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.

A novel factor for primary arteriovenous fistula failure: hyperinsulinism.

INTRODUCTION: Dysfunction of vascular access is an important reason of morbidity for dialysis patients and it is a major factor affecting the economical burden of hemodialysis. The preferred type of vascular access is creation of an arteriovenous fistula (AVF). However, the problem of fistula maturation rate is still a challenge. Herein, we tried to search the role of hyperinsulinism and insulin resistance as a new predictor of primary AVF failure (pAVFF) that may be a cause of intimal damage. METHODS: We included 119 patients (73 male and 46 female) with a diagnosis of end-stage renal disease (ESRD) who had undergone an AVF operation by a vascular surgeon. The AVF was examined for presence of thrill on the first postoperative day. A successful cannulation with two fistula needles with a blood flow of 250 mL/min for at least one complete dialysis session, after 4 weeks of AVF surgery was defined as functioning access. Insulin resistance in our patients was determined by the standard homeostasis model assessment (homa-IR). A logistic-regression analysis was performed to investigate the independent factors related with pAVFF. FINDINGS: Detection of pAVFF occurred in 27 (22.7%) patients. The presence of thrill, amount of daily proteinuria, insulin levels, homa-IR, and serum albumin levels were found to be significantly different between patients with fistula failure and those without pAVFF. The logistic-regression analysis of preoperative factors revealed the following OR (odds ratio) and 95% CI values: homa-IR 1.205 (1.063-1.366) (p = 0.004), serum albumin 0.398 (0.178-0.892) (p = 0.025), and the amount of daily proteinuria 1.307 (1.012-1.688) (p = 0.041). Even after addition of the presence of postoperative thrill on AVF, which was a postoperative strong clinical factor to the analysis, mean homa-IR and mean serum albumin continued to be independent predictors of pAVFF. DISCUSSION: Insulin resistance or hyperinsulinism may be a significant cause of pAVFF, which emphasizes the role of endothelium in fistula dysfunction.

Efficacy and safety of Sinovac vaccine administered in patients undergoing hemodialysis.

INTRODUCTION: COVID-19 disease is more serious and fatal in patients on dialysis treatment due to their immunosuppressive status. In this study, we aimed to evaluate the protection and safety of Sinovac vaccine, which is an inactivated vaccine, in patients undergoing hemodialysis. METHODOLOGY: A control group consisting of 220 hemodialysis patients (HD group) and 648 healthcare professionals who were healthy in our institution were included in the study. Quant II IgG anti-Spike antibody was measured 3 weeks after two doses of Sinovac vaccine were administered to both groups. RESULTS: The antibody response after two doses of Sinovac vaccine was 85.2% in the HD group and 99.8% in the control group. The mean antibody level before vaccination in the HD group was 3.5 ± 7.2 AU/mL and increased significantly 3 weeks after two doses of vaccine (mean 751 ± 1196 AU/mL). The control group's mean antibody level after vaccination was 1723 ± 1878 AU/mL. The mean antibody level after vaccination in the control group was significantly higher than the HD group (p < 0.0001). Despite higher levels of anti-Spike antibodies in the control group, post-vaccination antibody response was acceptable in both HD and control groups. The HD group was significantly older (mean 64 ± 12 years) than the control group (36 ± 10 years) (p < 0.0001). CONCLUSIONS: Although dialysis patients are immunocompromised, and some may not develop antibodies to the virus as strongly as healthy people, this study revealed that dialysis patients developed significant amounts of antibodies. Being old or on dialysis is an independent predictor of low antibody response to the Sinovac vaccine.

Thrombophilias and arteriovenous fistula dysfunction in maintenance hemodialysis.

Most episodes of fistula thrombosis are consequences of underlying physioanatomic abnormalities. However, some dialysis access thrombosis develops independent from any obvious anatomic cause. We aimed to clarify the role of thrombophilias in primary and secondary AVF failure. One hundred eighty nine arteriovenous fistulas in 116 adults on maintenance hemodialysis were analyzed. All subjects were evaluated for many thrombotic factors. Fistula information was obtained both from the patients' self reports, and from their medical records. Twenty-seven AVFs in 18 cases (14.3%) had pAVFF. The percentage of subjects with a BMI < 20 kg/m(2) was significantly lower than no-pAVFF group (P = 0.03). ATIII levels and albumin values were significantly lower in patients with sAVFF compared to those with no sAVFF. Other parameters were similar. There was no statistically significant difference between pAFFF versus No-pAFFF and sAFFF versus No-sAFFF groups with respect to all mutant alleles count. Routine extended analyses of all thrombophillic factors are not recommended in AVFF.

An unresolved issue: The relationship between spot urine protein-to-creatinine ratio and 24-hour proteinuria.

OBJECTIVE: To investigate the relationship between spot urine protein-to-creatinine (sP/Cr) ratio and 24-h protein excretion in patients with different diagnoses. METHODS: This retrospective study analysed data from the medical records of patients admitted for24-h proteinuria determination who also had sP/Cr ratio data for the same day. RESULTS: A total of 1222 urine samples obtained from 694 adult outpatients were analysed. The mean ± SD age of the patients was 53.6 ± 15.9 years. The mean ± SD 24-h proteinuria and sP/Cr were 1.7 ± 2.4 g/day and 1.8 ± 2.4, respectively. The correlation between the sP/Cr and 24-h protein excretion was high (R2 = 0.89). The sP/Cr ratio accounted for 72% of the variability in 24-h proteinuria in the entire study population. Areas under the curve for 24-h proteinuria at 0.3 g/day, 1.0 g/day and 3.0 g/day were 0.940, 0.966, and 0.949, respectively. The mean + 2SD limits of agreement were between +2.99 and -2.73 g/day according to the Bland Altman analysis. CONCLUSION: This current study found a clinically unacceptable deviation between 24-h proteinuria and sP/Cr ratio. Therefore, the sP/Cr ratio cannot replace 24-h proteinuria. A new method using spot urine protein and creatinine values that is able to minimize under or over estimation is still warranted.

Ureterosigmoidostomy[corrected]-associated quadriparesis, non-traumatic rhabdomyolysis, and tetany in an adult.

We report an adult case of ureterosigmoidostomy-associated quadriparesis, rhabdomyolysis, and tetany which may be the first such case in the literature. A 32-year-old female patient was brought to the emergency room of our hospital, having been unable to walk or use her arms for 24 h. Neurological examination revealed quadriparesis. She had severe hypokalemia (1.27 mmol/l) and metabolic acidosis (pH=7.05). Creatine kinase value was 2,590 U/l on the third day. She received intensive therapy to correct the hypokalemia and acidosis. On the third day of hospitalization tetany was detected in her upper extremities. The patient regained full muscle power after intravenous potassium chloride, bicarbonate, and calcium replacement therapy.

[Investigation of anticardiolipin antibodies in chronic hepatitis B infection together with total anti-delta positivity]. / Kronik hepatit B enfeksiyonu ile birlikte total anti-delta pozitifliginde antikardiyolipin antikorlarinin arastirilmasi.

Anticardiolipin antibodies (ACAs) are formed against phospholipids in various clinical conditions such as autoimmune diseases, malignancy, infectious diseases, alcohol-related and hepatic cirrhosis. The aims of this study were to investigate the presence of ACAs in patients with chronic hepatitis B together with positive total anti-delta antibodies, and to investigate the relationship between age, gender, and some laboratory parameters (ALT, AST, albumin, globulin, platelet number) of patients with chronic hepatitis delta virus (HDV) infection, who were positive or negative for ACAs. A total of 60 patients (43 male, 17 female) with chronic hepatitis D infection [HBsAg positive, HBeAg negative, anti-HBe positive, anti-HBc IgG positive, anti-HBc IgM negative, total anti-delta positive, anti-HCV negative] and 30 patients (21 male, 9 female) without hepatitis D infection [HBsAg positive, HBeAg negative, anti-HBe positive, anti-HBc IgG positive, anti-HBc IgM negative, total anti-delta negative, anti-HCV negative] as control group were included to the study. ACA IgG and IgM were searched by a commercial microELISA kit (Euroimmun, Germany). The statistical evaluation was performed with Pearson's chi-square test, Student's t-test, and Fisher's exact test. Total ACAs positivity rate of 60 patients with chronic HDV infection, was found as 13.3%, in which four of the patients were positive for only ACA IgM, while four was positive for only IgG. Positivity for both ACA IgG and ACA IgM could not be detected in these patients. No patients in the control group had positivity for ACAs (IgG and/or IgM). A statistically significant difference was observed in terms of ACA positivity between patients with and without HDV infection (p< 0.05). After all, there was no statistically significant correlation between ACAs positivity and the age, sex, and laboratory parameters of the patients with chronic HDV infection, except lower serum albumin levels (p= 0.004). Although the data of this study revealed a statistically significant positive correlation between chronic HDV infection and anticardiolipin antibodies, it is clear that there is a need for further studies on this subject.

Hyaluronic Acid as a New Biomarker to Differentiate Acute Kidney Injury From Chronic Kidney Disease.

INTRODUCTION: It may be difficult to differentiate acute kidney injury from chronic kidney disease in patients with no past medical reports of kidney function. This study aimed to investigate the role of serum hyaluronic acid (HA), which is known as a marker of fibrosis, in differential diagnosis of kidney failure. MATERIALS AND METHODS: A total of 90 patients (52 women and 38 mne) admitted to our renal unit with uremia for the first time were included. Serum HA level was measured. The diagnostic role of the test was investigated using the receiver operator curve curves. RESULTS: The mean age of the patients was 54.6 ±17.9 years. The diagnosis was chronic kidney disease (CKD) in 41.1%, acute kidney injury (AKI) in 48.9%, AKI on CKD in 6.7% (3 died without a diagnosis). The mean serum HA was significantly higher in the CKD group (146.1 ± 119.3 ng/mL) than the AKI group (68.9 ± 69.1 ng/mL; P < .001). Serum HA significantly correlated with proteinuria (r = 0.717, P < .001) and serum albumin level (r = - 0.599, P < .001) in the CKD group only. Serum HA cutoff level of 61 ng/dL had a sensitivity of 82% and specificity of 67% for differential diagnosis of AKI and CKD. CONCLUSIONS: Serum HA level may be used as tool to differentiate AKI from CKD. Further larger studies are warranted to clarify the definite the role of this marker.

Factors affecting responsiveness to hepatitis B immunization in dialysis patients.

BACKGROUND: Hepatitis B virus (HBV) and hepatitis C virus (HCV) infections are widespread health problems all over the world and have high morbidity and mortality. Hemodialysis patients are more frequently exposed to these viruses as they have poor immune system and frequently undergo parenteral interventions. The vaccination against HBV prevents infection and it has been recommended for the prevention of HBV infection in all susceptible dialysis patients. This study aimed to determine the seroprevalence of HBV and HCV infections and analyzed the factors affecting inadequate response to HBV vaccine in dialysis patients. METHODS: The data for 584 dialysis patients that were followed up at seven dialysis centers were analyzed. The patients received four doses of 40 µg recombinant hepatitis B vaccine at 0, 1, 2, and 6 months and were tested for anti-HBs titer after one month of completion of vaccination. If patients showed a titer of anti-HBs <10 IU/mL, an additional 40 µg in four vaccine doses was administered immediately and they were retested for the anti-HBs following the same schedule. The patients were divided into two groups: responders and non-responders. RESULTS: HBsAg and anti-HCV seroprevalence was 3.4% and 10.3%, respectively. After vaccination schedule, 264 (83.5%) patients had antibody response to HBV vaccine and 52 (16.5%) had no response. Hepatitis B vaccine unresponsiveness is more common in the patients with hepatitis C positivity (p = 0.011), BMI >30 (p = 0.019), over the age of 65 years (p = 0.009), and duration of dialysis treatment >5 years (p = 0.001). There was no statistically significant difference between gender, causes of renal disease, erythropoietin treatment, and the type of dialysis. CONCLUSION: Hepatitis C infection, obesity, being elderly, and having long hemodialysis period reduced the hepatitis B vaccination response in hemodialysis patients.

Rapid tumor lysis syndrome in a patient with metastatic colon cancer as a complication of treatment with 5-fluorouracil/leucoverin and irinotecan.

Tumor lysis syndrome is a potentially fatal complication of anti-cancer therapy that is usually seen in patients with bulky, rapidly proliferating, treatment-sensitive tumors such as hematological malignancies, but it rarely occurs in a variety of solid tumors such as colorectal carcinoma. Combination chemotherapy with infusional 5-fluorouracil/leucoverin and irinotecan has been recently accepted as the first treatment option for metastatic colorectal cancer. We present a case of tumor lysis syndrome in a patient with metastatic colon carcinoma that occurred 72 hrs after the initial course of a combination chemotherapy with irinotecan and 5-fluorouracil/leucoverin. Despite the immediate treatment with aggressive hydration by a sodium bicarbonate infusion, followed by forced diuresis and uricolytic therapy, he died of a sudden cardiac arrest complicated by acute renal failure. Our case indicates that administration of 5-fluorouracil/leucoverin and irinotecan for bulky tumors of colorectal origin with a rapid doubling time may induce an acute tumor lysis syndrome, which necessitates frequent laboratory monitoring and a close follow-up of the patient as well as prompt initiation of appropriate therapeutic measures.

Type 4 renal tubular acidosis in a patient with lupus nephritis.

Although renal tubular acidosis (RTA) is a rare complication of systemic lupus erythematosus (SLE), type 4 RTA associated with lupus nephritis is extremely rare. A 20-year-old woman presented with malaise and edema in the lower extremities and face. She had multiple lymphadenopathies. There were 20% eosinophil in blood smear and 32% in bone marrow aspiration. Serology revealed positive antinuclear antibody at 1:1000 titer, positive double-stranded DNA antibodies, and low complements C3 and C4 levels. Urinary sediment was active and urinary protein excretion was 4.8 g/d. The SLE Disease Activity Index score was 23. A high SLE Disease Activity Index scores was proposed as a potential risk factor for type 4 RTA. Type 4 RTA may complicate SLE, and specifically, patients with high SLEDAI scores and lymphadenopathy may pose a high risk. Our patient responded successfully to immunomodulatory therapy.

Role of lean body mass for estimation of glomerular filtration rate in patients with chronic kidney disease with various body mass indices.

OBJECTIVE: Glomerular filtration rate (GFR) is the main tool used to diagnose, treat and follow up renal diseases. Age, gender, ethnicity and obesity all affect the relationship between serum creatinine, muscle mass/body weight and GFR. This study aimed to investigate the role of lean body mass for GFR estimation in patients with chronic kidney disease (CKD) with various body mass indices. MATERIAL AND METHODS: In total, 110 Caucasian adult subjects with CKD referred for GFR measurement by (99m)Tc-DTPA renography were enrolled in the study. The patients were categorized according to body mass index values: <18.5 kg/m(2) (underweight), 18.5-24.9 kg/m(2) (normal), 25-29.9 kg/m(2) (overweight) and >30 kg/m(2) (obese). Lean body mass (LBM) and fat mass were measured by leg-to-leg bioimpedance. Predictive factors were identified by linear regression analysis in each group. RESULTS: GFR measured by DTPA, creatinine clearance, Cockcroft and Gault, and Modification of Diet in Renal Disease (four-variable) equations was 37+/-27, 42+/-30, 42+/-27, and 49+/-35 ml/min/1.73 m(2), respectively. The predictive role of 1/SCr, age, serum albumin, amount of proteinuria, LBM and fat mass was investigated all groups. None of the factors was significant in underweight and healthy weight groups except for 1/serum creatinine (SCr). LBM/SCr was an independent predictive factor for both overweight and obese groups. 1/SCr accounted for 96.2% of the variability in measured GFR for underweight subjects but only 58.1% of the variability in GFR of obese subjects. CONCLUSIONS: The formulae derived from SCr should be used cautiously in overweight and obese subjects. LBM measured by bioimpedance was an independent predictive factor of GFR in obese/overweight subjects and added clinically important diagnostic value to 1/SCr. It needs to be investigated as a parameter in further studies attempting to develop formulae for estimating GFR in larger obese and overweight populations.

Thrombosis of temporal artery and renal vein in Kimura-disease-related nephrotic syndrome.

Kimura disease (KD) is an angiolymphoid proliferative disorder of unknown etiology, occurs mainly in Asian patients, presenting with subcutaneous slowly growing masses, with a predilection for preauricular and submandibular regions. The clinical course of the disease is thought to be benign. Concomitant peripheral blood eosinophilia and elevated serum immunoglobulin E levels are often observed. Main systemic manifestation of the KD is renal involvement. Renal abnormalities, notably proteinuria and nephrotic syndrome have been found to be associated with KD. We report a 42-year-old man with KD and a steroid-sensitive membraneous nephrotic syndrome with bilaterally temporal artery and renal vein thrombosis. This is the first reported case of KD associated nephrotic syndrome complicated with wide arterial and venous thrombosis from Anatolia.

Snakebite-induced acute kidney injury: data from Southeast Anatolia.

Renal failure is an important complication of snakebite and a major cause of mortality. We aimed to study the clinical profile of snake envenomation in Southeast Anatolia, Turkey, in an adult population. We retrospectively analyzed the records of 200 snakebite victims from 1998 to 2006 at the Dicle University School of Medicine, Diyarbakir, Turkey. Sixteen patients (8%) developed AKI (acute kidney injury). Of those, 25% required dialysis and 18% died. There was no difference between groups in age, arrival time to hospital, and hospital stay time. Both groups received similar hydration and therapy at admission. Disseminated intravascular coagulation (DIC) was observed in 25% of the AKI group and was significantly higher than the non-AKI group (7.1%; p = 0.014). There was no significant difference regarding hemoglobin, platelet levels, and prothrombin time at admission. The prevalence of thrombocytopenia (<150,000 K/UL ) was 60% in the AKI group and 40% in the non-AKI group (p > 0.05). WBC count was significantly higher in the AKI group than in those without AKI (p = 0.001); serum albumin was significantly lower in the AKI group than in those without AKI (p = 0.013). AKI is an important complication of snakebite that may lead to mortality. Despite some troublesome aspects due to its retrospective design, this is a large series from Southeast Anatolia of Turkey in an adult population. Subjects with high WBC, low albumin, and DIC should be closely followed up for the development of AKI.