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.<...

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 ...

hrp0095rfc6.5 | Sex Development and Gonads | ESPE2022

The effect of GnRHa and testosterone treatment on growth in trans boys

Willemsen Lieve , Boogers Lidewij , Wiepjes Chantal , van Trotsenburg Paul , Klink Daniel , den Heijer Martin , Hannema Sabine

Background: Pubertal growth and adult height are important to many transgender adolescents undergoing medical transition. However, few studies are available on the impact of puberty suppression (PS) with GnRH analogues (GnRHa) and hormonal therapy (HT) with testosterone on growth. In this longitudinal cohort study, we investigated the effect of PS and HT on growth and adult height in transgender boys.Methods: A total of ...

hrp0094p2-446 | Thyroid | ESPE2021

The efficacy and short- and long-term side effects of radioactive iodine treatment in pediatric Graves’ disease: a systematic review

Lutterman Sarah , Zwaveling-Soonawala Nitash , Verberne Hein , Verburg Frederik , van Trotsenburg Paul , Mooij Christiaan

Background: Graves’s disease (GD) is the most common cause of hyperthyroidism. Maximal 30% of pediatric GD patients achieve remission with anti-thyroid drugs. The majority of patients therefore require definitive treatment. Both thyroidectomy and radioactive iodine (RAI) are often used as definitive treatment for GD. However, data on efficacy, and short- and long-term side effects of RAI treatment for pediatric GD are relatively scarce.Methods: A ...

hrp0097p1-367 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2023

Changes in body composition in transgender adolescents during puberty suppression and hormone treatment

Boogers Lidewij , Reijtenbagh Sterre , Wiepjes Chantal , van Trotsenburg Paul , den Heijer Martin , Hannema Sabine

Context: Transgender adolescents can be treated with puberty suppression (PS) using GnRH agonists (GnRHa), and subsequent hormone therapy (HT). Up to this date, it has not been described at what rate body composition in transgender adolescents changes during the first years of treatment. Also, it is unknown whether Tanner stage at which treatment is initiated, might affect this treatment outcome.Methods: Transgender adol...

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...

hrp0092rfc9.2 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Age-Specific Reference Values for Plasma FT4 and TSH Concentrations in Healthy, Term Neonates at Day Three to Seven, and 13 to 15 of Life

Naafs J.C. , Heinen C.A. , Zwaveling-Soonawala N. , van der Schoor S.R.D. , Tellingen V. van , Heijboer A.C. , Fliers E. , Boelen A. , van Trotsenburg A.S.P.

Background: Congenital hypothyroidism (CH) is a common and preventable cause of mental retardation in children, and is detected using dried blood spots in many neonatal screening programs. Upon suspicion of CH, plasma free thyroxine (FT4) and thyroid stimulating hormone (TSH) concentrations are measured. CH can be of thyroidal or central origin (CH-T and CH-C, respectively). While CH-T diagnosis is based on an elevated plasma TSH in combination with a low FT4,...

hrp0094p2-452 | Thyroid | ESPE2021

Complications after pediatric thyroidectomy: lymph node dissection is a risk factor for permanent hypocalcemia

van Rooijen Jesse J. , Paul van Trotsenburg A.S. , Zwaveling-Soonawala Nitash , Nieveen van Dijkum Els J.M. , Engelsman Anton F. , Derikx Joep P.M. , Mooij Christiaan F.

Background: Thyroidectomy is a definitive treatment option in some benign thyroid disorders and the definitive treatment option for thyroid cancer. As postoperative mortality is extremely rare data on postoperative complications and long-term health consequences are important.Objective: To evaluate the frequencies of short- and long-term complications, and their risk factors in pediatric patients (0-18 years) who underwent a thyroidectomy in a tertiary c...