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Hypothalamic syndrome
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DOI：10.1038/s41572-022-00351-z.png)
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摘要
En 中文
Hypothalamic syndrome (HS) is a rare disorder caused by disease-related and/or treatment-related injury to the hypothalamus, most commonly associated with rare, non-cancerous parasellar masses, such as craniopharyngiomas, germ cell tumours, gliomas, cysts of Rathke's pouch and Langerhans cell histiocytosis, as well as with genetic neurodevelopmental syndromes, such as Prader-Willi syndrome and septo-optic dysplasia. HS is characterized by intractable weight gain associated with severe morbid obesity, multiple endocrine abnormalities and memory impairment, attention deficit and reduced impulse control as well as increased risk of cardiovascular and metabolic disorders. Currently, there is no cure for this condition but treatments for general obesity are often used in patients with HS, including surgery, medication and counselling. However, these are mostly ineffective and no medications that are specifically approved for the treatment of HS are available. Specific challenges in HS are because the syndrome represents an adverse effect of different diseases, and that diagnostic criteria, aetiology, pathogenesis and management of HS are not completely defined. Hypothalamic syndrome is a rare disorder caused by injury to the hypothalamus from various diseases or their treatment. This Primer provides an overview of the epidemiology, pathophysiology, diagnosis and management of the syndrome, as well as the challenges in preventing hypothalamic injury and treating its sequelae.
Keyword:
PRADER-WILLI-SYNDROME
QUALITY-OF-LIFE
GROWTH-HORMONE THERAPY
LANGERHANS CELL HISTIOCYTOSIS
CENTRAL-NERVOUS-SYSTEM
SEPTO-OPTIC DYSPLASIA
INCREASED DAYTIME SLEEPINESS
AXIS TOPOGRAPHICAL MODEL
SIXTO OBRADOR AWARD.
LONG-TERM SURVIVORS
期刊
N
IF:
60.6
论文数:
650
被引数:
3.8W
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