Ectodermal and fibrous dysplasia. Kidney Dysplasia

Under dysplasia (translated from Greek, as dys - negation, wplasis - education) implies the wrong and deforming development of an organ. This is a collective concept, covering any cases of violations.

Fibrous dysplasia refers to diseases of the osseous system and is characterized by the fact that the bone tissue is replaced by a fibrous tissue. This process deforms the bones. The main cause of this disease is the appearance of a tumor-like process, which begins in the child, and then begins to progress. Fibrous dysplasia usually occurs in women.

There are several classifications of the disease. The most common is the unit, depending on the level of the pathological process:

• Mono-osseous fibrous dysplasia affects only one bone;
• Polyosseous - changes are observed in several bones (usually spread on one side of the body and often occurs in childhood).

The first form of the disease affects the cranial bones, scapula, tubular bones (long), ribs. The second is simultaneously more than fifty percent of the bones of the entire skeleton.

To the main complications can be attributed frequent fractures, which quickly grow together, but increased deformation in the bones, as well as sarcoma.

Fibrous dysplasia does not give any treatment, only orthopedic procedures (bone grafting, curettage, osteotomy) are possible, as well as the appointment of drugs that relieve pain.

Ectodermal dysplasia covers a large number of syndromes and diseases (about 100), the essence of which lies in the presence of abnormal development of ectoderm derivatives. The first sign is the presence of hyperthermia, which, if expressed and frequent attacks, can lead to either a disruption of intellectual development or a fatal outcome.

The following types and their symptoms can be distinguished.

Anhydratic begins to manifest in the baby-boy and lasts a lifetime. The main features are:

• Hereditary nature of the disease;
• Severe deformation of teeth;
• The presence of a slow pace of nail development;
• Atrophy of the mucous membrane (throat, mouth, nose);
• The appearance of the child is similar to a patient with congenital syphilis.

Treatment is appointed simpomatic. Forbidden: rest in the southern countries, work in hot and chemical shops.

Hydraulic exo-dermal dysplasia is characterized by the presence of changes in the palms, soles, and hair development. As a treatment, vitamin A is usually prescribed externally and inward, softening the cream and keratolytic. In addition, enzymatic agents, methyluracil, anabolic steroids, zinc, amino acids and biostimulants are used.

Kidney dysplasia has several types that differ:

• On morphological manifestations (cystic and simple);
• Localization (cortico-medullary, medullary and cortical);
• By prevalence - total, segmental, focal).

The main causes of this disease are genetic factors and teratogenic external and internal effects on the embryo. Therefore, the disease is based on congenital malformations, which, regardless of the species, lead to a decrease in the kidney in size, a vicious development of the parenchyma, a significant decrease in its functions.

Treatment of this disease is symptomatic and often there are cases when there is an indication for transplantation of this organ.

In general, dysplasia combines a group of diseases that imply the incorrect development of a particular organ or whole system. As a rule, the main causes are genetic and congenital factors.

Treatment is symptomatic or there are recommendations for organ or bone transplantation.