Primary amyloidosis and severe intrahepatic cholestatic jaundice.

Peters, Richard; Koukoulis, George Κ.; Gimson, A.; Portmann, Bernard; Westaby, David

doi:10.1136/gut.35.9.1322

Cited by 70 publications

(44 citation statements)

References 24 publications

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“…It is most commonly due to intrahepatic cholestasis26 although obstruction of the bile ducts by amyloid deposits has also been reported 27. Only four of our patients with AL disease died of hepatic insufficiency, and none of the AA patients died.…”

Section: Discussionmentioning

confidence: 51%

The liver in systemic amyloidosis: insights from ¹²³I serum amyloid P component scintigraphy in 484 patients

Lovat

Persey

Madhoo

et al. 1998

Gut

109

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Background and aims-The liver is frequently involved in amyloidosis but the significance of hepatic amyloid has not been systematically studied. We have previously developed scintigraphy with 123 I serum amyloid P component ( 123 I-SAP) to identify and monitor amyloid deposits quantitatively in vivo and we report here our findings in hepatic amyloidosis. Methods-Between 1988 and 1995, 805 patients with clinically suspected or biopsy proven systemic amyloidosis were evaluated. One hundred and thirty eight patients had AA amyloidosis, 180 had AL amyloidosis, 99 had hereditary amyloid syndromes, and 67 had dialysis related ( 2 microglobulin) amyloid. One hundred and ninety two patients with amyloidosis were followed for six months to eight years. Results-Hepatic amyloid was found in 98/180 (54%) AL and 25/138 (18%) AA patients but in only 1/53 patients with familial transthyretin amyloid polyneuropathy and in none with dialysis related amyloidosis. There was complete concordance between hepatic SAP scintigraphy and the presence or absence of parenchymal amyloid deposits on liver histology. Amyloidosis was never confined to the liver. Mortality was rarely due to hepatic failure, although hepatic involvement with AA amyloid carried a poor prognosis. Successful therapy to reduce the supply of amyloid fibril protein precursors was followed by substantial regression of all types of amyloid. Conclusions-SAP scintigraphy is a specific and sensitive method for detecting and monitoring hepatic amyloid. Liver involvement is always associated with major amyloid in other organ systems and carries a poor prognosis in AA type. Appropriate therapy may substantially improve prognosis in many patients. (Gut 1998;42:727-734)

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Section: Discussionmentioning

confidence: 51%

The liver in systemic amyloidosis: insights from ¹²³I serum amyloid P component scintigraphy in 484 patients

Lovat

Persey

Madhoo

et al. 1998

Gut

109

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“…Ascites may develop in approximately 10-20% of patients with primary systemic (AL) amyloidosis [11,12]. Ascites in patients with AL amyloid has been attributed to congestive heart failure, nephrotic syndrome, or portal hypertension from advanced hepatocellular disease.…”

Section: Discussionmentioning

confidence: 99%

Familial amyloid with a transthyretin leucine 33 mutation presenting with ascites

1998

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A 65-year-old female presented with symptomatic ascites. Light and electron microscopy examination of omental and peritoneal tissue obtained at exploratory laparotomy revealed amyloidosis. Immunochemical studies of the amyloid tissue showed positive staining with antibodies to transthyretin. Polymerase chain reaction (PCR), single strand conformation polymorphism analysis, and direct DNA sequencing demonstrated a transthyretin phenylalanine to leucine substitution at codon 33. This is only the second reported case of a transthyretin leucine 33 mutation. Moreover, this patient is unique among cases of transthyretin-associated amyloidosis with the clinical presentation of ascites. Am.

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“…Cholestasis is encountered in 5% of cases during the course of disease and elevation in liver enzymes especially alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT) is detected. 2,6 Large quantities of amyloid are deposited between sinusoidal wall in the space of Disse and columns of liver cells interfere with bile and blood flow giving the appearance of cholestatic jaundice, which occurred in our case. In whom had major increase in ALP and GGT but bilirubin level was slightly elevated.…”

Section: Discussionmentioning

confidence: 91%

“…Cholestatic jaundice is an infrequent manifestation of amyloidosis and found in only approximately 5% of cases of amyloidosis. 2,3 Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic premalignant clonal plasma cell or lymphoplasmacytic proliferative disorder. MGUS related amyloid is very rare.…”

Section: Introductionmentioning

confidence: 99%

Primary amyloidosis presenting intrahepatic cholestasis and fulminant hepatic failure

et al. 2015

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A 69-year-old man noticed abdominal pain located on right upper quadrant. Physical examination showed hepatosplenomegaly and icteric discoloration of sclera. On evaluation, patient was diagnosed to have hepatic amyloidosis related monoclonal gammopathy of undetermined significance (MGUS) and sinusoidal obstruction syndrome with intrahepatic cholestasis. In this case report we emphasize fulminant hepatic failure due to primer amyloidosis in diagnosed with MGUS patient.

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Primary amyloidosis and severe intrahepatic cholestatic jaundice.

Cited by 70 publications

References 24 publications

The liver in systemic amyloidosis: insights from ¹²³I serum amyloid P component scintigraphy in 484 patients

The liver in systemic amyloidosis: insights from ¹²³I serum amyloid P component scintigraphy in 484 patients

Familial amyloid with a transthyretin leucine 33 mutation presenting with ascites

Primary amyloidosis presenting intrahepatic cholestasis and fulminant hepatic failure

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Primary amyloidosis and severe intrahepatic cholestatic jaundice.

Cited by 70 publications

References 24 publications

The liver in systemic amyloidosis: insights from 123I serum amyloid P component scintigraphy in 484 patients

The liver in systemic amyloidosis: insights from 123I serum amyloid P component scintigraphy in 484 patients

Familial amyloid with a transthyretin leucine 33 mutation presenting with ascites

Primary amyloidosis presenting intrahepatic cholestasis and fulminant hepatic failure

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The liver in systemic amyloidosis: insights from ¹²³I serum amyloid P component scintigraphy in 484 patients

The liver in systemic amyloidosis: insights from ¹²³I serum amyloid P component scintigraphy in 484 patients