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Vol. 12. Issue 3.
Pages 293-302 (May - June 2006)
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Vol. 12. Issue 3.
Pages 293-302 (May - June 2006)
Caso Clínico\Clinical Case
Open Access
A propósito de um caso de imunodeficiência comum variável – Revisão das hipogamaglobulinemias
About a case of common variable immunodeficiency – Revision of hypogammaglobulinemias
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Alexandra Lopes*,***, Fernando Barata**
* Internato Complementar de Medicina Interna, Serviço de Medicina do Centro Hospitalar de Coimbra. Director: Dr. Ricardo Conceição
** Centro Hospitalar de Coimbra, Serviço de Pneumologia do Centro Hospitalar de Coimbra (Director: Dr. Rui Pato)
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Resumo

A imunodeficiência comum variável é uma imunodeficiência primária caracterizada por distúrbios imunológicos heterogéneos, de etiologia desconhecida. As suas manifestações clínicas incluem infecções recorrentes, doenças auto-imunes, hiperplasia linfóide, doenças granulomatosas e neoplasias; pode aparecer em doentes com deficiência de IgA. Os autores apresentam o caso clínico de doente com imunodeficiência comum variável e história de infecções respiratórias de repetição desde os 9 meses, associadas ao aparecimento de bronquiectasias.

Rev Port Pneumol 2006; XII (3): 293-301

Palavras-chave:
Imunodeficiência comum variável
Abstract

Common variable immunodeficiency is a primary immune deficiency characterized by heterogeneous immunologic disorders of unknown etiology. Its clinical manifestations include recurrent infections, autoimmune diseases, lymphoid hyperplasia, granulomatous diseases and malignancy. It can appear in patients with immunoglobulin A deficiency. The authors report the clinical case of a patient with common variable immunodeficiency and history of respiratory infections from the age of 9 months old, associated with the appearing of bronchiectasis.

Rev Port Pneumol 2006; XII (3): 293-301

Key-words:
Common variable immunodeficiency
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Bibliografia
[1.]
M.C. Sneller.
Common variable immunodeficiency.
Am J Med Sci, 321 (2001), pp. 42-48
[2.]
I. Vorechovsky, H. Zetterquist, R. Paganelli, et al.
Family and linkage study of selective IgA deficiency and common variable immunodeficiency.
Clin Immunol Immunopathol, 77 (1995), pp. 185-192
[3.]
T. Nijenhuis, I. Klasen, C.M. Weemaes, et al.
Common variable immunodeficiency (CVID) in a family: an autosomal dominant mode of inheritance.
Neth J Med, 59 (2001), pp. 134-139
[4.]
Primary immunodeficiency diseases. Report of a WHO scientific group.
Clin Exp Immunol, 109 (1997), pp. 1
[5.]
L. Hammarstrom, I. Vorechovsky, D. Webster.
Selective IgA deficiency (SigAD) and common variable immunodeficiency (CVID).
Clin Exp Immunol, 120 (2000), pp. 225
[6.]
R.A. Hermaszewski, A.D.B. Webster.
Primary hypogammaglobulinemia: A survey of clinical manifestations and complications.
Q J Med, 86 (1993), pp. 31
[7.]
C. Cunningham- Rundles, C. Bodian.
Common Variable immunodeficiency: clinical and immunological features of 248 patients.
Clin Immunol, 92 (1999), pp. 34-48
[8.]
G.P. Spickett, A.D.B. Webster, J. Farrant.
Cellular abnormalities in common variable immunodeficiency.
Imunodeficiencies, pp. 111-126
[9.]
M.E. Conley, C.L. Park, S.D. Douglas.
Childhood common variable immunodeficiency with autoimmune disease.
J Pediatr, 108 (1986), pp. 915-922
[10.]
C. Alvarez-Cuesta, L. Molinos, J.Á. Cascante, et al.
Cutaneous granulomas in a patient with common variable immunodeficiency.
Acta Derm Venereol, 79 (1999), pp. 334
[11.]
A.L. Krupnick, H. Shim, R.G. Phelps, et al.
Ctaneous granulomas masquerading as tuberculoid leprosy in a patient with congenital combined immunodeficiency.
Mt Sinai J Med, 68 (2001), pp. 326-330
[12.]
J.C. Pierson, C. Camisa, K.B. Lawlor, D.M. Elston.
Cutaneous and visceral granulomas in common variable immunodeficiency.
Cutis, 52 (1993), pp. 221-222
[13.]
E.M. Ziegler, L.M. Seung, K. Soltani, M.M. Medenica.
Cutaneous granulomas with two clinical presentations in a patient with common variable immunodeficiency.
J Am Acad Dermatol, 37 (1997), pp. 499-500
[14.]
C. Ariatti, D. Rossi, D. Vivenza, et al.
Molecular characterization of common variable immunodeficiency-related lymphomas.
Ann Ital Med Int, 16 (2001), pp. 163-169
[15.]
K.S. Elenitoba jonhnson, E.S. Jaffe.
Lymphoproliferative disorders associated with congenital immunodeficiencies.
Semin Diagn Pathol, 14 (1997), pp. 35-47
[16.]
M.L. Johnson, L.G. Keeton, Z.B. Zhu, et al.
Age-related changes in serum imunoglobulins in patients with familial IgA deficiency and common variable immunodeficiency (CVID).
Clin Exp Immunol, 108 (1997), pp. 477-483
[17.]
O. Olerup, Smith Cl, J. Bjorkander, Hammarstrom L. Shared.
HLA class II-associated genetic susceptibility and resistance, related to the HLA-DQB1 gene, in IgA deficiency and common variable immunodeficiency.
Proc Natl Acad Sci USA, 89 (1999), pp. 10653-10657
[18.]
M.G. Gutierrez, C.H. Kirkpatrick.
Progressive immunodeficiency in a patient with IgA deficiency.
Ann Allergy Asthma Immunol, 79 (1997), pp. 297
[19.]
J. Litzman, M. Burianova, V. Thon, J. Lokaj.
Progression of selective IgA deficiency to common variable immunodeficiency in a 16 year old boy.
Allergol Immunopathol, 24 (1996), pp. 174
[20.]
T. Espanol, M. Catala, M. Hernandez, et al.
Development of common variable immunodeficiency in IgA-deficient patients.
Clin Immunol Immunopathol, 80 (1996), pp. 333
Copyright © 2006. Sociedade Portuguesa de Pneumologia/SPP
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