Isolated laryngeal tuberculosis: A diagnostic dilemma.

INTRODUCTION AND IMPORTANCE: Primary laryngeal tuberculosis (PLTB) is a rare condition. The symptoms and findings are not specific in most of the cases. Patients are diagnosed essentially based on histopathological examination and mycobacterial culture. CASE REPORT: We report the case of a 42-year-old woman who presented to our hospital with dysphonia and dysphagia. Direct laryngoscopy revealed a lesion of the supraglottis. CT scan found a diffuse thickening of the entire surface of the larynx. A biopsy confirmed the diagnosis of tuberculosis. CLINICAL DISCUSSION: Primary laryngeal tuberculosis is a rare clinical entity despite its close anatomical and physiological proximity to the lungs. CONCLUSION: Clinician should keep in mind the existence of primary laryngeal tuberculosis to avoid delayed diagnosis and treatment, which can lead to high morbidity and mortality.

[Thrombotic microangiopathies. Incidence, pathogenesis, diagnosis, treatment and prognosis]. / Les microangiopathies thrombotiques. Incidence, physiopathologie, tableau clinique, prise en charge thérapeutique et pronostic.

INTRODUCTION: The objective of this work was to review current data about the pathophysiology, clinical features, and treatment of thrombotic microangiopathies. CURRENT KNOWLEDGE: Thrombotic microangiopathies are microvascular occlusive disorders characterized by systemic or intrarenal aggregation of platelets, thrombocytopenia, and mechanical injury to erythrocytes. In thrombotic thrombocytopenic purpura, systemic microvascular aggregation of platelets causes ischemia in the brain and other organs. In the hemolytic-uremic syndrome, platelet-fibrin thrombi occlude predominantly the renal circulation. Thrombotic microangiopathy is a rare disorder whose varied clinical manifestations result from the formation of platelet-rich thrombi within the microvasculature and consequent tissue ischemia. The clinical features are acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. This diagnosis is of considerable importance because of the possible fulminant clinical course. Some atypical forms may be unrecognized. Plasma exchange is the current reference treatment of thrombotic thrombocytopenic purpura. However, in the light of recent publications, either infusions of concentrates of purified enzyme or more intensive immunosuppressive therapy would be more specific.

[Acute fatty liver of pregnancy. About 22 cases]. / Stéatose hépatique aiguë gravidique. A propos de 22 cas.

OBJECTIVE: To report the clinical experience, biochemical findings, complications and maternal outcome in patients with acute fatty liver of pregnancy (AFLP). PATIENTS AND METHODS: Retrospective study over a period of 11 years (1993-2003). The diagnosis of AFLP was confirmed by liver biopsy in 15 women. However, in 7 women a medical committee that took into account clinical symptoms, and laboratory findings assessed the diagnosis. RESULTS: Were included in this study, 22 women with a mean age of 30+/-5.4 years. Only 22.7% of cases were primigravid. The mean gestational age was 36+/-2.76 weeks (range 31-41 weeks). The fetus was a male infant in 75% of cases. Ten women were admitted in the hospital without jaundice. However 15 women had developed an icterus since their hospital admission or during ICU stay. The mean SAPS II on the ICU admission was of 24.86+/-11.2 points. Biological disturbances observed were mainly: liver cytolysis in 91% of cases, a trend to hypoglycaemia in 86%, a hypoprotidemia in 66.7% and CIVD in 32%. During their ICU stay, 19 women (86.4%) developed one or several organ failures associated to the hepatic failure and 18 women required blood transfusion. After an average stay of 7.5 days, evolution was marked by the death of seven patients (31.8%). Factors correlated with a poor prognosis were: the delay of medical consultation, the development of jaundice, the development of encephalopathy, respiratory or a circulatory failure. DISCUSSION AND CONCLUSION: AFLP is a rare but life-threatening complication. Furthermore AFLP shares features with other more common and less perilous illnesses. An early diagnosis and appropriate therapy of this pathology should improve the poor prognosis in our country.

[Isolated synovial chondromatosis of the subacromial bursa: report of a new case and review of the literature]. / La chondromatose synoviale isolée de la bourse sous acromiodeltoïdienne: un nouveau cas et revue de la littérature.

Synovial chondromatosis is a rare metaplasia of the synovium of unknown etiology that may involve occasionally the subacromial bursa. We report a new case diagnosed by ultrasound in a 30-year-old man and we present pathogenetic, diagnostic and therapeutic features of this disease with a literature review.

[Study of cardiovascular morbidity in nurses exposed to cytostatic drugs: Multivaried approach analysis]. / Étude de la morbidité cardiovasculaire chez les infirmiers exposés aux cytostatiques : approche par l'analyse multivariée.

OBJECTIVE: To investigate the relationship between cardiovascular morbidity and exposure to cytostatic drugs. MATERIALS AND METHODS: A descriptive analytical study was conducted with 74 nurses exposed to cytostatic drugs in oncology and 215 unexposed. A medical questionnaire was applied. Exposure to cytostatic drugs was estimated by the exposure time and the index of cytostatic contact (ICC). The statistical tests used are: relative risk, odds ratio, multivariate analysis: descriptive (ACM) and predictive (AIC system). RESULTS: It is a young population; the average age is 42±9.9years with a female predominance (81%). The average length was 18.4±11.11years. The average of the ICC ranged from 0.60 to 12.6 with a highly significant difference. For morbidity, there was no difference for most cardiovascular disease (RR, 1.03; 95% CI [0.59; 1.82]) outside of hypertension and venous thrombosis. ACM objectified separation between the terms and the comments of the two groups for HTA. The interpretation of results at alpha=0.05 showed an association with cardiovascular disease. The study of the association between cardiovascular morbidity and exposure to cytostatic objectified association with seniority and the ICC with a statistically significant difference (P=0.01). CONCLUSION: Multivariate analysis helped to eliminate confounding factors and retain the ICC and length of exposure to cytostatic in the onset of cardiovascular morbidity.

[Celiac disease, cerebral venous thrombosis and protein S deficiency, a fortuitous association?]. / Maladie coeliaque, thrombose veineuse cérébrale et déficit en protéine S, une association fortuite?

INTRODUCTION: Celiac disease is a pathology which is rarely associated with thrombosis complications. Cerebral vascular thrombosis has never been described in patients with a celiac disease. OBSERVATION: We report an observation of a 21-year-old girl with a history of celiac disease who was hospitalized in the intensive care unit for convulsive status epilepticus secondary to a cerebral venous thrombosis. The etiologic investigation of this thrombo-embolic complication revealed protein S deficit. Our patient improved under symptomatic treatment. COMMENT: This original observation confirms that celiac disease can be associated with cerebral venous thrombosis.

[Primary pulmonary angiosarcoma]. / Angiosarcome pulmonaire primitif.

Pulmonary angiosarcoma is an uncommon vascular and usually secondary tumor. Only a few primary cases of pulmonary angiosarcoma have been described. We report a case of primary pulmonary angiosarcoma in an adult man who presented with hemoptysis. Chest x-ray film and chest CT showed a right para-cardiac opacity associated with diffuse alveolar consolidation of the right basal lobe. Right inferior lobectomy has been performed. Pulmonary angiosarcoma was diagnosed on histological and immuno-histochemical studies of the operative specimen. The primitive character was retained after ruling out all other tumor localizations. The clinical outcome was rapidly fatal. This observation is added to the other rare cases published of primitive pulmonary angiosarcoma. It confirms the poor prognosis and the extremely hemorrhagic nature of this tumor. Isolated necrotic parenchymatous mass was an original radiological pattern observed in this patient.

Respiratory system reactance as an indicator of the intrathoracic airway response to methacholine in children.

The upper airways may contribute to increases in airway resistance in response to a bronchial challenge, and thus decrease the specificity of such challenge tests to diagnose airway hyperresponsiveness when forced oscillation techniques are used to evaluate changes in respiratory system resistance (Rrs). A concomitent decrease in respiratory system reactance (Xrs) may indicate a change in the intrathoracic airways and/or lung parenchyma, provided that extrathoracic airway wall motion is prevented. To test the value of Xrs in the evaluation of bronchial hyperresponsiveness, we studied the respiratory impedance response to methacholine in 38 children with a history of asthma (aged 6-14.5 years), and compared the results to changes in the forced expiratory volume in one second (FEV1). Rrs and Xrs were measured by the forced oscillation technique with pseudorandom (11 subjects) or sinusoidal (27 subjects) pressure variations applied around the child's head to minimize upper airway wall motion. Changes in Rrs and in Xrs at 12 Hz (Rrs12, Xrs 12) correlated significantly with changes in FEV1 (P < 0.005). A decrease in FEV1 > or = 20% was observed in 23 subjects. When these 23 subjects were compared with the 15 children who did not show significant changes in FEV1, the responding group had larger mean +/- SEM changes in Rrs (116.0 +/- 13.2% vs 60.4 +/- 11.4%, P < 0.006) and in Xrs (-2.1 +/- 0.4 hPa.s/L vs -0.9 +/- 0.3 hPa.s/L, P < 0.03) than the nonresponders. The receiver operating characteristics (ROC) curve analysis was used to assess the diagnostic value, i.e., specificity and sensitivity, of different levels of change in Rrs and Xrs, with reference to FEV1. The overall incidence of false results was similar for Rrs and Xrs. The optimum diagnostic value for Rrs was a 70% increase, which corresponded to a sensitivity of 87% and a specificity of 67%. For Xrs the optimum decision level was -1 hPa.s/L, corresponding to a sensitivity of 70% and a specificity of 80%. It is concluded that Xrs may improve the specificity of the forced oscillation technique in interpreting the airway response to methacholine. This may be of particular interest in young children unable to perform forced expirations.

Role of the endothelium and blood stasis in the development of varicose veins.

Today, chronic venous insufficiency affects millions of people but the investigation of veins and of venous diseases is still very poor. Additionally, the mechanism of the occurrence of varicose veins is not understood. Blood stasis is often associated with these pathological situations and we propose that resulting ischemic conditions can trigger the endothelium to release inflammatory mediators and growth factors. On one hand, the inflammatory mediators will recruit and activate neutrophils, which then infiltrate the venous wall and damage components of the extracellular matrix. On the other hand, growth factors induce smooth muscle cell migration, proliferation and de-differentiation into the synthetic phenotype, all together leading to the formation of neointima. These processes, being repeated over time, would eventually lead to alterations of the venous wall as observed in varicose veins. Venotropic drugs are used to treat chronic venous insufficiency. They are able to increase venous tone and to decrease vein and capillary permeability but they are also able to protect the endothelial cells against ischemia. Indeed, they target complexes of the mitochondrial respiratory chain and maintain ATP production during hypoxia. Hence, the cells are resistant to ischemia and do not release inflammatory mediators and growth factors. These drugs should thus be able to prevent the alterations of the venous wall induced by blood stasis.

Role of the endothelium and blood stasis in the appearance of varicose veins.

Today, chronic venous insufficiency affects millions of people but the investigation of veins and of venous diseases is still very poor. Additionally, the mechanism of the occurrence of varicose veins is not understood. Blood stasis is often associated with these pathological situations and we propose that resulting ischemic conditions can trigger the endothelium to release inflammatory mediators and growth factors. On one hand, the inflammatory mediators will recruit and activate neutrophils, which then infiltrate the venous wall and damage components of the extracellular matrix. On the other hand, growth factors induce smooth muscle cell migration, proliferation and de-differentiation into the synthetic phenotype, all together leading to the formation of neointima. These processes, being repeated over time, would eventually lead to alterations of the venous wall as observed in varicose veins. Venotropic drugs are used to treat chronic venous insufficiency. They are able to increase venous tone and to decrease vein and capillary permeability but they are also able to protect the endothelial cells against ischemia. Indeed, they target complexes of the mitochondrial respiratory chain and maintain ATP production during hypoxia. Hence, the cells are resistant to ischemia and do not release inflammatory mediators and growth factors. These drugs should thus be able to prevent the alterations of the venous wall induced by blood stasis.

Perfused human saphenous veins for the study of the origin of varicose veins: role of the endothelium and of hypoxia.

If venous stasis due to blood stagnation has been recognized to be involved in the development of varicose veins, the mechanism linking this situation to the modifications of the venous wall observed in varicoses is still unclear. In order to study this mechanism, human saphenous veins were incubated in normoxic or hypoxic conditions and the interactions between the endothelium and neutrophils were investigated. We observed that many neutrophils adhered to the endothelium of veins incubated in hypoxic conditions rather than in normoxia and that these adherent neutrophils were activated: they released high amounts of superoxide anion and of leukotriene B4. Studies in scanning electron microscopy confirmed the increased neutrophil adherence to the endothelium as well as their activation. These results were then related to the histological observation of varicose veins. These veins show a thickening of the media with extracellular matrix deposit as well as an alteration of the elastic lamina with the presence of smooth muscle cells in the intima. These results are in agreement with in vitro studies on isolated endothelial cells. They all show that hypoxia is able to activate endothelial cells: they release inflammatory mediators and become adhesive for neutrophils which are then activated. These activated leukocytes release free radicals and proteases which are able to degrade the extracellular matrix. In addition, hypoxia-activated endothelial cells secrete growth factors which will trigger smooth muscle cell proliferation and the synthesis of extracellular matrix components. Altogether and because they are frequently repeated, these processes could eventually lead to alterations of the venous wall similar to those observed in varicose veins.

Effect of Ruscus extract and hesperidin methylchalcone on hypoxia-induced activation of endothelial cells.

BACKGROUND: Ruscus aculeatus extract and the flavonoid hesperidin methylchalcone (HMC) are drugs used in the treatment of chronic venous insufficiency. METHODS: In the present study, we investigated their effects on the activation of endothelial cells by hypoxia, a condition which mimics venous blood stasis. RESULTS: We observed that Ruscus extract was able to inhibit the activation of endothelial cells by hypoxia: the decrease in ATP content, the activation of phospholipase A2 as well as the subsequent increase in neutrophil adherence with a maximal protection obtained at 50 microg/ml. HMC was also able to inhibit the hypoxia-induced decrease in ATP content. Furthermore, the effects of Ruscus extract and of HMC on this decrease seem to be additive. CONCLUSIONS: The biochemical mechanism evidenced in this work might explain some of the beneficial therapeutic effects of these products in the treatment of chronic venous insufficiency patients.

[Determination of anti-transglutaminase antibodies in the diagnosis of coeliac disease in children: results of a five year prospective study]. / Dosage des anticorps anti-transglutaminase dans le diagnostic de la maladie coeliaque de l'enfant: résultats d'une étude prospective sur cinq ans.

AIM: To evaluate the interest of IgA antibodies to tissue transglutaminase in the diagnosis of children coeliac disease compared with anti-endomysium and anti-gliadin antibodies. SUBJECTS AND METHODS: Seventy children with coeliac disease (mean age: 5 years and 8 months) and 99 disease controls (mean age: 4 years and 5 months). IgA anti-transglutaminase were tested by ELISA using a human recombinant tissue transglutaminase. IgA anti-endomysium were detected by indirect immunofluorescence on monkey oesophagus. RESULTS: The middle rate of IgA anti-transglutaminase was 101.06 units in patients and only 0.47 unit in controls. IgA anti-transglutaminase and IgA anti-endomysium were in agreement in 98.8% of cases; only two cases were discordant (+/- and -/+). Globally, the two markers had the same sensitivity (90%), specificity (98%), negative (93.2%) and positive (96.9%) predictive values. For anti-gliadin antibodies, the IgG were more sensitive (88.6%) and the IgA more specific (93.9%). CONCLUSION: IgA anti-tissue transglutaminase can be used instead of IgA anti-endomysium as a serological marker of screening and diagnosis of coeliac disease in children after 3 years.

[Carotid body tumors: contribution of the various imaging techniques. A report of six cases]. / Tumeurs du corpuscule carotidien: apport des différentes techniques d'imagerie. A propos de six observations.

INTRODUCTION: Carotid body tumors are rare hypervascular lesions arising from neural crest paraganglion cells. MATERIALS AND METHODS: We retrospectively analyzed the imaging features of six patients with carotid body tumors to precise their imaging characteristics in order to find specific signs and elaborate a strategy for diagnosis. RESULTS: Imaging features detect the vascular nature of these tumors of the carotid space causing characteristic widening of the carotid bifurcation. Two patients had multiple (two or more) localizations and one had a family history of glomus tumor. CONCLUSION: MRI with MRA is considered as the gold standard imaging technique for the evaluation of glomus tumors as it allows a multiplanar approach which is important in the preoperative study.

[Pseudotumoral eyelid inflammation. Apropos of an anatomo-clinical case]. / Inflammation palpébrale pseudotumorale. A propos d'une observation anatomo-clinique.

A clinicopathological case of a 76-year-old male patient with a chronic inflammatory change of the inferior left eyelid is reported. The inflammation appeared as a reddish area of the inner part of the eyelid, without sharp limits, but with loss of lashes. Numerous local treatments did not to cure this condition. As some true eyelid tumors may mimic an inflammation during growth and, for example, sebaceous carcinoma may clinically present as chronic unilateral blepharitis, a surgical excisional biopsy was performed on this left eyelid. Its histopathological study showed a granulomatous inflammation, which was typical of a simple chalazion. This case clearly illustrates that the chalazion may not always appear as a limited nodular inflammation of the eyelid, but may have a more diffuse clinical presentation.

[Epidemiology of severe asthma]. / Épidémiologie de l'asthme grave.

Prevalence of severe acute asthma can be evaluated through mortality statistics, hospitalization rates and epidemiological studies. Risk factors include personnal characteristics (age, socio-economic status, disease denial, depression, medical features associated with increased severity), environment factors as life events and exposure to specific (allergens) and as non-specific factors (viral or microbial infections, passive smoking, air polluants) and the health care system (care delivery, quality of medical management). Such factors are inter-related. Their knowledge should allow a better management leading to a lower incidence of severe acute asthma attacks.

[Evaluation of a questionnaire on the home environment of asthmatic patients]. / Evaluation d'un questionnaire sur l'environnement domestique des asthmatiques.

Indoor environment outpatient asthmatics plays a major role in modulating asthma severity. Therefore many surveys devoted to studying risk factors for asthma include a questionnaire about housing characteristics. However no such questionnaire has been to date validated. In this study, we tried to validate such a questionnaire by comparing answers of a group of asthmatics outpatient to a questionnaire to de visu finding performed by a field worker during a home visit. Thirty asthmatic patients were included in the study. Housing characteristics were generally correctly acknowledged. Questions on the heating system should be clearly and simply formulated. Energetic supply of stoves and ventilation system is not always known. In the field of house equipment, especially bedding, photographs or drawings can be useful.

Polyunsaturated fatty acids deficits are associated with psychotic state and negative symptoms in patients with schizophrenia.

The study was aimed to examine membrane polyunsaturated fatty acids (PUFAs) profile in patients with schizophrenia (SZ) before and after antipsychotic medication and test their association with psychopathology. Erythrocyte membrane fatty acids were analysed by gas chromatography in 36 drug-free patients with SZ and 36 controls. Psychometric evaluation and blood sampling were achieved at baseline and after 3 months of antipsychotic treatment. At enrolment, levels of total PUFAs and arachidonic (AA) and docosahexaenoic (DHA) acids were significantly lower, but ω6/ω3 PUFAs ratio was higher in patients. AA and DHA were negatively related to the Andreason's scale for assessment of negative symptoms (SANS) score. DHA was inversely related to "alogia", "anhedonia", "avolition", and "blunted affect" subitems of SANS. After 3 months under typical antipsychotic drugs, fatty acid profile turned into comparable to controls in parallel with psychopathology improvement. Data indicate that PUFAs deficits are associated with psychotic state and negative symptoms of SZ.