Hospital pediatrics. Diagnosis of primary immunodeficiencies. Principles of therapy (most important)

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Hospital pediatrics. Diagnosis of primary immunodeficiencies. Principles of therapy (most important)

Lecture notes, cheat sheets

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2. Diagnosis of primary immunodeficiencies. Principles of therapy

Diagnosis of primary immunodeficiency states is as follows:

1) selection of children at risk of primary CHD (attention must be paid):

a) the pedigree of the child, indicating in it cases of death of children in the family at an early age from inflammatory diseases;

b) the development of vaccination, repeated, chronic, multifocal and unusual current infections, parasitic and fungal diseases;

c) the presence in the pedigree of autoimmune, allergic and tumor processes, hemopathy and pathology associated with sex;

d) the presence of associated syndromes (lagging behind in physical development, endocrinopathy, skin and neurological manifestations, etc.);

2) when assessing the immune system of children, it is necessary to take into account:

a) negative Mantoux tests after vaccination and BCG revaccination;

b) thymus dysplasia in young and middle-aged children;

c) the absence of an increase in regional lymph nodes in response to the inflammatory process;

d) hypoplasia of the tonsils or, on the contrary, a pronounced hypertrophy of the tonsil tissue and lymph nodes in combination with recurrent inflammatory processes;

3) assessment of routine laboratory tests:

a) detection in a clinical blood test: hemolytic or hypoplastic anemia, neutropenia, thrombocytopenia, absolute lymphopenia (less than 1000 in 1 mm3), absence of plasma cells in response to an acute infection - all this may indicate a deficiency syndrome in T- and B- systems. b) analysis of the proteinogram - detection of low-level hypoproteinemia and hypoalbuminemia allows us to roughly but reliably judge the state of certain immune functions;

4) identification of bright clinical non-immunological markers:

a) ataxia and bulbar telangiectasias - with Louis-Barr syndrome;

b) malformations of the main vessels and convulsions against the background of hypocalcemia - with Di George's syndrome.

Treatment. Principles of therapy for primary immunodeficiency conditions:

1) hospitalization for in-depth immunological and molecular studies and the choice of the method of therapy;

2) adequate replacement immunotherapy, which allows many patients to lead a normal life;

3) bone marrow transplantation - a radical and almost routine treatment for many forms of IDS;

4) refusal to consider a patient with congenital IDS as therapeutically unpromising.

Author: Pavlova N.V.

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