- Management of children with primary and secondary hypogammaglobulinemia (literature review)
Management of children with primary and secondary hypogammaglobulinemia (literature review)
Modern pediatrics. Ukraine. 2019.5(101):123-132; doi 10.15574/SP.2019.101.123
T.A. Marunchyn, A.P. Volokha
Shupyk National Medical Academy of Postgraduate Education, Kyiv, Ukraine
For citation: Marunchyn TA, Volokha AP. (2019). Management of children with primary and secondary hypogammaglobulinemia (literature review). Modern Pediatrics.Ukraine. 5(101): 123-132. doi 10.15574/SP.2019.101.123
Article received: Apr 23, 2019. Accepted for publication: Sep 12, 2019.
Primary hypogammaglobulinemiae are the most frequent forms of primary immunodeficiency, count more than 50% of congenital pathology of the immune system. Secondary hypogammaglobulinemia occur as a reason of hematological malignancies, intake of immunosuppressive or anticonvulsant drugs, exudative enteropathy, nephrotic syndrome. The prevalence of secondary hypogammaglobulinemia is 30 times higher according to primary hypogammaglobulinemia. The overall therapeutic goals of immunoglobulin replacement therapy are to reduce morbidity and mortality and to provide a patient with the best quality of life. There is a need for clinical research to evaluate the efficacy of immunoglobulin replacement therapy in children with primary hypogammaglobulinemia and to provide clear recommendations for the prevention of complications of recurrent infections, in particular bronchiectasis. The clinical and immunological indicators of hypogammaglobulinemia that develop in patients with nephrotic syndrome and intestinal lymphangioectasia have not been sufficiently studied. Clear guidelines and protocols for the management of children with secondary hypogammaglobulinemia are lacking in Ukraine.
No conflict of interest was declared by the authors.
Key words: primary and secondary hypohammaglobulinemia, antibody deficiency, infectious syndrome, bronchiectasis, children.
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