RESUMO
In this study, we aimed to review retrospectively the records of 5 patients who were treated in our hospital and to review the current approaches in diagnosis and management of autoimmune pancreatitis [AIP]. The series of patients diagnosed with AIP during the last seven years [January 2006 - August 2012] was the basis of this study. All records were retrieved and analyzed. The diagnosis of AIP was established on the basis of imaging studies, serology, cytology and response to treatment. Five patients were diagnosed with AIP pancreatitis during this 7-years period. Four of the 5 patients were males. The most common presenting symptom was abdominal pain [4/5]. Two patients with the preliminary diagnosis of pancreatic mass underwent surgery. Histological analysis of the surgical resection did not reveal any malignancy. During the follow-up, one of them has developed Crohn's disease and Sjogren syndrome. One of the patients had obstructive jaundice and abdominal pain for several months. Abdominal contrast enhanced computed tomography [CECT] suggested the diagnosis of AIP, cholangitis with renal atrophy and retroperitoneal fibrosis. He was started on steroids to which he responded dramatically. One patient had been diagnosed as primary sclerosing cholangitis few months earlier on the basis of abdominal CECT features showing a dominant stricture in the common bile duct. During the follow-up, the diagnosis of AIP was suspected and finally established on the basis of repeated magnetic resonance imaging [MRI]. The last patient had history of acute pancreatitis, obstructive jaundice and abdominal pain for 3 months. An abdominal CECT suggested autoimmune pancreatitis which was confirmed by MRCP. He was started later on steroids to which he responded significantly. IgG4 was done in all cases, high in four patients. AIP is a disease with increasing incidence and characterized by lymphoplasmacytic cells infiltration and fibrosis. It is necessary to evaluate patients in terms of AIP serologically to avoid wrong diagnosis and the morbidity of surgery
RESUMO
The Budd-Chiari syndrome is a rare disease, often fatal if not treated optimally. It is characterized by a blocked hepatic venous outflow tract. This review attempted to present pathophysiology, aetiologies,diagnosis and therapeutic modalities of the Budd-Chiari syndrome. Review of literature. Budd-Chiari syndrome is a complex disease with a wide spectrum of aetiologies and presentations. Hematologic abnormalities, particularly myeloproliferative disorders, are the most common causes of the Budd-Chiari syndrome. The clinical presentation is governed by the extent and rapidity of the hepatic vein occlusion. Doppler-ultrasound, computed tomography or magnetic resonance imaging of hepatic veins and inferior vena cava are usually successful in demonstrating non-invasively the obstacle or its consequences. A therapeutic strategy has been proposed where anticoagulation, correction of risk factors, diuretics and prophylaxis for portal hypertension are used first; then angioplasty for shortlength venous stenosis; then Transjugular Intrahepatic Portosystemic Shunt [TIPS]; and ultimately liver transplantation. Treatment progression is dictated by the response to previous therapy. This strategy has achieved 5-year survival rates approaching 70%.Medium-term prognosis depends on the severity of liver disease. The diagnosis of the Budd-Chiari syndrome must be considered in any patients with acute or chronic liver disease. Management of this syndrome should follow a step by step strategy