[Chronic pheochromocytoma-induced myocardial injuries: What should we expect from modern and classical echocardiographic tools?] / Apport du 2D strain et des outils échocardiographiques classiques pour la recherche d'anomalies myocardiques induites par l'exposition chronique à un phéochromocytome.

INTRODUCTION: Pheochromocytoma is a rare disease, which may manifest as severe cardiac complications. Apart from these situations, the "chronic" cardiac impact is not clearly defined. A cardiac MRI study suggests that these patients are carrying areas of fibrosis and foci of left ventricular myocarditis. Since these abnormalities are usually associated with altered left ventricular longitudinal systolic strain, we hypothesize that this strain is altered in patients with a "chronic" pheochromocytoma. METHOD: This retrospective case-control study was performed using patients from the Bordeaux University Hospital database, included between 2008 and 2016. We compared the left ventricular global longitudinal strain (GLS), radial and circumferential systolic strain and classic echocardiographic parameters between patients with pheochromocytoma and controls matched for age, sex, body mass index and systolic blood pressure. RESULTS: The analysis included 47 patients and 47 correctly matched controls. There were no statistically significant differences between the 2 groups in terms of GLS (-20.7±2.4% vs. -20.2±2.7%, P=0.40), radial strain, left ventricular mass or diastolic function. Left ventricular ejection fraction and circumferential strain were significantly higher in patients than in controls, with a significantly lower telediastolic diameter. CONCLUSION: No significant changes in GLS were observed in our pheochromocytoma patients, compared with controls. Several hypotheses may explain these results. The presence of fibrosis foci and areas of left ventricular myocarditis being associated with a poor cardiological prognosis, a systematic cardiac MRI could be discussed in these patients, until further studies are performed.

Diagnostic accuracy of computed tomography to identify adenomas among adrenal incidentalomas in an endocrinological population.

CONTEXT: The recent recommendations of the European Endocrine Society states that the performance of computed tomography (CT) to characterize 'true' adrenal incidentalomas (AIs) remains debatable. OBJECTIVE: To determine relevant thresholds for usual CT parameters for the diagnosis of benign tumors using robust reference standard among a large series of 'true' AIs recruited in an endocrinological setting. DESIGN: Retrospective study of 253 AIs in 233 consecutive patients explored in a single university hospital: 183 adenomas, 33 pheochromocytomas, 23 adrenocortical carcinomas, 5 other malignant tumors and 9 other benign tumors. Reference standard was histopathology in 118 AIs, biological diagnosis of pheochromocytoma in 2 AIs and size stability after at least 1 year of follow-up in 133 AIs. METHODS: Sensitivity, specificity and positive and negative predictive values were estimated for various thresholds of size, unenhanced attenuation (UA), relative and absolute wash-out (RPW, APW) of contrast media. 197 scans were reviewed independently in a blinded fashion by two expert radiologists to assess inter-observer reproducibility of measurements. RESULTS: Criteria associated with a 100% positive predictive value for the diagnosis of benign AI were: a combination of size and UA: 30 mm and 20 HU or 40 mm and 15 HU, respectively; RPW >53%; and APW >78%. Non-adenomatous AIs with rapid contrast wash-out were exclusively benign pseudocysts and pheochromocytomas, suggesting that classical thresholds of 60% and 40% for APW and RPW, respectively, can be safely used for patients with normal metanephrine values. Inter-observer reproducibility of all parameters was excellent (intra-class correlation coefficients: 0.96-0.99). CONCLUSIONS: Our study, the largest conducted in AIs recruited in an endocrinological setting, suggests safe thresholds for quantitative CT parameters to avoid false diagnoses of benignity.

La chirurgie d'épargne surrénalienne : du cortex à la médulla: Cortical sparing surgery: from cortex to medulla.

The 2017 Endocrine Society annual meeting included several communications and debates on the conservative adrenal surgery in bilateral hereditary pheochromocytomas (BHP), bilateral adrenal macronodular hyperplasia (BAMH) and primary hyperaldosteronism (PHA). The general principle is to preserve a part of the adrenal cortex to prevent the occurrence of a definitive adrenal insufficiency. In BHP, cortical sparing surgery allows more than 50% of patients to maintain normal corticotropic function at 10 years with a low recurrence rate (~ 10%). Since the adrenal medulla cannot be removed entirely, recurrence seems inevitable and long-term follow-up is essential. Individual risk of malignancy must be taken into account. In BAMH responsible for Cushing syndrome, unilateral adrenalectomy induces a normalization of urinary free cortisol in 92 to 100% of cases and even corticotropic insufficiency in 40 to 100% of cases. This is most often transient. Late recurrences of Cushing's syndrome may occur in 13 to 60% of cases. Prolonged patient monitoring is therefore essential. In PAH with lateralized aldosterone production, minimally invasive partial adrenal surgery, which consists of removing only the adrenal adenoma visualized at TDM, allows an improvement blood pressure in about 94% of patients. However, failure or recurrence may occur. Its place therefore remains marginal in the treatment of the lateralized PAHs.

Cabergoline for Cushing's disease: a large retrospective multicenter study.

OBJECTIVE: The efficacy of cabergoline in Cushing's disease (CD) is controversial. The aim of this study was to assess the efficacy and tolerability of cabergoline in a large contemporary cohort of patients with CD. DESIGN: We conducted a retrospective multicenter study from thirteen French and Belgian university hospitals. METHODS: Sixty-two patients with CD received cabergoline monotherapy or add-on therapy. Symptom score, biological markers of hypercortisolism and adverse effects were recorded. RESULTS: Twenty-one (40%) of 53 patients who received cabergoline monotherapy had normal urinary free cortisol (UFC) values within 12 months (complete responders), and five of these patients developed corticotropic insufficiency. The fall in UFC was associated with significant reductions in midnight cortisol and plasma ACTH, and with clinical improvement. Compared to other patients, complete responders had similar median baseline UFC (2.0 vs 2.5xULN) and plasma prolactin concentrations but received lower doses of cabergoline (1.5 vs 3.5 mg/week, P < 0.05). During long-term treatment (>12 months), cabergoline was withdrawn in 28% of complete responders because of treatment escape or intolerance. Overall, sustained control of hypercortisolism was obtained in 23% of patients for 32.5 months (19-105). Nine patients on steroidogenesis inhibitors received cabergoline add-on therapy for 19 months (1-240). Hypercortisolism was controlled in 56% of these patients during the first year of treatment with cabergoline at 1.0 mg/week (0.5-3.5). CONCLUSIONS: About 20-25% of CD patients are good responders to cabergoline therapy allowing long-term control of hypercortisolism at relatively low dosages and with acceptable tolerability. No single parameter, including the baseline UFC and prolactin levels, predicted the response to cabergoline.

[Cystic adenomatoid malformation of the lung revealed in a newborn infant by an image of a lung abscess]. / Malformation kystique adénomatoïde du poumon révélée chez un nouveau-né par une image d'abcès pulmonaire.

BACKGROUND: Cystic adenomatoid malformation, a rare pulmonary malformation, usually appears as a cystic mass, radiologically. It may be infected and confusion has also arisen in distinguishing it from pneumonia with pneumatoceles. CASE REPORTS: A full-term boy suffered from severe neonatal respiratory distress. Pregnancy had been uneventful despite the fact that his mother had insulin-dependent diabetes. Prenatal ultrasonographies did not reveal any abnormality. On day 2, X-rays showed a right pulmonary mass that appeared solid. The patient was treated for E Coli sepsis. Subsequently, the pulmonary mass became lacent, cystic, fluid-filled, resembling an abscess; the CT scan confirmed these features. As the lesion increased in volume, a limited resection was performed. Histologic examination showed adenomatoid proliferation of bronchiolar elements with formation of cysts and necrosis. CONCLUSION: Infection of cystic adenomatoid malformation may supervene the first days of life resulting in a lung abscess appearance.

Inhaled terbutaline administered via a spacer fully prevents exercise-induced asthma in young asthmatic subjects: a double-blind, randomized, placebo-controlled study.

The effects of inhaled terbutaline, a beta 2-adrenergic agonist, administered via a 750-ml spacer device were studied in young asthmatic subjects with exercise-induced asthma. A double-blind, randomized, placebo-controlled study of the effects of inhaled 0.5 mg terbutaline and placebo was conducted in 10 asthmatic children (age range 6-16 years) with documented exercise-induced asthma. Forced expiratory volume in 1 s (FEV1) was measured at baseline, 15 min after inhaling terbutaline or placebo, and at intervals up to 60 min after exercising. Subjects exercised using a cycle ergometer for 5 min at a submaximal, constant work-load while breathing dry air at room temperature. Terbutaline induced bronchodilation at rest in all subject and fully prevented exercise-induced asthma in nine out of the 10 subjects; the exercise-induced fall in FEV1 was markedly reduced in the remaining subject. It is concluded that exercise-induced asthma can be inhibited by pretreatment with inhaled terbutaline, administered via a spacer, in a majority of young asthmatics.

[Bronchial response to acetylcholine and Dermatophagoides pteronissimus in asymptomatic asthmatics]. / Réponse bronchique a l'acétylcholine et au Dermatophagoides pteronissimus chez les asthmatiques asymptomatiques.

Bronchial reactivity to increasing concentrations of acetylcholine (from 0.01 to 10 mg X ml-1) was studied in 84 asthmatic patients during a remission period. Three groups were identified based on decreasing reactivity : group 1 : 57 patients (68%) who exhibited a decrease equal or of more than 20% of forced expiratory volume per second (FEV1) and a decrease equal or of more than 35% of specific airway conductance (sGaw); group 2: 16 patients (19%) with a decrease in FEV1 of less than 20%, but a decrease in sGaw still equal or superior to 35%; group 3 : 11 patients (13%) with a less than 20% decrease in FEV1 and a less than 35% decrease of sGaw. Basal measurements of FEV1 and sGaw gave significantly lower values in group 1 than in group 2 and in group 2 than in group 3. A strong positive relationship was found between initial FEV1 (% of predicted) and acetylcholine concentration giving a fall of 20% in FEV1 (PD20-FEV1) (r = 0.87; p less than 0.001); a weak but still statistically significant relationship existed between initial sGaw and PD35-sGaw (r = 0.38; p less than 0.01). Twenty-five patients with allergic asthma were submitted to an inhalation test with Dermatophagoides pteronissimus (Dpt). Bronchial reactivity to Dpt was significantly correlated with acetylcholine (r = 0.9; p less than 0.001). Twenty-four hours after Dpt exposure, seven patients (28%) still presented bronchial obstruction. Eleven patients without persistent obstruction 24 h after Dpt exposure were resubmitted to acetylcholine; bronchial reactivity was found increased when compared to the initial acetylcholine-induced bronchospasm.(ABSTRACT TRUNCATED AT 250 WORDS)