Clinical characteristics and effects of GH replacement therapy in adults with childhood-onset craniopharyngioma compared with those in adults with other causes of childhood-onset hypothalamic–pituitary dysfunction

Abstract

Objective: Adults with childhood-onset (CO) craniopharyngioma (COCP) have poor quality of life (QoL) and clinical outcomes, but few studies have compared these patients with adults with other causes of CO hypothalamic-pituitary dysfunction. In this study, we compared baseline clinical characteristics and patient-reported outcomes before starting GH replacement therapy in adults with GH deficiency (GHD) due to COCP with those of adults either with CO idiopathic/congenital hypopituitarism (COH) or with CO extrasellar (COE) tumours, and evaluated the 1-and 5-year effects of GH replacement therapy. Subjects and methods: Retrospective analysis of the data recorded in KIMS (Pfizer International Metabolic Database) was carried out. Patients with COCP, COH and COE tumours were evaluated at baseline, and after 1 and 5 years of therapy. Results: Compared with COH and COE patients, more COCP patients underwent surgery, had greater abnormalities of body composition and higher prevalence of pituitary hormone deficits (all P!0.001), but comparable fasting glucose, HbA1c, total cholesterol and LDL-cholesterol levels, marital status, parenthood, living arrangements, education, employment and annual sick-leave days. After 1 and 5 years of GH replacement therapy, similar changes were evident with regard to body composition, fasting glucose and HbA1c levels, QoL, and the level of and satisfaction with physical activity across the three groups. Conclusions: Adults with untreated COCP with GHD at baseline demonstrated more co-morbidities including greater abnormalities of body composition, pituitary hormone deficits and visual field defects. Overall, adults with COCP, COH and COE tumours responded comparably to short-and long-term GH replacement therapy, suggesting that patients with GHD due to COCP benefited from GH replacement therapy to a similar degree as those with other causes of CO hypothalamic-pituitary dysfunction did.

Systematic review and meta‐analysis of health‐related quality of life in pediatric CNS tumor survivors

“…Sixteen percent of papers included in the review (n = 12) were longitudinal in design . The results of these studies varied widely from reporting a progressive decline in HRQOL over time to reporting stable HRQOL over time to a reported increase in HRQOL over time .…”

“…Sixteen percent of papers included in the review (n = 12) were longitudinal in design. [26][27][28][29][30][31][32][33][34][35][36][37] The results of these studies varied widely from reporting a progressive decline in HRQOL over time 29,34 to reporting stable HRQOL over time 31,32,36 to a reported increase in HRQOL over time. 26,28,30,31,36 Importantly, the majority of studies reporting improved HRQOL over time were in the context of an intervention study (e.g., Head Start).…”

Management of hypothalamic disease in patients with craniopharyngioma

“…Growth hormone replacement therapy is not associated with increased CP tumour recurrence . Children with CP and GHD gain height on GH therapy . Adult CP patients on GH therapy require similar doses of GH to NFPA patients and show improved quality of life .…”

Cтан Серцево-Судинної Системи Та Ліпідний Спектр При Соматотропній Недостатності У Молодих Дорослих Із Маніфестацією В Дитячому Віці (Огляд Літератури Та Власні Спостереження)

“…Якщо терапія рГР не від-новлюється, у молодих дорослих з дит-ДГР можливе виникнення множинних метаболічних порушень, а саме: гіпертригліцеридемії, неалкогольної жирової дистрофії печінки, гіперхолестеринемії, ЦД, АГ, що діагностуються вже в перші роки після припинення терапії рГР. Більше двох супутніх захворювань вияв-ляється у 30-32 % хворих [20,41,42].…”