Pituitary dwarfism - Causes, Symptoms, Diagnosis and Treatment

The term dwarfism (microsomes Nanosomes, dwarfism) broadly indicate a number of pathological conditions, expressed in backlog growth and physical development compared to the average age, sex, and racial population norm. Dwarfism may be independent genetic disease or a symptom of a number of endocrine and not endocrine diseases.

There are dwarfism with proportional and non-proportional physique. The group of diseases with proportional physique include pituitary dwarfism; myxedema (thyrogenous) dwarfism; adrenal dwarfism when adrenogenital syndrome; dwarfism, associated with damage to the thymus gland; infantile dwarfism, developing under the influence of exogenous factors (nutritional deficiency or toxicity); dwarfism associated with precocious puberty and early closure of growth zones. Dwarfism disproportionate constitution represented by the following forms: rachitic, hondrodistroficheskoy, dwarfism with osteogenesis imperfecta (brittle bones congenital). From cases to be distinguished family dwarfism (constitutional) form of stunting, which are regarded as a variant of physical development of the individual and not a pathology.

Pituitary dwarfism - a clinical syndrome caused by an absolute or relative pituitary growth hormone deficiency (lack of growth hormone secretion or impaired tissue sensitivity to it). Pituitary dwarfism occurs in a population at a frequency of 1:15000 to 1: 20000 slightly more among men.

STG) acquired giposomatotropnym state and peripheral tissue resistance to the action of growth hormone.

Congenital deficiency of growth hormone may be associated with genetic defects (mutations in the gene of growth hormone receptor gene mutation to somatoliberin et al.) Or developmental abnormalities of the hypothalamic-pituitary system (anencephaly, a congenital aplasia, hypoplasia or pituitary ectopia).

Acquired pituitary dwarfism develops in tumors of the hypothalamic-pituitary region (craniopharyngioma, hamartoma, neurofibroma, germinomas, pituitary adenoma) or other tumors of the brain (such as the optic chiasm glioma). In some cases, pituitary dwarfism could cause head injuries, birth injuries, neural infections (viral, bacterial encephalitis and meningitis), autoimmune pituitary damage to the pituitary stalk in surgical interventions, hydrocephalus, aneurysms pituitary blood vessels and so on. D. The pituitary dwarfism may be associated with the toxic effects of chemotherapy or radiation (leukemia, retinoblastoma, bone marrow transplant, etc d.).

Pituitary dwarfism due to the insensitivity of the target tissue to growth hormone deficiency can develop during growth hormone receptor, no biological activity of growth hormone, resistance to insulin like growth factor 1.

Along with isolated growth hormone deficiency, with pituitary dwarfism can be marked decrease in the synthesis of other pituitary hormones, gonadotropins, TSH, ACTH, which is accompanied by lack of relevant functions of peripheral endocrine glands (sex, thyroid, adrenal glands), which also affects the growth and physical development. In this case, endocrinology say pangipopituitarnoy form of dwarfism.

dry, wrinkled, with a yellowish tint or marble, which is caused by thyroid insufficiency. Hair color is often subtle, dry, brittle. Underdevelopment of the facial bones of the skull leads to the presence of small facial features ( "doll face"), retraction of the nose.

The distribution of subcutaneous fat may be different - from underdevelopment to excess fat deposits on "Cushingoid" type (chest, abdomen, thighs). There skeletal ossification delay, the weak development of the muscular system, violation of the change of teeth. Due to the underdevelopment of the larynx in adults with pituitary dwarfism children remains high tone of voice. In accordance with the short stature and body mass observed decrease in the size of internal organs (splanhnomikriya). Often, the pituitary dwarfism detected bradycardia and hypotension.

Violation of gonadotropic pituitary function causes a delay and inadequacy of sexual development (hypogonadism). In boys with pituitary dwarfism there is a lack of body hair of male type, testicular hypoplasia, cryptorchidism, micropenis; girls - amenorrhea, mikromastiya, hypoplasia of the uterus and ovaries.

Intellectual development with pituitary dwarfism are usually stored. May include the psychological characteristics such as negativism, isolation, self-reduction, infantile behavior.

If there are additional complaints of headache, vomiting, visual disturbances should be suspected intracranial pathology. When pangipopituitarnom dwarfism defined attributes of the secondary hypocorticoidism and hypothyroidism.

insulin, thyrotropin-releasing hormone, arginine, etc. rises slightly.

Radiography sella reveal his children's form ( "stand-up oval") and the general (ie. N. Juvenile) back. By increasing the size of the sella turcica, and the presence of calcification sites primarily to think about a tumor. X-ray of the wrist and hand to determine the radiological ( "bone") age. In pituitary dwarfism has been a significant slowdown in the processes of ossification of the skeleton.

To determine the form of dwarfism is necessary to study insulin-like growth factor (somatomedin-C), TSH, LH, FSH, T3 T4 cortisol in plasma and urine, antibodies to thyroglobulin, microsomal fraction thyrocytes and others. To eliminate the tumor lesion is performed CT (MRI) brain.

In pituitary dwarfism patient should be consulted an endocrinologist, gynecologist (women), andrology (male), a neurologist or neurosurgeon, ophthalmologist, geneticist.

pituitary tumors demonstrated their surgical removal, sometimes - radiotherapy.

Prediction and prevention of pituitary dwarfism



In pituitary dwarfism, caused by genetic factors, prognosis is good. Timely and rational HRT allows patients to be sufficiently physically developed, socially active and capable. When choosing a profession is not recommended to work, providing increased physical and neuro-psychological stress. In the absence of appropriate treatment for patients remains small stature and physical underdevelopment. In the case of organic brain lesions prognosis is determined by the underlying disease and its dynamics of development. Patients with pituitary dwarfism are on life-long dispensary endocrinologist and have to visit it every 2-3 months.

Prevention of pituitary dwarfism is the prevention of infections, physical and mental trauma in pregnant women; preventing birth defects, neuroinfections, head injury, intoxication in children.