Eculizumab experience in an adult patient with atypical hemolytic uremic syndrome.

Atypical hemolytic-uremic syndrome is a disease characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal failure. In this study, we present a case of a patient with atypical hemolytic-uremic syndrome treated successfully with eculizumab. A 20-year-old female was admitted with clinical signs of atypical hemolytic syndrome. The laboratory findings were as follows: hemoglobin 9.2 g/dL, platelet count 18 × 103/µL, creatinine 4.69 mg/dL, schistocytes were in peripheral blood smear, lactate dehydrogenase 2080 U/L, and emergency plasmapheresis procedure with fresh frozen plasma were initiated. The patient was anuric within 12 h of her admittance. ADAMTS13 activity was normal. Her mother's cousin developed acute rejection immediately after receiving a renal transplant and died two months later. As she did not respond to the treatment and considering her family history, eculizumab was initiated which resulted in platelet counts starting to rise on day 5, and the patient no longer needed dialysis after 22 days.

High serum soluble CD200 levels in patients with autosomal dominant polycystic kidney disease.

CD200 is a novel immune-effective molecule, existing in a cell membrane-bound form, as well as in a soluble form in serum, which performs to modulate inflammatory and acquired immune responses. Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the development of large renal cysts and progressive loss of renal function. As defects in cell cycle arrest and apoptosis of renal tubular epithelial cells occur in ADPKD, we asked whether serum soluble CD200 might underlie and effect on ADPKD. Serum soluble CD200 levels were measured in 44 patients with ADPKD and 24 healthy volunteers. Concentrations of soluble CD200 in the serum samples were quantified using an ELISA kit. The mean serum soluble CD200 levels were higher in patients with ADPKD than in the control group (71.4±29.2 and 21.4±5.6 pg/mL, p<0.001). Positive correlation was detected between serum soluble CD200 levels and glomerular filtration rate (r=0.772, p<0.001), and serum albumin level (r=0.466, p=0.001). Negative correlation was detected between serum soluble CD200 levels and serum creatinine levels (r=-0.761, p<0.001), and C reactive protein levels (r=-0.364, p=0.015). In the ADPKD patients group, serum soluble CD200 levels were lower in patients with stage 5 chronic kidney disease (CKD) than in patients with stages 1-2 (p<0.001), 3 (p=0.005) and 4 CKD (p=0.006). Serum soluble CD200 levels were similar in patients with stages 1-2, 3, and 4 CKD (p>0.05). Our results show that patients with ADPKD have activated soluble CD200 levels which were related to renal function and inflammation.

Autosomal Dominant Polycystic Disease is Associated with Depressed Levels of Soluble Tumor Necrosis Factor-Related Apoptosis-Inducing Ligand.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by multiple, large renal cysts and impaired kidney function. Although the reason for the development of kidney cysts is unknown, ADPKD is associated with cell cycle arrest and abundant apoptosis of renal tubular epithelial cells. AIMS: We asked whether serum-soluble TNF-related apoptosis-inducing ligand (sTRAIL) might underlie ADPKD. STUDY DESIGN: Case-control study. METHODS: Serum sTRAIL levels were measured in 44 patients with ADPKD and 18 healthy volunteers. The human soluble TRAIL/Apo2L ELISA kit was used for the in vitro quantitative determination of sTRAIL in serum samples. RESULTS: Mean serum sTRAIL levels were lower in patients with ADPKD as compared to the control group (446.9±103.1 and 875.9±349.6 pg/mL, p<0.001). Serum sTRAIL levels did not differ among stages of renal failure in patients with ADPKD. There was no correlation between serum sTRAIL levels and estimated glomerular filtration rate in patients with ADPKD (p>0.05). CONCLUSION: Our results show that ADPKD patients have depressed sTRAIL levels, indicating apoptosis unrelated to the stage of chronic renal failure.

Soluble Klotho and fibroblast growth factor 23 levels in diabetic nephropathy with different stages of albuminuria.

The relationship between soluble Klotho (s-Klotho) levels, fibroblast growth factor 23 (FGF23) levels, and albuminuria in patients with diabetic chronic kidney disease (CKD) remains unclear. A total of 109 patients with type 2 diabetes (mean age 61.63±9.77 years), at the outpatient clinic of the Antalya Research and Training Hospital Nephrology Unit between January and June 2014, as well as 32 healthy controls (mean age 49.53±7.32 years) were enrolled for this cross-sectional study. Patients were classified into three groups according to their urinary albumin creatinine ratio (UACR), normoalbuminuria (UACR<30 mg/g), microalbuminuria (UACR 30-300 mg/g), and macroalbuminuria (UACR>300 mg/g). The blood was analyzed for FGF23, s-Klotho, parathyroid hormone (PTH), P, Ca, creatinine, and 25-hydroxyvitamin D3 (25hD) levels. Creatinine, s-Klotho, FGF23, and PTH levels were significantly higher and 25hD levels were significantly lower in the patient group than in the healthy controls (p<0.001). Between the groups according to UACR, 1-way analysis of variance revealed statistically significant differences for creatinine (p<0.001), 25hD (p<0.001), PTH (p=0.002), Ca (p=0.002), and albumin levels (p<0.001). A statistically significant positive correlation was found between s-Klotho and FGF23 (r=0.768; p=0.001), and between FGF23 levels and UACR (r=0.768; p=0.001). In conclusion, the results of the present study suggest that s-Klotho levels are significantly elevated in patients with diabetes and s-Klotho levels decreased with increasing albumin excretion in our patients despite a reduction in estimated glomerular filtration rate.

Effects of ivabradine on 6-minute walk test and quality of life in patients with previously implanted CRT-D.

BACKGROUND: We aimed to evaluate clinical effects of additional heart rate control by ivabradine on life quality score and 6-minute walking test in patients with previously implanted biventricular cardiac resynchronization therapy defibrillator (CRT-D) with ischemic heart failure under regular treatment. METHODS: Fifteen men and 14 women with a median age of 63 years (range, 48-79 years) were studied. Twenty-one patients were in New York Heart Association class II (8 patients were in class III), CRT-D implanted previously, and with resting heart rates greater than 70 beats per minute with sinus rhythm despite conventional medication. Patients were given 2.5- to 7.5-mg ivabradine orally twice a day, and drug dosage was titrated to decrease the patients' average heart rate to 70 beats per minute. Before and 3 months after ivabradine treatment, all patients underwent extensive clinical, echocardiographic, and laboratory evaluation. RESULTS: Ivabradine treatment produced dose-dependent reductions in heart rate at rest and at peak exercise (91.9 ± 6.3 to 71.7 ± 4.8 and 114.4 ± 7.6 to 96.8 ± 4.8; P = 0.001 and P = 0.001, respectively). There were also significant improvements in life quality score (52.4 ± 9.5 to 37.9±7.8; P = 0.001) and 6-minute walking distance (278.7 ± 85.8 to 373.3 ± 94.0; P = 0.001) of patients. All patients with New York Heart Association class III became class II after 3 months of ivabradine treatment. CONCLUSION: Heart rate reduction in a short-term period by ivabradine produced significant improvements in exercise capacity and life quality in patients with CRT-D and conventional therapy.

Serum prolactin and macroprolactin levels in diabetic nephropathy.

OBJECTIVE: Three molecular forms of prolactin with molecular weights of 23 (monomeric), 50 - 60 and > 100 kDA (macroprolactin) have been defined. Prolactin levels have been shown to be reduced in especially poorly controlled diabetes mellitus and the prevalence of macroprolactinemia in diabetic patients has been higher than the non-diabetic population. PATIENTS AND METHODS: A total 234 Type 2 diabetic patients with different nephropathy stage was included in the study. Serum prolactin levels were analyzed by the Electrochemiluminescense method. Following polyethylene glycol (PEG) precipitation, recovery less than or equal to 40% was taken as evidence that a significant level of macroprolactin was present in the serum. RESULTS: Hyperprolactinemia and macroprolactinemia were detected in 40 (17%) and 13 (5.5%) patients, respectively. Macroprolactinemia was detected 13 of 40 patients with hyperprolactinemia (32.5%). Increased prolactin and macroprolactin levels in patients with moderate and severe renal failure (Stage 3, 4, and 5) according to the U.S. NKF-DOQI classification (p < 0.001). Prolactin and macroprolactin levels were not increased in patients with normoalbuminuria, microalbuminuria and macroalbuminuria (p > 0.05). Serum creatinine levels correleted positively with both prolactin (r = 0.51, p < 0.001) and macroprolactin levels (r = 0.43, p < 0.001). On the other hand, glomerular filtration rate correlated negatively with both prolactin (r = -0.54, p < 0.001) and macroprolactin levels (r = -0.44, p < 0.001). Albuminuria significantly related with neither prolactin nor macroprolactin levels (p > 0.05). CONCLUSION: In the present study, we found that not only serum prolactin but also serum macroprolactin levels increased especially in moderate to severe renal failure which was due to decreased glomerular filtration and renal parenchymal function resulting in an increased amount of monomeric prolactin and macroprolactin in the circulation in patients with Type 2 diabetes mellitus.

Renal failure in a patient with autosomal dominant polycystic kidney disease and coexisting dermato-polymyositis: first report in the literature.

Autosomal dominant polycystic kidney disease is a multisystem disorder characterized by multiple, bilateral renal cysts and is also associated with cysts in other organs, such as the liver, pancreas, and arachnoid membranes. Dermatomyositis is a disease which mainly involves the skin and muscles, although occasionally other organs are affected. In this report, a 56-year-old male patient with a four-year history of autosomal dominant polycystic kidney disease was presented. Renal failure was exacerbated by a coexisting dermato-polymyositis. Prednisone treatment with hemodialysis improved the situation. This is the first report renal failure in a patient with autosomal dominant polycystic kidney disease and dermato-polymyositis.

Complete heart block in a patient with Wegener's granulomatosis in remission--a case report.

Wegener's granulomatosis is a systemic inflammatory disorder of unknown cause that usually affects the upper and lower respiratory tracts as well as the kidney. Cardiac involvement is rare, although electrocardiographic abnormalities, coronary artery vasculitis, cardiac arrhythmias, and myocardial infarction have been reported in the literature. A 27-year-old female patient with Wegener's granulomatosis in remission is described in whom complete heart block developed in the 13th month of treatment with cyclophosphamide. A temporary pacing was applied and pulse methylprednisolone and cyclophosphamide were commenced. On the ninth day of treatment, normal sinus rhythm was achieved. In conclusion, cardiac rhythm abnormalities should always be kept in mind both in the diagnosis and follow-up of Wegener's granulomatosis.

Bismuth subcitrate nephrotoxicity. A reversible cause of acute oliguric renal failure.

Bismuth subcitrate is a known nephrotoxic agent that may lead to acute oliguric renal failure when ingested in toxic doses. We report a 17-year-old girl who was admitted to the emergency room with complaints of nausea, vomiting, and anuria. She had taken 25 tablets containing 300 mg bismuth subcitrate (total 7.5 g). The patient was managed with hemodialysis started a week after ingestion. Bismuth subcitrate nephrotoxicity should be considered in the differential diagnosis of acute renal failure.