Multiple Myeloma in a Patient With Familial Mediterranean Fever

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent episodes of painful inflammation in the abdomen, chest, or joints. The coexistence of multiple myeloma (MM) and FMF is an extremely rare event. Here, we report a case of FMF with concurrent MM. A 63-year-old woman was diagnosed with FMF since 15 years earlier. She was admitted with a complaint of low back pain. Regarding the presence of back pain, anemia, hypercalcemia, and kidney failure, a diagnosis of MM was suspected. A skeletal survey showed punched-out lesions in the skull. Serum protein electrophoresis demonstrated an immunoglobulin G kappa monoclonal gammopathy, and bone marrow aspiration revealed 30% involvement by abnormally appearing plasma cells, suggestive of MM. Although the association between FMF and MM may be a mere coincidence, further studies are necessary to understand their concurrent development

Seizure, Deafness, and Renal Failure: A Case of Barakat Syndrome

Etiologic diagnosis of seizure requires proper consideration of apparently unrelated clinical features of the patient. Here, we report the case of a patient of status epilepticus with moderate-to-severe bilateral sensorineural deafness. Investigations showed extensive intracranial calcification, hypoparathyroidism and unilateral renal agenesis. The features were consistent with Barakat syndrome, a rare developmental disorder associated with mutations in the GATA3 gene. To the best of our knowledge, this is the first reported case of Barakat syndrome from India

Association of glomerular C4d deposition with various demographic data in IgA nephropathy patients; a preliminary study.

BACKGROUND IgA nephropathy (IgAN) is the most prevalent primary chronic glomerulopathy worldwide. Thus, it is of vital importance to search for factors aggravating the disease progress, monitor disease activity and predict disease-specific therapy. C4d is a well-known biomarker of the complement cascade with a potential to meet the above needs. OBJECTIVES The aim of our study was, therefore, to determine whether C4d staining at the time of kidney biopsy had any correlation with the demographic, clinical and biochemical variables in IgAN. PATIENTS AND METHODS The definition of IgAN requires the presence of diffuse and global IgA deposits which were graded ≥2+ and weak C1q deposition. C4d immunohistochemical staining was conducted retrospectively on 29 renal biopsies of patients with IgAN, which were selected randomly from all biopsies. C4d immunohistochemical staining was performed on 3-μm deparaffinized and rehydrated sections of formaldehyde-fixed, paraffin-embedded renal tissues. RESULTS Of 29 selected patients, 68% were male. In this study, 54.2±25 percent of glomeruli in all biopsies were positive for C4d. The mean and standard deviation (SD) of serum creatinine and the magnitude of proteinuria were 1.72±1.2 mg/dl and 1582±1214 mg/day, respectively. In this study, we observed statistically significant correlations of percent C4d positivity with the serum creatinine (r=0.61, p=0.0005), magnitude of proteinuria (r=0.72, p=0.0001), the proportion of globally sclerotic glomeruli (r=0.43, p=0.02) and the proportion of tubulointerstitial fibrosis (r=0.54, p=0.0023). CONCLUSIONS The results from our investigation on C4d positivity in biopsy-proven cases of IgAN are in accord with some of the previous studies. These findings, however, require further validation in larger samples

The theme of the world diabetes day 2014; healthy living and diabetes; a nephrology viewpoint.

Annually, on November 14, the world diabetes day (WDD) is celebrated. WDD is a campaign led by the International Diabetes Federation (IDF) and its member associations throughout the world. It was created in 1991 by IDF and World Health Organization (WHO) in response to increasing concerns about the intensifying threat of diabetes worldwide. The WDD 2014 organization marks the first of a three-year (2014-16) emphasis on "healthy living and diabetes". Replacement of whole grain and cereal-based foods with refined grains in diet planning could be an operative and practical strategy in type II diabetic patients. This strategy beyond the development of glycemic control, leads to more benefits for management of other features of diabetes, diminution of diabetes-induced metabolic disorders, and prevents long-term complications especially diabetic kidney disease and cardiovascular disease

Renal replacement therapy in patients with familial Mediterranean fever

One of the causes of end-stage renal disease (ESRD) in some countries is the renal involvement caused by the complications of the familial Mediterranean fever (FMF). Amyloidosis is of the most important complications of FMF that can lead to ESRD. We report our experiences of FMF patients with ESRD who received hemodialysis and kidney transplantation over the past 17 years. Of nine patients, four had amyloidosis, two had focal segmental glomerulosclerosis (FSGS), one had IgA nephropathy, and two did not undergo biopsy because their kidneys were atrophic. Of the nine patients, one died of amyloidosis. Seven patients had kidney transplantation and one patient is currently undergoing dialysis. Six patients were transplanted from a living donor and one from a cadaver. The ultrasound examination revealed that five patients had bilateral kidney atrophy, four patients had normal size kidney with amyloidosis. None of the seven patients who underwent renal transplantation had a history of rejection. Kidney transplantation and hemodialysis in patients with FMF is similar to those with other ESRD etiologies

The Diagnostic Accuracy of High Resolution Ultrasound Imaging for Detection of Secondary Hyperparathyroidism in Patients with Chronic Renal Failure

Ultrasound is one of the preferred modalities for evaluation of the parathyroid glands. This study was undertaken to determine the accuracy of high resolution ultrasound for secondary hyperparathyroidism in patients with chronic renal failure. From March 2008 to March 2009, ninety-one hemodialysis patients were examined by high resolution ultrasound (14 MHz) of the parathyroid glands in comparison to parathyroid hormone level. 43.9% of patients showed enlarged parathyroid glands with an average of 8.7 mm. The mean parathyroid hormone level of patients with enlarged parathyroid glands on sonography was 503 ± 450 pg/ml. We observed a significant correlation between parathyroid hormone level and enlarged parathyroid glands (P<0.0001). Sensitivity and specificity of sonography for detection of secondary hyperparathyroidism were 62.5% and 85.7% respectively. In conclusion, our study showed that high resolution sonography is a useful noninvasive method for the evaluation of secondary hyperparathyroidism in patients on hemodialysis and that sonographically enlarged glands may be a measure of severity of secondary hyperparathyroidism

The effect of oral N-acetylcysteine on serum high sensitive CRP and plasma hemoglobin levels in end-stage renal disease patients under routine hemodialysis; a randomized placebocontrolled clinical trial

Background: Oxidative stress and systemic inflammation is increased in end-stage renal disease (ESRD) patients. Due to the various effects of oxidative stress in ESRD patients, different antioxidants have been evaluated. Objectives: In this study, we evaluated the effect of oral N-acetylcysteine (NAC) as an antioxidant on the serum high-sensitive C-reactive protein (hs-CRP) and plasma hemoglobin levels in the ESRD patients who were under routine hemodialysis. Patients and Methods: In this randomized placebo-controlled clinical trial, 51 ESRD patients under routine hemodialysis were randomly assigned to receive NAC 1200 mg daily for 1 month (n=26) or placebo (n=25). Laboratory findings including hemoglobin, ferritin, hsCRP were measured in patients before and after treatment. Results: NAC group compared to placebo group had significantly higher ferritin levels before treatment (p=0.02) and lower phosphorus levels after treatment (p=0.03). Comparing the results before and after treatment in each group, a significant reduction in hematocrit (p=0.002), ferritin (p=0.006), hs-CRP (p=0.02) and an increase in alkaline phosphatase levels (p=0.005) in NAC group and significant reduction in calcium levels (p<0.001) in placebo group was detected. No major side effects were seen. Conclusions: One month treatment with oral NAC resulted in reduced levels of hematocrit, ferritin and Hs-CRP, indicative of role of NAC in controlling inflammation in ESRD patients under hemodialysis. However, NAC was not effective in treatment of anemia, although the treatment duration was low

Impact of vitamin C supplementation on serum ferritin level in hemodialysis patients

Background: Iron deficiency anemia is common in patients on maintenance hemodialysis (HD). Serum ferritin is increased in HD patients. Vitamin C supplementation has shown to improve functional iron deficiency and anemia in HD patients. Objectives: In this study we aimed to evaluate the effect of vitamin C supplementation on serum ferritin level. Patients and Methods: In this randomized clinical trial, 39 HD patients were randomly assigned to receive routine therapy with (n=24) or without (n=15) vitamin C 500 mg IV twice a week after each dialysis session for 2 months. Changes in serum ferritin, iron, total iron-binding capacity (TIBC) and transferrin saturation (TS) before and after treatment were measured in each group. Results: Serum ferritin levels before treatment in case and control groups were 1859±1398.5 and 2567.5±1907.8 ng/mL which was significantly decreased to 1084.8±727.9 and 1313.5±1151.5 ng/mL, respectively (P=0.006 and P=0.007). Serum iron, TIBC and TS did not change significantly in each group after treatment. Comparing the mean changes in serum ferritin, iron, TIBC and TS, there was no significant difference between groups. Conclusions: Vitamin C had no effects on serum ferritin, iron and TS

The Therapeutic Effects of Sevelamer on Blood Sugar, HbA1c, Lipid Profile, and hs-CRP in Patients with Diabetic Nephropathy

Background: Diabetic nephropathy (DN) is one of the major complications of diabetes. Hyperglycemia, inflammation and oxidative stress as well as advanced glycation end products (AGEs) are reported as the risk factors for DN. Sevelamer has shown promising results in reducing inflammation and even HbA1c levels in DN. Objectives: We aim to evaluate the therapeutic effects of sevelamer on blood sugar, HbA1c level, lipid profile, and high sensitivity CRP (hs-CRP) in patients with diabetic nephropathy. Patients and Methods: In this clinical trial, 18 patients (13 female with mean age of 61.22±9.32 years) with stages 2-4 of diabetic nephropathy were recruited. Patients were administered with 800 mg of sevelamer twice a day for three months. Blood sugar, HbA1C, hs-CRP, lipid profile and other laboratory findings were measured before and 1, 2 and three months after initiation of the treatment. Results: There was significant decline in HbA1c (p=0.001), postprandial blood sugar (p=0.02) and phosphorus (p<0.001) at the end of the study period. Sevelamer had no effect on fasting blood sugar, lipid profile, blood urea nitrogen, creatinine, Na, K and hs-CRP. Conclusion: Our results indicated that along with its phosphorus reducing effects, sevelamer plays an important role in decreasing postprandial blood sugar and HbA1c level. Sevelamer had no effects on lipid profile and hs-CRP