Document Detail

Severe intrahepatic cholestasis, erythrocytosis and hypoglycemia: unusual presenting features of systemic AL amyloidosis.
MedLine Citation:
PMID:  18938770     Owner:  NLM     Status:  MEDLINE    
We report the case of a 68-year-old African woman who presented with jaundice, hepatomegaly and anasarca. Clinical investigation disclosed severe intrahepatic cholestasis, nephrotic syndrome, erythrocytosis and hypoglycemia. Diagnosis of systemic AL amyloidosis was established by percutaneous liver biopsy. Bone marrow biopsy showed 32% of myeloma cells. The patient started treatment with melphalan and prednisolone, but liver function deteriorated and she died in hepatic failure complicated by septic shock three weeks after the diagnosis. We present possible explanations for the unusual clinical and laboratory findings, which required a multidisciplinary approach and posed challenging problems in differential diagnosis and management.
Sara Ferreira; Cilénia Baldaia; Narcisa Fatela; Fernando Ramalho; Maria Adília Costa; Delfina Brito; Graça Esteves; Estela Monteiro
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Scandinavian journal of gastroenterology     Volume:  43     ISSN:  0036-5521     ISO Abbreviation:  Scand. J. Gastroenterol.     Publication Date:  2008 Mar 
Date Detail:
Created Date:  2008-10-20     Completed Date:  2008-12-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0060105     Medline TA:  Scand J Gastroenterol     Country:  Norway    
Other Details:
Languages:  eng     Pagination:  375-9     Citation Subset:  IM    
Department of Gastroenterology, Liver Unit, Hospital de Santa Maria, E.P.E. Avenida Professor Egas Moniz, Lisbon, Portugal.
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MeSH Terms
Amyloidosis / complications*,  diagnosis
Cholestasis, Intrahepatic / diagnosis,  etiology*
Diagnosis, Differential
Erythrocyte Count
Fatal Outcome
Hypoglycemia / diagnosis,  etiology*
Polycythemia / diagnosis,  etiology*
Severity of Illness Index
Tomography, X-Ray Computed

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