Polycythemia and increased erythropoietin in a patient with chronic kidney disease

Stark, Simone; Winkelmann, B.; Kluthe, Christof; Roigas, Jan; Querfeld, Uwe; Müller, Dominik

doi:10.1038/ncpneph0437

Cited by 7 publications

(5 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Several other deficiencies such as Egf receptor, Claudin-4 , Dlg1 and 17q12 microdeletion have also been linked to abnormal kidney development [ 14 – 17 ]. Moreover, in a previous human case report Stark et al [ 18 ] presented similar findings in a 16 year old white male with chronic kidney disease and a history of obstructive uropathy. The patient had high red blood cells count and high erythropoietin but normal platelet and leukocyte count.…”

Section: Resultssupporting

confidence: 64%

Kidney epithelium specific deletion of kelch-like ECH-associated protein 1 (Keap1) causes hydronephrosis in mice

et al. 2016

View full text Add to dashboard Cite

BackgroundTranscription factor Nrf2 protects from experimental acute kidney injury (AKI) and is promising to limit progression in human chronic kidney disease (CKD) by upregulating multiple antioxidant genes. We recently demonstrated that deletion of Keap1, the endogenous inhibitor of Nrf2, in T lymphocytes significantly protects from AKI. In this study, we investigated the effect of Keap1 deletion on Nrf2 mediated antioxidant response in the renal tubular epithelial cells.MethodsWe deleted Keap1 exon 2 and 3 in the renal tubular epithelial cells by crossing Ksp-Cre mice with Keap1 floxed (Keap1f/f) mice. Deletion of Keap1 gene in the kidney epithelial cells of Ksp-Keap1-/- mice and its effect on Nrf2 target gene expression was performed using PCR and real-time PCR respectively. Histological evaluation was performed on H&E stained sections. Complete blood count, serum and urine analysis were performed to assess systemic effects of defective kidney development. Student’s T test was used to determine statistical difference between the groups.ResultsKsp-Cre resulted in the deletion of Keap1 exon 2 and 3 and subsequent upregulation of Nrf2 target genes, Nqo1, Gclm and Gclc in the kidney epithelial cells of Ksp-Keap1-/- mice at baseline. Renal epithelial cell specific deletion of Keap1 in Ksp-Keap1-/- mice caused marked renal pelvic expansion and significant compression of medullary parenchyma consistent with hydronephrosis in both (3 month-old) males and females. Kidneys from 6 month-old Ksp-Keap1-/- mice showed progressive hydronephrosis. Hematological, biochemical and urinary analysis showed significantly higher red blood cell count (p = 0.04), hemoglobin (p = 0.01), hematocrit (p = 0.02), mean cell volume (p = 0.02) and mean cell hemoglobin concentration (p = 0.003) in Ksp-Keap1-/- mice in comparison to Keap1f/f mice.ConclusionsThese unexpected findings demonstrate that Keap1 deletion in renal tubular epithelial cells results in an abnormal kidney development consistent with hydronephrosis and reveals a novel Keap1 mediated signaling pathway in renal development.

show abstract

Section: Resultssupporting

confidence: 64%

Kidney epithelium specific deletion of kelch-like ECH-associated protein 1 (Keap1) causes hydronephrosis in mice

et al. 2016

View full text Add to dashboard Cite

show abstract

“…Bu kronik böbrek yetmezliği hastalarında anemi prevalansı %71,8 bulunmuştur. Anemi kronik böbrek yetmezliği olan hastalarda polisitemiye göre çok daha sık karşılaşılan bir durumdur, fakat polikistik böbrek hastalığı ve hidronefrozu olan hastalarda artmış eritropoetin düzeylerine bağlı polisitemik olgular bildirilmiştir (28,29). Bu yapılan çalışmada beklenenin aksine kronik böbrek yetmezliği hastalarının ikisinde polisitemi saptanmıştır.…”

Section: Discussionunclassified

The Influence of Anemia on Mortality in Patients with Chronic Obstructive Pulmonary Disease

Metan¹,

Tutar²,

Kanbay³

et al. 2013

Solunum

View full text Add to dashboard Cite

Bu çalışmanın amacı Kronik Obstruktif Akciğer Hastalığı (KOAH) tanısı ile izlenen hastalarda anemi sıklığının ve aneminin yaşam süresi üzerine etkisinin belirlenmesidir. Yöntemler: KOAH tanısı ile 01 Ocak 2004-31 Aralık 2008 tarihleri arasında izlenen hastalar, ICD-10 kodları kullanılarak hastane bilgi işlem sistemi veri tabanı aracılığıyla saptandı. Anemi tanısı Dünya Sağlık Örgütü kriterlerine göre konuldu. Hastalarda KOAH evrelemesi GOLD kriterlerine uygun olarak FEV 1 /FVC değerlerine göre yapıldı. Mortalite 1 Ocak 2004 ile 1 Ocak 2010 tarihleri arasında herhangi bir tarihte yaşamın kaybedilmesi olarak tanımlandı. Bulgular: Çalışmaya 452 hasta dahil edildi. Hastalardan 222'sinin (%49,1) öldüğü belirlendi. Anemi 145 (%32,07) hastada saptandı. Ölen hastalar ile hayatta kalan hastalar karşılaştırıldığında, yaş, hemoglobin, FEV 1 ve %FEV 1 değerlerinde istatistiksel olarak anlamlı farklılık saptandı. Ölen hastaların %76,1'inde ağır ve çok ağır KOAH saptanırken, yaşayan hastalarda bu oran %60,5 idi (p<0,001). Ölen hastalarda solid tümör daha sık saptandı (p<0,001). Anemik hastalar, polisitemik ve normal hemoglobine sahip hastalara göre daha yaşlı bulunurken (p=0,017), her üç hasta grubunda FEV 1 , %FEV 1 ve %FEV 1 /FVC değerleri arasında istatistiksel olarak anlamlı bir fark saptanmadı. Anemik hastalarda ölüm oranı polisitemik ve normal hemoglobine sahip hastalara göre daha yüksek bulundu (p=0,002). Anemik hastaların %62,8'inde, polisitemik hastaların %72,7'sinde ve normal hemoglobine sahip hastaların %70,3'ünde ağır ve çok ağır KOAH saptanmış olup, her üç grup arasında bu açıdan istatistiksel olarak anlamlı bir fark bulunmadı. Sonuç: Bu retrospektif çalışmada anemi KOAH hastalarında polisitemiye göre daha sık saptanmış ve anemik hastalarda mortalite oranının arttığı görülmüştür. Ayrıca KOAH hastalarında FEV 1 değerleri düştükçe mortalitenin arttığı belirlenmiştir.

show abstract

“…In rare cases, hydronephrotic kidney disease [16], obstructive sleep apnea [17], or renal artery thrombosis [18] can cause Epo-dependent secondary polycythemia in a dialysis patient. A high serum Epo level in polycythemic patients suggests secondary erythrocytosis as a possible diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Erythropoietin-producing tubercle granuloma in a hemodialysis patient

et al. 2013

View full text Add to dashboard Cite

BackgroundWe describe a case of a fever of unknown etiology that was caused by a caseating tubercle granuloma which produced erythropoietin. To our knowledge, this is the first report of an erythropoietin- producing granuloma.Case presentationA 48-year-old Japanese man with a 5-year history of maintenance hemodialysis for diabetic nephropathy presented with an intermittent fever over a few months. During febrile periods he developed erythema nodosum on his legs. Computed tomography showed axillary lymph node enlargement and this was further corroborated by a gallium scan that revealed high gallium uptake in these nodes. A Mantoux test was positive and an interferongamma release assay for tuberculosis diagnosis was also positive. Lymph node tuberculosis was suspected and the patient underwent lymphadenectomy. Histological analysis of the lymph nodes revealed a caseating granuloma that showed positive results on an acid-fast bacteria stain and a Mycobacterium tuberculosis polymerase chain reaction test. After lymphadenectomy, however, the patient’s hemoglobin levels rapidly decreased from 144 to 105 g/L, and this was further compounded by a decrease in serum erythropoietin from 223 mIU/mL to 10.7 mIU/mL by postoperative day 21. We suspected the tubercle to be a source of the erythropoietin and this was further confirmed by in situ hybridization.ConclusionsWe report for the first time ectopic erythropoietin production by a tuberculous lymph node. Our observations are substantiated by a postoperative decline in his erythropoietin level and a clinical requirement for erythropoietin treatment.

show abstract

Polycythemia and increased erythropoietin in a patient with chronic kidney disease

Abstract: Serial phlebotomies and laparoscopic removal of the right hydronephrotic kidney.

Cited by 7 publications

References 15 publications

Kidney epithelium specific deletion of kelch-like ECH-associated protein 1 (Keap1) causes hydronephrosis in mice

Kidney epithelium specific deletion of kelch-like ECH-associated protein 1 (Keap1) causes hydronephrosis in mice

The Influence of Anemia on Mortality in Patients with Chronic Obstructive Pulmonary Disease

Erythropoietin-producing tubercle granuloma in a hemodialysis patient

Contact Info

Product

Resources

About