Ps Priyamvada

Subcutaneous phaeohyphomycosis in kidney transplant recipients: A series of seven cases

Superficial and deep fungal infections are more frequent in transplant recipients primarily because of the failure of cell‐mediated immunity and lesser amount of antigen‐presenting Langerhans cells in their epidermis. Here, we report seven cases of post‐renal transplant subcutaneous phaeohyphomycosis, all of which manifested within 1 year after transplantation and were unresponsive to prolonged courses of itraconazole. This is the first case series, to our knowledge, of phaeohyphomycosis in transplant recipients in India.

Karyomegalic interstitial nephropathy following ifosfamide therapy

Ifosfamide (IFO), an alkylating agent used for the management of solid organ tumors, can cause reversible Fanconis syndrome and acute kidney injury. Karyomegalic interstitial nephropathy (KIN) is a rare form of chronic tubulointerstitial nephritis, initially described as a familial nephropathy in adults. So far, four cases of KIN have been reported in pediatric and adolescent population following treatment with IFO. We report a 22-year-old man who developed renal dysfunction following IFO therapy for relapsed Hodgkins lymphoma. Renal biopsy revealed chronic tubulointerstitial nephritis with atypical tubular epithelial cells showing nuclear enlargement and hyperchromasia, consistent with a diagnosis of KIN. The renal function improved following a short course of corticosteroids.

Medullary sponge kidney and isolated hemihyperplasia.

The term hemihyperplasia refers to an enlargement of body parts beyond the normal asymmetry. Hemihyperplasia can be isolated or associated with various well-described malformation syndromes. Medullary sponge kidney (MSK) has been described with isolated and syndromic hemihyperplasia; the actual prevalence is not known The hemi hypertrophy can be so subtle that it may be easily overlooked. MSK need not be limited to the side of hemihyperplasia – most often it is bilateral. Around 33 cases has been reported from different parts of the world of which 15 cases are isolated hemi hyperplasia (IHH), the remaining occurring in the context of various malformation syndromes So far only one case has been reported from India. We report a case of IHH involving right side of the body, recurrent renal stones, incomplete distal renal tubular acidosis hypercalciuria and imaging showing bilateral MSKs.

Correction of metabolic acidosis improves muscle mass and renal function in chronic kidney disease stages 3 and 4: a randomized controlled trial

Background Metabolic acidosis (MA) is associated with a loss of muscle mass and faster deterioration of kidney function in patients with chronic kidney disease (CKD). A few single-centre randomized trials have reported favourable outcomes following correction of MA. Additional good quality evidence on the safety and efficacy of alkali supplementation is required in epidemiologically different patient subsets with CKD. Methods A single-centre, open-label, randomized, prospective parallel-group study was conducted to assess the effect of correction of MA on body composition and kidney function. A total of 188 patients with CKD stages 3 and 4, with venous bicarbonate levels <22 mEq/L were randomized. The intervention arm received standard care as per Kidney Disease: Improving Global Outcomes (KDIGO) 2012 guidelines along with oral sodium bicarbonate supplementation to maintain venous bicarbonate levels at 24-26 mEq/L, whereas the control group received standard care alone. The mid-arm muscle circumference (MAMC), lean body mass (LBM) and estimated glomerular filtration rate (eGFR) were compared between the groups at the end of 6 months. Results The intervention arm showed a higher LBM {36.8 kg [95% confidence interval (CI) 36.5-37.1] versus 36 [35.7-36.4]; P = 0.002} and MAMC [22.9 cm (95% CI 22.8-23) versus 22.6 (22.5-22.7); P = 0.001] when compared with the control group. The GFR in the intervention arm was higher [32.74 mL/1.73 m2 (95% CI 31.5-33.9) versus 28.2 (27-29.4); P ≤ 0.001]. A rapid decline in GFR was documented in 39 (41.5%) patients in the control arm and 19 (20.2%) patients in the intervention arm (P = 0.001). Conclusions Alkali supplementation to increase venous bicarbonate levels to 24-26 mEq/L is associated with preservation of LBM and kidney function in patients with CKD stages 3 and 4.

Cerebral venous thrombosis in a patient with acute postinfectious glomerulonephritis

Thrombosis of the cerebral venous sinuses (CVT) is described in nephrotic syndrome. A 13-year-old girl was admitted with acute post-infectious glomerulonephritis (APIGN). Subsequently she developed recurrent seizures with focal neurological deficits. On evaluation, she was found to have CVT. To the best of our knowledge, this is the first report of CVT in APIGN. Identifying this complication is imperative, as timely diagnosis and treatment could be lifesaving.

Acute gastric dilatation in a patient with lupus nephritis: An uncommon presentation of lupus mesenteric vasculitis

Abdominal symptoms are common in patients with lupus nephritis and are often attributed to drugs or uremia per se. Lupus mesenteric vasculitis (LMV) or lupus enteritis is a rare entity reported in patients with active systemic lupus erythematosus. It usually occurs in patients with a long-standing history of lupus with high disease activity. Usually, small bowel is predominantly affected. The stomach and rectum are spared in view of significant collateral circulation. Here, we describe an 18-year-old boy who presented with nephrotic syndrome without any extrarenal features of lupus. On subsequent evaluation, he was found to have active lupus nephritis. He developed acute gastric dilatation secondary to extensive LMV. Imaging showed an extensive involvement of gastrointestinal tract from the stomach to the sigmoid colon, sparing the rectum. To the best of our knowledge, this is the first report of LMV presenting as acute gastric dilatation.

Assessment of health-related quality of life and its determinants in patients with chronic kidney disease

Health-related quality of life is an important, yet neglected aspect of chronic kidney disease (CKD) care. We evaluated the quality of life and its determinants across CKD 3 to 5D using a kidney disease specific tool (Kidney Disease Quality of Life-SF™) in an underprivileged, predominantly rural population with high rates of illiteracy and unemployment. The scores of individual domains were summarized to three composite scores – physical composite summary (PCS), mental composite summary (MCS), and kidney disease component summary score (KDCS). A total number of 204 participants were recruited from nephrology outpatient clinics. About 68.1% of participants were males. The mean age of the study population was 49.14 ± 13.63 years. There was a high proportion of illiteracy (36.3%) and unemployment (80.9%). KDCS showed a significant decline (P = 0.01) from CKD 3 to CKD 5D whereas MCS and PCS showed a nonsignificant decrease. There was no difference in KDCS, PCS, or MCS scores between patients treated by hemodialysis and CAPD. Illiteracy and unemployment were associated with significantly lower KDCS, PCS, and MCS scores. Age ≥50 years was associated with poor PCS (29.49 ± 8.20 vs. 34.17 ± 9.99 ; P< 0.001). Hemoglobin <10 g/dL was associated with poor KDCS (58.93 ± 13.09 vs. 65.55 ± 13.38 ; P< 0.001) and PCS (29.56 ± 8.13 vs. 33.37 ± 9.82; P< 0.001). The presence of comorbidities such as diabetes and hypertension had no impact on the composite scores. KDCS, MCS, or PCS scores did not vary among patients having high serum phosphorus (≥4.5 mg/dL), low albumin (<3.5 g/dL), and elevated parathyroid hormone (≥150 pg/ml). On multiple linear regression analysis, the predictors of KDCS were unemployment (P < 0.001) and illiteracy (P = 0.03). Unemployment (P < 0.001) and age (P < 0.001) were predictors of PCS whereas literacy level (P < 0.001) was predictive of MCS.

Early eosinophilic antibody-mediated rejection in a renal allograft recipient

Although the predominant component of acute allograft rejection is the T-cells, the milieu is not devoid of other inflammatory cells including plasma cells, eosinophils, and histiocytes. Apart from the CD8 T cell and CD4 T cell-FasL cytotoxicity, experimental models had proven a pivotal role of Th-2 cells in acute rejection, and these have been associated with marked tissue eosinophilia. Herein, we present a unique case of severe eosinophilic acute antibody-mediated rejection in a 22 years old deceased donor renal allograft recipient, within 4 days of transplantation without peripheral eosinophilia. The pathology was successfully dealt with the prevailing modalities of therapy, including steroids, plasmapheresis, intravenous immunoglobulin, and bortezomib. Concurrently, we have briefly reviewed the literature about the role of eosinophils in graft rejection and its prognostication.

Acute Interstitial Nephritis Following Snake Envenomation: A Single-Center Experience.

OBJECTIVES To identify the clinical and histopathological characteristics of patients who develop acute interstitial nephritis (AIN) following snake envenomation. METHODS A retrospective analysis of patients diagnosed with snake envenomation-induced AIN from October 2013 to November 2014. RESULTS After snake envenomation, 88 patients developed acute kidney injury (AKI). Biopsies were performed on 7 patients due to nonrecovery of kidney function. Among these, 5 patients had AIN. Thus, AIN accounted for 5.7% of snakebite-related acute kidney injury. All patients had severe envenomation at presentation and had prolonged renal failure. Kidney biopsy found a mixed infiltrate composed of predominantly lymphocytes, with variable proportions of other cells including eosinophils neutrophils and plasma cells. The response rate to corticosteroids was 80%. CONCLUSIONS AIN after snake bite is not uncommon. AIN needs to be considered in patients with persistent renal failure after snake envenomation. Identifying this complication is of utmost importance because of the potentially reversible nature.

Clinical spectrum and outcomes of crescentic glomerulonephritis: A single center experience

There is limited data on the etiology, clinical and histopathological spectrum and outcomes of crescentic glomerulonephritis (CrGN) in adult Indian population. This prospective study was done to evaluate the etiology, clinicohistological patterns and predictors of outcome of CrGN in South Indian population. All the patients received standard protocol based immunosuppression in addition to supportive care. Immune-complex glomerulonephritis (ICGN) was the most common etiology (n = 31; 77.5%) followed by pauci-immune glomerulonephritis (PauciGN; n = 8; 20%) and anti-glomerular basement membrane disease (n = 1; 2.5%). The most common etiology of ICGN was IgA nephropathy (n = 11; 27.5%) followed by lupus nephritis (n = 7; 17.5%) and post-infectious glomerulonephritis (PIGN) (n = 7; 17.5%). The patients with PauciGN were significantly older compared to those with ICGN (44.5 ± 15 years vs. 31.8 ± 11 years; P = 0.01). The patients with PauciGN presented with significantly higher serum creatinine (9.7 ± 4.4 vs. 6.6 ± 3.3 mg/dl; P = 0.03). The histopathologic parameters of ICGN and PauciGN were comparable except for a higher proportion of sclerosed glomeruli in ICGN. At the end of 3 months follow-up, only two patients went into complete remission (5.4%). Majority of the patients had end-stage renal failure (48.6%) and were dialysis dependent and seven patients (18.9%) expired. There was no signifi difference in the renal survival (10.9 ± 1.9 vs. 9.6 ± 3.3 months) or patient survival (17.5 ± 2.1 vs. 17.3 ± 4.3 months). The parameters associated with adverse outcomes at 3 months were hypertension (odds ratio [OR]: 0.58; confidence interval [CI]: 0.36–0.94), need for renal replacement therapy (OR: 0.19; CI: 0.04–0.9), serum creatinine at admission (P = 0.019), estimated glomerular filtration rate (P = 0.022) and percentage of fibrocellular crescents (P = 0.022).