{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T20:26:17Z","timestamp":1780086377436,"version":"3.54.0"},"reference-count":27,"publisher":"The Endocrine Society","issue":"7","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2006,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Context: Changes in body weight, statural growth rate, and puberty may be the presenting symptoms of hypothalamic-pituitary tumors.<\/jats:p>\n               <jats:p>Objective: The objective of the study was to assess the relationship between the tumor and its treatment and the weight, growth rate, and onset of puberty, using the diencephalic syndrome of emaciation as model.<\/jats:p>\n               <jats:p>Patients: Eleven patients seen before 1 yr of age, except one aged 9 yr, for diencephalic syndrome of emaciation due to hypothalamic pilocytic astrocytoma, were treated by surgical resection (n = 9), cranial irradiation (n = 7), and\/or chemotherapy (n = 10).<\/jats:p>\n               <jats:p>Results: At diagnosis, growth rate was normal, despite the emaciation, and there was no hypothalamic-pituitary deficiency, except in the oldest patient. After tumor treatment, all had GH and thyroid-stimulating hormone deficiencies, but only three, who underwent major surgical resection, also had ACTH deficiency and diabetes insipidus. Eight became obese, and all but the oldest had transient precocious puberty. Plasma leptin concentrations were very low at diagnosis, increased after tumor treatment, and decreased transiently in one boy when the testosterone increased. The plasma soluble leptin receptor concentrations changed in the opposite direction, leading to an increase in the free leptin index, including in the three patients whose tumor was reduced without surgery. The body mass index was correlated positively with plasma leptin (rho = 0.73, P = 0.0004) and free leptin index (rho = 0.63, P &amp;lt; 0.004) and negatively with ghrelin (rho = \u22120.49, P &amp;lt; 0.03) concentrations.<\/jats:p>\n               <jats:p>Conclusions: The obesity that occurs after treatment of hypothalamic tumors is not due to dysregulation of leptin secretion because it and plasma soluble leptin receptor remain regulated by factors like testosterone. This study also shows the influence of weight, possibly via leptin secretion, on the transient hypothalamic-pituitary-gonadal activation that occurs during the first year of life.<\/jats:p>","DOI":"10.1210\/jc.2006-0322","type":"journal-article","created":{"date-parts":[[2006,4,19]],"date-time":"2006-04-19T01:26:29Z","timestamp":1145409989000},"page":"2467-2473","source":"Crossref","is-referenced-by-count":46,"title":["Diencephalic Syndrome due to Hypothalamic Tumor: A Model of the Relationship between Weight and Puberty Onset"],"prefix":"10.1210","volume":"91","author":[{"given":"Raja","family":"Brauner","sequence":"first","affiliation":[{"name":"Universite\u0301 Paris-Descartes (R.B.), Assistance Publique-Ho\u0302pitaux de Paris, Ho\u0302pital Bice\u0302tre, Unite\u0301 d\u2019Endocrinologie Pe\u0301diatrique, 94270 Le Kremlin-Bice\u0302tre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Trivin","sequence":"additional","affiliation":[{"name":"Assistance Publique-Ho\u0302pitaux de Paris (C.T., J.-C.S.), Ho\u0302pital Necker-Enfants Malades, Service d\u2019Explorations Fonctionnelles, 75743 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Zerah","sequence":"additional","affiliation":[{"name":"Universite\u0301 Paris-Descartes (M.Z., C.S.-R.), Assistance Publique-Ho\u0302pitaux de Paris, Ho\u0302pital Necker-Enfants Malades, Service de Neurochirurgie Pe\u0301diatrique, 75743 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Claude","family":"Souberbielle","sequence":"additional","affiliation":[{"name":"Assistance Publique-Ho\u0302pitaux de Paris (C.T., J.-C.S.), Ho\u0302pital Necker-Enfants Malades, Service d\u2019Explorations Fonctionnelles, 75743 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franc\u0327ois","family":"Doz","sequence":"additional","affiliation":[{"name":"Universite\u0301 Paris-Descartes (F.D.), Institut Curie, De\u0301partement d\u2019Oncologie Pe\u0301diatrique, 75248 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chantal","family":"Kalifa","sequence":"additional","affiliation":[{"name":"De\u0301partement d\u2019Oncologie Pe\u0301diatrique (C.K.), Institut Gustave Roussy, 94805 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Sainte-Rose","sequence":"additional","affiliation":[{"name":"Universite\u0301 Paris-Descartes (M.Z., C.S.-R.), Assistance Publique-Ho\u0302pitaux de Paris, Ho\u0302pital Necker-Enfants Malades, Service de Neurochirurgie Pe\u0301diatrique, 75743 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","reference":[{"key":"2020071613403479900_R1","first-page":"421","article-title":"Influence of tumor location on the presentation and evolution of craniopharyngiomas","volume":"103","author":"Meuric","year":"2005","journal-title":"J Neurosurg"},{"key":"2020071613403479900_R2","first-page":"2734","article-title":"Obesity in childhood craniopharyngioma: relation to post-operative hypothalamic damage shown by magnetic resonance imaging","volume":"81","author":"De Vile","year":"1996","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071613403479900_R3","first-page":"274","article-title":"A diencephalic syndrome of emaciation in infancy and childhood","volume":"26","author":"Russell","year":"1951","journal-title":"Arch Dis Child"},{"key":"2020071613403479900_R4","volume-title":"Clin Endocrinol (Oxf)","author":"Trivin"},{"key":"2020071613403479900_R5","doi-asserted-by":"crossref","first-page":"4572","DOI":"10.1200\/JCO.2003.03.043","article-title":"Progression-free survival in children with optic pathway tumors: dependence on age and the quality of the response to chemotherapy. 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