hrp0084p2-389 | Fat | ESPE2015

Childhood Craniopharyngioma with Hypothalamic Obesity – No Long-term Weight Reduction due to Rehabilitation Programs

Sterkenburg Anthe S , Hoffmann Anika , Gebhardt Ursel , Muller Hermann L

Background: Severe obesity due to hypothalamic involvement has major impact on prognosis in long-term survivors of childhood-onset craniopharyngioma. The long-term effects of rehabilitation efforts on weight development and obesity in these patients are not analysed up to now.Method: 108 patients with childhood-onset craniopharyngioma recruited in HIT Endo before 2001 were included in the study. Long-term weight development (BMI SDS after ...

hrp0084p2-519 | Pituitary | ESPE2015

Eating Behaviour, Weight Problems and Eating Disorders in 101 Long-Term Survivors of Childhood-Onset Craniopharyngioma

Hoffmann Anika , Sterkenburg Anthe S , Gebhardt Ursel , Muller Hermann L

Background: As a result of hypothalamic involvement and/or treatment-related hypothalamic damage, up to 75% of childhood craniopharyngioma patients develop hypothalamic obesity.outcome is important for optimization of treatment.Method: Eating behaviour was analysed in 101 survivors of childhood craniopharyngioma, recruited from 1980 to 2001 in the HIT-Endo multicentre study, and in 85 BMI-matched healthy controls using the Inventory for eating behavior a...

hrp0082p1-d3-192 | Pituitary | ESPE2014

Diencephalic Syndrome in Childhood Craniopharyngioma: Results of German Multicenter Studies on 485 Long-Term Survivors of Childhood Craniopharyngioma

Hoffmann Anika , Gebhardt Ursel , Sterkenburg Anthe , Warmuth-Metz Monika , Muller Hermann L

Background: Childhood craniopharyngiomas are known to be associated with an increased risk of excessive weight gain and hypothalamic obesity. Atypical clinical manifestations include the development of a diencephalic syndrome (DS) with a failure to thrive or weight loss.Objective and hypotheses: In a retrospective study we analyzed 21 of 485 childhood craniopharyngioma patients (4.3%) who presented with a low weight (<−2 BMI SDS) at time of dia...

hrp0084p2-509 | Pituitary | ESPE2015

Survival, Hypothalamic Obesity, and Neuropsychological/Psychosocial Status after Childhood-Onset Craniopharyngioma: Newly Reported Long-Term Outcomes

Sterkenburg Anthe S , Hoffmann Anika , Gebhardt Ursel , Warmuth-Metz Monika , Daubenbuchel Anna M M , Muller Hermann L

Background: Quality of life and long-term prognosis are frequently, often severely impaired in craniopharyngioma (CP) patients.Objective and hypotheses: Knowledge of risk factors for long-term outcome is important for optimisation of treatment.Method: Overall survival (OS) and progression-free survival (PFS), BMI, neuropsychological status (EORTCQLQ-C30, MFI-20), and psychosocial status were analysed in 261 patients with childhood-...

hrp0082p1-d3-195 | Pituitary | ESPE2014

Childhood Craniopharyngioma: Changes of Treatment Strategies in Multinational Trials During the Last 12 Years

Hoffmann Anika , Warmuth-Metz Monika , Gebhardt Ursel , Pietsch Torsten , Pohl Fabian , Kortmann Rolf-Dieter , Calaminus Gabriele , Muller Hermann L

Background: Despite high survival rates in childhood craniopharyngioma, prognosis is frequently impaired due to sequelae. Radical surgery was the treatment of choice for several decades. However, even at experienced surgical facilities radical surgery can result in hypothalamic disorders such as severe obesity.Objective and hypotheses: We analyzed, whether treatment strategies for childhood craniopharyngioma patients recruited in German studies (KRANIOPH...

hrp0084p2-514 | Pituitary | ESPE2015

Hydrocephalus and Hypothalamic Involvement in Paediatric Patients with Craniopharyngioma or Cysts of Rathke’s Pouch: Impact on Long-term Prognosis

Daubenbuchel Anna M M , Hoffmann Anika , Gebhardt Ursel , Warmuth-Metz Monika , Sterkenburg Anthe S , Muller Hermann L

Background: Paediatric patients with sellar masses such as craniopharyngioma (CP) or cyst of Rathke’s pouch (CRP) frequently suffer disease- and treatment-related sequelae.Objective and hypotheses: We analysed the impact and prognostic relevance of initial hydrocephalus (HY) and hypothalamic involvement (HI) on long-term survival and functional capacity (FC) in children with CP or CRP.Method: Using retrospective analysis of pa...

hrp0084p3-1196 | Thyroid | ESPE2015

Hoffmann Syndrome in a Boy with Severe Acquired Primary Hypothyroidism

Lorenzo Lucia Garzon , Rojo Jaime Cruz , del Pozo Cristina Martinez , Enamorado Noemi Nunez , del Pozo Jaime Sanchez

Background: Hoffmann syndrome is a specific and rare form of hypothyroid myopathy in adults characterized by presence of muscle stiffness, proximal weakness and pseudohypertrophy. When this occurs in a cretin child it is known as Kocher–Debré–Sémélaigne syndrome. Patients with more severe or longstanding untreated hypothyroidism are more likely to develop clinically significant muscle disease. Serum muscle enzyme levels as CK, myoglobin and lactate deh...

hrp0092p1-231 | Growth and Syndromes (to include Turner Syndrome) (1) | ESPE2019

Height and Weight Dynamics in Preschool Boys with Constitutional Delay of Growth and Puberty

Reinehr Thomas , Hoffmann Elisa , Rothermel Juliane , Lehrian Thersia , Binder Gerhard

Background: Constitutional delay of growth and puberty (CDGP) is one of the most frequent norm variants in children presenting with short stature. Knowing the height, growth, and weight pattern of CDGP in the first years of life is important to distinguish CDGP from growth hormone deficiency (GHD) or other diseases.Methods: We studied height and weight in the first 5 years of life in 54 boys with CDGP including measureme...

hrp0092p1-380 | Growth and Syndromes (to include Turner Syndrome) (2) | ESPE2019

A New Model of Adult Height Prediction Validated in Boys with Constitutional Delay of Growth and Puberty

Reinehr Thomas , Hoffmann Elisa , Rothermel Juliane , Lehrian Theresia , Binder Gerhard

Background: For children with retarded bone ages such as in constitutional delay of growth and puberty (CDGP) there are no specific methods to predict adult height based on bone age. Widely used methods such as Bayley-Pinneau (BP) tend to overestimate adult height in CDGP. Therefore, we aimed to develop a specific adult height prediction model for boys in pubertal age with retarded bone age >1 year.Methods</...

hrp0084p3-865 | Fat | ESPE2015

Waist Circumference to Body Height is a Suitable Measure of Cardiovascular Risk in Overweight and Obese Children

Yakovenko Vira , Teufel Ulrike , Henn Laura , Bettendorf Markus , Hoffmann Georg F , Grulich-Henn Juergen

Background: Several methods have been used to evaluate the risk of cardiovascular diseases in obese children. Both BMI and waist-to-hip ratio were suggested as risk factors. However, they did not prove to estimate the risk for cardiovascular events in adulthood. Recent studies suggest that the ratio of waist circumference to body height (WHtR) is a more reliable predictor for cardiovascular risk in 6-10-year old children (Kuba et al. 2013).Objec...