Increased cholesterol and decreased fluidity of red cell membranes (spur cell anemia) in progressive intrahepatic cholestasis.
Pediatrics
; 67(4): 461-6, 1981 Apr.
Article
en En
| MEDLINE
| ID: mdl-7254967
ABSTRACT
Progressive hemolytic anemia occurred in a 4 1/2-year-old girl with familial intrahepatic cholestasis; a peripheral smear contained bizarre spiculated "spur" red cells. Analysis of this patient's fresh red cells revealed a 59% increase in cholesterol content with a normal phospholipid content and therefore an increase in the cholesterol/phospholipid molar ratio to 1.35 (normal = 0.92). A similar abnormality of lipid composition was present in serum lipoproteins. The lipid abnormality in red cell membrane was associated with a decrease in membrane fluidity, as assessed by the fluorescence polarization of the hydrophobic probe 1,6-diphenyl-1,3,5-hexatriene. Following incubation with patient's plasma, normal cells acquired a spur-shaped morphology with an associated decrease in osmotic fragility and a 25% increase in cholesterol content. The patient's cells, during incubation with normal plasma, acquired morphologic features of spiculated spherocytes with an increase in osmotic fragility and a 21% decrease in cholesterol content. Chenodeoxycholate and lithocholate were present in markedly elevated concentrations in serum. These studies show that a process identical to spur cell anemia in alcoholic cirrhosis may accompany severe liver disease in children with intrahepatic cholestasis.
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Banco de datos:
MEDLINE
Asunto principal:
Colestasis Intrahepática
/
Colesterol
/
Eritrocitos Anormales
/
Anemia Hemolítica
Límite:
Child, preschool
/
Female
/
Humans
Idioma:
En
Año:
1981
Tipo del documento:
Article