hrp0094p2-451 | Thyroid | ESPE2021

A girl with Trisomy 21 presents with Van Wyk-Grumbach syndrome. A rare diagnosis

Dikaiakou Eirini , Vlachopapadopoulou Elpis Athina , Kosteria Ioanna , Panos Alexandros , Dede Eirini , Koutrouveli Eleni , Zouridaki Christina , Michalacos Stefanos

Background: Van Wyk-Grumbach syndrome (VWGS) is a rare diagnosis that should be suspected in children with signs of peripheral precocious puberty and hypothyroidism. It is characterized by multicystic enlarged ovaries, in the presence of long-term severe hypothyroidism. Treatment of VWGS consists of hormonal replacement with levothyroxine. Usually, ovarian cysts and increased ovarian volume subside within an average of 2 months but can persist up to 12 months after treatment.<...

hrp0097rfc8.2 | Fat, metabolism and obesity 2 | ESPE2023

Acute rise of leptin after five days of dexamethasone and its association with hunger, fat mass, sleep and fatigue, in children with acute lymphoblastic leukemia

van Hulst Annelienke , Verwaaijen Emm , van den Berg Sjoerd , van Litsenburg Raphaele , Grootenhuis Martha , Fiocco Marta , Neggers Sebastian , van den Heuvel-Eibrink Marry , van den Akker Erica

Background & Aims: Children with acute lymphoblastic leukemia (ALL) frequently receive high doses dexamethasone during treatment, which may induce acute side effects. The aims of the current study were to determine the influence of a five-day dexamethasone course on changes in leptin, fat mass, body mass index (BMI), hunger, sleep and fatigue and to explore the associations between these changes.Methods: Pediatric AL...

hrp0094p2-21 | Adrenals and HPA Axis | ESPE2021

The reliability of Salivary Cortisol compared to Serum Cortisol for diagnosing adrenal insufficiency in the gold standard ACTH stimulation test in children

Ciancia Silvia , van den Berg Sjoerd A.A. , van den Akker Erica L.T. ,

Introduction: The gold standard for the diagnosis of adrenal insufficiency (AI) is the ACTH stimulation test. According to the guidelines approved by the European Endocrine Society, the ACTH stimulation test should be performed with the administration of the standard dose of i.v. Synachten® 250 µg for adults and children aged ≥ 2 years (infants should receive 15 µg/kg, children ≤ 2 years 125 µg). In many clinical settings a Syna...

hrp0086p1-p914 | Thyroid P1 | ESPE2016

Van Wyk Grumbach Syndrome with Kocher Smeglaine Debre Syndrome: Case Report of a Rare Association

Syed Mohd. Razi , Gupta Abhinav Kumar , Gupta Deepak Chand , Gupta Keshav Kumar

Background: Van Wyk Grumbach Syndrome (VWGS) is a rare presentation of juvenile hypothyroidism which manifests in females as chronic autoimmune hypothyroidism, isosexual pseudo-precocious puberty and multicystic ovaries. It uniquely presents with short stature and delayed bone age unlike other causes of precocious puberty. Kocher-Debre-Smeglaine Syndrome (KDSS) is a rare presentation of juvenile hypothyroidism manifesting as calf muscle pseudo-hypertrophy, delayed contraction ...

hrp0094p2-337 | Multisystem endocrine disorders | ESPE2021

Van Wyk Grumbach Syndrome in an Egyptian Female Pediatric Patient: a rare presentation of a common disease

Ibrahim Amany , Abdel Rahman Alshaimaa Mahfouz ,

Background: Van Wyc-Grumbach syndrome is a rare presentation of long standing pre-pubertal hypothyroidism characterized by bilateral multicystic ovaries, vaginal bleeding and delayed skeletal growth in case of female.Case Presentation: we present a clinical course, diagnosis and management of a case of Van Wyc-Grumbach syndrome in a 10-years old Egyptian counselled for recurrent vaginal bleeding and pubertal advancement for age. She was ...

hrp0095p1-398 | Thyroid | ESPE2022

Unusually Enlarged Ovaries Associated with Severe Hypothyroidism - Van Wyk and Grumbach Syndrome Case Report

Cvetković Dimitrije , Todorović Slađana , Milenković Tatjana , Mitrović Katarina , Vuković Rade , Panić Zarić Sanja , Savić Đorđe , Mijović Tanja , Smolović Dijana , Tončev Jovana

Introduction: Since 1960, enlarged polycystic ovaries associated with hypothyroidism, delayed bone age and precocious puberty are recognized as Van Wyk and Grumbach syndrome. Although it is rare entity, it should be considered in differential diagnosis of the enlarged polycystic ovaries.Case: An eleven-year-old girl was presented with sudden onset of abdominal pain and vomiting. A large mass with multiple cysts was seen ...

hrp0086p2-p969 | Thyroid P2 | ESPE2016

An Unusual Presentation of Hashimoto Thyroiditis (HT) and Precocious Puberty: The Van Wyk-Grumbach Syndrome

Leka-Emiri Sofia , Karachaliou Feneli , Fotinou Aspasia , Petrou Vassilis , Michalakos Stefanos

Background: The association of primary hypothyroidism and isosexual precocious pseudopuberty in females was first described in 1960 by Van Wyk and Grumbach. The unique elements that lead to the diagnosis are FSH-dominated sexual precocity with non advanced bone age in the presence of hypothyroidism.Objective and methods: Describe an 8.5 years old girl with hypothyroidism due to HT and clinical and hormonal features of Van-Wyk and Grumbach syndrome.<p...

hrp0094p2-180 | Fat, metabolism and obesity | ESPE2021

Impact of the COVID-19 pandemic and related lockdown measures on lifestyle behaviours and quality of life in children and adolescents with severe obesity

Welling MS , Abawi O , van den Eynde E , van Rossum EFC , Halberstadt J , Brandsma AE , Kleinendorst L , van den Akker ELT , van der Voorn B ,

Introduction: Lockdown measures following the COVID-19 pandemic are shown to have greatly affected lifestyle behaviours and health-related quality of life (HRQoL) of children. The impact on children with severe obesity has not yet been described. Aim of this study was to investigate the impact of COVID-19 lockdown on eating behaviours, physical activity (PA), screen time, and HRQoL of children (including adolescents) with severe obesity....

hrp0082p3-d1-936 | Puberty and Neuroendocrinology | ESPE2014

Association of Van Wyk Grumbach and Debre Semelaigne Syndromes in Two Cases with Severe Hypothroidism

Demirel Fatma , Oden Alkim , Tayfun Meltem , Ucakturk Ahmet , Gungor Ali

Background: Van Wyk Grumbach (VWG) and Kocher Debre Semelaigne (KDS) syndromes are rare syndromes with clinical manifestation of hypothyroidism associated with precious pseudo puberty and myopathic pseudomuscular hypertrophy. We present two cases that have the characteristic of both VWGS and KDSS syndromes developed in association with a long-term untreated hypothyroidism.Case 1: Seventeen years old girl was referred to our hospital due to menstrual irre...