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Renal involvement in paroxysmal nocturnal hemoglobinuria (PNH) is usually clinically not apparent but, in cases with clinical involvement, varies from reversible acute dysfunction to chronic irreversible damage. We report two cases of acute kidney injury and one case of chronic kidney disease due to PNH. In all three cases a diagnosis of PNH was made after detection of renal dysfunction. The renal involvement was documented on both histology and on imaging studies. Finally, we briefly review the main literature data on renal involvement in PNH.

Background: Coronary artery disease accounts for significant morbidity and mortality in patients with chronic kidney disease (CKD). Besides the higher prevalence of traditional risk factors, several uremia-related factors may play a role in accelerated atherosclerosis, such as elevated levels of lipoprotein (a) (Lp(a)). The effect of maintenance hemodialysis (MHD) on Lp(a) levels is not well understood. The present work was carried out to study the Lp(a) levels in Stage 4 and Stage 5 CKD patients as well as the effect of MHD on Lp(a) levels in patients with Stage 5 CKD. Methods: The study subjects included 15 patients with Stage 4 CKD, 15 patients with Stage 5 CKD, and 15 age- and sex-matched healthy controls. Plasma Lp(a) was measured by ELISA in all the subjects at the time of entry into the study and after 4 weeks of MHD in patients with Stage 5 CKD. Patients on MHD were dialyzed two to three times weekly for 4 hr during each session. Results: Mean Lp(a) levels were significantly higher in patients with CKD than in control patients. In patients with Stage 4 CKD, the Lp(a) level was 34.0 ± 19.5 mg/dL, whereas in Stage 5 CKD the level was 49.0 ± 30.9 and in healthy controls it was 22.2 ± 16.4. In patients with Stage 5 CKD, 4 weeks of MHD led to a significant fall in Lp(a) levels by 23.6% (P

A retrospective analysis of 584 cases of acute poisoning admitted with a medical emergency to the Department of Medicine, GTB Hospital, Delhi, over a three-year period. The patients were analysed with respect to the age, sex, mode of poisoning, type of poison consumed and mortality. Of these, 42.63% were aged 20–30 years. Poisoning was used as a suicidal agent by 63.8% of the patients. The nature of the poison could not be ascertained in 15.92% of patients. Sedatives were involved in 13.36%. Aluminium phosphide poisoning was found in 11.82%. The overall mortality was estimated to be 13.18% with 53.2% being caused by the consumption of aluminium phosphide. There has been a change in the nature of poisons consumed and the number of cases of aluminium phosphide poisoning is declining. However, aluminium phosphide poisoning still remains a major threat as it carries a high mortality rate.

Interaction of patient in marital dyad may have bearing on long-term patient outcome. Depression, subjective stress, and marital discord have been reported in healthy spouses of patients with end-stage renal disease (ESRD). Depressed patients on dialysis along with their spouses can function as depressed dyad. We looked at the incidence and factors associated with depression and marital stress among Indian hemodialysis patients and their spouses.A total of 49 (32 males, 17 females) patients on maintenance hemodialysis and their spouses were independently administered Beck Depression Inventory (BDI), Revised Dyadic Adjustment Scale, and self-rated subjective quality-of-life scale. Their demographic parameters, socioeconomic status, and type of family (nuclear or joint) were also noted.About 57.1% of patients were depressed compared with 42.8% of spouses (p = 0.133). In both patients and spouses, BDI correlated with quality of life and perceived marital stress. About 36.7% of patients and 24.4% of spouses reported marital stress (p = 0.69). Male spouses had more marital stress compared with female spouses (p0.0001). Depression and marital stress in patients and spouses was not associated with socioeconomic status, literacy levels, and employment. Depression in patients had direct correlation with depression in spouse (r = 0.572, p0.0001) and degree of marital dissatisfaction in spouse (r = 0.623, p0.0001). Patients living in nuclear family were more depressed and had more marital stress.Married ESRD patients and their spouses function as a complex psychosocial dyad with significant two-way interactions. Social support, as is seen in joint families, leads to significantly lesser depression and better marital understanding.

We present a case of a 64-year-old man, a kidney transplant recipient with acute pyelonephritis and acute graft deterioration. He was diagnosed with Actinobaculum schaalii infection in urine cultures. He was treated with antibiotics for 3 weeks and recovered well. The case describes an unusual pathogenic infection in a kidney transplant patient.

Oxidative stress (OS) and endothelial dysfunction are implicated in accelerated atherosclerosis in chronic kidney disease (CKD). We assessed endothelial function, OS, and carotid intimal medial thickness (CIMT) and their correlates in 44 CKD stage 5 patients (group III) before and after hemodialysis (HD), 40 patients of CKD stages 3 and 4 (group II), and 25 matched controls (group I).OS was measured by serum concentration of antioxidants; vitamin C and fractional reducing ability of plasma (FRAP) and pro-oxidant; thiobarbituric acid reactive substances (TBARS). Ultrasonography of carotid artery for CIMT and of brachial artery for flow-mediated dilatation (endothelium-dependent dilatation, EDD) was done.TBARS increased significantly with severity of CKD. Antioxidants FRAP and vitamin C were significantly lower in CKD patients as compared with controls, but there was no significant difference between groups II and III. EDD decreased significantly with severity of CKD, whereas CIMT though higher in CKD patients as compared with controls was not significantly different between groups II and III. After a session of HD as compared with predialysis, levels of TBARS decreased, whereas those of FRAP, vitamin C, and EDD increased. On multivariate analysis, there was negative correlation of TBARS with glomerular filtration rate (GFR), serum albumin, hemoglobin, and EDD. Vitamin C had positive correlations with GFR, serum albumin, hemoglobin, and EDD. EDD had direct correlation with GFR, whereas CIMT correlated negatively with EDD.Endothelial dysfunction and OS occur early in CKD, are closely related to each other and structural atherosclerosis, and are proportional to decline in GFR.

Dengue virus infection can clinically manifest as dengue fever, dengue shock syndrome and dengue hemorrhagic fever. Acute kidney injury as a result of dengue virus infection can occur due to various reasons including hypotension, rhabdomyolysis, sepsis and rarely immune complex mediated glomerular injury. However, glomerulonephritis associated with IgA Nephropathy in dengue virus infection has not been reported previously. We report a case of 15-year-old boy who was admitted with dengue fever and dialysis dependant acute kidney injury. Urine examination showed microscopic glomerular hematuria and proteinuria. Kidney biopsy showed mesangial proliferation with mesangial IgA dominant immune complex deposits and acute tubular necrosis. A repeated kidney biopsy 6 weeks after clinical recovery showed reversal of glomerular changes as well as resolution of mesangial IgA deposits.

Inherited deficiency of certain factors is responsible for increased tendency to vascular thrombosis; however two genetic defects in the coagulation pathway may coexist and cause recurrent thrombosis. Previously studies of thrombophilia have focused on the identification of single gene defects with the concept that familial thrombophilia is a single gene disorder. Now it has become accepted that familial thrombosis in protein C-deficient families is caused by co-segregation of one or more additional genetic factors that increase the risk of thrombosis. Co-existence of two or more genetic abnormalities increases the risk of thrombotic tendencies in affected persons. Simultaneous presence of factor V Leiden and deficiency of protein C results in higher risk of thrombosis. We report two such cases with additional analysis of the family tree highlighting that dual abnormality results in higher penetrance of the disease among family members.

Sternum is resistant to infections and thus infrequent site of osteomyelitis. Involvement of sternum by Mycobacterium tuberculosis is rare. We report case of 37-year-old male with isolated tuberculosis of the sternum, a 14-year-old girl with tuberculosis of the sternum and Potts spine and a 55-year-old male with tubercular sinus of the sternum, three different presentations of the same disease.

Renal involvement in leprosy has been reported rarely in the literature. Acute kidney injury in patients with leprosy is uncommon and may occur due to acute tubular necrosis, drug-induced interstitial nephritis and rarely crescentic glomerulonephritis. The latter with histologic confirmation of the diagnosis has been reported in very few cases of leprosy. A 25-year-old male, on therapy for multibacillary leprosy, was found to have deranged renal functions on evaluation for a history of nausea, vomiting, swelling and episode of haematuria. Kidney biopsy was performed twice over a period of 2 weeks, showing progression from diffuse proliferative glomerulonephritis to crescentic glomerulonephritis, pauci-immune in nature. The patient was treated aggressively with intravenous steroids, following which his renal functions stabilized. Crescentic glomerulonephritis, an extremely rare phenomenon in leprosy, should be considered in these patients presenting with features of acute kidney injury. Timely performed renal biopsy assists in accurate diagnosis and appropriate management of the patient, hence preserving renal parenchyma. Rapid progression from diffuse proliferative glomerulonephritis to crescentic glomerulonephritis in a patient with leprosy is described herein for the first time in the literature.

Coronary artery disease accounts for significant morbidity and mortality in patients with chronic kidney disease (CKD). Besides the higher prevalence of traditional risk factors, several uremia-related factors may play a role in accelerated atherosclerosis, such as elevated levels of lipoprotein (a) (Lp(a)). The effect of maintenance hemodialysis (MHD) on Lp(a) levels is not well understood. The present work was carried out to study the Lp(a) levels in Stage 4 and Stage 5 CKD patients as well as the effect of MHD on Lp(a) levels in patients with Stage 5 CKD.The study subjects included 15 patients with Stage 4 CKD, 15 patients with Stage 5 CKD, and 15 age- and sex-matched healthy controls. Plasma Lp(a) was measured by ELISA in all the subjects at the time of entry into the study and after 4 weeks of MHD in patients with Stage 5 CKD. Patients on MHD were dialyzed two to three times weekly for 4 hr during each session.Mean Lp(a) levels were significantly higher in patients with CKD than in control patients. In patients with Stage 4 CKD, the Lp(a) level was 34.0 +/- 19.5 mg/dL, whereas in Stage 5 CKD the level was 49.0 +/- 30.9 and in healthy controls it was 22.2 +/- 16.4. In patients with Stage 5 CKD, 4 weeks of MHD led to a significant fall in Lp(a) levels by 23.6% (P0.001).The results of this study show that increases in Lp(a) levels start early during the course of CKD and become more pronounced with increased severity of disease. Initiation of MHD lowers Lp(a) levels and may have a long-term beneficial effect on cardiovascular morbidity and mortality.

While the use of anti epileptic drugs (AEDs) for a long period is a known risk factor for bone loss and pathological fractures, yet the physicians are not yet sensitized to this possibility. It is now believed that the patients who have fractures due to long-term treatment with anticonvulsants have osteomalacia as the predominant lesion. This has been attributed to the alterations in the levels of circulating calcium and calcitropic hormones. Here we report a case of a young male who had been on anticonvulsants for 11 years and was admitted with us with severe bone pains, multiple pathological pseudo fractures and a severe degree of disability secondary to phenytoin induced osteomalacia.

The hepatitis B virus (HBV) is estimated to have infected about 350 million people worldwide, making it one of the most common human pathogens. Renal involvement is among its most common extra hepatic manifestations and usually manifests in the form of immune complex mediated glomerulopathy, such as membranous glomerulonephritis (MGN), membranoproliferative glomerulonephritis (MPGN), mesangioproliferative glomerulonephritis and immunoglobulin A (IgA) nephropathy. Occurrence of focal and segmental glomerular sclerosis (FSGS) with HBV infection is rare and only five cases have been reported earlier. We report two cases of hepatitis B associated FSGS. In both the cases, HBsAg was demonstrated in the renal tissue and both the cases showed response to treatment with lamivudine, thus indicating a possible causal association between the viral infection and occurrence of nephrotic syndrome.

We aimed to study the clinical profile, prognostic factors, and the 6-month outcome of patients with emphysematous pyelonephritis (EPN) METHODS: All patients admitted with a diagnosis of emphysematous pyelonephritis between January 2001 and July 2007 were included.Overall 19 cases were diagnosed to have emphysematous pyelonephritis. There were 16 females and three males. Fourteen cases had type 2 diabetes mellitus. Fourteen cases had unilateral involvement and five had bilateral involvement. Eleven cases were classified as having class 1 or 2 disease and eight cases had class 3 and 4 disease. E. coli was the most common organism cultured (68.4%). Five cases underwent percutaneous drainage of the collecting system and three cases had nephrectomy of which 10.5% (two with advanced disease) expired. Shock at admission (p = 0.03), serum creatinine5.0 mg/dl (p = 0.035) and DIC (p = 0.017) were independent poor prognostic factors. There was no difference in the prognosis between patients who hador=2 or2 poor prognostic factors (p = 0.16). However, prognosis was not related to disease class, unilateral vs. bilateral involvement, sepsis or the age of the patient. At 6 months, two patients were on maintenance hemodialysis.In cases of EPN, shock, serum creatinine5.0 mg/dl and DIC at admission are poor prognostic factors. Larger prospective studies are needed to confirm our findings.

A 14-year-old girl presented with a 10-year history of a large crusted plaque over the right thigh for 10 years and small reddish plaque over the left upper back for 3 months. On routine evaluation, she was found to have hematuria. Skin biopsy from the lesion was suggestive of skin tuberculosis (lupus vulgaris), and kidney biopsy showed features of IgA nephropathy (IgAN). Fine-needle aspiration from the inguinal lymph node was consistent with granulomatous disease. The patient has been on anti-tubercular treatment, and the hematuria has subsided.

Noonan syndrome is characterised by short stature, typical facial dysmorphology and congenital heart defects. Urogenital abnormalities are reported in 10% of the cases. We present a 14-year-old girl with characteristic features of Noonan syndrome and nephrotic-range proteinuria. She had crossed fused ectopic kidneys. Renal biopsy showed focal segmental glomerulosclerosis. Oral steroids were instituted and she responded well. The case highlights this novel renal presentation of Noonan syndrome.

To the Editor: The management of subnephrotic proteinuria in membranous nephropathy is challenging. We retrospectively analyzed fifteen biopsy-proven membranous nephropathy adults with proteinuria of 1–3 g/day (mean 2.4 g/day) and mean serum creatinine of 0.8 mg/day. Secondary causes were ruled out. All the cases were on conservative therapy receiving angiotensin-converting enzyme inhibitors (ACEI) and angiotensin receptor blockers (ARB). The mean blood pressure was 124/ 82 mm/Hg and mean serum potassium was 4.0 meq/l. Aldosterone antagonists or receptor blockers were not used. After six months of ACEI and ARB, nine cases were still proteinuric in the range of 1–3 g/day. Aliskiren (150 mg/day) was added and titrated to a maximum tolerated dose of 300 mg/day. Three months later, in eight cases mean blood pressure was 120/76 mm/Hg and mean proteinuria decreased to \500 mg/day which was independent of the systemic blood pressure-lowering effect of the drug (Table 1). The cases were followed for additional three months. The benefits continued and there was no relapse of the disease or worsening of renal functions. None of the cases developed side-effects needing the drug to be stopped. This is a preliminary analysis of the use of aliskiren in membranous nephropathy. It highlights the fact that despite adequate blood pressure control and use of ACEI and ARB, there is a need to block renin angiotensin pathway by other steps. Aliskiren interferes with first and rate-limiting step in renin enzyme cascade. The high specificity of renin for its substrate reduces likelihood of unwanted interactions and side-effects. Preliminary data in humans show that aliskiren reduces blood pressure and proteinuria [1]. It has also been found useful in hypertensive diabetics with proteinuria [2]. The antiproteinuric benefit is likely to be related to both lowering of intraglomerular pressure and the anti-inflammatory/antiproliferative effects of the drug. Our analysis has some limitations. The types of ACEI and ARB were based on the physician’s preference and aldosterone blockade was not achieved in any of the cases. Second, we had a small sample size and short duration of follow up. A larger randomized controlled trial is needed to fully explore the benefits of aliskiren in proteinuric diseases. To conclude, aliskiren has proved a useful adjunct to the combination of ACEI and ARB in management of membranous nephropathy and the benefits may possibly be extrapolated to other glomerular diseases. Ankur Gupta, act as guarantor for the other authors.

To the Editor: The occurrence of parasitic infections of the gastrointestinal tract is well known in renal transplant recipients. Some of the infections manifest as chronic diarrhea and pain in the abdomen; the seemingly easy diagnosis often proves difficult. We describe here a 50-year-old live related renal transplant recipient on prednisolone 10 mg/day, mycophenolate mofetil 1,500 mg/day and tacrolimus 2 mg/day with a baseline serum creatinine of 1.3 mg/dl 3 months posttransplant. He was admitted with a painful abdomen and increased frequency of stools for the past 4 weeks. Stools were watery, occurring four to five times per day, and were without mucus. On examination, the patient was dehydrated with a blood pressure of 106/60 mmHg. Systemic examination was noncontributory except for diffuse abdominal tenderness. Investigations showed hemoglobin 13.5gm/dl, leukocyte count 13,200/mm, blood urea 94 mg/dl, serum creatinine 2.2 mg/dl and tacrolimus trough level 8.5 ng/ml. Blood culture was sterile. Ultrasound of the abdomen was normal. Initial stool microscopy was normal. He was given intravenous fluids, levofloxacin 250 mg/day and metronidazole 500 mg thrice daily. Antibiotics were stopped after 5 days as there was no response. Mycophenolate mofetil was changed to mycophenolate sodium preparation, and his frequency of stool decreased to two to three times/day for the next 3 days, but again his diarrhea worsened. A viral screen, including cytomegalovirus PCR, hepatitis B surface antigen, anti-hepatitis C antibody, IgM anti-hepatitis A and E antibodies, and ELISA for HIV were negative. Stool examination for clostridium difficile toxin was negative. Sigmoidoscopic biopsy showed mucosa with focal extension of chronic inflammation in the lamina propia and mild edema without any evidence of infection or viral inclusion bodies. A repeat stool microscopy showed eggs of Hymenolepis nana (H. nana) in simple wet mount preparation. No other parasite or pathogenic bacteria were found by either stool examination or culture. Nitazoxanide 500 mg twice daily for 3 days was given along with praziquantel 25 mg/kg body weight once. The patient’s condition improved over the next couple of days, and repeat stool samples were negative for H. nana. Renal functions returned to baseline within a week. H. nana can cause troublesome diarrhea in renal transplant recipients. A simple detailed stool analysis can diagnose it. In one of the studies, 4.5% renal transplant recipients were positive for parasitic infections, of which 0.3% had ova related to H. nana [1]. Immunosuppression breaks the balance between the agent and the host. In laboratory models, cyclosporine reduces survival, growth and fecundity in a wide range of protozoans and helminths [2], but this has not been reported with tacrolimus and needs evaluation. However, the role of H. nana as an opportunistic infection is unclear and requires more We certify that a written consent for publication of the case has been obtained from the patient.

To the Editor: The article 'Early steroid treatment improves the recovery of renal function in patients with drug-induced acute interstitial nephritis' has great significance in clinical practice but needs to be interpreted with caution.1

Diabetic nephropathy is a potential consequence of mitochondrial dysfunction and localized tissue oxidative stress. A number of pathways that generate reactive oxygen species (ROS), such as glycolysis, specific defects in the polyol pathway, uncoupling of nitric oxide synthase, xanthine oxidase, NAD(P)H oxidase, and advanced glycation, have been identified as potential major contributors to the pathogenesis of diabetic chronic kidney disease (CKD). 1 Nrf2 is an active transcription factor for the expression of mRNA that codes for synthesis of a host of cytoprotective molecules. These molecules include enzymes that directly destroy ROS, as well as enzymes that synthesize small molecules such as glutathione that scavenges potentially destructive electrophiles. Animal models have suggested the role of genetic deficiency of Nrf2 in pathogenesis and histology of renal disease including glomerulosclerosis. Also, Nrf2 activation is suppressed in animal models of CKD. 2,3 In search for a specific, well-tolerated agent for the therapeutic induction of Nrf2, a new class of compounds—synthetic oleanane triterpenoids (SO), specifically the methyl ester of 2-cyano-3, 12-dioxooleana-1,9(11)-dien-28-oic acid (bardoxolone methyl) as the most potent representative of this group of molecules, is being focused. The cytoprotective effects are believed to be mediated by binding of the SO to the inhibitory protein Keap1, which then releases its partner, Nrf2 transcription factor, that results in the upregulation of several antioxidant genes including NAD(P)H: quinone oxidoreductase 1, thioredoxin, catalase, superoxide dismutase, and heme oxygenase. This results in the reduction of intracellular ROS and proinflammatory activity of the nuclear factor kappa-light-chain-enhancer of activated B cells pathway, thereby restoring redox homeostasis in areas of inflammation. 4 A recent, phase 2a, multicenter, openlabel study in 20 patients with moderate to severe diabetic CKD showed an apparent increase in kidney function following relatively short-term treatment with bardoxolone methyl with no life-threatening adverse events. 5 Apart from diabetes, SO have been used favorably in experimental animal models of kidney injury caused by toxic agents, cystic fibrosis, and emphysema induced by cigarette smoke, disease states characterized by hyperactivity of the immune system, cancer, including prevention and treatment, Parkinson’s disease, Huntington’s disease, and Alzheimer’s disease. 6 To conclude, bardoxolone methyl and other SO could form the basis of therapies halting the progression of CKD and may be even reversing them. Larger studies are needed to confirm beneficial effect of this drug. Internists should be ready to greet this drug with next sunrise.

We evaluated important nontraditional cardiovascular risk factors, endothelial function and oxidative stress (OS) among stable peritoneal dialysis (PD) patients. Their association with carotid intimal medial thickness (CIMT) was also assessed. Thirty-eight adult patients (13 diabetics, 20 males) on PD for >6 months and 15 age and sex-matched controls were studied. Duration of dialysis (DOD), residual urine output (UO), weekly Kt/V urea, detailed biochemical and lipid profile were noted. OS was measured by serum concentration of antioxidants; vitamin C and ferric reducing ability of plasma (FRAP) and pro-oxidant; thiobarbituric acid-reactive substances (TBARS). High-resolution ultrasonography was used to determine CIMT and flow-mediated dilatation of brachial artery [endothelium-dependent dilatation (EDD)] and dilatation subsequent to nitrate spray [endothelium-independent dilatation (EID)]. Mean age, DOD, UO and Kt/V of study population were 49.3 ± 11.6 years, 19.4 ± 11.8 months, 508.2 ± 422.9 ml/day and 1.73 ± 0.24, respectively. As compared to controls PD patients had higher CIMT (0.46 ± 0.05 vs 0.50 ± 0.07 mm, P = 0.003) and TBARS (1.5 ± 0.4 vs 5.1 ± 2.3 nM/ml, P < 0.001) but lower Vitamin C (1.7 ± 0.3 vs 0.6 ± 0.2 mg%, P < 0.001), FRAP (990.8 ± 78.1 vs 328.7 ± 183.5 μM/L, P < 0.001) and EDD (26.2 ± 5.4 vs 9.8 ± 4.6 %, P < 0.001). TBARS correlated positively with DOD and negatively with hemoglobin. Vitamin C and FRAP correlated positively with serum albumin. EDD correlated positively with UO, Kt/V and hemoglobin. CIMT correlated negatively with Kt/V and hemoglobin. Among themselves CIMT correlated negatively with EDD and vitamin C. EDD correlated positively with vitamin C, while FRAP correlated positively with vitamin C and negatively with TBARS. PD patients have higher OS, poorer endothelial function and higher structural atherosclerosis. These parameters are closely linked to each other, hemoglobin, DOD, residual UO, serum albumin and small solute clearances.