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. 1971 Oct;46(249):665–670. doi: 10.1136/adc.46.249.665

IgA deficiency in children

A clinical study with special reference to intestinal findings

E Savilahti, P Pelkonen, J K Visakorpi
PMCID: PMC1647824  PMID: 5118054

Abstract

Clinical, immunological, and intestinal studies on 26 children with IgA deficiency in the age range 2 to 16 years are reported. 9 of these children were suffering from autoimmune disease, namely thyroiditis (5), thyrotoxicosis (1), rheumatoid arthritis (2), and probable Sjögren's syndrome (1). The last-mentioned patient had defective cellular immunity. Altogether 11 patients were subject to recurrent respiratory tract infections. The symptomatology of the remaining patients was variable. In a boy with growth retardation, a chromosome anomaly was found, and endocrinological studies indicated total absence of growth hormone.

In 21 patients IgA was undetectable, while 5 had trace amounts of IgA in their sera. IgG was raised in 11 patients, and one patient had low serum IgG. IgM levels were mostly normal. Precipitating antibodies to cow's milk proteins were present in all but one serum.

Small intestinal biopsy was performed on all patients. In 3 cases total villous atrophy was detected and these probably had coeliac disease, though malabsorption symptoms were not always evident. Disaccharidase assay of biopsy specimens revealed 2 cases of isolated lactase deficiency among 8 tested.

Results show that the increased incidence of autoimmune disease reported in IgA deficiency in adults also holds true in children; i.e. that there is a raised incidence of coeliac disease with or without symptoms in IgA deficiency.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ammann A. J., Hong R. Selective IgA deficiency and autoimmunity. Clin Exp Immunol. 1970 Dec;7(6):833–838. [PMC free article] [PubMed] [Google Scholar]
  2. BACHMANN R., LAURELL C. B., SVENONIUS E. STUDIES ON THE SERUM GAMMA 1A-GLOBULIN LEVEL. II. GAMMA 1A-DEFICIENCY IN A CASE OF SYSTEMIC LUPUS ERYTHEMATOSUS. Scand J Clin Lab Invest. 1965;17:46–50. doi: 10.3109/00365516509077282. [DOI] [PubMed] [Google Scholar]
  3. Bachmann R. Studies on the serum gamma-A-globulin level. 3. The frequency of A-gamma-A-globulinemia. Scand J Clin Lab Invest. 1965;17(4):316–320. doi: 10.3109/00365516509077057. [DOI] [PubMed] [Google Scholar]
  4. Buckley R. H., Dees S. C. Correlation of milk precipitins with IgA deficiency. N Engl J Med. 1969 Aug 28;281(9):465–469. doi: 10.1056/NEJM196908282810903. [DOI] [PubMed] [Google Scholar]
  5. CROWLE A. J. A simplified micro double-diffusion agar precipitin technique. J Lab Clin Med. 1958 Nov;52(5):784–787. [PubMed] [Google Scholar]
  6. Cassidy J. T., Burt A., Petty R., Sullivan D. Selective IgA deficiency in connective tissue diseases. N Engl J Med. 1969 Jan 30;280(5):275–275. doi: 10.1056/nejm196901302800520. [DOI] [PubMed] [Google Scholar]
  7. Claman H. N., Merrill D. A., Peakman D., Robinson A. Isolated severe gamma A deficiency: immunoglobulin levels, clinical disorders, and chromosome studies. J Lab Clin Med. 1970 Feb;75(2):307–315. [PubMed] [Google Scholar]
  8. Crabbé P. A., Heremans J. F. Lack of gamma A-immunoglobulin in serum of patients with steatorrhoea. Gut. 1966 Apr;7(2):119–127. doi: 10.1136/gut.7.2.119. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Crabbé P. A., Heremans J. F. Selective IgA deficiency with steatorrhea. A new syndrome. Am J Med. 1967 Feb;42(2):319–326. doi: 10.1016/0002-9343(67)90031-9. [DOI] [PubMed] [Google Scholar]
  10. Dubois R. S., Roy C. C., Fulginiti V. A., Merrill D. A., Murray R. L. Disaccharidase deficiency in children with immunologic deficits. J Pediatr. 1970 Mar;76(3):377–385. doi: 10.1016/s0022-3476(70)80476-0. [DOI] [PubMed] [Google Scholar]
  11. Finley S. C., Cooper M. D., Finley W. H., Uchida I. A., Noto T. A., Roddam R. F. Immunological profile in a chromosome 18 deletion syndrome with IgA deficiency. J Med Genet. 1969 Dec;6(4):388–393. doi: 10.1136/jmg.6.4.388. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Gelzayd E. A., McCleery J. L., Melnykh C. S., Kraft S. C. Intestinal malabsorption deficiency. Arch Intern Med. 1971 Jan;127(1):141–147. [PubMed] [Google Scholar]
  13. Goldberg L. S., Barnett E. V., Fudenberg H. H. Selective absence of IgA: a family study. J Lab Clin Med. 1968 Aug;72(2):204–212. [PubMed] [Google Scholar]
  14. Hermans P. E., Huizenga K. A., Hoffman H. N., Brown A. L., Jr, Markowitz H. Dysgammaglobulinemia associated with nodular lymphoid hyperplasia of the small intestine. Am J Med. 1966 Jan;40(1):78–89. doi: 10.1016/0002-9343(66)90189-6. [DOI] [PubMed] [Google Scholar]
  15. Hobbs J. R. Immune imbalance in dysgammaglobulinaemia type IV. Lancet. 1968 Jan 20;1(7534):110–114. doi: 10.1016/s0140-6736(68)92721-9. [DOI] [PubMed] [Google Scholar]
  16. Huntley C. C., Robbins J. B., Lyerly A. D., Buckley R. H. Characterization of precipitating antibodies to ruminant serum and milk proteins in humans with selective IgA deficiency. N Engl J Med. 1971 Jan 7;284(1):7–10. doi: 10.1056/NEJM197101072840102. [DOI] [PubMed] [Google Scholar]
  17. Huntley C. C., Thorpe D. P., Lyerly A. D., Kelsey W. M. Rheumatoid arthritis with IgA deficiency. Am J Dis Child. 1967 Apr;113(4):411–418. doi: 10.1001/archpedi.1967.02090190057002. [DOI] [PubMed] [Google Scholar]
  18. Immonen P. Levels of the serum immunoglobulins gamma-A, gamma-G and gamma-M in the malabsorption syndrome in children. Ann Paediatr Fenn. 1967;13(4):115–153. [PubMed] [Google Scholar]
  19. LAUNIALA K., PERHEENTUPA J., VISAKORPI J., HALLMAN N. DISACCHARIDASES OF INTESTINAL MUCOSA IN A PATIENT WITH SUCROSE INTOLERANCE. Pediatrics. 1964 Nov;34:615–620. [PubMed] [Google Scholar]
  20. Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
  21. ROCKEY J. H., HANSON L. A., HEREMANS J. F., KUNKEL H. G. BETA-2A AGLOBULINEMIA IN TWO HEALTHY MEN. J Lab Clin Med. 1964 Feb;63:205–212. [PubMed] [Google Scholar]
  22. ROITT I. M., DONIACH D. Human autoimmune thyroiditis: serological studies. Lancet. 1958 Nov 15;2(7055):1027–1033. doi: 10.1016/s0140-6736(58)90530-0. [DOI] [PubMed] [Google Scholar]
  23. Rowe D. S., Anderson S. G., Grab B. A research standard for human serum immunoglobulins IgG, IgA and IgM. Bull World Health Organ. 1970;42(4):535–552. [PMC free article] [PubMed] [Google Scholar]
  24. Schlegel R. J., Bernier G. M., Bellanti J. A., Maybee D. A., Osborne G. B., Stewart J. L., Pearlman D. S., Ouelette J., Biehusen F. C. Severe candidiasis associated with thymic dysplasia, IgA deficiency, and plasma antilyMPHOCYTE EFFECTS. Pediatrics. 1970 Jun;45(6):926–936. [PubMed] [Google Scholar]
  25. South M. A., Copper M. D., Wollheim F. A., Good R. A. The IgA system. II. The clinical significance of IgA deficiency: studies in patients with agammaglobulinemia and ataxia-telangiectasia. Am J Med. 1968 Feb;44(2):168–178. doi: 10.1016/0002-9343(68)90148-4. [DOI] [PubMed] [Google Scholar]
  26. Stewart J. M., Go S., Ellis E., Robinson A. Absent IgA and deletions of chromosome 18. J Med Genet. 1970 Mar;7(1):11–19. doi: 10.1136/jmg.7.1.11. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Visakorpi J. K., Kuitunen P., Pelkonen P. Intestinal malabsorption: a clinical study of 22 children over 2 years of age. Acta Paediatr Scand. 1970 May;59(3):273–280. doi: 10.1111/j.1651-2227.1970.tb09003.x. [DOI] [PubMed] [Google Scholar]

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