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Biomedical subjects

S Guez

Publications and source records attributed to S Guez.

33 records · Page 2Linked to original sources
PubMed1998

Creutzfeldt-Jakob disease with long duration and panencephalopathic lesions: molecular analysis of one case.

A 49-year-old woman presented with isolated aphasia followed by dementia and ataxia with a duration of 4 years. Histopathologically there was panencephalic involvement, status spongiosus, and kuru-type plaques. Molecular analysis showed heterozygosity at codon 129 in the prion protein (PrP) gene, and type 2 protease-resistant PrP. The comparison between this case and those previously reported suggests that the panencephalopathic variant of Creutzfeldt-Jakob disease (CJD) is an aspecific end-stage condition displayed by most if not all CJD variants in individual patients with an unusually prolonged course.

Biopsy↗
PubMed1997

[Prevalence of clinical manifestations of allergic reactions in HIV infection. Cross sectional study of 115 subjects].

A cross sectional survey was set up to study the relation between the prevalence of allergic-type reactions during HIV infection course. For each patient, a standardized interview about recent allergic-type manifestations (RATM), skin prick-tests to six common airborne allergens, IgE serum level were done. Among the 115 included patients, the mean CD4 lymphocyte count (CD4) was 214.7/mm3 (range: 0-1328/mm3). RATM were found in 8.8% of patients with CD4 < 50, in 30% of patients with CD4 between 51 and 200, in 36% of patients with CD4 between 201 and 350 and in 11.5% of patients with CD4 < 350 (p = 0.03). The risk of presenting RATM was 4.8 times (95% confidence interval = 1.7-13.5) higher in patients with CD4 between 51 and 350 than in other patients (p = 0.003). The proportion of positive prick-tests did not significantly vary according to the level of CD4. The increased frequency of RATM in patients with CD4 between 51 and 350/mm3 could be due to an allergic predisposition acquired during the course of HIV infection. The mechanisms explaining the reduced frequency of allergic manifestations when immunodeficiency is profound (CD4 < 50/mm3) remain to be explained.

Adult↗
PubMed1996

[Prostacyclin in the treatment of hemolytic-uremic syndrome: apropos of a case].

A 52 year-old man was hospitalised for acute renal failure with thrombocytopenia and hemolytic anemia without oliguria. A haemolytic-uremic syndrome was diagnosed and prostacyclin infusion was started. Twenty-four hours later, the renal function improved as well as thrombocytopenia and anemia. Recovery occurred after 11 days of treatment. Haemolytic-uremic syndrome treatment is not well codified: plasmaphoresis, fresh frozen plasma, transfusions showed inconstant efficiency and data about prostacycline treatment are rare and often contradictory. Multicentric studies must be started in order to determine the precise benefit of this treatment.

Epoprostenol↗
PubMed1996

Shortcomings in predicting postnatal renal function using prenatal urine biochemistry in fetuses with congenital hydronephrosis.

Ten fetuses with hydronephrosis underwent one to seven urine sampling procedures at 23 to 36 weeks' gestation to evaluate renal function. Postnatally, the infants' renal function was assessed by a combination of serum creatinine measurement, ultrasonography and renal scintigraphy. Six infants had pyelo-ureteric junction obstruction, two had megabladder with megaureter, and two had vesico-ureteric reflux. All infants had normal serum creatinine levels at the time of postnatal follow-up, but five of the seven with unilateral involvement had moderate or severe renal damage. Abnormal urinary electrolyte concentrations were found antenatally in only two of them. For the three infants with bilateral hydronephrosis, postnatal evaluation showed moderately or severely damaged kidneys despite prenatal evidence of normal biochemical indexes. Fetal urine electrolyte measurement may be accurate in the diagnosis of renal dysplasia, but its sensitivity is poor in predicting moderate renal dysfunction.

Female↗
PubMed1995

Acute pyelonephritis as a cause of hyponatremia/hyperkalemia in young infants with urinary tract malformations.

Obstructive uropathy causes tubular resistance to aldosterone and severe metabolic imbalance may be precipitated by an episode of pyelonephritis. In the last 3 years we investigated 52 episodes of pyelonephritis (positive urine culture, elevated C reactive protein, fever, elevated neutrophil count) in 50 children between 15 days and 15 months of age. Ultrasonography voiding cystography and renal scintiscan were performed in all cases and i.v. urography in some. A salt-losing syndrome with hyponatremia and hyperkalemia (Na < 125 meq/liter; K > 6.3 meq/liter) was observed in 17 infants < 3 months, accompanied by plasma aldosterone concentration of 5000 to 23,000 pg/ml (normal value, < 1000 pg/ml). All these children had a severe urinary tract (UT) malformation (ureteropelvic junction stenosis in 7 cases, vesicoureteral reflux in 7, posterior urethral valves in 2, double system in 1). Thirteen infants < 3 months, 7 with no urinary tract malformations, did not have electrolyte imbalance. Pyelonephritis was diagnosed in 20 other patients ages 4 to 15 months, including 16 with severe UT malformations; 4 had normal UTs. We conclude that a salt-losing syndrome with tubular resistance to aldosterone can occur during pyelonephritis in young infants with congenital UT malformation, that the risk diminishes considerably or disappears after 3 months of age and that in the absence of UT malformation pyelonephritis does not cause acute sodium loss of clinical relevance.

Age Factors↗
PubMed1993

Evaluation of the peritoneal equilibration test in children on chronic peritoneal dialysis.

The peritoneal equilibration test (PET) is routinely performed in adults treated with chronic peritoneal dialysis to assess the peritoneal transport rate and to optimize treatment prescription. Only a few and not well-standardized studies on the PET have been reported in children. Twenty-six PET's were performed in 16 children, mean age 10.9 +/- 4.9 years, mean body weight (BW) 26.8 +/- 11.9 kg, treated with nightly intermittent peritoneal dialysis (NIPD). The PET was performed according to Twardowski. Forty mL/kg BW of 2.27% glucose solution were infused at a rate of 10 mL/kg BW every 2 minutes. In the simplified test, dialysate samples were taken at dwell times 0, 2 and 4 hours for glucose and creatinine. A blood sample was taken after a 2-hour dwell time for the same parameters. The standard PET (8 patients) consisted of dialysate samples at 0, 15, 30, 60, 120, and 240 minutes and blood samples at 0 and 240 minutes. Mean D/P ratio for corrected creatinine and D/D0 ratio for glucose at 2 hours were 51.6 +/- 11.6 and 50.4 +/- 9, respectively; at 4 hours 69.6 +/- 12 and 34.4 +/- 9.8, respectively. There was good correlation between D/P creatinine and D/D0 glucose at 4 hours (p < 0.0001). Patients were classified as high (3 cases), high-average (5), low-average (6), and low (2) transporters. A statistically significant difference was found between the curves obtained by the simplified PET and those of the standard PET in the first hour (r = 0.66; p < 0.05). In conclusion, the PET, modified for use in children, gave reliable and reproducible results.(ABSTRACT TRUNCATED AT 250 WORDS)

Biological Transport↗
PubMed1992

In vitro study of platelets and circulating mononuclear cells of subjects presenting an intolerance to aspirin.

Aspirine-sensitive asthma (ASA) is a disease defined only by clinical criteria. It is an intrinsic asthma related to a hypersensitivity to aspirin. The illness is linked to abnormalities in platelet and macrophage arachidonic acid metabolism. We assessed in vitro the platelet chemoluminescence (CL) and lymphocyte proliferative response of ASA patients. We observed that platelets from patients and control do not generate any CL in the presence of aspirin. Concerning the proliferative response of lymphocytes, the in vitro effect of aspirin depends upon the origin of the lymphocytes tested. Thus aspirin clearly enhances the proliferative response of lymphocytes from normal subjects but diminishes the thymidine uptake by lymphocytes from ASA patients. This discrepancy in the in vitro response of lymphocytes from normal subjects and patients might be useful for in vitro diagnosis of ASA.

Aged↗
PubMed1991

[Bronchial symptoms and respiratory function in the natural gas extraction industry. The role of Pi phenotype and chemical pollution].

The aim of this study was to measure, in workers in the chemical industry, the frequency of symptoms and/or alteration in respiratory function according to the Pi phenotype and the occupational environment of each subject. 188 men (mean age 33) participated in a cross sectional study which included: a questionnaire on the working conditions, smoking habits and respiratory symptoms, a lung function test assessing bronchial flow rates, residual volume and CO lung transfer, laboratory investigations with determination of the Pi phenotype, blood concentration of alpha-1-antitrypsin (alpha-1-AT) and antielastase activity in the serum, a study of outdoor environment in each occupation. The Pi phenotype was divided as MM (75.5%) and non MM (24.5%). Both serum alpha 1AT concentration and antielastase activity were lower in non MM subjects than in MM ones. There was no difference between the 2 groups for age, smoking, working conditions, bronchial symptoms or respiratory function values that were within a normal range. The MM subjects stated that they were more exposed to dust, gas and cold; their absence from work for respiratory disorders was more frequent although in a non significant manner and their flow rates at low lung volumes was paradoxically worse than in non MM subjects. It is concluded that neither the outdoor environment nor the Pi phenotype play a role in the respiratory risk which requires, to be more comprehensively evaluated, a prospective study.

Adult↗
PubMed1987

An unusual complication of the Le Veen shunt: a right atrial ventricular chamber pseudotumor.

We report the case of a right atrial-ventricular chamber thrombus discovered four years after insertion of a Le Veen shunt for treatment of refractory ascites. A two-dimensional echocardiogram, performed after the discovery of an isolated systolic murmur, demonstrated a "tumorlike" mass seated in both the right atrium and the right ventricle. The mass was surgically removed and histologic examination confirmed that it was a thrombus developed at the tip of the catheter. We propose that two-dimensional echocardiography should be performed periodically for the survey of such intracardiac devices.

Adult↗
PubMed1979

Modifications in the sarcoplasmic reticulum and subcellular calcium distribution in skeletal muscle in a case of Westphal's disease (hypokalemic periodic paralysis).

In a case of hypokalemic periodic paralysis with characteristic alterations of the sarcoplasmic reticulum (SR) in the skeletal muscle, subcellular calcium re-partition, as revealed with the pyroantimonate technique, appears disturbed during paralysis. Pyroantimonate precipitates, normally concentrated in the terminal cisternae of the SR, were localized in the T tubules, whereas the terminal cisternae appeared empty. The increase (about 14%) in muscular calcium during paralysis may result from the accumulation of calcium in the extracellular compartment (T tubules). Defects in calcium uptake and storage by the SR may be involved in the pathogenesis of the periodic paralysis syndrome.

Adolescent↗
PubMedDate not supplied

[Apropos of 2 cases of Scedosporium apiospermum nasosinusal infection].

We report two cases of nasosinusal infection caused by Scedosporium apiospermum and involving the nasal fossa in one case and the maxillary sinus in the other. The course of the disease varied according to the patient's immune status. The otherwise healthy patient (case n. 1) was completely cured by surgery, whereas the immunosuppressed patient (case n. 2) had local extension with pseudotumoral symptoms and lysis of the inter-sinusonasal septum; an early surgical treatment combined with local administration of a specific antifungal agent resulted in an apparent cure, but the patient died a few months later. A review of the literature confirms the invasive potential of this fungus which in immunosuppressed patients (by therapeutic immunosuppressants or by a debilitating disease) becomes a dangerous opportunistic organism. Cultures on Sabouraud's medium provide an accurate diagnosis and enable antifungal drugs to be tested, miconazole being the most regularly effective of them. In healthy subjects surgery is the sole treatment of nasal or sinusal lesions, while in all immunocompromised patients it must be combined with an antifungal treatment.

Aged↗