Children with multiple endocrine neoplasia type 2B: Not tall and marfanoid, but short with normal body proportions

التفاصيل البيبلوغرافية
العنوان: Children with multiple endocrine neoplasia type 2B: Not tall and marfanoid, but short with normal body proportions
المؤلفون: Annemarie A. Verrijn Stuart, Medard F M van den Broek, Gerlof D. Valk, Hanneke M van Santen
المصدر: Clinical Endocrinology
سنة النشر: 2021
مصطلحات موضوعية: Adult, medicine.medical_specialty, Pediatrics, Endocrinology, Diabetes and Metabolism, growth, Adrenal Gland Neoplasms, Multiple Endocrine Neoplasia Type 2a, Short stature, Endocrinology, Pediatric Endocrinology, Internal medicine, medicine, Humans, Thyroid Neoplasms, marfanoid body habitus, Multiple endocrine neoplasia, Child, Retrospective Studies, Body proportions, business.industry, Marfanoid, multiple endocrine neoplasia type 2B, Tall Stature, medicine.disease, Carcinoma, Neuroendocrine, short stature, Arm span, Original Article, final height, medicine.symptom, ORIGINAL ARTICLES, business, Body mass index, Multiple endocrine neoplasia type 2b
الوصف: Objective Multiple endocrine neoplasia 2B (MEN2B) is characterised by early-onset medullary thyroid carcinoma (MTC), pheochromocytoma and several nonendocrine manifestations. Unfortunately, MEN2B is often diagnosed late, after the development of clinically significant MTC. Marfanoid habitus is considered an important related feature, which may lead to the assumption that patients with MEN2B have tall stature. Here, we describe the longitudinal growth and body proportions of eight MEN2B patients during childhood. Design It is a retrospective case series. Methods Patients were under the care of a Dutch MEN expertise centre. Growth patterns were assessed and interpreted in relation to body mass index (BMI), age at diagnosis and at thyroidectomy, extensiveness of disease manifestations and parental height. Results Seven patients were short during childhood, of whom four showed growth below target height range (THR) and three at the lowest margin of THR. Only one patient grew well within THR. All patients who attained final height (n = 4) ended within THR, despite short stature during childhood. Arm span/height ratio was not increased and upper segment/lower segment ratio was not reduced in any patient. Short stature in childhood in this study did not seem to be associated with age at diagnosis, age at thyroidectomy, extensiveness of MTC, endocrine deficiencies or BMI. Conclusions This study shows that children with MEN2B may well present with short rather than tall stature. Thereafter, final height within THR was attained in those who already reached adulthood, but none had tall stature. Finally, body proportions were normal in all children and adults in this case series, not underlining a 'marfanoid' body habitus.
تدمد: 1365-2265
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ba84a1886901f47cfa4004dc6c4470e0Test
https://pubmed.ncbi.nlm.nih.gov/34160841Test
حقوق: OPEN
رقم الانضمام: edsair.doi.dedup.....ba84a1886901f47cfa4004dc6c4470e0
قاعدة البيانات: OpenAIRE