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Types of primary immunodeficiency states

A.V. Zhestkov 1, O.S. Kozlova 1, M.O. Zolotov 1, V.V. Kulagina 2

1 Samara State Medical University, Ministry of Health of Russia, Samara, Russia
2 Private institution educational organization of higher education “Мedical University Reaviz”, Samara, Russia

ALLERGOLOGY AND IMMUNOLOGY IN PEDIATRICS, Volume 54 • Number 3 • September 2018, pp. 40 – 46
DOI: 10.24411/2500-1175-2018-00015

Abstract

Research objective: To study clinical and anamnestic signs used for early diagnosis of primary immunodeficiency conditions (PID).
Materials and methods. We have studied the case histories of 29 patients diagnosed with PID. The following data were evaluated: the occurrence of different PID groups, the manifestation of the disease, the presence of pathology in the antenatal period, and the frequency of occurrence of these alarming signs of PID in these patients, as recommended by the Society of Primary Immunodeficiencies.
Results. The lag in growth and physical development was revealed in 54% of patients, repeated deep abscesses of the skin and internal organs, as well as two or more episodes of severe generalized infection – in 46% of patients. Frequent otitis, sinusitis and thrush occurred in only 31% of cases, two or more pneumonia a year – in 28% of patients, the presence of FID in family members – in 10% of cases. Lymphadenopathy was detected in 62% of patients, hepatosplenomegaly in 59% of patients, anemia in 52% of patients and instability of stool in 39% of patients.
The conclusion. PID refers to rare diseases with a diverse clinical picture. The delay in diagnosis is an average of 9.6 years. The late age of diagnosis is indicative of PID hypodiagnosis. Not always the early and only sign of PID is the infectious syndrome. Such non-specific signs as lymphadenopathy, hepatosplenomegaly and anemia are quite common and may become additional criteria in the early diagnosis of PID.

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For citation

Zhestkov AV, Kozlova OS, Zolotov MO, Kulagina VV. Types of primary immunodeficiency states. Allergology and Immunology in Pediatrics. 2018;54(3):40-46. (In Russ.) https://doi.org/10.24411/2500-1175-2018-00015

For correspondense

A.V. Zhestkov
email: avzhestkov2015@yandex.ru.

cellular immune deficiency humoral immune deficiency immunopathological syndroms innate immunity system рrimary immunodeficiency conditions
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