Leading with dysphagia An unusual view of a common symptom.

Plummer-Vinson syndrome (PVS) is characterized by the classic triad of post-cricoid dysphagia, iron-deficiency anemia and esophageal webs. PVS is commonly found in women of middle age especially in the fourth and fifth decade of life. The prevalence of PVS has decreased due to early detection of iron deficiency and repletion of iron stores. We report a case of a 81-year-old female patient who had a classic presentation of PVS, treated successfully with endoscopic procedure. To our knowledge, the current case is the fourth case of dysphagia related to Plummer-Vinson syndrome reported in an octogenarian in the literature so far. Iron supplementation can resolve dysphagia in many patients, but dilation of esophageal webs may sometimes be required. PVS should be part of the differential diagnosis of sideropenic dysphagia, especially due the risk of pharyngeal and esophageal epidermoid neoplasia.

When eating becomes a real nightmare.

.We present the case of a 46-year-old female with dysphagia to solids and retrosternal pain that worsened after eating. Due to mediastinal lymphadenopathies, she underwent endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) 3 weeks before, mentioning the complaints started afterwards. On physical examination she had fever (38.3ºC). Gastroscopy revealed three 10-20mm fistulous orifices with purulent discharge at 26-32cm from the incisors and another four partially covered by fibrin in the distal esophagus. EBUS-TBNA report was reviewed, mentioning 6 needle passes through the esophagus, due to failed endotracheal intubation, without immediate complications. A cervicothoracic CT scan identified 2 mediastinal abscesses, the largest with 9cm, communicating with the esophageal fistulas. She was admitted, underwent intravenous antibiotics and endoscopy-guided nasogastric tube placement. The histopathological analysis diagnosed Castleman's disease. There was clinical and imagological improvement during admission. After 16 days she was released. Upper endoscopy was repeated one month later showing complete closure of the fistulous orifices.

Predictors for incomplete response to ursodeoxycholic acid in primary biliary cholangitis. Data from a national registry of liver disease.

BACKGROUND: The current standard of treatment in primary biliary cholangitis (PBC) is ursodeoxycholic acid (UDCA), although a considerable proportion of patients show incomplete response resulting in disease progression. OBJECTIVE: This study aimed to assess the prevalence of incomplete response to UDCA and determine associated patients' characteristics. METHODS: Patients with PBC as main diagnosis were included from a national multicentric patient registry-Liver.pt. Main endpoints included incomplete response to UDCA treatment according to Barcelona, Paris I and Paris II criteria, Globe and UK PBC scores and the association between baseline characteristics and incomplete response according to Paris II criteria. RESULTS: A total of 434 PBC patients were identified, with a mean age of 55 years and 89.2% females. Nearly half of patients were asymptomatic at diagnosis and 93.2% had positive anti-mitochondrial antibodies. Almost all patients (95.6%) had been prescribed at least one drug for PBC treatment. At the last follow-up visit, 93.3% were under treatment of which 99.8% received UDCA. Incomplete response to UDCA was observed in 30.7%, 35.3%, 53.7% and 36.4% of patients according to Barcelona, Paris I, Paris II criteria and Globe score, respectively. After adjusting for age and sex, and accordingly to Paris II criteria, the risk for incomplete biochemical response was 25% higher for patients with cirrhosis at diagnosis (odds ratio [OR] = 1.25; 95% confidence interval [95%CI]: 1.02-1.54; p = 0.033) and 35% (95%CI:1.06-1.72; p = 0.016) and 5% (OR = 1.05; 95%CI:1.01-1.10; p = 0.013) for those with elevated gamma-glutamyl transferase (GGT) and alkaline phosphatase (ALP). CONCLUSION: A considerable proportion of patients showed incomplete biochemical response to UDCA treatment according to Paris II criteria. Cirrhosis, elevated GGT and ALP at diagnosis were identified as associated risk factors for incomplete response. Early identification of patients at risk of incomplete response could improve treatment care and guide clinical decision to a more careful patient monitorization.

POEMS syndrome and idiopathic portal hypertension: a possible association

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POEMS syndrome and idiopathic portal hypertension: a possible association.

A 48-year old female patient was admitted to the emergency department with upper gastrointestinal bleeding. Endoscopy showed large esophageal varices that were treated with band ligation. She had been treated with cyclophosphamide, melphalan, lenalidomide and corticosteroids for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy or edema, M protein, skin changes). She had no other risk factors for chronic liver disease. Laboratory and radiological examinations could not confirm the etiology of portal hypertension. The liver biopsy suggested hepatoportal sclerosis, compatible with idiopathic portal hypertension (IPH). Under a band ligation program, with beta-blocker, diuretics and prophylactic anticoagulation, the patient remains stable. In POEMS syndrome, a rare multi-systemic disease, the most frequent liver manifestation is hepatomegaly(1). To the best of authors' knowledge, IPH has been reported only 3 times in POEMS syndrome(2,3,4) and this is the second case reporting the occurrence of histological findings compatible with IPH. As the precise etiopathogenesis of IPH is not fully elucidated and as it is an extremely uncommon manifestation in POEMS, it is not yet certain whether IPH is part of the syndrome or whether it develops independently. While some investigators believe that there is a congenital vascular anomaly involving the changes in the portal tract, the majority support an acquired vascular defect hypothesis(5). The acquired IPH hypothesis emphasizes a possible role of various pathogenic determinants: infections, prothrombotic states, prolonged exposure to several medications and toxins, immunological basis and progressive fibrosis of the portal veins (5). In this hypothesis, a hepatic circulation defect secondary to POEMS syndrome, which includes pro-inflammatory (VEGF, interleukin (IL)-6, IL-1ß, tumor necrosis factor-α, tumor growth factor-ß) and pro-thrombotic factors (aberrations in the thrombin-antithrombin complex)(1) which have been documented in the IPH and POEMS syndrome, may contribute to a possible association between these entities. Although the association between these two entities is yet to be confirmed, the authors present this case in order to contribute to its elucidation.

Refractory Celiac Disease Type II: A Case Report that Demonstrates the Diagnostic and Therapeutic Challenges.

Refractory celiac disease is an uncommon but serious complication of celiac disease. We describe a case of a severe refractory celiac disease type II, complicated with ulcerative jejunoileitis, in a 68 years old female, unresponsive to consecutive treatments with budesonide, prednisolone, cladribine and autologous stem cell transplantation. The patient maintained severe malnutrition, advanced osteoporosis, anaemia, vitamin deficiencies and hydro-electrolytic imbalances, necessitating consecutive hospitalizations for total parenteral nutrition. The patient also developed life-threatening complications, namely respiratory and urinary septic shock and also episodes of haemorrhagic shock secondary to ulcerative jejunoileitis. The progression to enteropathy associated T-cell lymphoma was never demonstrated, but the patient died 7 years after the diagnosis due to a septic shock secondary to a nosocomial pneumonia and osteomyelitis related to a spontaneous hip fracture. This case highlights the difficulties in the diagnostic process, therapeutic management and surveillance of this rare condition associated with very poor prognosis.

A doença celíaca refratária é uma complicação rara mas muito severa da doença celíaca. Apresentamos o caso de uma doente de 68 anos, com doença celíaca refratária tipo II complicada de jejunoileíte ulcerativa, que não respondeu a tratamentos sucessivos com budesonido, prednisolona, cladribina e transplante autólogo de medula óssea. A doente manteve desnutrição e osteoporose severas, deficiências vitamínicas e desequilíbrios hidro-eletrolíticos, necessitando de múltiplas hospitalizações para receber nutrição parentérica total. Também desenvolveu diversas complicações potencialmente fatais nomeadamente sépsis com origem respiratória e urinária e choque hemorrágico secundário à jejunoileíte ulcerativa. Contudo, nunca se demonstrou a presença de um linfoma de células T. A doente faleceu 7 anos após o diagnóstico devido a choque sético secundário a pneumonia nosocomial e osteomielite relacionada com fratura espontânea da anca. Este caso ilustra as dificuldades sentidas no decurso do diagnóstico, terapêutica e vigilância desta entidade clínica rara, a qual está associada a um péssimo prognóstico.

Ampullary Metastasis From Breast Cancer: A Rare Cause of Obstructive Jaundice.

Breast cancer is the most common tumor in women and the first cause of death for malignancy in the female. Bile ducts are not among the common sites of metastasis from breast cancer. Few cases of obstructive jaundice due to metastatic breast cancer have been described in the literature and they mostly resulted from widespread liver metastases that eventually involved the bile ducts. We report an exceptional case of ampullary metastasis in the absence of liver metastases. Sporadic reports have been published about the involvement of the ampulla by breast cancer metastasis. This case emphasizes the need to consider this diagnosis in women presenting with obstructive jaundice, especially when there is a clinical possibility of breast cancer.

O cancro da mama é o tumor mais comum em mulheres e a principal causa de morte por neoplasia nesta população. A via biliar não é um local comum de metastização desta neoplasia. Poucos casos de icterícia obstrutiva devido a metástases mamárias têm sido descritos na literatura e ocorrem principalmente devido a metástases hepáticas que comprimem a via biliar. Relatamos um caso excepcional de metástase ampular na ausência de metástases hepáticas.Existem apenas relatos esporádicos do envolvimento da ampola por metástase mamárias. Este caso enfatiza a necessidade de considerar este diagnóstico perante um quadro de icterícia obstrutiva, especialmente em doentes com possível neoplasia mamária.

Portal Hypertensive Biliopathy: An Infrequent Cause of Biliary Obstruction.

INTRODUCTION: Biliary obstruction is usually caused by choledocholithiasis. However, in some circumstances, alternative or concurring unusual ethiologies such as portal hypertensive biliopathy (PHB) must be considered. CLINICAL CASE: We present the case of a 36-year-old female complaining of jaundice and pruritus. Liver function tests were compatible with biliary obstruction and the ultrasound scan of the abdomen showed dilatation of the intrahepatic biliary ducts, a dilated common bile duct (CBD) and biliary calculi. The computed tomography of the abdomen revealed a portal cavernoma encasing the CBD. DISCUSSION: Portal cavernoma, the hallmark of extrahepatic portal venous obstruction, can cause PHB. When symptomatic, chronic cholestasis is present if a dominant stricture exists whereas biliary pain and acute cholangitis occur when choledocholithiasis prevails. Management must be individualized and usually includes endoscopic therapy to address choledocholithiasis and shunt surgery for definitive treatment.

INTRODUÇÃO: A causa mais comum de icterícia obstrutiva é a coledocolitíase. No entanto, no contexto clínico adequado, devem ser consideradas etiologias alternativas ou concomitantes, nomeadamente a biliopatia hipertensiva portal (BHP). CASO CLÍNICO: Apresentamos o caso de uma doente do sexo feminino com 36 anos de idade com icterícia e prurido. O estudo bioquímico era compatível com icterícia obstrutiva e o estudo ecográfico do abdómen revelou dilatação das vias biliares intra-hepáticas e do colédoco associados a litíase biliar. A tomografia computorizada abdominal realizada mostrou a existência um cavernoma da veia porta a envolver o colédoco. DISCUSSÃO: O cavernoma da porta, no contexto de obstrução portal venosa extra-hepática pode complicar-se com BHP. Quando sintomática manifesta-se por colestase crónica caso exista uma estenose dominante ou dor biliar e/ou colangite aguda quando predomina a litíase. O tratamento definitivo é individualizado, incluindo terapêutica endoscópica visando a litíase associada e uma derivação cirúrgica venosa porto-sistémica.

Results from the International Silver Graft Registry for high-risk patients treated with a metallic-silver impregnated vascular graft.

The purpose of this postmarket surveillance registry was to document the efficacy of a vascular prosthesis coated with metallic silver in high-risk patients undergoing vascular reconstructions. Patency (primary endpoint) and freedom from graft infection (secondary endpoint) data were assessed at a minimum of 12 months in patients with significant co-morbidity and/or confirmed graft infections or infected native vessels. Between November 2006 and December 2009, 230 patients with high-risk factors underwent aortic,peripheral and/or extra-anatomic reconstructions with Silver Graft® (SG) in six German, one French and one Polish vascular center.All participating centers used the metallic silver-coated polyester graft (SG) in various diameters and lengths including tubular and bifurcate vascular grafts. Doppler ultrasound follow-ups to determine graft patency were planned at 12 months or done at an earlier time in case the patient became symptomatic. A total of 230 patients were studied. Ten of these 230 patients had graft infections at baseline whereas the remaining 220 subjects had significant risk factors such as coronary artery disease (62.7%, 138/220), vascular access in scar tissue (27.3%, 60/220), Fontaine III/IV (38.2%, 84/220), chronic renal insufficiency (26.8%, 59/220) and diabetes (21.0%,46/220). The long-term follow-up at 15.5 ± 8.3 months revealed a secondary patency rate of 93.2% (205/220) and an 'all cause' mortality rate of 18.6% (41/220). There was a freedom from de novo graft infection rate of 95.9% (211/220) in the high-risk group without graft infections at baseline. One regraft infection occurred distal of the revisional reconstruction in the 10 patients with graft infection at baseline. The presence of perigraft fluid at follow-up and Fontaine III/IV at baseline were found to be predictors for graft patency whereas perigraft fluid presence was the only predictor for de novo graft infections. This registry revealed favorable patency and freedom from de novo infections rates in a 'high-risk' population with significant co-morbidities.

Fatal hepatitis B reactivation treated with entecavir in an isolated anti-HBs positive lymphoma patient: a case report and literature review.

Hepatitis B virus (HBV) reactivation is a well-recognized complication that occurs in lymphoma patients who undergo chemotherapy. Only very few cases of HBV reactivation in patients with isolated antibody against hepatitis B surface antigen (anti-HBs) have been reported. We present a case of a 78-year-old woman diagnosed with diffuse large B cell non-Hodgkin's lymphoma who only displayed a positive anti-HBs, as the single possible marker of occult HBV infection, before starting therapy. She was treated with several chemotherapeutic regimens (including rituximab) for disease relapses during 3 years. Forty days after the last cycle of chemotherapy, she presented with jaundice, markedly elevated serum aminotransferase levels, and coagulopathy. HBV serology showed positivity for HBsAg, anti-HBc and anti-HBs. HBV DNA was positive. Antiviral treatment with entecavir was promptly initiated, but the patient died from liver failure. A review of the literature of HBV reactivation in patients with detectable anti-HBs levels is discussed.

Habituation and short-term repeatability of thermal testing in healthy human subjects and patients with chronic non-neuropathic pain.

We investigated habituation effects during thermal quantitative sensory testing (tQST) using 8 repetitive measurements for thermal detection and pain thresholds. The same measurements were repeated two days later. 39 healthy subjects and 36 patients with chronic non-neuropathic pain syndromes (migraine, tension-type headache, non-radicular back pain) were enrolled. The pain intensity was assessed using an 11-point (0-10) numerical rating scale. Measurements correlated significantly over the two days in both groups (r=0.41...0.62). Warm detection (WDT) and heat pain threshold (HPT) revealed no significant differences over these days. Cold detection (CDT) and pain thresholds (CPT) showed significant differences but these were small compared to the range of normal variability (CDTDelta -0.28 degrees C; CPTDelta 1.51 degrees C). On both days, WDT showed no habituation during measurements. Although there was a small difference in CDT and CPT between first and second measurement, there was no habituation beyond the second stimuli. In contrast, HPT significantly increased between first and sixth stimuli, indicating pronounced habituation. Average HPT of first to third measurement was significantly lower than HPT of the fourth to sixth assessment (45.9 degrees C; 47.7 degrees C) with a good day-to-day repeatability. Repeatability and habituation was identical in both groups. Ongoing pain intensity in the patient groups correlated significantly with CDT/WDT but not with CPT, HPT, indicating that ongoing pain might suppress the sensitivity to non-painful stimuli. In summary, tQST proved a reliable diagnostic tool for clinical practice. Day-to-day differences were small but without clinical relevance. Habituation was most pronounced for HPT, probably due to peripheral fatigue of the receptors.