Neutropenia is a disease, characterized by an abnormally low number of neutrophils. Neutrophils usually make up 50-70% circulating white blood cells and serve as the primary protection against infection, destroying bacteria in blood. Thus, patients with neutropenia are more susceptible to bacterial infection.
Early childhood neutropenia occur often enough, and although in most cases the flow easily and are not subject to treatment, all require timely identification, spending differential diagnosis and determine the optimum tactics of patients.
Acute neutropenia (formed within hours or days) may develop as a result of rapid consumption, destruction or disturbance of production neitrofilov. Chronic neutropenia (for a period of months and years), usually, due to the decline in the formulation of cells or their excessive sekvestraciej in the spleen. Neutropenia can be classified as primary if there is an internal deficit of myeloid cells in the bone marrow or as secondary (due to the influence of external factors on kostnomozgovye myeloid cells).
Neutropenia can go unnoticed, but with the development of severe infections or sepsis patients, they manifest. Some common infections can take an unexpected stroke in patients with neutropenia (the formation of pus).
Some common symptoms include fever nejtropenij and frequent infections. These infections can lead to ulcers in the mouth, diarrhea symptoms, the feeling of burning sensation during urination, unusual redness, pain or swelling around the wound, sore throat.
Diagnostic tactics when identifying neutropenia in the child in early childhood may be next:
If any of these signs of no, то наиболее вероятный диагноз – ХДНДВ. Если в наличии хотя бы один – следует искать другие причины нейтропении.
The nature and scope of laboratory tests patient with granulocytopenia depends not so much on the severity of neutropenia, how much the frequency and severity of associated infections.
For patients with HDNDV the important point is the documentation of the duration of the neutropenia more 6 months, no other changes in gemogramme, as well as raising the level of neutrophils during intercurrent infections.
The minimum diagnostic program with isolated neutropenia also includes a determination of the level of immunoglobulins in the blood.
Bone marrow biopsy may be needed to exclude other diseases.
Define routinely in patients with HDNDV in the blood level of antinej-trofilnyh antibodies is not necessary, so as not to reveal their all. On the other hand, in case of secondary autoimmune neutropenia these tests, as the definition of other autoantibodies, should be conducted. Determination of antibody titer to NA1 and NA2 in child and maternal serum may be useful to confirm the diagnosis izoimmunnoj neutropenia.
Congenital nejtropenijah may require genetic study.
Management of patients of early age with HDNDV provides, Firstly, explanation of the essence of the problem parents, to avoid unnecessary anxiety on their part. Recommend to pay more attention to oral hygiene of the child to prevent stomatitis, Gingivitis. Preventive vaccinations are carried out according to the calendar, recommend also to inculcate children flu Additionaly, pneumococcal and meningococcal disease. In the vast majority of cases, HDNDV other measures not required.
Izvesny three severity of neutropenia based on the absolute number of neutrophils (ANC), measured in cells at the microliter of blood:
Treatment depends on the cause, on which it originated. Therefore, treat the infection, that led to the development of neutropenia. Depending on the severity and the form of the disease, doctor makes a decision on the treatment of neutropenia in the hospital or at home. The main emphasis of doing on the strengthening of the immune system. Of the medications used antibiotics, vitamins, medicines for strengthening immunity. When very severe patient placed in solitary confinement, where support sterility and spend ultraviolet irradiation.
There are many ways to, based on common sense, for, to reduce the risk of complications when passing treatment, eg:
Antibacterials are appointed only in identifying the child's focus of bacterial infection, and if neutropenia and fever without apparent infection.
With frequent relapses of bacterial infection offer to prevent trimetoprimom/sulfometaksazolom, However, the dose, course duration, efficacy and safety of this method has not been studied.
Frequent repeated infections, resistant to antibiotic therapy, а также определенные формы врожденных нейтропении – показания для применения Г-КСФ и внутривенных иммуноглобулинов.
Glucocorticoids are able to raise the level of neutrophils. However, the use of them when nejtropenijah can be justified only in the event of failure of all other methods, and, in General, is the exception rather, than the rule. Categorically not recommended to appoint glukokortikoida children with uncomplicated HDNDV with a view to adjusting the level of neutrophils.
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