GYNECOLOGIC AND OBSTETRIC ASPECTS OF GAUCHERS-DISEASE - A SURVEY OF 53 PATIENTS

OBJECTIVE: We report our experience on the gynecologic and obstetric f eatures of 53 female patients with Gaucher's disease. STUDY DESIGN: Ea ch patient was interviewed for a detailed medical history, and all und erwent a complete physical examination and laboratory work-up. RESULTS : Delay of puberty onset was encountered in two thirds of the patients without subsequent infertility. Heavy menstrual bleeding was a major problem and was best treated with low-dose oral contraceptives. Of the 102 spontaneous pregnancies' 25 (24.5%) ended in spontaneous first-tr imesters abortions; 72 continued beyond the twenty-second week. Nine p atients (27.7%) were diagnosed as having Gaucher's disease during thei r first pregnancies. Aggravation of thrombocytopenia and anemia were p rominent features, but antepartum blood transfusion was not required. Early postpartum hemorrhage and fever were increased after both cesare an and vaginal deliveries. Development of bone crisis in seven women d uring the third trimester and early postpartum periods recurred in sub sequent pregnancies. Genotypes had not influenced the gynecologic or o bstetric manifestations. CONCLUSIONS: Gynecologic and obstetric compli cations play a significant role in this patient population, representi ng an additional burden to female patients with Gaucher's disease.