Percent of cases[1-4] | Typical age of onset◊[2,4] | |
Hypothalamic endocrine manifestations | ||
Early- and rapid-onset obesity | 100 | 3 to 7 years |
Deceleration of growth rate | 38 | 5 to 14 years |
Failed growth hormone stimulation test | 62 | |
Adrenal insufficiency | 29 | |
Cushing's syndrome | 5 | |
Hypernatremia* | 62 | 2 to 12 years |
Diabetes insipidus | 24 | 2 to 12 years |
Hyperprolactinemia | 52 | 4 to 12 years |
Central hypothyroidism | 52 | |
Hypogonadotropic hypogonadism | 43 | |
Precocious puberty | 10 | |
Respiratory manifestations¶ | 100 | |
Cardiorespiratory arrest | 57 | 3 to 8 years |
Autonomic dysregulationΔ | 52 | 3 to 13 years |
Tumors of neural crest origin | 40-56 | 3 to 7 years |
Adrenal ganglioneuroma | 29 | |
Neurobehavioral disorders | 48 | Median 4.8 years |
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