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Partial IgA deficiency

MedGen UID:
868762
•Concept ID:
C4023167
•
Finding
HPO: HP:0011837

Definition

Detectable but decreased IgA levels that are more than 2 standard deviations below normal age-adjusted means. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPartial IgA deficiency

Recent clinical studies

Etiology

Respiratory and allergic disorders in children with severe and partial immunoglobulin A immunodeficiency.
Živković J, Lipej M, Banić I, Bulat Lokas S, Nogalo B, Lulić Jurjević R, Turkalj M
Scand J Immunol 2019 Dec;90(6):e12828. Epub 2019 Oct 24 doi: 10.1111/sji.12828. PMID: 31520490
Follow-up and outcome of symptomatic partial or absolute IgA deficiency in children.
Moschese V, Chini L, Graziani S, Sgrulletti M, Gallo V, Di Matteo G, Ferrari S, Di Cesare S, Cirillo E, Pession A, Pignata C, Specchia F
Eur J Pediatr 2019 Jan;178(1):51-60. Epub 2018 Sep 29 doi: 10.1007/s00431-018-3248-1. PMID: 30269248
Children with partial IgA deficiency: clinical characteristics observed in the pediatric rheumatology clinic.
Shakkottai A, Bupathi K, Patel AP, Chalom E, Chamarthi S, Lehman TJ, Peterson MG, Gaur S, Moorthy LN
Clin Pediatr (Phila) 2012 Jan;51(1):46-50. Epub 2011 Aug 25 doi: 10.1177/0009922811417287. PMID: 21868598
Selective IgA deficiency in early life: association to infections and allergic diseases during childhood.
Janzi M, Kull I, Sjöberg R, Wan J, Melén E, Bayat N, Ostblom E, Pan-Hammarström Q, Nilsson P, Hammarström L
Clin Immunol 2009 Oct;133(1):78-85. Epub 2009 Jun 21 doi: 10.1016/j.clim.2009.05.014. PMID: 19541543
Autoimmune paediatric liver disease.
Mieli-Vergani G, Vergani D
World J Gastroenterol 2008 Jun 7;14(21):3360-7. doi: 10.3748/wjg.14.3360. PMID: 18528933Free PMC Article

Diagnosis

The Prevalence of Selective and Partial Immunoglobulin A Deficiency in Patients with Autoimmune Polyendocrinopathy.
Jamee M, Alaei MR, Mesdaghi M, Noorian S, Moosavian M, Dolatshahi E, Taghavi Kojidi H, Chavoshzadeh Z, Fallahi M, Parviz S, Aghamahdi F, Azizi G
Immunol Invest 2022 May;51(4):778-786. Epub 2021 Jan 12 doi: 10.1080/08820139.2021.1872615. PMID: 33432864
Respiratory and allergic disorders in children with severe and partial immunoglobulin A immunodeficiency.
Živković J, Lipej M, Banić I, Bulat Lokas S, Nogalo B, Lulić Jurjević R, Turkalj M
Scand J Immunol 2019 Dec;90(6):e12828. Epub 2019 Oct 24 doi: 10.1111/sji.12828. PMID: 31520490
Follow-up and outcome of symptomatic partial or absolute IgA deficiency in children.
Moschese V, Chini L, Graziani S, Sgrulletti M, Gallo V, Di Matteo G, Ferrari S, Di Cesare S, Cirillo E, Pession A, Pignata C, Specchia F
Eur J Pediatr 2019 Jan;178(1):51-60. Epub 2018 Sep 29 doi: 10.1007/s00431-018-3248-1. PMID: 30269248
Children with partial IgA deficiency: clinical characteristics observed in the pediatric rheumatology clinic.
Shakkottai A, Bupathi K, Patel AP, Chalom E, Chamarthi S, Lehman TJ, Peterson MG, Gaur S, Moorthy LN
Clin Pediatr (Phila) 2012 Jan;51(1):46-50. Epub 2011 Aug 25 doi: 10.1177/0009922811417287. PMID: 21868598
Autoimmune paediatric liver disease.
Mieli-Vergani G, Vergani D
World J Gastroenterol 2008 Jun 7;14(21):3360-7. doi: 10.3748/wjg.14.3360. PMID: 18528933Free PMC Article

Therapy

Autoimmune paediatric liver disease.
Mieli-Vergani G, Vergani D
World J Gastroenterol 2008 Jun 7;14(21):3360-7. doi: 10.3748/wjg.14.3360. PMID: 18528933Free PMC Article
Effects of sulfasalazine treatment on serum immunoglobulin levels in children with juvenile chronic arthritis.
van Rossum MA, Fiselier TJ, Franssen MJ, ten Cate R, van Suijlekom-Smit LW, Wulffraat NM, van Luijk WH, Oostveen JC, Kuis W, Dijkmans BA, van Soesbergen RM; Dutch Juvenile Chronic Arthritis Study group
Scand J Rheumatol 2001;30(1):25-30. doi: 10.1080/030097401750065283. PMID: 11252688
Follow-up of pediatric patients with recurrent infection and mild serologic immune abnormalities.
Herrod HG
Ann Allergy Asthma Immunol 1997 Nov;79(5):460-4. doi: 10.1016/S1081-1206(10)63044-X. PMID: 9396982
Neutrophil and lymphocyte locomotion in patients with selective IgA deficiency.
Coşkun Y, Sanal O, Ersoy F
Turk J Pediatr 1990 Apr-Jun;32(2):79-83. PMID: 2091374

Prognosis

Follow-up and outcome of symptomatic partial or absolute IgA deficiency in children.
Moschese V, Chini L, Graziani S, Sgrulletti M, Gallo V, Di Matteo G, Ferrari S, Di Cesare S, Cirillo E, Pession A, Pignata C, Specchia F
Eur J Pediatr 2019 Jan;178(1):51-60. Epub 2018 Sep 29 doi: 10.1007/s00431-018-3248-1. PMID: 30269248
Bronchial hyperreactivity in children with antibody deficiencies.
Özcan C, Metin A, Erkoçoğlu M, Kocabas CN
Allergol Immunopathol (Madr) 2015 Jan-Feb;43(1):57-61. Epub 2014 Jan 30 doi: 10.1016/j.aller.2013.09.014. PMID: 24485938
Selective IgA deficiency in early life: association to infections and allergic diseases during childhood.
Janzi M, Kull I, Sjöberg R, Wan J, Melén E, Bayat N, Ostblom E, Pan-Hammarström Q, Nilsson P, Hammarström L
Clin Immunol 2009 Oct;133(1):78-85. Epub 2009 Jun 21 doi: 10.1016/j.clim.2009.05.014. PMID: 19541543
Autoimmune paediatric liver disease.
Mieli-Vergani G, Vergani D
World J Gastroenterol 2008 Jun 7;14(21):3360-7. doi: 10.3748/wjg.14.3360. PMID: 18528933Free PMC Article
Clinical findings and intestinal immunoglobulins in children with partial IgA deficiency.
Savilahti E, Pelkonen P
Acta Paediatr Scand 1979 Jul;68(4):513-9. doi: 10.1111/j.1651-2227.1979.tb05049.x. PMID: 463533

Clinical prediction guides

The Prevalence of Selective and Partial Immunoglobulin A Deficiency in Patients with Autoimmune Polyendocrinopathy.
Jamee M, Alaei MR, Mesdaghi M, Noorian S, Moosavian M, Dolatshahi E, Taghavi Kojidi H, Chavoshzadeh Z, Fallahi M, Parviz S, Aghamahdi F, Azizi G
Immunol Invest 2022 May;51(4):778-786. Epub 2021 Jan 12 doi: 10.1080/08820139.2021.1872615. PMID: 33432864
Follow-up and outcome of symptomatic partial or absolute IgA deficiency in children.
Moschese V, Chini L, Graziani S, Sgrulletti M, Gallo V, Di Matteo G, Ferrari S, Di Cesare S, Cirillo E, Pession A, Pignata C, Specchia F
Eur J Pediatr 2019 Jan;178(1):51-60. Epub 2018 Sep 29 doi: 10.1007/s00431-018-3248-1. PMID: 30269248
B-cell subsets in patients with transient hypogammaglobulinemia of infancy, partial IgA deficiency, and selective IgM deficiency.
Cipe FE, Doğu F, Güloğlu D, Aytekin C, Polat M, Biyikli Z, Ikincioğullari A
J Investig Allergol Clin Immunol 2013;23(2):94-100. PMID: 23654075
Children with partial IgA deficiency: clinical characteristics observed in the pediatric rheumatology clinic.
Shakkottai A, Bupathi K, Patel AP, Chalom E, Chamarthi S, Lehman TJ, Peterson MG, Gaur S, Moorthy LN
Clin Pediatr (Phila) 2012 Jan;51(1):46-50. Epub 2011 Aug 25 doi: 10.1177/0009922811417287. PMID: 21868598
Selective IgA deficiency in early life: association to infections and allergic diseases during childhood.
Janzi M, Kull I, Sjöberg R, Wan J, Melén E, Bayat N, Ostblom E, Pan-Hammarström Q, Nilsson P, Hammarström L
Clin Immunol 2009 Oct;133(1):78-85. Epub 2009 Jun 21 doi: 10.1016/j.clim.2009.05.014. PMID: 19541543

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