Pregnancy and its outcomes in hemodialysis patients in Turkey.

BACKGROUND/AIM: This study aimed to investigate pregnancy frequency and evaluate the factors affecting live births in hemodialysis (HD) patients. MATERIALS AND METHODS: Female HD patients whose pregnancy was retrospectively reported between January 1, 2014, and December 31, 2019. The duration of HD, primary disease, whether the pregnancy resulted in abortion, stillbirth, or live birth, whether the HD duration was prolonged after diagnosing the pregnancy and whether it accompanied preeclampsia were recorded. RESULTS: In this study, we reached 9038 HD female patients? data in the study. A total of 235 pregnancies were detected in 145 patients. The mean age was 35.42 (35 ± 7.4) years. The mean age at first gestation was 30.8 ± 6.5 years. The average birth week was 32 (28 - 36) weeks. 53.8% (no = 78) of the patients had live birth, 51.7% (no = 70) had at least one abortion in the first 20 weeks, and 13.1% (no = 19) had at least one stillbirth after 20 weeks. The rate of patients' increased numbers of dialysis sessions during pregnancy was 71.7%. The abortion rate was 22.4% in those with increased HD sessions, whereas 79.3% in those not increased HD sessions (p < 0.001). Live birth frequency was 67.2% in the increased HD sessions group and 3.4% in those who did not differ in HD sessions (p < 0.001). CONCLUSION: For the first time, we reported pregnancy outcomes in HD female patients, covering all regions of Turkey. It has been observed that; increasing the number of HD sessions in dialysis patients will decrease fetal and maternal complications and increase live birth rates.

Characteristics and outcomes of acute kidney injury in hospitalized COVID-19 patients: A multicenter study by the Turkish society of nephrology.

BACKGROUND: Acute kidney injury (AKI) is common in coronavirus disease-2019 (COVID-19) and the severity of AKI is linked to adverse outcomes. In this study, we investigated the factors associated with in-hospital outcomes among hospitalized patients with COVID-19 and AKI. METHODS: In this multicenter retrospective observational study, we evaluated the characteristics and in-hospital renal and patient outcomes of 578 patients with confirmed COVID-19 and AKI. Data were collected from 34 hospitals in Turkey from March 11 to June 30, 2020. AKI definition and staging were based on the Kidney Disease Improving Global Outcomes criteria. Patients with end-stage kidney disease or with a kidney transplant were excluded. Renal outcomes were identified only in discharged patients. RESULTS: The median age of the patients was 69 years, and 60.9% were males. The most frequent comorbid conditions were hypertension (70.5%), diabetes mellitus (43.8%), and chronic kidney disease (CKD) (37.6%). The proportions of AKI stages 1, 2, and 3 were 54.0%, 24.7%, and 21.3%, respectively. 291 patients (50.3%) were admitted to the intensive care unit. Renal improvement was complete in 81.7% and partial in 17.2% of the patients who were discharged. Renal outcomes were worse in patients with AKI stage 3 or baseline CKD. The overall in-hospital mortality in patients with AKI was 38.9%. In-hospital mortality rate was not different in patients with preexisting non-dialysis CKD compared to patients without CKD (34.4 versus 34.0%, p = 0.924). By multivariate Cox regression analysis, age (hazard ratio [HR] [95% confidence interval (95%CI)]: 1.01 [1.0-1.03], p = 0.035], male gender (HR [95%CI]: 1.47 [1.04-2.09], p = 0.029), diabetes mellitus (HR [95%CI]: 1.51 [1.06-2.17], p = 0.022) and cerebrovascular disease (HR [95%CI]: 1.82 [1.08-3.07], p = 0.023), serum lactate dehydrogenase (greater than two-fold increase) (HR [95%CI]: 1.55 [1.05-2.30], p = 0.027) and AKI stage 2 (HR [95%CI]: 1.98 [1.25-3.14], p = 0.003) and stage 3 (HR [95%CI]: 2.25 [1.44-3.51], p = 0.0001) were independent predictors of in-hospital mortality. CONCLUSIONS: Advanced-stage AKI is associated with extremely high mortality among hospitalized COVID-19 patients. Age, male gender, comorbidities, which are risk factors for mortality in patients with COVID-19 in the general population, are also related to in-hospital mortality in patients with AKI. However, preexisting non-dialysis CKD did not increase in-hospital mortality rate among AKI patients. Renal problems continue in a significant portion of the patients who were discharged.

Characteristics and outcomes of hospitalised older patients with chronic kidney disease and COVID-19: A multicenter nationwide controlled study.

OBJECTIVE: Older adults with co-morbidities have been reported to be at higher risk for adverse outcomes of coronavirus disease 2019 (COVID-19). The characteristics of COVID-19 in older patients and its clinical outcomes in different kidney disease groups are not well known. METHODS: Data were retrieved from a national multicentric database supported by Turkish Society of Nephrology, which consists of retrospectively collected data between 17 April 2020 and 31 December 2020. Hospitalised patients aged 18 years or older with confirmed COVID-19 diagnosis suffering from stage 3-5 chronic kidney disease (CKD) or on maintenance haemodialysis (HD) treatment were included in the database. Non-uraemic hospitalised patients with COVID-19 were also included as the control group. RESULTS: We included 879 patients [388 (44.1%) female, median age: 63 (IQR: 50-73) years]. The percentage of older patients in the CKD group was 68.8% (n = 188/273), in the HD group was 49.0% (n = 150/306) and in the control group was 30.4% (n = 70/300). Co-morbidities were higher in the CKD and HD groups. The rate of presentation with severe-critical disease was higher in the older CKD and HD groups (43.6%, 55.3% and 16.1%, respectively). Among older patients, the intensive care unit (ICU) admission rate was significantly higher in the CKD and HD groups than in the control group (38.8%, 37.3% and 15.7%, respectively). In-hospital mortality or death and/or ICU admission rates in the older group were significantly higher in the CKD (29.3% and 39.4%) and HD groups (26.7% and 30.1%) compared with the control group (8.6% and 17.1%). In the multivariate analysis, in-hospital mortality rates in CKD and HD groups were higher than control group [hazard ratio (HR): 4.33 (95% confidence interval [CI]: 1.53-12.26) and HR: 3.09 (95% CI: 1.04-9.17), respectively]. CONCLUSION: Among older COVID-19 patients, in-hospital mortality is significantly higher in those with stage 3-5 CKD and on maintenance HD than older patients without CKD regardless of demographic characteristics, co-morbidities, clinical and laboratory data on admission.

Impact of hospital-acquired acute kidney injury on Covid-19 outcomes in patients with and without chronic kidney disease: a multicenter retrospective cohort study

Background/aim: Hospital-acquired acute kidney injury (HA-AKI) may commonly develop in Covid-19 patients and is expected to have higher mortality. There is little comparative data investigating the effect of HA-AKI on mortality of chronic kidney disease (CKD) patients and a control group of general population suffering from Covid-19. Materials and methods: HA-AKI development was assessed in a group of stage 3­5 CKD patients and control group without CKD among adult patients hospitalized for Covid-19. The role of AKI development on the outcome (in-hospital mortality and admission to the intensive care unit [ICU]) of patients with and without CKD was compared. Results: Among 621 hospitalized patients (age 60 [IQR: 47­73]), women: 44.1%), AKI developed in 32.5% of the patients, as stage 1 in 84.2%, stage 2 in 8.4%, and stage 3 in 7.4%. AKI developed in 48.0 % of CKD patients, whereas it developed in 17.6% of patients without CKD. CKD patients with HA-AKI had the highest mortality rate of 41.1% compared to 14.3% of patients with HA-AKI but no CKD (p < 0.001). However, patients with AKI+non-CKD had similar rates of ICU admission, mechanical ventilation, and death rate to patients with CKD without AKI. Adjusted mortality risks of the AKI+non-CKD group (HR: 9.0, 95% CI: 1.9­44.2) and AKI+CKD group (HR: 7.9, 95% CI: 1.9­33.3) were significantly higher than that of the non-AKI+non-CKD group. Conclusion: AKI frequently develops in hospitalized patients due to Covid-19 and is associated with high mortality. HA-AKI has worse outcomes whether it develops in patients with or without CKD, but the worst outcome was seen in AKI+CKD patients.

Evaluation of Dipper and Non-dipper Blood Pressure Patterns and Quality of Life Among Patients with Chronic Obstructive Pulmonary Disease. / Avaliação de Padrões Pressóricos Dipper e Não-Dipper e Qualidade de Vida entre Pacientes com Doença Pulmonar Obstrutiva Crônica.

BACKGROUND: Non-dipper blood pressure is defined by less than a 10% reduction in nighttime blood pressure, and it is associated with cardiovascular disease. Inflammation is thought to play a role in the pathogenesis of both chronic obstructive pulmonary disease (COPD) and non-dipper blood pressure pattern, and both diseases are associated with lower quality of life. OBJECTIVE: The aim of this study was to investigate the effects of non-dipper blood pressure pattern in patients with COPD. METHODS: A cross-sectional study was carried out with 142 patients with COPD. The Saint George Respiratory Questionnaire and the Euro Quality of Life Scale were used to collect data. To understand arterial stiffness, the augmentation index and pulse wave velocity were measured, and 24-hour ambulatory blood pressure monitoring was subsequently performed. A multivariable logistic regression model was used to understand the relationship between different independent variables and blood pressure pattern. P values lower than 0.05 were considered statistically significant. RESULTS: As a result, 76.1% (n = 108) of the patients had non-dipper blood pressure pattern. Non-dipper patients had higher C-reactive protein (OR:1.123; 95% CI:1.016;1.242), augmentation index (OR: 1.057; 95% CI: 1.011;1.105) and Saint George Respiratory Questionnaire total score (OR: 1.021; 95% CI: 1.001;1.042) than dipper patients. Also, as the number of people living at home increased, non-dipper blood pressure pattern was found to be more frequent (OR: 1.339; 95% CI: 1.009;1.777). CONCLUSION: Non-dipper blood pressure pattern may increase cardiovascular risk by triggering inflammation and may adversely affect the prognosis of COPD by lowering the disease-related quality of life. (Arq Bras Cardiol. 2020; [online].ahead print, PP.0-0).

FUNDAMENTO: O padrão pressórico não-dipper é definido por uma redução inferior a 10% na pressão arterial noturna e está associado a doenças cardiovasculares. Acredita-se que a inflamação desempenhe um papel na patogênese da doença pulmonar obstrutiva crônica (DPOC) e no padrão pressórico não-dipper e ambas as doenças estão associadas a uma qualidade de vida mais baixa. OBJETIVO: O objetivo deste estudo foi o de investigar os efeitos do padrão pressórico não-dipper em pacientes com DPOC. MÉTODOS: Foi realizado um estudo transversal incluindo 142 pacientes com DPOC. O Questionário Respiratório de Saint George e a Escala de Qualidade de Vida Euro foram utilizados para a coleta de dados. Para entender a rigidez arterial, o índice de aumento e a velocidade da onda de pulso foram medidos; subsequentemente, foi realizada a monitorização ambulatorial da pressão arterial de 24 horas. Foi aplicado um modelo de regressão logística multivariável para entender a relação entre as diferentes variáveis independentes e o padrão pressórico. Foram considerados estatisticamente significativos valores de p inferiores a 0,05. RESULTADOS: Como resultado, 76,1% (n = 108) dos pacientes apresentaram o padrão pressórico não-dipper. Os pacientes com padrão não-dipper apresentaram valores mais altos de proteína C reativa (OR: 1,123; IC 95%: 1,016;1,242), índice de aumento (OR: 1,057; IC 95%: 1,011;1,105) e pontuação total no Questionário Respiratório de Saint George (OR: 1,021; IC 95%: 1,001;1,042), em comparação com os pacientes com padrão dipper. Adicionalmente, com o aumento do número de pessoas que habitavam o domicílio, verificou-se que o padrão pressórico não-dipper era mais frequente (OR: 1,339; IC 95%:1,009;1,777). CONCLUSÃO: O padrão pressórico não-dipper pode aumentar o risco cardiovascular ao desencadear a inflamação e pode afetar adversamente o prognóstico da DPOC diminuindo a qualidade de vida relacionada à doença. (Arq Bras Cardiol. 2020; [online].ahead print, PP.0-0).

Mortality analysis of COVID-19 infection in chronic kidney disease, haemodialysis and renal transplant patients compared with patients without kidney disease: a nationwide analysis from Turkey.

BACKGROUND: Chronic kidney disease (CKD) and immunosuppression, such as in renal transplantation (RT), stand as one of the established potential risk factors for severe coronavirus disease 2019 (COVID-19). Case morbidity and mortality rates for any type of infection have always been much higher in CKD, haemodialysis (HD) and RT patients than in the general population. A large study comparing COVID-19 outcome in moderate to advanced CKD (Stages 3-5), HD and RT patients with a control group of patients is still lacking. METHODS: We conducted a multicentre, retrospective, observational study, involving hospitalized adult patients with COVID-19 from 47 centres in Turkey. Patients with CKD Stages 3-5, chronic HD and RT were compared with patients who had COVID-19 but no kidney disease. Demographics, comorbidities, medications, laboratory tests, COVID-19 treatments and outcome [in-hospital mortality and combined in-hospital outcome mortality or admission to the intensive care unit (ICU)] were compared. RESULTS: A total of 1210 patients were included [median age, 61 (quartile 1-quartile 3 48-71) years, female 551 (45.5%)] composed of four groups: control (n = 450), HD (n = 390), RT (n = 81) and CKD (n = 289). The ICU admission rate was 266/1210 (22.0%). A total of 172/1210 (14.2%) patients died. The ICU admission and in-hospital mortality rates in the CKD group [114/289 (39.4%); 95% confidence interval (CI) 33.9-45.2; and 82/289 (28.4%); 95% CI 23.9-34.5)] were significantly higher than the other groups: HD = 99/390 (25.4%; 95% CI 21.3-29.9; P < 0.001) and 63/390 (16.2%; 95% CI 13.0-20.4; P < 0.001); RT = 17/81 (21.0%; 95% CI 13.2-30.8; P = 0.002) and 9/81 (11.1%; 95% CI 5.7-19.5; P = 0.001); and control = 36/450 (8.0%; 95% CI 5.8-10.8; P < 0.001) and 18/450 (4%; 95% CI 2.5-6.2; P < 0.001). Adjusted mortality and adjusted combined outcomes in CKD group and HD groups were significantly higher than the control group [hazard ratio (HR) (95% CI) CKD: 2.88 (1.52-5.44); P = 0.001; 2.44 (1.35-4.40); P = 0.003; HD: 2.32 (1.21-4.46); P = 0.011; 2.25 (1.23-4.12); P = 0.008), respectively], but these were not significantly different in the RT from in the control group [HR (95% CI) 1.89 (0.76-4.72); P = 0.169; 1.87 (0.81-4.28); P = 0.138, respectively]. CONCLUSIONS: Hospitalized COVID-19 patients with CKDs, including Stages 3-5 CKD, HD and RT, have significantly higher mortality than patients without kidney disease. Stages 3-5 CKD patients have an in-hospital mortality rate as much as HD patients, which may be in part because of similar age and comorbidity burden. We were unable to assess if RT patients were or were not at increased risk for in-hospital mortality because of the relatively small sample size of the RT patients in this study.

Splenic infarction is not rare in granulomatosis with polyangiitis.

Splenic involvement is rarely reported in patients with ANCA-associated vasculitides (AAVs), particularly in those with granulomatosis with polyangiitis (GPA) and is in fact considered to be underestimated. We aimed to investigate the frequency of splenic lesions-mainly infarction-and related factors in patients with AAVs. Patients with AAV whose abdominal or thoracic computed tomographies (CTs) were performed at or after diagnosis were included in the study. CT images were examined for splenic lesions. Overall, 69 patients (median age at diagnosis 54 [IQR 24] years; 55% with GPA, 29% with microscopic polyangiitis, and 16% with renal-limited disease) were included in the analysis. Splenic pathologies were detected in 19 (28%) patients; 12/19 (63%) splenomegaly and 7/19 (37%) splenic infarction. All patients with splenic infarction exhibited GPA with PR3-ANCA-positive serology. Three of these seven patients had autosplenectomy. Patients with splenic infarction were younger at diagnosis (p = 0.018) with also significantly higher ear-nose-throat (ENT) (%100 vs 37; p = 0.002) and eye involvement (%50 vs %12; p = 0.044) than patients without splenic infarction. Splenic pathologies are not rare in AAVs. Furthermore, infarction can help separate GPA from MPA. In young patients with GPA, particularly those with ENT and eye involvements, physicians should consider splenic infarction.Key Points• Splenic infarction is more common than previously thought in ANCA-associated vasculitides, particularly in granulomatosis with polyangiitis.• Detecting splenic infarction can help differentiate granulomatosis with polyangiitis from other subgroups.

The role of mammalian target of rapamycin pathway in the pathogenesis of pauci-immune glomerulonephritis.

Background: The characteristic lesion of pauci-immune glomerulonephritis is focal necrotizing and crescentic glomerulonephritis. The underlying mechanisms in the formation or progression of crescent formation need further investigations. Therefore, we aimed to evaluate the role of mammalian target of rapamycin (mTOR), which might be a potential therapeutic target, in kidney biopsies of patients with pauci-immune glomerulonephritis. Methods: The patients diagnosed as pauci-immune glomerulonephritis at an outpatient nephrology clinic were retrospectively reviewed and those patients who had a kidney biopsy before receiving an immunosuppressive treatment were included in the study. Kidney biopsy specimens were immunohistochemically stained with mTOR, antibodies of phosphatase and tensin homolog (PTEN) and transforming growth factor-ß (TGF-ß) and scored by an experienced renal pathologist. Results: In total, 54 patients with pauci-immune glomerulonephritis (28 [52%] female) were included. According to the histopathologic examination, 22% of our cases were classified as focal, 33% crescentic, 22% mixed, and 22% as sclerotic. The mTOR was expressed in substantial percentages of glomeruli of patients with pauci-immune glomerulonephritis. However, we observed PTEN expression in all samples and mTOR in all tubulointerstitial areas. mTOR expression was found to be related with the presence of crescentic and sclerotic changes observed in glomeruli and the degree of fibrosis in interstitial areas. Serum creatinine level or response to treatment was not found to be associated with mTOR pathway expression. Conclusion: Our results suggest that mTOR pathway may play role in the pathogenesis of pauci-immune glomerulonephritis, besides targeting this signaling may be an alternative option for those patients.

Insulin Resistance in Nonobese Renal Allograft Recipients on Maintenance Doses of Cyclosporine or Tacrolimus.

PURPOSE: In comparison to cyclosporine (CsA), tacrolimus (Tac) seems to be more diabetogenic in renal transplant recipients, and post-transplant diabetes mellitus is more common in patients using Tac, especially during the first year after transplantation. However, at maintenance doses, there are no comparative data of insulin resistance (IR) in patients using Tac or CsA. The purpose of this study was to investigate the IR indexes in patients on maintenance doses of CsA or Tac. METHODS: Forty-five nondiabetic and nonobese renal transplant recipients participated in the study (M:F, 30:15; age, 36 ± 9 years); 27 patients were on CsA, and 18 were on Tac. All had stable graft function, were transplanted at least 6 months previously, and were receiving maintenance doses of steroids (5.0 mg/d), azathioprine or mycophenolate mofetil, and calcineurin inhibitors (CsA [2.14 ± 0.46 mg/kg/d] or Tac [0.06 ± 0.03 mg/kg/d]). IR was evaluated by the homeostasis model assessment (HOMA) index and composite body insulin sensitivity index. RESULTS: We did not determine any significant difference in the HOMA and composite body insulin sensitivity index levels among patients using CsA or Tac (1.5 ± 1.3 vs 1.5 ± 1.1, P > .05, and 9.9 ± 5.8 vs 14.6 ± 11.7, P > .05, respectively). There was a significant correlation between creatinine and HOMA values. CONCLUSION: There was no difference in IR indexes in renal transplant recipients receiving maintenance doses of either CsA or Tac.

Acute renal infarction in Turkey: a review of 121 cases.

PURPOSE: Renal infarction is a clinical condition which is caused by renal artery occlusion and leads to permanent renal parenchymal damage. In the literature, there are generally case reports on this subject, and few studies that include a large group of patients. Therefore, we aimed to present the data of a large group of patients who were diagnosed with acute renal infarction in our country in this retrospective study. METHODS: The data of patients who were diagnosed with acute renal infarction according to clinical and radiological findings in Turkey in the last 3 years were examined. For this purpose, we contacted with more than 40 centers in 7 regions and obtained support from clinically responsible persons. Demographic data of patients, laboratory data at the time of diagnosis, tests performed for etiologic evaluation, given medications, and patients' clinical status during follow-up were obtained from databases and statistical analysis was performed. RESULTS: One-hundred and twenty-one patients were included in the study. The mean age was 53 ± 1.4 (19-91) years. Seventy-one (58.7%) patients were male, 18 (14.9%) had diabetes, 53 (43.8%) had hypertension, 36 (30%) had atrial fibrillation (AF), and 6 had a history of lupus + antiphospholipid syndrome (APS). Forty-five patients had right renal infarction, 50 patients had left renal infarction, and 26 (21.5%) patients had bilateral renal infarction. The examinations for the ethiologies revealed that, 36 patients had thromboemboli due to atrial fibrillation, 10 patients had genetic anomalies leading to thrombosis, 9 patients had trauma, 6 patients had lupus + APS, 2 patients had hematologic diseases, and 1 patient had a substance abuse problem. Fifty-seven (57%) patients had unknown. The mean follow-up period was 14 ± 2 months. The mean creatinine and glomerular filtration rate (GFR) values at 3 months were found to be 1.65 ± 0.16 mg/dl and 62 ± 3 ml/min, respectively. The final mean creatinine and GFR values were found to be 1.69 ± 0.16 mg/dl and 62 ± 3 ml/min, respectively. CONCLUSIONS: Our study is the second largest series published on renal infarction in the literature. More detailed studies are needed to determine the etiological causes of acute renal infarction occurring in patients.

Role of the mTOR pathway in minor salivary gland changes in Sjogren's syndrome and systemic sclerosis.

BACKGROUND: To examine the activity of the mammalian target of rapamycin (mTOR) pathway and its regulators, transforming growth factor (TGF)-ß1 and phosphatase and tensin homolog (PTEN), in minor salivary gland biopsies of Sjogren's syndrome (SS) and systemic sclerosis (SSc) patients. METHODS: We retrospectively evaluated SS, SSc, and SS-SSc overlap patients admitted to our outpatient rheumatology clinic between January 2007 and December 2015 who underwent a minor salivary gland biopsy. Patient demographics and some clinical features were obtained from hospital records. Immunohistochemistry was used to analyze total mTOR, total PTEN, and TGF-ß1 expression in the biopsied tissues. The biopsy specimens were also examined for the presence and degree of fibrosis. RESULTS: Minor salivary gland biopsies of 58 SS, 14 SSc, and 23 SS-SSc overlap patients were included in the study. There was no significant difference in mTOR expression between these groups (P = 0.622). PTEN protein was expressed in 87.2% of patients with SS, 57.9% with overlap syndrome, and 100% of the SSC patients, and these differences were statistically different (P = 0.023). Although ductal epithelial TGF-ß1 expression was similar between the groups (P = 0.345), acinar cell expression was found to be more frequent in the SSc (72.7%) and overlap patients (85.7%) in comparison with the SS cases (58.2%; P = 0.004). CONCLUSION: mTOR may be one of the common pathways in the pathology of both SS and SSc. Hence, there may be a role for mTOR inhibitors in the treatment of both diseases. Additionally, PTEN and TGF-ß1 expression may be a distinctive feature of SSc.

Screening Fabry's disease in chronic kidney disease patients not on dialysis: a multicenter study.

OBJECTIVES: Fabry's disease is an X-linked inherited, rare, progressive, lysosomal storage disorder, affecting multiple organs due to the deficient activity of α-galactosidase A (α-Gal A) enzyme. The prevalence has been reported to be 0.15-1% in hemodialysis patients; however, the information on the prevalence in chronic kidney disease not on dialysis is lacking. This study aimed to determine the prevalence of Fabry's disease in chronic kidney disease. METHODS: The patients older than 18 years, enclosing KDIGO 2012 chronic kidney disease definitions, not on dialysis, were enrolled. Dried blood spots on Guthrie papers were used to analyze α-Gal A enzyme and genetic analysis was performed in individuals with enzyme activity ≤1.2 µmol/L/h. RESULTS: A total of 1453 chronic kidney disease patients not on dialysis from seven clinics in Turkey were screened. The mean age of the study population was 59.3 ± 15.9 years. 45.6% of patients were female. The creatinine clearance of 77.3% of patients was below 60 mL/min/1.73 m2, 8.4% had proteinuria, and 2.5% had isolated microscopic hematuria. The mean value of patients' α-Gal A enzyme was detected as 2.93 ± 1.92 µmol/L/h. 152 patients had low levels of α-Gal A enzyme activity (≤1.2 µmol/L/h). In mutation analysis, A143T and D313Y variants were disclosed in three male patients. The prevalence of Fabry's disease in chronic kidney disease not on dialysis was found to be 0.2% (0.4% in male, 0.0% in female). CONCLUSION: Fabry's disease should be considered in the differential diagnosis of chronic kidney disease with unknown etiology even in the absence of symptoms and signs suggestive of Fabry's disease.

The relationship between weight, height and body mass index with hemodynamic parameters is not same in patients with and without chronic kidney disease.

BACKGROUND: Although anthropometric measurements are related with clinical outcomes; these relationships are not universal and differ in some disease states such as in chronic kidney disease (CKD). The current study was aimed to analyze the relationship between height, weight and BMI with hemodynamic and arterial stiffness parameters both in normal and CKD patients separately. METHODS: This cross-sectional study included 381 patients with (N 226) and without CKD (N 155) with hypertension. Routine laboratory and 24-h urine collection were performed. Augmentation index (Aix) which is the ratio of augmentation pressure to pulse pressure was calculated from the blood pressure waveform after adjusted heart rate at 75 [Aix@75 (%)]. Pulse wave velocity (PWV) is a simple measure of the time taken by the pressure wave to travel over a specific distance. Both [Aix@75 (%)] and PWV which are measures of arterial stiffness were measured by validated oscillometric methods using mobil-O-Graph device. RESULTS: In patients without CKD, height is inversely correlated with [Aix@75 (%)]. Additionally, weight and BMI were positively associated with PWV in multivariate analysis. However, in patients with CKD, weight and BMI were inversely and independently related with PWV. In CKD patients, as weight and BMI increased stiffness parameters such as Aix@75 (%) and PWV decreased. While BMI and weight are positively associated with arterial stiffness in normal patients, this association is negative in patients with CKD. CONCLUSION: In conclusion, height, weight and BMI relationship with hemodynamic and arterial stiffness parameters differs in patients with and without CKD.

Endothelial nitric oxide synthase gene intron 4 polymorphism predicts new onset diabetes mellitus after transplantation in kidney allograft recipients treated with cyclosporin A.

BACKGROUND: Nitric oxide (NO), synthesized from LS: -arginine by the enzyme endothelial nitric oxide synthase (eNOS), is a potent vasodilator and has been implicated in mediating insulin-induced uptake and metabolism of glucose in skeletal muscle. Polymorphisms of the eNOS gene have been associated with altered eNOS activity and NO levels. Although several factors have been demonstrated for new onset diabetes mellitus after transplantation (NODAT), determining a genetic susceptibility for all patients requires further study. In our study, we evaluated the relationship between eNOS gene intron 4 polymorphism and NODAT in kidney allograft recipients. METHODS: A total of 82 consecutive patients who received their first kidney transplantation and maintained graft function for at least a 12-month post-transplant period and who used triple therapy including cyclosporin A (CsA) for maintenance immunosuppression were included. PCR-RFLP was used for genetic analyses. RESULTS: Nine of 82 patients (11%) developed NODAT. Concerning the prevalence of eNOS intron 4 gene polymorphism, a significantly higher percentage of 4a allele carriers developed NODAT than non-carriers [6/26 (23.1%) versus 3/56 (5.4%), P = 0.02]. Compared with non-diabetics, NODAT patients were older (P = 0.04), had higher rate of hepatitis C (P < 0.05) and higher body mass index at the time of transplantation (P = 0.03). In regression analyses, having a 4a allele of the eNOS gene intron 4 polymorphism (P = 0.02) and HCV seropositivity (P = 0.03) were found to be independent risk factors for the development of NODAT. CONCLUSIONS: These findings suggest that carrying a 4a allele of the eNOS gene intron 4 polymorphism is associated with NODAT. This may help us to further understand the individual risk for development of NODAT in kidney allograft recipients under CsA treatment.

First case of continuous ambulatory peritoneal dialysis peritonitis due to Candida sake.

Fungal peritonitis is a relatively uncommon complication of peritoneal dialysis that contributes significantly to morbidity, drop out from the continuous ambulatory peritoneal dialysis (CAPD) program, and mortality. Candida sake infections were rarely published in literature. We present the first case of peritonitis due to C. sake. A 41-year-old man was admitted to our hospital with abdominal pain, nausea, vomiting, fever, weakness. Abdominal ultrasonography demonstrated a fistula tract, which has an opening at inferolateral of the umbilicus extending 5 cm from the skin into the abdominal cavity with a foreign body (11 x 10 mm length) inside the fistula. The foreign body was removed by surgery being apparently a part of a previously inserted peritoneal catheter. Postoperative specimens revealed polymorph leucocytes and yeast cells in Gram stain, and culture on Sabouraud dextrose agar (SDA) yielded a growth of a fungus, subsequently identified as C. sake with Api ID 32C. Fluconazole (200 mg/day) therapy was started. He recovered after two weeks of therapy. In conclusion, C. sake, a rare type of Candida species, should be considered as a probable peritoneal pathogen in patients with multiple episodes of bacterial peritonitis, previous broad-spectrum antibiotic therapy and diabetes mellitus.

Fatal outcome of disseminated invasive aspergillosis in kidney allograft recipients.

Fungal infections in solid organ transplant recipients are of concern due to the related high mortality and morbidity. Aspergillus species are one of the major opportunistic fungal pathogens causing invasive pulmonary infections which rarely involve extrapulmonary organs. The occurrence varies by type of transplantation, with aspergillosis more frequently associated with heart, liver and lung transplantation cases than those involving kidney recipients. Several risk factors have been proposed, with cases occurring early and late after the transplantation. Although pulmonary involvement is the main presentation, invasive extrapulmonary aspergillosis can on rare occasions be observed and is associated with poor prognosis. Herein, we report two cases that presented with extrapulmonary invasive aspergillosis, i.e., one presented with cerebral abscess and the second with soft tissue abscess in the right posterior thigh. While the cerebral abscess was not surgically treated, the soft tissue abscess was surgically drained. When the primary focus was investigated, pulmonary nodulars were found in both cases. Both patients were treated with long-term amphotericin B; however, one patient was lost with functioning graft and the kidney of the second patient failed due to decreased immunosuppression and he died while on maintenance hemodialysis. Invasive extrapulmonary presentation of aspergillosis rarely occurs in kidney transplant recipients and is associated with a high mortality rate.

Pregnancy-associated plasma protein A in dialysis patients.

BACKGROUND: Pregnancy-associated plasma protein A (PAPP-A) was recently described as a new marker of cardiovascular events and of inflammation in uremic patients. The aim of this study was to determine levels of PAPP-A in chronic dialysis patients and its possible relationships with renal osteodystrophy. METHODS: A total of 99 adult chronic hemodialysis patients, 14 peritoneal dialysis patients and 41 control subjects were included in the study. Serum PAPP-A, intact parathormone (iPTH), calcium, phosphorus and alkaline phosphatase (ALP) were measured. The correlations between PAPP-A and iPTH, calcium, phosphorus and ALP were determined. RESULTS: PAPP-A levels were significantly higher in peritoneal dialysis [4.5 (3.2-6.7) mU/L, median (interquartile range)], and hemodialysis patients [4.7 (3.8-6.5) mU/L] in comparison to control subjects [3.4 (3.0-5.0) mU/L] (p<0.05). In hemodialysis patients, post-dialysis PAPP-A levels [6.2 (4.7-9.4) mU/L] were significantly higher than pre-dialysis levels [4.7 (3.8-6.5) mU/L] (p<0.05). There was a weak but statistically significant positive correlation between serum PAPP-A and iPTH (r=0.216; p=0.041) and ALP (r=0.205; p=0.044) in the hemodialysis group. Correlation between the duration of dialysis therapy and PAPP-A levels was also significant (r=0.267; p=0.008) in the hemodialysis group. CONCLUSIONS: PAPP-A levels are elevated in acute coronary syndromes and are closely related to inflammation and oxidative stress. We conclude that PAPP-A levels are increased in dialysis patients and may reflect a greater degree of chronic inflammation than osteodystrophy in uremic patients.