Portal hypertensive gastropathy

There is now substantial clinical evidence to suggest that portal hypertensive gastropathy is an important source of gastrointestinal bleeding in patients with portal hypertension. Although a relatively uncommon presenting feature in such patients, it appears to become progressively more frequent and important the longer such patients with bleeding oesophageal varices survive after treatment by endoscopic sclerotherapy. It is now being increasingly recognized as the most important cause of haemorrhage after oesophageal varices in such patients. The endoscopic and histological characteristics of the condition are now well established but from a clinical point of view it is important to distinguish it from a number of other disorders. The pathogenesis of portal hypertensive gastropathy is poorly understood; venous congestion secondary to portal hypertension undoubtedly plays an important role but this is not thought to account entirely for the condition since abnormalities in the arterial blood supply are also observed. Many abnormalities in gastric mucosal function have been reported but it is unclear whether these are secondary disturbances or whether they play an important primary role in the development of the condition. Animal studies to date have not been helpful due to the lack of a satisfactory experimental model.Portocaval shunt surgery cures portal hypertensive gastropathy but propranolol has been shown to be highly effective in controlling haemorrhage from this condition and should now be considered the treatment of choice. The mechanism of action is unclear, and it remains to be shown whether other R-blockers, or indeed any other drugs, are useful in treating this disorder.     

门静脉高压性胃病

门静脉高压症所致消化道疾病除食管和胃底静脉曲张外,还有非静脉曲张疾病,如消化性溃疡、门静脉高压性胃病(PHG)等.1985年McCormack等[1]总结了127例门静脉高压患者的内镜表现,发现胃黏膜有特征性胃炎样表现,其显微镜下的特征是胃黏膜组织内小血管扩张,而无明显炎症,作者将这种门静脉高压胃黏膜的淤血性改变称为"充血性胃病".     

Portal hypertensive bleeding

Portal hypertension bleeding is a common and serious complication of cirrhosis. All patients with cirrhosis should undergo endoscopy and be evaluated for possible causes of current or future portal hypertensive bleeding. Possible causes of bleeding include esophageal varices, gastric varices, and PHG. Patients with esophageal varices at high risk of bleeding should be treated with nonselective beta-blockers for primary prevention of variceal hemorrhage. HVPG measurements represent the optimal way to monitor the success of pharmacologic therapy. EVL may be used in those with high-risk varices who do not tolerate beta-blockers. When active bleeding develops, simultaneous and coordinated attention must be given to hemodynamic resuscitation, prevention and treatment of complications, and active control of bleeding. In cases of acute esophageal variceal (Fig. 5) and PHG bleeding, terlipressin, somatostatin, or octreotide should be started. Endoscopic treatment is provided for those with bleeding esophageal varices. If first-line therapy fails, TIPS or surgery may need to be performed. Unlike esophageal variceal or PHG bleeding, there is no established optimal treatment for gastric variceal bleeding. Individual and specific treatment modalities for acute gastric variceal bleeding must be calculated carefully after considering side effects.     

门脉高压性胃病

肝硬化门脉高压致上消化道主要的病变是食管 -胃底静脉曲张 ,最严重且死亡率高的是静脉曲张破裂出血 ,此外引起出血的尚有非静脉曲张破裂性疾病 ,其中较常见的为门脉高压性胃病 (ＰｏｒｔａｌＨｙｐｅｒｔｅｎｓｉｖｅＧａｓｔｒｏｐａｔｈｙ ,ＰＨＧ) ,其产生机理是门脉高压致胃粘膜毛细血管、小静脉扩张及粘膜水肿 ,此病只有在胃镜下才能做出诊断。肝硬化门脉高压性胃粘膜病变并非炎症性 ,无糜烂性变化 ,但可有显性或非显性出血。组织学特点为无炎症性或只有轻度炎性细胞浸润 ,具有特征性的镜下所见系粘膜或粘膜下小血管扩张。关于门脉高…     

Portal hypertensive enteropathy

Portal hypertensive enteropathy(PHE) is a condition that describes the pathologic changes and mucosal abnormalities observed in the small intestine of patients with portal hypertension. This entity is being increasingly recognized and better understood over the past decade due to increased accessibility of the small intestine made possible by the introduction of video capsule endoscopy and deep enteroscopy. Though challenged by its diverse endoscopic appearance, multiple scoring systems have been proposed to classify the endoscopic presentationand grade its severity. Endoscopic findings can be broadly categorized into vascular and non-vascular lesions with many subtypes of both categories. Clinical manifestations of PHE can range from asymptomatic incidental findings to fatal gastrointestinal hemorrhage. Classic endoscopic findings in the setting of portal hypertension may lead to a prompt diagnosis. Occasionally histopathology and cross sectional imaging like computed tomography or magnetic resonance imaging may be helpful in establishing a diagnosis. Management of overt bleeding requires multidisciplinary approach involving hepatologists, endoscopists, surgeons, and interventional radiologists. Adequate resuscitation, reduction of portal pressure, and endoscopic therapeutic intervention remain the main principles of the initial treatment. This article reviews the existing evidence on PHE with emphasis on its classification, diagnosis, clinical manifestations, endoscopic appearance, pathological findings, and clinical management. A new schematic management of ectopic variceal bleed is also proposed.     

门脉高压性小肠病

小肠改变是门脉高压门静脉高压症是一组由门静脉压力持续增高引起的症候群,最常见表现是消化道出血,特别是食管胃静脉曲张破裂出血.随着小肠检查手段的发展,特别是胶囊内镜和双气囊小肠镜,发现门脉高压下小肠发生了病变,被定义为门脉高压性小肠病(PHE),这种改变也是消化道出血的重要原因.消化道出血的患者,胃或食管没有静脉曲张情况...     

Portal hypertensive colopathy

The aim of this paper was, first, to show in a case control study that in alcoholic cirrhotic patients colonic vascular ectasias (VE) are a complication of portal hypertension and, second, to establish in a histomorphometric study that colonic vascular ectasias and rectal varices (RV) are only endoscopic features of a new entity: portal hypertensive colopathy, the pathologic basis of which is colonic mucosal capillary ectasia. In the case control study, for each case, three age- and sex-matched controls selected from consecutive patients were used. Sixteen alcoholic cirrhotic patients, 12 men, 4 women (mean age ±sd: 62±10 years) had colonic vascular ectasias. The prevalence of esophageal varices (88% vs 44%,P<0.005), esophageal varices (5 mm) (44% vs 12.5%,P<0.01), previous history of bleeding from esophageal varices (44% vs 8%,P<0.005), and rectal varices (63% vs 6%,P<0.001) was significantly greater in cases with colonic vascular ectasias than in controls without colonic vascular ectasias. The relative risk of colonic vascular ectasias in alcoholic cirrhotic patients with esophageal varices versus cirrhotic patients without esophageal varices was 14.4 (95% confidence interval 2.8–75.3). In the histomorphometric study, cirrhotic patients with vascular ectasias and/or rectal varices had a significantly higher mean diameter of vessels (20.3±1.5 m vs 18.7±1.6 m,P<0.05) and a higher mean cross-sectional vascular area (2143±396 m² vs 1676±345 m²,P<0.05) than cirrhotic patients without vascular ectasias and/or rectal varices. These results suggest that colonic vascular ectasias seem to be a complication of portal hypertension and that colonic vascular ectasias and rectal varices are endoscopic features of a new entity the portal hypertensive colopathy.     

Portal hypertensive gastropathy: a review

Portal hypertensive gastropathy (PHG) occurs as a complication of cirrhotic or non‐cirrhotic portal hypertension. Although the pathogenesis of PHG is not completely understood, evidence suggests that the key factor for the development of PHG is portal hypertension. PHG is clinically important because it may cause acute (and even) massive or insidious, blood loss. The diagnosis of PHG is (only) made endoscopically; it is most often characterized by an abnormality of the gastric mucosa described as a mosaic‐like pattern resembling ‘snake‐skin’, with or without red spots and the endoscopic pattern is key its diagnosis. Unfortunately, standardization of the endoscopic diagnostic criteria for PHG is poor and consensus is generally lacking, resulting in a wide range of reported prevalence. Pharmacological therapies, presumably reducing portal pressure and gastric blood flow, have been used to treat acute bleeding; propanolol, a non‐selective β‐blocker (24–480 mg/day), has been used most frequently. Endoscopic treatment for PHG bleeding plays a small, if any, role in the treatment of PHG. TIPS and shunt surgery have not been extensively analysed as a treatment for acute or chronic PHG bleeding, but they appear to lessen the severity of PHG. Secondary prophylaxis of PHG bleeding with non‐selective β‐blockers is recommended. There is not enough evidence to support the use of β‐blockers in primary prophylaxis of PHG bleeding, even in cases of severe PHG (however, non‐selective β‐blockers are recommended if varices are present). Further studies are needed to clarify the role of PHG in suspected chronic gastrointestinal bleeding.     

Immunoglobulin D amyloidosis: a distinct entity

IgD monoclonal gammopathies are uncommon. They are seen rarely as a monoclonal gammopathy of undetermined significance and are present in 1%-2% of patients with multiple myeloma. In light-chain amyloidosis, IgD monoclonal proteins are found in ap-proximately 1% of patients. When an IgD monoclonal protein is found, amyloidosis is often omitted from the differential diagnosis. In the present study, we reviewed the natural history of IgD-associated amyloidosis among 53 patients seen over 41 years. The distribution of clinical syndromes suggests that these patients have a lower frequency of renal and cardiac involvement. The overall survival of these patients does not appear to be different from that of patients who have light-chain amyloidosis associated with another monoclonal protein.     

4 Portal hypertensive gastropathy

The term portal hypertensive gastropathy (PHG) defines a wide spectrum of diffuse macroscopic lesions that appear in the gastric mucosa of patients with portal hypertension. Histologically, these lesions correspond to dilated vessels in the mucosa and submucosa in the absence of erosions or inflammation. Endoscopically, the lesions are classified as mild when mosaic pattern or superficial reddening are present, and severe when gastric mucosa appear with diffuse cherry red spots. Mild lesions are highly prevalent (65–90%), whereas severe lesions are present in only 10–25% of cirrhotic patients.The pathogenesis of PHG is not well known, but both venous congestion related with raised portal pressure and increased gastric blood flow seem to be crucial factors for its development. Variceal sclerosis may contribute to the development or aggravation of the lesions.Bleeding is the unique clinical manifestation of PHG, and occurs only in those patients with severe lesions. During a 5-year follow-up, the risk of overt bleeding or chronic bleeding, which induces anaemia, is 60% and 90%, respectively, for patients with severe PHG.Propranolol is the only pharmacological treatment that has been proven useful in preventing bleeding from PHG. Porto-systemic shunts and liver transplantation are also effective.     

Eosinophilic fasciitis: a distinct clinical entity?

Eosinophilic Fasciitis is a syndrome characterized by exertion related scleroderma-like skin changes, peripheral eosinophilia, hypergammaglobulinemia and diffuse faciitis. Controversy exists as to the precise classification of the syndrome, i.e., whether it is a distinct entity or a variant of scleroderma. We describe a patient with eosinophilic faciitis but with several unique features: 1) progressive skin changes unresponsive to corticosteroid therapy; 2) elevated anti-DNA antibodies; 3) hypocomplementemia; and 4) a followup biopsy showing sclerodermatoid skin changes. These features and others relating to the controversial aspects of classification of eosinophilic fasciitis are discussed.     

Primary pulmonary Hodgkin's disease: a distinct entity

A 30-year-old lady presented with fever, dry cough and weight loss for the preceding five months. Radiological investigations revealed a solitary nodular lesion in the lingula of the left lung. Guided fine needle aspiration cytology failed to yield any diagnostic material. Bronchoscopic cytology was also not contributory. As a last resort open lung biopsy was done and a diagnosis of Hodgkin's disease was made. Hilar and pre aortic lymph node biopsies showed only reactive change. The final diagnosis was primary pulmonary Hodgkin's disease.