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63 Cards in this Set

  • Front
  • Back
What is Budd-Chiari syndrome?
Hepatic Vein Thrombosis
What is the maximum normal liver edge in newborn?
3.5 cm below RCM
max liver edge in child
2 cm below RCM
Normal Liver Span at 1 week
4.5 to 5 cm
What is a Riedel Lobe?
normal variant of right lobe of liver extends far below RCM
5 mechanisms that can produce hepatomegaly
1)inflammation, 2)excessive storage, 3)infiltration, 4) congestion, 5) obstruction
What are Kupffer cells?
specialized macrophages located in the liver that form part of the reticuloendothelial system. The primary function of Kupffer cells is to recycle old red blood cells that no longer are functional.
Causes of inflammation of the liver
Infections, Toxins, radiation, autoimmune disease, and Kupffer cell hyperplasia
causes of biliary obstruction
Cholelithiasis, Choledochal cyst , Biliary atresia, tumors
hepatomegaly in neonate with hyperbilirubinemia after 2 weeks of age - 3 diseases to r/o
extrahepatic biliary atresia, CF, alpha 1 antitrypsin deficiency
systemic signs of liver disease
jaundice, fatigue, anorexia, weight loss, abdominal distention, blood in stool,
signs of chronic liver disease
spider angiomas, palmar erythema, xanthomas. More common in adults
signs that hepatomegaly is related to congenital infection
A neonatal history of IUGR, microcephaly, chorioretinitis, and purpura
hepatic bruit
Reticuloendothelial hyperplasia or Hemangiomatosis
Wolman disease
lysosomal storage disease; large amounts of lipids (particularly cholesteryl esters and glycerides) accumulate in the liver, spleen, lymph nodes and other tissues; Sx include HSM, an excessive amount of fat in stool; death usually occurs by 6 months
Reye and Reye-like syndromes
encephalopathy and fatty liver
What causes both hepatomegaly and splenomegaly?
Portal hypertension, hepatic infiltration by malignant cells, or storage disease
signs of portal hypertension
HSM, ascites, prominent venous pattern to abdomen
Ddx of Failure to thrive and hepatomegaly in infancy
metabolic disease such as glycogen storage, hereditary fructose intolerance, galactosemia, or cystic fibrosis
hepatomegaly and distinctive breath or urine odor
consider organic acidemias.
signs of hemangiomatosis
Cutaneous hemangiomas or a hepatic bruit
progressive neurologic deterioration and hepatomegaly
may have glycogen or lipid storage disease or Wilson disease
Zellweger syndrome
disorder of peroxisomal function; mongoloid facies, hypotonia, and neurologic deterioration suggests
ocular findings in Wilson disease
Kayser-Fleischer rings or cataracts
Gianotti-Crosti syndrome
Papular acrodermatitis - a self-limiting dermatosis that may be seen in patients who have viral hepatitis
basic lab eval of hepatomegaly
Urinalysis, CBC, Reticulocyte count; chem 20; alk phos; PT
Which is more liver-specific: Alanine aminotransferase or aspartate aminotransferase?
ALT. AST is also found in muscle
Labs to assess synthetic function
serum albumin and prothrombin time
prolonged prothrombin time
decrease in liver synthetic function or malabsorption of vitamin k
labs to order if you suspect hepatomegaly related to metabolic defect
glucose, ketones, lactic acid, pyruvic acid, amino acids, and uric acid along with urine organic acids
Calcifications in the liver, the vasculature, or the biliary tree seen on x-ray
malignancy or parasitic infection, portal vein thrombosis, or gallstones
most helpful radiology study for hepatomegaly
ultrasound with doppler
study to distinguish biliary atresia from neonatal hepatitis
Radionuclide scan; biliary atresia: hepatic uptake of the radionuclide is normal, but excretion into the intestine is absent. In neonatal hepatitis, uptake by the diseased liver parenchyma is impaired, but there is excretion into the intestines
definitive study for biliary atresia
cholangiography; usually intra-operative
Hepatomegaly plus conjugated hyperbilirubinemia without splenomegaly in neonate
1) r/o biliary atresia. radionuclide scan asap; if no excretion into duodenum, do liver biopsy with intraop cholangiography prior to Kasai
hepatomegaly w/o splenomegaly elevated conjugated hyperbili in neonate and biliary atresia ruled out
ultrasound for choledochal cyst; tests for causes of neonatal hepatitis; idiopathic neonatal hepatitis
in infant: Hepatomegaly plus conjugated hyperbilirubinemia with splenomegaly (and usually vomiting and FTT)
congenital infection, sepsis, or metabolic disease
in infant: Unconjugated or mixed hyperbilirubinemia associated with hepatosplenomegaly
congenital infections, increased portal pressures, or extramedullary hematopoiesis
Hepatosplenomegaly in an infant who has no hyperbilirubinemia
suggests an obstructive or infiltrative cause
suspect infilatrative disease; what tests?
Abdominal ultrasonography to look at liver consistency, patency of venous flow, and mass lesions. Liver biopsy is diagnostic for infiltrative diseases
Labs c/w Viral hepatitis:
hyperbilirubinemia, elevated conjugated bilirubin, and elevated aminotransferases plus positive serology for a viral hepatitis
Labs c/w viral hepatitis but neg serologies. Ddx?
drug or toxin exposures, autoimmune hepatitis, and Wilson disease
Ddx for child with HSM and no hyperbilirubinemia:
malignancy, storage disease, parasitic cysts
work up for child with HSM and no hyperbilirubinemia:
CBC with smear, bone marrow biopsy
Lab signs of hemolytic disease:
elevated unconjugated bilirubin level and an elevated reticulocyte count
What else causes elevated unconjuated bili, elevated reticulocytes besides hemolysis?
CHF, restrictive pericardial disease, and infections
a child with hepatomegaly but not hyperbilirubinemia or splenomegaly
do ultrasonography and serology to rule out cystic or mass lesions and viral or autoimmune hepatitis
Ddx of hepatomegaly plus fever:
Systemic illness; Acute and chronic hepatitis; Viral infections; Hepatic abscess; Hemophagocytic lymphohistiocytosis
Ddx of hepatomegaly plus vomiting and diarrhea:
Reye and Reye-like syndromes; Fatty acid oxidation disorders; Congenital lactic acidemias; Organic acidemias; Urea cycle defects; Glycogen storage disease types 1 and 3; Hereditary fructose intolerance; Fulminant hepatic failure; Wolman disease
What causes cavernous transformation of the portal vein?
portal venous thrombosis
DDX of enlarged liver and spleen
Hepatic disease with portal hypertension leading to splenomegaly
Infection of liver and spleen
Storage disease
Causes of hepatic scarring and portal hypertension in childhood
Biliary atresia
alpha-1-antitrypsin deficiency,
Alagille syndrome
congenital hepatic fibrosis
infections that cause hepatomegaly with splenomegaly
cat-scratch disease, EBV, malaria
malignancy leading to hepatosplenomegaly
histiocytic disorders
storage disorders leading to hepatosplenomegaly
Gaucher disease, Niemann-Pick disease, mucopolysaccharidoses
Hepatosplenomegaly without infantile cholestasis
Niemann-Pick disease
What is Niemann-Pick disease?
Deficiency of sphingomyelinase
What kind of cells are found in Niemann-Pick disease?
Lipid filled histiocytes “foam cells”
How does Niemann Pick disease type A differ from type B?
Type A is more severe
How does type A present?
Severe hypotonia, lymphadenopathy, developmental regression by 6 months of age
How does type B present?
Hepatosplenomegaly in early childhood
symptoms of alagille syndrome
jaundice, peripheral pulmonic stenosis, HSM
when and how does wolman disease present
first few months after birth, with severe FTT, HSM and bilateral calcified adrenal glands