Gastrinoma de Localização Pilórica numa Doente com Úlcera Péptica Recorrente

Apresenta-se o caso clínico de uma mulher de 56 anos, raça branca, com úlcera duodenal e esofagite com 3 anos de evolução apesar de terapêutica mantida com bloqueadores dos receptores H2. O valor da gastrina sérica basal estava ligeiramenteacima dos limites superiores do normal. O teste de estimulação de secretina foi sugestivo de gastrinoma. Com tomografia axial computorizada e arteriografia selectiva não se localizou o tumor. Na laparotomia exploradora encontrou-se um nódulo pilórico que se demonstrou corresponder ao gastrinoma. Dezoito meses após a cirurgia a doente encontrou-se assintomática e os exames de controle são negativos.

Stress-Related Mucosal Disease: Incidence of Bleeding and the Role of Omeprazole in its Prophylaxis

BACKGROUND: Upper gastrointestinal bleeding is the severe complication of stress-related mucosal disease in hospitalized patients. In intensive care units (ICU), risk factors are well defined and only mechanical ventilation and coagulopathy proved to be relevant for significant bleeding. On the contrary, in non-ICU settings there is no consensus about this issue. Nevertheless, omeprazole is still widely used in prophylaxis of bleeding. The objective of our study was to evaluate the relevance of stress-related mucosal disease bleeding in patients admitted to an internal medicine ward, and the role of omeprazole in its prophylaxis. METHODS: We conducted a retrospective study in which we analysed consecutive patients who were admitted to our ward over a year. We recorded demographic characteristics of the patients, potential risk factors for stress-related mucosal disease (clinical data, laboratory, and medication), administration of prophylactic omeprazole, and total cost of this prophylaxis. Patients with active gastrointestinal bleeding on the admission were excluded. We recorded every upper gastrointestinal bleeding event with clinical relevance. RESULTS: Five hundred and thirty-five patients, mean age 70 years, mean length of stay 9.6+/-7.7 days; 140 (26.2%) patients were treated with 40 mg of omeprazole intravenously, 193 (36.1%) with 20mg of omeprazole orally, and 202 (37.8%) patients had no prophylaxis. There was only one episode (0.2%) of clinically relevant bleeding. CONCLUSION: In patients admitted to an internal medicine ward, incidence of upper gastrointestinal bleeding as a complication of stress-related mucosal disease is low. We found that there is no advantage in prophylaxis with omeprazole.

Onychomycosis in Patients with Chronic Leg Ulcer and Toenail Abnormalities

Nails have a limited number of reactive patterns to disease. Accordingly, toenail changes of different etiologies may mimic onychomycosis. OBJECTIVE To determine the prevalence of toenail onychomycosis among patients with leg ulcer and toenail abnormalities attending a dermatology clinic. METHODS A cross-sectional study was conducted through the analysis of clinical records and results of mycological examination. RESULTS A total of 81 patients were included, with a median age of 76.0 years. Most ulcers were of venous etiology, followed by those of mixed and arterial pathogenesis. The mycological evaluation confirmed the diagnosis of onychomycosis in 27.2% of the patients. The etiologic agent was a dermatophyte in 59.1% of isolates in nail samples, while Trichophyton interdigitale was the most frequent fungal species (40.9%). CONCLUSIONS Most toenail abnormalities in patients with chronic leg ulcer were not onychomycosis. This study highlights the importance of systematic mycological examination in these patients, in order to avoid overtreatment with systemic antifungals, unnecessary costs and side effects.

A Nonhealing Ulcer on the Nose. A Case Report

Dermatitis artefacta is a disease characterized by self-inflicted skin lesions as the result or manifestation of psychiatric disorders or specific stress situations. Clinical manifestations range from superficial erosions to deep wounds. Because of its rarity and the polymorphism of the lesions, dermatitis artefacta is often a challenge for the clinicians. This report presents the case of a 62-year-old woman who had an ulcer of the nose lasting for three years caused by digital manipulation. Early recognition of dermatitis artefacta is difficult but avoids unnecessary treatments. A multidisciplinary approach to this entity is necessary to obtain the best results.

Hand Involvement in Ollier Disease and Maffucci Syndrome: a Case Series

Ollier Disease and Maffucci Syndrome are two rare diseases that can cause tumors in several organs, having a special predilection for the hand. However, there have been very few reports in the literature focusing on hand manifestations of these diseases. We report the cases of three female patients: one with Ollier Disease, and two other with Maffucci Syndrome. All patients had hand involvement as their initial primary complaint. The Ollier Disease patient developed chondrosarcomas of two digits and had to have these fingers amputated. One of the Maffucci patients died one year after presentation from a brain glioblastoma. These cases emphasize the importance of early diagnosis of Ollier Disease and Maffucci Syndrome, as these two conditions are associated not only to crippling hand deformity, but also to a significant risk of chondrosarcoma, and other malignant tumors.

Risk of Surgery for Congenital Heart Disease in the Adult: a Multicentered European Study

BACKGROUND: Surgery for congenital heart disease (CHD) has changed considerably during the last three decades. The results of primary repair have steadily improved, to allow treating almost all patients within the pediatric age; nonetheless an increasing population of adult patients requires surgical treatment. The objective of this study is to present the early surgical results of patients who require surgery for CHD in the adult population within a multicentered European study population. METHODS: Data relative to the hospital course of 2,012 adult patients (age > or = 18 years) who required surgical treatment for CHD from January 1, 1997 through December 31, 2004 were reviewed. Nineteen cardiothoracic centers from 13 European countries contributed to the data collection. RESULTS: Mean age at surgery was 34.4 +/- 14.53 years. Most of the operations were corrective procedures (1,509 patients, 75%), followed by reoperations (464 patients, 23.1%) and palliative procedures (39 patients, 1.9%). Six hundred forty-nine patients (32.2%) required surgical closure of an isolated ostium secundum atrial septal defect. Overall hospital mortality was 2%. Preoperative cyanosis, arrhythmias, and NYHA class III-IV, proved significant risk factors for hospital mortality. Follow-up data were available in 1,342 of 1,972 patients (68%) who were discharged home. Late deaths occurred in 6 patients (0.5%). Overall survival probability was 97% at 60 months, which is higher for corrective procedures (98.2%) if compared with reoperations (94.1%) and palliations (86.1%). CONCLUSIONS: Surgical treatment of CHD in adult patients, in specialized cardiac units, proved quite safe, beneficial, and low-risk.

Úlceras de Perna em Portugal: um Problema de Saúde Subestimado

To determine the prevalence and aetiology of leg ulceration in a population of patients registered with five health centres within Lisbon, a study was undertaken to identify patients receiving care from community and hospital. Identification of patients was through health professionals, with a simple questionnaire completed for all patients identified who were registered with the five health centres. In 263 patients were identified in a population of 186,000 (total prevalence 1.41/1,000 population). The prevalence was similar between men and women (1.3 and 1.46/1,000, respectively). As expected this was highly age dependent, being most common in patients aged over 80 years (6.5 and 4.9/1,000, respectively). The ulceration was highly chronic in nature, with median ulceration of 18 months. Of the 240 with ulcer duration recorded, 158 (66%) had the present ulcer for longer than one year, and 40 (17%) for longer than five years. The cause of ulceration was unknown to the health professional treating the patient in 86 (33%) of the cases. Of those with a cause, most commonly this was venous (80%) with 10% mixed arterial/venous ulceration and 3% frank arterial disease. Most care was provided by community services, with 145 (55%) treated in health centres and 77 (29%) treated in the patient's home. The mean number of treatments per week was 3.0, with 21 (9%) of patients being seen on a daily basis. Most patients (80%) had seen a specialist doctor for their ulceration, most often a dermatologist (48%) and a vascular surgeon (33%). The prevalence of chronic leg ulceration is similar to other reported studies in western Europe, and indicates that approximately 14,000 patients suffer from leg ulceration at any one time in Portugal. This produces a high burden on both hospital and community services.

Annular Lichen Planus in Association with Crohn Disease

BACKGROUND: Lichen planus is an idiopathic inflammatory disease of the skin and mucous membranes. Although the etiology is not established, it has been associated with autoimmune diseases, viral infections, drugs and dental restoration materials. However, the association with inflammatory bowel disease has been very rarely reported in the literature. CASE REPORT: A 19-year-old female patient presented with annular lesions on her upper body and limbs, with a sharply defined border and non-atrophic skin in the center. The lesions were hyperpigmented and had been stable for over one year. The histopathology confirmed the diagnosis of annular lichen planus. She had weight loss, occasional diarrhea, and a severe anemia. The investigation of these symptoms led to the diagnosis of Crohn disease and a sickle cell trait. Therapy with systemic corticosteroids and mesalazine controlled the intestinal disease, with concomitant improvement of the skin lesions. CONCLUSIONS: As lichen planus can be associated with other immunological disorders, the association with inflammatory bowel disease should be considered in the evaluation of the patient.

Does Metabolic Syndrome Predict Significant Angiographic Coronary Artery Disease?

INTRODUCTION: Metabolic syndrome (MS) is an independent predictor of acute cardiovascular events. However, few studies have addressed the relationship between MS and stable angiographic coronary artery disease (CAD), which has a different pathophysiological mechanism. We aimed to study the independent predictors for significant CAD, and to analyze the impact of MS (by the AHA/NHLBI definition) on CAD. METHODS: We prospectively included 300 patients, mean age 64±9 years, 59% male, admitted for elective coronary angiography (suspected ischemic heart disease), excluding patients with known cardiac disease. All patients underwent assessment of demographic, anthropometric, and laboratory data and risk factors, and subsequently underwent coronary angiography. RESULTS: In the study population, 23.0% were diabetic, 40.5% had MS (and no diabetes) and 36.7% had neither diagnosis. Significant CAD was present in 51.3% of patients. CAD patients were older and more frequently male and diabetic, with increased triglycerides and glucose and lower HDL cholesterol. Abdominal obesity was also less prevalent. MS was not associated with the presence of CAD (OR 0.94, 95% CI 0.59-1.48, p=0.778). Of the MS components, the most important predictors of CAD were increased glucose and triglycerides. Abdominal obesity was associated with a lower risk of CAD. In a multivariate logistic regression model for CAD, independent predictors of CAD were age, male gender, glucose and triglycerides. Body mass index had a protective effect. CONCLUSIONS: Although MS is associated with cardiovascular events, the same was not found for stable angiographically proven CAD. Age, gender, diabetes and triglycerides are the most influential factors for CAD, with abdominal obesity as a protective factor.

Prevalence of Metabolic Syndrome and Diabetes on a High-Risk Population with Suspected Coronary Artery Disease

Objectivos: A prevalência de Sindroma Metabólica (SM) e diabetes é variável consoante a definição utilizada, assim como com a região geográfica e o grupo étnico estudado. Não existem estudos em indivíduos portugueses com suspeita de doença arterial coronária. Analisámos a prevalência de SM e diabetes nesta população específica de doentes, comparando também definições. Métodos: Incluíram-se no estudo 300 indivíduos, com uma idade media de 64 ± 9 anos, 59% do género masculino, admitidos para angiografia coronária electiva, tendo sido excluídos os doentes com doença cardíaca previamente conhecida. Avaliou-se a prevalência de SM e de diabetes. Resultados: A prevalência ajustada de SM foi de 39,3% (critério NCEP-ATP III), 53,8% (critério IDF) e 48,4% (critério AHA/NHLBI). A prevalência ajustada de diabetes foi de 14,8% pela definição prévia da ADA e de 36.4% com a definição mais recente. A concordância global entre as definições de SM foi de 45,3%, sendo mais elevada entre as definições da AHA/NHLBI e da NCEP-ATP III (Kappa 0,821). A prevalência de SM está altamente dependente da idade em ambos os géneros, sendo mais prevalente no género feminino. A prevalência de diabetes é também dependente da idade, sendo semelhante em ambos os géneros. O componente de SM mais frequente é a hipertensão arterial, seguido pela obesidade abdominal, elevação da glicose, colesterol-HDL baixo e finalmente elevação dos triglicéridos. É também importante referir que 60% dos doentes estavam sob terapêutica hipolipemiante (56,6% com estatinas, 1,7% com fibratos e 1,7% com ambos). A diferença mais significativa entre géneros no que diz respeito aos componentes de SM é a elevada prevalência de obesidade abdominal no género feminino. Conclusões: Nesta população de alto risco, a prevalência de SM é elevada, sendo contudo a prevalência de diabetes semelhante à registada em estudos epidemiológicos na população geral.

Mesenchymal Stem Cell Therapy in Heart Disease

Cardiovascular disease is among the main causes of mortality and morbidity worldwide. Despite significant advances in medical and interventional therapy, the prognosis of conditions such as ischemic heart disease is still dismal. There is thus a need to investigate new therapeutic tools, one of which is stem cell therapy. Hematopoietic stem cells are the most studied type, and the fact that their biology is relatively well understood has led to their being used in preclinical research and clinical trials. However, the results of some of these studies have been controversial, which has opened the way for studies on other cell types, such as mesenchymal stem cells. These cells have immunomodulatory properties which suggest that they have therapeutic potential in cardiology. In the present article, the authors review the state of the art regarding mesenchymal stem cells, from basic and translational research to their use in clinical trials on ischemic heart disease, heart failure and arrhythmias, and discuss possible future uses.

Clinical and Pathological Findings in Women with Fabry Disease

Introduction. Fabry disease is a rare metabolic disorder caused by the genetic deficiency of the lysosomal hydrolase alpha-galactosidase A, located on chromosome X. Females with the defective gene are more than carriers and can develop a wide range of symptoms. Nevertheless, disease symptoms generally occur later and are less severe in women than in men. The enzyme deficiency manifests as a glycosphingolipidosis with progressive accumulation of glycosphingolipids and deposit of inclusion bodies in lysosomes giving a myelinlike appearance. Patients and Methods. Records of renal biopsies performed on adults from 1st January 2008 to 31st August 2011, were retrospectively examined at the Renal Pathology Laboratory. We retrieved biopsies diagnosed with Fabry disease and reviewed clinical and laboratory data and pathology findings. Results. Four female patients with a mean age of 49.3±4.5 (44-55) years were identified. The mean proteinuria was 0.75±0.3 g/24h (0.4-1.2) and estimated glomerular filtration rate (CKD EPI equation) was 71±15.7 ml/min/1.73m2 (48-83). Three patients experienced extra-renal organ involvement (cerebrovascular, cardiac, dermatologic, ophthalmologic and thyroid) with distinct severity degrees. Leukocyte α-GAL A activity was below normal range in the four cases but plasma and urinary enzymatic activity was normal. Light microscopy showed predominant vacuolisation of the podocyte cytoplasm and darkly staining granular inclusions on paraffin and plastic-embedded semi-thin sections. Electron microscopy showed in three patients the characteristic myelin-like inclusions in the podocyte cytoplasm and also focal podocyte foot process effacement. In one case the inclusions were also present in parietal glomerular cells, endothelial cells of peritubular capillary and arterioles. Conclusion. Clinical signs and symptoms are varied and can be severe among heterozygous females with Fabry disease. Intracellular accumulation of glycosphingolipids is a characteristic histologic finding of Fabry nephropathy. Since this disease is a potentially treatable condition, its early identification is imperative. We should consider it in the differential diagnosis of any patient presenting with proteinuria and/or chronic kidney disease, especially if there is a family history of kidney disease.

Orthotopic Liver Transplantation in Familial Amyloidotic Polyneuropathy Is Associated with Long-Term Progression of Renal Disease

Orthotopic liver transplantation has become the treatment of choice for familial amyloidotic polyneuropathy. The aims of this study were to evaluate the renal complications post orthotopic liver transplantation in familial amyloidotic polyneuropathy and their impact. We retrospectively studied 185 recipients who underwent 217 orthotopic liver transplants. Mean age 36.8±9.5 years, 59% males, 14.3% with renal dysfunction pre orthotopic liver transplantation. Mean follow-up 3.6±3.7 years. Thirty-two patients died. Univariate and multivariate analysis were performed, and p<0.05 was considered significant. Acute kidney injury occurred in 57 patients and renal replacement therapy was needed in 16/57. In multivariate analysis, acute kidney injury was correlated with development of chronic kidney disease (p<0.001). Relating to development of chronic kidney disease, 23.5% had progress to stage 3, 6% to stage 4 and 5.1% to stage 5d. According to Spearmen correlation, risk factors for chronic kidney disease development were age (p<0.001), renal dysfunction pre orthotopic liver transplantation (p<0.001) and acute kidney injury post orthotopic liver transplantation (p<0.001). Mortality was correlated with age (p<0.001), retransplantation need (p=0.004), renal dysfunction pre orthotopic liver transplantation (p<0.001), acute kidney injury post orthotopic liver transplantation (p=0.04), and chronic kidney disease stage 5 (p<0.001). Using binary regression, mortality was correlated with chronic kidney disease development (p=0.02). In conclusion, familial amyloidotic polyneuropathy patients are disposed to renal complications that have a negative impact on the survival of these patients.