SUMMARY Bleeding of esophageal varices have a high mortality rate, and medical management is usualy unsuccessful. Surgical methods such as vascular ligation or shunting are expensive. High risk procedures with high mortality. Endoscopic sclerotherapy was applied in 104 patients with acute bleeding of esophageal varices during a period of 5 years in a prospettive and retrospective study. Th...

case presentation we herein describe a case of a 7 year-old child affected by an overlap syndrome between type 2 autoimmune hepatitis and small duct primary sclerosing cholangitis. although characterized by a severe onset, the disease showed a good response to treatment with prednisone and azathioprine. conclusions the association of type 2 autoimmune hepatitis and small duct primary cholangiti...

Langerhans' cell histiocytosis (LCH) of the liver is uncommon. When seen, it is part of multifocal disease and can present as biliary obstruction. We present a case of sclerosing biliary disease with a solitary LCH lesion and no evidence of systemic disease. We postulate that the LCH is a secondary phenomenon, arising against a background of a complex, familial liver disease. This case also rai...

Immunoglobulin G4-related sclerosing disease is a recently recognized disease entity most commonly associated with autoimmune pancreatitis. This condition can also manifest as extra-pancreatic disease involving the bile ducts, kidney, lung, and retroperitoneum. The disease entity consists of elevated serum IgG4 levels, extensive IgG4-positive plasma cells, and lymphocyte infiltration of the aff...

Three Chinese patients, one female aged 63 years, two males aged 52 and 54 years, respectively, all presented with chronic sclerosing sialadenitis (CSS) as the initial clinical manifestation, followed by IgG4-related sclerosing pancreatitis. The kidneys and lungs were involved in case 1. Moreover, enlargement of hilar lymph nodes was also noted in this patient. After steroid therapy, the clinic...

IgG4-related systemic sclerosing disease (IRSSD) is a new clinical entity that is characterized by elevated levels of serum IgG4; plasma cell infiltrates in tissues, including the pancreas, lung, liver, kidney and breast; and a good response to corticosteroid therapy. We report a case of a patient with sclerosing cholangitis and pulmonary inflammatory pseudotumors. The diagnosis was made twelve...

This study reports on the outcome of four patients having ileal pouch anal anastomosis after orthotopic liver transplantation for sclerosing cholangitis complicating ulcerative colitis. There were no deaths on follow up. Early postoperative bleeding was a complication of colectomy or rectal excision in three, one of whom developed hepatic artery thrombosis and the overall complication rate was ...

We report 5 cases of sclerosing rhabdomyosarcoma. The patients included 4 adults and 1 adolescent. In the 5 cases, 3 tumors occurred in the head and neck region and 2 in an extremity. Histologically, all 5 tumors were characterized by the presence of abundant extracellular hyaline matrix, mimicking osteoid or chondroid tissue. They were composed mostly of primitive small round cells that displa...

Sclerosing stromal tumours of the ovary have recently been described as a histologically and clinically distinct subgroup within the thecoma-fibroma spectrum of benign ovarian sex cord stromal tumours. Reported cases occurred predominantly in young women and only occasional tumours showed evidence of hormonal activity. The present series of five cases expands the spectra of both histological pa...