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Growth hormone deficiency

Growth hormone deficiency is the medical condition of inadequate production of growth hormone (GH) and its effects on children and adults. Growth hormone, also called somatotropin, is a polypeptide hormone which stimulates growth and cell reproduction. See separate articles on GH physiology and GH treatment. more...

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Deficiency of GH produces significantly different problems at various ages. In newborn infants the primary manifestations may be hypoglycemia or micropenis. In later infancy and childhood, growth failure may be major effect. Adults with growth hormone deficiency may have diminished lean body mass and poor bone density and a number of physical and psychological symptoms.

GH deficiency can be congenital or acquired in childhood or adult life. It can be partial or complete. It is usually permanent, but sometimes transient. It may be an isolated deficiency or occur in association with deficiencies of other pituitary hormones.

GH deficiency is treated by growth hormone replacement.

Terminology

The term hypopituitarism is often used interchangeably with GH deficiency by endocrinologists but more often denotes GH deficiency plus deficiency of at least one other anterior pituitary hormone. When GH deficiency (usually with other anterior pituitary deficiencies) is associated with posterior pituitary hormone deficiency (usually diabetes insipidus) the condition is termed panhypopituitarism.

HGH also refers to human growth hormone but this older abbreviation has begun to develop paradoxical connotations (see fuller discussion of HGH in GH treatment and HGH quackery).

Causes of GH deficiency

There are many causes of GH deficiency. Some examples include:

  • mutations of specific genes (e.g., GHRHR, GH1)
  • congenital malformations involving the pituitary (e.g., septo-optic dysplasia, posterior pituitary ectopia)
  • damage to the pituitary from incracranial disease (e.g., hydrocephalus),
  • intracranial tumors in or near the sella turcica, especially craniopharyngioma,
  • damage to the pituitary from radiation therapy to the head for leukemia or brain tumors,
  • surgery in the area of the pituitary,
  • autoimmune inflammation (hypophysitis),
  • severe head trauma,
  • ischemic or hemorrhagic infarction from low blood pressure (Sheehan syndrome) or hemorrhage pituitary apoplexy.

Many cases of isolated growth hormone deficiency (IGHD) recognized in childhood are idiopathic. IGHD has been reported to affect about 1 in 4000 children, but IGHD is difficult to distinguish from other causes of shortness such as constitutional delay, and the true incidence is unsettled.

Severe prenatal deficiency of GH, as occurs in congenital hypopituitarism, has little effect on fetal growth. However, prenatal and congenital deficiency can reduce the size of a male's penis, especially when gonadotropins are also deficient. Besides micropenis, additional consequences of severe deficiency in the first days of life can include hypoglycemia and exaggerated jaundice (both direct and indirect hyperbilirubinemia). Female infants will lack the microphallus of course but may suffer from hypoglycemia and jaundice.

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Growth hormone deficiency - Tips From Other Journals
From American Family Physician, 8/1/91

Growth delay affects not only the biologic development of a child, but is also typically associated with significant psychosocial and educational problems. Stabler and colleagues review the psychosocial and educational aspects of growth hormone deficiency in children and present a comprehensive approach to the management of children with this disorder.

Children with growth hormone deficiency are considered to be at risk of developmental stress psychologically, educationally and socially. The social behavior of children with growth hormone deficiency has been frequently described as immature, reflecting low self-esteem and depression. Their problem solving abilities are often less than their peers. Twenty to 40 percent of children with growth hormone deficiency are held back at least one grade in elementary school, compared with 4 to 5 percent of normal children. Children with growth hormone deficiency are at risk for marked academic deficiencies and behavioral problems, and require a more specific education prescription than simply repeating a school year.

Treatment of growth hormone deficiency should include recognition of the social, psychologic and educational issues commonly faced by the patients. Ideally, a multidisciplinary team of health professionals should perform a comprehensive evaluation of intelligence, academic achievement and general psychologic function. The primary care physician should explain the medical condition, treatment protocol and prognosis to the patient and parents while providing anticipatory guidance. Important strategies include carefully explaining the medical condition and helping the family to maintain realistic treatment expectations. Pharmacologic treatment consists of long-term growth hormone replacement therapy.

The treatment focus should not be limited to adding additional inches in height. A specific final height should not be guaranteed. Rather, treatment goals should be to increase growth velocity, to keep the child's stature from becoming even more discrepant from peers and to address the psychologic vulnerability of these children.

The etiology of the short stature should be discussed with the parents and child so that misinformation or guilt can be confronted. Concerns about the long-term side effects of medication and methods to facilitate compliance should be specifically addressed. To reduce apprehension about injections of growth hormone, various methods to reduce the pain of injection should be discussed. Readiness for school entry, discussion of the pros and cons of grade retention, comprehensive psychometric assessment by a pediatric psychologist and family counseling may also be useful.

Parents should be encouraged to treat their child according to chronologic age, to discourage and prohibit the use of pejorative nicknames associated with short stature and to encourage participation in sports, such as swimming or skiing, which are not dependent on size. Referral to support organizations, such as the Human Growth Foundation, may also be valuable. (Clinical Pediatrics, March 1991, vol. 30, p. 156.)

COPYRIGHT 1991 American Academy of Family Physicians
COPYRIGHT 2004 Gale Group

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