Questions & answers

Questions & answers

1. On the basis ofthe history and physical examination, what would you consider in your diferential diagnosis? Among the possible diagnoses to be cons...

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1. On the basis ofthe history and physical examination, what would you consider in your diferential diagnosis? Among the possible diagnoses to be considered for Allen are mononucleosis, anemia, hepatitis, viral syndrome, recurrent strep pharyngitis, biliary duct abnormalities, and Gilbert syndrome. Mononucleosis, anemia, and viral syndrome would all present with fatigue, sore throat, and possibly nausea. The absence of lymphadenopathy decreases the likelihood that Allen has one of these acute illnesses. The presence of scleral icterus with a duration of 3 years should lead the clinician to suspect a hepatic cause of the illness. Viral hepatitis is a clinical condition commonly found in the United States; more than 300,000 cases are reported each year. The infection can be caused by at least four distinct agents: hepatitis A, hepatitis B, non-A/non-B hepatitis, and hepatitis C. The disease prodrome may resemble a respiratory or gastrointestinal infection, followed by symptoms of malaise, fever, anorexia, nausea, and vomiting. The liver becomes enlarged. Within 4 to 5 days, the patient becomes jaundiced. The patient’s urine becomes unusually darker, and weight loss may occur. Liver enzymes, especially serum glutamate oxaloacetate transaminase/aspartate aminotransferase (SGOT/AST) and serum glutamate pyruvate transaminase/ alananine aminotransferase (SGPT/ ALT), are abnormal (Nelson, 1996). Gilbert syndrome is a relatively common and benign congenital liver disorder. It occurs in a 4:l ratio in males/ females, can be accompanied by intermittent episodes of nausea and fatigue, and is characterized by a mild and fluc-

March/P\pril 1998

mating increase in serum bilirubin levAn estimated 3.7% of the adult population has Gilbert syndrome. Onset usually occurs in the teen years or early adulthood (age 20 to 30 years). Patients rarely have significant symptoms, but occasionally mild jaundice may appear. The sclerae may be icteric. Gilbert syndrome occasionally presents as an incidental laboratory finding, with serum bilirubin levels rising when the patient fasts or during an intercurrent illness, such as a cold or flu. Gilbert syndrome can be diagnosed by documenting a consistently elevated serum bilirubin level when results of all other liver function tests are normal (American Liver Foundation, 1996). Biliary duct abnormalities and other anatomic defects of the liver normally would have presented themselves earlier in life. Two defects of inherited conjugated hyperbilirubinemia, DubinJohnson syndrome and Rotor’s syndrome, are both autosomal recessive disorders characterized by mild jaundice and an elevated direct bilirubin and are exacerbated by infection. Both of these syndromes usually present in the adolescent period (Nelson, Behrman, Klugman, & Arvin, 1996). els.

2. What additional information would you need to confirm your diagnosis? A more complete to be obtained, rence of jaundice

Ann L. Komelasky

medical history needs including the occurin the past or in other

is a Certified Pediatric Nurse Practitioner

Reprint requests: Ann L. Komelasky, Manassas, VA 22 110. J Pediatr Health Care. (1998). Copyright

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family members, use of alcohol or illegal substances, and travel outside the United States in the past 3 to 5 years. A complete blood cell count would be indicated, with an elevation in the white blood cell count supporting the diagnosis of acute infection. The sedimentation rate may be elevated or normal. A mono spot, Epstein-Barr IgM titers, cytomegalovirus IgG titers, and a throat culture would rule out those entities as the source of infection. An acute hepatitis screen and liver function studies are essential in the workup for the scleral ictems. Allen’s family history reveals no relatives with intercurrent jaundice or hepatic disease. He denies use of alcohol or illegal substances. He has not traveled outside the United States. Allen’s laboratory results are all negative, except for a viral shift on the differential, a bilirubin level of 8.2, an ALT of 68, and a gamma-glutamyl transpeptidase (GGT) of 59. Results of the hepatitis screen are negative. Allen was also sent for an abdominal sonogram to rule out hepatobiliary masses or abnormalities; findings of the sonogram were normal. Allen continued to have symptoms for 2 weeks after the initial presentation. On day 5 of his illness, his bilirubin level had decreased to 3.3, his ALT to 58, and his GGT to 79. His physical examination had returned to normal with no icterus and only mild cold symptoms. By 1 month after his illness

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Komelasky

his serum bilirubin level had to returned to 1.6 (normal, 0.2 to 1.3). 3.

What treatment would you recommend?

The immediate treatment for Gilbert syndrome is directed toward the relief of symptoms. In Allen’s case this included rest, fluids, fever and pain control, and decongestants. His activities and sports were limited until his jaundice and dizziness had abated. The most important portion of medical management is education, reassurance, and support. ln some cases, the use of phenobarbital has been shown to reduce the serum bilirubin levels (Black and Sherlock, 1970). Allen and his mother were given written information about Gilbert syndrome. They were reassured that the jaundice occurring with intercurrent viral illnesses would be benign but could be accompanied with nausea and fatigue. Several studies have looked at the prognosis of Gilbert syndrome and the need to avoid the use or overuse of

certain drugs toxic to the liver in patients with Gilbert syndrome, with no conclusive results (Testa et al., 1995; Herman, Chaudhary, & Szakacs, 1994; and Durst, Jabotinsky-Rubin, Dorevitch, Kikinson, and Tur-Kaspa, 1993). The effect of religious fasting has also been examined and was found to exacerbate the jaundice in these patients (Ashraf, van Someren, Quigley, Saboor, & Farrow, 1994). In addition, some correlation of an increased serum alkaline phosphatase in some patients with Gilbert syndrome has been found (Lieverse et al., 1990). It is most important that the patient and the clinician understand this disease and that the medical history/record be clearly marked to avoid future unnecessary laboratory testing and patient inconvenience.

REFERENCES Liver Foundation. (1996). Gilbert’s syndrome (pamphlet). Cedar Grove, New Jersey. A&d, W., van Someren, N., Quigley, E.M., Saboor,

American

S.A., & Farrow, L.J. (1994). Gilbert’s syndrome and Ramadan: Exacerbation of unconjugated hyperbilirubinemia by religious fasting. Journal

ofCZinical GasfuoeMferoZogy, 19,122-124. Black, M.M., Gilbert’s

& Sherlock, S. (1970). Treatment of syndrome with phenobarbitome. Lancef, I, 1359-1361. Durst, R., Jabotinsky-Rubin, K., Dorevitch, A., Kikinson, L., & Tur-Kaspa, R. (1993). Idiopathic unconjugated hyperbilirubinemia (Gilbert’s syndrome) and concurrent psychotropic drug administration. Pharmacopychiafry, 2649-52. Herman, R.J., Chaudhary, A., & Szakacs, C.B. (1994). Disposition of lorazepam in Gilbert’s syndrome: Effects of fasting, feeding, and enterohepatic circulation. Journal of Clinical Phanrcology, 34,978-984. Lieverse, A.G., va&ssen, G.G., Beukeveld, G.J., Gazendam, J., Dompeling, EC., tenKate, L.P., VW Belle, S.A., & Weits, J. (1990). Familial increased serum intestinal alkaline phosphatase: A new variant associated with Gilbert’s syndrome. Journal ofClinical Pafhology, 43,125-128. Nelson, W.E., Behnnan, R.E., K&man, R.M., &

Awin,

A.M. (1996). Nelson’s Textbook of Pedi-

atrics (15th ed.) Philadelphia: Saunders. Testa, R., Bardellini, E., Borzone, S., Montani, I’., F&so, D., Bindi, l’., Calieris, S., Campo, N., Alvarez, S., & Piieddu, M. (1995). Caffeine clearance in subjects with constitutional unconjugated hyperbilirobinemia. lfalian Journal of

Gasfroenferology,

27,129-132.

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