[Homozygous familial hypercholesterolemia. Response to combined cholestyramine and lovastatin therapy].
Homozygous familiar hypercholesterolemia (FH) is a serious inherited disease caused by a genetic defect in the cell surface receptor that controls the degradation of low density lipoprotein (LDL). These patients often have myocardial infarction in their teens or early adulthood and are usually unresponsive to drugs. Recently it has been reported promising results using combined drugs regimens in patients with residual receptor activity. We report a new additional patient with receptor-defective homozygous FH treated with a combination of lovastatin and cholestyramine. The cholesterol levels were reduced in a 67% and there were adverse events related to treatment during a 7 month period of follow-up.
Obando Santaella
I
I
Servicio de Pediatría, Hospital G B de Pozoblanco, Córdoba.
Fernández Gómez
E
E
Mongil Ruiz
I
I
Escobar Gallego
A
A
García Alegría
J J
JJ
Gascón Luna
F
F
Masana Marín
L
L
D002675:Child, Preschool; D002792:Cholestyramine Resin / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000188:drug therapy / Q000235:genetics*; D008148:Lovastatin / Q000627:therapeutic use*; D008297:Male
D002792:Cholestyramine Resin; D008148:Lovastatin
Hipercolesterolemia familiar homocigota. Respuesta al tratamiento combinado con colestiramina y lovastatina.