Portal hypertension and ascites in systemic mastocytosis

We report a case of systemic mastocytosis (SM) presenting as ascites and portal hypertension. The haematological picture at presentation was suggestive of chronic myelomonocytic leukaemia. Initial difficulties in making a diagnosis of SM were encountered as the cutaneous signs were atypical. The correct diagnosis was established only after tissue sections were appropriately stained for mast cells. The liver biopsy showed portal and sinusoidal mast cell infiltration, portal fibrosis and evidence of hepatic venous outflow obstruction. The disease progressed rapidly and recurrent massive ascites was a dominant problem. This case illustrates again the problems of making a diagnosis of SM especially when the clinical picture is atypical. Ascites as a presenting manifestation of SM has been reported previously in only six patients. Published cases of SM with portal hypertension or ascites or both are reviewed.     

Portal hypertension secondary to splenic arteriovenous fistula

Portal hypertension is a common condition in association with chronic liver disease; however, it is rarely caused by vascular shunting from splenic arteriovenous fistula (SAVF). We are presenting a case of non-cirrhotic portal hypertension due to SAVF. This condition is usually seen in multiparity women; however, other causes like trauma or mycotic infection are reported in the literatures with equal gender prevalence. Most of the patients present with gastric and esophageal varices, splenomegaly or upper abdominal pain. Clinical history and examination are the first step for diagnosis followed by ultrasound or contrast enhanced computer tomography. Splenic arteriovenous fistula must be then confirmed by selective celiac or splenic arteriography which is the gold standard tool. The condition is treatable either surgical ligation or intra-arterial embolization. Due to less invasive procedure, the later one is more favorable in unstable patients. Both procedures have been reported to be equally successful in managing SAVF.     

Portal hypertension secondary to azathioprine in myasthenia gravis

A 52 year old man with myasthenia gravis and normal liver function was treated with neostigmine, prednisolone and azathioprine. Three years after starting azathioprine he developed clinical evidence of portal hypertension. A liver biopsy showed nodular regenerative hyperplasia (NRH). The development of NRH following azathioprine treatment in a patient with myasthenia gravis strengthens the case for a causal role of azathioprine in producing NRH and portal hypertension.     

Portal hypertension. Update on pathophysiology and management

A lot has been learned about the pathophysiology of portal hypertension and its complications. This knowledge has led to vast advancements in therapy for this serious disease. I believe the future will carry more breakthroughs in therapy intercepting earlier steps in the disease process such as arresting fibrogenesis, inducing fibrinolysis or possibly targeting vascular remodeling and neovessel formation or employing hepatocytes transplantation.     

门奇断流术和肠腔侧侧分流术治疗门脉高压症的探讨

目的:探讨门奇断流术和肠腔侧侧分流术治疗门脉高压症的疗效。方法;采用先进的实验方法,吲哚氰绿负荷实验对门脉高压症患者分别行门奇断流术和肠腔侧侧分流术,进行手术前、后肝血流量、KICG、FPP、胃壁压的测定。结果:门奇断流手术前、后肝血流量、KICG、FPP无明显变化,胃壁压手术后较手术前有所增高,平均增高8.7％;肠腔侧侧分流手术前、后肝血流量、KIOG均有不同程度的降低,平均降低17％。结论:门奇断流术对肝血流量和肝机能无明显影响;肠腔侧侧分流术对肝血流量和肝机能有一定影响,但影响并不很大。     

Portal hypertension complicating myelofibrosis: reversal following splenectomy.

Portal hypertension occurs in approximately 10% of patients with myelofibrosis. Increased portal blood flow secondary to splenomegaly has been proposed to explain its development. In a 60-year-old woman with proven myelofibrosis of 10 years' duration and gross splenomegaly, portal hypertension developed with esophageal varices and ascites. There was no demonstrable obstruction to portal blood flow. Following splenectomy the ascites and esophageal varices disappeared. Despite the presence of splenic myeloid metaplasia, splenectomy did not impair the patient's hematologic status. Portal hypertension complicating myelofibrosis has a poor prognosis, so careful attention should be given to its detection. Splenectomy may be preferable to portal-systemic shunting in the management of this complication.     

Portal hypertension in a patient with chronic myeloid leukaemia.

A patient with chronic myeloid leukaemia treated with busulphan for 4-5 years, developed signs of busulphan toxicity and portal hypertension with ascites, oesophageal varices and jaundice. At post-mortem there was minimal leukaemic infiltration but there were alterations in the liver architecture sufficient to explain the portal hypertension. The pathogenesis of the liver changes and their possible relationship to splenomegaly and busulphan toxicity are considered.     

Portal hemodynamics in cirrhotics with portal hypertension using color Doppler velocity profile

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