Background-Malignancy, hypercoagulability, and conditions leading to decreased portal flow have been reported to contribute to the aetiology of extrahepatic portal vein thrombosis (EPVT). Mortality of patients with EPVT may be associated with these concurrent medical conditions or with manifestations of portal hypertension, such as variceal haemorrhage. Patients and methods-To determine which variables have prognostic significance with respect to survival, we performed a retrospective study of 172 adult EPVT patients who were followed over the period 1984-1997 in eight university hospitals. Results-Mean follow up was 3.9 years (range 0.1-13.1). Overall survival was 70% (95% confidence interval (CI) 62-76%) at one year, 61% (95% CI, 52-67%) at five years, and 54% (95% CI, 45-62%) at 10 years. The one, five, and 10 year survival rates in the absence of cancer, cirrhosis, and mesenteric vein thrombosis were 95% (95% CI 87-98%), 89% (95% CI 78-94%), and 81% (95% CI 67-89%), respectively (n=83). Variables at diagnosis associated with reduced survival according to multivariate analysis were advanced age, malignancy, cirrhosis, mesenteric vein thrombosis, absence of abdominal inflammation, and serum levels of aminotransferase and albumin. The presence of variceal haemorrhage and myeloproliferative disorders did not influence survival. Only four patients died due to variceal haemorrhage and one due to complications of a portosystemic shunt procedure. Conclusion-We conclude that mortality among patients with EPVT is related primarily to concurrent disorders leading to EPVT and not to complications of portal hypertension. (Gut 2001;49:720-724)

Liver transplantation should be considered for patients with glycogen storage disease who have developed liver malignancy or hepatic failure, and for type IV patients with the classical and progressive hepatic form.

and early adulthood, the symptoms seem to regress and norType III glycogen storage disease (GSD) is a disorder mal adulthood appears possible in most patients. 3 With reof carbohydrate metabolism caused by a deficiency of spect to the liver it has been noticed that the hepatomegaly debranching enzyme. Different subtypes with different gradually resolves concurrent with decreases in elevated clinical pictures have been recognized. During childtransaminase levels. 3 Histologically, besides glycogen storhood and early adulthood, the symptoms generally reage, the development of more or less fibrosis seems to be the gress, and normal adulthood appears possible in most rule. Few reports present data on the development of cirrhopatients without symptoms or signs of cirrhosis. We resis with or without the sequelae of portal hypertension. 3-9 port on an adult patient with GSD who developed endDeath caused by end-stage cirrhosis is unusual. In Japan one stage cirrhosis and a small hepatocellular carcinoma.adult patient has been reported with the combination GSD She had GSD subtype IIIb, i.e., there were no signs of III, cirrhosis, and hepatocellular carcinoma. 9 cardiomyopathy, myopathy, or neuropathy. She under-We report on an adult patient with GSD IIIb who received went a successful transplantation, representing the first a transplant because of end-stage cirrhosis and a small hepacase treated this way for this indication to our knowltocellular carcinoma. To our knowledge, this is the first paedge, and she is doing well after 1 year. Debranching tient to receive a transplant for GSD IIIb cirrhosis. enzyme activity was absent both in the liver and in the leukocytes before transplantation. The debranching en- PATIENTS AND METHODS zyme activity remained absent in the leukocytes after transplantation. We conclude that patients with GSDWe describe the case of a woman with GSD IIIb up to 1 year after orthotopic liver transplantation. sponsible deficient debranching enzyme system was identihomogenates were prepared according to standard laboratory pracfied by Illingworth et al. 2 The debranching enzyme complex tice. A post-concanavalin A fraction from a leukocyte and liver hocomprises two different activities: oligo-1,4-1,4 glucan trans-mogenate was prepared according to the method of Van Diggelen et ferase (EC 2.4.1.25), which transfers three glucose residues al. 10 Limit dextrin was prepared from rabbit liver (type III; Sigma, as a unit from a side chain to the main glycogen chain, and Bornem, Belgium), essentially as described 11 using phosphorylase b amylo-1,6-a glucosidase (EC 3.2.1.33), which hydrolyzes the from rabbit liver (Boehringer Mannheim, Almere, The Netherlands). glucose at the a-1,6 position. 3 Different subtypes with differEnzyme Assay. Debranching enzyme was determined by mixing 25 mL of post-concanavalin A supernatant with 25 mL of potassium ent clinical pictures have been recognized. Approximately buffer (10 mmol/L, pH 6.5) and 50 mL of substrate solution (15 mg 80% of patients have subtype GSD IIIa, and ...

After orthotopic liver transplantation (OLT) bone mass rapidly declines and vertebral fracture rate increases. We studied bone loss and parameters of bone turnover in 53 consecutive patients. In an attempt to reduce bone loss the patients were prophylactically treated with cyclical etidronate in addition to daily 1 alpha-hydroxyvitamin D3 and calcium. During the first 3 months after transplantation median lumbar spinal bone mineral density (BMD) decreased 4.5%; subsequently no significant changes occurred. Median hip BMD continued to fall during the first post-transplantation year and deteriorated 7% over the whole study period. New vertebral fractures were seen in 25% of the patients, which is not lower than previously reported rates in patients not receiving cyclical etidronate. Parathyroid hormone levels increased after OLT (p = 0.01), but remained within normal ranges. Urinary hydroxyproline levels were increased and normalized in the second half-year after OLT. Elevated fasting calciuria increased further after OLT. 1,25-Dihydroxy-vitamin D3 levels were lowered pre-OLT (25 vs 66 pmol/ 1, p< 0.001) and normalized at 3 months after OLT. Serum osteocalcin concentrations remained unchanged and were reduced compared with levels in healthy controls. In summary, increased bone resorption occurs after OLT with persistent decreased bone formation, leading to vertebral fracture in 25% of patients. Etidronate, 1 alpha-calcidol and calcium treatment did not prevent bone loss.

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