Growth hormone deficiency

From a growth hormone deficiency ( hyposomatotropism , English growth hormone deficiency (GHD)) occurs when the pituitary growth hormone formed (pituitary) (Growth Hormone or GH, and Somatropin or somatotropic hormone or STH called) is paid insufficient. As a result, growth retarded in children and adolescents . Because somatropin also has an effect on the protein and energy balance, reduced muscle mass and increased fat deposits can be observed at the same time, and patients tend to have low blood sugar levels . This applies to both children and adults. The administration of somatropin normalizes growth and metabolism . Somatropin works in the body through insulin-like growth factors (IGF).

Spread and causes

Somatropin deficiency occurs in 1 in 3,000 to 5,000 children. It can be isolated or combined with other hormonal failures, as the pituitary gland produces several hormones.

The causes of a deficiency are very complex and can be inherited or acquired:

• inherited:
  • genetic malformation of the secretory cells
  • genetic changes in the structure of the hormone (e.g. Kowarski syndrome )
  • Receptor defects (e.g. Laron syndrome )
• acquired:
  • Birth complications
  • Injury to the pituitary gland / hypothalamus as a result of trauma
  • Shock syndromes
  • psychosocial aspects
  • Brain tumors (Lorain dwarfism, no longer commonly used name)

Of idiopathic hormone deficiency occurs when the cause can not be determined. Somatropin deficiency often occurs in combination with other hypothalamic / pituitary hormone disorders:

• Hypothyroidism (lack of thyroid hormones)
• Overactive adrenal cortex ( Cushing's syndrome )
• Deficiency of antidiuretic hormone (also known as ADH or adiuretin)

Somatropin deficiency (hypothalamic / pituitary) can be severe or mild. In the case of hypothalamus disorder, somatropin can be formed, but not released regularly, leading to so-called neurosecretory dysfunction (functional disturbance of the release). If the cause is pituitary (anterior pituitary insufficiency), one speaks of pituitary dwarfism or Nanosomia pituitaria (English pituitary dwarfism ).

Disease emergence

If there is no somatropin, the “carrier hormone” IGF-1 (insulin-like growth factor 1) cannot be formed in the organism. IGF-1 promotes cell division in the cartilage layers of the growth plates . Growth hormone also plays a role in the division of cells in the skin, in the release of fatty acids from adipose tissue and in increasing blood sugar.

Clinical manifestations

Slowed growth, which leads to short stature, is the main symptom. Children with a somatropin deficiency are usually proportionately short with a delayed bone age . They have small hands and feet, and usually have a doll-like face. The muscles are poorly developed and the skin is very thin. Adults with a growth hormone deficiency since childhood often only grow to be 120-130 cm tall. In the absence of puberty, growth in the absence of growth plate closure can also lead to final size in the lower normal range.

A microdontia can produce clinical symptoms.

Investigation methods

If the preliminary diagnosis reveals a justified suspicion of a hormone deficiency, two stimulation tests are usually carried out to dynamically determine the somatropin level and to check the other hormone axes. In most children's endocrinological centers, these are the arginine test and the insulin tolerance test , and in some cases also the clonidine test or the glucagon test . Very rarely, a nocturnal somatropin profile is also measured in the blood to rule out a slight impairment of hormone secretion (so-called neurosecretory dysfunction). In addition, a magnetic resonance tomographic examination of the pituitary gland is required if the deficiency is proven .

pathology

A destruction of the pituitary gland can be caused by tumors (mostly craniopharyngiomas ), the destruction or destruction of growth hormone-producing cells in the anterior lobe of the pituitary gland due to a lack of oxygen, for example during birth ( breeches ) or inflammation in the brain area. A disruption in the area of ​​the hypothalamus, which is responsible for regulating hormone release in the pituitary gland, can also be a cause.

treatment

The treatment is carried out with genetically engineered somatropin, which is identical to the human hormone. It must be administered daily. So far it is technically not possible to manufacture somatropin in tablet or drop form. The treatment usually lasts for several years and ends with the closure of the growth plates, i.e. the completion of the body's growth.

However, we now know that humans produce growth hormone not only as children but also in adulthood. The amount gradually decreases in the course of life; a physiological deficiency situation only occurs at an advanced age. Somatropin not only plays an important role in height growth and development in childhood, but also as a metabolic hormone in adulthood. In the meantime it has been clearly proven that even in adults with growth hormone deficiency, somatropin therapy has a positive effect on many functions (bone stability, muscle strength, cardiovascular function, general vitality). In the event of a proven hormone deficiency, which was confirmed again at the end of the therapy of a child or adolescent, the treatment can also be continued in adulthood.

prevention

It is not possible to prevent the occurrence of a somatropin deficiency. Only if the breech is known should the pregnancy be terminated by a caesarean section , which can prevent an insufficient supply of oxygen to the brain.

Prospect of healing

The hormone deficiency cannot be cured. If a tumor is found to be the cause, a neurosurgical procedure is required. The consequences of the deficiency can be remedied or mitigated by hormone therapy .

literature

• O. Butenandt: Etiology of hypothalamo-pituitary short stature (hypopituitarism) . In: Social Pediatrics 12 . 1990, p. 168-173 .

• O. Butenandt, MB Range: Final height after growth hormone therapy. Pediatric Endocrinology: Past and Future . Ed .: MB Ranke, JR Bierich. MD-Verlag, Munich 1986, p. 36 .

• H. Cushing: The pituitary body and is dissorders . Lippincot, Philadelphia 1912.

• K. Kruse: Pediatric Endocrinology . Georg Thieme Verlag, Stuttgart / New York 1999.

• Z. Laron, O. Butenandt: Optimum use of growth hormone in children . In: Drugs 42 . 1991, p. 1-8 .

• MS Raben: Treatment of a piutuitary dwarf with human growth hormone . In: J. Clin Endocrinol 18 . 1958, p. 883 .

Individual evidence

1. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
2. ↑ Ludwig Weissbecker: The anterior pituitary insufficiency (hypopituitarism). In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition, ibid. 1961, pp. 1001-1004, here: p. 1003.

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