familial nonhemolytic jaundice(redirected from constitutional hepatic dysfunction)
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Jaundice is not a disease; it is a symptom of a number of different diseases and disorders of the liver and gallbladder and of hemolytic blood disorders. One such disorder is the presence of a gallstone in the common bile duct, which carries bile from the liver to the intestine. This may obstruct the flow of bile, causing it to accumulate and enter the bloodstream. The obstruction of bile flow may cause bile to enter the urine, making it dark in color, and also decrease the bile in the stool, making it light and clay-colored. This condition requires surgery to remove the gallstone before it causes serious liver injury.
The pigment causing jaundice is called bilirubin. It is derived from hemoglobin that is released when erythrocytes are hemolyzed and therefore is constantly being formed and introduced into the blood as worn-out or defective erythrocytes are destroyed by the body. Normally the liver cells absorb the bilirubin and secrete it along with other bile constituents. If the liver is diseased, or if the flow of bile is obstructed, or if destruction of erythrocytes is excessive, the bilirubin accumulates in the blood and eventually will produce jaundice. Determination of the level of bilirubin in the blood is of value in detecting elevated bilirubin levels at the earliest stages before jaundice appears, when liver disease or hemolytic anemia is suspected.
Patients with severe jaundice are at risk for encephalopathic changes that produce confusion, impaired mentation, and altered levels of consciousness. The potential for injury is increased and demands vigilance and safety measures to protect the patient.
fa·mil·i·al non·he·mo·lyt·ic jaun·dice[MIM*143500]
familial nonhemolytic jaundice
fa·mil·i·al non·he·mo·lyt·ic jaun·dice(fă-mil'ē-ăl non'hē-mō-lit'ik jawn'dis)
Synonym(s): Gilbert disease.
jaundice(jon'dis) [Fr. jaunisse, fr. jaune, yellow]
Deposits of bilirubin in the skin often cause itching. Other symptoms of jaundice depend on whether the bilirubin is direct (conjugated [i.e., soluble in body fluids]) or indirect (unconjugated). Obstructive jaundice causes conjugated hyperbilirubinemia; in this disease, bile pigments dissolve in the urine, which turns bright green, and the stool appears gray or white owing to the deprivation of bile.
Tests to determine the cause of jaundice include a carefully performed history and physical exam, urinalysis (positive for bilirubin only in conjugated hyperbilirubinemia), liver function tests, blood tests for hepatitis, and abdominal ultrasonography. Invasive diagnostic testing with cholangiography, endoscopic retrograde cholangiopancreatography, or percutaneous trans-hepatic cholangiography is performed when occult biliary obstruction is suspected.
The precise cause of jaundice must be determined in each patient to provide suitable therapies. For example, patients with gallstones obstructing the cystic duct need surgical treatment, and newborns with severe jaundice may require treatment with ultraviolet light to prevent kernicterus, but jaundiced patients with acute hepatitis A usually heal with symptomatic rather than specific remedies.
breast milk jaundice, breast-feeding jaundice
familial nonhemolytic jaundiceGilbert syndrome.
hematogenous jaundiceHemolytic jaundice.
hemorrhagic jaundiceLeptospiral jaundice.
infectious jaundiceInfectious hepatitis.
Levels of bilirubin less than 2 in the first week of life are common, occurring in about 80% of premature babies and half of all full-term babies. They are typically not hazardous to the developing infant. When jaundice develops in the first 24 hr of life, however, or when bilirubin levels exceed published guidelines, kernicterus (neurotoxicity caused by bilirubin) may develop. Infants with potentially damaging levels of bilirubin in the blood are treated with phototherapy (bili lights). Synonym: icterus neonatorum; jaundice of newborn; physiologic jaundicehemolytic disease of the newborn; isoimmunization; kernicterus; phototherapy;
jaundice of newbornNeonatal jaundice.
The condition is marked by yellow staining of the skin, mucous membranes, sclera, and secretions. The patient may complain of pruritus caused by bile pigments in the skin. The urine is yellow or green, but the stools turn light or clay-colored because of absence of bile pigment in the intestinal tract. Acute obstruction to the flow of bile causes right upper quadrant pain and may be associated with biliary colic due to entrapment of gallstones.
Cholecystectomy with common bile duct exploration (choledochostomy) is used to resolve obstructive jaundice caused by gallstones. Radical surgeries (as the Whipple procedure) or stenting of the biliary tract with or without external damage may temporarily relieve obstructive jaundice caused by cancer.