Membranoproliferative glomerulonephritis and interstitial nephritis in the setting of Epstein-Barr virus-related hemophagocytic syndrome
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BACKGROUND: Hemophagocytic syndrome (HPS) is a rare, aggressive disorder characterized by dysregulation of lymphocyte and macrophage activity, which culminates in tissue infiltration with hemophagocytosis and ultimately organ failure. Renal involvement frequently ensues and usually results in acute tubular necrosis with associated interstitial inflammation. Less frequently, glomerulopathy can also be found. CASE: We report a case of a 24-year-old Caucasian woman with previous asymptomatic hematuria, mild proteinuria, and normal renal function who presented to us with fever. Laboratory findings revealed pancytopenia, elevated lactate dehydrogenase, and ferritin as well as liver and kidney failure. Renal biopsy showed a tubulointerstitial nephritis superimposed in a membranoproliferative glomerulonephritis with crescents. Extensive etiologic investigation was negative except for Epstein-Barr virus (EBV) viral load. EBV-DNA was then identified by in situ hybridization in the renal biopsy. HPS could be diagnosed with the presence of six criteria: fever, splenomegaly, bicytopenia, high ferritin, hypertriglyceridemia, and high levels of soluble CD25. Steroid therapy was initiated with resolution of HPS as well as complete recovery of renal and liver function. CONCLUSION: In this case, we believe that EBV triggered both HPS and tubulointerstitial nephritis. Steroid therapy successfully treated the inflammatory syndrome, allowing renal function recovery without compromising EBV infection resolution.
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Multiple brown tumors - a rare presentation in the modern era
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Chronic kidney disease (CKD) commonly evolves with disturbances in mineral and bone metabolism, currently defined as CKD-MBD. Management strategies have progressed over the years, but our knowledge regarding evaluation and treatment is still sparse. Herein, we describe a rare case of a hemodialysis patient with apparently fairly controlled hyperparathyroidism (HPTH), who developed multiple symptomatic brown tumors involving the scull, mandible, vertebrae, pelvis, and metacarpus. Parathyroidectomy allowed complete resolution of the bone lesions preventing disastrous consequences.
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Diabetes, deafness and renal disease.

Deafness, kidney disease and diabetes are not a usual association, neither is a family history of these diseases. We present the case of a 47-year-old woman with non-nephrotic proteinuria, no haematuria, normal renal function, sensorineural hearing loss, recently diagnosed diabetes and maculopathy. There was a maternal family history of deafness, diabetes and renal disease. Renal biopsy revealed focal and segmental glomerulosclerosis (FSGS), leading to the pursuit of an m.3243A > G mitochondrial mutation and diagnosis of maternally inherited diabetes and deafness. The association of FSGS with mitochondrial diseases is not well known among nephrologists. Its timely diagnosis is important to avoid exposure to ineffective and unnecessary immunosuppression.

Pulmonary-renal syndrome as a clinical expression of multiple myeloma / Síndrome pulmón-riñón como una expresión clínica de mieloma múltiple

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Recurrent ischemic stroke in an adult with cystinosis: a clinical-pathological case.

A 32-year-old woman with infantile nephropathic cystinosis presented with cystinosis and recurrent ischemic stroke. The neuropathological description demonstrates that recurrent stroke was caused by intracranial stenosis and showed evidence of cystinosis brain involvement. There are few reports of cerebrovascular disease in patients with longstanding nephropathic cystinosis. This case reinforces that cerebrovascular disease can be a cause of neurological impairment and disability in patients with longstanding nephropathic cystinosis, with implications on primary stroke prevention strategies in these patients.

Evaluation of an instrument for screening patients at risk for chronic kidney disease: testing SCORED (Screening for Occult Renal Disease) in a Portuguese population.

Prevalence of chronic kidney disease (CKD) is increasing and CKD has a long asymptomatic phase suitable for screening. SCORED (Screening for Occult Renal Disease) is a prescreening test which has compared favorably with KEEP. We report the results of SCORED testing in subjects attending a World Kidney Day event. After SCORED, subjects were tested for creatinine, urinary albumin and creatinine, and renal ultrasound. Eighty-eight subjects participated (32 men; mean age 59.7 ± 14.8 years; 58% hypertensive and 15.9% diabetics) of which 60 had a high score for kidney disease. Thirty-eight of 47 (80.8%) subjects that were further evaluated had a high-risk score. All subjects with CKD had a high score (100% sensitivity). SCORED showed low specificity (24.3%), but a high negative predictive value (100%). Including albuminuria in the definition of CKD increased the positive predictive value to 43.6%. In conclusion, SCORED is good for prescreening subjects for CKD in a European population as it captures all patients with CKD. Moreover, in subjects with low risk, the probability of CKD is low. SCORED is useful in alerting the general population and the medical community about the risk factors of CKD.

Acute kidney injury in hospitalized HIV-infected patients: a cohort analysis.

BACKGROUND: Acute kidney injury (AKI) in hospitalized human immunodeficiency virus (HIV)-infected patients in the highly active antiretroviral therapy (HAART) era has not been extensively addressed. The aim of the present study was to analyze the incidence, etiology, risk factors and the impact of AKI on in-hospital mortality in this population. METHODS: A total of 489 HIV-infected patients hospitalized in the Department of Infectious Diseases of the Hospital de Santa Maria (Lisbon, Portugal) between January 2005 and December 2007 were retrospectively studied. AKI was defined by 'Risk Injury Failure Loss of kidney function End-stage kidney disease'(RIFLE) criteria based on serum creatinine. Comparisons between patients with and without AKI were performed using the Student's t-test or the χ2 test. Logistic regression method was used to determine predictors of AKI and in-hospital mortality. A two-tailed P-value <0.05 was considered significant. RESULTS: Eighty-eight patients (18%) had AKI within the hospitalization period. The most common etiologies of AKI were sepsis (59%), nephrotoxic drug administration (37.5%), volume depletion (21.6%) and radiocontrast use (20.5%). Preexisting hypertension [adjusted odds ratio (OR) 2.4, 95% confidence interval (CI) 1.04-5.6, P = 0.04], acquired immunodeficiency syndrome (adjusted OR 2.7, 95% CI 1.2-6, P = 0.02), sepsis (adjusted OR 23, 95% CI 11-45.3, P < 0.001) and nephrotoxic drug administration (adjusted OR 2.8, 95% CI 1.4-5.8, P = 0.004) were risk factors of AKI. Patients with AKI had higher in-hospital mortality than patients without AKI (27.3 versus 8%, P < 0.001). In multivariate analysis, AKI was a risk factor of in-hospital mortality (adjusted OR 2.7, 95% CI 1.3-5.6, P = 0.008). CONCLUSION: AKI occurred in 18% of hospitalized HIV-infected patients and it was independently associated with increased in-hospital mortality.

Long-term risk of mortality after acute kidney injury in patients with sepsis: a contemporary analysis.

BACKGROUND: Acute kidney injury (AKI) is associated with increased short-term mortality of septic patients; however, the exact influence of AKI on long-term mortality in such patients has not yet been determined. METHODS: We retrospectively evaluated the impact of AKI, defined by the "Risk, Injury, Failure, Loss of kidney function, End-stage kidney disease" (RIFLE) classification based on creatinine criteria, on 2-year mortality in a cohort of 234 hospital surviving septic patients who had been hospitalized at the Infectious Disease Intensive Care Unit of our Hospital. RESULTS: Mean-follow-up was 21 +/- 6.4 months. During this period, 32 patients (13.7%) died. At 6 months, 1 and 2 years of follow-up, the cumulative probability of death of patients with previous AKI was 8.3, 16.9 and 34.2%, respectively, as compared with 2.2, 6 and 8.9% in patients without previous AKI (log-rank, P < 0.0001). In the univariate analysis, age (hazard ratio 1.4, 95% CI 1.2-1.7, P < 0.0001), as well as pre-existing cardiovascular disease (hazard ratio 3.6, 95% CI 1.4-9.4, P = 0.009), illness severity as evaluated by nonrenal APACHE II (hazard ratio 1.3, 95% CI 1.1-1.6, P = 0.002), and previous AKI (hazard ratio 4.2, 95% CI 2.1-8.5, P < 0.0001) were associated with increased 2-year mortality, while gender, race, pre-existing hypertension, cirrhosis, HIV infection, neoplasm, and baseline glomerular filtration rate did not. In the multivariate analysis, however, only previous AKI (hazard ratio 3.2, 95% CI 1.6-6.5, P = 0.001) and age (hazard ratio 1.4, 95% CI 1.2-1.6, P < 0.0001) emerged as independent predictors of 2-year mortality. CONCLUSIONS: Acute kidney injury had a negative impact on long-term mortality of patients with sepsis.