Araştırma Makalesi
BibTex RIS Kaynak Göster
Yıl 2023, Cilt: 3 Sayı: 1, 13 - 19, 29.01.2023
https://doi.org/10.56016/dahudermj.1225606

Öz

Kaynakça

  • Schroeder, HW, Jr. “Genetics of IgA deficiency and common variable immunodeficiency.” Clin Rev Allergy Immunol 2000;19(2): 127-140.
  • Notarangelo, LD, A Fischer, RS Geha, JL Casanova, H Chapel, ME Conley, et al. “Primary immunodeficiencies: 2009 update.” J Allergy Clin Immunol 2009;124(6): 1161-1178.
  • Conley, ME, J Rohrer, L Rapalus, EC Boylin and Y Minegishi. “Defects in early Bcell development: comparing the consequences of abnormalities in pre-BCR signaling in the human and the mouse.” Immunol Rev 2000;178: 75-90.
  • Hammarstrom, L, I Vorechovsky and D Webster. “Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID).” Clin Exp Immunol 2000;120(2): 225-231.
  • Chapel, H, M Lucas, M Lee, J Bjorkander, D Webster, B Grimbacher, et al. “Common variable immunodeficiency disorders: division into distinct clinical phenotypes.” Blood 2008;112(2): 277-286.
  • Quinti, I, A Soresina, G Spadaro, S Martino, S Donnanno, C Agostini, et al. “Longterm follow-up and outcome of a large cohort of patients with common variable immunodeficiency.” J Clin Immunol 2007;27(3): 308-316.
  • Ardeniz, O, CB Avci, A Sin, G Ozgen, F Gunsar, N Mete, et al. “Vitamin D deficiency in the absence of enteropathy in three cases with common variable immunodeficiency.” Int Arch Allergy Immunol 2008;147(1): 74-83.
  • Urschel, S, L Kayikci and U Wintergerst. “Common variable immundeficiency disorders in children: delayed diagnosis despite typical clinical presentation.” J Pediatr 2009;(154): 888.
  • Conley, ME, LD Notarangelo and A Etzioni. “Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies).” Clin Immunol 1999;93(3): 190-197.
  • Cunningham-Rundles, C and C Bodian. “Common variable immunodeficiency: clinical and immunological features of 248 patients.” Clin Immunol 1999;92(1): 34-48.
  • Bates, CA, MC Ellison, DA Lynch, CD Cool, KK Brown and JM Routes. “Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency.” J Allergy Clin Immunol 2004;114(2): 415-421.
  • Wood, P, S Stanworth, J Burton, A Jones, DG Peckham, T Green, et al. “Recognition, clinical diagnosis and management of patients with primary antibody deficiencies: a systematic review.” Clin Exp Immunol 2007;149(3): 410-423.
  • Daniels, JA, HM Lederman, A Maitra and EA Montgomery. “Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review.” Am J Surg Pathol 2007;31(12): 1800-1812.
  • Agarwal, S and L Mayer. “Gastrointestinal manifestations in primary immune disorders.” Inflamm Bowel Dis 2010;16(4): 703-711.
  • Resnick, ES, EL Moshier, JH Godbold and C Cunningham-Rundles. “Morbidity and mortality in common variable immune deficiency over 4 decades.” Blood 2012;119(7): 1650-1657.
  • Notarangelo, LD, A Fischer, RS Geha, JL Casanova, H Chapel, ME Conley, et al. (2009). “Primary immunodeficiencies: 2009 update.” J Allergy Clin Immunol 124(6): 1161-1178.
  • Agarwal, S and C Cunningham-Rundles. “Autoimmunity in common variable immunodeficiency.” Curr Allergy Asthma Rep 2009; 9(5): 347-352.
  • Busse, PJ, S Razvi and C Cunningham-Rundles. “Efficacy of intravenous immunoglobulin in the prevention of pneumonia in patients with common variable immunodeficiency.” J Allergy Clin Immunol 2002;109(6): 1001-1004.
  • Chapel, HM, GP Spickett, D Ericson, W Engl, MM Eibl and J Bjorkander. “The comparison of the efficacy and safety of intravenous versus subcutaneous immunoglobulin replacement therapy.” J Clin Immunol 2000;20(2): 94-100

Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience

Yıl 2023, Cilt: 3 Sayı: 1, 13 - 19, 29.01.2023
https://doi.org/10.56016/dahudermj.1225606

Öz

Objectives: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by immunoglobulin production defect. Our study aimed to create awareness of primary immunodeficiency in adult patients, establish standard approaches for clinical follow-up of CVID patients, and reveal the clinical characteristics of CVID patients in our region.
Method: The study was conducted in patients with diagnosed and newly diagnosed CVID. The demographic and clinical characteristics of the patients and their treatment data were analyzed retrospectively and prospectively.
Results: Thirteen of our patients were female and 12 were male. The mean age at diagnosis of the patients was 30.32 (2-57) and the mean delay in diagnosis was 9.32 months (0-30). The most common clinical finding of our patients at the time of admission was an infection. Among the infections identified, 3 patients had URTI, 19 had LRTI, and 2 had gastroenteritis. In 16 of our patients, bronchiectasis was detected at the time of diagnosis, and in 1 during the follow-up period. In the examinations performed in terms of organomegaly, splenomegaly was found in 11 patients and hepatomegaly was found in 8 patients. When patients were screened for autoimmune disease, ITP and celiac were found in 2 patients at the beginning, while autoimmune thyroiditis was developed in 1 patient and SLE in 1 patient during follow-up. Our patients were given IVIG treatment at regular intervals. The number of reactions seen in a total of 421 IVIG infusions was two.
Conclusion: Primary immunodeficiencies should definitely be considered in patients with recurrent infections and resistance to antibiotic therapy. Patients should be followed according to established follow-up and treatment protocols in order to reduce and diagnose complications.

Kaynakça

  • Schroeder, HW, Jr. “Genetics of IgA deficiency and common variable immunodeficiency.” Clin Rev Allergy Immunol 2000;19(2): 127-140.
  • Notarangelo, LD, A Fischer, RS Geha, JL Casanova, H Chapel, ME Conley, et al. “Primary immunodeficiencies: 2009 update.” J Allergy Clin Immunol 2009;124(6): 1161-1178.
  • Conley, ME, J Rohrer, L Rapalus, EC Boylin and Y Minegishi. “Defects in early Bcell development: comparing the consequences of abnormalities in pre-BCR signaling in the human and the mouse.” Immunol Rev 2000;178: 75-90.
  • Hammarstrom, L, I Vorechovsky and D Webster. “Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID).” Clin Exp Immunol 2000;120(2): 225-231.
  • Chapel, H, M Lucas, M Lee, J Bjorkander, D Webster, B Grimbacher, et al. “Common variable immunodeficiency disorders: division into distinct clinical phenotypes.” Blood 2008;112(2): 277-286.
  • Quinti, I, A Soresina, G Spadaro, S Martino, S Donnanno, C Agostini, et al. “Longterm follow-up and outcome of a large cohort of patients with common variable immunodeficiency.” J Clin Immunol 2007;27(3): 308-316.
  • Ardeniz, O, CB Avci, A Sin, G Ozgen, F Gunsar, N Mete, et al. “Vitamin D deficiency in the absence of enteropathy in three cases with common variable immunodeficiency.” Int Arch Allergy Immunol 2008;147(1): 74-83.
  • Urschel, S, L Kayikci and U Wintergerst. “Common variable immundeficiency disorders in children: delayed diagnosis despite typical clinical presentation.” J Pediatr 2009;(154): 888.
  • Conley, ME, LD Notarangelo and A Etzioni. “Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies).” Clin Immunol 1999;93(3): 190-197.
  • Cunningham-Rundles, C and C Bodian. “Common variable immunodeficiency: clinical and immunological features of 248 patients.” Clin Immunol 1999;92(1): 34-48.
  • Bates, CA, MC Ellison, DA Lynch, CD Cool, KK Brown and JM Routes. “Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency.” J Allergy Clin Immunol 2004;114(2): 415-421.
  • Wood, P, S Stanworth, J Burton, A Jones, DG Peckham, T Green, et al. “Recognition, clinical diagnosis and management of patients with primary antibody deficiencies: a systematic review.” Clin Exp Immunol 2007;149(3): 410-423.
  • Daniels, JA, HM Lederman, A Maitra and EA Montgomery. “Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review.” Am J Surg Pathol 2007;31(12): 1800-1812.
  • Agarwal, S and L Mayer. “Gastrointestinal manifestations in primary immune disorders.” Inflamm Bowel Dis 2010;16(4): 703-711.
  • Resnick, ES, EL Moshier, JH Godbold and C Cunningham-Rundles. “Morbidity and mortality in common variable immune deficiency over 4 decades.” Blood 2012;119(7): 1650-1657.
  • Notarangelo, LD, A Fischer, RS Geha, JL Casanova, H Chapel, ME Conley, et al. (2009). “Primary immunodeficiencies: 2009 update.” J Allergy Clin Immunol 124(6): 1161-1178.
  • Agarwal, S and C Cunningham-Rundles. “Autoimmunity in common variable immunodeficiency.” Curr Allergy Asthma Rep 2009; 9(5): 347-352.
  • Busse, PJ, S Razvi and C Cunningham-Rundles. “Efficacy of intravenous immunoglobulin in the prevention of pneumonia in patients with common variable immunodeficiency.” J Allergy Clin Immunol 2002;109(6): 1001-1004.
  • Chapel, HM, GP Spickett, D Ericson, W Engl, MM Eibl and J Bjorkander. “The comparison of the efficacy and safety of intravenous versus subcutaneous immunoglobulin replacement therapy.” J Clin Immunol 2000;20(2): 94-100
Toplam 19 adet kaynakça vardır.

Ayrıntılar

Birincil Dil İngilizce
Konular İç Hastalıkları
Bölüm Research Articles
Yazarlar

Zeynep Yüksel 0000-0002-2929-5039

Ahmet Zafer Calıskaner Bu kişi benim 0000-0002-9084-8704

Yayımlanma Tarihi 29 Ocak 2023
Gönderilme Tarihi 28 Aralık 2022
Yayımlandığı Sayı Yıl 2023 Cilt: 3 Sayı: 1

Kaynak Göster

EndNote Yüksel Z, Calıskaner AZ (01 Ocak 2023) Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience. DAHUDER Medical Journal 3 1 13–19.



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