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.

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.

Hepatitis C infection, obesity, being elderly, and having long hemodialysis period reduced the hepatitis B vaccination response in hemodialysis patients.

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.

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.