학술논문
Approach to the Patient: Management of Pituitary Hormone Replacement Through Transition
Approach to the Patient
Approach to the Patient
Document Type
Academic Journal
Source
Journal of Clinical Endocrinology & Metabolism. July 2022, Vol. 107 Issue 7, p2077, 15 p.
Subject
Language
English
ISSN
0021-972X
Abstract
Hypopituitarism and Transition--Introduction Hypopituitarism is a rare and complex endocrine disorder associated with significant morbidity and mortality (1, 2). It is highly heterogeneous, and clinical manifestations range from isolated pituitary [...]
Hypopituitarism in childhood is a rare, complex disorder that can present with highly variable phenotypes, which may continue into adult life. Pituitary deficits can evolve over time, with unpredictable patterns resulting in significant morbidity and mortality. Hypopituitarism and hypothalamic dysfunction may be associated with challenging comorbidities such as obesity, learning difficulties, behavioral issues, sleep disturbance, and visual impairment. Transition is the purposeful planned movement of adolescents and young adults with chronic conditions from child-centered to adult-oriented health care systems with a shift from parent- to patient-focused care. To achieve effective transition within a health care setting, the inherent challenges involved in the evolution from a dependent child to an independent adult must be recognized. Transition is a critical time medically for patients with hypopituitarism. Complex issues with respect to puberty, attainment of optimal stature, adherence to treatment, and acceptance of the need for life-sustaining medications need to be addressed. For health care professionals, transition is an opportunity for reassessment of the pituitary deficits and the need for lifelong replacement therapies, often against a background of complex psychological issues. We present 4 illustrative cases of hypopituitarism of differing etiologies with diverse clinical presentations. Diagnostic and management processes from clinical presentation to young adulthood are discussed, with a particular focus on needs and outcomes through transition. Key Words: hypopituitarism, transition, hormone replacement, adolescence Abbreviations: AP, anterior pituitary; BMI, body mass index; BMD, bone mineral density; CDI, central diabetes insipidus; CHH, congenital hypogonadotropic hypogonadism; CNS, clinical nurse specialist; DDAVP, desmopressin; DVT, deep vein thrombosis; EPP, ectopic posterior pituitary; FT4, free T4; GHD, GH deficiency; HD, hypothalamic dysfunction; HH, hypogonadotropic hypogonadism; HP, hypothalamopituitary; HRT, combined cyclical hormone replacement therapy; IGHD, isolated GH deficiency; ITT, insulin-tolerance test; LCH, langerhans cell histiocytosis; MRI, magnetic resonance imaging; ONH, optic nerve hypoplasia; PSA, pituitary stalk absence; QoL, quality of life; SDS, SD score; SOD, septo-optic dysplasia; TRH, thyrotropin-releasing hormone
Hypopituitarism in childhood is a rare, complex disorder that can present with highly variable phenotypes, which may continue into adult life. Pituitary deficits can evolve over time, with unpredictable patterns resulting in significant morbidity and mortality. Hypopituitarism and hypothalamic dysfunction may be associated with challenging comorbidities such as obesity, learning difficulties, behavioral issues, sleep disturbance, and visual impairment. Transition is the purposeful planned movement of adolescents and young adults with chronic conditions from child-centered to adult-oriented health care systems with a shift from parent- to patient-focused care. To achieve effective transition within a health care setting, the inherent challenges involved in the evolution from a dependent child to an independent adult must be recognized. Transition is a critical time medically for patients with hypopituitarism. Complex issues with respect to puberty, attainment of optimal stature, adherence to treatment, and acceptance of the need for life-sustaining medications need to be addressed. For health care professionals, transition is an opportunity for reassessment of the pituitary deficits and the need for lifelong replacement therapies, often against a background of complex psychological issues. We present 4 illustrative cases of hypopituitarism of differing etiologies with diverse clinical presentations. Diagnostic and management processes from clinical presentation to young adulthood are discussed, with a particular focus on needs and outcomes through transition. Key Words: hypopituitarism, transition, hormone replacement, adolescence Abbreviations: AP, anterior pituitary; BMI, body mass index; BMD, bone mineral density; CDI, central diabetes insipidus; CHH, congenital hypogonadotropic hypogonadism; CNS, clinical nurse specialist; DDAVP, desmopressin; DVT, deep vein thrombosis; EPP, ectopic posterior pituitary; FT4, free T4; GHD, GH deficiency; HD, hypothalamic dysfunction; HH, hypogonadotropic hypogonadism; HP, hypothalamopituitary; HRT, combined cyclical hormone replacement therapy; IGHD, isolated GH deficiency; ITT, insulin-tolerance test; LCH, langerhans cell histiocytosis; MRI, magnetic resonance imaging; ONH, optic nerve hypoplasia; PSA, pituitary stalk absence; QoL, quality of life; SDS, SD score; SOD, septo-optic dysplasia; TRH, thyrotropin-releasing hormone