(jī′găn-tizm) [Gr. gigas, stem gigant-, giant + -ism] The excessive development of the body or a body part. SYN: giantism.
(jēl′yē) [Leonardo Gigli, Italian gynecologist, 1863–1908] A flexible wire saw with specialized teeth used for cutting bony structures. It is operated manually by pulling its handles back and forth. It was first used to section the symphysis pubis as a way of making difficult deliveries easier.
(zhēl-bār′) [Nicolas A. Gilbert, Fr. physician, 1858–1927] A benign, autosomal dominant form of jaundice with variable penetrance, secondary to glucuronyl-transferase deficiency, and resulting in elevated levels of unconjugated bilirubin.
INCIDENCE: The disease is common, present in about 5% to 10% of people of European ancestry.
CAUSES: It is caused by a deficiency in the enzyme system that conjugates bilirubin (bilirubin-uridine diphosphate glucuronyl transferase), making it soluble in water. In the absence of this enzyme, unconjugated bilirubin accumulates in the body.
SYMPTOMS AND SIGNS: Most patients have no symptoms; some have nonspecific abdominal pain. Patients may have mild elevations of serum bilirubin levels that may or may not be visible on casual examination of the skin.
DIAGNOSIS: Gilbert syndrome is diagnosed when a patient is found to have elevated levels of unconjugated bilirubin on blood testing, i.e., a bilirubin level above normal, but typically less than 6 mg/dL. The conjugated bilirubin level is typically within normal range. Bilirubin is not found on dipstick testing of a urine specimen because unconjugated bilirubin is not soluble in water. If the patient has no other existing conditions, liver enzymes and serum lactate dehydrogenase levels are normal. Red blood cell morphology and red blood cell counts are normal, in contrast to patients with hemolytic anemias. In the past, when the diagnosis was in doubt, serum bilirubin levels were tested when the patient was well hydrated, and those levels were compared to serum levels after a prolonged fast. Fasting, dehydration, fevers, and other stressors increase levels of unconjugated bilirubin in patients with Gilbert syndrome.
PREVENTION: The disease cannot be prevented, and it affects the metabolism of some drugs, e.g., the chemotherapeutic agent irinotecan. Drugs whose metabolism can be affected by Gilbert syndrome should be administered at altered doses.
IMPACT ON HEALTH: Gilbert syndrome has been associated with an increased risk of gallstones, but the syndrome is generally considered to be benign. As a result, transplanted livers taken from patients with Gilbert syndrome are used without concern that the transplanted livers may affect the survival of the organ transplant recipients.
TREATMENT: No treatment is indicated.
PATIENT CARE: Patients should be reassured that, although they have high levels ...