This scientific work is devoted to improving the study of primary and secondary
hypogammaglobulinemias with the aim of increasing the efficacy of treatment of
children with this pathology, improving their quality of life, preventing the occurrence
of infectious diseases and their complications, as well as reducing mortality.
On the basis of a retrospective and prospective study of children with primary
hypogammaglobulinemia receiving intravenous immunoglobulin (IVIG) replacement
therapy an assessment of various treatment regimens was carried out, taking into
account the dosage of intravenous immunoglobulin and the regularity of the therapy, as
well as specified criteria for the efficacy of immunoglobulin replacement therapy in
children with primary hypogammaglobulinemia (mainly antibody immunodeficiencies),
and hypogammaglobulinemias associated with other primary immunodeficiencies. The
scientific value of the work lies in the fact that, for the first time the clinical and
immunological indicators of children with secondary hypogammaglobulinemia are
described and the appointment of IVIG replacement is scientifically justified. As a
result, the clinical characteristics are presented and the analysis of immunological
parameters in children with secondary hypogammaglobulinemias is carried out, the
tactics of dynamic monitoring of patients with secondary hypogammaglobulinemias is
determined, the timeliness of the correction of antibody deficiency in such patients and
the impact of various schemes of IVIG replacement therapy on the course of the disease
are evaluated. Also comparative characteristic of hypogammaglobulinemias depending
on the degree of severity in children with primary and secondary
hypogammaglobulinemia was provided.
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The efficacy of the treatment was evaluated by analyzing the level of serum
immunoglobulins, the frequency of infectious diseases, the use of antibiotic therapy,
and the number of hospitalizations before and after IVIG replacement therapy.
53 children with various forms of primary hypogammaglobulinemia were included
in this study. They were divided into groups: hereditary hypogammaglobulinemia (16
children), common variable immunodeficiency (CVID) (6 children), primary combined
immunodeficiency (4 children), transient hypogammaglobulinemia (10 children),
unspecified hypogammaglobulinemia (4 children), selective deficiency of
immunoglobulin IgG subclasses (3 children), other defined immunodeficiency
syndromes (6 children: one with Netherton syndrome, one with Louis-Bar syndrome
(ataxia telangiectasia), two with Nijmegen breakage syndrome, two with DiGeorge
syndrome). The study also included: one child with hyper-IgM syndrome, two children
with specific antibody deficiency (SAD), and one child with a defect in immune
regulation (X-linked lymphoproliferative disease (XLP), Duncan’s syndrome).
The average age of diagnosis of patients in the group of hereditary
hypogammaglobulinemia was 4.1 years, in the group of transient
hypogammaglobulinemia – 0.8 years, in the group of CVID – 10.1 years, in the group
of unspecified hypogammaglobulinemia – 3.7 years, in the group of other defined
primary immunodeficiencies – 0.8 years, in the group of primary combined
immunodeficiencies – 1 year, in the group of deficiency of IgG subclasses – 8.3 years,
in the group of SAD – 6.8 years. A child with hyper-IgM syndrome was diagnosed at
the age of 2 years, and a child with XLP was diagnosed with hypogammaglobulinemia
at the age of four.
So, among 53 children with primary hypogammaglobulinemia, 32% (17 children)
were infants, 26% (14 children) – of early childhood, 17% (9) – children of preschool
age, and 17% (9) – children of primary school age, older children – 8% (4 children). At
the same time, there were 34/64% boys, and 19/36% girls. As a result of X-linked
transmission only boys were observed in the group of children with hereditary
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hypogammaglobulinemia, hyper-IgM syndrome and X-linked lymphoproliferative
syndrome.
Among the infectious diseases most often in children with primary
hypogammaglobulinemia were infections of the upper (sinusitis) and lower respiratory
tract (pneumonia, bronchitis), bacterial diseases of the skin, its appendages and soft
tissues (pyoderma, paronychia, panaritium, furunculosis, abscess, phlegmon,
suppurative lymphadenitis, purulent mastitis), supurrative otitis, invasive bacterial
diseases (sepsis, meningitis, osteomyelitis). Infections of the oral cavity, infectious eye
diseases, infections of the urinary tract, chronic diarrhea, fungal diseases and
mycobacterial infections did not occur so often. Allergic (bronchial asthma, atopic
dermatitis, allergic rhinitis, erythroderma) and autoimmune diseases (autoimmune
hemolytic anemia, idiopathic thrombocytopenic purpura, autoimmune neutropenia,
juvenile idiopathic arthritis, hemorrhagic vasculitis) were also relevant for children of
this group.
