Two Different Presentation of C3 Glomerulonephritis Treated with Eculizumab: Two Cases and Brief Overview

MakaleWOS:000925209300013C3 glomerulopathy is a newly defined glomerular disease dominated by C3 complement storage and uncertain C1, C4, and immunoglobin accumulations. Hereditary mutations associated with Complement Factor H (CFH) causing hyperactivation of the alternative complement pathway were identified. Most mutations associated with C3 glomerulopathy are associated with the N-terminal end. Whether mutations are pathogenic or not will direct diagnosis and treatment. We present 2 cases, one 61-year-old and one 24-year-old attending our clinic at different times with hematuria, proteinuria, edema, and kidney failure. Both patients had C3 glomerulopathy diagnosed based on the results of kidney biopsy and were treated with eculizumab. Both cases had CFH-associated mutations

A Rare Case: Improved Heart Failure with Anti-Complement Therapy in Complement-Dependent Hemolytic Uremic Syndrome

MakaleWOS:000925209300014xtrarenal involvement occurs in approximately 20% of patients with complement-mediated hemolytic-uremic syndrome. The involvement is usually of the nervous system, and cardiac involvement occurs in 3%-10% of patients. Cardiac manifestations vary, including myocardial infarction, cardiomyopathy, and acute decompensated heart failure. Among these patients, thrombotic microangiopathy-related cardiac dysfunction is mainly due to the continuous activation of the complement system, which leads to endothelial damage and thrombosis in the coronary microvessels. We wanted to highlight the importance of cardiac evaluation at the time of diagnosis or during follow-up in thrombotic micro-angiopathy patients by presenting a case of heart failure with low ejection fraction in a 24-year-old young patient in whom we detected complement-mediated hemolytic-uremic syndrome, a secondary mutation of complement factor H receptor. It is still an unknown issue because of the rarity of cardiac involvement in complement-mediated hemolytic-uremic syndrome patients. Primary myocardial involvement is increasingly recognized as a possible concomitant feature of hemolytic-uremic syndrome. Failure to perform a detailed cardiac evaluation both at diagnosis and during follow-up in complement-mediated hemolytic-uremic syndrome patients can lead to fatal outcomes. Anti-complement therapy can also lead to good cardiac outcomes in these patients

Evaluation of subclinical atherosclerosis in obese patients with three noninvasive methods: Arterial stiffness, carotid intima-media thickness, and biomarkers of endothelial dysfunction

ABSTRACT Objective: In this study, we aimed to evaluate subclinical atherosclerosis in patients with obesity who had cardiovascular disease risk indicators such as arterial stiffness, which is evaluated using pulse wave velocity (PWV), carotid intima-media thickness (CIMT), and biomarkers of endothelial dysfunction such as endocan, ADAMTS97, and ADAMTS9. Subjects and methods: Sixty obese subjects, including 23 subjects with body mass index (BMI) ≥ 40, 37 subjects with BMI ≥ 30 but < 40, and 60 age-and sex-matched control subjects, were included in our study. Serum endocan, ADAMTS97, and ADAMTS9 levels as well as PWV and CIMT measurements of the subjects in the obese and control groups were performed. Results: In the obesity group, PWV levels were significantly higher than they were in the control group and endocan levels were significantly lower than they were in the control group. When we compared the obese group with BMI ≥ 40 and the control group, the BMI ≥ 40 group had significantly higher PWV and CIMT levels than the control group had, whereas endocan, ADAMTS7, and ADAMTS9 levels were similar to those of the control group. When we compared the obese group with BMI ≥ 30 < 40 to the control group, endocan levels were lower in the group with BMI ≥ 30 < 40, and PWV and CIMT levels were similar to the control group. Conclusions: We found that arterial stiffness and CIMT increased in obese patients with BMI ≥ 40 and that increased arterial stiffness was associated with age, systolic blood pressure, and HBA1C. In addition, we found that the endocan levels were lower in obese patients than they were in nonobese control individuals

The efficacy of cinacalcet in the treatment of hyperparathyroidism in Turkish hemodialysis patient population

WOS: 000393291900012OBJECTIVE: Cinacalcet reduces parathyroid hormone levels by increasing the sensitivity of the parathyroid gland to calcium. in this study, we firstly aimed to evaluate the efficacy of cinacalcet in Turkish hemodialysis patients. MATERIAL and METHODS: 4483 hemodialysis patients were screened and 469 patients who had used cinacalcet were included in the study. the patients were divided into 4 groups according to drug usage durations (Group 1: 3 months, Group 2: 6 months, Group 3: 9 months and Group 4: 12 months). the patients' Parathormone, Ca, P and CaxP levels at the 3rd, 6th, 9th and 12th months were compared to the start of treatment and previous months. RESULTS: the levels of Parathormone, Ca, P and CaxP significantly decreased compared to their initial levels in all groups (from 1412 pg/ml to 1222 pg/mL for Parathormone, p< 0,001) in the 3rd month. However, this reduction was not continued in the subsequent months (Parathormone: 1381 pg/ml for the 12th month). CONCLUSION: Cinacalcet may not provide adequate benefit in control of hyperparathyroidism in Turkish hemodialysis patient population

The Effect of Two Different Bicarbonate-Containing Dialysis Solutions on Acidosis Control in Hemodialysis Patients

AMAÇ: Hemodiyaliz hastalarında kronik bir metabolik asidoz eğilimi söz konusudur. Yüksek olgu sayılı çalışmalarda diyaliz öncesi serum bikarbonat konsantrasyonu ile mortalite arasında ilişki saptanmıştır. Çalışmamızda 32 ve 36 mmol/L bikarbonat içerikli diyaliz solüsyonu kullanımının asidoz kontrolüne etkisi araştırıldı.GErEÇ ve YÖnTEMLEr: Bir merkezde haftada üç kez hemodiyalize giren 91 (43E,48K) hastaya ilk altı ay 32 mmol/L diyalizat, ikinci altı ayda aynı hastalara 36 mmol/L bikarbonat diyalizat kullanılmaya başlandı ve diyaliz öncesi ve sonrası plazma bikarbonat düzeyleri karşılaştırıldı. BULGULAr: İlk altı ayda ortalama bikarbonat düzeyi 20,552,55 mmol/L olup sadece 30 hastada bikarbonat düzeyi 22 mmol/L ve üzerindeydi. İkinci altı aylık dönemde ise ortalama bikarbonat düzeyi 23,593,3 mmol/L olup hedef düzeylerdeydi. Ancak 19 hastada diyaliz sonu ortalama bikarbonat düzeyi 30 mmol/L'nin üzerinde bulundu. Prediyaliz asidozu olan hastaların ortalama interdiyalitik kilo artışı 2,80,5 kg olup, asidozu olmayan gruptan daha yüksekti (p0,05). Prediyaliz üre, Kt/V, albumin, potasyum ve CRP düzeyleri bakımından iki periyot arasında anlamlı farklılık yoktu. SOnUÇ: 36 mmol/L bikarbonat içeren diyaliz solüsyonu kullanımı ile asidoz kontrolü daha iyi yapılmakta ancak hastaların yaklaşık yüzde yirmisinde diyaliz sonunda ciddi metabolik alkaloz gelişmektedir. Bu durum aritmiyi tetikleyebilir. Bu nedenle aritmisi olan veya metabolik alkaloza eğilimi olan hastalarda yüksek bikarbonatlı solüsyon kullanımında bikarbonat içeriğinin ayarlanmasının gerektiğini düşünmekteyiz.OBJECTIVE: In hemodialysis patients; there is a continuing tendency to metabolic acidosis. In studies, the relationship between serum bicarbonate concentration and dialysis mortality has been determined. In our study, the effect of 32 and 36 mmol/L bicarbonate-containing dialysis solution on acidosis control was investigated. MATErıAL and METHODS: In a hemodialysis clinic, a 32 mmol/L bicarbonate-containing dialysate was used in the first six months and a 36 mmol/L bicarbonate-containing dialysate was used in the second six months. The study was performed on 91 (43M/48F) patients. Plasma bicarbonate levels before and after dialysis were compared. rESULTS: In the first period, only 30 patients' bicarbonate levels were 22 mmol/L or more. In the second period, the mean bicarbonate level was 23.59±3.3 mmol/L. However, the level of bicarbonate after dialysis was above 30 mmol/L in 19 patients. In patients with predialysis acidosis, the interdialytic mean weight gain was higher than in the group without acidosis (p>0.05). There were no significant differences between the two periods in terms of predialysis urea, Kt/V, albumin, potassium and CRP levels.CONClUSION: Acidosis control is better with the use of a dialysis solution containing 36 mmol/L bicarbonate. But at the end of dialysis, severe metabolic alkalosis developed on approximately twenty percent of patients. This situation can trigger arrhythmia. We therefore think that it is necessary to adjust the machine bicarbonate in the use of high bicarbonate solution in patients with arrhythmia or alkalosis tendency

Böbrek Nakilli Hastalarda Osteoprotegerin ile Arteryal Damar Sertliği ve Osteoporoz Arasındaki İlişki

Öz AMAÇ: Kardiyovasküler hastalıklar böbrek transplantasyonu (Btx) olan hastalarda mortalitenin ana nedenleri arasındadır. Osteoprotegerin (OPG), osteoblastlar tarafından üretilir ve BTxli hastalarda artmış kardiyovasküler risk ile bağlantılıdır. Normal popülasyonda OPG, serumda NF-?B ligandının (RANKL) bir reseptörü olarak davranır ve bu etkileşimin kemik erimesi ve vasküler fonksiyonlar üzerinde önemli bir rol oynadığı tespit edilmiştir. Literatürde Btxli hastalarda bu etkileşimle ilgili yeterli bilgi bulunmamaktadır. Bu nedenle, çalışmamızda Btxli hastalarda OPG, RANKL, osteoporoz ve arteriyel sertlik arasındaki ilişki araştırılmıştır. GEREÇ ve YÖNTEMLER: Bu kesitsel çalışmaya 80 erişkin Btxli hasta dahil edilmiştir. Femur boynu mineral yoğunluğu dual-enerji X-ışını soğurma (DEXA) yöntemiyle elde edildi. Serum OPG ve RANKL ELISA yöntemi ile ölçüldü. Nabız dalga analizi karotid ve nabız dalga hızı (NDH) makinesi kullanılarak femoral arterlerden ölçüldü. BULGULAR: Hastalar osteopeni/osteoporoz grubu (n:56) ve normal (n: 24) olarak iki gruba ayrıldı. Vücut kitle indeksi normal gruba göre osteopenik/osteoporotik grupta anlamlı olarak daha düşüktü. Nabız dalga hızı ile yaş (r: 0,204, p: 0,072), OPG (r: 0,219, p: 0,052), kalsiyumxfosfat çarpımı (r: 605, p 0,001) ve sistolik kan basıncı (r: 0,198 , p: 0,058) arasında pozitif korelasyon saptanırken, NDH ile RANKL (r: -0,261, p: 0,020) ve kreatinin klirensi (r: -0,220, p: 0,051) arasında negatif korelasyon tespit edildi. Lineer regresyon analizinde nabız dalga hızının bağımsız belirleyicisi olarak kalsiyum x fosfat çarpımı bulundu. Ancak kreatinin klirensi, RANKL osteoprotegerin ve sistolik kan basıncı NDHnın bağımsız öngördürücüsü olarak tespit edilmedi. SONUÇ: Çalışmamızda, böbrek nakilli hastalarda arteriyel sertliği gösteren nabız dalga hızı OPG ve RANKL ile değil fakat kalsiyum ve fosfor çarpımı ile bağımsız olarak ilişkili bulunmuştur. Anahtar sözcükler: Böbrek nakli, Osteoporoz, Osteoprotegerin, RANKL, Nabız dalga hızıAbstract OBJECTIVE: Cardiovascular diseases are the main reason of death in patients with renal transplantation (Rtx). Osteoprotegerin (OPG) is produced by osteoblasts and is linked to increased cardiovascular risk in Rtx. OPG acts as a decoy receptor binding receptor activator of nuclear factor kappa-B ligand (RANKL) and this interaction plays a role in bone resorption and vascular function. This study aimed to investigate the relation between OPG, RANKL, osteoporosis and arterial stiffness in Rtx patients. MATERIAL and METHODS: This cross-sectional study included 80 adult Rtx recipients. Femoral neck mineral density was obtained by dual-energy X-ray absorptiometry. Serum OPG and RANKL were measured by the ELISA method. Pulse-wave analysis was measured in the carotid and femoral arteries using a pulse wave velocity (PWV) machine. RESULTS: Patients were divided into two groups as normal (n:24) and osteopenia/osteoporosis group (n:56). Body mass index was significantly lower in the osteopenic/osteoporotic group compared to the normal group. Pulse wave velocity was positively correlated with age (r:0.204,p:0.072), osteoprotegerin (r:0.219,p:0.052), calcium x phosphate product (r:0.605,p:>0.001), and systolic blood pressure (r:0.198,p:0.058) and negatively correlated with RANKL (r:-0.261,p:0.020) and creatinine clearance (r:-0.220,p:0.051). PWV was independently predicted by calcium x phosphate product but not creatinine clearance, RANKL, osteoprotegerin and systolic blood pressure. CONCLUSION: In our study, serum calcium x phosphate product but not OPG and RANKL levels were found to be the main predictor of arterial stiffness in Rtx patients. Key words: Renal transplantation, Osteoporosis, Osteoprotegerin, RANKL, Pulse wave velocit

The Relationship of Red-Cell Distribution Width and Carotid Intima Media in Chronic Kidney Disease

AMAÇ: Eritrosit dağılım genişliği (RDW) rutin olarak tam kan sayımından elde edilen bir parametredir. Son yıllarda yapılan çalışmalarda kardiyovasküler hastalığı olanlarda ve sağlıklı gönüllülerde RDW'nin kardiyovasküler hastalıklarla ilişkili olduğu gösterilmiştir. Böbrek yetmezliği hastalarında RDW ile ilgili yeterli çalışma yoktur. Biz kronik böbrek yetmezliği hastalarında RDW'nin aterosklerozun bir ön göstergesi olan karotis intima media kalınlığı (IMK) ile ilişkisini araştırdık.GEREÇ ve yÖNTEMLER: 30 sağlıklı kontrol, 30 kronik böbrek yetmezliği hastası, 37 hemodiyaliz hastası çalışmaya alındı. Hastaların ultrasonografik yöntem ile IMK değerleri ölçüldü. BULGULAR: CRP (p0,039), hemoglobin (p0,001), IMK (p0,001), RDW(p0,001), üre (p0,001), kreatinin (p0,001), albumin (p0,001), ürik asit (p0,001) ve ferritin (p0,001) değerlerinde her üç grup arasında istatistiksel olarak anlamlı farklılık tespit edildi. Posthok analizde IMK değeri prediyaliz grubunda (p0,001) ve hemodiyaliz grubunda (p0,001) sağlıklı kontrollere göre istatistiksel olarak anlamlı yüksek tespit edildi. Hemodiyaliz ve prediyaliz gruplarının ortalama IMK değerleri arasında istatistiksel farklılık tespit edilmedi (p:0,988). RDW değeri hemodiyaliz grubunda control grubuna (p:0,046) ve prediyaliz grubuna (p:0,03) göre yüksek saptandı. RDW değeri prediyaliz grubunda control grubuna göre istatistiksel anlalmlılık sınırına yakın yüksek tespit edildi (p: 0,067). RDW değeri IMK (r: 0,356 P: 0,012 ) ve CRP (r: 0,361 P: 0,004 ) ile pozitif korelasyon, gösterdi.SONUÇ: Kronik böbrek yetmezliği hastalarında RDW inflamasyon ve intima media kalınlığı ile ilişkilidir.OBJECTIVE: Red-cell distribution width (RDW) is a parameter routinely used for diagnosis of different anemia types. Recent studies have shown the RDW relationship with mortality in general population and patients with cardiovascular disease. However, the number of studies on RDW in chronic kidney disease (CKD) is insufficient. We evaluated the relationship between RDW and carotid intima media thickness (IMT), which is a predictor of atherosclerosis, in patients with CKD.MATERIAL and METHODS: 30 healthy controls, 30 patients with CKD, 37 hemodialysis patients were included. IMT was measured with ultrasonography. RESULTS: We identified statistically significant differences in CRP (p: 0.039), hemoglobin (p>0.001), IMK (p>0.001), RDW (p>0.001), urea (p>0.001), creatinine (p>0.001), albumin (p>0.001), uric acid (p>0.001) and ferritin (p>0.001) levels among three groups. In post hoc analysis, the IMK value was statistically significantly higher in the predialysis (p>0.001) and hemodialysis group than healthy controls. IMK value was not statistically different in the hemodialysis group than the predialysis group (p: 0.988). The RDW value was higher in the predialysis group than controls with a trend to statistical significance (p: 0.067). RDW value showed positive correlation with IMK (r: 0.356 P: 0.012) and CRP (r: 0.361 P: 0.004). CONCLUSION: RDW is associated with inflammation and intima media thickness in patients with CKD

The Relationship of Red-Cell Distribution Width and Carotid Intima Media in Chronic Kidney Disease

AMAÇ: Eritrosit dağılım genişliği (RDW) rutin olarak tam kan sayımından elde edilen bir parametredir. Son yıllarda yapılan çalışmalarda kardiyovasküler hastalığı olanlarda ve sağlıklı gönüllülerde RDW'nin kardiyovasküler hastalıklarla ilişkili olduğu gösterilmiştir. Böbrek yetmezliği hastalarında RDW ile ilgili yeterli çalışma yoktur. Biz kronik böbrek yetmezliği hastalarında RDW'nin aterosklerozun bir ön göstergesi olan karotis intima media kalınlığı (IMK) ile ilişkisini araştırdık.GEREÇ ve yÖNTEMLER: 30 sağlıklı kontrol, 30 kronik böbrek yetmezliği hastası, 37 hemodiyaliz hastası çalışmaya alındı. Hastaların ultrasonografik yöntem ile IMK değerleri ölçüldü. BULGULAR: CRP (p=0,039), hemoglobin (p<0,001), IMK (p<0,001), RDW(p<0,001), üre (p<0,001), kreatinin (p<0,001), albumin (p<0,001), ürik asit (p<0,001) ve ferritin (p<0,001) değerlerinde her üç grup arasında istatistiksel olarak anlamlı farklılık tespit edildi. Posthok analizde IMK değeri prediyaliz grubunda (p<0,001) ve hemodiyaliz grubunda (p<0,001) sağlıklı kontrollere göre istatistiksel olarak anlamlı yüksek tespit edildi. Hemodiyaliz ve prediyaliz gruplarının ortalama IMK değerleri arasında istatistiksel farklılık tespit edilmedi (p:0,988). RDW değeri hemodiyaliz grubunda control grubuna (p:0,046) ve prediyaliz grubuna (p:0,03) göre yüksek saptandı. RDW değeri prediyaliz grubunda control grubuna göre istatistiksel anlalmlılık sınırına yakın yüksek tespit edildi (p: 0,067). RDW değeri IMK (r: 0,356 P: 0,012 ) ve CRP (r: 0,361 P: 0,004 ) ile pozitif korelasyon, gösterdi.SONUÇ: Kronik böbrek yetmezliği hastalarında RDW inflamasyon ve intima media kalınlığı ile ilişkilidir.OBJECTIVE: Red-cell distribution width (RDW) is a parameter routinely used for diagnosis of different anemia types. Recent studies have shown the RDW relationship with mortality in general population and patients with cardiovascular disease. However, the number of studies on RDW in chronic kidney disease (CKD) is insufficient. We evaluated the relationship between RDW and carotid intima media thickness (IMT), which is a predictor of atherosclerosis, in patients with CKD.MATERIAL and METHODS: 30 healthy controls, 30 patients with CKD, 37 hemodialysis patients were included. IMT was measured with ultrasonography. RESULTS: We identified statistically significant differences in CRP (p: 0.039), hemoglobin (p<0.001), IMK (p<0.001), RDW (p<0.001), urea (p<0.001), creatinine (p<0.001), albumin (p<0.001), uric acid (p<0.001) and ferritin (p<0.001) levels among three groups. In post hoc analysis, the IMK value was statistically significantly higher in the predialysis (p<0.001) and hemodialysis group than healthy controls. IMK value was not statistically different in the hemodialysis group than the predialysis group (p: 0.988). The RDW value was higher in the predialysis group than controls with a trend to statistical significance (p: 0.067). RDW value showed positive correlation with IMK (r: 0.356 P: 0.012) and CRP (r: 0.361 P: 0.004). CONCLUSION: RDW is associated with inflammation and intima media thickness in patients with CKD

Diyaliz hastalarında asimetrik dimetilarjinin ve homosistein düzeyleri

Kardiyovasküler hastalıklar ve endotelyal disfonksiyon son dönem böbrek yetmezliği hastalarında en önemli ölüm nedenleridir. Periton diyalizi ve hemodiyaliz gibi tedavi yöntemlerinin farklı parametreler üzerine farklı etkileri vardır. Asimetrik dimetilarjinin endojen nitrik oksit sentaz inhibitörüdür ve kronik böbrek yetmezliğinin progresyonunda yeni bir belirteç olduğu gösterilmiştir. Homosistein endotel hasarı, damar düz kas hücre proliferasyonu ve koagülasyon anormallikleri yoluyla trombogenez ve atero- geneze neden olur. Daha önceki çalışmalarda periton diyalizi (PD) ve hemodiyalizin (HD) oksidan ve antioksidan sistemler üzerine etkisiyle ilgili çelişkili bulgular vardır. Bu çalışmada HD, PD ve sağlıklı kişilerde ADMA, homosistein ve CRP düzeylerini belirlemeyi amaçladık. Çalışmaya 44 PD (23E,21K), 26HD (13E,13K) hastası ve 29 (15E,14K) sağlıklı kişi katıldı. ADMA, homosistein, arjinin ve CRP düzeyleri ölçüldü. Son dönem böbrek yetmezliği (SDBY) hastalarının ADMA, homosistein ve CRP düzeyleri sağlıklı kontrol grubuna göre yüksek (p0.001), arjinin düzeyleri düşük olarak bulundu(p0.001). HD ve PD gruplarında ADMA, homosistein ve CRP seviyeleri açısından farklılık bulunamadı (sırasıyla p:0.287, p: 0.587, p: 0.835) Bizim sonuçlarımız HD,PD hastalarında sağlıklı kontrole göre ADMA ve homosistein düzeylerinin yüseldiğini gösterdi. Bu bulgular; diyaliz hastalarında endotel disfonksiyonu, inflamasyon ve oksidatif stresin arttığını ve ADMA ve homosistein düzeylerinin diyaliz tedavi yönteminden etkilenmediğini gös- termektedirCardiovascular diseases and endothelial disfunction are major causes of mortality in patients with end stage renal disease (ESRD). Treatment strategies like continuous ambulatory peritoneal dialysis (CAPD) and hemodialysis (HD) have different effects on dif- ferent parameters. Asymmetric dimethylarginine (ADMA) is an endogenous nitric oxide (NO) synthase inhibitor and it has been reported to be a novel marker for the progression of chronic kidney disease (CKD). Homocysteine is believed to cause atherogen- esis and thrombogenesis via endothelial damage, vascular smooth muscle proliferation and coagulation abnormalities. In previ- ous studies, conflicting findings have been reported about the effect of HD and CAPD on oxidant and antioxidant systems. In this study, we aimed to investigate ADMA, homocysteine and C- reactive protein (CRP) levels in patients with ESRD having HD and CAPD treatment and healthy individuals. This study was performed on 44 (23M, 21F) CAPD patients, 26 (13M, 13F) HD patients and 29 (15M, 14F) age and sex matched healthy control subjects. The lipid profile, ADMA, homocysteine, arginine and CRP levels were measured. Serum ADMA, homocysteine and CRP levels of the ESRD patients were significantly higher, whereas serum arginine levels were significantly lower in both HD and CAPD patients compared to control subjects. No differences were found between serum ADMA, homocysteine and CRP levels of the CAPD and HD patients. Our results suggest that ADMA, homocysteine and CRP levels were increased in HD and CAPD patients compared to the control subjects. These findings suggest that ESRD patients are prone to inflammation, oxidative stress and endothelial dysfunction. We conclude that endothelial dysfunction, inflammation and oxidative stress are increased in dialysis patients and ADMA concentrations are not affected by the modality of dialysis treatment