Serra SÜRMELİ DÖVEN, Selma ÜNAL, Serap DEMİR, Simge BARDAK, Feryal KARAHAN, Ayşe GÜNEŞ, Ali DELİBAŞ

Kronik Böbrek Hastalığı Olan Bireylerde Orak Hücreli Anemi Taşıyıcı Sıklığının Belirlenmesi

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.

Anahtar Kelimeler:

Kronik böbrek hastalığı, Orak hücreli anemi taşıyıcılığı, Orak hücreli anemi hastalığı

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

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.

Keywords:

Chronic kidney disease, sickle cell trait, sickle cell disease,

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