Thymus gland aplasia
Contents:
Description:
The aplasia of a thymus gland is a group of diseases caused by genetic defects of immune system.
Thymus gland Aplasia symptoms:
1. Di-George's syndrome. Along with an aplasia of gland the aplasia of epithelial bodies with manifestations of a hypoparathyrosis is possible. In a pathogeny deficit of the circulating T lymphocytes, sharp oppression of reaction of cellular immunity, relative increase in number of V-lymphocytes and preservation of reaction of humoral immunity takes place (normal level of immunoglobulins in blood, a hypocalcemia).
Characteristic symptoms of a disease are spasms, since the neonatal period, recurrent infections of respiratory and digestive systems. It is usually combined with anomalies of development of an aortic arch, mandible, ear lobes, with a hypoplasia of lymph nodes and an underdevelopment of timuszavisimy zones.
2. Nezelof's syndrome — an autosomal and recessive aplasia of a thymus gland with a lymphopenia, without aplasia of parathyroids, but with an underdevelopment of timuszavisimy zones in lymph nodes and a spleen.
Also sharp decrease in reactivity of T lymphocytes (deficit of cellular system of immunity) comes to light.
Since the neonatal period recurrent bronchitis, pneumonia, coloenterites of a virus or fungal etiology, herpetic rashes, sepsis are noted. Deficit of T lymphocytes and oppression of reaction of cellular immunity are more expressed, than at Di-George's syndrome. Patients perish at early age.
3. Louis's syndrome — Bara — immunological insufficiency at an ataxy teleangiectasia, is characterized by autosomal and recessive inheritance of an aplasia of gland, proceeds with reduction of lymphocytes in timuszavisimy zones of lymph nodes and a spleen, demyelination in a cerebellum.
Multisystem difficult frustration:
1) neurologic (ataxy, lack of coordination etc.);
2) vascular (teleaniektaziya of skin and conjunctiva);
3) mental (mental retardation);
4) endocrine (disturbance of functions of adrenal glands, gonads). Since the early childhood recurrent sinuso-pulmonic infections develop.
Disturbance of cellular immunity is followed by defeat of T - and V-systems of immunity, deficit of IgA In blood serum oonaruzhivatsya emorionalny oyelka (α-and β-fetoproteins)))))))))). At such patients malignant new growths develop more often (more often lymphosarcomas, a lymphogranulomatosis).
4. "The Swiss syndrome" — the autosomal and recessive heavy combined immunological insufficiency. The Limfopenichesky agammaglobulinemia, an aplasia or a hypoplasia of a thymus gland are combined with a hypoplasia of all adenoid tissue. Sharp hypoplasia of a thymus, hypoplasia of lymph nodes and lymphoid formations of a spleen, intestines.
Since the neonatal period recurrent fungal, viral and bacterial damages of skin and mucous membranes of a nasopharynx, respiratory tracts, intestines. At such children it is difficult to identify a thymus.
Along with sharp oppression of reactions of cellular immunity deficit of humoral immunity comes to light (deficit of T - and V-lymphocytes). Children usually perish in the first half a year of life.
Thymus gland Aplasia reasons:
This group of diseases is caused by genetic defects of immune system.
Inborn, or primary, the aplasia (or a hypoplasia) a thymus is characterized by total absence of a timichesky parenchyma or its extremely poor development that defines existence of the heavy combined immunological nedostatochknost owing to sharp reduction of maintenance of T - both V-lymphocytes and lack of little bodies of a thymus.
All these diseases are followed by recurrent inflammatory diseases, is more often than pulmonary or intestinal localization which quite often are a proximate cause of death of patients. Therefore the children, especially early age having recurrent inflammatory diseases have to be carefully inspected on a functional condition of a thymus gland.
Similar changes find at children at a number of the diseases united in group of immunodeficient. The most expressed defects of development of a thymus are found at the following syndromes.
Treatment of the Aplasia of a thymus gland:
Recovery and replaceable immunotherapy. Transplantation of a thymus or marrow, administration of immunoglobulins, thymus hormones is for this purpose carried out. Use of the corticosteroids possessing an immunodepressive effect, contraindicated.
