hrp0089p2-p326 | Pituitary, Neuroendocrinology and Puberty P2 | ESPE2018

Pituitary Adenomas in Children and Adolescents: A Retrospective Single-Centre Analysis

Breil Thomas , Lorz Catherine , Choukair Daniela , Mittnacht Janna , Inta Ioana , Klose Daniela , Jesser Jessica , Schulze Egbert , Bettendorf Markus

Background: Paediatric pituitary adenomas are rare and mostly benign disorders which may secrete pituitary hormones. Prolactinomas account for half of all pituitary adenomas (PROLA), followed by non-secreting adenomas (20–40%; incidentalomas), adrenocorticotropic-hormone secreting adenomas (10–30%; ACTHA) and growth-hormone-secreting adenomas (5–15%; GHA).Methods: In this single-centre retrospective analysis clinical, biochemical and radio...

hrp0086p1-p99 | Bone & Mineral Metabolism P1 | ESPE2016

Automated Greulich-Pyle Bone Age Determinations in Children with Chronic Endocrine Diseases

Choukair Daniela , Huckmann Annette , Mittnacht Janna , Inta Ioana , Klose Daniela , Schenk Jens Peter , Bettendorf Markus , Thodberg Hans Henrik

Background: Prediction of adult height is a standard procedure in pediatric endocrinology, but it is associated with considerable interrater variability.Objective and hypotheses: To compare the new adult height prediction (PAH) method by automated bone age determination (BoneXpert™) with the conventional PAH method by Bayley Pinneau (BP) based on bone age determination according to Greulich & Pyle. Furthermore, to assess measures of bone health ...

hrp0092mte5 | Managing Endocrinopathies in McCune-Albright Syndrome | ESPE2019

Managing Endocrinopathies in McCune-Albright Syndrome

Tessaris Daniele

McCune Albright Syndrome (MAS, OMIM # 174800) is a rare congenital sporadic disorder with an estimated prevalence ranging from 1 in 1,00,000 to 1 in 100,000. MAS is caused by a post-zygotic somatic activating mutation of the GNAS1 gene resulting in an increased GSα protein signaling leading to hyperfunction of glycoprotein hormone receptors, autonomous cell proliferation, and hormonal hypersecretion. The mosaic constitutive activation of this signal transducer is clinical...

hrp0095rfc3.4 | Early Life and Multisystem Endocrinology | ESPE2022

Immunophenotype and proinflammatory cytokines in ROHHAD(NET) patients compared to age- and sex-matched obese patients

Angelelli Alessia , Morandi Fabio , Prigione Ignazia , Fava Daniela , Napoli Flavia , Maghnie Mohamad

Background: ROHHAD(NET) syndrome has been proposed as an acquired neuroimmunological dysfunction. Aim of the study is to compare the phenotype of lymphocyte subpopulations in patients vs controls and to evaluate the plasma levels of pro-inflammatory cytokines/chemokines in ROHHADNET syndrome.Patients and Methods: We included 14 patients (9F;5M), selected by clinical criteria, matched with 11 controls with simple obesity ...

hrp0095p1-407 | Adrenals and HPA Axis | ESPE2022

Agreement between Acth-low test and Insuline Tolerance Test in Patients with Risk Factors for Central Adrenal Insufficiency

Panciroli Marta , Angelelli Alessia , Fava Daniela , Patti Giuseppa , Napoli Flavia , Di Iorgi Natascia , Maghnie Mohamad

Background: Central adrenal insufficiency(CAI) is a potential life-threatening condition and a prompt diagnosis represents a clinical challenge; indeed, the Insulin Tolerance Test(ITT), gold standard for CAI diagnosis, can be contraindicated in some conditions. Aims were to evaluate the diagnostic value of the ACTH low test(ACTH-LT) compared to the ITT and to identify eventual determinants(primary diagnosis, risk factors, symptoms, baseline cortisol, hypothala...

hrp0095p1-565 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Adult Height is impaired in females with Medulloblastoma and hypogonadism: Impact of Pubertal Timing

Ferraro Paola , Casalini Emilio , Fava Daniela , Maghnie Mohamad , Patti Giuseppa , Di Iorgi Natascia

Introduction and Aim of the Study: girls with previous pediatric medulloblastoma (MB) are at risk of short stature and impairment of the hypothalamic pituitary gonadal function due to multiple risk factors;our aim was to assess the impact of hypergonadotropic hypogonadism (HH) on growth up to final height (FH) in females with or without growth hormone deficiency (GHD).Methods: anthropometrics (height-Ht-SDS, BMI-SDS, Tan...

hrp0095p2-28 | Bone, Growth Plate and Mineral Metabolism | ESPE2022

Longitudinal assessment of bone health index (BHI) as a measure of bone health in short-statured children before and during treatment with recombinant growth hormone

Holzapfel Lukas , Bettendorf Markus , Rabe Michael , Mittnacht Janna , Choukair Daniela , Schenk Jens-Peter

Introduction: Bone Health Index (BHI) determined by measurement of cortical thickness of metacarpalia II-IV in x-rays of the left hand represents a method to estimate bone health in children. Aim of this study was to investigate changes of BHI SDS in the course of growth hormone (GH) treatment.Method: 256 consecutive children with short stature (isolated GH deficiency (IGHD) n = 121, multiple pituitary hormone deficiency...

hrp0092p1-365 | GH and IGFs (2) | ESPE2019

De Novo Formation of Neutralizing IGF-I Antibodies During rhIGF-1 Treatment in a Girl with IGFALS Deficiency as Distinct Adverse Event Interfering with Growth Promotion

Mittnacht Janna , Breil Thomas , Choukair Daniela , Duffert Christin , Hwa Vivian , Rosenfeld Ron , Bettendorf Markus

Background: IGFALS deficiency is a rare cause of GH insensitivity (GHI). We report a German girl with short stature who was born as 2nd child at 40 weeks of gestation. Her Caucasian parents were unrelated and healthy (target height 168 cm, SDS 0.2). She was born appropriately sized for gestational age (49 cm, SDS -1.2; 2950 g, SDS-1.2). Height (104.8 cm; SDS -2.94) and height velocity (5.1 cm / year; SDS -1.67) were reduced at the age of 6 years.</p...

hrp0092p1-428 | Thyroid (2) | ESPE2019

Anti-Gastric Parietal Cells Antibodies for Autoimmune Gastritis Screening: A Follow-Up Study in Young Patients with Autoimmune Thyroid Disease.

Calcaterra Valeria , Montalbano Chiara , Vinci Federica , Miceli Emanuela , Regalbuto Corrado , Albertini Riccardo , Larizza Daniela

Introduction: The association between ATD and AIG is very poorly characterized in pediatric age. We review the prevalence of the anti-gastric parietal cells antibodies (APCA) in young patients with ATD and we evaluated the development of AIG during follow-up, in order to define the usefulness of these markers for AIG screening in these patients.Patients and Methods: We evaluated 220 children and adolescents (11.28 ±...

hrp0092p2-64 | Diabetes and Insulin | ESPE2019

Autoimmune Thyroid Diseases in Children and Adolescents with Maturity Onset Diabetes of the Young

Calcaterra Valeria , Regalbuto Corrado , Dobbiani Giulia , Vinci Federica , Montalbano Chiara , Larizza Daniela

Background and Aim: The relationship between T1DM and autoimmune thyreopathies is known and described, but the relationship between thyreopathies and other type of diabetes is not sufficiently clarified in pediatric age. The aim of our study was to assess the prevalence of autoimmune thyroid diseases (ATD) in children and adolescents with maturity onset diabetes of the young (MODY) in comparison with patients with T1DM and control group....