Jorge Pratas

Risk factors for acute rejection in 806 cyclosporine-treated renal transplants: a multivariate analysis.

IT is known that acute rejection (AR) is responsible for serious graft injury, causing parenchymal deterioration that can end in early graft failure or in long-term development of chronic dysfunction. Both mechanisms contribute negatively to graft survival. The aim of this study was to investigate the incidence, the causes, and the effects of AR on the outcome of transplants under cyclosporine (CyA)based therapy.

Severe acute kidney injury and multiple myeloma: Evaluation of kidney and patient prognostic factors

BACKGROUND Patients with multiple myeloma (MM) manifesting acute kidney injury (AKI) and who later recover renal function and independence from renal replacement therapy (RRT) are considered to have a better outcome. The aim of this work was to study the factors associated with renal function recovery (independence of hemodialysis) and longer survival in these patients. METHODS A retrospective single center study including patients with a diagnosis of MM and severe AKI, defined as stage 3 of the Kidney Disease: Improving Global Outcomes (KDIGO) criteria: 3.0 times baseline increase in serum creatinine (sCr) or increase in sCr to ≥4.0mg/dL or initiation of RRT, was conducted. Data was registry-based and collected between January 2000 and December 2011. We examined demographic and laboratorial data, presenting clinical features, precipitating factors, need for RRT and chemotherapy. Death was considered the primary endpoint. RESULTS Lower serum β2-microglobulin was the only independent factor associated with recovery of renal function and independence of RRT (OR 0.95, 95% CI: 0.91-0.99, P=0.02). The median survival after AKI was 10.7±12.1months. The factors associated with longer survival were independence of RRT (HR 2.21; 95% CI: 1.08-4.49; P=0.02), lower CRP (HR 1.07; 95% CI: 1.03-1.12; P=0.001) and younger age (HR 1.03; 95% CI: 1.01-1.06; P=0.005). CONCLUSIONS Our study suggests that MM patients with lower serum β2-microglobulin have a higher likelihood of recovering renal function after severe AKI. Independence of RRT, lower CRP and younger age are associated with longer survival.

Hypouricaemia and hyperuricosuria in familial renal glucosuria

Familial renal glucosuria is a rare co-dominantly inherited benign phenotype characterized by the presence of glucose in the urine. It is caused by mutations in the SLC5A2 gene that encodes SGLT2, the Na+-glucose cotransporter responsible for the reabsorption of the bulk of glucose in the proximal tubule. We report a case of FRG displaying both severe glucosuria and renal hypouricaemia. We hypothesize that glucosuria can disrupt urate reabsorption in the proximal tubule, directly causing hyperuricosuria.

Membranoproliferative glomerulonephritis in a puerperal with Sjögren's syndrome: Rare finding or something else?

We report on a case of a 35-year-old Caucasian woman with primary Sjögren’s Syndrome (pSS) diagnosed by salivary gland biopsy and IgM monoclonal gammopathy of undetermined significance (MGUS), treated with hydroxychloroquine 200 mg daily since she was 22 year-old. After 24 weeks into her first pregnancy, she developed marked asthenia with muscle pain and arthralgia, hypertension, oedema and palpable purpura of the lower limbs. Laboratorial testing revealed an increase in serum creatinine (sCr) (0.75 → 1.18 mg/dL), anaemia (haemoglobin (Hb) = 8.6 g/dL), active urinary sediment and nephrotic-range proteinuria (3.8 g/24 h). She was diagnosed with preeclampsia with foetal distress and underwent a caesarean at 29 weeks of pregnancy. One week post-partum the patient was discharged home with sCr = 0.84 mg/dL, Hb = 12 g/dL, 24-hour proteinuria = 1.4 g and normotensive under nifedipine 30 mg daily. Three months post-partum, she was admitted to the emergency room presenting acute pulmonary oedema requiring invasive ventilatory support. Pulmonary auscultation showed fine crackles over both lung bases, she had moderate peripheral oedema and after urinary catheterization, oligoanuria was confirmed. Laboratory tests revealed sCr = 4.39 mg/dL, K+ = 6.6 mmol/L, brain natriuretic peptide = 5013 pg/mL, Hb = 8.1 g/dL, platelet count = 248x109/L and negative blood cultures. Urinalysis showed 3+ proteinuria on a dipstick test. On echocardiogram there were no signs of endocarditis and she had a ventricular ejection fraction of 50%, renal ultrasound revealed normal kidneys and chest X-ray showed large bilateral pleural effusion. Continuous haemodiafiltration was initiated and the patient was admitted to the intensive care unit, until she gained ventilatory autonomy and suspended haemodiafiltration, being transferred to the Nephrology Department three days later for continued care. A kidney biopsy was performed revealing type I membranoproliferative glomerulonephritis with IgM (++), IgG (+) and C3 (++) deposits, glomerular capillary endotheliosis, focal and segmental thrombotic microangiopathy, tubulointerstitial nephritis and injuries of focal and discrete vasculitis. (Fig. 1). Serologies for human immunodeficiency virus, hepatitis B virus and hepatitis C virus (HCV) were negative, as well as HCV RNA testing. Complement study showed low C4 (C4<0,01) and normal C3 (C3=1,36) and high rheumatoid factor (195 IU/mL; N = ). Cryoglobulins were positive with polyclonal IgG and monoclonal IgM and kappa light chains. The patient was treated with 1 g methylprednisolone pulses on three consecutive days and subsequent oral prednisolone at a dosage of 1 mg/kg/day. One year has passed and the patient has been weaned off steroid therapy maintaining clinical stability. Analytically, the values of sCr have stabilized at 1.0 mg/dL, with proteinuria of 150 mg/24 h (Table 1). The histological findings of our patients’ kidney biopsy revealed distinct injury patterns that could be inserted in various clinical pictures: glomerular capillary endotheliosis and focal and segmental thrombotic microangiopathy were expected in the context of previous preeclampsia1 while tubulointerstitial nephritis with injuries of vasculitis were compatible with pSS.2 Regarding the immune complex mediated MPGN, which might have been the cause of acute kidney injury, the differential diagnosis of its underlying cause was challenging and included autoimmune diseases, chronic infection and monoclonal gammopathy. MPGN associated with autoimmune diseases is rarely seen in patients with SS and we also excluded chronic infection. Considering monoclonal gammopathies, although the immunofluorescence microscopy on renal biopsy was not typical, our patient was diagnosed with IgM MGUS.3 A growing number of pathologic renal conditions are being attributed to a clonal plasma cell disorder that is less myeloma-like and more MGUS-like in terms of its bulk and proliferative rate and the term monoclonal gammopathy of