174 results match your criteria: "Centre de Reference des Maladies Endocriniennes Rares de la Croissance[Affiliation]"
PLoS One
August 2018
Service de Diabétologie et Endocrinologie Pédiatrique, Departement de Pédiatrie, et Centre de Reference des Maladies Endocriniennes Rares de la Thyroïde et de l'Hypophyse, Centre Hospitalier Universitaire d'Angers, Angers, France.
Objective: The objective was to determine whether maternal nutritional factors are associated with transient neonatal hyperinsulinism (HI).
Design And Setting: Case control study in 4 French tertiary Obstetrics and Neonatology Departments between 2008 and 2015.
Methods: Sixty-seven mothers of neonates diagnosed with transient hyperinsulinism and 113 mothers of controls were included.
Heart
November 2018
AP-HP, Pitié-Salpêtrière Hospital Department of Pharmacology CIC-1421 Pharmacovigilance Unit INSERM UMR ICAN 1166 Sorbonne Université UPMC, Univ Paris 06, Institute of CArdiometabolism and Nutrition (ICAN), Paris, France.
Objective: A prolonged QTc (LQT) is a surrogate for the risk of torsade de pointes (TdP). QTc interval duration is influenced by sex hormones: oestradiol prolongs and testosterone shortens QTc. Drugs used in the treatment of breast cancer have divergent effects on hormonal status.
View Article and Find Full Text PDFEndocr Connect
May 2018
Assistance Publique-Hôpitaux de Paris (AP-HP)Hôpital Universitaire Robert-Debré, Department of Pediatric Endocrinology and Diabetology, and Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Paris, France
Objective: There is a scarcity of data from randomised controlled trials on the association of growth hormone (GH) with gonadotrophin-releasing hormone agonists in idiopathic short stature (ISS), although this off-label use is common. We aimed to test whether delaying pubertal progression could increase near-adult height (NAH) in GH-treated patients with ISS.
Methods: Patients with ISS at puberty onset were randomised to GH with leuprorelin (combination, = 46) or GH alone ( = 45).
J Pediatr Endocrinol Metab
April 2018
Department of Paediatrics, 2nd Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic.
Background: We investigated time trends in age, gender, growth hormone (GH) dose and height standard deviation score (SDS) in children with GH deficiency (GHD), born small for gestational age (SGA) or with Turner syndrome (TS) starting GH treatment.
Methods: Data were obtained from children enrolled in the NordiNet® International Outcome Study (IOS) between 2006 and 2015 in the Czech Republic, France, Germany, Serbia and Montenegro (all indications), and Switzerland and the UK (GHD only). Trends were analyzed by linear regression.
Ann Biol Clin (Paris)
April 2018
Service des explorations fonctionnelles, Hôpital Necker-Enfants malades, Assistance Publique des Hôpitaux de Paris, Paris, France.
Ann Endocrinol (Paris)
February 2018
Service d'endocrinologie diabétologie pédiatrique, hôpital Robert-Debré, centre de référence des maladies endocriniennes rares de la croissance et du développement, université Paris Diderot, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75019 Paris, France.
The French endocrinology society (SFE) and the French pediatric endocrinology society (DFSDP) have drawn up recommendations for the management of primary and secondary adrenal insufficiency in the adult and child, based on an analysis of the literature by 19 experts in 6 work-groups. A diagnosis of adrenal insufficiency should be suspected in the presence of a number of non-specific symptoms except hyperpigmentation which is observed in primary adrenal insufficiency. Diagnosis rely on plasma cortisol and ACTH measurement at 8am and/or the cortisol increase after synacthen administration.
View Article and Find Full Text PDFJ Endocr Soc
March 2017
Context: Cabergoline (CAB) is very effective in the treatment of macroprolactinomas, but there are few data on the CAB dose necessary to achieve and maintain normal prolactin (PRL) levels.
Design And Patients: We retrospectively studied 260 patients. CAB was introduced at a mean dose of 0.
J Am Soc Echocardiogr
February 2018
Sorbonne Universités, UPMC Univ Paris 06, AP-HP, Pitié-Salpêtrière Hospital, Department of Cardiology, Echocardiography Unit, Paris, France; UMR ICAN 1166, Paris, France.
Background: Normal values of left ventricular ejection fraction (LVEF) and absolute values of global longitudinal strain (GLS) are lower in men than in women. Data concerning the association of sex hormone levels on these left ventricular systolic function surrogates are scarce. The aim of this study was to determine the association of sex hormones with systolic left ventricular function in healthy subjects and patients with congenital adrenal hyperplasia (CAH) as a model of testosterone dysregulation.
View Article and Find Full Text PDFAnn Endocrinol (Paris)
December 2017
Service des explorations fonctionnelles, hôpital Necker-Enfants malades, Assistance publique des hôpitaux de Paris (AP-HP), 149, rue de Sèvres, 75015 Paris, France.
Ann Endocrinol (Paris)
December 2017
Service d'endocrinologie diabétologie pédiatrique, hôpital Robert-Debré, université Paris-Diderot, centre de référence des maladies endocriniennes rares de la croissance et du développement, Assistance publique-hôpitaux de Paris, 48, boulevard Sérurier, 75019 Paris, France.
Patients with adrenal insufficiency require regular, specialised monitoring in order to optimise their replacement therapy, to detect signs of under- and over-dosage, and to examine for possible associated disorders (auto-immune disorders in the case of auto-immune primary adrenal insufficiency either isolated or as part of auto-immune polyendocrinopathy syndrome type 1; illnesses with underlying monogenic causes). The transition period between adolescence and adulthood represents an added risk of a breakdown in monitoring which requires particular attention from medical teams and coordination between adult and pediatric medical teams. It is essential to encourage patient autonomy in the management of their illness, notably their participation in treatment education programs, in particular programs that target avoidance of, or early treatment of acute adrenal insufficiency.
View Article and Find Full Text PDFBr J Dermatol
October 2017
Département de Dermatologie, CHU de Montpellier, Université de Montpellier, 191, avenue du Doyen-Gaston-Giraud, 34295, Montpellier, France.
J Clin Endocrinol Metab
December 2017
Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, Service d'Endocrinologie Diabétologie Pédiatrique, Centre de Référence des Maladies Endocriniennes Rares de la Croissance, France.
Context: Growth hormone (GH) therapy may improve statural growth outcomes in patients with severe juvenile idiopathic arthritis (JIA).
Objectives: To evaluate the effect of GH treatment on adult height and to identify determinants of growth outcomes in JIA.
Design And Patients: Data from 58 patients with JIA, including 53 receiving GH, enrolled in three prospective clinical trials between 1997 and 2002 were analyzed.
Eur J Pediatr
February 2018
Department of Pediatric Endocrinology and Diabetology, and Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Universitaire Robert-Debré, F-75019, Paris, France.
Unlabelled: Clinical precocious puberty (PP) is a disease, reputed to be on the increase and suspected to be linked to endocrine disrupting chemicals (EDC) exposure. Population-based epidemiological data are lacking in France and scarce elsewhere. We accessed the feasibility of monitoring PP nationwide in France in this context, using a nationwide existing database, the French National Health Insurance Information System.
View Article and Find Full Text PDFEur J Endocrinol
November 2017
Department of Pediatric Endocrinology, University Children's Hospital, Saarland University Medical Center, Homburg, Germany.
Objective: To investigate the effect of age at growth hormone (GH) treatment start on near adult height (NAH) in children with isolated GH deficiency (GHD).
Design: NordiNet® International Outcome Study (IOS) (Nbib960128), a non-interventional, multicentre study, evaluates the long-term effectiveness and safety of Norditropin® (somatropin) (Novo Nordisk A/S) in the real-life clinical setting.
Methods: Patients ( = 172) treated to NAH (height at ≥18 years, or height velocity <2 cm/year at ≥16 (boys) or ≥15 (girls) years) were grouped by age (years) at treatment start (early (girls, <8; boys, <9), intermediate (girls, 8-10; boys, 9-11) or late (girls, >10; boys, >11)) and GHD severity (<3 ng/mL or 3 to ≤10 ng/mL).
Eur J Endocrinol
September 2017
Assistance Publique-Hôpitaux de Paris, Hôpital Universitaire Robert Debré, Service d'Endocrinologie Diabétologie Pédiatrique, Centre de Référence des Maladies Endocriniennes Rares de la Croissance et du développement, Paris, France.
Context: Regular monitoring of serum IGF-I levels during growth hormone (GH) therapy has been recommended, for assessing treatment compliance and safety.
Objective: To investigate serum IGF-I SDS levels during GH treatment in children with GH deficiency, and to identify potential determinants of these levels.
Design, Patients And Methods: This observational cohort study included all patients ( = 308) with childhood-onset non-acquired or acquired GH deficiency (GHD) included in the database of a single academic pediatric care center over a period of 10 years for whom at least one serum IGF-I SDS determination during GH treatment was available.
Eur J Endocrinol
September 2017
William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London, UK.
Ann Endocrinol (Paris)
July 2017
IE3M, endocrinologie et médecine de la reproduction, centre de référence des maladies endocriniennes rares de la croissance, hôpitaux universitaires La Pitié-Salpêtrière-Charles-Foix, AP-HP, 75013 Paris, France. Electronic address:
Eur J Endocrinol
August 2017
Assistance Publique-Hôpitaux de Paris, Hôpital Universitaire Robert Debré, Service d'Endocrinologie Diabétologie Pédiatrique, Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Paris, France.
Graves' disease is an autoimmune disorder. It is the leading cause of hyperthyroidism, but is rare in children. Patients are initially managed with antithyroid drugs (ATDs), such as methimazole/carbimazole.
View Article and Find Full Text PDFJ Clin Endocrinol Metab
April 2017
Université Paris Diderot, Sorbonne Paris Cité, Paris 75019, France.
Context: Growth hormone (GH) is known to be diabetogenic, but the risk of diabetes in individuals treated with GH in childhood has been little evaluated, and conflicting results have been obtained.
Objective: To investigate the prevalence of diabetes and gestational diabetes in a population-based cohort of patients treated with GH for short stature in childhood in France.
Design, Setting, And Participants: Participants were a population-based cohort of 5100 children with idiopathic isolated GH deficiency, idiopathic short stature, or short stature in children born short for gestational age who started GH treatment between 1985 and 1996.
Semin Nucl Med
March 2017
Service de Médecine Nucléaire Pédiatrique, Hôpital d'Enfants Armand Trousseau, Paris, France; UPMC Univ Paris 06, Biophysique, Paris, France. Electronic address:
Thyroid scintigraphy holds a key place in the etiologic workup of neonatal hypothyroidism. Routine screening for this disorder in maternity hospitals in industrialized countries, for nearly 40 years, has permitted early treatment and thereby helped to prevent its physical and mental complications. Neonatal hypothyroidism affects approximately 1 in 3000 births.
View Article and Find Full Text PDFJ Clin Endocrinol Metab
May 2017
Dutch Growth Research Foundation, Westzeedijk 106, 3016 AH Rotterdam, The Netherlands.
Context: Growth hormone (GH) is prescribed for an increasing range of indications, but there has been concern that it might raise cancer risk. Published data are limited.
Objective: To examine cancer risks in relation to GH treatment.
Eur J Endocrinol
April 2017
AP-HPIE3M, Hôpital Pitié-Salpêtrière, Department of Endocrinology and Reproductive Medicine and Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Centre de Référence des Pathologies Gynécologiques Rares, ICAN, Paris, France.
Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is characterized by cortisol and in some cases aldosterone deficiency associated with androgen excess. Goals of treatment are to replace deficient hormones and control androgen excess, while avoiding the adverse effects of exogenous glucocorticoid. Over the last 5 years, cohorts of adults with CAH due to 21-hydroxylase deficiency from Europe and the United States have been described, allowing us to have a better knowledge of long-term complications of the disease and its treatment.
View Article and Find Full Text PDFHorm Res Paediatr
April 2017
Service d'Endocrinologie Diabétologie Pédiatrique, Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Hôpital Robert Debré, and Université Paris Diderot, Sorbonne Paris Cité, and Institut National de la Santé et de la Recherche Médicale (INSERM), Unité 1141, DHU Protect, Paris, France.
Fetal and neonatal autoimmune hyperthyroidism is a rare, serious but transient disorder. Early diagnosis and treatment are key objectives for an optimal prognosis and the well-being of the child. This review focuses on the management of these patients during the fetal and neonatal periods.
View Article and Find Full Text PDFPLoS One
July 2017
AP-HP, IE3M, Hôpital Pitié-Salpêtrière, Department of Endocrinology and Reproductive Medicine, Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Centre de Référence des Pathologies Gynécologiques Rares, Paris, France.
Premature ovarian insufficiency leads to through infertility and estrogen deficiency. Optimal management encompasses estrogen replacement therapy. Long-term outcome of women with POI is not known.
View Article and Find Full Text PDFAnn Biol Clin (Paris)
December 2016
Service d'endocrinologie et médecine de la reproduction et Centre de référence des maladies endocriniennes rares de la croissance, AP-HP, Hôpital Pitié-Salpêtrière, Paris, France ; UPMC Université Paris 6, Paris, France.
Polycystic ovary syndrome (PCOS) is the most common ovarian disorder associated with androgen excess in women, which justifies the growing interest of endocrinologists. This syndrome leads to clinical hyperandrogenism and/or a biological dysovulation and infertility. Its diagnosis is based on consensual diagnostic criteria, but which are likely to change in the near future with the rise of the interest of new markers such as AMH.
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