familial nonhemolytic jaundice

jaundice

[jawn´dis] yellowness of skin, sclerae, mucous membranes, and excretions due to hyperbilirubinemia and deposition of pigments" >bile pigments. It is usually first noticeable in the eyes, although it may come on so gradually that it is not immediately noticed by those in daily contact with the jaundiced person. Called also icterus.
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.Patient Care. Assessment of the patient with jaundice includes observations of the degree and location of yellowing, noting the color of urine and stools, and the presence of itching. Since jaundice can be accompanied by severe itching, frequent skin care is important to preserve skin integrity. Tepid sponge baths can help reduce discomfort and promote rest.
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.

Jaundice may be attributable to prehepatic (A), hepatic (B), or posthepatic (C) causes. From Damjanov, 2000.acholuric jaundice jaundice without bilirubinemia, associated with elevated unconjugated bilirubin that is not excreted by the kidney. Familial acholuric jaundice is another name for the hereditary form of hemolytic jaundice" >hemolytic jaundice.breast milk jaundice elevated unconjugated bilirubin in some breast-fed infants due to the presence of an abnormal pregnane that inhibits glucuronyl transferase conjugating activity.cholestatic jaundice that resulting from abnormality of bile flow in the liver.familial nonhemolytic jaundice Gilbert disease.hematogenous jaundice hemolytic jaundice.hemolytic jaundice see hemolytic jaundice.hepatocellular jaundice jaundice caused by injury to or disease of the liver cells.leptospiral jaundice Weil's syndrome.neonatal jaundice (jaundice of the newborn) icterus neonatorum.nonhemolytic jaundice that due to an abnor-mality in bilirubin metabolism.obstructive jaundice that due to blockage of the flow of bile.physiologic jaundice mild icterus neonatorum during the first few days after birth.

fa·mil·i·al non·he·mo·lyt·ic jaun·dice

[MIM*143500] mild jaundice due to increased amounts of unconjugated bilirubin in the plasma without evidence of liver damage, biliary obstruction, or hemolysis; thought to be due to an inborn error of metabolism in which the excretion of bilirubin by the liver is defective, ascribed to decreased conjugation of bilirubin as a glucuronide or impaired uptake of hepatic bilirubin; autosomal dominant inheritance. Synonym(s): benign familial icterus, constitutional hepatic dysfunction, Gilbert syndrome

fa·mil·i·al non·he·mo·lyt·ic jaun·dice

(fă-mil'ē-ăl non'hē-mō-lit'ik jawn'dis) Mild form of the hepatic disorder due to increased amounts of unconjugated bilirubin in the plasma without evidence of liver damage, biliary obstruction, or hemolysis; thought to be due to an inborn error of metabolism in which the excretion of bilirubin by the liver is defective.
Synonym(s): Gilbert disease.

jaundice

(jon'dis) [Fr. jaunisse, fr. jaune, yellow]

JAUNDICE: Skin of the abdomenYellow staining of body tissues and fluids, due to excessive levels of bilirubin in the bloodstream. Jaundice is not usually visible until the total bilirubin level rises above 3 mg/dl. It is a symptom of many illnesses, including those marked by any of the following: obstruction of the biliary tract by gallstones, inflammatory masses, or tumors (such as cholecystitis, pancreatic carcinoma); slowing of the release of bile from hepatic portals (such as cholestasis); alteration of bile metabolism at the cellular level (such as in genetic diseases such as Gilbert's disease); release of bilirubin because of liver cell injury by toxins or viruses (such as acetaminophen overdose; hepatitis B virus infection); release of bile pigments as a result of the destruction or ineffective manufacturing of red blood cells (such as hemolysis; hereditary spherocytosis); or resorption of bile from hematomas within the body, esp. after trauma. See: illustrationjaundiced (jon'dist), adjective Synonym: icterus

Symptoms

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.

Diagnosis

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.

Treatment

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.

acholuric jaundice

Jaundice without bile pigment in the urine. Most of the excess bilirubin is unconjugated.

breast milk jaundice

, breast-feeding jaundiceHyperbilirubinemia resulting from pregnanediol or free fatty acids that inhibit bilirubin conjugation. Serum bilirubin level usually peaks above 20 ml/dl by 14 to 21 days of age. Some pediatricians recommend stopping breast-feeding for 24 to 36 hr if the level exceeds 20 ml/dl. If the infant's bilirubin level drops rapidly, the mother may resume nursing. It typically lasts for the first week of life or until the mother produces adequate milk.

cholestatic jaundice

Jaundice produced by failure of bile to flow to the duodenum. It may be caused by intrahepatic bile duct obstruction (as in certain drug reactions), liver cell damage (as in viral hepatitis), or extrahepatic obstruction to the flow of bile (as in cholecystitis).

congenital jaundice

Jaundice occurring at or shortly after birth.

familial nonhemolytic jaundice

Gilbert syndrome.

hematogenous jaundice

Hemolytic jaundice.

hemolytic jaundice

Jaundice caused by the fragmentation of red blood cells and the release of unconjugated bilirubin in the bloodstream. This finding is associated with hemolytic anemia (HA). Because the bilirubin is not conjugated by the liver, it is not soluble in water and does not discolor the urine. Many conditions may be responsible, including congenital HA; sickle cell anemia; autoimmune HA (as in infectious mononucleosis or Mycoplasma pneumoniae infections); microangiopathic HA (as in hemolytic uremic syndrome); or transfusion-associated HA. Synonym: hematogenous jaundice; hemolytic icterus; nonobstructive icterus

hemorrhagic jaundice

Leptospiral jaundice.

hepatocellular jaundice

Jaundice resulting from disease of liver cells, e.g., in acute hepatitis. Synonym: parenchymatous jaundice

infectious jaundice

Infectious hepatitis.

leptospiral jaundice

Jaundice caused by leptospirosis. Synonym: hemorrhagic jaundice

neonatal jaundice

Nonpathological jaundice affecting newborns, usually resulting from the destruction of red blood cells by the immature liver at birth. The destruction of red blood cells causes unconjugated bilirubin to accumulate in the blood and skin. Benign neonatal jaundice manifests 48 to 72 hr after birth, lasts only a few days, and typically does not require therapy.

Patient care

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 newborn

Neonatal jaundice.

nonhemolytic jaundice

Jaundice due to abnormal metabolism of bilirubin or to biliary tract obstruction, and not to excessive destruction of red blood cells.

obstructive jaundice

Jaundice caused by a mechanical impediment to the flow of bile from the liver to the duodenum. Gallstones are the most common cause. Cholangitis, obstructing cancers, cysts, parasites in the bile ducts, or hepatic abscesses are responsible less frequently. Synonym: obstructive icterus; postobstructive jaundice; regurgitation jaundice

Symptoms

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.

Treatment

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.

parenchymatous jaundice

Hepatocellular jaundice.

pathological jaundice of newborn

Hemolytic disease of the newborn.

physiologic jaundice

Neonatal jaundice.

postobstructive jaundice

Obstructive jaundice.

regurgitation jaundice

Obstructive jaundice.

retention jaundice

Jaundice resulting from the inability of liver cells to remove bile pigment from circulation.

spirochetal jaundice

Leptospirosis.

toxic jaundice

Jaundice resulting from chemical injury to the liver or sepsis.

Gilbert,

Nicholas A., French physician, 1858-1927. Gilbert disease - Synonym(s): familial nonhemolytic jaundiceGilbert syndrome - Synonym(s): familial nonhemolytic jaundice

Hebra,

Ferdinand von, Austrian dermatologist, 1816-1880. Hebra disease - an acute eruption of macules, papules, or subdermal vesicles presenting a multiform appearance. Synonym(s): erythema multiforme; familial nonhemolytic jaundiceHebra prurigo - a severe form of chronic dermatitis with secondary infection. Synonym(s): prurigo agria; prurigo ferox

fa·mil·i·al non·he·mo·lyt·ic jaun·dice

(fă-mil'ē-ăl non'hē-mō-lit'ik jawn'dis) [MIM*143500] Mild icterus due to increased amounts of unconjugated bilirubin in the plasma without evidence of liver damage, biliary obstruction, or hemolysis.

单词	familial nonhemolytic jaundice
释义	DictionarySeejaundice familial nonhemolytic jaundice jaundice [jawn´dis] yellowness of skin, sclerae, mucous membranes, and excretions due to hyperbilirubinemia and deposition of pigments" >bile pigments. It is usually first noticeable in the eyes, although it may come on so gradually that it is not immediately noticed by those in daily contact with the jaundiced person. Called also icterus. 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.Patient Care. Assessment of the patient with jaundice includes observations of the degree and location of yellowing, noting the color of urine and stools, and the presence of itching. Since jaundice can be accompanied by severe itching, frequent skin care is important to preserve skin integrity. Tepid sponge baths can help reduce discomfort and promote rest. 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.Jaundice may be attributable to prehepatic (A), hepatic (B), or posthepatic (C) causes. From Damjanov, 2000.acholuric jaundice jaundice without bilirubinemia, associated with elevated unconjugated bilirubin that is not excreted by the kidney. Familial acholuric jaundice is another name for the hereditary form of hemolytic jaundice" >hemolytic jaundice.breast milk jaundice elevated unconjugated bilirubin in some breast-fed infants due to the presence of an abnormal pregnane that inhibits glucuronyl transferase conjugating activity.cholestatic jaundice that resulting from abnormality of bile flow in the liver.familial nonhemolytic jaundice Gilbert disease.hematogenous jaundice hemolytic jaundice.hemolytic jaundice see hemolytic jaundice.hepatocellular jaundice jaundice caused by injury to or disease of the liver cells.leptospiral jaundice Weil's syndrome.neonatal jaundice (jaundice of the newborn) icterus neonatorum.nonhemolytic jaundice that due to an abnor-mality in bilirubin metabolism.obstructive jaundice that due to blockage of the flow of bile.physiologic jaundice mild icterus neonatorum during the first few days after birth. fa·mil·i·al non·he·mo·lyt·ic jaun·dice [MIM143500] mild jaundice due to increased amounts of unconjugated bilirubin in the plasma without evidence of liver damage, biliary obstruction, or hemolysis; thought to be due to an inborn error of metabolism in which the excretion of bilirubin by the liver is defective, ascribed to decreased conjugation of bilirubin as a glucuronide or impaired uptake of hepatic bilirubin; autosomal dominant inheritance. Synonym(s): benign familial icterus, constitutional hepatic dysfunction, Gilbert syndrome fa·mil·i·al non·he·mo·lyt·ic jaun·dice (fă-mil'ē-ăl non'hē-mō-lit'ik jawn'dis) Mild form of the hepatic disorder due to increased amounts of unconjugated bilirubin in the plasma without evidence of liver damage, biliary obstruction, or hemolysis; thought to be due to an inborn error of metabolism in which the excretion of bilirubin by the liver is defective. Synonym(s): Gilbert disease. jaundice (jon'dis) [Fr. jaunisse, fr. jaune, yellow] JAUNDICE: Skin of the abdomenYellow staining of body tissues and fluids, due to excessive levels of bilirubin in the bloodstream. Jaundice is not usually visible until the total bilirubin level rises above 3 mg/dl. It is a symptom of many illnesses, including those marked by any of the following: obstruction of the biliary tract by gallstones, inflammatory masses, or tumors (such as cholecystitis, pancreatic carcinoma); slowing of the release of bile from hepatic portals (such as cholestasis); alteration of bile metabolism at the cellular level (such as in genetic diseases such as Gilbert's disease); release of bilirubin because of liver cell injury by toxins or viruses (such as acetaminophen overdose; hepatitis B virus infection); release of bile pigments as a result of the destruction or ineffective manufacturing of red blood cells (such as hemolysis; hereditary spherocytosis); or resorption of bile from hematomas within the body, esp. after trauma. See: illustration*jaundiced (jon'dist), adjective Synonym: icterus Symptoms 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. Diagnosis 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. Treatment 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. acholuric jaundice Jaundice without bile pigment in the urine. Most of the excess bilirubin is unconjugated. breast milk jaundice , breast-feeding jaundiceHyperbilirubinemia resulting from pregnanediol or free fatty acids that inhibit bilirubin conjugation. Serum bilirubin level usually peaks above 20 ml/dl by 14 to 21 days of age. Some pediatricians recommend stopping breast-feeding for 24 to 36 hr if the level exceeds 20 ml/dl. If the infant's bilirubin level drops rapidly, the mother may resume nursing. It typically lasts for the first week of life or until the mother produces adequate milk. cholestatic jaundice Jaundice produced by failure of bile to flow to the duodenum. It may be caused by intrahepatic bile duct obstruction (as in certain drug reactions), liver cell damage (as in viral hepatitis), or extrahepatic obstruction to the flow of bile (as in cholecystitis). congenital jaundice Jaundice occurring at or shortly after birth. familial nonhemolytic jaundice Gilbert syndrome. hematogenous jaundice Hemolytic jaundice. hemolytic jaundice Jaundice caused by the fragmentation of red blood cells and the release of unconjugated bilirubin in the bloodstream. This finding is associated with hemolytic anemia (HA). Because the bilirubin is not conjugated by the liver, it is not soluble in water and does not discolor the urine. Many conditions may be responsible, including congenital HA; sickle cell anemia; autoimmune HA (as in infectious mononucleosis or Mycoplasma pneumoniae infections); microangiopathic HA (as in hemolytic uremic syndrome); or transfusion-associated HA. Synonym: hematogenous jaundice; hemolytic icterus; nonobstructive icterus hemorrhagic jaundice Leptospiral jaundice. hepatocellular jaundice Jaundice resulting from disease of liver cells, e.g., in acute hepatitis. Synonym: parenchymatous jaundice infectious jaundice Infectious hepatitis. leptospiral jaundice Jaundice caused by leptospirosis. Synonym: hemorrhagic jaundice neonatal jaundice Nonpathological jaundice affecting newborns, usually resulting from the destruction of red blood cells by the immature liver at birth. The destruction of red blood cells causes unconjugated bilirubin to accumulate in the blood and skin. Benign neonatal jaundice manifests 48 to 72 hr after birth, lasts only a few days, and typically does not require therapy. Patient care 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 newborn Neonatal jaundice. nonhemolytic jaundice Jaundice due to abnormal metabolism of bilirubin or to biliary tract obstruction, and not to excessive destruction of red blood cells. obstructive jaundice Jaundice caused by a mechanical impediment to the flow of bile from the liver to the duodenum. Gallstones are the most common cause. Cholangitis, obstructing cancers, cysts, parasites in the bile ducts, or hepatic abscesses are responsible less frequently. Synonym: obstructive icterus; postobstructive jaundice; regurgitation jaundice Symptoms 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. Treatment 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. parenchymatous jaundice Hepatocellular jaundice. pathological jaundice of newborn Hemolytic disease of the newborn. physiologic jaundice Neonatal jaundice. postobstructive jaundice Obstructive jaundice. regurgitation jaundice Obstructive jaundice. retention jaundice Jaundice resulting from the inability of liver cells to remove bile pigment from circulation. spirochetal jaundice Leptospirosis. toxic jaundice Jaundice resulting from chemical injury to the liver or sepsis. Gilbert, Nicholas A., French physician, 1858-1927. Gilbert disease - Synonym(s): familial nonhemolytic jaundiceGilbert syndrome - Synonym(s): familial nonhemolytic jaundice Hebra, Ferdinand von, Austrian dermatologist, 1816-1880. Hebra disease - an acute eruption of macules, papules, or subdermal vesicles presenting a multiform appearance. Synonym(s): erythema multiforme; familial nonhemolytic jaundiceHebra prurigo - a severe form of chronic dermatitis with secondary infection. Synonym(s): prurigo agria; prurigo ferox fa·mil·i·al non·he·mo·lyt·ic jaun·dice (fă-mil'ē-ăl non'hē-mō-lit'ik jawn'dis) [MIM*143500] Mild icterus due to increased amounts of unconjugated bilirubin in the plasma without evidence of liver damage, biliary obstruction, or hemolysis.
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