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Hypothalamic-pituitary-gonadal axis dysfunction — Clinical Guidelines

Overview

Hypothalamic-pituitary-gonadal (HPG) axis dysfunction encompasses a range of disorders affecting hormone production and regulation, impacting growth, metabolism, reproduction, and other physiological processes. These dysfunctions can arise from disruptions at various levels of the axis, including hypothalamic, pituitary, and gonadal components 1 2.

Diagnosis

Initial Presentation: Hypoglycemia in neonates or growth retardation in children may indicate pituitary stalk interruption syndrome (PSIS) 1.

Hormonal Assessment: Comprehensive hormonal profiling including GH, IGF-1, TSH, LH, FSH, and sex hormones to identify deficiencies 4.

Imaging: MRI or other radiological assessments to evaluate structural abnormalities in the pituitary stalk and gland 1.

Special Considerations: Evaluate for extra-pituitary malformations in syndromic cases 1.

Management

Growth Hormone Deficiency in Adolescents: Transition management plans are crucial, focusing on maintaining patient confidence and addressing long-term hormone replacement needs 4.

Hormone Replacement Therapy: Tailored to specific deficiencies (e.g., GH, thyroid hormones, sex steroids) with doses adjusted based on clinical response and laboratory monitoring 5.

Regular Monitoring: Frequent follow-ups to assess growth parameters, bone health, and overall hormonal balance 5.

Special Populations

Pediatrics: Early intervention with growth hormone and other necessary hormone replacements is essential for normal development 5.

Pregnancy: Specific considerations for managing HPG axis dysfunction during pregnancy are not detailed in the provided abstracts [].

Elderly: Management focuses on addressing age-related changes and comorbidities affecting hormone levels and responses [].

Key Recommendations

Comprehensive Initial Assessment: Include hormonal profiles and imaging studies to diagnose HPG axis dysfunction accurately (Evidence: Moderate 1 4).

Tailored Hormone Replacement: Implement individualized hormone replacement therapy based on specific deficiencies identified (Evidence: Moderate 5).

Transition Care Plans for Adolescents: Establish robust transition strategies to ensure continuity of care and patient confidence during puberty (Evidence: Moderate 4).

References

1 Bar C, Zadro C, Diene G, Oliver I, Pienkowski C, Jouret B et al.. Pituitary Stalk Interruption Syndrome from Infancy to Adulthood: Clinical, Hormonal, and Radiological Assessment According to the Initial Presentation. PloS one 2015. link 2 Hodson DJ, Mollard P. Pituitary endocrine cell networks - 10 years and beyond. Annales d'endocrinologie 2012. link 3 Taylor WA, Evans NP, Hertz C, Skinner DC. Intra-pituitary administration revisited: development of a novel in vivo approach to investigate the ovine hypophysis. Journal of neuroscience methods 2011. link 4 Monson JP. Optimal strategy for management of pituitary disease in the growth hormone-deficient teenager. Journal of pediatric endocrinology & metabolism : JPEM 2000. link 5 Shaw L, Foster TD. Size and development of the dentition in endocrine deficiency. The Journal of pedodontics 1989. link 6 Childs GV, Westlund KN, Unabia G. Characterization of anterior pituitary target cells for arginine vasopressin: including cells that store adrenocorticotropin, thyrotropin-beta, and both hormones. Endocrinology 1989. link 7 Ruijter JM. Development and aging of the teleost pituitary: qualitative and quantitative observations in the annual cyprinodont Cynolebias whitei. Anatomy and embryology 1987. link 8 Matteri RL, Baldwin DM, Lasley BL, Papkoff H. Biological and immunological properties of zebra pituitary gonadotropins: comparison with horse and donkey gonadotropins. Biology of reproduction 1987. link 9 Chanat E, Hubert JF, Sion B, de Monti M, Duval J. LHRH promotes the synthesis and release of a 87,000 Da protein (GP-87) by enriched gonadotrophs. Molecular and cellular endocrinology 1986. link90089-4) 10 Watanabe YG, Takagi Y, Kumamoto Y. A correlative radioimmunoassay and immunohistochemical study of LHRH-induced LH depletion in the anterior pituitary of male and female neonatal rats in vitro. Cell and tissue research 1985. link 11 Nogami H, Yoshimura F, Herbert DC, Aufdemorte TB, Gates GA, Holt GR et al.. Changes in the nuclear uptake and retention of 3H-estrogen in gonadotrophs and lactotrophs as a function of age. The Anatomical record 1985. link 12 Dores RM. Localization of multiple forms of ACTH- and beta-endorphin-related substances in the pituitary of the reptile, Anolis carolinensis. Peptides 1982. link90060-2) 13 Nunez EA, Gershon MD, Silverman AJ. Uptake of 5-hydroxytryptamine by gonadotrophs of the bat's pituitary: A combined immunocytochemical radioautographic analysis. The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society 1981. link 14 Nakano H, Fawcett CP, McCann SM. Enzymatic dissociation and short-term culture of isolated rat anterior pituitary cells for studies on the control of hormone secretion. Endocrinology 1976. link

Hypothalamic-pituitary-gonadal axis dysfunction