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Kronik Böbrek Hastalığı Olan Bireylerde Orak Hücreli Anemi Taşıyıcı Sıklığının Belirlenmesi

Yıl 2020, Cilt: 4 Sayı: 3, 55 - 59, 27.11.2020

Öz

Amaç: Orak Hücreli Anemi Taşıyıcılığı Türkiye’de özellikle Çukurova Bölgesi’nde yaygın olarak görülmektedir. Orak Hücreli Anemi Taşıyıcısı olan bireylerde en sık görülen böbrek anormallikleri renal konsantrasyon defektleri, renal papiller nekroz ve hematüridir. Orak Hücreli Anemi taşıyıcılığının kronik böbrek yetmezliğine yol açmasıyla ilgili tartışmalı sonuçlar yayınlanmıştır. Türkiye’de Kronik Böbrek Hastalığı olan bireylerde Orak Hücreli Anemi Taşıyıcı sıklığının araştırıldığı herhangi bir çalışma bulunmamaktadır. Bu çalışmada, Türkiye’de tek bir merkezdeki kronik böbrek hastalığı olan bireylerde Orak Hücreli Anemi Taşıyıcılığı’ nın sıklığını belirlemek amaçlanmıştır.
Gereç ve Yöntemler: Merkezimizde Erişkin ve Çocuk Nefroloji Polikliniklerinde takip edilen, kronik böbrek yetmezliği, tübülopati, proteinüri ve hematürisi olan 164 hasta çalışmaya dahil edildi. Hastaların tanıları, hemoglobin düzeyleri, glomerüler filtrasyon hızları, idrar dansiteleri, proteinüri, hematüri varlığı ve ailede orak hücreli anemi öyküsünün olup olmaması gibi veriler kayıtlardan elde edildi. Hemoglobin elektroforezi ile HbS ve HbA değerleri elde edildi.
Bulgular: Hastaların yaş ortalaması 33.18±27.33 yıl (min.-maks. 1-92 yıl) idi. Hastaların 92’sinde (%56,1) kronik böbrek yetmezliği, 19’unda (%11,6) tübülopati, 22’sinde (%13,4) proteinüri ve 31’inde (%18,9) hematüri vardı. Orak hücreli anemi taşıyıcılığı kronik böbrek yetmezliği olan üç erişkin bireyde saptandı (%1,8).
Sonuç: Bu çalışmaya göre Kronik Böbrek Hastalığı olan bireylerde Orak Hücreli Anemi Taşıyıcılığı sıklığında artış görülmemiştir. Bu bulguları desteklemek için daha büyük hasta gruplarıyla çalışmalar yapılmalıdır.

Kaynakça

  • 1. Shaw C, Sharpe C.C, Could sicle cell trait be a predisposing risk factor for CKD? Nephrol Dial Transplant, 2010: 25; 2403-2405.
  • 2. Ashley-Koch A, Yang Q, Olney RS, Sickle hemoglobin (HbS) allele and sickle cell disease: a HuGE review, Am J Epidemiol 2000: 151; 839–845.
  • 3. Connes P, Hardy-Dessources MD, Hue O, Counterpoint: sickle cell trait should not be considered asymptomatic and as a benign condition during physical activity, J Appl Physiol 2007: 103; 2138–2140.
  • 4. Fabritius H, Millan J, Le Corroller Y, Systematic screening of hemoglobinopathies in blood donors in Guadeloupe (French West Indies), Rev Fr Transfus Immunohematol 1978: 21; 937–950.
  • 5. Dünya’da ve Türkiye’de talasemi ve hemoglobinopatiler. Arcasoy A. (editör). Hemoglobinopati ve Talasemi Önlem-Tanı-Tedavi. 2. Baskı. Antalya: Ulusal Hemoglob inopati Konseyi Sağlık Bakanlığı 2003. p.11-19.
  • 6. Gümrük F, Altay Ç, Orak hücre anemisi, Katkı,1995:16; 327-345.
  • 7. Arpacı A, Aksoy K, Dikmen N. Çukurova’da orak hücre anemisi ve talasemi taraması, XXII. Ulusal Hematoloji Kongresi, İstanbul 1991: 115.
  • 8. Eraslan S. Beta Talaseminin Moleküler Tanısı. Düzen Genetik Hastalıklar Tanı Merkezi, Ankara-Türkiye, 2005.
  • 9. Kılınç Y, Akmanlar N, Kümi M, Köker I, The incidences of hemoglobinopathies and thalassemias in cord blood of newborns from Çukurova Province, Med Bull İstanbul Medical Faculty 1992: 25; 9- 14.
  • 10. Buckalew VM Jr, Someren A, Renal manifestations of sickle cell disease, Arch Intern Med. 1974:133;660–669.
  • 11. Statius van Eps L, Earley L. Strauss and Welt's Diseases of the Kidney. 3rd ed, Boston: Little, Brown & Company, 1979: 1229-1240.
  • 12. Key NS, Derebail VK, Sickle cell trait: novel clinical significance, Hematology Am Soc Hematol Educ Program, 2010: 418-422.
  • 13. Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y. Complications associated with sickle cell trait: a brief narrative review, Am J Med. 2009: 122: 507–512.
  • 14. Heller P, Best WR, Nelson RB, Becktel J, Clinical implications of sickle cell trait and glucose-6-phosphate dehydrogenase deficiency in hospitalized black male patients, N Engl J Med. 1979: 300 (18); 1001-1005.
  • 15. Statius van Eps LW, Pinedo-Veels C, de Vries GH, de Koning J, Nature of concentrating defect in sickle cell nephropathy: microradioangiographic studies. Lancet. 1970: 1 (7644); 450-452.
  • 16. Alvarez O, Rodriguez MM, Jordan L, Sarnaik S, Renal medullary carcinoma and sickle cell trait: A systematic review. Pediatr Blood Cancer, 2015: 62(10); 1694-1699.
  • 17. Naik R.P, Derebail V.K, Grams M.E, Franceschini N, Auer P.L, Peloso G.M, Association of Sickle Cell Trait With Chronic Kidney Disease and Albuninuria in African Americans, JAMA, 2014: 312 (20) ; 2115-2125.
  • 18. Mukendi K, Lepira FB, Makulo JR, Sumaili KE, Kayembe PK, Nseka MN, Sickle cell trait is not associated with chronic kidney disease in adult Congolese patients: a clinic-based, cross-sectional study, CVJ Africa, 2015: 26 (3); 125-129.
  • 19. Hicks P.J, Langefeld C.D, Lu L, Bleyer A.J, Divers J, Nachman P.H, Sickle cell trait is not independently associated with susceptibility to end-stage renal disease in African Americans, Kidney Int, 2011: 80 (12);1339-1343.
  • 20. Piccone C, Dell KM. Sickle Cell Nephropathy in Children, Pedaitric Nephrology (7nd ed) Vol.2. Berlin Heidelberg: Springer Verlag, 2016: 1526.
  • 21. Maigne G, Ferlicot S, Galacteros F, Belenfant X, Ulinski T, Niaudet P et al. Glomerular lesions in patients with sickle cell disease, Medicine 2010:89 (1) ;18-27.
  • 22. Eckert DE, Jonutis AJ, Davidson AJ, The incidence and manifestations of urographic papillary abnormalities in patients with S hemoglobinopathies. Radiology, 1974: 113; 59–63.
  • 23. Gupta AK, Kirchner KA, Nicholson R, Adams JG, III, Schechter AN, Noguchi CT, et al, Effects of alpha-thalassemia and sickle polymerization tendency on the urine-concentrating defect of individuals with sickle cell trait, J Clin Invest 1991: 88(6);1963-1968.
  • 24. Statius Van Eps LW, De Jong PE, Sickle Cell Disease, In: Schrier RW, Gottschalk, CW, eds. Disease of the Kidney, 1997; p. 2201-2219.
  • 25. Sesso R. Almeida M.A., Figueiredo M. S, Bordin J.O, Renal dysfunction in patients with sickle cell anemia or sickle cell trait, Braz J Med Biol Res. 1998: 31; 1257-1262.

Frequency of Sickle Cell Trait in the patients with chronic kidney diseases in a tertiary center in Turkey

Yıl 2020, Cilt: 4 Sayı: 3, 55 - 59, 27.11.2020

Öz

Aim: Sickle Cell Disease and Sickle Cell Trait (SCT) is commonly seen in Çukurova region in Turkey. Renal manifestations of the sickle cell trait (SCT) include renal concentration defects, renal papillary necrosis, and hematuria. Conflicting results were reported regarding whether the SCT leads to chronic renal failure. Frequency of Sickle Cell Trait in the patients with chronic kidney diseases has yet to be reported in Turkey. This study evaluated the SCT frequency in patients with chronic kidney diseases in a single center in Turkey.
Materials and Methods: A total of 164 patients with chronic kidney disease such as chronic renal failure, tubulopathy, proteinuria, and hematuria who were followed-up in the Pediatric and Adult Nephrology Departments in a center were enrolled in the study. Information regarding diagnosis, hemoglobin levels, glomerular filtration rate, urine density, proteinuria, hematuria, and family history for sickle cell disorder were collected from the records retrospectively. HbS and HbA values were evaluated using hemoglobin electrophoresis.
Results: The mean age of the patients was 33.18±27.33 years (min.-maks. 1-92 years). Ninety-two (56.1%) had chronic renal failure, 19 (11.6%) had tubulopathy, 22 (13.4%) had proteinuria, and 31 (18.9%) had hematuria. SCT was detected in three adult patients (1.8%) with chronic renal failure.
Conclusion: The SCT was not associated with increased frequency of chronic kidney diseases according to this study. Further studies with a larger sample size are needed to support this result.

Kaynakça

  • 1. Shaw C, Sharpe C.C, Could sicle cell trait be a predisposing risk factor for CKD? Nephrol Dial Transplant, 2010: 25; 2403-2405.
  • 2. Ashley-Koch A, Yang Q, Olney RS, Sickle hemoglobin (HbS) allele and sickle cell disease: a HuGE review, Am J Epidemiol 2000: 151; 839–845.
  • 3. Connes P, Hardy-Dessources MD, Hue O, Counterpoint: sickle cell trait should not be considered asymptomatic and as a benign condition during physical activity, J Appl Physiol 2007: 103; 2138–2140.
  • 4. Fabritius H, Millan J, Le Corroller Y, Systematic screening of hemoglobinopathies in blood donors in Guadeloupe (French West Indies), Rev Fr Transfus Immunohematol 1978: 21; 937–950.
  • 5. Dünya’da ve Türkiye’de talasemi ve hemoglobinopatiler. Arcasoy A. (editör). Hemoglobinopati ve Talasemi Önlem-Tanı-Tedavi. 2. Baskı. Antalya: Ulusal Hemoglob inopati Konseyi Sağlık Bakanlığı 2003. p.11-19.
  • 6. Gümrük F, Altay Ç, Orak hücre anemisi, Katkı,1995:16; 327-345.
  • 7. Arpacı A, Aksoy K, Dikmen N. Çukurova’da orak hücre anemisi ve talasemi taraması, XXII. Ulusal Hematoloji Kongresi, İstanbul 1991: 115.
  • 8. Eraslan S. Beta Talaseminin Moleküler Tanısı. Düzen Genetik Hastalıklar Tanı Merkezi, Ankara-Türkiye, 2005.
  • 9. Kılınç Y, Akmanlar N, Kümi M, Köker I, The incidences of hemoglobinopathies and thalassemias in cord blood of newborns from Çukurova Province, Med Bull İstanbul Medical Faculty 1992: 25; 9- 14.
  • 10. Buckalew VM Jr, Someren A, Renal manifestations of sickle cell disease, Arch Intern Med. 1974:133;660–669.
  • 11. Statius van Eps L, Earley L. Strauss and Welt's Diseases of the Kidney. 3rd ed, Boston: Little, Brown & Company, 1979: 1229-1240.
  • 12. Key NS, Derebail VK, Sickle cell trait: novel clinical significance, Hematology Am Soc Hematol Educ Program, 2010: 418-422.
  • 13. Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y. Complications associated with sickle cell trait: a brief narrative review, Am J Med. 2009: 122: 507–512.
  • 14. Heller P, Best WR, Nelson RB, Becktel J, Clinical implications of sickle cell trait and glucose-6-phosphate dehydrogenase deficiency in hospitalized black male patients, N Engl J Med. 1979: 300 (18); 1001-1005.
  • 15. Statius van Eps LW, Pinedo-Veels C, de Vries GH, de Koning J, Nature of concentrating defect in sickle cell nephropathy: microradioangiographic studies. Lancet. 1970: 1 (7644); 450-452.
  • 16. Alvarez O, Rodriguez MM, Jordan L, Sarnaik S, Renal medullary carcinoma and sickle cell trait: A systematic review. Pediatr Blood Cancer, 2015: 62(10); 1694-1699.
  • 17. Naik R.P, Derebail V.K, Grams M.E, Franceschini N, Auer P.L, Peloso G.M, Association of Sickle Cell Trait With Chronic Kidney Disease and Albuninuria in African Americans, JAMA, 2014: 312 (20) ; 2115-2125.
  • 18. Mukendi K, Lepira FB, Makulo JR, Sumaili KE, Kayembe PK, Nseka MN, Sickle cell trait is not associated with chronic kidney disease in adult Congolese patients: a clinic-based, cross-sectional study, CVJ Africa, 2015: 26 (3); 125-129.
  • 19. Hicks P.J, Langefeld C.D, Lu L, Bleyer A.J, Divers J, Nachman P.H, Sickle cell trait is not independently associated with susceptibility to end-stage renal disease in African Americans, Kidney Int, 2011: 80 (12);1339-1343.
  • 20. Piccone C, Dell KM. Sickle Cell Nephropathy in Children, Pedaitric Nephrology (7nd ed) Vol.2. Berlin Heidelberg: Springer Verlag, 2016: 1526.
  • 21. Maigne G, Ferlicot S, Galacteros F, Belenfant X, Ulinski T, Niaudet P et al. Glomerular lesions in patients with sickle cell disease, Medicine 2010:89 (1) ;18-27.
  • 22. Eckert DE, Jonutis AJ, Davidson AJ, The incidence and manifestations of urographic papillary abnormalities in patients with S hemoglobinopathies. Radiology, 1974: 113; 59–63.
  • 23. Gupta AK, Kirchner KA, Nicholson R, Adams JG, III, Schechter AN, Noguchi CT, et al, Effects of alpha-thalassemia and sickle polymerization tendency on the urine-concentrating defect of individuals with sickle cell trait, J Clin Invest 1991: 88(6);1963-1968.
  • 24. Statius Van Eps LW, De Jong PE, Sickle Cell Disease, In: Schrier RW, Gottschalk, CW, eds. Disease of the Kidney, 1997; p. 2201-2219.
  • 25. Sesso R. Almeida M.A., Figueiredo M. S, Bordin J.O, Renal dysfunction in patients with sickle cell anemia or sickle cell trait, Braz J Med Biol Res. 1998: 31; 1257-1262.
Toplam 25 adet kaynakça vardır.

Ayrıntılar

Birincil Dil İngilizce
Konular Klinik Tıp Bilimleri
Bölüm ARAŞTIRMA MAKALESİ
Yazarlar

Serra Sürmeli Döven 0000-0001-9109-859X

Selma Ünal 0000-0002-9951-0291

Serap Demir 0000-0002-2189-6825

Simge Bardak 0000-0002-6851-4871

Feryal Karahan 0000-0002-1729-9585

Ayşe Güneş 0000-0003-4078-6039

Ali Delibaş 0000-0002-1469-9276

Yayımlanma Tarihi 27 Kasım 2020
Yayımlandığı Sayı Yıl 2020 Cilt: 4 Sayı: 3

Kaynak Göster

APA Sürmeli Döven, S., Ünal, S., Demir, S., Bardak, S., vd. (2020). Frequency of Sickle Cell Trait in the patients with chronic kidney diseases in a tertiary center in Turkey. Balıkesir Medical Journal, 4(3), 55-59.