L' Hypothyroïdie constitue un facteur de risque surajouté d'athérosclérose carotidienne chez les patients hypercholestérolémiques

L'EXISTENCE D'UNE RELATION CAUSALE ENTRE L'HYPOTHYROÏDIE, NOTAMMENT DANS SA FORME FRUSTE, ET LA PATHOLOGIE CARDIOVASCULAIRE ISCHEMIQUE EST ENCORE DEBATTUE A CE JOUR. CETTE ETUDE A POUR BUT D'EVALUER S'IL EXISTE UNE CORRELATION ENTRE L'HYPOTHYROÏDIE ET L'ATHEROSCLEROSE CAROTIDIENNE. AU SEIN D'UNE POPULATION CONSULTANT POUR DYSLIPIDEMIE, 794 PATIENTS AYANT UNE TSH SUPERIEURE A 4 MUI/L ONT ETE SELECTIONNES, ET ONT ETE APPARIES A 1588 SUJETS EN EUTHYROÏDIE, SUR LES PRINCIPAUX FACTEURS DE RISQUE CARDIOVASCULAIRES (AGE, SEXE, BILAN LIPIDIQUE, HYPERTENSION ARTERIELLE, DIABETE, OBESITE ET TABAGISME ACTIF). PLUS DE 90 % DES PATIENTS HYPOTHYROÏDIENS AVAIENT UNE TSH INFERIEURE A 10 MUI/L. L'EVALUATION DE L'ATHEROSCLEROSE CAROTIDIENNE A MONTRE QUE LES PATIENTS DONT LE TAUX DE TSH ETAIT SUPERIEUR A 10 MUI/L AVAIENT UNE ELEVATION SIGNIFICATIVE DE LEUR EPAISSEUR INTIMA-MEDIA CAROTIDIENNE, PAR RAPPORT AUX PATIENTS DONT LA TSH ETAIT COMPRISE ENTRE 4 ET 10 MUI/L. PAR CONTRE, L'ENSEMBLE DES PATIENTS EN HYPOTHYROÏDIE AVAIT DES PLAQUES CAROTIDIENNES ET UNE EPAISSEUR INTIMA-MEDIA COMPARABLES A CELLES LES PATIENTS TEMOINS. CETTE ETUDE A DONC ETABLI QUE L'HYPOTHYROÏDIE EST ASSOCIEE A UNE MAJORATION DE L'EPAISSEUR INTIMA-MEDIA CAROTIDIENNE CHEZ LES PATIENTS DYSLIPIDEMIQUES DONT LE TAUX DE TSH EST SUPERIEUR A 10 MUI/L. L'HYPOTHYROÏDIE APPARAIT DONC CONSTITUER, CHEZ LES PATIENTS DYSLIPIDEMIQUES, UN FACTEUR DE RISQUE CARDIOVASCULAIRE SURAJOUTEPARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Etude du retentissement osseux dans un modèle de résistance partielle aux androgènes (la maladie de Kennedy)

La maladie de Kennedy est une maladie génétique rare récessive liée à l X, due à une mutation du gène du récepteur aux androgènes. Elle entraine des troubles neurologiques secondaires à l accumulation de ce récepteur dans les motoneurones périphériques et des troubles endocriniens secondaires à la perte de fonction partielle du récepteur. Nous avons voulu évaluer si ces patients ayant un hypogonadisme secondaire à une résistance partielle aux androgènes et souffrant de sédentarité avaient une ostéoporose. Notre étude a été réalisée chez dix patients présentant une maladie de Kennedy confirmée génétiquement. Ces patients, âgés en moyenne de 62,6ans, présentaient une élévation des taux moyens d estrogènes, estradiol à 43pg/mL (+/-14) et estrone à 68pg/mL (+/-21), et des taux moyens normaux de testostérone, testostérone totale à 5,7ng/mL (+/-2,3) et testostérone biodisponible à 2ng/mL (+/-0,6). Neuf patients sur dix avaient une réponse explosive au test à la GnRH. Aucun patient n a d ostéoporose ou d élévation des marqueurs osseux, les moyennes des Tscores au niveau du rachis, du fémur et du col fémoral sont normales, respectivement +0,71DS (+/-1,75), -0,25DS (+/-1,12) et -0,38DS (+/-1,33). Lorsqu on a comparé ces résultats à une population témoin hypogonadique hypogonadotrope (n=9), on retrouve une différence significative du Tscore au niveau du rachis (p=0,01). Ce résultat est important chez ces patients qui sont du fait de leur maladie neurologique, potentiellement à risque de chute. Aucune étude n avait jusqu à présent évalué l atteinte osseuse dans ce modèle de résistance partielle aux androgènes, dans lequel l élévation des estrogènes pourrait avoir un rôle protecteur.PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocSudocFranceF

Modalités de prise en charge d'une gynécomastie en pratique médicale courante

PARIS-BIUM (751062103) / SudocCentre Technique Livre Ens. Sup. (774682301) / SudocSudocFranceF

Transient Visual Loss induced by Pregnancy

During pregnancy, because of estrogen stimulation, Pituitary gland almost always enlarge and can sometimes exceeding 100 % and return to normal within six month after post partum. It usually does not expand to the extrasellar area to cause symptoms such as headache or vision impairement. In some rares cases the mass effect threaten the vision

Patients with the metabolic syndrome and a disturbed cortisol balance display more microalbuminuria

The objective of this study was to investigate whether patients with the metabolic syndrome (MetS) and an imbalance in cortisol metabolism express increased urinary albumin excretion compared to those patients with metabolic syndrome alone. Seventy-four patients with MetS were evaluated using a low-dose dexamethasone suppression test (LDDST) to identify disturbed cortisol balance (cortisol levels > 50 nmol/L after LDDST). The level of albumin in the urine was also evaluated. Disturbed cortisol balance was found in 8% of all evaluated patients with MetS. Microalbuminuria was present significantly more often (p <0.01) in those patients with MetS and an imbalance in cortisol metabolism compared with patients suffering MetS alone (urine albumin: 210 mg/L vs. 26 mg/L, respectively, p <0.01). A substantial percentage of patients with MetS had inappropriate cortisol homeostasis. Of importance, excretion of urinary albumin was increased in these patients. This observation may indicate that this subgroup within the MetS population has a higher cardiovascular risk and possible increased endothelial dysfunction, with a subsequent need for stricter control to prevent cardiovascular morbidity and mortalit

Laparoscopic Revision of Bariatric Surgeries in Two Patients with Severe Resistant Hypocalcemia After Endocrine Cervical Surgery

International audienc

The French national protocol for Kennedy’s disease (SBMA): consensus diagnostic and management recommendations

International audienceAbstract Background Kennedy’s disease (KD), also known as spinal and bulbar muscular atrophy (SBMA), is a rare, adult-onset, X-linked recessive neuromuscular disease caused by CAG expansions in exon 1 of the androgen receptor gene (AR). The objective of the French national diagnostic and management protocol is to provide evidence-based best practice recommendations and outline an optimised care pathway for patients with KD, based on a systematic literature review and consensus multidisciplinary observations. Results The initial evaluation, confirmation of the diagnosis, and management should ideally take place in a tertiary referral centre for motor neuron diseases, and involve an experienced multidisciplinary team of neurologists, endocrinologists, cardiologists and allied healthcare professionals. The diagnosis should be suspected in an adult male presenting with slowly progressive lower motor neuron symptoms, typically affecting the lower limbs at onset. Bulbar involvement (dysarthria and dysphagia) is often a later manifestation of the disease. Gynecomastia is not a constant feature, but is suggestive of a suspected diagnosis, which is further supported by electromyography showing diffuse motor neuron involvement often with asymptomatic sensory changes. A suspected diagnosis is confirmed by genetic testing. The multidisciplinary assessment should ascertain extra-neurological involvement such as cardiac repolarisation abnormalities (Brugada syndrome), signs of androgen resistance, genitourinary abnormalities, endocrine and metabolic changes (glucose intolerance, hyperlipidemia). In the absence of effective disease modifying therapies, the mainstay of management is symptomatic support using rehabilitation strategies (physiotherapy and speech therapy). Nutritional evaluation by an expert dietician is essential, and enteral nutrition (gastrostomy) may be required. Respiratory management centres on the detection and treatment of bronchial obstructions, as well as screening for aspiration pneumonia (chest physiotherapy, drainage, positioning, breath stacking, mechanical insufflation-exsufflation, cough assist machnie, antibiotics). Non-invasive mechanical ventilation is seldom needed. Symptomatic pharmaceutical therapy includes pain management, endocrine and metabolic interventions. There is no evidence for androgen substitution therapy. Conclusion The French national Kennedy’s disease protocol provides management recommendations for patients with KD. In a low-incidence condition, sharing and integrating regional expertise, multidisciplinary experience and defining consensus best-practice recommendations is particularly important. Well-coordinated collaborative efforts will ultimately pave the way to the development of evidence-based international guidelines

The French national protocol for Kennedy’s disease (SBMA): consensus diagnostic and management recommendations

International audienceBackground:Kennedy’s disease (KD), also known as spinal and bulbar muscular atrophy (SBMA), is a rare, adult-onset, X-linked recessive neuromuscular disease caused by CAG expansions in exon 1 of the androgen receptor gene (AR). Theobjective of the French national diagnostic and management protocol is to provide evidence-based best practicerecommendations and outline an optimised care pathway for patients with KD, based on a systematic literature review andconsensus multidisciplinary observations.Results:The initial evaluation, confirmation of the diagnosis, and management should ideally take place in a tertiary referralcentre for motor neuron diseases, and involve an experienced multidisciplinary team of neurologists, endocrinologists,cardiologists and allied healthcare professionals. The diagnosis should be suspected in an adult male presenting with slowlyprogressive lower motor neuron symptoms, typically affecting the lower limbs at onset. Bulbar involvement (dysarthria anddysphagia) is often a later manifestation of the disease. Gynecomastia isnot a constant feature, but is suggestive of asuspected diagnosis, which is further supported by electromyography showing diffuse motor neuron involvement oftenwith asymptomatic sensory changes. A suspected diagnosis is confirmed by genetic testing. The multidisciplinary assessmentshould ascertain extra-neurological involvement such as cardiac repolarisation abnormalities (Brugada syndrome), signs ofandrogen resistance, genitourinary abnormalities, endocrine and metabolic changes (glucose intolerance, hyperlipidemia). Inthe absence of effective disease modifying therapies, the mainstay of management issymptomatic support usingrehabilitation strategies (physiotherapy and speech therapy). Nutritional evaluation by an expert dietician is essential, andenteral nutrition (gastrostomy) may berequired. Respiratory management centres on the detection and treatment ofbronchial obstructions, as well as screening for aspiration pneumonia (chest physiotherapy, drainage, positioning, breathstacking, mechanical insufflation-exsufflation, cough assistmachnie, antibiotics). Non-invasive mechanical ventilation isseldom needed. Symptomatic pharmaceutical therapy includes pain management, endocrine and metabolic interventions.There is no evidence for androgen substitution therapy