Browsing by Issue Date, starting with "2017-10"
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- Postinfectious Anti-Myelin Oligodendrocyte Glycoprotein Antibody Positive Optic Neuritis and MyelitisPublication . Vieira, JP; Sequeira, J; Brito, MJWe report the case of a 9-year-old girl admitted with fever, headache, and a cerebrospinal fluid lymphocytic pleocytosis. Polymerase chain reaction was positive for human herpes virus 6. She subsequently developed ataxia and bilateral loss of vision. Magnetic resonance imaging (MRI) showed bilateral optic nerve lesions with extension to optic chiasm and a short-segment myelitis. Serologic studies were positive for Borrelia burgdorferi IgM. Anti-aquaporin 4 antibody was negative and anti-myelin oligodendrocyte glycoprotein antibody (MOG) positive. After intravenous methylprednisolone, ceftriaxone, and intravenous immunoglobulin, her vision slowly recovered. The patient was discharged with only mild visual acuity loss, 1 month after admission. Brain MRI was repeated later and was normal and MOG assay became negative. In our view, this patient suffered from a postinfectious, anti-MOG-mediated, spinal cord and optic nerve demyelination.
- A Rash Decision. The Hazards of the Wrongful Use of AdrenalinePublication . Coutinho Cruz, M; Sá Carvalho, R; Modas Daniel, P; Cruz Ferreira, RAnaphylaxis is life-threatening and should be addressed urgently. Its treatment is not without side effects and an accurate diagnosis must be made to prevent potential harm by the wrongful use of medication. A 46-year-old woman with hypertension treated with angiotensin converting enzyme inhibitor (ACEI) presented to the emergency department with non-pitting oedema of the face and limbs. A hasty diagnosis of anaphylaxis was made and intravenous adrenaline administered. The patient developed a myocardial infarction caused by coronary artery spasm that required invasive intervention. The initial clinical picture was resolved when the ACEI was discontinued unmasking a case of ACEI-induced angioedema. The correct differentiation of these two apparently similar clinical entities is of utmost importance in the management of emergency department patients.
- Association of Vitiligo and Halo NevusPublication . Correia, C; Pires, E; Valejo Coelho, M; Paiva Lopes, MJ
- Simultaneous Kidney-Pancreas Transplantation With an Original "Transverse Pancreas" Technique: Initial 9 Years' Experience With 56 CasesPublication . Paulino, J; Martins, A; Vigia, E; Marcelino, P; Nobre, AM; Bicho, L; Filipe, E; Barroso, EAn innovative technique for pancreas transplantation is described. The main aspect consists of the horizontal positioning of the pancreas, which allows a better venous outflow, thus preventing thrombosis and graft loss. The program of pancreas transplantation in this national reference center for pancreatic and liver surgery was started in 2007; the initial results were considered poor, resulting in the loss of half of the grafts due to venous thrombosis. After analyzing the possible causes, this technique was proposed and successfully implemented, reducing the postoperative complications, particularly the problem of venous thrombosis. A detailed description of the new surgical technique is provided. The main clinical and demographic characteristics of the 56 patients who underwent the surgery are analyzed. The incidence of venous thrombosis was 5.3% (3 patients) and graft loss was 3.5% (2 patients). Due to the good results, this technique became the standard surgery for transplantation of the pancreas in our center. The technique proved to be safe and successful. Due to the unique pancreas graft implantation, we called it "transverse pancreas surgery."
- Direct Percutaneous Endoscopic Jejunostomy Using Single-Balloon Enteroscopy without Fluoroscopy: a Case SeriesPublication . Bernardes, C; Pinho, R; Rodrigues, A; Proença, L; Carvalho, JBACKGROUND: Direct percutaneous endoscopic jejunostomy (DPEJ) is a useful method to provide enteral nutrition to individuals when gastric feeding is not possible or contraindicated. The aim of this study was to analyze the efficacy and safety of DPEJ tube placement with the Gauderer-Ponsky technique by the pull method, using single-balloon enteroscopy (SBE) without fluoroscopy. METHODS: This is a retrospective analysis of patients undergoing SBE for DPEJ placement in a referral hospital between January 2010 and March 2016. Technical success, clinical success and procedure related complications were recorded. RESULTS: Twenty-three patients were included (17 males, median age 71 years, range 37-93 years). The most frequent indications for DPEJ were gastroesophageal cancer (n = 10) and neurological disease (n = 8). Eighty-seven percent of the patients had a contraindication to percutaneous endoscopic gastrostomy (PEG) and PEG was unsuccessful in the remaining patients. The technical success rate was 83% (19/23), transillumination was not possible in three patients and an accidental exteriorization of the bumper resulting in a jejunal perforation occurred in one patient. The clinical success was 100% (19/19). The median follow-up was five months (range 1-35 months). Apart from the case of jejunal perforation and the two cases of accidental exteriorization, there were no other complications during follow-up. The 6-month survival was 65.8% and the 1-year survival was 49.3%. CONCLUSION: DPEJ can be carried out successfully via SBE without fluoroscopy with a low rate of significant adverse events. Although, leaving the overtube in place during the bumper pulling can be useful for distal jejunal loops, it can be safely removed in proximal loops to minimize complications.
- Parvovirus B19 Infection Associated with Hemolytic Anemia and Cranial PolyneuropathyPublication . Sequeira, J; Calado, A; Dias, M; Manita, MParvovirus B19 (PB19) is a common, widespread, small, single-stranded DNA virus which has been linked with a broad spectrum of clinical illnesses, including a variety of neurological complications such as encephalitis, meningitis, myelitis, stroke, cerebellar ataxia, and neuropathy. The authors describe a case of PB19 infection associated with hemolytic anemia and cranial polyneuropathy involving the second and third cranial nerves in a 23-year-old immunocompetent woman. The diagnosis of acute PB19 infection was established with detection of positive DNA and anti-PB19 IgM antibodies in blood samples. Antiganglioside antibody studies were performed and serum anti-GD1b IgG was strongly positive. Further investigation was normal or negative, excluding other infectious or autoimmune disorders. The patient was initially treated with a 5-day course of intravenous immunoglobulin (IGIV). Because of incomplete neurological recovery, methylprednisolone was also administered 7 days after IGIV therapy initiation. Complete resolution of clinical symptoms was observed 3 months after disease onset at follow-up visit, despite the persistence of PB19 DNA and anti-PB19 IgM antibodies in serum 5 months after the initial presentation. Our report provides evidence that PB19 could affect both the central and peripheral nervous system, possibly by triggering an autoimmune mechanism that leads to autoantibody production.
- Does Permanent Atrial Fibrillation Modify Response to Cardiac Resynchronization Therapy in Heart Failure Patients?Publication . Abreu, A; Oliveira, MM; Cunha, PS; Santa Clara, H; Portugal, G; Rodrigues, I; Santos, V; Almeida-Morais, L; Selas, M; Soares, R; Branco, LM; Cruz Ferreira, R; Mota Carmo, MINTRODUCTION: The benefits of cardiac resynchronization therapy (CRT) documented in heart failure (HF) may be influenced by atrial fibrillation (AF). We aimed to compare CRT response in patients in AF and in sinus rhythm (SR). METHODS: We prospectively studied 101 HF patients treated by CRT. Rates of clinical, echocardiographic and functional response, baseline NYHA class and variation, left ventricular ejection fraction, volumes and mass, atrial volumes, cardiopulmonary exercise test (CPET) duration (CPET dur), peak oxygen consumption (VO2max) and ventilatory efficiency (VE/VCO2 slope) were compared between AF and SR patients, before and at three and six months after implantation of a CRT device. RESULTS: All patients achieved ≥95% biventricular pacing, and 5.7% underwent atrioventricular junction ablation. Patients were divided into AF (n=35) and SR (n=66) groups; AF patients were older, with larger atrial volumes and lower CPET dur and VO2max before CRT. The percentages of clinical and echocardiographic responders were similar in the two groups, but there were more functional responders in the AF group (71% vs. 39% in SR patients; p=0.012). In SR patients, left atrial volume and left ventricular mass were significantly reduced (p=0.015 and p=0.021, respectively), whereas in AF patients, CPET dur (p=0.003) and VO2max (p=0.001; 0.083 age-adjusted) showed larger increases. CONCLUSION: Clinical and echocardiographic response rates were similar in SR and AF patients, with a better functional response in AF. Improvement in left ventricular function and volumes occurred in both groups, but left ventricular mass reduction and left atrial reverse remodeling were seen exclusively in SR patients
- Síndrome de Stevens-Johnson. Apresentação de Caso ClínicoPublication . Caixeirinho, P; Fonseca, L; Machado, C; Fernandes, AIntrodução: A Síndrome de Stevens-Johnson (SSJ) é uma doença mucocutânea rara e potencialmente fatal, mais frequente no sexo masculino, cuja incidência aumenta com a idade e em determinados grupos de risco. A SSJ e a Necrólise Tóxica Epidérmica (NET) são duas entidades da mesma doença, com severidade diferente. A etiologia não é clara, mas pensa-se que se deva maioritariamente a reacções adversas a fármacos. Caso clínico: Um jovem de 17 anos de idade, sem antecedentes pessoais relevantes, foi observado no Serviço de Urgência por surgimento de lesões maculopapulares, com 3 dias de evolução, dispersas pela face, cavidade oral, tronco e extremidades, com prostração e taquicardia. Foi internado com o diagnóstico de SSJ. Discussão e Conclusões: O SSJ e a NET têm grande morbilidade e considerável mortalidade. O rápido reconhecimento desta identidade, com a remoção do fármaco desencadeador é essencial. A perda da função de barreira da pele, com a consequente alteração da homeostasia, implica muitas vezes a manutenção da terapêutica de suporte em Unidades de Cuidados Intensivos ou de Queimados.
- Bone Biopsy Practice Patterns Across Europe: the European Renal Osteodystrophy Initiative - a Position PaperPublication . Evenepoel, P; D'Haese, P; Bacchetta, J; Cannata-Andia, J; Ferreira, A; Haarhaus, M; Mazzaferro, S; Lafage Proust, MH; Salam, S; Spasovski, G; Cozzolino, MRenal osteodystrophy (ROD) is a heterogeneous group of metabolic bone diseases complicating progressive chronic kidney disease (CKD). Bone biomarkers and bone imaging techniques may help to assess bone health and predict fractures in CKD but do have important inherent limitations. By informing on bone turnover and mineralization, a bone biopsy may help to guide prevention and treatment of ROD and its consequences. According to a recent survey conducted among European nephrologists, bone biopsies are performed rather exceptionally, both for clinical and research purposes. Obviously, clinical research in the field of ROD is threatened by vanishing clinical and pathological expertise, small patient cohorts and scientific isolation. In March 2016, the European Renal Osteodystrophy (EU-ROD) initiative was created under the umbrella of the ERA-EDTA CKD-mineral and bone disorder (MBD) Working Group to revitalize bone biopsy as a clinically useful tool in the diagnostic workup of CKD-MBD and to foster research on the epidemiology, implications and reversibility of ROD. As such, the EU-ROD initiative aims to increase the understanding of ROD and ultimately to improve outcomes in CKD patients.
- Beyond Polycystic Kidney DiseasePublication . Santos, SF; Francisco, T; Cordeiro, AI; Lopes, MJTuberous sclerosis(TS) is an autosomal dominant disease caused by mutations in TSC1 and TSC2 genes. TSC2 gene is located in chromosome 16p13.3, adjacent to PKD1 gene, responsible for the autosomal dominant polycystic kidney disease. In a rare subgroup of patients, the presence of a deletion which simultaneously affects the TSC2 and PKD1 genes has been confirmed. TSC2/PKD1-Contiguous Gene Syndrome is characterised by the early appearance of autosomal dominant polycystic kidney disease in combination with several phenotypic manifestations of TS. We present a 13-year-old girl with bilateral renal cysts detected at the age of 9 months. At the age of 13, she was referred to the Dermatology Outpatients Clinic due to a facial cutaneous eruption. She presented with facial erythema, fibroadenomas with malar distribution and disseminated hypomelanotic macules, meeting the criteria for TS. TSC2/PKD1 Contiguous Gene Syndrome deletion was suspected, being later confirmed by genetic testing.