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].

• Anemia

  A good way to differentiate between them is to determine the type of the anemia; ZS causes hemolytic anemia while alcoholic hepatitis causes macrocytic anemia. [cureus.com]

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

  Profligate, active, nucleated red cells could be seen in a blood smear, and there were signs of hyperplastic anemia, indicating that the anemia was due to hemolysis. [ncbi.nlm.nih.gov]

• Fatigue

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

  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]

  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]

• 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]

  […] body's serotonin level (serotonergic drug) and have at least three of the following signs or symptoms: Agitation Abnormal eye movements (ocular clonus, this is a key finding in establishing a diagnosis of SS) Diarrhea Heavy sweating not due to activity Fever [medlineplus.gov]

• Weakness

  Symptoms Symptoms of Sturge-Weber syndrome include: Port-wine stain (more common on the face than the rest of the body) Seizures Paralysis or weakness on one side Learning disabilities Glaucoma (very high fluid pressure in the eye) Exams and Tests Glaucoma [ufhealth.org]

  At presentation, he complained of nausea, vomiting, weakness, and dark urine over the prior 2 weeks. He denied abdominal pain, fevers, chills, hematemesis, coffee ground vomitus, or melena. [ncbi.nlm.nih.gov]

  Other nonspecific symptoms include nausea, vomiting, malaise, weakness, and low-grade fever [1]. ZS can mimic alcoholic hepatitis as the two conditions share findings such as alcohol use, jaundice, and anemia [3,7]. [cureus.com]

• Anorexia

  She was admitted to the hospital with anorexia, asthenia and jaundice. Physical examination showed liver stigmata and hepatomegaly. [pubmed.ncbi.nlm.nih.gov]

  Symptoms Most common symptoms due to long-term history of chronic alcoholism include: Nausea Vomiting after heavy drinking Anorexia Abdominal pain Hepatomegaly, enlarged spleen, late cirrhosis Skin and yellow sclera Hemolytic Anemia, Hemoglobinuria (hemoglobin [athena.targetwoman.com]

• Cough

  He had diarrhea for one week, Pain in the left lower portion of the chest, and mild cough. He had from eight to 12 watery, dark brown bowel movements a day. The chest pain and cough subsided two days after onset. [jamanetwork.com]

  Older antidepressants called monoamine oxidase inhibitors (MAOIs) can also cause SS with the medicines described above, as well as meperidine (Demerol, a painkiller) or dextromethorphan (cough medicine). [medlineplus.gov]

  Teeth decay and gum inflammation Hoarseness Other symptoms may include: Fatigue Fever Change in the color of hands or feet with cold exposure (Raynaud phenomenon) Joint pain or joint swelling Swollen glands Skin rash Numbness and pain due to neuropathy Cough [pennmedicine.org]

• Abdominal Pain

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

  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]

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

  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. [scienceopen.com]

• 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]

• Jaundice

  Three classical features of ZS are jaundice, hyperlipidemia, and hemolytic anemia. Jaundice and hyperbilirubinemia result from hemolysis and cholestasis due to alcohol-induced liver injury [4]. [cureus.com]

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

  She was admitted to the hospital with anorexia, asthenia and jaundice. Physical examination showed liver stigmata and hepatomegaly. [pubmed.ncbi.nlm.nih.gov]

  Keywords: alcoholic liver disease, case report, hemolytic anemia, jaundice, Zieve syndrome 1.  [ncbi.nlm.nih.gov]

• Suggestibility

  Heinle Medicine The American journal of the medical sciences 1968 Results suggest that an unidentified, extracorpuscular factor develops during the acute illness and subsides during remission, and whether this factor is unique to patients whose illness [semanticscholar.org]

  - In view of the diminished ATP and raised 2,3-diphosphoglycerate (2.3 DPG) levels - a combination which suggests a pyruvate kinase (PK) deficiency - additional procedures were carried out in order to detect an abnormal activity of the red cell PK. [ncbi.nlm.nih.gov]

  Zieve1 initially suggested that the presence of haemolytic anaemia could be secondary to the exposure of the erythrocyte membranes to a high presence of haemolysins (lysolecithin and lysocephalin). [elsevier.es]

  The current case demonstrates a variant on the theme of Zieve's syndrome in that the patient's lipid panel was essentially normal suggesting that the exact mechanism of hemolysis is still uncertain. [mayoclinic.pure.elsevier.com]

  Zieve suggested that hyperlipidemia and presence of abnormal lipids (possibly lysolecithin) plays a role in hemolysis by disrupting RBC membrane [1]. [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].

• Acanthocytes

  It is defined by hemolytic anemia (with spur cells and acanthocytes/schistocytes), hyperlipoproteinemia (excessive blood lipoprotein), jaundice (elevation of unconjugated bilirubin), and abdominal pain. The underlying cause is liver delipidization. [quizlet.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]

  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. [howlingpixel.com]

  A peripheral blood smear showed acanthocytes and schistocytes (Figure 2). Two units of packed RBCs were transfused on day 1 of admission. [cureus.com]

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

  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]

• Liver Biopsy

  A liver biopsy revealed mild fatty degeneration, Mallory bodies, and cholestasis (Figs. 2–5). Viral hepatitis, nonhepatotropic viral infection, autoimmune liver disease, and metabolic liver disease were ruled out. Liver biopsy at low magnification. [ncbi.nlm.nih.gov]

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]

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]

[…] 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]

[…] 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]

[…] 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]

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]

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]

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]

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]

[…] 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.