The pituitary gl& is called the master endocrine gland of the body because it controls the function of other endocrine organs. The anterior pituitary produces the hormones thyrotropin (thyroid-stimulating hormone
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Panhypopituitarism is a condition of inadequate or absent production of the anterior pituitary hormones. It is frequently the result of other problems that affect the pituitary gland và either reduce or destroy its function or interfere with hypothalamic secretion of the varying pituitary-releasing hormones. Panhypopituitarism can be the kết thúc result of various clinical scenarquả táo. The signs và symptoms are diverse. Manifestations that suggest congenital anterior hypopituitarism include micropenis, midline defects, optic atrophy, hypoglycemia, & poor growth. <1, 2, 3>
Diagnosis & management
The diagnostic evaluation of a child with hypopituitarism is divided inkhổng lồ 2 portions: recognition of the hormonal deficiencies và determination of the cause. Genetic testing is available at various commercial và academic laboratories for mutations associated with hypopituitarism.
Cortisol deficiency requires prompt recognition và treatment. This is particularly true for the child who may be facing surgery or experiencing other significant stresses related to lớn the cause of hypopituitarism.
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In TSH deficiency, the dose of L-thyroxine replacement is age dependent. In cases of gonadotropin deficiency, sex steroid replacement should begin at puberty, while in GH deficiency, GH replacement doses should be administered in doses of 0.18-0.3 mg/kg/wk subcutaneously, divided in 6-7 doses. Higher doses of up to lớn 0.7 mg/kg/wk may be beneficial in puberty.
Surgical treatment should be used for operable pituitary và hypothalamic tumors.
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The effects of hypopituitarism in children depend on the affected hormones. GH deficiency can result in hypoglycemia và short stature. Gonadotropin deficiency leads lớn prenatal micropenis and delayed or interrupted puberty in older children. Corticotropin deficiency interferes with normal carbohydrate, protein, & lipid metabolism and may result in weight loss, hypoglycemia, fatigue, hypotension, & death. Thyrotropin deficiency leads khổng lồ hypothyroidism.
Hypopituitarism is caused by various conditions and is associated with various hormonal deficiencies. Thus, data are limited regarding frequency rates of the various etiologies và components. An Italian study reported GH deficiency prevalence to be approximately 9 cases per 1000 individuals in a pediatric population. <4> Data from the Northwest Regional Screening program estimate the frequency of congenital TSH deficiency at 1 case per 29,000 live sầu births. <5>
A study by Kao et al indicated that adults with childhood-onphối multiple pituitary hormone deficiencies (COMPHD) have a significantly worse chất lượng of life than vì healthy adults. Compared with healthy controls, adults with COMPHD were shorter và more overweight, had less education, were less likely lớn be married, và had a higher unemployment rate, lower incomes, & fewer children. They also scored lower on the Female Sexual Function Index & Male Sexual Quotient. <6>
Setian N, Aquiar CH, Galvao JA. Rathke"s cleft cyst as a cause of growth hooc môn deficiency và micropenis. Child"s Nervous System. 1999. Vol 5: 271-3.
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