Recurrent Acute Kidney Injury Caused by Idiopathic Renal Hypouricemia: The First Report from Iran with A Novel Mutation.

Numerous factors have been involved in exercise-induced acute kidney injury (EIAKI), such as using non-steroidal anti-inflammatory drugs following exercise and idiopathic renal hypouricemia (IRHUC). IRHUC is an autosomal recessive inherited disorder characterized by impaired tubular uric acid transfer, impaired reabsorption, and accelerated uric acid secretion. Some IRHUC patients have been shown to have EIAKI. A 27-year-old police officer was admitted to the hospital due to anorexia and a serum creatinine level of 18 mg/dL, after a "tug-ofwar" game. After one dialysis sessions per day over five days, his creatinine dropped to 1.3 mg/dL. Six months later, he developed bilateral flank pain and red discoloration of urine, following a 300-meter chase of a convict, and his creatinine level increased to 2.3 mg/dL, which was corrected with proper hydration alone. Recurrent acute kidney injury can be due to hereditary renal hypouricemia, which should be considered among differential diagnoses for patients.  DOI: 10.52547/ijkd.6992.

Treatment Effect of Ergocalciferol on Bone Metabolism Indexes and Parathyroid Hormone in Hemodialysis Patients.

INTRODUCTION: Vitamin D deficiency is a common problem in end-stage renal disease patients under hemodialysis. Both active and nutritional vitamin D supplementation have been recommended for its treatment. In this study we aimed to evaluate the effects of treatment with ergocalciferol on bone metabolism indexes in hemodialysis patients. MATERIALS AND METHODS: In a randomized controlled trial, 40 hemodialysis patients were randomly allocated to the intervention (n = 20) and placebo (n = 20) groups. During the study, 4 patients in the placebo and 1 in the intervention group were excluded. Patients received calcitriol, 0.25 mg/d, with ergocalciferol, 50 000 IU, or placebo weekly for 3 months. Serum levels of 25-hydroxyvitamin D, calcium, parathyroid hormone, and alkaline phosphatase were measured before and after treatment. RESULTS: 25-hydroxyvitamin D levels were significantly improved in the intervention group (12.00 ± 4.90 ng/mL versus 29.89 ± 9.48 ng/mL, P < .001), but the placebo group had no improvement (14.23 ± 7.62 ng/mL versus 13.87 ± 8.04 ng/mL, P > .05). There was no significant changes in serum calcium, parathyroid hormone, or alkaline phosphatase levels in each group. Eight patients (42.1%) in the intervention compared to zero cases in the placebo group had normal 25-hydroxyvitamin D levels after treatment (P = .004). No cases of hypercalcemia were seen in the studied patients. CONCLUSIONS: Treatment with ergocalciferol could significantly improve vitamin D deficiency with no significant effects of serum calcium or parathyroid hormone levels.

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.

Successful Treatment of Severe Metabolic Acidosis Due to Acute Aluminum Phosphide Poisoning With Peritoneal Dialysis: a Report of 2 Cases.

Aluminum phosphide poisoning is common in our region. It can cause severe metabolic acidosis and persistent hypotension, which lead to cardiogenic shock and subsequently mortality. Oliguric or anuric acute kidney injury is seen in almost all patients with aluminum phosphide poisoning. Renal replacement therapies are recommended in these patients to improve metabolic acidosis and increase the rate of survival. We report 2 cases of severe acute aluminum phosphide poisoning treated successfully with peritoneal dialysis.

Association between hepcidin, haemoglobin level and iron status in stage 4 chronic kidney disease patients with anaemia.

OBJECTIVE: To explore the probable association of serum hepcidin and haemoglobin levels with iron and inflammation statuses in patients of chronic kidney disease stage 4 with anaemia. METHODS: The cross-sectional study was conducted at Tabriz University of Medical Sciences, Iran, from March 2011 to October 2012, and comprised patients of chronic kidney disease stage 4 with anaemia. Serum biochemical factors as well as hepcidin, ferritin, interleukin 6, high sensitivity C-reactive protein and iron levels were measured using standard methods. Statistical correlations were established using regression analysis and Pearson's correlation coefficient. RESULTS: There were 40 patients among whom 15(37.5%) were males and 25(62.5%) were females with an overall mean age of 55.68±14.4 years. There was a significant inverse relationship between hepcidin and haemoglobin levels (p<0.05). There were significant correlations between hepcidin with iron status, nutritional and inflammatory markers such as ferritin, Total iron binding capacity, albumin and interleukin 6 (p<0.05 each). CONCLUSIONS: Hepcidin had negative correlation with haemoglobin level in stage 4 chronic kidney disease patients with adequate iron stores, which could be effective in the development of anaemia in such patients.

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.

Diabetes mellitus, diabetes insipidus, optic atrophy, and deafness: A case of Wolfram (DIDMOAD) syndrome.

PURPOSE: To report a case of Wolfram syndrome (WS) characterized by diabetes mellitus, diabetes insipidus, progressive optic atrophy, and deafness. CASE REPORT: A 19-year-old female patient, a known case of diabetes mellitus type I from six years before, presented with progressive vision loss since four years earlier. On fundoscopic examination, she had bilateral optic atrophy without diabetic retinopathy. The patient also had diabetes insipidus, neurosensory deafness, and neurogenic bladder. CONCLUSION: WS should be considered a differential diagnosis in patients with diabetes mellitus who present with optic atrophy, and it is necessary to perform a hearing test as well as collecting 24-h urine output.

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.

Seizure, deafness, and renal failure: a case of barakat syndrome.

Barakat syndrome (also known as HDR syndrome) is an autosomal dominant disorder characterized by hypoparathyroidism, sensorineural deafness, and renal disease caused by mutation of the GATA3 gene located at chromosome 10p15. The exact prevalence of this disorder is not known but is very rare, with only about a dozen cases reported in the literature. Here, we report a case of 58-year-old man from Ardabil who presented with seizure due to hypocalcemia. Further history revealed bilateral deafness. Audiogram confirmed sensorineural hearing loss of both sides. His laboratory data were consistent with hypoparathyroidism and renal failure. He was diagnosed to have Barakat syndrome based on his clinical and laboratory data. In conclusion, we need to be aware of rare inherited conditions in a patient with abnormal physical and laboratory findings even though their initial presentation was seizure and hypocalcemia.

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.