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Of 178 patients with sclerosing cholangitis treated since 1950, 88 patients had associated inflammatory bowel disease, 72 had no such history, and 18 had iatrogenic injury or stone disease. A total of 233 biliary operations were performed, with a 75% rate of temporary improvement after initial operation. Subsequent operations resulted in a lower success rate and a higher mortality rate. Radiologic findings included predominant extrahepatic, intrahepatic, and diffuse disease in 29%, 28%, and 43% of patients, respectively; no survival differences were noted. Seventy-five of one hundred three deaths (73%) were related to liver failure, bleeding, or sepsis. Of 14 patients undergoing portosystemic shunt, 13 died of surgical complications or related disease. Orthotopic liver transplantation was performed in 16 patients and resulted in eight deaths, mainly in patients who had previously undergone extensive surgical treatment. No survival differences were seen between the patients with inflammatory bowel disease, those without the condition, or those who had colectomy. Surgical treatment in patients with sclerosing cholangitis should be minimized. Orthotopic liver transplantation should be offered as the treatment of choice for patients with portal hypertension, refractory cholangitis, advanced cirrhosis, or progressive liver failure.
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PMID:Surgical aspects of sclerosing cholangitis. Results in 178 patients. 222 20

A 11-year-old white girl presented with a diagnosis of thrombosis of the portal vein after newborn septicemia. Duplex sonography revealed significant narrowing of the portal vein and its right and left branches. A Doppler signal could only be obtained in certain short segments of the portal vein and indicated hepatopetal flow. Color-coded Doppler sonography showed extensive varicose veins in the gallbladder with a bigger draining vessel running to the porta hepatis. Documentation of varices like those in the gallbladder wall confirms the diagnosis of portal hypertension and may increase the sensitivity of Doppler sonography. Color mapping has the potential to detect unexpected flow and to analyze blood flow to better advantage.
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PMID:Color-coded Doppler evaluation of cholecystic varices in portal hypertension. 228 47

Postoperative treatment after pancreas surgery is concentrated on the function of the exocrine and endocrine part of the gland. While functional disturbances of the endocrine pancreas may give rise to serious problems associated with diabetes, functional disturbances of the exocrine pancreas are less important. On the other hand, flow disorders of the exocrine pancreas may lead to pancreatitis, fistulas, cysts, and abdominal sepsis. Pancreatic tumours are not infrequently apudomas whose biology has an important bearing on the after-treatment. Thrombophlebitic splenomegaly may lead to portal and possibly to segmental portal hypertension. In this event, a careful follow-up examination will be needed to decide whether further surgery is necessary.
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PMID:[After-care following surgery of the pancreas]. 267 64

Renal vein thrombosis in early infancy is a complication of dehydration and prolonged hypotension. The onset is usually acute and the most common clinical signs are uni- or bilateral frank masses, hematuria, proteinuria and thrombocytopenia. In most cases, with conservative management, the late outcome is favorable. In the adult, renal vein thrombosis is often a silent complication of the nephrotic syndrome, the hypercoagulability of which may be an important factor in the pathogenesis of the thrombosis. Clinically, the presentation of a sudden complete occlusion is that of severe abdominal and lumbar pain with hematuria and loss of function of the kidney that suffers hemorrhagic infarction. Physical examination often reveals an enlarged kidney. With gradual occlusion, renal function is preserved. The initial diagnostic approach is with ultrasound studies and computed tomography; definitive diagnosis is established by renal venography or by selective renal arteriography. In general, a conservative approach including the use of anticoagulant treatment is preferred to surgical intervention. Priapism is a persistent painful penile erection due to ischemic or non-ischemic causes; therapeutic intracavernosal injection of papaverine is becoming the most common cause. In early and mild stages, aspiration of blood from the corpora cavernosa supplemented with intracavernosal irrigation with alpha-stimulating agents is the procedure of first choice; in late and severe ischemia, a shunt procedure may become necessary. Hepatic vein thrombosis occurs in association with a number of conditions considered predisposing factors including the use of oral contraceptives. The clinical picture may be that of an acute illness with abdominal pain, hepatomegaly, ascites and hepatic failure as well as early death. More often, the onset is insidious with slowly developing ascites and wasting. For the diagnosis, hepatic scintigraphy may be helpful but, at present, ultrasonography, computed tomography and magnetic resonance scanning are procedures of choice. There is, as yet, no adequate treatment. A fatal outcome may be prevented by surgical decompression of the congested liver and, in recent years, liver transplantation has been employed. Portal vein thrombosis, in children, is usually considered a complication of umbilical sepsis or a result of a congenital abnormality of the portal vein. In adults, the most frequent causes are hepatic cirrhosis and neoplasia. Clinically, there may be a sudden appearance of ascites with resolution in a symptom-free interval until the onset of other features of portal hypertension occur. Currently, ultrasound real-time imaging supplemented with Doppler capability, computed tomography and magnetic resonance scanning provide the necessary diagnostic information. Variceal hemorrhage is often the first major complication requiring treatment.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Thrombosis in particular organ veins. 268 Aug 53

Two neonates underwent staphylococcal septicemia with multiple intrahepatic abscesses, following umbilical catheterization in one case and due to superinfected heel puncture in the other case. At the first examination, liver ultrasonography showed multiple hypoechogenic areas and assessed patency of the portal vein flow. In spite of clinical, biological and sonographic recovery within the following weeks, portal hypertension due to seemingly late portal vein thrombosis occurred. These clinical reports indicate the need for a protracted echosonographic supervision of neonatal intrahepatic abscesses and portal vein patency before asserting complete recovery.
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PMID:[Portal system obstruction of delayed onset following neonatal Staphylococcus aureus infection]. 271 56

Variceal hemorrhage is frequently a lethal event. Mortality among patients who have bled is high, with survival over the short term of only 25% to 50%. We retrospectively reviewed the records of 177 patients in whom variceal bleeding was treated with variceal sclerosis during a 5-year period from 1981 to 1986. All patients were treated by freehand injection of 25% sodium morrhuate with 35% dextrose, 4 ml per injection, through a fiberoptic endoscope. Of this group, 46 patients were treated with sclerosis followed by liver transplantation (group 1). These were compared to 36 nonalcoholic Child's class B and C patients treated with sclerosis alone (group 2). Survival at 4 years was poor in group 2 (17%). Liver failure and continued gastrointestinal bleeding were the most frequent causes of death. Survival among the liver-transplant group was significantly better (73%, p less than 0.001). Causes of death in this group were primarily due to sepsis, often in the setting of acute graft rejection. Group 1 patients were younger (39.8 +/- 10.8 vs 49.8 +/- 16.5 years, p less than 0.01); this difference is influenced by the deliberate selection of younger patients for liver transplantation. We conclude that sclerotherapy followed by liver transplantation significantly improves survival compared to conventional therapy in selected patients with advanced liver disease and portal hypertension. Donor organ availability will seriously limit the applicability of this approach to patients with bleeding esophageal varices.
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PMID:Bleeding esophageal varices: treatment by sclerotherapy and liver transplantation. 305 93

Between 1976 and 1984, 136 patients with portal hypertension due to extrahepatic obstruction were operated on. Twenty two patients had emergency and 114 elective operations. The operative mortality was 9% and 1%, respectively. Altogether 117 patients (86%) were followed up for from two to 10 years: 17 rebled, none developed encephalopathy or sepsis after splenectomy, and 90% and 75% were alive at five and 10 years respectively. Unlike endoscopic sclerotherapy and treatment with propranolol, operative treatment of variceal bleeding can usually be completed during one admission and carries a low mortality and a fairly low morbidity. Operation seems to be the best form of treatment for poor patients living far from medical facilities in developing countries and may be the treatment of choice in developed countries as well.
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PMID:Operations for portal hypertension due to extrahepatic obstruction: results and 10 year follow up. 312 Sep 6

We report the clinical features of 7 men (mean age 22 years, range 7-53 years) with congenital hepatic fibrosis (CHF). Five patients presented with variceal bleeding and/or hepatosplenomegaly due to portal hypertension. Cholangitis was the presenting symptom in the other 2 cases. Diagnosis was established by histological examination of a surgical wedge biopsy (4 patients) or needle biopsy (3 patients). A portal-systemic shunting was performed in 6 patients, three times prophylactically. None of the 5 survivors developed chronic hepatic encephalopathy. Recurrent bouts of cholangitis with septicemia and hepatic abscesses were a major complication in 5 patients with a fatal outcome in 2 cases. Six patients had associated small and large cysts in the cortex of both kidneys, compatible with adult-type polycystic disease. One patient developed terminal renal insufficiency. In 3 patients kidney function remained normal at a mean follow-up time of 7.5 years (range 1-18 years). In 2 families (4 cases) an autosomal dominant inheritance of renal disease was suggested. This study demonstrates that CHF is a rare cause of portal hypertension in late childhood and in adults. Cholangitis is a severe and frequently fatal complication. Association with a variety of congenital renal abnormalities is very frequent. However, the association with adult-type polycystic disease as reported in 4 cases is very rare.
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PMID:Congenital hepatic fibrosis. 341 Nov 2

A retrospective necropsy survey of 13 patients who had received endoscopic injection sclerotherapy was carried out to study tissue changes induced and to determine the causes of death. These results were compared with autopsy findings in nine patients with portal hypertension, comparable for age, sex, and nature and severity of underlying liver disease, who had not received sclerotherapy. Although all treated patients had variceal thrombosis with an associated vasculitis, residual varices were usually present, probably reflecting the brief duration of treatment (median, 12 days). The major complications of sclerotherapy resulted from necrosis, with resultant mucosal ulceration and abscess formation. These features were not present in the control group. Complications contributing to death were hemorrhage in three patients, and in one sepsis with deep necrosis and periesophageal abscess formation.
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PMID:Endoscopic sclerotherapy: lessons from a necropsy study. 348 38

Pancreatic abscess remains the most lethal form of intra-abdominal abscess despite a wide variety of operative approaches that have been advocated for its control. Mortality is frequent, and recurrent abscesses after operative drainage are common. Death often results from ongoing uncontrolled sepsis. The role of percutaneous drainage (PCD) of pancreatic abscesses is controversial. Recent experience with five patients who had pancreatic abscess and in whom a combination of operative drainage and PCD proved instrumental in survival leads the authors to recommend the consideration of both forms of drainage dependent upon the circumstances. Specifically, indications for PCD may include the following: use as a temporizing measure prior to celiotomy in a critically ill patient; use in postoperative patients who have recurrent abscesses and in whom the presence of dense inflammation precludes safe evacuation of pus; and use in the patient who has known portal hypertension and in whom massive bleeding is likely to result from celiotomy and abscess drainage.
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PMID:The role of percutaneous drainage of pancreatic abscesses. 360 66


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