed. Treatment is based on corticotherapy which is less efficient in myopathic form and in this case we had to use methotrexate with success.

Aged↗

[Male infertility caused by bilateral agenesis of the vas deferens: a new clinical form of cystic fibrosis?].

Congenital bilateral absence of vas deferens causes male excretory infertility and represents 1 to 2% of male infertility. Because of a genotypic similarity with cystic fibrosis, the possible in vitro fertilization with epididymal sperm requires careful genetic counselling. We studied genotype, sweat chloride concentration, respiratory function tests, sinus abnormalities, pancreatic and hepatic functions in 22 subjects with congenital bilateral absence of vas deferens. Among them, four were compound heterozygotus, all of them with the R117H mutation. Ten had a positive sweat test, one of them also being compound heterozygotus. Congenital bilateral absence of vas deferens and double mutation or positive sweat test led to high probable cystic fibrosis diagnosis in 13 subjects. Six subjects were heterozygotus for one cystic fibrosis mutation, criterium which is not sufficient for cystic fibrosis diagnosis; five of them had sinus abnormalities, present in 11 of the 22 subjects. Only three patients had no mutation nor sweat chloride abnormalities. This work confirms the high frequency of cystic fibrosis mutations in males with congenital bilateral absence of vas deferens, with a higher frequency of positive sweat test than in other publications, and a high frequency of sinus abnormalities. This monosymptomatic phenotype of cystic fibrosis suggests new hypotheses for a relationship between genotype and phenotype.

Adult↗

Preexisting dementia in stroke patients. Baseline frequency, associated factors, and outcome.

BACKGROUND AND PURPOSE: The link between stroke and degenerative dementia, especially Alzheimer's disease, is closer than expected by chance. Dementia after stroke may be due to the cumulative effect of vascular and degenerative changes. The prevalence of dementia just before stroke onset remains unsettled. The aim of this study was to determine the frequency of preexisting dementia in stroke patients, associated factors, and consequences on outcome. METHODS: We evaluated the cognitive functioning prior to stroke in 202 consecutive patients with ischemic or hemorrhagic stroke by means of the Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE). We classified in the dementia group patients with IQCODE scores of 104 or more. Six months after stroke onset, survivors underwent a battery of neuropsychological tests. RESULTS: Thirty-three patients were demented before stroke (16.3%; 95% confidence interval, 11.2 to 21.4). There was no diagnosis of dementia in 32 of these 33 patients. We determined by logistic regression analysis that female sex, family dementia, leukoaraiosis, and cerebral atrophy are independently associated with prestroke dementia. All survivors who had IQCODE scores of 104 or more at the acute stage met criteria for dementia 6 months later. CONCLUSIONS: Our study showed that one sixth of stroke patients have preexisting dementia. Therefore, some patients with so-called "poststroke dementia" probably had unrecognized preexisting dementia.

Adult↗

[Dermatopolymyositis and primary biliary cirrhosis. A rare association].

We report a case of the uncommon association of dermatomyositis and primary biliary cirrhosis in a causasion male of 48-year-old. Diagnosis of dematomyositis was made because of muscle weakness, loss of weight, skin telangiectasia, elevated serum concentration of creatine kinase, polyphasic low amplitudes waves on electromyography and histologic confirmation on muscle biopsy. Diagnosis of primary biliary cirrhosis was made because of elevated values of alcaline phosphatase and gamma glutamyl transferase, elevated values of type II mitochondrial antibody and compatible histological lesions on liver biopsy. We found only ten case reports associating polymyositis and primary biliary cirrhosis. We hypothesize that hepatic and muscle mitochondrial dysfonction may be involved.

Dermatomyositis↗

Dementia in stroke.

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Cerebrovascular Disorders↗