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].

• Fatigue

  Patient concerns: A 30-year-old Chinese woman presented with a 4-month history of fatigue, yellowish… CONTINUE READING [semanticscholar.org]

  Patient concerns: A 30-year-old Chinese woman presented with a 4-month history of fatigue, yellowish discoloration of the eyes, and tea-colored urine. [ncbi.nlm.nih.gov]

  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. [symptoma.com]

• Fever

  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]

  He denied fever, chills, diaphoresis, hematemesis, melena, or tremors. The patient had been a heavy drinker for the past 20 years, consuming 56–84 g of alcohol per day. He admitted to drinking before coming to the hospital. [journals.lww.com]

• Refractory Anemia

  Clinicians need to maintain a high index of suspicion for ZS in patients with alcoholic hepatitis who have transfusion refractory anemia. Alcohol abstinence is essential for recovery. [link.springer.com]

• Abdominal Pain

  Consideration of appendicitis as the leading cause of emergency abdominal surgery; Contribution of the position of the appendix on the location of pain; Indications of appendicitis; Importance of seeking medical advice on persistent abdominal pains. [connection.ebscohost.com]

  It can present acutely with abdominal pain. [ncbi.nlm.nih.gov]

  The syndrome is defined by excessive blood lipoprotein, jaundice and abdominal pain. [athena.targetwoman.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]

• Right Upper Quadrant Tenderness

  CASE REPORT A 46-year-old white man presented with dyspnea and right upper quadrant abdominal pain of 2-month duration. [journals.lww.com]

• Epigastric Pain

  In the following report, we present the case of a man in his 30s who was admitted to our emergency department with severe epigastric pain and signs of alcohol intoxication. Blood samples showed signs of severe hyperlipidaemia and jaundice. [casereports.bmj.com]

• Poor Oral Hygiene

  The physical exam was significant for slurred speech, severe jaundice, scleral icterus, poor oral hygiene, and a distended abdomen. The patient reported no personal or family history of autoimmune disorders or congenital hemolytic anemias. [cureus.com]

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

  "Zieveʼs Syndrome Presenting With Severe Hypertriglyceridemia". ACG Case Reports Journal. 6 (7): 1–3. doi:10.14309/crj.0000000000000133. ^ Zieve, L (March 1958). [en.wikipedia.org]

  We report a case of ZS with severe hypertriglyceridemia. [unboundmedicine.com]

  Because the hypertriglyceridemia was not severe in those cases, no aggressive measures were taken in addressing the hypertriglyceridemia such as plasmapheresis or intravascular insulin therapy. The exact pathogenesis of ZS is not known. [journals.lww.com]

  We report a case of ZS with severe hypertriglyceridemia. Among the reported cases of ZS in English literature, we believe this is the first case of the syndrome presenting with severe hypertriglyceridemia requiring plasmapheresis. [insights.ovid.com]

  We suggest that this decrease in erythrocyte turnover helps to ameliorate hypertriglyceridemia. Malnutrition is another factor that may contribute to hypertriglyceridemia in infants with thalassemia or PK deficiency. [indianpediatrics.net]

In the following case report, we discuss plasmapheresis as a possible treatment for patients with severe Zieve syndrome in addition to conventional symptomatic therapy. [casereports.bmj.com]

The treatment of Zieve syndrome consists of abstinence from alcohol, blood transfusions as needed, and general supportive treatment [2]. [cureus.com]

The early diagnosis and treatment of such patients is of paramount importance to their management. [ncbi.nlm.nih.gov]

Medical Therapy Definitive treatment for Zieve's syndrome is alcohol cessation. [wikidoc.org]

Treatment[edit] Definitive treatment for Zieve's syndrome is alcohol cessation. [en.wikipedia.org]

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]

[…] underlying chronic liver disease and who recover spontaneously in 3-4 weeks after stopping drinking alcohol,5 compared to severe alcoholic hepatitis, which can require invasive diagnostic tests and administration of corticosteroids, as well as having a worse prognosis [elsevier.es]

The Maddrey discriminant function for alcoholic hepatitis is a commonly used scoring system that incorporates total bilirubin and prothrombin time to calculate disease severity and prognosis [13,14]. [cureus.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]

[…] to replace minerals in your teeth Saliva substitutes Drugs that help your salivary glands make more saliva To prevent dental decay caused by mouth dryness: Brush and floss your teeth often Visit the dentist for regular checkups and cleanings Outlook (Prognosis [pennmedicine.org]

Publication: 1997 Revue: Annales de medecine interne ISSN: 0003-410X NLM-ID: 0171744 Volume: 148 Num: 3 Page: 292-3 MeSH: Acute Disease|Adult|Anemia, Hemolytic/complications|Humans|Hyperlipidemias/complications|Jaundice/complications|Male|Pancreatitis/etiology [drci-chu-clermontferrand.fr]

Document type Article Language French Keyword (fr) ZIEVE SYNDROME HEPATITE AIGU ALCOOLISME ETIOLOGIE COMPLICATION HOMME PATHOLOGIE DIGESTIVE GASTROENTEROLOGIE Keyword (en) ZIEVE SYNDROME HEPATITIS ACUTE ALCOHOLISM ETIOLOGY COMPLICATION HUMAN GASTROENTEROLOGY [pascal-francis.inist.fr]

[…] 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]

We review the existing literature to explain the epidemiology, pathogenesis, diagnosis, and treatment of Zieve syndrome. [cureus.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]

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]

The pathophysiology of hemolysis in ZS is not fully understood. Dr. Zieve suggested that hyperlipidemia and presence of abnormal lipids (possibly lysolecithin) plays a role in hemolysis by disrupting RBC membrane [1]. [cureus.com]

In order to prevent pancreatitis, plasmapheresis was performed. Subsequently, triglyceride concentration dropped and the patient was discharged a few days later in significantly improved physical condition. [casereports.bmj.com]

McCullough Medicine The American Journal of Gastroenterology 2010 Intended for use by physicians, these recommendations suggest preferred approaches to the diagnostic, therapeutic, and preventive aspects of care intended to be flexible, in contrast to [semanticscholar.org]

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]

[…] unnecessary invasive testing or referrals and provide definitive therapy such as alcohol cessation counseling and enrollment into programs that would prevent relapse of alcoholism. [ncbi.nlm.nih.gov]

Surgery Prevention References ↑ ZIEVE L (1958). "Jaundice, hyperlipemia and hemolytic anemia: a heretofore unrecognized syndrome associated with alcoholic fatty liver and cirrhosis". Annals of Internal Medicine. 48 (3): 471–96. [wikidoc.org]

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.