Gigantism is a pathological tallness caused by excessive production of growth hormone (somatotropic hormone) by the anterior pituitary gland and manifested already in childhood. There is an increase in height over 2 m, a disproportionality of the physique with a predominant elongation of the limbs, while the head seems very small. Patients have a disorder of the physical and mental state, sexual function. With this disease, the ability to work is limited, the risk of infertility is high. The main diagnostic criterion of disease, in addition to a vivid clinical picture, is the detection of an increase in STH in the blood.
Meaning
Gigantism (or macrosomia) develops in children with incomplete processes of skeletal ossification, is more common in male adolescents, is determined already at the age of 9-13 years and progresses throughout the entire period of physiological growth. With gigantism, the child’s growth rate and its indicators far exceed the anatomical and physiological norm and by the end of puberty reach more than 1.9 m in women and 2 m in men while maintaining a relatively proportional physique. The frequency of gigantism is from 1 to 3 cases per 1000 population.
Parents of patients suffering from this pathology are usually of normal height. Pathology should be differentiated from hereditary tallness.
Classification
Modern endocrinology identifies the following variants of the development of gigantism:
- acromegaly – with signs of acromegaly;
- splanchnomegaly – accompanied by an increase in the size and mass of internal organs;
- eunuchoid – gigantism in patients with hypogonadism (hypofunction or complete cessation of the functions of the genital glands), manifested by disproportionately elongated limbs, open growth zones in the joints, the absence of secondary sexual characteristics;
- true – characterized by a proportional increase in body size and the absence of deviations from physiological and mental functions;
- partial or partial – with an increase in individual parts of the body;
- half – accompanied by an increase in one half of the trunk;
- cerebral – caused by organic brain damage and accompanied by impaired intelligence.
Causes
Gigantism and acromegaly are two age-related variations of the same pathological processes: hyperplasia and hyperfunction of pituitary cells producing somatotropic hormone (growth hormone). Increased secretion of growth hormone can be observed in pituitary lesions as a result of tumors of the gland (pituitary adenoma), intoxication, neuroinfections (encephalitis, meningitis, meningoencephalitis), traumatic brain injuries.
Often the cause of gigantism is a decrease in the sensitivity of epiphyseal cartilage, which ensures the growth of bones in length, to the effects of sex hormones. As a result, the bones retain the ability to increase in length for a long time, even in the post-puberty period. Hypersecretion of somatotropin after the closure of bone growth zones and the completion of ossification of the skeleton leads to acromegaly.
Symptoms of gigantism
A jump in the increase in body length with gigantism occurs in 10-15 years. Patients are characterized by high growth and the rate of its increase, complain of rapid fatigue and weakness, dizziness, headaches, visual impairment, pain in joints and bones. A decrease in memory and performance leads to a deterioration in academic performance at school. Gigantism is characterized by hormonal disorders, disorders of mental and sexual function (infantilism). Women with gigantism develop primary amenorrhea or early termination of menstrual function, infertility, men – hypogonadism. Other hormonal manifestations of gigantism are diabetes insipidus, hypo- or hyperthyroidism, diabetes mellitus. There is first an increase in muscle strength, and then muscle weakness and asthenia.
With gigantism, it is possible to develop arterial hypertension, myocardiodystrophy, heart failure, emphysema of the lungs, dystrophic liver changes, infertility, diabetes mellitus, thyroid disorders.
Diagnostics
The diagnosis of gigantism is established on the basis of an external examination of the patient, the results of laboratory, radiological, neurological and ophthalmological studies. With gigantism, a high content of STH in the blood is determined in the laboratory.
To detect pituitary tumors, skull x-ray, CT and brain MRI are performed. In the presence of pituitary adenoma is determined. Radiographs of the hands show a discrepancy between bone and passport age. Ophthalmological examination of patients with gigantism reveals a limitation of visual fields, stagnation in the fundus.
Treatment
The principles of treatment of gigantism are similar to the treatment of acromegaly. To normalize the level of growth hormone in gigantism, somatostatin analogues are used, and sex hormones are used for faster closure of bone growth zones. Etiological treatment of gigantism in pituitary adenomas involves the use of radiation therapy or their surgical removal in combination with drug support by dopamine agonists.
With the eunuchoid type of gigantism, treatment is aimed at eliminating sexual infantilism, accelerating ossification of the skeleton and stopping its further growth. The treatment of partial gigantism includes orthopedic correction with the help of plastic surgery. The combined treatment of gigantism used by endocrinologists combines hormonal and radiation therapy and allows achieving positive results in a significant number of patients.
Prognosis and prevention
With adequate therapy of gigantism, the prognosis for life is relatively favorable. However, many patients do not live to old age and die from complications of the disease. Most patients suffering from gigantism are infertile, their ability to work is sharply reduced.
Parents should be concerned about a sharp, significant increase in the growth of a child during puberty compared to his peers. Timely medical intervention will prevent complications.