Browsing by Author "Martins, A"
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- Abordagem clínica da cirrose hepática: protocolos de atuaçãoPublication . Reis, J; Alves, N; Martins, A; Horta, D; Alberto, S; Santos, L; Carvalho, R; Rodrigues, C; Oliveira, A; Costa, M; Lourenço, L; Branco, J; Cardoso, M; Anapaz, V; Alexandrino, G; Figueiredo, L; Rafael, M
- Acute Hepatitis in the DRESS SyndromePublication . Oliveira, AM; Carvalho, R; Martins, A; Reis, JDrug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe, idiosyncratic reaction characterized by diffuse maculopapular rash, facial edema, lym- phadenopathy, fever, eosinophilia and/or other leukocyte abnormalities, and involvement of internal organs as liver, kidney, heart and lung. Diagnosing this entity is specifically complicated due to the multiplicity of organs involved. DRESS syndrome must be recognized promptly and the causative drug withdrawn in order to improve patient outcomes. Indeed, it is a potentially life-threatening condition, with a reported mortality between 5 and 20%. We describe a case of a 22-year old woman admitted to our hospital with acute diffuse, pruritic rash associated with crampy abdominal pain, vomiting, diarrhea and fever three weeks after starting sulfasalazine therapy. Initially, laboratory parameters revealed normal white blood cell count and normal liver enzymes, but during hospitalization, eosinophilia developed and liver enzymes, including transaminases and cholestatic parameters, dramatically increased. The diagnostic of DRESS syn- drome was made and sulfasalazine was withdrawn and as there were signs of disease severity, systemic corticotherapy was initiated, with gradually improvement of the rash and symptoms resolution. The patient was discharged home after thirty days of hospitalization.
- Brain mapping of epileptic activity in a case of idiopathic occipital lobe epilepsy (Panayiotopoulos syndrome)Publication . Leal, A; Nunes, S; Martins, A; Secca, M; Jordão, CThe Panayiotopoulos type of occipital lobe epilepsy has generated great interest, but the particular brain areas involved in the peculiar seizure manifestations have not been established. We studied a patient with the syndrome, using high-resolution EEG and simultaneous EEG and functional magnetic resonance imaging (fMRI). Resolution of the scalp EEG was improved using a realistic spline Laplacian algorithm, and produced a complex distribution of current sinks and sources over the occipital lobe. The spike-related blood oxygen level dependent (BOLD) effect was multifocal, with clusters in lateral and inferior occipital lobe and lateral and anterior temporal lobe. We also performed regional dipole seeding in BOLD clusters to determine their relative contribution to generation of scalp spikes. The integrated model of the neurophysiologic and vascular data strongly suggests that the epileptic activity originates in the lateral occipital area, spreading to the occipital pole and lateral temporal lobe.
- Can red cell distribution width be used as a marker of Crohn's disease activity?Publication . Oliveira, AM; Cardoso, F; Rodrigues, C; Santos, L; Martins, A; Deus, JR; Reis, JIntroduction: Recently, it has been suggested an association between red cell distribution width (RDW) and Crohn’s disease activity index (CDAI), but its use is not yet performed in daily clinical practice. Objectives: To determine whether RDW can be used as a marker of Crohn’s disease (CD) activity. Methods: This was a cross-sectional study including patients with CD, observed consecutively in an outpatient setting between January 1st and September 30th 2013. Blood cell indices, erythrocyte sedimentation rate (ESR), and C-reactive protein were measured. CD activity was determined by CDAI (active disease if CDAI ≥ 150). Associations were analyzed using logistic regression (SPSS version 20). Results: 119 patients (56% female) were included in the study with a mean age of 47 years (SD 15.2). Twenty patients (17%) had active disease. The median RDW was 14.0 (13---15). There was an association between RDW and disease activity (p = 0.044). After adjustment for age and gender, this association remained consistent (OR 1.20, 95% CI 1.03---1.39, p = 0.016). It was also found that the association between RDW and disease activity was independent of hemoglobin and ESR (OR 1.36, 95% CI 1.08---1.72, p = 0.01) and of biologic therapy (OR 1.19, 95% CI 1.03---1.37, p = 0.017). A RDW cutoff of 16% had a specificity and negative predictive value for CDAI ≥ 150 of 88% and 86%, respectively. Conclusion: In this study, RDW proved to be an independent and relatively specific marker of CD activity. These results may contribute to the implementation of this simple parameter, in clinical practice, aiming to help therapeutic decisions.
- Celiac disease and upper tract Crohn's disease: a rare associationPublication . Alberto, S; Martins, A; Deus, JRThe authors present the case of a 47 years old female, with no past or familial history, hospitalized due to vomiting and significant weight loss over the last 9 months, without abdominal pain, blood loss, constipation or diarrhea. Blood tests results revealed iron deficiency anaemia, seropositivity for ASCA, anti-transglutaminase antibody and HLA-DQ2. Upper GI endoscopy showed several ulcers in duodenal mucosa and an ulcerated stricture in D3; biopsies were taken and histopathology revealed transmural inflammatory chronic infiltrate, crypt loss and some areas of villous atrophy and intraepithelial lymphocytosis. The small bowel follow through confirmed 2 main strictures in duodenum and proximal jejunum. She was started on prednisolone, azathioprine and a gluten-free diet with an initial good response although relapse was detected one month later. In this last admission, parenteral nutrition was necessary due to her deteriorated nutritional state and she also started infliximab, with subsequent significant clinical and endoscopic improvement.
- Chronic abdominal pain with a difficult diagnosisPublication . Oliveira, AM; Rodrigues, C; Santos, L; Martins, A; Deus, JR
- Clinical and microbiological characteristics associated with mortality in spontaneous bacterial peritonitis: a multicenter cohort study.Publication . Oliveira, AM; Branco, JC; Barosa, R; Rodrigues, JA; Ramos, L; Martins, A; Karvellas, C; Cardoso, FOBJECTIVES: Spontaneous bacterial peritonitis (SBP) is a prevalent and high mortality complication of cirrhosis. We aimed to describe these patients' clinical and microbiological characteristics and evaluate their impact on outcomes. METHODS: This was a retrospective cohort study including 139 consecutive patients with positive culture SBP from three Portuguese centers diagnosed between 2009 and 2014. Multivariate logistic regression was used to study associations with 30-day mortality. RESULTS: The mean age of the patients was 62 years and 81% of patients were men. The mean model for end-stage liver disease score was 19. Hepatic encephalopathy, hepatorenal syndrome, and variceal bleeding developed in 47, 30, and 21% of patients, respectively. Gram-positive bacteria were isolated in the ascitic fluid of 42% of patients. Resistance to quinolones and multiresistance were found in 33 and 17% of patients, respectively. C-reactive protein level (adjusted odds ratio, 1.16 per 1 mg/l increment) and development of hepatorenal syndrome (adjusted odds ratio, 2.86) were associated independently with 30-day mortality (model's area under the curve, 0.78). CONCLUSION: In this cohort, SBP portended high early mortality. Gram-positive bacteria, bacteria resistant to quinolones, and multiresistant bacteria were identified in considerable proportions of patients. In the setting of the high early mortality and changing microbiological profile, SBP management strategies need to be improved.
- A Complex Case of Cholestasis in a Patient with ABCB4 and ABCB11 MutationsPublication . Cardoso, M; Branco, J; Anapaz, V; Rodrigues, C; Carvalho, R; Horta, D; Martins, A; Reis, JThe low-phospholipid-associated cholelithiasis (LPAC) syndrome is a form of symptomatic cholelithiasis occurring in young adults, characterized by recurrence of symptoms after cholecystectomy and presence of hepatolithiasis. The case refers to a healthy 39-year-old Caucasian male who presented with abdominal pain and jaundice. His blood tests showed conjugated hyperbilirubinemia and elevated liver enzymes (total bilirubin 6.65 mg/dL, γ-glutamyltransferase 699 IU/L) and abdominal computed tomography revealed dilation of common bile duct and left intrahepatic ducts. Magnetic resonance cholangiopancreatography identified choledocholithiasis, retrieved by endoscopic retrograde cholangiopancreatography, after which there was a worsening of jaundice (total bilirubin 23 mg/dL), which persisted for several weeks, possibly due to ciprofloxacin toxicity. After an extensive workup including liver biopsy, the identification of two foci of hepatolithiasis on reevaluation abdominal ultrasound raised the hypothesis of LPAC syndrome and the patient was started on ursodeoxycholic acid, with remarkable improvement. Genetic testing identified the mutation c.1954A>G (p.Arg652Gly) in ABCB4 gene (homozygous) and c.1331T>C (p.Val444Ala) in ABCB11 gene (heterozygous). In conclusion, we describe the unique case of an adult male with choledocholithiasis, hepatolithiasis, and persistent conjugated hyperbilirubinemia after retrieval of stones, fulfilling the criteria for LPAC syndrome and with possible superimposed drug-induced liver injury, in whom ABCB4 and ABCB11 mutations were found, both of which had not been previously described in association with LPAC.
- A complex case of low-phospholipid-associated cholelithiasis syndrome.Publication . Figueiredo, LM; Lourenço, L; Horta, D; Martins, AThe low-phospholipid-associated cholelithiasis (LPAC) syndrome is a form of symptomatic and recurring cholelithiasis occurring in young adults, associated with mutations in the ABCB4 gene. It is a clinical syndrome characterized by at least two of the following criteria: age at onset of biliary symptoms below 40 years, intrahepatic echogenic foci or microlithiasis and recurrence of biliary symptoms after cholecystectomy. In the rare cases progressing to end-stage liver disease, a liver transplant may be indicated. We report a case of a 40-year-old female patient with clinical criteria for LPAC syndrome and with ABCB4 gene mutation. She had a complex history of choledocholithiasis recurrence despite treatment with ursodeoxycholic acid and multiple therapeutic endoscopic retrograde cholangiopancreatography, and she developed portal vein thrombosis.
- Endoscopic therapy in disconnected duct syndrome: re-connecting the duct.Publication . Figueiredo, LM; Lourenço, L; Horta, D; Martins, AWe report the case of a 61-year-old male patient with a history of acute necrotizing biliary pancreatitis and a disconnected duct syndrome. He underwent transgastric drainage using a luminal apposing metal stent and transgastric necrosectomy with complete resolution of the necrosis. A pancreatic fistula was identified during pancreatography and a pancreatic plastic stent was placed in order to manage the disconnected duct syndrome. The tip of the pancreatic stent could be seen inside the pancreatic collection, which is an unusual finding. There was a resolution of the collection and the pancreatic stent was removed.