Primary systemic amyloidosis with giant hepatomegaly and a swiftly progressive course (137 visite)
J Clin Gastroenterol (ISSN: 0192-0790), 1997 Apr; 24(3): 173-175.
Tipo di articolo: Journal Article,
Impact factor: 0.936, Impact factor a 5 anni: 2.61
Url: http://www.scopus.com/inward/record.url?eid=2-s2.0-0030958389&partnerID=40&md5=dbb52df05e1834fcc9ff67a5981a6314
Parole chiave: Amyloidosis, Cholestasis, Hepatomegaly, Portal Hypertension, Alkaline Phosphatase, Aminotransferase, Adult, Aminotransferase Blood Level, Article, Ascites, Case Report, Esophagus Varices, Female, Human, Human Tissue, Priority Journal, Biopsy, Intrahepatic, Fatal Outcome, Liver, Liver Function Tests, Middle Aged,
Affiliazioni:
Nuclear Medicine Department, University Federico II, Naples, Italy
Gastroenterology Unit, University Federico II, Naples, Italy
Corso Vittorio Emanuele, 670, 80122 Napoli, Italy
Riferimenti:
Gertz, M.A., Kyle, R.A., Hepatic amyloidosis (primary [AL] immunoglobulin light chain): The natural history in 80 patients (1988) Am J Med, 85, pp. 73-8
Kyle, R.A., Gertz, M.A., Primary systemic amyloidosis: Clinical and laboratory features in 474 cases (1995) Semin Hematol, 32, pp. 45-59
Brandt, K., Cathacart, E.S., Cohen, A.S., A clinical analysis of the course and prognosis of forty-two patients with amyloidosis (1968) Am J Med, 44, pp. 955-969
Kyle, R.A., Greipp, P.R., Amyloidosis (AL): Clinical and laboratory features in 229 cases (1983) Mayo Clin Proc, 58, pp. 665-683
Merlini, G., Treatment of primary amyloidosis (1995) Semin Hematol, 32, pp. 60-79
Serra, L., Poppi, M.C., Criscuolo, M., Zandomenighi, R., Primary systemic amyloidosis with giant hepatomegaly and portal hypertension: A case report and a review of the literature (1993) Ital J Gastroenterol, 25, pp. 435-438
Bion, E., Brenard, R., Pariente, E.A., Sinusoidal portal hypertension in hepatic amyloidosis (1991) Gut, 32, p. 227
Rubinow, A., Koff, R.S., Cohen, A.S., Severe intrahepatic cholestasis in primary amyloidosis: A report of four cases and a review of the literature (1978) Am J Med, 64, pp. 937-946
Levy, M., Fryd, C.H., Eliakim, M., Intrahepatic obstructive jaundice due to amyloidosis of the liver: A case report and a review of the literature (1971) Gastroenterology, 61, pp. 234-238
Itescu, S., Hepatic amyloidosis: An unusual cause of ascites and portal hypertension (1984) Arch Intern Med, 144, pp. 2257-2259
Menke, D.M., Kyle, R.A., Fleming, C.R., Wolfe, J.T., Kurtin, P.J., Oldenburg, W.A., Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis (1993) Mayo Clin Proc, 68, pp. 763-767
Gertz, M. A., Kyle, R. A., Hepatic amyloidosis (primary [AL] immunoglobulin light chain): The natural history in 80 patients (1988) Am J Med, 85, pp. 73-8
Kyle, R. A., Gertz, M. A., Primary systemic amyloidosis: Clinical and laboratory features in 474 cases (1995) Semin Hematol, 32, pp. 45-59
Kyle, R. A., Greipp, P. R., Amyloidosis (AL): Clinical and laboratory features in 229 cases (1983) Mayo Clin Proc, 58, pp. 665-683
Levy, M., Fryd, C. H., Eliakim, M., Intrahepatic obstructive jaundice due to amyloidosis of the liver: A case report and a review of the literature (1971) Gastroenterology, 61, pp. 234-238
Menke, D. M., Kyle, R. A., Fleming, C. R., Wolfe, J. T., Kurtin, P. J., Oldenburg, W. A., Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis (1993) Mayo Clin Proc, 68, pp. 763-767
J Clin Gastroenterol (ISSN: 0192-0790), 1997 Apr; 24(3): 173-175.
Tipo di articolo: Journal Article,
Impact factor: 0.936, Impact factor a 5 anni: 2.61
Url: http://www.scopus.com/inward/record.url?eid=2-s2.0-0030958389&partnerID=40&md5=dbb52df05e1834fcc9ff67a5981a6314
Parole chiave: Amyloidosis, Cholestasis, Hepatomegaly, Portal Hypertension, Alkaline Phosphatase, Aminotransferase, Adult, Aminotransferase Blood Level, Article, Ascites, Case Report, Esophagus Varices, Female, Human, Human Tissue, Priority Journal, Biopsy, Intrahepatic, Fatal Outcome, Liver, Liver Function Tests, Middle Aged,
Affiliazioni:
Nuclear Medicine Department, University Federico II, Naples, Italy
Gastroenterology Unit, University Federico II, Naples, Italy
Corso Vittorio Emanuele, 670, 80122 Napoli, Italy
Riferimenti:
Gertz, M.A., Kyle, R.A., Hepatic amyloidosis (primary [AL] immunoglobulin light chain): The natural history in 80 patients (1988) Am J Med, 85, pp. 73-8
Kyle, R.A., Gertz, M.A., Primary systemic amyloidosis: Clinical and laboratory features in 474 cases (1995) Semin Hematol, 32, pp. 45-59
Brandt, K., Cathacart, E.S., Cohen, A.S., A clinical analysis of the course and prognosis of forty-two patients with amyloidosis (1968) Am J Med, 44, pp. 955-969
Kyle, R.A., Greipp, P.R., Amyloidosis (AL): Clinical and laboratory features in 229 cases (1983) Mayo Clin Proc, 58, pp. 665-683
Merlini, G., Treatment of primary amyloidosis (1995) Semin Hematol, 32, pp. 60-79
Serra, L., Poppi, M.C., Criscuolo, M., Zandomenighi, R., Primary systemic amyloidosis with giant hepatomegaly and portal hypertension: A case report and a review of the literature (1993) Ital J Gastroenterol, 25, pp. 435-438
Bion, E., Brenard, R., Pariente, E.A., Sinusoidal portal hypertension in hepatic amyloidosis (1991) Gut, 32, p. 227
Rubinow, A., Koff, R.S., Cohen, A.S., Severe intrahepatic cholestasis in primary amyloidosis: A report of four cases and a review of the literature (1978) Am J Med, 64, pp. 937-946
Levy, M., Fryd, C.H., Eliakim, M., Intrahepatic obstructive jaundice due to amyloidosis of the liver: A case report and a review of the literature (1971) Gastroenterology, 61, pp. 234-238
Itescu, S., Hepatic amyloidosis: An unusual cause of ascites and portal hypertension (1984) Arch Intern Med, 144, pp. 2257-2259
Menke, D.M., Kyle, R.A., Fleming, C.R., Wolfe, J.T., Kurtin, P.J., Oldenburg, W.A., Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis (1993) Mayo Clin Proc, 68, pp. 763-767
Gertz, M. A., Kyle, R. A., Hepatic amyloidosis (primary [AL] immunoglobulin light chain): The natural history in 80 patients (1988) Am J Med, 85, pp. 73-8
Kyle, R. A., Gertz, M. A., Primary systemic amyloidosis: Clinical and laboratory features in 474 cases (1995) Semin Hematol, 32, pp. 45-59
Kyle, R. A., Greipp, P. R., Amyloidosis (AL): Clinical and laboratory features in 229 cases (1983) Mayo Clin Proc, 58, pp. 665-683
Levy, M., Fryd, C. H., Eliakim, M., Intrahepatic obstructive jaundice due to amyloidosis of the liver: A case report and a review of the literature (1971) Gastroenterology, 61, pp. 234-238
Menke, D. M., Kyle, R. A., Fleming, C. R., Wolfe, J. T., Kurtin, P. J., Oldenburg, W. A., Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis (1993) Mayo Clin Proc, 68, pp. 763-767
Although the involvement of the liver is common in systemic amyloidosis (AL), clinical features of hepatic dysfunction and liver chemistry abnormalities are often mild or absent. A mild increase in the serum alkaline phosphatase value is the most common finding. Hypertransaminasemia, hyperbilirubinemia, and portal hypertension with ascites and gastroesophageal varices occur late in the course of the disease and predict a short survival. We describe the case of a 58-year-old woman with AL, whose dramatic and unusual clinical picture, consisting of giant hepatomegaly, hypertransaminasemia, increase in alkaline phosphatase, esophageal varices, and ascites, was rapidly complicated by severe obstructive cholestasis.
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