Internal Medicine
Recertification / MOC Exam Review

Educational Objective:

Diagnose intrahepatic cholestasis of pregnancy.

Key Point:

Intrahepatic cholestasis of pregnancy is a benign condition occurring in the second or third trimester and characterized by generalized pruritus and elevated serum bile acids and aminotransferases.

Explanation:

ICP is typically a benign condition that occurs in approximately one in 100 pregnancies in the United States. In addition to bilirubin, other liver function tests are elevated, particularly alkaline phosphatase. Pruritus results from retention of bile salts. Symptoms and signs of cholestasis generally subside within 48 hours of delivery. Preeclampsia does not cause pruritus. Hemolytic anemia may result in jaundice, but, again, pruritus would be unlikely. Cholelithiasis is common in pregnancy but jaundice, from bile duct obstruction, without pain, would be highly unusual.

References:

Geenes V, Williamson C. Intrahepatic cholestasis of pregnancy. World J Gastroenterol. 2009;15(17):2049-2066

Grone AK, Smith JF Jr. Intrahepatic cholestasis of pregnancy Neo Rev. 2012;13(3):e145--e150.

Geenes, Victoria, et al. "Association of severe intrahepatic cholestasis of pregnancy with adverse pregnancy outcomes: A prospective population-based case-control study." Hepatology 59.4 (2014): 1482-1491.

Educational Objective:

Use serum tryptase measurement to support a diagnosis of anaphylaxis in equivocal cases.

Key Point:

Serum tryptase levels are elevated in an anaphylactic reaction when compared to levels obtained from an asymptomatic baseline.

Explanation:

The best laboratory test to order to clarify whether this patient is having an anaphylactic reaction is a serum mature/total tryptase level. Mature/total tryptase levels can be obtained in situations where it is unclear whether a patient is having an anaphylactic reaction or some other condition such as a septic shock, carcinoid syndrome, or cardiogenic shock. Tryptase levels are elevated in the setting of anaphylaxis. Mature tryptase levels should be obtained along with a total tryptase level. If the ratio of total to mature tryptase levels is below 10, anaphylaxis is suggested. If only a total tryptase level can be obtained, then it is best to obtain an initial level during the event of concern and then to obtain a repeat level after the acute symptoms have resolved. Metanephrines are used to diagnose pheochromocytoma, not anaphylaxis.  Serum serotonin levels are not used clinically. Vasoactive intestinal peptide levels are used in the diagnosis of neuroendocrine tumors and not anaphylaxis.

References:

Schwartz LB. Laboratory tests to support the clinical diagnosis of anaphylaxis. Revised December 12, 2014. Accessed February 5, 2016.

Tran PT, et al. Allergy, hypersensitivity, angioedema, and anaphylaxis. In: Marx JA, et al. Rosen's Emergency Medicine: Concepts and Clinical Practice. 8th ed.,2014.1543-1557.e.2.

Educational Objective:

Diagnose and treat intrahepatic cholestasis of pregnancy.

Key Point:

Intrahepatic cholestasis of pregnancy is distinguished by severe pruritus and hyperbilirubinemia.

Explanation:

Intrahepatic cholestasis of pregnancy usually occurs during the second or third trimester and its hallmark symptom is pruritus, often with isolated elevations of bilirubin. The exact etiology is unknown but is likely to be multifactorial.

Diagnosis is made by distinguishing this entity from other liver conditions in pregnancy, such as acute fatty liver and viral hepatitis. Cholestasis is found more often in patients infected with hepatitis C virus. Treatment involves controlling symptoms with medications such as hydroxyzine, cholestyramine, or ursodeoxycholic acid.

Although intrahepatic cholestasis of pregnancy poses a slight increase in risk to the fetus, no indication for urgent cesarean delivery exists at this time. Diagnostic endoscopic retrograde cholangiopancreatography should be avoided during pregnancy and would be of low yield in a woman with normal findings on ultrasonography.

References:

Williamson C, Geenes V. Intrahepatic cholestasis of pregnancy. Obstet Gynecol. 2014;124:120-133.

Brouwers, L, Koster MPH, Intrahepatic cholestasis of pregnancy: maternal and fetal outcomes associated with elevated bile acid levels. American Journal of Obstetrics and Gynecology, 2015; 212:100.e1-100.e7

Educational Objective:

Discuss the diagnosis of malingering.

Key Point:

Suspect malingering in a patient presenting with a simulated illness in order to obtain external benefits (e.g., money, avoidance of prosecution). Such patients are commonly uncooperative with diagnostic evaluations and treatments.

Explanation:

The clinician should suspect malingering. According to the diagnostic criteria set forth on the DSM- V, clinicians should suspect malingering when an evaluation occurs in a medicolegal context, such as referral from an attorney, when a marked discrepancy exists between the person's claims and objective findings, when the person is uncooperative in the diagnostic evaluation and in complying with the prescribed treatment regimen, or when antisocial personality disorder is present. The circumstances in this case contain all of the warning signs except antisocial personality disorder. Furthermore, there is no evidence that the person evaluated seeks the patient role; therefore, a factitious disorder can be excluded. The context points to the intentional production of the symptoms described by the person, ruling out the somatoform disorders hypochondriasis and conversion disorder.

References:

American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, Fifth edition, 2013

Heer JS. Factitious Disorders and Malingering. Rosen’s Emergency Medicine: Concepts and Clinical Practice. 2018; 104: 1361-1365.e1

Educational Objective:

Diagnose Mollaret’s syndrome.

Key Point:

Mollaret’s syndrome is a benign, recurrent, aseptic lymphocytic meningitis frequently caused by HSV-2 virus.

Explanation:

This patient has Mollaret syndrome, which is benign, recurrent, aseptic meningitis. The most common cause of this condition is HSV-2 infection. Use of antiviral prophylaxis does not appear to affect the risk of meningeal recurrence, although it can prevent more frequent outbreaks of genital lesions, as seen in this patient. The disease is usually self-limited.

HSV-1 infection is most likely to cause a single episode of severe encephalitis with fever, focal deficits, seizures, and abnormal neuroimaging affecting the frontotemporal lobe. Enterovirus can cause recurrent or chronic meningitis in patients with immunoglobulin deficiency and, occasionally, in otherwise apparently healthy infants. An epidermoid cyst can release irritating chemicals in the CSF and cause recurrent meningitis, but the neuroimaging is normal in this patient. Patients with complement deficiency can have recurrent bacterial meningitis with Neisseria meningitides infection, but the prompt resolution of the symptoms and the negative cultures argue against this possibility.

References:

Aurelius E, Franzen-Röhl E, Glimåker M, et al; HSV-2 Meningitis Study Group. Long-term valacyclovir suppressive treatment after herpes simplex virus type 2 meningitis: a double-blind, randomized controlled trial. Clin Infect Dis. 2012;54(9):1304-1313.

Schlesinger Y, Tebas P, Gaudreault-Keener M, Buller RS, Storch GA. Herpes simplex virus type 2 meningitis in the absence of genital lesions: improved recognition with use of the polymerase chain reaction. Clin Infect Dis. 1995;20(4):842-848.