Browsing by Author "Cunha, L"
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- Atypical renal presentation of antiphospholipid syndromePublication . Gaspar, A; Manso, RT; Pereira, F; Cunha, L; Inchaustegui, L; Serra, A; Rodrigues, B; Correia, PAntiphospholipid syndrome (APS) is a systemic autoimmune disease which can occur as a primary disease or in association with other autoimmune diseases, the most frequent being Systemic Lupus Erythematosus (SLE). Although renal manifestations of SLE are well known, antiphospholipid syndrome renal manifestations such as antiphospholipid syndrome nephropathy and glomerulopathies have yet to be better characterized. The authors present the case of a 39 -year -old Caucasian woman with antiphospholipid syndrome diagnosis and a previous history of deep venous thrombosis and intermittent polyarthralgia, who was referred to a nephrology consultation for proteinuria and microscopic haematuria with preserved renal function. The renal biopsy showed a pattern of membranous glomerulopathy and thrombotic microangiopathy in association with antiphospholipid syndrome nephropathy. This case report illustrates a complex clinical and anatomopathological case of a 39 - year -old woman with a previous antiphospholipid syndrome diagnosis who presented with unspecific manifestations such as proteinuria and microscopic haematuria and preserved renal function. The histological findings alert us to the range of possible renal manifestations of APS and the need to better characterize these patients by preforming renal biopsy.
- Cellular Variant of Focal Segmental Glomerulosclerosis Treated with Plasma ExchangePublication . Cunha, L; Pereira, F; Manso, RT; Fervenza, F; Soto, KFocal segmental glomerulosclerosis (FSGS) is the most common primary glomerular disease in nephrotic patients in the United States, frequently leading to end stage renal disease (ESRD). The cellular variant is a rare form of FSGS commonly associated with poor outcome. We report a case of cellular variant FSGS with progressive kidney dysfunction successfully treated with plasma exchange (PE). A 49-year-old Caucasian female presented with two days of ankle edema and hypertension. Laboratory findings showed serum creatinine (SCr) 1.6 mg/dL, urine albumin/creatinine ratio (uACR) 2.8 g/g, haematuria 3+ and no immunological abnormalities. Kidney biopsy revealed a cellular FSGS variant with segmental endocapillary proliferation on light microscopic, negative immunofluorescence and widespread foot process effacement by electronic microscopic. Prednisolone 1 mg/Kg was started. Four days later the SCr worsened (3.6 mg/dL) and the patient became severely nephrotic with uACR of6.8g/g, quickly attaining a maximum of 24.6 g/g in a short time and albumin of 2.15g/dL. Pulsed methyl prednisolone was started. Despite a 10 course of steroids, no clinical improvement was observed. Considering the rapidly worsening renal function and severe nephrotic syndrome, PE was begun in association with mycophenolate mofetil and tacrolimus. Kidney function recovered after one week. Complete remission was achieved at 3rd week and remains in complete remission at 27 months follow-up. Prolonged remission is a challenge in primary FSGS. PE associated with combined immunosuppression was effective in the present case. The short and long-term effects of plasma exchange in primary FSGS should be evaluated in prospective studies.
- Cystatin C as a marker of acute kidney injury in the emergency departmentPublication . Soto, K; Coelho, S; Rodrigues, B; Martins, H; Frade, F; Lopes, S; Cunha, L; Papoila, A; Devarajan, PBACKGROUND AND OBJECTIVES: The diagnosis of acute kidney injury (AKI) is usually based on changes in serum creatinine, which is a poor marker of early renal dysfunction. The discriminative and predictive abilities of serum and urinary cystatin C were examined for the prediction of AKI. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: In this prospective cohort study, serum and urinary cystatin C were serially measured in a heterogeneous group of patients (n = 616) presenting to a tertiary care emergency department. The primary outcome was AKI, classified according to RIFLE and AKIN criteria. The secondary outcome was an adjudication based on clinical criteria to AKI, prerenal azotemia, chronic kidney disease (CKD), and normal kidney function. RESULTS: Patients were adjudicated to have AKI in 21.1%, prerenal azotemia in 25.8%, CKD in 2.4%, and normal kidney function in 50.7%. For the diagnosis of AKI, the discriminatory ability of urinary creatinine and cystatin C was marginal. Both serum cystatin C and serum creatinine (at presentation and 6 hours later) showed high discriminatory ability for the diagnosis of AKI. However, only serum cystatin C attained a significant early predictive power (Hosmer-Lemeshow P value > 0.05). Serum cystatin C could differentiate between AKI and prerenal azotemia, but not between AKI and CKD. CONCLUSIONS: Serum cystatin C is an early, predictive biomarker of AKI, which outperforms serum creatinine in the heterogeneous emergency department setting. However, neither biomarker discriminated between AKI and CKD. Additional biomarkers continue to be needed for improved specificity in the diagnosis of community-acquired AKI.
- Is Manchester (MTS) more than a triage system? A study of its association with mortality and admission to a large Portuguese hospital.Publication . Martins, H; Cunha, L; Freitas, PTBACKGROUND: The Manchester Triage System (MTS) is a 5-point triage scale used to triage patients presenting to the emergency department. It was introduced in the UK in 1996 and is now widespread, especially in Europe, and has been in use in our hospital since 2000 via a computerised protocol. A study was undertaken to determine whether the subgroups created by the application of MTS have different propensities for indirect triage outcomes such as death in the A&E department or being admitted to hospital. METHODS: A database of 321 539 patients triaged during a 30-month period (from January 2005 to June 2007) was used. MTS codes, death outcomes, admission and admission route were used to estimate the proportions and association between MTS codes and the remaining variables by chi(2) univariate analysis. RESULTS: There was a clear association between the priority group and short-term mortality as well as with the proportion of patients admitted to hospital. CONCLUSIONS: The MTS provides information that extends beyond its immediate usefulness as a prioritisation mechanism. It is a powerful tool for distinguishing between patients with high and low unadjusted risk of short-term death as well as those who will stay in hospital for at least 24 h and those who will return home. Its discriminatory power is not equal for medical and surgical specialities, which may be linked to the nature of its inbuilt discriminators.
- Kidney biopsy in Lupus Nephritis: still essential in clinical practicePublication . Pereira, F; Cunha, L; Manso, RT; Soto, K; Rovin, BRenal involvement in Systemic Lupus Erythematous is common and its management remains a daily challenge for clinical providers. Percutaneous kidney biopsy remains the gold standard for diagnosis of lupus nephritis. More recently, we have seen the role of the biopsy being challenged, considering the widespread use of corticosteroids and mycophenolate mofetil for all forms of lupus nephritis. We present a review of published evidence regarding first and repeat kidney biopsies for patients with lupus nephritis. Based on the available literature, we recommend a kidney biopsy to guide treatment and determine prognosis and we also suggest an algorithm for kidney rebiopsy in lupus nephritis.
- Late Cytomegalovirus Infection in Kidney Transplant Recipients after a Six-Month Prevention ProtocolPublication . Cunha, L; Laranjinha, I; Birne, R, et al.BACKGROUND: Despite a reduction in the incidence of cytomegalovirus (CMV) infections after kidney transplantation, less is known about late CMV infection in kidney transplant recipients. OBJECTIVE: To assess incidence of CMV infection in a cohort of patients under a high surveillance CMV prevention protocol and identify factors associated with late CMV infection. METHODS: Analysis of a consecutive cohort of 181 kidney allograft recipients between January 2012 and Aug 2015. CMV prevention-protocol consisted of 6-month universal prophylaxis and pre-emptive therapy for high-risk group (D+/R- or patients submitted to lymphocyte-depleting agent for induction or rejection treatment) and pre-emptive therapy for standard-risk group (D±/R+). Stopping valganciclovir was followed by CMV screening in the next two appointments. RESULTS: CMV infection was identified in 73 of 181 patients; the rate in high-risk group and standard-risk group was similar (p=0.443). However, in the latter group, the infection occurred mostly in the first 6 months. Late CMV infection occurred in 25 of 181 patients (5 of standard-risk group and 20 of high-risk group), after a median (IQR) of 253 (230.3-312.3) days after transplantation and 55 (41-89.5) days after the protocol period. Screening for CMV after valganciclovir discontinuation revealed 56% of late CMV infections. In high-risk group, D+/R- was associated with late CMV infection (HR 2.7, p=0.039) and in standard-risk group; lower age was associated with late CMV infection (HR 0.89, p=0.02). CONCLUSION: The incidence of CMV infection was similar to that reported in the literature. In high-risk patients, antigenemia surveillance during prophylaxis did not appear to reduce late CMV infections. Antigenemia screening after valganciclovir had limited results in the diagnosis of late CMV infection. D+/R- was associated to late CMV infection in high-risk group. Lower age appeared to influence late CMV infection in standard-risk group.
- Magnesium – association with inflammation and renal disease in systemic lupus erythematosusPublication . Cunha, L; Marcelino, G; Amaral, M; Alves, JDIntroduction: Recent studies suggest that magnesium deficiency may play a role in inflammation. In diabetes and cardio-vascular diseases, conditions with a component of chronic inflammation, C–reactive protein levels are higher and associated with low serum magnesium. The objective of this study is to evaluate serum magnesium levels in patients with systemic lupus erythematosus and its potential association with inflammation and renal manifestations. Methods: All patients with systemic lupus erythematosus followed in a Systemic Immune Diseases Unit, from January 2012 until January 2014, were included in this cross sectional analysis. Patients with infection, neoplasia, liver failure and chronic kidney disease (stage > 3) were excluded. Clinical information and laboratory results (serum magnesium, C-reactive protein, erythrocyte sedimentation rate, serum creatinine and spot urine test) were collected. A multivariate analysis was performed to explore possible predictive factors for hypomagnesaemia. Results: One hundred and two patients were included (94.1% female, 21-86 years). 33.4% had hypertension, 8.8% had diabetes and 20.6% had hypomagnesaemia (< 1.8mg/dL). There were no significant differences between the inflammatory parameters of patients with hypomagnesaemia or normomagnesaemia. Serum magnesium was significantly lower with increasing comorbidities (p = 0.01). Leukocyturia was significantly higher in the hypomagnesaemia group (p = 0.03) and haematuria had a negative correlation with serum magnesium (-0.23, p < 0.05). Multivariate analysis showed that patients with hypertension and diabetes had higher risk of hypomagnesaemia: OR 42.29 (95% CI, 1.43-1249.31). Leukocyturia was also individually and independently associated with hypomagnesaemia: OR 8.37 (95% CI, 1.40-49.97). Conclusion: The presence of hypomagnesaemia in our patients with systemic lupus erythematosus was high. There was no association between the levels of serum magnesium and the inflammatory parameters. Increasing comorbidities and leukocyturia were independent predictors of lower serum magnesium. Finally, the association of leukocyturia and haematuria with lower serum magnesium may suggest a relationship with a higher disease activity.
- Severe Systemic Lupus Erythematosus presentation in patient with alternative complement pathway mutationsPublication . Pereira, F; Cunha, L; Campos, P; Gaspar, A; Manso, RT; Soto, KSystemic lupus erythematosus (SLE) is an autoimmune disease which can involve almost any organ, making its difficult therapeutic approach. Immune complex deposition can often activate complement, accounting for many of SLE clinical manifestations and laboratory findings. We present a case of a patient who presented with acute pancreatitis and acute kidney injury as onset manifestations of SLE, later developing neurological manifestations, who was successfully treated with rituximab, plasma exchange and steroids as induction therapy. Persistently low C3 level led to a genetic analysis of the complement system components. We found three polymorphisms in the alternative pathway of complement regulators (complement factor H c2669 G>T, p.Ser890Ile and c3019 G>T, p.Val1007Leu and complement factor I c.482+6 G>T), two of which have been correlated with atypical haemolytic uraemic syndrome and dense deposit disease and also complement factor H -related protein (CFHR1 and CFHR3) mutations by deletion. This raises the question whether these polymorphisms and mutations played any role in our patient’s clinical course.