Open AccessSlovenski nacionalni register bolnikov s primarno imunsko pomanjkljivostjo
Author(s) -
Štefan Blazina,
Gašper Markelj,
Maruša Debeljak,
Anja Koren Jeverica,
Nataša Toplak,
Nevenka Bratanič,
Marko Pokorn,
Peter Kopač,
Andreja Pikelj Pečnik,
Mitja Košnik,
Janez Jazbec,
Marijana Kuhar,
Alojz Ihan,
Tadej Avčin
Publication year - 2016
Publication title -
zdravniški vestnik
Language(s) - English
Resource type - Journals
eISSN - 1581-0224
pISSN - 1318-0347
DOI - 10.6016/zdravvestn.1321
Subject(s) - medicine , primary immunodeficiency , clinical immunology , bloodstream infection , rheumatology , pediatrics , immunology , allergy , disease
Data from Slovene national primary immunodeficiency (PID) registry are presented. Besides clinical and genetic data of patients with PID, quality indicators in patient care are included. Data are systematically collected in Department of Allergology, Rheumatology and Clinical Immunology in collaboration with physicians of different specialities. Increasing number and spectrum of PID are recognised in Slovenia. After establishment of Slovene multidisciplinary group in PID care in 2007 thorough immunological and genetic diagnostics, subcutaneous immunoglobulin replacement and treatment of PID with haematopoietic stem cell transplantation were introduced in routine clinical practice in Slovenia. Increased medium age of PID patients reflects improved survival and better recognition of PID in adults. According to the data in comparable registries percentages of patients with predominantly antibody deficiencies and complement deficiencies are low and high, respectively.