» Articles » PMID: 6978347

Primary Immunodeficiency Disorders in Sweden: Cases Among Children, 1974-1979

Overview
Journal J Clin Immunol
Publisher Springer
Date 1982 Apr 1
PMID 6978347
Citations 42
Authors
Affiliations
Soon will be listed here.
Abstract

A nationwide survey of symptomatic primary immunodeficiency disorders in children in Sweden during the 6-year period 1974-1979 resulted in 201 reported cases. The reported data for 174 children were analyzed. Antibody deficiencies were the most frequent (45.0%), followed by phagocytic disorders (22.0%) and combined T- and B-cell disorders (20.8%). Thirty-two children (18.4%) died, with the highest mortality for combined T- and B-cell defects. The sex ratio for all disorders was 2:1 for boys:girls. Neutropenia was significantly more common in the northern part of Sweden.

Citing Articles

Autoimmunity in Wiskott-Aldrich Syndrome: Updated Perspectives.

Sudhakar M, Rikhi R, Loganathan S, Suri D, Singh S Appl Clin Genet. 2021; 14:363-388.

PMID: 34447261 PMC: 8384432. DOI: 10.2147/TACG.S213920.


The Importance of Primary Immune Deficiency Registries: The United States Immunodeficiency Network Registry.

Lopes J, Cunningham-Rundles C Immunol Allergy Clin North Am. 2020; 40(3):385-402.

PMID: 32654688 PMC: 8183626. DOI: 10.1016/j.iac.2020.03.002.


Characteristics of the patients followed with the diagnosis of common variable immunodeficiency and the complications.

Erdem S, Gulez N, Genel F, Karaman S, Nacaroglu H Cent Eur J Immunol. 2019; 44(2):119-126.

PMID: 31530980 PMC: 6745547. DOI: 10.5114/ceji.2019.87060.


Primary immunodeficiency in infection-prone children in southern Sweden: occurrence, clinical characteristics and immunological findings.

Brodszki N, Jonsson G, Skattum L, Truedsson L BMC Immunol. 2014; 15:31.

PMID: 25318568 PMC: 4159572. DOI: 10.1186/s12865-014-0031-6.


Prevalence and morbidity of primary immunodeficiency diseases, United States 2001-2007.

Kobrynski L, Powell R, Bowen S J Clin Immunol. 2014; 34(8):954-61.

PMID: 25257253 PMC: 4820073. DOI: 10.1007/s10875-014-0102-8.


References
1.
Spector B, Perry 3rd G, Kersey J . Genetically determined immunodeficiency diseases (GDID) and malignancy: report from the immunodeficiency--cancer registry. Clin Immunol Immunopathol. 1978; 11(1):12-29. DOI: 10.1016/0090-1229(78)90200-3. View

2.
Ammann A, Hong R . Selective IgA deficiency: presentation of 30 cases and a review of the literature. Medicine (Baltimore). 1971; 50(3):223-36. View

3.
Conley M, Beckwith J, Mancer J, TENCKHOFF L . The spectrum of the DiGeorge syndrome. J Pediatr. 1979; 94(6):883-90. DOI: 10.1016/s0022-3476(79)80207-3. View

4.
Kostman R . Infantile genetic agranulocytosis. A review with presentation of ten new cases. Acta Paediatr Scand. 1975; 64(2):362-8. DOI: 10.1111/j.1651-2227.1975.tb03847.x. View

5.
Oxelius V . Chronic infections in a family with hereditary deficiency of IgG2 and IgG4. Clin Exp Immunol. 1974; 17(1):19-27. PMC: 1554046. View