Membranous Nephropathy in a Patient with Charcot-Marie-Tooth Disease: Association of Myelin Mutations.

A 40-year-old female presented to the neurologist with gradually progressive weakness of distal and proximal muscles of both lower limbs and cramps for 2 years. She gave a history of similar illness in her paternal grandmother and her father. Her examination revealed bilateral foot drop and mild proximal muscle weakness. She was diagnosed to have peripheral neuropathy and subsequently treated conservatively. Over the next year, she noticed progressive swelling of both lower limb and frothy urine. A nephrology consultation was obtained, and a renal biopsy was done, which showed membranous nephropathy. She was started on steroids and subsequently on tacrolimus as the proteinuria progressively worsened. Her anti-phospholipase A2 receptor antibody was negative both in blood and in the kidney biopsy tissue. A search for a genetic basis of this rare clinical condition was made, and heterozygous mutation was detected in the myelin gene. This mutation was confirmed with genetic sequencing. The mutation is associated with MPZ gene and is associated with multiple hereditary sensorimotor neuropathy. MPZ knockout mice have been shown to have increased glomerular permeability and proteinuria.

Lupus Nephritis with Coexistent Antiphospholipid Antibodies Associated Nephropathy: A Case Report and Literature Review.

Antiphospholipid syndrome (APS) associated nephropathy is a distinct clinical entity and can occur in patients with systemic lupus erythematosus (SLE) independent of or associated with lupus nephritis. Associated APS nephropathy in a patient with lupus predicts poor renal outcome, especially if left untreated. Recognizing a coexistent APS nephropathy in a patient with lupus nephritis is of utmost importance. Here, we present a patient with severe lupus nephritis with antiphospholipid antibodies (aPLs) who had no thrombotic manifestations of APS clinically. On renal biopsy, she was found to have APS nephropathy. Remission was achieved after 3 months of anticoagulation and immunosuppression. This case illustrates the importance of renal biopsy in a patient of SLE with aPLs. Renal biopsy often alerts a treating rheumatologist of the presence of thrombotic involvement in such patients, thereby altering the treatment of such patients.

Xanthogranulomatous Pyelonephritis: Intrahepatic and Intrathoracic Extension.

A 32-year-old female presented to us with worsening cough and expectoration, low-grade fever, and malaise for 3 months. She gave a history of pregnancy loss secondary to urinary tract infection (UTI) a year back. At that time, she was told to have an obstructive right renal calculus. She also had a history of recurrent UTI in the past 1 year. She had no other comorbidities. Her clinical evaluation revealed an enlarged right kidney and reduced air entry in the right hemithorax. Radiological investigations revealed a large right kidney invading into the inferior surface of the right lobe of the liver and the right pleural space. A clinical diagnosis of xanthogranulomatous pyelonephritis was made, and she was advised nephrectomy. Intraoperatively, the right kidney was found to invade both the right lobe of the liver and the right pleural cavity through a right diaphragmatic defect. Histopathology of the kidney revealed the presence of foamy histiocytes suggestive of xanthogranulomatous pyelonephritis. Invasive xanthogranulomatous pyelonephritis is known, however, invasion into the extra-abdominal structures has not been reported in the literature. Our case is a rare manifestation of a rare clinical entity - xanthogranulomatous pyelonephritis.

Nontubercular Mycobacterial Infection in a Renal Allograft Recipient.

A 71-year-old male, a renal allograft recipient, presented to us with a history of fever and right palm swelling. He had a history of fever 7 years back when he was treated with antitubercular treatment (ATT). Three years back, he was diagnosed to have gout and he was started on allopurinol. He developed severe bone marrow toxicity and allopurinol was changed to febuxostat. On admission, routine investigations did not reveal any focus of infection. The fluid aspirate from the palm revealed acid-fast bacilli (AFB). He was started on ATT; however, he did not show significant improvement. Two months later, he developed multiple subcutaneous lesions, and the pus again came positive for AFB. Due to lack of improvement, the aspirate was sent for molecular diagnostic identification. The mycobacteria was identified as Mycobacterium haemophilum. His treatment was changed to rifampicin, clarithromycin, and ciprofloxacin. As he showed slow improvement, his immunosuppression was tapered slowly. At 7 months of therapy, he is clinically better and his lesions are healing. His renal functions stayed stable despite tapering of cyclosporine in a patient who is on rifampicin. This case, the first report of M. haemophilum infection in a kidney transplant recipient in India, illustrates the difficulty in diagnosing nontubercular mycobacterial infection in transplant recipients. It also emphasizes the dilemma in tapering immunosuppressive drugs in disseminated nontubercular mycobacterial infections where there are considerable interactions between ATT and immunosuppressives.

Unusual Case of Acute Lung Injury in a Renal Allograft Recipient.

A renal allograft recipient developed cough with hemoptysis on the 1st postoperative day. A chest X-ray was performed which was suggestive of fluid overload. His fluid was restricted and diuretics were added. On the same day, his pulmonary infiltrates worsened and a computed tomography (CT) of the chest was carried out, which was suggestive of the right lower lobe consolidation and left pleural effusion. He underwent a bronchoscopy and the lavage was sent for cultures, which did not grow any infective organism. Besides routine antibiotics, treatment for possible cytomegalovirus, fungal infections, and pneumocystis infection was instituted. Noninvasive ventilation was started on day 8. A repeat CT of the chest on the postoperative day 8 showed further worsening of the pulmonary infiltrates. As all the initial cultures and serology were negative, a possibility of interstitial pneumonitis was considered. Mycophenolate sodium was considered as a possible cause of the lung infiltrates and was withdrawn. The patient showed progressive improvement. His antibiotics were withdrawn. He was discharged on day 14. A repeat CT 4 weeks post transplant showed significant improvement in his pulmonary pathology. The acute lung injury was considered to be a drug reaction secondary to mycophenolate sodium. In a renal allograft recipient with persistent pulmonary infiltrates, interstitial involvement secondary to drugs should be considered if the patient does not improve with the standard treatment measures.

Hemophagocytic lymphohistiocytosis secondary to hemodialysis catheter-related blood stream infection.

A 57-year-old man on dialysis presented with fever due to Pseudomonas septicemia. Workup revealed very high triglycerides and serum ferritin levels. A bone marrow examination showed hemophagocytosis. A diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made and steroids were started. He was put on automated peritoneal dialysis. Patients' condition continued to deteriorate and he succumbed to his illness. This case illustrates the development of HLH secondary to infections which are increasingly being recognized in the literature. Often this diagnosis is missed as it becomes difficult to differentiate between sepsis and HLH. The presence of high ferritin, hypertriglyceridemia, and hemophagocytosis in the bone marrow confirms the diagnosis.

Fibroblast growth factor-23 levels in maintenance hemodialysis patients in India.

Fibroblast growth factor-23 (FGF-23) levels start rising early in patients with chronic kidney disease and is implicated in cardiovascular and overall mortality of hemodialysis patients. We conducted a prospective observational cohort study in stable dialysis patients looking into the levels of FGF-23 in hemodialysis patients and its association with various demographic and biochemical variables and mortality. A total of 91 patients were enrolled in the study. The mean FGF-23 levels were very high (1152.7 pg/ml). FGF-23 levels were significantly associated with serum phosphorus and parathyroid hormone (PTH) levels in univariate and multivariate analysis. No significant association between FGF-23 and cardiovascular comorbidities and overall mortality was seen. FGF-23 levels rise exponentially in maintenance hemodialysis patients. There is a strong association between FGF-23 and phosphorus and PTH levels. No association between FGF-23 and mortality was noted in our patients.

Mixed acid-base disorder secondary to topiramate use in traumatic brain injury.

We report a case of a man with traumatic brain injury. He was started on to prophylactic topiramate which led to a mixed acid-base disorder. He had severe metabolic acidosis secondary to renal tubular acidification defect and respiratory alkalosis secondary to hyperventilation. Withdrawal of the offending drug led to the prompt resolution of the acid-base disturbance.

Mesenteric ischemia masquerading as refractory peritonitis in continuous ambulatory peritoneal dialysis patients.

We report two cases of mesenteric ischemia in patients on long term peritoneal dialysis both of which were associated with poor outcomes. Both were diabetic and on peritoneal dialysis for a long time. On evaluation of refractory peritonitis we found evidence of non occlusive mesenteric ischemia. Despite adequate treatment both succumbed to their illness. Abdominal pathology, especially mesenteric ischemia leading to gut infarction, should be considered in patients with refractory peritonitis.

Transjugular renal biopsy in a case of nephrotic syndrome with extrahepatic portal venous obstruction.

Renal biopsy in patients with nephrotic syndrome helps to establish the pathological diagnosis and subsequent treatment. In certain circumstances, biopsies are difficult to obtain because of the risk of bleeding. We report a case where renal biopsy was obtained through the transjugular route in a patient who had nephrotic syndrome with extrahepatic portal venous obstruction.

Acute renal failure in the tropics: emerging trends from a tertiary care hospital in South India.

AIM: To study epidemiology, laboratory parameters, outcome and factors determining outcome of patients presenting with acute renal failure in our hospital. MATERIALS AND METHODS: A prospective study between December 1997-December 1999 in which all patients presenting with acute renal failure were included. Demographics, laboratory parameters, etiology, outcome and prognostic factors determining outcome were studied. RESULTS: 642 patients were part of the study. The predominant underlying cause for ARF was sepsis (153/642); overall mortality was 26.5%. Patients who had sepsis were admitted in the ICU, and patients with oliguria had poorer outcome. CONCLUSION: Sepsis continues to be the predominant cause of ARF. Overall mortality of our patients is better, because of the case mix, a large percentage of patients had acute gastroenteritis as a cause of CRF.

Granulocyte-macrophage colony-stimulating factor as an adjuvant to hepatitis B vaccination in maintenance hemodialysis patients.

Patients on maintenance hemodialysis (HD) have poor seroconversion rate after hepatitis B vaccination. The present study was designed to test the efficacy of granulocyte-macrophage colony-stimulating factor (GM-CSF) as an adjuvant to hepatitis B vaccination for improving seroconversion rate in maintenance HD patients. Twelve chronic HD patients were randomly assigned to receive either hepatitis B vaccination alone or hepatitis B vaccination 24 h after 1 dose of GM-CSF for primary immunization. A group of 16 chronic HD patients who had not seroconverted after a standard two-dose hepatitis B vaccination were randomly assigned either to a booster dose of hepatitis B vaccine alone or a booster dose given 24 h after one dose of GM-CSF. In the primary immunization group only 2 of 6 patients (33%) who had received vaccination alone, versus 5 of 6 patients (83%) who had received hepatitis B vaccine after one dose of GM-CSF, developed seroprotective antibody titers. Moreover, seroprotective antibody titers (IU/ml) were significantly higher in the latter group (275 +/- 286.5 vs. 14 +/- 22, p < 0.05). In patients who had not seroconverted with prior hepatitis B vaccination, GM-CSF adjuvant therapy significantly increased the seroconversion rate versus booster dose alone (87.5 vs. 25%, respectively, p < 0.02), with significantly higher seroprotective antibody titers (84 +/- 80 vs. 19 +/- 33 IU/ml, respectively, p < 0. 05). These findings suggest that administration of one dose of GM-CSF, as adjuvant therapy, prior to primary or booster dose hepatitis B vaccination may significantly increase seroconversion rate and seroprotective antibody titers in chronic HD patients.

Infective endocarditis as a cause of fever in hemodialysis patients.

Vascular access infections are common in maintenance hemodialysis patients especially with dual lumen cuffed catheter. Persistent infections may lead to valvular seeding and the development of infective endocarditis. Though antibiotic therapy may often suffice, many patients may require surgical correction which carries a high risk of mortality. However appropriate preoperative therapy may considerably reduce the risk of surgery in maintenance hemodialysis patients.

A randomised controlled trial of intradermal hepatitis B vaccination and augmentation of response with erythropoietin.

AIM: Intradermal administration of Hepatitis B vaccine (HBV) achieves better seroconversion in patients on dialysis compared to intramuscular administration. The aim of the study was to determine whether twice weekly intradermal injections of the vaccine can further augment the vaccine response as compared to once weekly injections. Patients with end stage renal failure on haemodialysis were randomly allocated over a period of 22 months to receive 20 mu gms of recombinant HBV by intradermal injections once a week (group 1) or twice a week (group 2) for 6 weeks. The patients recruited during the first 12 months of the study did not receive recombinant human erythropoietin (Epo) as it was not available (phase 1). During the last 10 months of study all patients received Epo (phase 2) in addition to HBV. RESULTS: A total of 85 patients were enrolled of whom 77 completed the study. There were 41 patients in group 1 and 36 patients in group 2. Seroprotection (anti HBs > 10 mIU/ml in the absence of HBs Ag and anti HBc) was achieved in 56.1% patients of group I compared to 77.8% of group 2 (p < 0.05). The seroprotection rate was 78.1% among patients receiving Epo (phase 2) compared to 60% among 45 who did not receive Epo (phase 1). Anti HBs titre in responders was 308.5 +/- 148.7 mIU/ml in patients of phase 2 compared to 198 +/- 112.8 mIU/ml in patients of phase 1 (p < 0.05). The subgroup receiving both Epo and twice weekly vaccine (group 2 of phase 2) had the highest seroprotection rate of 86.7%. CONCLUSION: Twice weekly intradermal vaccination is more effective than once weekly regime in achieving rapid seroconversion. The vaccine response may be augmented by use of Epo probably due to reduction in transfusion requirement and concomitant immunosuppression.