COMMON VARIABLE IMMUNODEFICIENCY: CLINICAL, LABORATORY DATA AND OUTCOME OF OUR PATIENTS

Volume: 40 Number: 1 March 1, 2001
  • Ferah Genel
  • Güzide Aksu
  • Arzu Kütükçüler
  • Ceyhun Dizdarer
  • Suat Çağlayan
  • Necil Kütükçüler
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COMMON VARIABLE IMMUNODEFICIENCY: CLINICAL, LABORATORY DATA AND OUTCOME OF OUR PATIENTS

Abstract

Common variable immunodeficiency (CVID) is a condition characterized by an impairment in antibody production resulting in recurrent bacterial infections in untreated patients. Abnormalities of both B and T cells have been described. Affected individuals exhibit an increased incidence of inflammatory bowel disease, autoimmunity and malignancy. In this study, the clinical and laboratory data for 8 patients with CVID diagnosed and treated between January 1994 and January 2001 were evaluated. The onset of infectious signs and symptoms were 3.5 ± 2.67 years, the mean age of diagnosis was 8.79 ± 3.23 years and the diagnosis was delayed for 5.25 ± 2.52 years Recurrent sinopulmonary infections (%75) and chronic diarrhea (%50) were the most significant clinical features. In one patient, polyarthritis was the initial clinical manifestation. All patients presented with hypogammaglobulinemia and absence of specific antibody (antitetanus) responses. In 5 patients CD4/CD8 ratio was inverted. The patients were followed for a period of 21.5+18.5 months Three patients (%37.5) with pulmonary complaints progressed to bronchiectasis, while one patient develope inflammatory bowel disease in follow up. This patient died with bronchiectasis, pulmonary fibrosis and respiratory tailu despite adequate immunoglobulin replacement therapy. Minimalization of the complications in such a disease with hig mortality and morbidity rates is achieved by adequate treatment on a periodic basis in the remaining seven patients.

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Primary Language

Turkish

Subjects

-

Journal Section

-

Authors

Ferah Genel

Güzide Aksu

Arzu Kütükçüler

Ceyhun Dizdarer

Suat Çağlayan

Necil Kütükçüler

Publication Date

March 1, 2001

Submission Date

March 1, 2001

Acceptance Date

-

Published in Issue

Year 2001 Volume: 40 Number: 1

IZ

https://izlik.org/JA89TP29SD

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RIS / Bibtex
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1.Ferah Genel, Güzide Aksu, Arzu Kütükçüler, Ceyhun Dizdarer, Suat Çağlayan, Necil Kütükçüler. SIK GÖRÜLEN DEĞİŞKEN İMMUN YETERSİZLİK: OLGULARIN KLİNİK, LABORATUVAR VE İZLEM BULGULARININ DEĞERLENDİRİLMESİ. EJM [Internet]. 2001 Mar. 1;40(1):31-6. Available from: https://izlik.org/JA89TP29SD

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