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Zieve's Syndrome

Zieve Syndrome

Zieve's syndrome is a disorder that manifests with anemia, jaundice, and hyperlipidemia, in the setting of alcoholism and hepatic cirrhosis.


Presentation

Zieve's syndrome is observed in suspected or known alcoholic patients and leads to the manifestation of transient jaundice due to acute hemolytic anemia and hyperlipidemia [1]. Individuals affected by the syndrome typically suffer from steatohepatitis as well.

Anemia is always present in the case of Zieve's syndrome and follows a hemolytic pathophysiological route, causing the appearance of jaundice. The symptoms such as fatigue and malaise may be seen in mild cases. A considerable number of patients, however, are affected by severe anemia, which is responsible for a profound set of symptoms, including tachycardia, tachypnea, and hypotension. Loss of consciousness and dizziness may be present due to the anemic condition of the patient in the Zieve's syndrome.

Pain in the right upper quadrant abdominal region is also a common symptom, leading to confusion between Zieve's syndrome and acute cholecystitis [2]. Patients have also reported myalgia, which occurs due to an increased serum viscosity, and retinal damage has also been observed, even though there may be no symptoms accompanying the latter [3] [4]. Some patients diagnosed with Zieve's syndrome present with intracranial hemorrhage, that has mainly been attributed to the increase in serum lipids, with this symptom being documented in only four cases [5].

Whipple Disease
  • Whipple’s disease is a cause of gastrointestinal (GI) malabsorption usually in middle-aged men, and is caused by the Gram-positive bacteria Tropheryma whippelii. Crigler–Najjar syndrome is a rare congenital disorder of bilirubin conjugation.[drsuemedicalnotes.blogspot.com]
Abdominal Pain
  • It can present acutely with abdominal pain.[ncbi.nlm.nih.gov]
  • All patients presented with acute abdominal pain and fever. Four had a history of a recent alcohol binge. Hyperlipidaemia was present in two patients; this subsided before the onset of haemolysis.[ncbi.nlm.nih.gov]
  • The main symptoms of acute ascending cholangitis are jaundice, abdominal pain, chills, and fever.... Is that stomach pain appendicitis? // Consumer Reports on Health;Dec2000, Vol. 12 Issue 12, p7 Focuses on the medical symptoms of appendicitis.[connection.ebscohost.com]
  • Clinical presentation includes the constellation of symptoms of hemolytic anemia, hyperlipidemia, abdominal pain and jaundice in the context of known alcohol abuse.[mayoclinic.pure.elsevier.com]
  • It is defined by hemolytic anemia (with spur cells and acanthocytes), hyperlipoproteinaemia (excessive blood lipoprotein ), jaundice (elevation of unconjugated bilirubin ), and abdominal pain. [1] The underlying cause is liver delipidization.[en.wikipedia.org]
Murphy's Sign
  • Patients affected by the syndrome do not display a positive Murphy's sign and are typically afebrile, in contradistinction to acute cholecystitis.[symptoma.com]
Retinal Hemorrhage
  • Given that Zieve's syndrome may also induce intracranial or retinal hemorrhage, fundoscopy and a complete neurological examination may be required. Neurological deficits may indicate the need for further investigation.[symptoma.com]
Retinal Damage
  • Patients have also reported myalgia, which occurs due to an increased serum viscosity, and retinal damage has also been observed, even though there may be no symptoms accompanying the latter.[symptoma.com]
Tremulousness
  • In Zieve's 20 patients, aged 26 to 65 years, tremulousness was common and frank delirium tremens occurred at times. The condition improved once the drinking stops. Leslie Zieve in Citation Classic, September 26, 1983, 39: 18.[whonamedit.com]

Workup

The diagnosis of Zieve's syndrome may be difficult, due to the similarities with other conditions and due to the habit of alcoholism often being withheld by the patients. The primary aim is to distinguish the syndrome from other medical conditions, such as acute cholecystitis and acute alcoholic hepatitis.

The initial laboratory evaluation of any patient presenting with jaundice, signs of anemia and possibly upper right abdominal quadrant pain includes the following:

  • Complete blood count: It will illustrate moderately or severely diminished hemoglobin levels, increased reticulocytes. Spherocytes and target cells may also be revealed.
  • Biochemical profile: Increased serum indirect bilirubin, alkaline phosphatase, cholesterol, and triglycerides. Liver function tests render abnormal results.

Distinguishing Zieve's syndrome from acute cholecystitis requires an initial thorough physical examination. Patients affected by the syndrome do not display a positive Murphy's sign and are typically afebrile, in contradistinction to acute cholecystitis. Furthermore, indirect serum bilirubin levels are profoundly elevated in Zieve's syndrome, amounting to over 5 mg/dL, whereas values between 2 and 5 mg/dL are expected in acute cholecystitis. A right upper quadrant ultrasonographic scan (RUQ US) or cholescintigraphy are also required: a lack of gallstones or pericholecystic fluid direct the diagnostic process towards Zieve's syndrome. These procedures, however, may render inconclusive results, such as gallbladder distention, findings which do not aid in the differential diagnosis of Zieve's syndrome and acute cholecystitis [6].

Zieve's syndrome also needs to be differentiated from acute alcoholic hepatitis. These two medical entities are distinguished, due to the fact that the former leads to hyperlipidemia in combination with hemolytic anemia, whereas the latter is characterized by a remarkable increase in transaminase levels (AST< 300 IU/mL), hyperbilirubinemia and a prolonged international normalized ratio (INR) [6]. Moreover, patients suffering from acute alcoholic hepatitis exhibit a steadily declining course, despite appropriate treatment, whereas Zieve's syndrome tends to regress, once the alcoholic trigger has been removed [6].

Given that Zieve's syndrome may also induce intracranial or retinal hemorrhage, fundoscopy and a complete neurological examination may be required [7]. Neurological deficits may indicate the need for further investigation.

Also, in the cases of individuals who have not yet been diagnosed with fatty liver disease or steatohepatitis, a liver biopsy will illustrate a fatty infiltration of the liver. In general, Zieve's syndrome must be considered as a possible pathology, when a patient with a history presents with indirect hyperbilirubinemia, a medical history of alcoholism and no other probable causes for the biochemical anomaly [8].

Hypertriglyceridemia
  • History [ edit ] Zieve's syndrome was initially described in 1958. [5] Dr Leslie Zieve described patients with a combination of alcoholic liver disease , hemolytic anemia and hypertriglyceridemia . References [ edit ] Mehta, AB; N McIntyre (2004).[en.wikipedia.org]
  • Historical Perspective Zieve's syndrome was initially described by Leslie Zieve in patients with a combination of alcoholic liver disease , hemolytic anemia and hypertriglyceridemia . [1] Classification Pathophysiology The proposed mechanism of the characteristic[wikidoc.org]
  • ., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… … Medical dictionary Zieve syndrome — a syndrome of hypercholesterolemia, hepatosplenomegaly, fatty liver, hemolytic anemia, and hypertriglyceridemia seen in alcoholics[translate.academic.ru]
  • Leslie Zieve described patients with a combination of alcoholic liver disease , hemolytic anemia and hypertriglyceridemia . References Mehta, AB; N McIntyre (2004). Oxford Textbook of Clinical Hepatology . Oxford University Press. pp. 1786–1787.[howlingpixel.com]
  • Leslie Zieve described patients with a combination of alcoholic liver disease , hemolytic anemia and hypertriglyceridemia . Mehta, AB; N McIntyre (2004). Oxford Textbook of Clinical Hepatology . Oxford University Press. pp. 1786–1787.[wikiwand.com]
Bone Marrow with Hyperplasia
  • There could be drop in hemoglobin, reticulocytes, bone marrow erythroblastic hyperplasia, and increased erythrocyte fragility and shortened life of red blood cells.[athena.targetwoman.com]

Treatment

  • Treatment Summary of Treatment of Zieve Syndrome Department of Geriatrics: Department of Geriatrics, Department of Geriatrics, Department of Geriatrics, Department of Geriatrics, Treatment: drug treatment supportive treatment Treatment cycle: 3 months[healthfrom.com]
  • (November 2017) Treatment [ edit ] Definitive treatment for Zieve's syndrome is alcohol cessation.[en.wikipedia.org]
  • Definitive treatment for Zieve's syndrome is alcohol cessation.[quizlet.com]
  • Medical Therapy Definitive treatment for Zieve's syndrome is alcohol cessation.[wikidoc.org]
  • PY - 2013 Y1 - 2013 N2 - The aim of this case is to make the primary care physician and hospitalist aware of the clinical presentation and treatment protocol for Zieve's syndrome.[mayoclinic.pure.elsevier.com]

Prognosis

  • Natural History Complications Prognosis Diagnosis Diagnostic Criteria History and Symptoms Physical Examination Laboratory Findings Imaging Findings Other Diagnostic Studies Treatment Medical Therapy Definitive treatment for Zieve's syndrome is alcohol[wikidoc.org]
  • Prognosis: symptoms can disappear. Basic Nursing Nursing of Zieve Syndrome Etiology of alcohol damage caused by liver damage and varying degrees of cholestasis, causing jaundice.[healthfrom.com]
  • […] person's signs and symptoms, and may include: Anticonvulsant medicines for seizures Eye drops or surgery to treat glaucoma Laser therapy for port-wine stains Physical therapy for paralysis or weakness Possible brain surgery to prevent seizures Outlook (Prognosis[ufhealth.org]
  • […] factors used in the calculation of Child Pugh score in a patient with liver cirrhosis, EXCEPT: Variceal bleeding 133 Candidates Hepatic encephalopathy 5 Candidates Serum bilirubin 5 Candidates CHOOSE ANSWER & CLICK HERE MELD score used to determine prognosis[gatetomedicine.com]
  • Spur cell anaemia – seen in advanced chronic liver disease especially in alcoholics and has a poor prognosis. It is related to abnormal cholesterol loading of the red cell membrane and reduced deformability of the red cells.[tropicalgastro.com]

Etiology

  • Etiology Zieve syndrome etiology Due to alcohol damage caused by liver damage and varying degrees of cholestasis, resulting in jaundice, alcohol can cause increased plasma free fatty acids, and because of increased intrahepatic lipid formation, resulting[healthfrom.com]
  • Moreover, further exams were performed: •Study of iron status that showed a significant increase in serum ferritin (4144 mcg/L, so it was performed the genetic study that affirmed the alcoholic etiology of the hemochromatosis) •Peripheral smear showed[omicsonline.org]
  • Jaundice, gall bladder micro-calculi and a Coombs’ negative, non-spherocytic hemolytic anemia (Hb 11.6 g/dL, reticulocyte count 24%) were noted in the newborn period, but no specific etiology was elucidated. She was exclusively breast fed.[indianpediatrics.net]

Epidemiology

  • […] hemolysin such as lysolecithin . [2] Changes in membrane lipid compositions such as increased cholesterol and polyunsaturated fatty acid (PUFA) have been report during the hemolytic phase. [3] Causes Differentiating Zieve's syndrome from Other Diseases Epidemiology[wikidoc.org]
  • He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education.[lifeinthefastlane.com]
Sex distribution
Age distribution

Pathophysiology

  • The exact pathophysiologic mechanism is unknown but, as reviewed in the article, has been postulated to be secondary to alcohol induced hyperlipidemia that in turn predisposes red cell membranes to lysis.[mayoclinic.pure.elsevier.com]
  • Anemia is always present in the case of Zieve's syndrome and follows a hemolytic pathophysiological route, causing the appearance of jaundice. The symptoms such as fatigue and malaise may be seen in mild cases.[symptoma.com]
  • Historical Perspective Zieve's syndrome was initially described by Leslie Zieve in patients with a combination of alcoholic liver disease , hemolytic anemia and hypertriglyceridemia . [1] Classification Pathophysiology The proposed mechanism of the characteristic[wikidoc.org]

Prevention

  • Prevention Prevention of Zieve Syndrome Preventive measures: alcohol abstinence can fundamentally prevent the occurrence of this disease.[healthfrom.com]
  • Prevention There is no known prevention. Images References Sahin M. Sturge-Weber syndrome. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 596.[ufhealth.org]
  • This leads to instability of a critically balanced system leading to an increased bleeding tendency Prevention and treatment of bleeding in cirrhosis Our understanding of the coagulation cascade has undergone a change in recent times.[tropicalgastro.com]
  • […] macrophage-mediated clearance of antibody bound RBCs) cyclophosphamide or azathioprine (third line) role of IV Ig is uncertain (used in case reports) danazol (synthetic steroid, used previously) cold AIHA keep patient and fluids warm – avoidance of cold prevents[lifeinthefastlane.com]

References

Article

  1. Zieve L. Jaundice, hyperlipemia and hemolytic anemia: A heretofore unrecognized syndrome associated with alcoholic fatty liver and cirrhosis. Annals Intern Med. 1958;48(3):471–96.
  2. Hashmi S, Allison MG, McCurdy MT, Reed RM. Hyperbilirubinaemia and haemolytic anaemia in acute alcoholic hepatitis: there's oil in them thar veins. BMJ Case Rep. 2014.
  3. Martin JC, Ross A, Watson D, et al. A case of Zieve's syndrome presenting with myalgia: not to be confused with polymyalgia rheumatica. Br J Rheumatol. 1996;35:495–6.
  4. Gross C, Bohl B, Herfurth S. A case report of unusual retinal findings in a patient with Zieve syndrome. Ophthalmology. 2002;99:876–9.
  5. Hilz MJ, Bauer J, Druschky KF, et al. Zieve's syndrome and intracranial haemorrhage: coincidence or related disorders. J Neurol. 1990;237:120–1.
  6. Kurzweil SM, Shapiro MJ, Andrus CH, et al. Hyperbilirubinemia without common bile duct abnormalities and hyperamylasemia without pancreatitis in patients with gallbladder disease. Arch Surg. 1994;129:829–33.
  7. Lucey MR, Mathurin P, Morgan TR. Alcoholic hepatitis. N Engl J Med. 2009;360:2758–69.
  8. Nath S, Peck JR. Hemolytic Anemia in Alcohol-Induced Liver Disease: A Case Report on
    Zieve’s Syndrome. Int J Blood Res Disord. 2015, 2:1.

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Last updated: 2018-06-22 09:18