TJH-2018-1-baski

More documents

Recommendations

Info

BRIEF REPORTDOI: 10.4274/tjh.2017.0266Turk J Hematol 2018;35:66-70Glomerular and Tubular Functions in Children and Adults withTransfusion-Dependent ThalassemiaTransfüzyona Bağımlı Çocuk ve Erişkin Talasemi Hastalarında Glomerüler ve Tubuler BöbrekFonksiyonlarıAgageldi Annayev 1 , Zeynep Karakaş 1 , Serap Karaman 1 , Altan Yalçıner 2 , Alev Yılmaz 3 , Sevinç Emre 31İstanbul University İstanbul Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey2Düzen Laboratories, İstanbul, Turkey3İstanbul University İstanbul Faculty of Medicine, Department of Pediatric Nephrology, İstanbul, TurkeyAbstractThis study aimed at assessing renal functions in patients withtransfusion-dependent thalassemia (TDT). Fifty patients and 30controls were enrolled in this prospective study. Serum levels ofelectrolytes and albumin were measured by a spectrophotometer.Serum levels of cystatin-C and urinary levels of β2-microglobulinwere measured by nephelometric method. Thirty-eight patients werereceiving deferasirox and 8 were on deferiprone. Serum electrolytesand albumin levels of the patients were found to be within normalranges. Urinary β2-microglobulin and serum cystatin-C levels weresignificantly higher in patients than controls. They did not significantlydiffer between the subgroup of patients on deferiprone and thecontrol group, whereas they were found to be higher in patients usingdeferasirox compared to controls. Urinary β2-microglobulin levelssignificantly increased in patients who were receiving high-dosedeferasirox compared to those who were receiving a daily dose of15-20 mg/kg or controls. Subclinical renal injury may be present inTDT patients.Keywords: Thalassemia, Tubulopathy, Glomerulopathy, β2-Microglobulin, CystatinÖzBu çalışmada transfüzyona bağımlı talasemi (TBT) hastalarındaböbrek fonksiyonlarının değerlendirilmesi amaçlanmıştır. Prospektifçalışmaya, 50 TBT ve 30 kontrol grubu dahil edildi. Serum elektrolitlerive albumin düzeyleri spektrofotometre ile ölçüldü. Serum sistatin-C veidrar β2-mikroglobülin düzeyleri nefelometrik yöntemle ölçüldü. Otuzsekiz hasta deferasiroks, 8 hasta deferipron alıyordu. Hastaların serumelektrolitleri ve albumin düzeyleri normal sınırlardaydı. İdrar β2-mikroglobulin ve serum sistatin-C düzeyleri hasta grubunda kontrolgrubundakilere göre anlamlı derecede yüksekti. Serum Cys-C ve idrarβ2-mikroglobulin düzeyleri, deferipron kullananlar ve kontrol grubuarasında anlamlı farklılık göstermezken, deferasiroks kullananlardakontrol grubuna göre daha yüksek bulundu. İdrar β2 mikroglobulindüzeyleri, yüksek doz deferasiroks alan hastalarda, 15-20 mg/kg/gün deferasiroks alanlara veya kontrol grubuna göre anlamlı şekildeartmıştı. Transfüzyona bağımlı talasemi hastalarında subklinik olarakrenal hasar mevcut olabilir.Anahtar Sözcükler: Talasemi, Tubulopati, Glomerulopati, β2-Mikroglobulin, Sistatin CIntroductionIron accumulation may lead to renal damage in transfusiondependentthalassemia (TDT) [1,2]. Cystatin C (Cys-C), a smallmolecular weight protein, is filtered from the glomeruli,reabsorbed from the tubular cells, and metabolized from thekidneys. It is a good marker for glomerular filtration rate (GFR).β2-Microglobulin (β2MG), a single-chain, low-molecular-weightpolypeptide, is filtered by the glomeruli, then reabsorbed andcatabolized in the proximal tubular cells. Increased urinaryexcretion of β2MG may demonstrate tubular dysfunction. Ourstudy assessed kidney function in patients with TDT using serumCys-C (SCys-C) and urinary β2MG (Uβ2MG) measurements inaddition to routine tests, as well as the utility of these markers asindicators for early glomerulopathy and tubulopathy.Materials and MethodsFifty patients with TDT, 25 under and 25 above the age of 18,have been followed since their childhood by the Thalassemia©Copyright 2018 by Turkish Society of HematologyTurkish Journal of Hematology, Published by Galenos Publishing HouseAddress for Correspondence/Yazışma Adresi: Serap KARAMAN, M.D., İstanbul University İstanbulFaculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, TurkeyPhone : +90 533 437 81 30E-mail : drkaramans@yahoo.com ORCID-ID: orcid.org/0000-0002-7428-3897Received/Geliş tarihi: July 17, 2017Accepted/Kabul tarihi: July 28, 201766
Turk J Hematol 2018;35:66-70Annayev A, et al: Kidney Functions in Transfusion-Dependent Thalassemia PatientsCenter at the İstanbul Faculty of Medicine of İstanbul University.Twenty-two patients were male and 28 were female. The meanage was 18.4±11.8 years (range: 2-45). Thirty age- and sexmatchedsubjects were included in the control group. Ethicalapproval was granted by the institutional review board andpatient consent was obtained.Serum electrolytes, urine calcium/creatinine (uCa/Cr) andfractional excretion of sodium (FENa), GFR, proteinuria, serumCr, and albumin levels were measured and were compared totheir reference ranges in the patient group. SCys-C and Uβ2MGlevels in the patient group were compared to those of thecontrols and potential correlations between SCys-C or Uβ2MGlevels and the severity of anemia, ferritin levels, and chelationtherapy were also evaluated. Blood samples collected tomeasure SCys-C were cold-centrifuged at 4000 rpm for 10 min.A photometric analysis of serum albumin, urea, Cr, uric acid, andelectrolyte levels was conducted. Ferritin levels were measuredby the electrochemiluminescent immunoassay method. SCys-Cand Uβ2MG levels were measured by nephelometry.Statistical analysis was done using SPSS 17.0.ResultsNo significant differences were found between the patientsand controls in terms of age or sex (p>0.05). Demographic andclinical characteristics of the patients are summarized in Table1. In the patients, serum Na, K, Ca, P, Mg, urea, Cr, and albuminwere within normal limits. None of the patients had proteinuria.The mean uCa/Cr ratio was found to be higher than the normallevel. Estimated GFR was elevated in the patient group (Table2). SCys-C and Uβ2MG levels were higher in patients thancontrols (p=0.001, p=0.010) (Table 3). SCys-C was increased withage (r=0.376; p=0.007). No correlations were found betweenUβ2MG levels and age (r=-0.186, p=0.217).Renal dysfunction was detected in 30 out of 50 patients. FENalevels were increased in 8 patients, while Uβ2MG and SCys-Clevels were increased in 9 and 25 patients, respectively. In ourstudy, renal involvement was observed in 54% of the patientsunder the age of 18 and 64% of the patients above the age of18. No correlations were observed between the mean SCys-Cand Uβ2MG levels and pretransfusion hemoglobin and iron load(p>0.05) (Figures 1 and 2). No correlations were found betweenSCys-C and ferritin levels. The assessment of the correlationsbetween the Uβ2MG and ferritin levels in patients revealed thatUβ2MG levels were greater than in the controls, particularlyin those who had a ferritin level of <500 ng/mL or >1000 ng/mL (p=0.001). Although Uβ2MG levels were slightly elevated inthe patients who had ferritin levels between 500 and 1000 ng/mL, the difference between the patients and controls was notstatistically significant (p>0.05).The distribution of patients by their chelation therapy wasas follows: 38 patients (75.5%) were on deferasirox (DFX); 8(16.3%) were on deferiprone (DFP). Among the patients whowere on DFX, 11 (31%) were receiving a dose of 15-20 mg/kg/day, 13 (33%) were receiving a dose of 20-30 mg/kg/day, and 14(36%) were receiving a dose of 30-40 mg/kg/day. When SCys-Cconcentrations were categorized by iron chelation therapy, therewere no differences between the patients who were on DFP andthe controls, while significant differences were found betweenTable 1. Demographics and clinical characteristics of thepatient group.MeanAge 18.4±11.8yearsPretransfusion hemoglobin (g/dL) 8.7±0.78Serum ferritin (ng/mL) 1770.8±1883TSH (mIU/L) 2.7±1.14fT4 (pmol/L) 11.3±2.15n %Age <18 years 25 50>18 years 25 50Sex Male 22 44Female 28 56Ferritin (ng/mL) <500 8 -500-1000 10 -1000-2500 23 ->2500 9 -Chelator Deferasirox 38 75.5Deferiprone 8 16.3None 4 8.2Splenectomy No 33 66Yes 17 34Osteoporosis Yes 24 48Liver iron concentration(mg/g dry weight)No 26 52<7 21 507-15 11 26>15 10 24Non-MRI - 8 -Heart T2* (ms) <10 2 410-20 3 6>20 37 74Non-MRI - 8 -MRI: Magnetic resonance imaging.67
Page 1:
Volume 35 Issue 1 March 2018 80 TLI
Page 4 and 5:
Contact InformationEditorial Corres
Page 6 and 7:
TURKISH JOURNAL OF HEMATOLOGYINSTRU
Page 8 and 9:
subject may be posited. Do not publ
Page 10 and 11:
the questions. You may copy and pas
Page 12 and 13:
Images in Hematology71 Ascites in t
Page 14 and 15:
Gamaletsou MN, et al: Antifungal-Re
Page 16 and 17:
Page 18 and 19:
Page 20 and 21:
Page 22 and 23:
Page 24 and 25:
RESEARCH ARTICLEDOI: 10.4274/tjh.20
Page 26 and 27:
Aydınok Y, et al: A National Regis
Page 28 and 29: Aydınok Y, et al: A National Regis
Page 30 and 31: Aydınok Y, et al: A National Regis
Page 32 and 33: Genitsari S, et al: MSCs in Childho
Page 40 and 41: Tüfekçi Ö, et al: Juvenile Myelo
Page 48 and 49: Vural S, et al: Transformation of M
Page 54 and 55: RESEARCH ARTICLEDOI: 10.4274/tjh.20
Page 56 and 57: Nikkhah H, et al: BM-MSCs’ Effect
Page 62 and 63: Kumar D, et al: NPM1 Mutation Analy
Page 64 and 65: Kumar D, et al: NPM1 Mutation Analy
Page 66 and 67: RESEARCH ARTICLEDOI: 10.4274/tjh.20
Page 68 and 69: Özen M, et al: ABO Blood Group and
Page 74 and 75: Abur Ü, et al: CLLU1 Expression an
Page 76 and 77: Abur Ü, et al: CLLU1 Expression an
Page 80 and 81: Annayev A, et al: Kidney Functions
Page 82 and 83: Annayev A, et al: Kidney Functions
Page 84 and 85: Debski J, et al: Plasma Cell Myelom
Page 86 and 87: Singh C, et al: Pachymeningeal Invo
Page 88 and 89: LETTERS TO THE EDITORTurk J Hematol
Page 106: Advisory Board of This Issue (March
show all

TJH-2018-1-baski

You also want an ePaper? Increase the reach of your titles

Delete template?

Save as template?