R Ram

Renal Tubular Acidosis in Sjögren's Syndrome: A Case Series

Background: The exact frequency of distal and proximal renal tubular acidosis (RTA) in Sjögrens syndrome is unknown. Other features of Sjögrens syndrome like polyuria, glomerular manifestations, familial occurrence and pregnancy are not widely reported. The aim was to prospectively study the clinical features and outcome of distal and proximal RTA in Sjögrens syndrome and also report on other renal manifestations of Sjögrens syndrome. Methods: The present study is a prospective consecutive case series of patients who presented with a history suggestive of RTA and Sjögrens syndrome. All patients were followed for 1 year. The diagnosis of RTA was by fractional excretion of bicarbonate. The diagnosis of Sjögrens syndrome was according to the American-European classification system [modified by Tzioufas and Voulgarelis: Best Pract Res Clin Rheumatol 2007;21:989-1010]. Results: The total number of RTA patients diagnosed during this period was 149. Sjögrens syndrome accounted for 34.8% (52 of 149) of RTA patients. The important symptoms and laboratory parameters were oral and ocular symptoms in 23 (44.2%), dental caries in 12 (23%), body pains in 47 (90.3%), mean serum pH 7.202 ± 0.03, mean serum bicarbonate, 14.03 ± 1.66 mmol/l, and mean urine pH, 7.125 ± 0.54. There were 30 (57.6%) patients with distal RTA and 22 (42.3%) patients with proximal RTA. Conclusions: The clinical implication of the present study is that RTA is a common feature of Sjögrens syndrome. It may be missed if the presentation is not due to oral and ocular symptoms. The present study is also the only one with a 1-year follow-up.

Multiple metastatic infections in a hemodialysis patient with untunneled internal jugular catheter.

We present an end‐stage renal disease patient on dialysis with fever. The primary source was right internal jugular vein catheter which had metastatic infections in the body probably via an arteriovenous communication in a cavity in left lung. Patient had right psoas muscle abscess and a left kidney abscess. An 18F‐fluorodeoxyglucose‐positron emission spectroscopy scan was done to find out left kidney abscess. A search of literature did not reveal many patients of psoas abscess secondary to infection of hemodialysis access.

A 10-year follow-up of idiopathic membranous nephropathy patients on steroids and cyclophosphamide: a case series

Abstract The studies of idiopathic membranous nephropathy (IMN) require sufficiently long duration of follow-up to understand the effect of treatment on the development of end-stage renal disease (ESRD) in IMN. The aim was to assess the remission rates with steroids and cyclophosphamide regimen for IMN at the end of 10 years of follow-up. A prospective, open-label study performed in Nephrology department of a state run tertiary care centre in a southern state of India. Adult (age >18 years) patients with biopsy-proven IMN of at least 6-month duration were included in the study. Patients received a 6-month course of alternate months of steroid and cyclophosphamide. The patients were followed for 10 years. Study end points were doubling of serum creatinine, development of ESRD, or death. A total of 58 IMN patients were recruited from 1997 to 2001. Out of 58 patients included, only 48 patients could complete the treatment schedule in six months. The remission rate at the end of 10 years was 58.6% (34 in 58 patients). The probability of dialysis-free survival in our study was 89.6% at the end of 10 years follow-up. The regimen of steroids and cyclophosphamide in IMN had a remission rates not as high as reported before. It was associated with high relapse rates and more infections.

Renal manifestations in paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired chronic disorder characterized by a triad of clinical features - hemolytic anemia, pancytopenia, and thrombosis. Not many reports of renal involvement in PNH are available in literature. We present a case series of PNH with renal involvement. We present the data of PNH patients who attended to Departments of General Medicine and Nephrology at a government-run tertiary care institute in South India. The diagnosis of PNH in these patients during initial phase, between 1998 and 2004 was based on sucrose lysis and Hams test. After 2004, the diagnosis was based on flow cytometry to detect CD59 (membrane inhibitor of reactive lysis), a glycoprotein, and CD55 (decay accelerating factor) in regulation of complement action. The patient data were collected from 1998 to 2014. There were 14 patients of PNH in this period. The mean age was 37 years and the range was 16–68 years. There were eight females. Acute kidney injury (AKI) was noted in six patients. Dialysis was performed in four of them. The mean serum creatinine and urea at the initiation of dialysis were 5.4 ± 0.6 and 64.1 ± 6.1 mg/dl, respectively. The median number of hemodialysis sessions done was four. Renal biopsy was done in four patients. In three patients, the urinalysis and serum chemistry were suggestive of Fanconi syndrome. In our patients, three renal manifestations of PNH were identified. They were AKI, renal vessel thrombosis, and Fanconi syndrome. Chronic renal failure was not identified.

Isoniazid cerebellitis in a peritoneal dialysis patient

Isonicotinic acid hydrazide (Isoniazid, INH) is one of the primary drugs used in the treatment of tuberculosis. Adverse reactions to isoniazid occur in approximately 5%, including rash, fever, jaundice, peripheral neuritis, agranulocytosis, vasculitis and isoniazid hypersensitivity. From 75 to 95% of a dose of isoniazid is excreted in the urine. Slow acetylators rarely may accumulate toxic concentrations of INH if their renal function is impaired. In dialysis patients INH may cause cerebellitis, a rare adverse effect. A 53year-old patient of hypertension and end stage renal disease had been on peritoneal dialysis. He was on three 2.5% dextrose (Dianeal) exchanges. The peritoneal equilibration test revealed him to be a high transporter. He presented 15 days after initiation of isoniazid (5 mg/kg per day) and pyridoxine (40 mg/day) as a part of antituberculous treatment for pulmonary tuberculosis with clinical features like lack of finger-nose coordination, dysdiadokinesia, intentional tremor, wide based gait and impaired tandem walking. Brain MRI showed bilateral T2 weighted hyperintensities in dentate nuclei of cerebellar hemispheres (Fig. 1), hypointensity in T1 image sequences and there was no restriction in diffusion weighted sequences. The trough plasma pyridoxal5-phosphate (PLP) levels in fasting without abstaining from pyridoxine were < 5 μg/L (reference range; 5–50 μg/L by high performance liquid chromatography). Considering the low levels of PLP, the INH was stopped and pyridoxine supplemented at a dose, 120 mg/day. The clinical features disappeared in a week. In the last 5 years two more peritoneal dialysis patients have also had clinical features of cerebellitis and MRI for both of them revealed hyperintensities in dentate nuclei of cerebellar hemispheres. Both of them responded after stopping INH and increasing the dose of pyridoxine. Both of them were also high transporters. There have been a few reports of INH causing cerebellitis, but all patients were on haemodialysis. The increased sensitivity of the dialysis population to isoniazid neurotoxicity is predominantly due to inhibition the activation of pyridoxine to pyridoxal 5-phosphate by INH metabolites. In addition, there is rapid clearance of PLP by haemodialysis, resulting in a severe deficiency of this active metabolite. In peritoneal dialysis patients with high transporter membrane characteristic, the clearance of PLP may be profound. In addition

Disseminated Trichosporon infection in a renal transplant recipient

Trichosporon species are basidiomycetous yeast‐like anamorphic organisms (Basidiomycota, Hymenomycetes, Tremelloidae, Trichosporonales) that are widely distributed in nature. Trichosporon species colonize the skin and gastrointestinal tract of humans. We present a report of disseminated Trichosporon in a renal allograft recipient. Our patient satisfied the definitions of both “proven invasive trichosporonosis” and “probable pulmonary infection.” Only 2 reports of disseminated Trichosporon infection in renal transplant recipients, to our knowledge, have been published.

Cutaneous fungal infection in a renal transplantation patient due to a rare fungus belonging to order Pleosporales.

Fungal infections are being increasingly reported from immuno-compromised as well as immuno-competent patients. Transplant patients are on long term immunosuppressive therapy which makes them highly vulnerable to opportunistic fungal infections .These infections can be cutaneous or systemic. Several fungi have been reported to be the culprits such as Candida spp., Aspergillus spp., C. neoformans, P. carinii, and zygomycetes group of fungi. Cutaneous infections are most commonly caused by Pityriasis (tinea) versicolor, dermatophytes, and candida sp but these days the demtiaceous fungi are becoming more frequently reported .Here we report a case of post renal transplant cutaneous infection caused by dematiaceous fungus belonging to the order Pleosporales

Acute renal failure in a patient with mantle cell lymphoma.

Mantle cell lymphoma is a rare form of B‐cell lymphoma. We present a 54‐year‐old gentleman with mantle cell lymphoma. It was diagnosed based on the demonstration of B‐cell antigens CD20 and CD5. It was further confirmed by demonstration of overexpression of cyclin D1 on these atypical lymphocytes in the immunohistochemical staining. He also had acute renal failure and proteinuria. Renal biopsy revealed crescents and lymphomatous infiltration of tubulointerstitium. The presence of infiltrating cells with similar markers in both the lymph node and the kidney confirmed the infiltration of kidney with lymphomatous cells. Our present patient, after a thorough literature search, is found to be the second one with a glomerular lesion and tubulointerstitial infiltration by malignant lymphoma cells.

Emphysematous pyelonephritis: disappearing kidneys.

A 51-year-old man with type 2 diabetes mellitus for 10 years and hypertension for 8 years, who was a smoker and an alcoholic, presented with a history of fever, chills, and rigor of 4 days; abdominal pain and distension of 3 days; jaundice and anuria of 1 day duration. He had been binge drinking for 2 days before admission and had not taken insulin for several days. Examination revealed jaundice, tachypnea, tachycardia, a blood pressure of 90/70 mm Hg, and diffuse abdominal tenderness, with no guarding or rigidity. Investigations showed the following results: random blood glucose, 385 mg/dl; serum creatinine, 8.5 mg/dl; blood urea, 185 mg/dl; serum sodium, 142 mEq/l; serum potassium, 5.5 mEq/l; serum bilirubin, 8.0 mg/dl; hemoglobin, 13.5 g/dl; total leukocyte count, 30,800/μL; platelet count, 85,000/μL; and urine and blood cultures, sterile. Ultrasound abdomen showed multiple gas shadows in both the kidneys. Computerized tomography scan of the abdomen (Figure 1 and Supplementary Figure S1 online) revealed that the major parts of kidneys were replaced by gas, with only shreds of renal tissue visible. On the same day, bilateral ultrasound-guided percutaneous drainages were placed (Supplementary Figure S2 online). The pus revealed the growth of Escherichia coli. He was treated with pipercillin–tazobactum and aztreonam for 3 weeks. There was improvement in fever and hypotension. However, he remained dialysis dependent.

Renal infarction due to lupus vasculopathy

In the ISN/RPS 2003 classification of lupus nephritis (LN) renal vascular lesions are not mentioned. We present a patient with postpartum lupus vasculopathy. The renal biopsy in our patient showed concentric intimal thickening with narrowed lumen. No inflammatory changes were found. It also revealed immunoglobulin and complement deposition on the wall of the arteriole. These changes indicate lupus vasculopathy. The glomeruli revealed diffuse proliferative glomerulonephritis, with wire loops and cellular crescent in one glomerulus. The patient showed improvement with immunosuppression.