To evaluate the role of power Doppler in the identification and differentiation bet­ween acute renal transplant rejection and acute tubular necrosis (ATN), we studied 67 live donor renal transplant recipients. All patients were examined by spectral and power Doppler sono-graphy. Assessment of cortical perfusion (CP) by power Doppler was subjective, using our grading score system: P0 (normal CP); homogenous cortical blush extending to the capsule, P1 (reduced CP); cortical vascular cut-off at interlobular level, P2 (markedly reduced CP); scattered cortical color flow at the interlobar level. Renal biopsies were performed during acute graft dysfunction. Pathological diagnoses were based on Banff classification 1997. The Mann- Whitney test was used to test the difference between CP grades with respect to serum creatinine (SCr), and resistive index (RI). For 38 episodes of acute graft rejection grade I, power Doppler showed that CP was P1 and RI ranging from 0.78 to 0.89. For 21 episodes of acute graft rejection grade II, power Doppler showed that CP was P1, with RI ranging from 0.88 to >1. Only one case of grade III rejection had a CP of P2. Twelve biopsies of ATN had CP of P0 and RI ranging from 0.80 to 0.89 There was a statistically significant correlation between CP grading and SCr (P <0.01) as well as between CP grading and RI (P <0.05). CP grading had a higher sensitivity in the detection of early acute rejection compared with RI and cross-sectional area measurements. We conclude that power Doppler is a non-invasive sensitive technique that may help in the detection and differentiation between acute renal transplant rejection and ATN, particularly in the early post-transplantation period.

The influence of steroid maintenance on the outcomes of repeat kidney transplant (RKT) recipients with respect to induction type is unclear. Using the Organ Procurement and Transplant Network/United Network of Organ Sharing (OPTN/UNOS) database, we identified patients (≥18 years) who underwent deceased donor RKT from January 2000 to December 2008 after receiving induction with rabbit-antithymocyte globulin (r-ATG), alemtuzumab or an IL-2 receptor blocker (IL-2B) and were discharged on a calcineurin inhibitor/mycophenolate mofetil regimen with or without steroids. Of 5634 patients, 3643 received r-ATG (steroid = 3157, no-steroid = 486), 448 alemtuzumab (steroid = 196, no-steroid = 252) and 1543 an IL-2B (steroid = 1465, no-steroid = 78). Unadjusted graft survivals were similar for the no-steroid versus steroid groups for induction with r-ATG [hazard ratio (HR) 0.85 and 95% confidence interval (95% CI) 0.70-1.03, P = 0.10], alemtuzumab (HR 0.76, 95% CI 0.51-1.14, P = 0.18) and IL-2B (HR 0.77, 95% CI 0.56-1.70, P = 0.23). In the adjusted model, steroid use improved graft survival in alem­tuzumab (HR 0.44, 95% CI 0.25-0.76, P = 0.003) but not in the r-ATG (HR 0.86, 95% CI 0.68-1.09, P = 0.21) or IL-2B (HR 0.98, 95% CI 0.56-1.70, P = 0.94) groups. Steroid use was asso­ciated with inferior patient survival in unadjusted (HR 1.30, 95% CI 1.17-1.44, P <0.001) and adjusted (HR 1.29, 95% CI 1.14-1.45, P <0.001) models for r-ATG induction, whereas this was not observed with alemtuzumab (unadjusted HR 1.11, 95% CI 0.89-1.37, P = 0.36; adjusted HR 0.90, 95% CI 0.68-1.20, P = 0.49) or IL-2B (unadjusted HR 1.01, 95% CI 0.87-1.18, P = 0.87; adjusted HR 1.15, 95% CI 0.97-1.38, P = 0.12) inductions. Our study showed a graft survival benefit in the alemtuzumab- and patient death risk in the r-ATG-induced RKT recipients discharged on steroids.

Poor sleep quality is a common problem that can potentially predict mortality risk and quality of life in hemodialysis (HD) patients. Also, inflammation is a common feature in HD patients. To determine sleep quality and its correlation with serum C-reactive protein (CRP) level in these patients, we studied 132 chronic HD patients in two university-affiliated teaching hospitals in Sari, Iran during September 2010. Pittsburgh Sleep Quality Index was employed to assess the sleep quality of the patients in addition to CRP, albumin, cholesterol, phosphorus and hemoglobin levels. There were 104 (78.7%) patients who suffered from poor sleep quality. A significant correlation was found between serum CRP level and the patients' sleep quality (P <0.05). There was also a significant correlation between sleep quality and serum phosphorus and albumin levels and body mass index (P <0.05). Furthermore, a significant positive correlation existed between the different components of sleep quality and CRP (P <0.05). We conclude that there is a correlation between decreased sleep quality in HD patients and elevated CRP levels, which may have therapeutic implications.

Conventional echocardiographic (ECHO) parameters of systolic and diastolic func­tion of the left ventricular (LV) have been shown to be load dependent. However, the impact of pre-load reduction on tissue Doppler (TD) parameters of LV function is incompletely understood. To evaluate the effect of a single hemodialysis (HD) session on LV systolic and diastolic function using pulsed Doppler echocardiography and pulsed tissue Doppler imaging (TDI), we studied 81chronic HD patients (40 males; mean age 52.4 ± 16.4 years) with these tools. ECHO parameters were obtained 30 min before and 30 min after HD. Fluid volume removed by HD was 1640 ± 730 cm ^[3] . HD led to reduction in LV end-diastolic volume (P <0.001), end-systolic volume (P <0.001), left atrium area (P <0.001), peak early (E-wave) trans-mitral flow velocity (P <0.001), the ratio of early to late Doppler velocities of diastolic mitral inflow (P <0.001) and aortic time velocity integral (P <0.001). No significant change in peak S velocity of pulmonary vein flow after HD was noted. Early and late diastolic (E') TDI velocities and the ratio of early to late TDI diastolic velocities (E'/A') on the lateral side of the mitral annulus decreased signi­ficantly after HD (P = 0.013; P = 0.007 and P = 0.008, respectively). Velocity of flow progres­sion (Vp) during diastole was not affected by pre-load reduction. Pulmonary artery systolic pressure and the diameter of the inferior vena cava decreased significantly (P <0.001 and P <0.001, respectively) after HD. We conclude that most of the Doppler-derived indices of diastolic function are pre-load-dependent and velocity of flow progression was minimally affected by pre­load reduction in HD patients.

Medication adherence was assessed in 89 patients on hemodialysis (HD) at the King Abdul Aziz Medical City using an Arabic version of the Morisky Medication Adherence Scale (MASS-8). The results of the study revealed that 31.46% and 40.45% of the participants showed low and medium adherence, respectively, while 28.09% showed high medication adherence. Accordingly, 71.91% of the patients visiting the dialysis unit were considered medication non-adherent. While being of older age (P = 0.012), being married (P = 0.012) increased the level of adherence, being of medium level of education (P = 0.024) decreased adherence levels. On the other hand, gender, presence of a care-giver, number of members in the household and employment status seems to have no effect on the level of medication adherence. These results call upon the practitioners in HD units to develop intervention programs that can increase the level of medication adherence.

Fetuin-A, a hepatic glycoprotein present in the circulation, is a potential inhibitor for systemic calcification. The main aim of this study was to evaluate the association between fetuin-A and other biochemical parameters as facilitator factors for developing atherosclerosis in hemodialysis (HD) patients. This case-control study was conducted on 44 HD patients undergoing treatment in 2012. Parathormone (i-PTH) and fetuin levels were performed by the enzyme-linked immunosorbent assay method, high-sensitivity C-reactive protein (hs-CRP) by chemiluminescence, low-density lipoprotein by direct enzymatic, calcium and albumin by colorimetric and phosphorous by ultraviolet (UV) methods. Chi-square was used for evaluating the association between variables and t-test was used for comparing the mean of the quantitative variables for the two groups. SPSS-16 software was used for data analysis and P-value less than 5% was considered as significant. Mean of serum fetuin level was 23.25 ± 4.90 ng/mL in HD patients and 32.92 ± 5.21 in the control group. Median of hs-CRP was 2.45 mg/dL in the patients and 1.00 mg/dL in the control group and i-PTH was 74.3 pg/mL in the patients and 7.30 pg/mL in the control group. The calcium-phosphorous product was 46.77 ± 14.22 mg/dL in the patient and 31.73 ± 6.48 mg/dL in the control group. A reverse significant association was found between fetuin-A and hs-CRP in this study. In this study, serum fetuin-A level in HD patients was lower than controls. Therefore, a low level of fetuin-A seems to be associated with atherosclerosis, inflammation and malnutrition.

Sleep complaints are common in hemodialysis (HD) patients. Sleep quality (SQ) is a predictor of quality of life and mortality risk in HD. The aim of this study was to examine factors that may have a role in SQ. In this cross-sectional analytic study, 138 end-stage renal disease patients receiving maintenance HD for >3 months were included. The Pittsburgh Sleep Quality Index (PSQI) was used to measure individual's SQ. Patients with a global PSQI score >5 were assumed as poor sleepers. Eighty-eight patients (64%) were classified as poor sleepers. Poor sleepers were older and more likely had diabetes. They had significantly higher serum ferritin and calcium levels and lower serum parathyroid hormone level (all P-values <0.05). The global PSQI score was positively correlated with age, serum calcium level and presence of diabetes as the underlying cause of renal failure. In the multi-variable binary regression model, presence of diabetes (Odds Ratio (OR) = 3.67, P = 0.008) and body pain (OR = 1.182, P = 0.014) were the significant independent predictors for poor SQ. Poor SQ was common among our HD patients, especially among diabetic cases and, therefore, there is a need to pay more attention to the care of this subgroup with regard to the diagnosis and management of sleep complaints.

End-stage renal disease (ESRD) is a complex illness that involves different organs including the lungs. We studied the pulmonary function tests, arterial blood gases (ABG) and plasma endothelin-1 (ET-1) levels to check whether there is any change in their levels after hemodialysis (HD) in patients with ESRD. In this cross-sectional study (from July 2009 to April 2010), 20 patients with ESRD were evaluated. ABG, spirometric parameters and plasma ET-1 were measured before and after HD in these patients. Student's t-test was performed to clarify the differences and Pearson's test was used for correlations. P <0.05 was considered statistically significant. Significant reduction was seen in oxygen saturation (O ₂ sat), partial pressure of carbon-dioxide (PaCO ₂ ) and oxygen (PaO ₂ ) after a HD session (P <0.001). Also, improvement was seen in all spirometric parameters except forced expiratory volume (FEV1)/forced vital capacity (FVC) after HD. Plasma ET-1 levels decreased signi­ficantly after HD. Mean ET-1 before HD was 6.88 + 5.81 pg/mL while it was 3.91 + 2.76 pg/mL after HD (P = 0.009). Based on the plasma levels of ET-1, the patients were divided into two groups. The mean level of ET-1 was higher in the first group. Significant increase was seen in spirometric parameters in the second group. Our study suggests that, in patients with ESRD, plasma ET-1 level is higher than in the normal population, and this is closely related to deterioration of pulmonary function tests. Significant reduction of plasma ET-1 may be an important factor in the improvement of spiro­metry parameters after HD.

Patients on continuous ambulatory peritoneal dialysis (CAPD) are routinely eva­luated using the peritoneal equilibrium test (PET) to determine the best method for achieving target total dialysis clearance (T-Kt/V). In this study, we tested the hypothesis that standard CAPD prescription would achieve an initial T-Kt/V of more than 1.7 in all the patients regardless of their PET measurements. This is a retrospective study that included patients who started standard CAPD of four two-liter exchanges per day. The study included 118 patients; their mean age was 51.5 years with a standard deviation (SD) of 14.39 years. There were 83 males (70.3%) and 35 females (29.7%). PET and Kt/V were performed during the first four to six weeks of the study. The PET classified the patients into four categories: 24 (20.3%), high transporters; 65 (55.1%), high average; 28 (23.7%), low average; and one (0.8%), low transporter. Patients were then divided in two groups: Group 1 comprised of the high transporters while Group 2 included all the other patients. The T-Kt/V of the two groups was similar; in Group 1, it was 2.57 (±1.17) and in Group 2 it was 2.50 (±0.88) (P = 0.77). The T-Kt/V of patients with no residual renal function was also similar; in Group 1 and Group 2 it was 1.8 (±0.29) and 1.97 (±0.56), respectively (P = 0.45). All patients in our study who started on standard CAPD treatment had an adequate initial T-Kt/V. Thus, our data demonstrate that all patients with end-stage renal disease can safely begin standard CAPD without PET, which only needs to be performed if the patient encounters trouble in his/her T-Kt/V or fluid removal.

Visceral adiposity causes hypertension, hyperglycemia and dyslipidemia. This study was conducted to evaluate whether a correlation exists between body fat percentage (BFP) of chronic kidney disease (CKD) patients and their dietary intake. In this hospital-based, quasi-experimental study, 135 incident cases of CKD were included, of whom 76 completed the study. The patients included were aged 18 years and above and had a body mass index (BMI) between 18 and 25 kg/m ^[2] , had CKD of any etiology and serum creatinine of up to 5 mg/dL. Patients with acquired immunodeficiency syndrome, active hepatitis B or C, malignancy, previous kidney transplantation, current participation in any trial, diabetes mellitus and those who were on dia­lysis were excluded. The study patients were put on a diet of 25-30 kcal/kg/day, with 60% of the calories coming from carbohydrates and 20% each from protein and fat. Assessment was made at baseline (BL) and at 12 months (TM) for anthropometric parameters, skin-fold thickness, nutri­tional parameters, serum albumin and dietary intake (3-day dietary record) and clinical charac­teristics. No significant change was seen in BFP, waist circumference (WC) and BMI at BS and at TM. There was significant improvement in serum albumin (P <0.05) and e-GFR (P <0.01) while CRP was elevated both at BL and TM. The dietary intake was within the prescribed limit, with significant improvement in energy intake between BS and TM (P <0.05). The intake of delta dietary protein and fat positively correlated with delta e-GFR (P <0.001). There was a significant association between change in BFP and change in BMI (P <0.005). During follow-up, there was no significant change in biochemical parameters and BFP as well as stage of CKD of the study patients. This study supports the fact that dietary counseling is an important part of treatment in patients with CKD.

Renal biopsy plays an indispensable role in the diagnosis and management of patients with lupus nephritis (LN). A number of studies have evaluated the role of a repeat biopsy in case of disease relapse or treatment unresponsiveness. We studied 40 patients with LN with renal biopsies performed at baseline and after six months of therapy. The baseline and protocol biopsies were compared with respect to histological class transformation, crescents, tubular atrophy, interstitial fibrosis and glomerulosclerosis. We also compared serum creatinine, hemog­lobin, systemic lupus erythematosus disease activity index (SLEDAI) scores, 24-h urine protein excretion and C3levels as well as activity index (AI) and chronicity index (CI) at baseline and at six months. Comparison of means was made by paired t test, McNemar test and marginal homogeneity test (multinomial data). Histological class transformation was seen in 10 patients (25%). Intra-class progression to greater chronicity was seen in 10 other patients (25%).There was an increase in glomerulosclerosis, tubular atrophy, interstitial fibrosis and a reduction in cellularity, crescent formation and wire loop lesions in the protocol biopsy. A decline in AI (6.05 vs. 2.50, P <0.001) and SLEDAI scores (8.1 vs. 3.7, P <0.001) and an increase in CI (0.68 vs. 2.52, P <0.001) was observed at the time of protocol biopsy. Our study shows a trend toward greater chronicity in protocol biopsies in LN.

Cardiovascular diseases are a common cause of morbidity and mortality in subjects on regular hemodialysis. Insulin resistance is associated with increased cardiovascular diseases. Elevated serum ferritin is linked to insulin resistance. The aim of this work is to study the effect of desferoxamine therapy on some of the cardiovascular risk factors such as fasting insulin, B-cell function, insulin resistance, glucose, HbA1c%, lipid profile, blood pressure and carotid intima media thickness (CAIMT). Our study included ten subjects on regular hemodialysis with elevated serum ferritin. We measured the fasting serum glucose, HbA1c%, insulin, homeostatic model assessment of insulin resistance (HOMA-IR), fasting lipid profile, alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels and complete blood count (CBC). Statis­tically significant decreases in fasting serum insulin, B-cell function, glucose, HbA1c% and HOMA-IR were noted after deferoxamine therapy. No statistically significant difference was seen with regard to lipid profile, blood pressure and CAIMT. Iron overload increases insulin resistance and cardiovascular risk in hemodialysis subjects. Correction of anemia by iron therapy should keep target ferritin as per guidelines. Further studies are needed to determine the safest ferritin level among hemodialysis subjects.

To evaluate the effects of the co-administration of tacrolimus and ketoconazole to a group of kidney transplant recipients, we studied 30 kidney transplant recipients with stable kidney function who were maintained on tacrolimus-based immunosuppression. They were pres­cribed ketoconazole (100 mg/day) with a concomitant reduction in daily tacrolimus dose to maintain its level within the therapeutic range. The study included 19 males and 11 females with a mean age of 36 ± 12 years. All patients were at least three months post-transplant and had tacrolimus trough levels within the therapeutic range of 5-7 ng/mL. Desired tacrolimus trough levels could be achieved in 29/30 patients after the addition of ketoconazole. This resulted in a significant reduction of the median tacrolimus dose from 5 mg/day (range 3-20 mg/day) at baseline to 2 mg/day (range 1-4 mg/day) (P = 0.00). The median reduction in the tacrolimus dose was 63% (range 50-83%). The median monthly tacrolimus cost dropped from 375 US$ per patient (range 225-1440 US$) to 150 US$ per patient (range 120-300 US$). There were no reported adverse drug effects during the study period. After one year of follow-up, there was a small but significant improvement in the estimated glomerular filtration rate (72 ± 18 versus 78 ± 20 mL/min, P = 0.01) and a significant reduction in serum uric acid levels (7.7 ± 1.7 versus 5.9 ± 0.8 mg/dL, P = 0.003). The co-administration of ketoconazole and tacrolimus to kidney trans­plant recipients is safe and significantly reduces the cost of immunosuppression. In addition, this combination appears to have a beneficial effect on kidney function.

We conducted a single-center prospective double-arm open-labeled study on kidney transplant patients from 2010 to 2011 to evaluate the efficacy of induction therapy using low, single-dose rabbit-antithymocyte globulin (r-ATG), 1.5 mg/kg on Day 0 (n = 80, 60 males, mean age 35.9 years), versus basiliximab (Interleukin-2 blocker) 20 mg on Days 0 and 4 (n = 20, 12 males, mean age 45.1 years) on renal allograft function in terms of serum creatinine (SCr), rejec­tion and infection episodes and patient/graft survival and cost. Demographic and post-transplant follow-up including immunosuppression was similar in both groups. In the r-ATG group, donors were unrelated (spouse, n = 25), deceased (n = 31) and parents/siblings (others), with a mean HLA match of 1.58. Donors in the basiliximab group were living unrelated (spouse, n = 15) and deceased (n = 5), with a mean HLA match of 1.56. No patient/graft was lost in the r-ATG group over a mean of one year follow-up, and the mean SCr was 1.28 mg/dL with 7.5% acute rejection (AR) episodes; infections were also not observed. In the basiliximab group, over the same period of follow-up, there was 95% death-censored graft survival, and the mean SCr was 1.23 mg/dL with 10% AR episodes. One patient died due to bacterial pneumonia and one succumbed to coronary artery disease; one graft was lost due to uncontrolled acute humoral and cellular rejection. The cost of r-ATG and basiliximab were $600 and $2500, respectively. We conclude that induction immunosuppressive therapy with a low-dose r-ATG may be a better option as compared with basiliximab in terms of graft function, survival and cost benefit in kidney transplant patients.

Emphysematous pyelonephritis (EPN) is a group of potentially life-threatening conditions seen particularly in diabetics, leading to high morbidity and mortality. Our aim was to study the profile of emphysematous infections of the kidney and urinary tract and evaluate the effect of early surgical intervention on mortality. This is an observational study conducted in a tertiary care hospital between January 2009 and January 2013, in which the clinical, laboratory, microbiological and radiological profiles of patients with EPN (diagnosed based on clinical, laboratory and imaging findings) was studied. A total of 12 patients were studied, including 10 with diabetes. A total of 66.6% had pyelonephritis, 25% had both cystitis and pyelonephritis and 8.3% had only cystitis; involvement of the left kidney was more common and bilateral involvement was seen in two cases. The clinical features seen in the patients included fever (100%), features of urinary infection (66.6%) and flank pain (50%). Culture positivity was seen in only 50% of the cases. Ten patients underwent percutaneous drainage (PCD) within 24 h, and two of these patients required nephrectomy subsequently. All patients were followed-up for one month. There was one death (mortality 8.3%), and all other patients responded well and reco­vered. Our study suggests that EPN is a potentially life-threatening condition that requires aggressive and prompt medical therapy with early PCD to reduce morbidity and mortality. Nephrectomy should be reserved for cases that do not respond to PCD.

Steroid-resistant nephrotic syndrome (SRNS) patients are candidates for other alter­native drug regimes, and the non-responsiveness to steroid is more common among glomerulo-nephritides other than minimal change disease. Without performing biopsy and proper renal histology, progression of the disease cannot be assessed. Fractional excretion of magnesium (FE Mg) has been found to correlate directly with various renal histologies. The aim of this study is to evaluate the relationship of FE Mg in children with the histological pattern in SRNS. In this prospective observational study, 40 children of nephrotic syndrome, both with the first episode as well as relapse, aged 1-12 years were included in the study. Of them, 20 were steroid-responsive cases and 20 were steroid-resistant cases. FE Mg was determined in all the patients and renal histology was performed in the steroid-resistant cases. A correlation was found between FE Mg and renal histology. Data were analyzed in SPSS program version-16. Comparison of two groups was performed by the Fisher exact test and unpaired t test. P-value less than 0.05 were considered to be significant. The results of histo-pathology showed that the mean difference in FE Mg was significant (P <0.001), as FE Mg was 7.0 ± 2.3% in mesangiocapillary glomerulonephritis, 6.9 ± 1.3% in focal segmental glomerulosclerosis, 4.7 ± 0.6% in immunoglobulin M nephropathy, 4.5 ± 1.2% in focal segmental proliferative glomerulo-nephritis, 4.4 ± 1.6% in minimal change disease, 4.2 ± 0.4% in diffuse mesangial proliferative glome-rulonephritis and 3.8 ± 1.3% in mesangial proliferative glomerulonephritis. There was a statistically significant difference between FE Mg in steroid-resistant nephrotic syndrome (4.9 ± 1.9) and steroid-responsive syndrome (1.2 ± 0.3). FE Mg is a simple, minimally invasive screening marker for SRNS, and is an early predictor of clinical outcome. It can be considered as an initial investigation where biopsy cannot be performed or indications are not clear.

Emphysematous infection of the cysts in patients with polycystic kidney disease is very rare and seldom reported. We herein report a case of emphysematous polycystic infection in a 61-year-old male patient who was on peritoneal dialysis for end-stage renal disease caused by adult polycystic kidney disease.

Caroli's syndrome (CS) is a rare congenital disorder characterized by multiple segmental cystic or saccular dilatations of the intrahepatic bile ducts and congenital hepatic fibrosis. We report a 9-year-old boy who was diagnosed with CS and autosomal recessive poly-cystic kidney disease. On screening, his 5-month-old asymptomatic sister had multiple dilated biliary radicals with multiple bilateral renal cystic lesions. Both the patient and the affected sibling have been advised regular follow-up for monitoring the progression of the disease. In conclusion, patients with CS should be screened for renal cystic lesions and vice versa even if they are asymptomatic. Also, as the disease is inherited in an autosomal recessive manner, it is important to screen family members for early diagnosis and management.

Mixed connective tissue disease (MCTD) is an overlap syndrome first defined in 1972 by Sharp et al. In this original study, the portrait emerged of a connective tissue disorder sharing features of systemic lupus erythematosus, systemic sclerosis (scleroderma) and polymyositis. Scleroderma renal crisis (SRC) is an extremely infrequent but serious complication that can occur in MCTD. The histologic picture of SRC is that of a thrombotic micro-angiopathic process. Renal biopsy plays an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in MCTD patients, helping to predict the clinical outcome and optimizing patient management. We herewith report a rare case of SRC in a patient with MCTD and review the relevant literature.

Although hypercalcemia and hypercalciuria are known to occur in breast-fed pre-term infants, to the best of our knowledge, it has never been reported in a term baby previously. We report a term male baby who was followed-up during pregnancy for having bright kidneys, but a follow-up renal ultrasound (US) after birth had revealed normal scan. Laboratory inves­tigations revealed normal serum calcium (Ca), phosphorous (PO ₄ ) and alkaline phosphatase (ALP). The baby was being fed by breast milk. Follow-up US two months later showed early nephrocalcinosis along with hypercalcemia and hypercalciuria; by the age of three months, nephrocalcinosis was more extensive and the serum Ca level was more than 12 mg/L with hypercalciuria. Parathyroid hormone (PTH), phosphorous (PO ₄ ), ALP and thyroid function tests were all normal. Antenatal history revealed a hypothyroid mother who was maintained on L-thyroxin, calcium and vitamin D supplement during pregnancy. Her blood tests showed normal serum Ca, low PO ₄ and elevated PTH. The baby was diagnosed to have hypercalciuria and hypercalcemia secondary to maternal hypophosphatemia (maternal vitamin D deficiency). Breast feeding was stopped and the baby was started on formula, whereby he showed remarkable improvement both for his blood chemistry as well as his hypercalciuria.

The NPHS2 gene encodes podocin, a membrane protein that acts as the structural scaffold in podocyte foot processes. NPHS2 mutations are associated with steroid-resistant neph-rotic syndrome (SRNS), with the pathologic variant being focal and segmental glomerulosclerosis (FSGS), an emerging cause of end-stage renal disease in children. We describe a novel NPHS2 sequence variant in a girl with SRNS. Onset occurred at the age of seven years, with edema, hypo-proteinemia, hypoalbuminemia, hypercholesterolemia, hypertriglyceridemia and nephrotic protei-nuria. Renal function was normal and autoimmunity markers were negative. Proteinuria failed to decrease after standard steroid therapy. Renal biopsy showed FSGS. Cyclosporine therapy was instituted, but no remission of proteinuria was achieved and chronic renal failure developed. Mole­cular analysis of the NPHS2 gene revealed a homozygous nucleotide substitution in position c.451+3A>T in intron 3-4. This nucleotide substitution has not been reported in the literature till date. The effect of the detected substitution on podocin protein was demonstrated by renal biopsy RNA extraction and cDNA amplification analysis. This technique had never been applied to a NPHS2 mutation. Based on these results, immunosuppressive drugs were discontinued and conser­vative therapy was undertaken.

Henoch-Schonlein purpura (HSP) is the most common childhood vasculitis. Renal involvement in HSP is one of the major causes of chronic renal failure in children. It is important to start effective and relatively safe medication to prevent end-stage renal disease (ESRD). Mycophenolate mofetil (MMF) appears to be a promising therapeutic agent in many autoimmune diseases such as lupus nephritis and vasculitis. Herein, we describe the treatment with MMF of three patients with HSP nephritis. In two cases with rapidly progressive glomerulonephritis without response to steroid, after treatment with MMF, significant improvement in kidney function and proteinuria were observed. In another patient with HSP nephritic-nephrotic syn­drome who showed resistance to steroid, MMF offered a favorable effect. MMF seems to be a promising therapeutic agent in the treatment of the severe HSP nephritis.

We describe a 19-year-old male who developed diclofenac-induced acute inters­titial nephritis (AIN). Diffuse mononuclear cell infiltration was confirmed by renal biopsy and a Gallium (Ga)-67 scintigraphy revealed diffuse uptake of the isotope in both kidneys. His renal function had gradually and promptly recovered after initiation of low-dose prednisolone (0.5 mg/kg/day). There are no established criteria for the administration of corticosteroids in the treatment of drug-induced AIN. Moreover, no clear recommendations regarding the optimal dose and duration of steroid administration in the treatment for drug-induced AIN has been established. In addition, we discuss the clinical benefit of steroid treatment and the diagnostic impact of Ga scanning on the management of drug-induced AIN.

Liddle's syndrome is a rare cause of secondary hypertension. Identification of this disorder is important because treatment differs from other forms of hypertension. We report an interesting case of a 35-year-old lady, a known diabetic and hypertensive patient, who presented with features of hypertensive encephalopathy. The family history was unremarkable. Past treat­ment with various combinations of antihypertensive medications including spironolactone, all at high doses, failed to control her blood pressure. Upon evaluation, the patient had hypokalemic alkalosis, low 24-h urine potassium and suppressed plasma renin activity. Although these findings were similar to hyperaldosteronism, plasma aldosterone was lower than the normal range. Blood pressure decreased markedly after administration of amiloride. Along with hyporeninemic hypo-aldosteronism, the non-responsiveness to spironolactone and good response to amiloride esta­blished the diagnosis of Liddle's syndrome.

Renal involvement in tuberculosis occurs due to lympho-hematogenous dissemi­nation. However, glomerular involvement is an uncommon event. Crescentic nephritis compli­cating tuberculosis is a therapeutic dilemma and weighs the risk of worsening the infection after immunosuppressive therapy. We present here a case of miliary tuberculosis with immune com­plex crescentic nephritis with advanced renal injury requiring renal replacement therapy. A diagnosis of miliary tuberculosis was made on the basis of positive sputum AFB, lymph node biopsy showing caseating granulomas and urinary polymerase chain reaction being positive for mycobacterial antigens. The patient recovered renal function with anti-tuberculous therapy with­out requiring immunosuppressive therapy.

The success of a pediatric kidney transplantation program can only be judged by reviewing its results. We aim to audit our short-term outcome of pediatric kidney transplantation at the King Fahad Specialist Hospital-Dammam. A retrospective chart review was performed to collect data about recipient demographics, etiology of end-stage kidney disease, type of dialysis, type of donor and outcome. Between September 2008 and April 2012, 35 pediatric kidney trans­plantations (<16 year) were performed of a total of 246 kidney transplants (14.2%). The mean age was 8.1 years, with a mean weight of 23.3 kg, and there were 21 (60%) boys in the study. Kidney dysplasia/hypoplasia was the most common etiology (51.4%). Pre-emptive kidney transplantation was performed in six (17%) patients. Peritoneal dialysis was the most common mode of dialysis [24 (69%) children]. Living donation was the source of kidney allografts in 13 (37%) cases. During a mean follow-up of 1.5 years, one patient died and one graft was lost due to kidney vein thrombosis. The one year patient and graft survival rates were 97% and 94%, respectively. Efforts should now be focused on achieving optimal long-term results. There is also a need to encourage pre-emptive transplantation and living donation in this population.

Vesicoureteral reflux (VUR) is a common congenital renal tract anomaly in children. Reports from Sudan are scanty. We report the characteristics, presentation and outcome of primary VUR in a tertiary care hospital. The records of 30 patients (16 males; 53%) followed-up between January 2004 and December 2010 were reviewed. The mean age at the time of diagnosis was 4 ± 3.9 years and 47% were <2 years of age. Renal ultrasound scan (USS) failed to predict VUR in 17% of the patients. On voiding cysturethrogram (VCUG), VUR was bilateral in 57% and severe grade in 64%. Grades were not significantly associated with age, gender or site of VUR. Initial dimercaptosuccinic acid radionuclide scan showed renal damage in 61.5% of the patients. Renal damage was significantly associated with female gender and severe VUR, but not with age of onset or history of urinary tract infection (UTI). Thirteen patients (43.3%) presented with acute UTI, eight (26.6%) with non-specific urinary tract symptoms and nine (30%) with persistently elevated serum creatinine. Urine cultures were positive in 73% of patients, and E. coli was the most common pathogen. Renal impairment at presentation was significantly associated with bilateral severe VUR and history of UTI but not age or gender. After a mean follow-up period of 1.78 years (6 months to 5 years), 70% of patients remained with normal renal function and 30% progressed to chronic kidney disease; two of them died. In conclusion, our data is different from many studies. Features of primary VUR in Sudanese children are late age of onset, equal gender affection and predominance of severe grade. Presentation is associated with a high rate of UTI, renal damage and advanced renal impairment. Measures to improve early detection and treatment of VUR may reduce the risk of kidney damage.

Acute kidney injury (AKI) is one of the most challenging and serious complications of pregnancy. We present our experience on the clinical profile and outcome of 57 patients with pregnancy-related AKI, of a total of 580 patients with AKI seen during the study period. This is a prospective single-center study in a civil hospital conducted from January to December 2010. The most common age group of the study patients was 20-25 years; 43.8% of the patients had received antenatal care. AKI was observed in the puerperium (n = 34), early pregnancy (n = 10) and late pregnancy (n = 13). The cause of AKI included puerperal sepsis (63.1%), pregnancy-induced hypertension (PIH) (33.33%), post-abortion (22.80%), ante-partum hemorrhage (APH) (14%) and post-partum hemorrhage (PPH) (8%). Complete, partial and no renal recovery was observed in 52.64%, 21.05% and 26.31% of the patients, respectively. Low platelet count and plasma fibrinogen and high bilirubin, D-dimer and activated partial throm-boplastin time were observed more commonly in patients with partial recovery. Of the 57 patients, 50 received hemodialysis, three received peritoneal dialysis and seven patients were managed conserva­tively. A total of 13 patients developed cortical necrosis that was associated with sepsis in six, PPH and pre-eclampsia/eclampsia in three patients each and APH in one. Nine patients died, and the cause of death was septicemia in four, pre-eclampsia in three and APH and PPH in one patient each. In our study, puerperal sepsis was the most common etiological factor for pregnancy-related AKI. Prolonged oliguria or anuria were bad prognostic factors for renal recovery. Sepsis, thrombocytopenia, disseminated intra-vascular coagulation and liver involvement were associated with increased mortality.

Acute kidney injury (AKI) is a common and major complication of sepsis. Sepsis-induced AKI is associated with higher morbidity and mortality. A prospective study was designed to include all the patients with a provisional diagnosis of sepsis with AKI admitted in our inten­sive care unit from August 2009 to September 2010. Detailed demographic data including various clinical parameters, co-morbidities, investigations, complications and outcome were entered in a designated proforma and were analyzed. A total of 53 subjects with the provisional diagnosis of sepsis with AKI were included in the study. The majority of patients (60.37%) were female. The mean age of the study population was 45.84 ± 20.5 years. Forty-nine percent of the subjects were <45 years old and 26.4% patients were >65 years. Among the co-morbid conditions, 9.4% subjects had diabetes mellitus type 2. Among the primary causes of AKI, 72% of the cases were due to medical causes, in which pneumonia was the major cause, and 28% were due to surgical causes, in which cholecystitis was the major cause. 47.1% cases expired, 11.3% subjects left against medical advice and 41.5% cases had favorable outcome. Among the expired cases, 20.7% subjects expired within 24 h; for others, the median hospital stay was four days. This prospective study showed that the major causes of AKI were medical illness and pneumonia. Mortality in sepsis-induced AKI is significantly high. This highlights the importance of prevention of AKI in sepsis by early and renal-friendly aggressive treatment of sepsis and the need for improvement in the management of such AKI cases.