婴儿仍在母亲子宫内时发生的一种疾病。 IUGR 产生的足月婴儿在出生时体重和/或身长异常小。

Several hormones are involved in regulating growth. Some act directly on target organs, while others act by triggering the production of other hormones, which activate specific organ functions necessary for growth. This finely tuned system can malfunction in several ways, causing abnormal growth.

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垂体通常被称为主腺，因为它会产生多种激素来控制其他腺体的功能。它位于称为下丘脑的大脑部分下方的颅骨中部。脑垂体有两个不同的部分：前（前）叶和后（后）叶。垂体分泌激素以响应来自下丘脑的化学信息，下丘脑是与其相连的大脑部分。

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Growth hormone is an anterior pituitary hormone whose main effect is to promote the growth of body tissues. Other anterior pituitary hormones affect growth indirectly by working through other glands.

 

These other hormones include:

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Thyroid Stimulating Hormone (TSH) – causes the thyroid gland to produce thyroid hormone, which regulates body metabolism and is essential for normal growth.

Adrenocorticotropic Hormone (ACTH) – causes the adrenal glands to produce cortisol (stress hormone) and other hormones that enable the body to respond to stress. Too much cortisol will cause growth failure in a child.

Luteinizing Hormone (LH) and Follicle Stimulating Hormone (FSH) – cause the sex glands (ovaries or testes) to produce sex hormones, which are necessary for adolescent sexual development and the growth spurt that accompanies puberty.
The major hormone produced by the posterior pituitary gland -is called vasopressin, or anti-diuretic hormone (ADH). It controls water output through the kidneys.

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CAUSES OF GROWTH HORMONE DEFICIENCY

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Growth hormone deficiency may occur by itself or in combination with one or more other pituitary hormone deficiencies. It may be total (no growth hormone is produced) or partial (some growth hormone is produced, but not enough to support normal growth).

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Hypopituitarism may be congenital, resulting from abnormal formation of the pituitary or hypothalamus before the child is born, or acquired, stemming from damage to the pituitary or hypothalamus during or after birth. Congenital hypopituitarism is present at birth, although it may not be apparent for many months. Acquired hypopituitarism may become evident any time during infancy or childhood, and may occur after a severe head injury or a serious illness such as meningitis or encephalitis. Many cases of acquired hypopituitarism result from a tumor called craniopharyngioma. This tumor may press on the hypothalamus or pituitary, causing one or more hormone deficiencies. Deficiency consists of surgical removal of the tumor, which usually results in permanent hypopituitarism.

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有时无法确定垂体机能减退的原因，或者 - 如果怀疑有原因，则可能难以证明。研究人员正试图更多地了解生长激素缺乏症和垂体功能减退症的原因。

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生长激素缺乏症的诊断

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生长激素缺乏症的孩子通常个子小，面容不成熟，身材胖乎乎的。所有身体部位的生长速度都很慢，因此孩子的比例保持正常。智力正常。如果孩子的身高已经绘制在生长图表上，它看起来会趋于平稳并偏离孩子既定的生长曲线。如果长期存在生长障碍，孩子可能比同龄的其他孩子矮很多。这就是为什么绘制在生长图表上的身高和体重测量值如此重要——越早发现可治疗的生长问题，孩子就越有可能在整个童年时期保持正常身高并充分发挥其成长潜力。

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任何身高仅与小两岁或两岁以上的孩子一样高或偏离先前正常生长曲线的孩子都应由医生进行评估。儿科内分泌学家是专门治疗患有生长和激素问题的儿童的医生。根据情况，医生可能会在 6 到 12 个月的时间内对孩子进行测量，以准确确定孩子的生长速度。

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评估首先收集有关亲属身高和家庭中是否存在任何健康问题的信息。应提及家庭成员的早熟或晚熟史（性发育和生长突增）。医生会想知道母亲的怀孕、分娩和分娩情况。应该收集孩子从出生开始的所有身高和体重测量值，以便医生可以将它们绘制在生长图表上。医生会询问有关孩子的一般健康状况和营养状况、过去的疾病、受伤和压力的问题。

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将进行彻底的身体检查，并可能获得手和手腕的 X 光片，以了解骨骼发育与身高和实足年龄的比较情况。可能会抽取少量血液以寻找甲状腺激素缺乏和肾脏、骨骼和胃肠道（胃和肠）疾病的证据。可以测量血液中胰岛素样生长激素-I (IGF-I) 的量。 IGF-I是生长过程中的“中间人”。生长激素刺激肝脏和其他身体组织产生 IGF-I，然后 IGF-I 充当血液中生长激素与细胞内导致生长的机制之间的联系。血液中 IGF-I 的量提供了对存在的生长激素量的间接测量。

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这种简单的评估通常可以为医生提供足够的信息来确定生长问题的原因或确定不存在生长问题。如果医生怀疑可能存在垂体问题，则需要进一步检查。一系列的血液测试可以测量血液中激素的浓度和垂体对各种刺激的反应能力。这些测试可以在诊所或在短暂的住院期间进行。

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生长激素缺乏症很难诊断，因为脑垂体会爆发性地产生生长激素。这意味着单个随机血液样本中的生长激素水平可能非常低。测试生长激素缺乏症的一种方法是给孩子服用一种物质，这种物质会导致正常儿童突然释放生长激素，并测量在一段时间内获得的几个血液样本中存在的生长激素量。由于任何孩子都可能不会在某一天对任何给定的测试做出反应，因此可能需要不止一种刺激来评估孩子产生生长激素的能力。已经确定了几种生长激素刺激剂。这些包括剧烈运动和几种化学物质和药物（胰岛素、精氨酸、胰高血糖素、左旋多巴、可乐定）。

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另一种测试生长激素分泌的方法包括让孩子住院并测量在睡眠期间甚至整个 24 小时期间获得的血液样本中存在的生长激素量。由于大约三分之二的总生长激素产生发生在深度睡眠期间，因此该测试可以更好地反映孩子的脑下垂体正常产生多少生长激素。

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如果几项测试表明不存在生长激素或产生的生长激素量不足以支持正常生长，则可以确定生长激素缺乏症的诊断。正在进行大量研究以开发更准确和可靠的诊断生长激素缺乏症的方法。随着研究人员更多地了解可能导致部分生长激素缺乏症的情况，甚至生长激素缺乏症的定义也在被修订。

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生长激素缺乏症的治疗

生长激素缺乏症可通过注射生长激素来治疗。大多数儿童每天都接受注射；其他人每周收到六次；少数人每周接受 3 次治疗。* 治疗开始后，生长速度通常会迅速增加，这可能会在 3 到 4 个月后被孩子和父母注意到。这种比正常情况更快的生长速度会随着时间的推移而缓慢下降，但它仍然比没有治疗时发生的速度要快。许多父母注意到孩子在治疗开始后食欲增加和体内脂肪减少。

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生长激素缺乏症的治疗通常需要数年时间，直到孩子达到可接受的成人身高或达到最大生长潜力。与其他情况一样，儿童和父母可能会不耐烦地希望看到更快或更令人印象深刻的治疗结果。他们可能会变得气馁，即使治疗正在按计划进行。重要的是要记住，增长是一个缓慢的过程，需要数月才能衡量；刚开始虚就指望一夜长高的孩子，会失望的。您孩子的医生将与您讨论现实的短期和长期治疗期望。

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如果检测发现其他激素缺乏，可用药物替代；当发现甲状腺激素、皮质醇和性激素缺乏时，可以很容易地给予。重要的是按照指示服用这些激素，因为只有当所有激素都以适当的量存在时才能发生正常生长。良好的营养和充足的休息对所有儿童的正常成长都很重要。

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SOURCES OF HUMAN GROWTH HORMONE

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Until recently, the only source of human growth hormone was the pituitary glands of deceased people, obtained at autopsy. In April, 1985, pituitary-derived growth hormone was removed from distribution in the United States and many foreign countries following the deaths of several young adults from a very rare viral disease that may have been transmitted through the pituitary growth hormone they had received many years earlier. Fortunately, the first biosynthetic growth hormone, which is produced using recombinant DNA technology, was in the- final stages of testing and was approved as safe and effective for use in growth hormone deficient children by the Food and Drug Administration in October, 1985. Because this type of growth hormone does not come from human beings, there is little possibility that human diseases can be transmitted through it.

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Biosynthetic growth hormone is supplied as a powder in sterile vials. Parents and children are taught how to mix the powder into a solution and administer the injections. Treatment is continued as long as potential for growth exists and the child is responding to therapy. With early diagnosis and a good response to treatment, children with growth hormone deficiency can expect to reach normal adult height.

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PSYCHOLOGICAL ASPECTS OF SHORT STATURE

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OIur society places great emphasis on height. Children who are short for their age sometimes have problems because playmates and teachers treat them as though they are younger rather than just smaller. Parents tend to do this too and decrease their expectations of the child. These children then may not act their age because it’s not expected of them. Teasing and name-calling may be hard to take. Some of these problems may be helped by a frank and open discussion with teachers and classmates.

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It is very important to provide emotional support for the child with GH deficiency and to emphasize the child’s many good and valuable characteristics, so that the child’s stature does not limit his horizons. More about psychosocial adaptation to short stature can be learned from parents of short children and from your growth clinic doctor, nurse and psychologist.

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HOPE FOR THE FUTURE

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Biosynthetic growth hormone is available in unlimited quantity for the Deficiency of all growth hormone deficient children. It is possible that substitutes for growth hormone may become available as research continues. These may include growth hormone releasing factor (GHRF), the hypothalamic chemical that directs the pituitary to produce growth hormone, and IGF-I that links growth hormone with linear growth.

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Much research is being done to better understand the causes of growth hormone deficiency, and to develop more accurate ways of diagnosing it. Many children with growth hormone deficiency can look forward to reaching normal height as a result of the research that has been done over the years and is continuing today.

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