Cátia Cunha

Background: Hydroxychloroquine (HCQ) is a recommended drug in systemic lupus erythematosus (SLE). It has a long terminal half-life, making it an attractive target for therapeutic drug monitoring. The aim of this study was to establish a relationship between blood HCQ concentration and lupus nephritis activity. Methods: We conducted a retrospective observational study with data collected from clinical and laboratory records. Inclusion criteria were patients followed in the lupus clinic with biopsy-proven International Society of Nephrology/Renal Pathology Society Classes III, IV or V lupus nephritis on HCQ for at least 3 months (200-400 mg daily) and with HCQ levels measured during treatment. Exclusion criteria were patients on renal replacement therapy at baseline or patients lost to follow-up. Results: In 171 patients, the HCQ level was measured in 1282 samples. The mean HCQ blood level was 0.75±0.54mg/L and it was bimodally distributed. An HCQ level <0.20 mg/L [232 samples (18.1%)] appeared to define a distinct group of abnormally low HCQ levels. For patients in complete or partial remission at baseline compared with those remaining in remission, patients with renal flare during follow-up had a significantly lower average HCQ level (0.59 versus 0.81 mg/L; P= 0.005). Our data suggest an HCQ target level to reduce the likelihood of renal flares >0.6 mg/L (600 ng/mL) in those patients with lupus nephritis. Conclusion: HCQ level monitoring may offer a new approach to identify non-adherent patients and support them appropriately. We propose an HCQ minimum target level of at least 0.6 mg/L to reduce the renal flare rate, but this will require a prospective study for validation.

&lt;b&gt;&lt;i&gt;Introduction:&lt;/i&gt;&lt;/b&gt; Pancreatic surgery still carries a high morbidity and mortality even in specialized centers. The aim of this study was to evaluate the influence of patients’ body composition on postoperative complications and survival after pancreatic surgery. &lt;b&gt;&lt;i&gt;Methods:&lt;/i&gt;&lt;/b&gt; This was a retrospective study on patients undergoing pancreatic surgery between March 2012 and December 2017. Demographics, clinical data, and postoperative complications classified according to Clavien-Dindo were recorded. Body composition was assessed using routine diagnostic or staging computed tomography (CT). Multiple Cox proportional hazards models were adjusted. &lt;b&gt;&lt;i&gt;Results:&lt;/i&gt;&lt;/b&gt; Ninety patients were included, 55% were male, and the mean age was 68 ± 10.9 years. Of these 90, 92% had a total pancreatectomy or pancreaticoduodenectomy, 7% a distal pancreatectomy, and 1% a pancreaticoduodenectomy with multi-visceral resection; 84% had malignant disease. The incidence of major complications was 27.8% and the 90-day mortality was 8.8%. The ratio of visceral fat area/skeletal muscle area (VFA:SMA) was associated with an increased risk of complications (OR 2.24, 95% CI 1.14–4.87, &lt;i&gt;p&lt;/i&gt; = 0.03) and 90-day survival (HR 2.13, 95% CI 1.13–4.01, &lt;i&gt;p&lt;/i&gt; = 0.019). On simple analysis, shorter overall survival (OS) was observed in patients aged ≥70 years (&lt;i&gt;p&lt;/i&gt; = 0.0009), with postoperative complications ≥IIIb (&lt;i&gt;p&lt;/i&gt; = 0.01), an increased VFA:SMA (&lt;i&gt;p&lt;/i&gt; = 0.007), and decreased muscle radiation attenuation (&lt;i&gt;p&lt;/i&gt; = 1.6 × 10&lt;sup&gt;–5&lt;/sup&gt;). In an OS model adjusted for age, disease malignancy, postoperative complications, and body composition parameters, muscle radiation attenuation remained significantly associated with survival (HR 0.94, 95% CI 0.90–0.98, &lt;i&gt;p&lt;/i&gt; = 0.0016). A model which included only body composition variables had a discrimination ability (&lt;i&gt;C&lt;/i&gt;-statistic 0.76) superior to a model which comprised conventional clinical variables (&lt;i&gt;C&lt;/i&gt;-statistic 0.68). &lt;b&gt;&lt;i&gt;Conclusion:&lt;/i&gt;&lt;/b&gt; Body composition is a major determinant of postoperative complications and survival in pancreatic surgery patients.

BACKGROUND AND AIM: Preoperative biliary drainage (PBD) in patients with pancreatic cancer remains debatable. The aim of this study was to analyse the indications for PBD in patients performing pancreaticoduodenectomy (PD) and to evaluate the impact of this procedure on postoperative outcome. METHODS: Observational retrospective cohort study of patients undergoing PD for pancreatic cancer. Clinical data and postoperative outcome, namely complications and 90-day mortality, were prospectively collected and compared between patients performing PBD or direct surgery (DS). RESULTS: Eighty-two patients were included: 40 underwent PBD and 42 performed DS. Major complications (27.5% vs 33.3%, P=0.156) and 90-day mortality (10% vs 16.7%, P=0.376) were similar between the two groups. There was a trend for higher mean total bilirubin in patients with PBD (P=0.073). The indication for PBD was suspicion of cholangitis/choledocholithiasis or need to perform neoadjuvant chemotherapy in 24 (60%) patients. In the remaining, elevated bilirubin was probably the only reason to perform PBD. Length of hospital stay was longer in PBD group (P=0.003). On multiple logistic regression, 90-day mortality was not related with preoperative bilirubin levels, biliary drainage or its indication, but solely with age (OR 1.15, 95%CI 1.05-1.31, P=0.008). CONCLUSIONS: PBD is often performed in patients undergoing PD without a formal indication, mainly due to high bilirubin levels. No increased morbidity/mortality was observed but length of hospital stay was prolonged in patients performing PBD.

AA amyloidosis is a rare complication of chronic inflammatory disorders and has been associated with rheumatoid arthritis and ankylosing spondylitis. We present a case of AA amyloidosis secondary to Sjogren's syndrome (SS). A 79-year-old woman presented with rapidly progressive renal failure and complaints of asthenia, anorexia and generalized oedema. She had severe renal failure (creatinine 6.0 mg/dl), with microscopic haematuria, nephrotic proteinuria and low serum albumin levels, and an increased erythrocyte sedimentation rate. Serum protein electrophoresis revealed a peak in the gamma globulin zone. The patient was started on haemodialysis and corticosteroids. Clinical results showed the patient met the diagnostic criteria for primary SS, and neoplastic haematological disease was excluded. Renal biopsy revealed a diagnosis of AA amyloidosis. Renal AA amyloidosis is a rare condition in patients with primary SS. However, in patients with proteinuria and/or renal failure, it should be included in the differential diagnosis and a renal biopsy should be performed. LEARNING POINTS: Sjogren's syndrome should be regarded as a predisposing condition for the development of renal AA amyloidosis.Sjogren's syndrome and renal AA amyloidosis have been diagnosed simultaneously in some patients.A renal biopsy should be performed in patients with Sjogren's syndrome and proteinuria and/or renal failure.

Neste estudo introduzimos uma extensão do Ficheiro de Imagens Multicategoriais (FIM, Prada &amp; Garcia-Marques, 2006) em 280 imagens. Apresentamos, assim, dados normativos destas novas imagens em termos de valência, aferida através da média das respostas em duas dimensões contínuas referenciadas em 9 pontos, “Mau/Bom” e “Gosto Pouco/Gosto Muito”. Estas foram também avaliadas relativamente à sua familiaridade subjectiva, através da resposta a uma escala definida por “Pouco Familiar/Muito Familiar”. Cada imagem foi avaliada por uma amostra mínima de 20 estudantes, sendo sistematizadas as respectivas avaliações e tempos de resposta a cada escala (i.e., médias, desvios-padrão e intervalos de confiança a 95%).