Pregnancy after avascular necrosis of the femur complicating Gaucher's disease.
A patient with type I Gaucher's disease had avascular necrosis of the right femoral head that resulted in an altered bony pelvis and marked restriction of right hip abduction. Enzyme replacement therapy with alglucerase prevented further deterioration and improved thrombocytopenia. Vaginal delivery was achieved with the patient in the left lateral position with exaggerated flexion at the contralateral hip.
Cleary
J E
JE
Department of Obstetrics and Gynecology, Sloane Hospital for Women at the New York Presbyterian Hospital, College of Physicians and Surgeons at Columbia University, New York, New York, USA.
Burke
W M
WM
Baxi
L V
LV
D000328:Adult; D005260:Female; D005271:Femur Head Necrosis / Q000175:diagnosis / Q000209:etiology*; D005776:Gaucher Disease / Q000150:complications* / Q000188:drug therapy; D005962:Glucosylceramidase / Q000008:administration & dosage / Q000627:therapeutic use; D006529:Hepatomegaly / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D011247:Pregnancy; D011248:Pregnancy Complications; D013163:Splenomegaly / Q000175:diagnosis
C070292:alglucerase; D005962:Glucosylceramidase