Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Ogechukwu Hannah Nnabude, MD

Overview

The symptoms of adiposogenital dystrophy are due to deficiency of hypothalamic and pituitary hormones. The condition will not improve without treatment and often worsens over time.

Adiposogenital dystrophy natural history, complication, and prognosis

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Natural History

Patients develop prepubertal obesity with fat deposition at the breasts, hips, femoral regions, and abdomen, Dehydration and hypernatremia can occur as a result of antidiuretic hormone deficiency. Mental retardation may also be seen^[1]. Radiologic findings include delayed bone ossification. If a pituitary tumor is involved, there may also be visual disturbances, which may be seen with or without compression of the optic chiasm ^[2] ^[3].

Complications

Complications of adiposogenital syndrome include^[4]^[5]:

Obesity which can further lead to a variety of health conditions
Mental retardation which has socioeconomic impact on the patient, the patient's family, and the society
Diabetes insipidus which may lead to dehydration, electrolyte imbalance, organ dysfunction and death
Short stature
Infertility
Osteoporosis and an increased risk of fractures

Prognosis

Prognosis depends on the underlying cause. Without treatment, there is no improvement in the condition. Radiation and surgical removal of tumors may aid in treatment. Hormone replacement for the hormones that are underproduced as well as diet and exercise are important in the management of the condition^[6].

References

↑ Babinski-fröhlich syndrome. Bissonnette B, & Luginbuehl I, & Marciniak B, & Dalens B.J.(Eds.), (2006). Syndromes: Rapid Recognition and Perioperative Implications. McGraw Hill. https://accessanesthesiology.mhmedical.com/content.aspx?bookid=852&sectionid=49517244
↑ Wan MJ, Zapotocky M, Bouffet E, Bartels U, Kulkarni AV, Drake JM (2018). "Long-term visual outcomes of craniopharyngioma in children". J Neurooncol. 137 (3): 645–651. doi:10.1007/s11060-018-2762-3. PMID 29344823.
↑ Repka MX, Miller NR, Miller M (1989). "Visual outcome after surgical removal of craniopharyngiomas". Ophthalmology. 96 (2): 195–9. doi:10.1016/s0161-6420(89)32914-9. PMID 2704538.
↑ Babinski-fröhlich syndrome. Bissonnette B, & Luginbuehl I, & Marciniak B, & Dalens B.J.(Eds.), (2006). Syndromes: Rapid Recognition and Perioperative Implications. McGraw Hill. https://accessanesthesiology.mhmedical.com/content.aspx?bookid=852&sectionid=49517244
↑ Sanchez Jimenez JG, De Jesus O. Hypothalamic Dysfunction. [Updated 2021 Aug 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-
↑ Sanchez Jimenez JG, De Jesus O. Hypothalamic Dysfunction. [Updated 2021 Aug 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-

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[1] Babinski-fröhlich syndrome. Bissonnette B, & Luginbuehl I, & Marciniak B, & Dalens B.J.(Eds.), (2006). Syndromes: Rapid Recognition and Perioperative Implications. McGraw Hill. https://accessanesthesiology.mhmedical.com/content.aspx?bookid=852&sectionid=49517244

[pmid29344823-2] Wan MJ, Zapotocky M, Bouffet E, Bartels U, Kulkarni AV, Drake JM (2018). "Long-term visual outcomes of craniopharyngioma in children". J Neurooncol. 137 (3): 645–651. doi:10.1007/s11060-018-2762-3. PMID 29344823.

[pmid2704538-3] Repka MX, Miller NR, Miller M (1989). "Visual outcome after surgical removal of craniopharyngiomas". Ophthalmology. 96 (2): 195–9. doi:10.1016/s0161-6420(89)32914-9. PMID 2704538.

[4] Babinski-fröhlich syndrome. Bissonnette B, & Luginbuehl I, & Marciniak B, & Dalens B.J.(Eds.), (2006). Syndromes: Rapid Recognition and Perioperative Implications. McGraw Hill. https://accessanesthesiology.mhmedical.com/content.aspx?bookid=852&sectionid=49517244

[5] Sanchez Jimenez JG, De Jesus O. Hypothalamic Dysfunction. [Updated 2021 Aug 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-

[6] Sanchez Jimenez JG, De Jesus O. Hypothalamic Dysfunction. [Updated 2021 Aug 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-

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Adiposogenital dystrophy natural history, complications and prognosis