Whipple’s disease-A typical endoscopic finding of a rare disease

A 49-year-old female presented with a 5-month course of diarrhoea, nocturn abdominal pain, asthenia, and weight loss of 30% of her body mass in three months. The patient had also a four-year medical history of bilateral mechanic gonalgy and arthralgias of the metacarpophalangeal and interphalangeal joints, despite treatment with prednisolone. On examination the patient had hyperpigmentation of the face and thorax, low-grade fever, and a BMI of 15,8 Kg/m2. Diarrhoea was documented with watery stools seven times per day despite loperamide, brownish, with no visible blood or mucous. Since the upper GI endoscopy and colonoscopy had no macroscopic abnormalities, the patient underwent a capsule endoscopy, which revealed continuous mucosal lesion with lymphangiectasia, oedema, villous atrophy and areas of denudation with hematinic punctate from the duodenum to the ileum. Diagnosis of Whipple’s Disease was made with typical histology findings in duodenum material and a positive PCR for Tropheryma whipplei. (AU)

Whipple's disease - A typical endoscopic finding of a rare disease.

A 49-year-old female presented with a 5-month course of diarrhoea, nocturn abdominal pain, asthenia, and weight loss of 30% of her body mass in three months. The patient had also a four-year medical history of bilateral mechanic gonalgy and arthralgias of the metacarpophalangeal and interphalangeal joints, despite treatment with prednisolone. On examination the patient had hyperpigmentation of the face and thorax, low-grade fever, and a BMI of 15,8 Kg/m2. Diarrhoea was documented with watery stools seven times per day despite loperamide, brownish, with no visible blood or mucous. Since the upper GI endoscopy and colonoscopy had no macroscopic abnormalities, the patient underwent a capsule endoscopy, which revealed continuous mucosal lesion with lymphangiectasia, oedema, villous atrophy and areas of denudation with hematinic punctate from the duodenum to the ileum. Diagnosis of Whipple's Disease was made with typical histology findings in duodenum material and a positive PCR for Tropheryma whipplei.

Culture-Negative Endocarditis and Cerebral Demyelination: A Challenging Case of Whipple's Disease.

Whipple's disease is a rare condition that, when not recognized and properly treated, can be fatal. A 49-year-old female presented with a five-month history of diarrhoea with watery stools seven times per day, nocturnal abdominal pain, asthenia, and a weight loss of 30% of her body mass in three months. The patient had a four-year medical history of bilateral mechanic gonalgia, arthralgia of the metacarpophalangeal and interphalangeal joints, and anaemia. The examination was remarkable for hyperpigmentation, along with a body mass index (BMI) of 15.8 kg/m2. The diagnosis of Whipple's disease was made with upper gastrointestinal endoscopy, with typical histologic findings and a positive PCR for Tropheryma whipplei. Investigations also revealed cerebral demyelination and endocarditis in three valves. Treatment with intravenous ceftriaxone for four weeks and oral cotrimoxazole for one year resulted in complete resolution of the symptoms and endocarditis. This case report shows uncommon features of the disease and debates the challenging decisions toward diagnosis and effective treatment.

A case report: black oesophagus as a possible complication of transcatheter aortic valve implantation.

BACKGROUND: The increasing number of transcatheter aortic valve implantation (TAVI) in the last few years has unveiled a unique set of events and complications that need prompt recognition and management in order to improve patient outcomes, often involving a multidisciplinary team. CASE SUMMARY: We present a case of a 86-year-old woman with symptomatic severe aortic stenosis that underwent a TAVI and, in the post-procedure period, presented with acute abundant haematemesis, haemodynamic instability, and haemoglobin drop. The diagnosis of acute necrotizing oesophagitis (ANE) was made by upper gastrointestinal endoscopy. DISCUSSION: Acute necrotizing oesophagitis is a rare entity caused usually by an ischaemic insult in the presence of predisposing factors; it has a high rate of complications and mortality. To the best of our knowledge, this is the first clinical case report to describe the occurrence of ANE as a possible complication of TAVI and is also an example of the importance of the multidisciplinary approach of these complex patients, which extends even beyond the concept of Heart Team.

Incidence, predictive factors and clinical significance of development of portal vein thrombosis in cirrhosis: A prospective study.

BACKGROUND AND AIMS: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. There are few prospective studies validating risk factors for development of PVT. We analysed the incidence, factors associated with PVT development and its influence on cirrhosis decompensations and orthotopic liver transplant (OLT)-free survival. METHODS: In this prospective observational study between January 2014 and March 2019, 445 consecutive patients with chronic liver disease were screened and finally 241 with cirrhosis included. Factors associated with PVT development and its influence on cirrhosis decompensations and OLT-free survival by time dependent covariate coding were analysed. RESULTS: Majority of patients belonged to Child-Pugh class A 184 (76.3%) and the average MELD score was 10 ± 5. Previous cirrhosis decompensations occurred in 125 (52.1%), 63 (26.1%) were on NSBB and 59 (27.2%) had undergone banding for bleeding prophylaxis. Median follow-up was 29 (1-58) months. Cumulative incidence of PVT was 3.7% and 7.6% at 1 and 3 years. Previous decompensation of cirrhosis and low platelet counts but not NSBB independently predicted the development of PVT. During follow-up, 82/236 (34.7%) patients developed cirrhosis decompensations. OLT-free survival was 100% and 82.8% at 3 years, with and without PVT respectively. MELD score, but not PVT, independently predicted cirrhosis decompensations (HR 1.14; 95%CI:1.09-1.19) and OLT-free survival (HR 1.16;95%CI:1.11-1.21). CONCLUSION: Previous decompensations of cirrhosis and thrombocytopenia predict PVT development in cirrhosis suggesting a pathophysiologic role for severity of portal hypertension. PVT development did not independently predict cirrhosis decompensations or lower OLT-free survival.

Anticoagulation in Cirrhosis and Portal Vein Thrombosis Is Safe and Improves Prognosis in Advanced Cirrhosis.

BACKGROUND: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. AIMS: We analyzed the safety and effect of anticoagulant therapy (AT) on PVT recanalization and orthotopic liver transplant (OLT)-free survival. METHODS: Eighty consecutive patients from a prospective registry of cirrhosis and non-tumoral PVT at a tertiary center were analyzed. AT effect on PVT recanalization and OLT-free survival was determined by time-dependent Cox regression analysis. RESULTS: Average MELD score was 15 ± 7. Portal hypertension-related complications at PVT diagnosis were present in 65 (81.3%) patients. Isolated portal vein trunk/branch thrombosis was present in 53 (66.3%) patients. AT was started in 37 patients. AT was stopped in 17 (45.9%) patients, in 4 (10.8%) due to bleeding events. No variceal bleeding occurred while on AT. Anticoagulation was restarted in 6/17 (35.2%) patients due to rethrombosis. In 67 patients with adequate follow-up imaging, AT significantly increased the rate of PVT recanalization compared with those who did not receive anticoagulation [51.4% (18/35) vs 6/32 (18.8%), p = 0.005]. OLT-free survival after a median follow-up of 25 (1-146) months was 32 (40%). Although there was no significant effect of AT on overall OLT-free survival, OLT-free survival was higher among patients with MELD ≥ 15 receiving AT compared to those who did not (p = 0.011). Baseline MELD at PVT detection independently predicted PVT recanalization (HR 1.11, 95% CI 1.01-1.21, p = 0.027) and mortality/OLT (HR 1.12, 95% CI 1.05-1.19, p < 0.001). CONCLUSIONS: Although AT did not improve overall OLT-free survival, it was associated with higher survival in advanced cirrhosis. Anticoagulation increased PVT recanalization and should be maintained after PVT recanalization to avoid rethrombosis.

Identifying palliative care needs in a Portuguese liver unit.

BACKGROUND & AIMS: Chronic liver disease is a major worldwide cause of morbidity and mortality. Palliative care policies are not clearly established in chronic liver disease. The NECPAL CCOMS-ICO© (NECesidades PALiativas/Palliative Needs) is a tool to identify palliative care needs, including a section for liver disease. AIM: The aim of this study was to identify palliative care needs in liver patients hospitalised in a tertiary referral Liver Unit. METHODS: Single-centre prospective observational study. One hundred and twenty patients with cirrhosis were included and NECPAL questionnaire was applied to all patients in a 7-month period. RESULTS: 84.2% of patients were considered as requiring palliative intervention; however, clinicians identified those needs only in 65.8% of the cases and caregivers in 6.7% of the cases; less than 8% of the patients were referred for palliative care consultation. An excessive use of healthcare resources (positive answer to question 3) was strongly associated with a positive need for palliative care (positive NECPAL): OR 7.305, CI 95% 2.54-20.995, P < .001). An excessive use of healthcare facilities has a sensitivity of 84.2% and a specificity of 42.1% for prediction of a positive NECPAL result (AUC 0.710, 95% CI 0.570-0.850, P = .004). CONCLUSIONS: The NECPAL CCOMS-ICO© represents a feasible and easy-to-use tool to identify palliative care needs in patients with chronic liver disease.

Long-evolution ascites in a patient with constrictive pericarditis

Constrictive pericarditis (CP) is an uncommon disease resulting from chronic pericardial inflammation, fibrosis and calcification. Since there are atypical forms of presentation, with subtle or nonexistent cardiorespiratory symptoms, diagnosis may be challenging and difficult. Recurrent ascites in patients with congestive hepatopathy due to constrictive pericarditis is, in most cases, reversible after pericardiectomy. Nevertheless, development of persistent liver dysfunction may be a long-term complication. The present case describes a 23 years old man with growth delay, dyspnoea and long evolution ascites, whose exhaustive etiological investigation led to diagnosis. Afterwards the patient underwent elective surgery with symptom and general condition improvement. Ascites differential diagnosis and its association with constrictive pericarditis are briefly reviewed in this article (AU)

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Ascitic Calprotectin is a Novel and Accurate Marker for Spontaneous Bacterial Peritonitis.

BACKGROUND: Spontaneous bacterial peritonitis (SBP) is a known complication of advanced cirrhosis and presents a high mortality rate. A polymorphonuclear (PMN) cell count >250/µl in the ascitic fluid is the current gold standard for diagnosing SBP. AIM: We evaluated the accuracy of a point-of-care test (POCT) for ascitic calprotectin in diagnosing patients with SBP. METHODS: Eighty-eight patients admitted with decompensation of liver cirrhosis were studied including 41 patients (46.6%) with SBP. Ascitic calprotectin was measured using a quantitative POCT developed by Bühlmann® . RESULTS: Calprotectin levels correlated with PMN cell count and other inflammatory markers and were significantly higher in patients with SBP. An optimal cutoff of calprotectin above 1.57 µg/ml presented high sensitivity (87.8%), specificity (97.9%), and positive (97.3%) and negative (90.2%) predictive values for diagnosing SBP. Using calprotectin selectively in patients with a serum albumin-ascites gradient above 11 g/l further increased the sensitivity and negative predictive values of the test. CONCLUSION: Ascitic calprotectin appears to be a reliable method for diagnosing SBP in patients with liver cirrhosis. It may present an alternative to other conventional diagnostic methods.

Long-evolution ascites in a patient with constrictive pericarditis.

Constrictive pericarditis (CP) is an uncommon disease resulting from chronic pericardial inflammation, fibrosis and calcification. Once there are atypical forms of presentation, with subtle or nonexistent cardiorespiratory symptoms, diagnosis may be challenging and difficult. Recurrent ascites in patients with congestive hepatopathy due to constrictive pericarditis is common and, in most cases, reversible after pericardiectomy. Nevertheless, development of persistent liver dysfunction may be a long-term complication. The present work describes a 23 years old man with growth delay, dyspnoea and long evolution ascites, whose exhaustive etiological investigation led to diagnosis. Afterwards the patient underwent elective surgery with symptom and general condition improvement. Ascites differential diagnosis and its association with constrictive pericarditis are briefly reviewed in this article.

Case of acute hepatitis E with concomitant signs of autoimmunity.

Sporadic cases of acute viral hepatitis E have been described in developed countries, despite the more common occurrence in endemic areas and developing countries. We present the case of a 58 years old Portuguese female, with no epidemiological relevant factors, admitted with acute hepatitis with positive anti-nuclear antibodies, anti-smooth muscle antibody and high serum gamma globulin (> 1.5 fold increase). Serologies for hepatitis A virus, hepatitis B virus, hepatitis C virus, Epstein-Barr virus, cytomegalovirus, hereditary sensory neuropathy and varicella zoster virus were negative. Liver biopsy histology revealed changes compatible with autoimmune hepatitis. Prednisolone and azathioprine was started. She tested positive for immunoglobulin M anti hepatitis E virus (HEV) with detectable viremia by reverse transcription polymerase chain reaction (RT-PCR) technique. HEV-RNA was confirmed through RT-PCR in a liver specimen, establishing the diagnosis of acute hepatitis E. Immunosuppression was stopped. She clinically improved, with resolution of laboratory abnormalities. Therefore, we confirmed acute hepatitis E as the diagnosis. We review the literature to elucidate about HEV infection and its autoimmune effects.

Severe intrahepatic cholestasis, erythrocytosis and hypoglycemia: unusual presenting features of systemic AL amyloidosis.

We report the case of a 68-year-old African woman who presented with jaundice, hepatomegaly and anasarca. Clinical investigation disclosed severe intrahepatic cholestasis, nephrotic syndrome, erythrocytosis and hypoglycemia. Diagnosis of systemic AL amyloidosis was established by percutaneous liver biopsy. Bone marrow biopsy showed 32% of myeloma cells. The patient started treatment with melphalan and prednisolone, but liver function deteriorated and she died in hepatic failure complicated by septic shock three weeks after the diagnosis. We present possible explanations for the unusual clinical and laboratory findings, which required a multidisciplinary approach and posed challenging problems in differential diagnosis and management.