Zieve's syndrome
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| name = Zieve's syndrome
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Zieve's syndrome is an acute metabolic condition that can occur during withdrawal from prolonged heavy alcohol use. It is defined by hemolytic anemia (with spur cells and acanthocytes), hyperlipoproteinemia (excessive blood lipoprotein), jaundice (elevation of unconjugated bilirubin), and abdominal pain.{{cite book | last=Mehta | first=AB |author2=N McIntyre | title=Oxford Textbook of Clinical Hepatology | publisher=Oxford University Press | year=2004 | pages=1786–1787 | isbn=0-19-262515-2 }} The underlying cause is liver delipidization. This is distinct from alcoholic hepatitis which, however, may present simultaneously or develop later.{{cn|date=March 2022}}
Diagnosis of Zieve's syndrome should be considered in patients with prolonged alcohol use (especially after an episode of binge drinking) with an elevation of unconjugated bilirubin and without obvious signs of gastrointestinal bleeding.{{Cite journal|last1=Shukla|first1=Sandhya|last2=Sitrin|first2=Michael|date=2015-07-09|title=Hemolysis in Acute Alcoholic Hepatitis: Zieve's Syndrome|journal=ACG Case Reports Journal|volume=2|issue=4|pages=250–251|doi=10.14309/crj.2015.75|issn=2326-3253|pmc=4508957|pmid=26203455}}
Pathogenesis
The proposed mechanism of the characteristic haemolytic anemia in Zieve's syndrome is due to alteration of the red cell metabolism, namely pyruvate kinase instability leaving them susceptible to circulating hemolysin such as lysolecithin.{{Cite journal|last1=Melrose|first1=W. D.|last2=Bell|first2=P. A.|last3=Jupe|first3=D. M.|last4=Baikie|first4=M. J.|date=1990-01-01|title=Alcohol-associated haemolysis in Zieve's syndrome: a clinical and laboratory study of five cases|journal=Clinical and Laboratory Haematology|volume=12|issue=2|pages=159–167|doi=10.1111/ijlh.1990.12.2.159 |issn=0141-9854|pmid=2208946}} Changes in membrane lipid compositions such as increased cholesterol and polyunsaturated fatty acid (PUFA) have been reported during the hemolytic phase.{{Cite journal|last1=Kunz|first1=F.|last2=Stummvoll|first2=W.|date=1970-10-01|title=The significance of plasma phospholipids in Zieve syndrome|journal=Blut|volume=21|issue=4|pages=210–226|doi=10.1007/BF01632304 |issn=0006-5242|pmid=5531666}}
Diagnosis
The diagnosis is demonstrated by the triad of alcoholic hepatitis or cirrhosis, hemolytic anemia, and hyperlipidemia.{{cn|date=March 2022}}
Treatment
Definitive treatment for Zieve's syndrome is alcohol cessation. Individuals with markedly elevated triglycerides, particularly with a history of pancreatitis or intracerebral hemorrhage, may require plasmapharesis to avoid complications associated with hypertriglyceridemia.{{cite journal |last1=Choudhry |first1=Faiza |last2=Kathawa |first2=Jolian |last3=Kerton |first3=Kelsey |last4=Farshadsefat |first4=Seina |last5=Piper |first5=Marc |title=Zieveʼs Syndrome Presenting With Severe Hypertriglyceridemia |journal=ACG Case Reports Journal |date=July 2019 |volume=6 |issue=7 |pages=e00133 |doi=10.14309/crj.0000000000000133| pmc=6722365|doi-access=free |pmid=31620530 }}
History
Zieve's syndrome was initially described in 1958.{{cite journal | last=Zieve | first=L | title=Jaundice, hyperlipemia and hemolytic anemia: a heretofore unrecognized syndrome associated with alcoholic fatty liver and cirrhosis | journal=Annals of Internal Medicine | volume=48 | pages=471–476 | date=March 1958 | pmid=13521581 | issue=3 | doi=10.7326/0003-4819-48-3-471}} Leslie Zieve described patients with a combination of alcoholic liver disease, hemolytic anemia and hypertriglyceridemia.{{cn|date=March 2022}}
References
External links
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| ICD10 = K70.0
| ICD9 = {{ICD9|571.1}}
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