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CONTEXT: Lipoid congenital adrenal hyperplasia (LCAH) is characterized by a disorder of steroidogenesis in both adrenal glands and gonads. 46,XX patients with classic LCAH usually have thelarche and menarche but show anovulatory menstruations and subsequent premature menopause. Only three patients with classic LCAH have been reported to successfully achieve delivery with the aid of assisted reproductive therapies for conception and progesterone replacement therapy during early pregnancy. In contrast, pubertal development and pregnancy outcomes in patients with nonclassic LCAH have not been fully elucidated. CASE DESCRIPTION: We report four Japanese women who had a diagnosis of primary adrenal insufficiency during infancy or childhood and carried compound heterozygous STAR mutations (p.Gln258* and p.Arg188His, p.Gln258* and p.Met225Thr, and p.Gln258* and p.Arg272Cys). In all four patients, thelarche and menarche spontaneously occurred from 10 to 11 years of age and from 12 to 14 years of age, respectively. Subsequently, their menstruation cycles were regular at almost 1-month intervals. Patient 1 conceived naturally twice, and patient 2 conceived with the use of clomiphene citrate for ovulation induction. These two patients maintained the pregnancies without progesterone replacement therapy and successfully delivered children. CONCLUSION: Patients with nonclassic LCAH maintain ovarian function, which enables normal pubertal development and a successful pregnancy outcome without progesterone replacement therapy.

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Cervicoisthmic pregnancy has a high risk of abortion or preterm delivery, and only 11 cases of live birth have been reported since 1980. In addition, almost all cases require blood transfusion and hysterectomy because of profuse bleeding after delivery of the placenta. A 39-year-old nulliparous woman who became pregnant after a fourth intracytoplasmic sperm injection was diagnosed with cervicoisthmic pregnancy on ultrasonography at 6 weeks' gestation. A healthy neonate was delivered by cesarean section at 32 weeks, but hysterectomy and blood transfusion were required. Perinatal management is discussed.

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Aims: It is well documented that maternal morbidity and neonatal morbidity and mortality increase alike in high-order multiple (HOM) births. There have, however, been few reports concerning the costs of maternal and neonatal medical care associated with HOM births. This is the first such report on the situation in Japan. Materials and methods: All triplet and quadruplet pregnancies managed at this institution from before 16 weeks' gestation, and delivered at no earlier than 22 weeks' gestation, between 1997 and 2002 were included. Prophylactic cervical ligature, hospitalization to prevent premature labor from 23 weeks' gestation until delivery, and delivery by cesarean section, were all routine for HOM pregnancies. All women with singleton and twin pregnancies, who underwent in vitro fertilization (IVF) or intracytoplasmic sperm injection (ICSI) and also delivered their babies at no earlier than 22 weeks' gestation at this institution, between 1997 and 2002, were also examined as controls. Prophylactic cervical ligature, preventive hospitalization, and cesarean section were not routine in the control group. Results: The average gestational ages at delivery in singleton (n = 58), twin (n = 21), triplet (n = 14) and quadruplet (n = 1) pregnancies, were 39.4, 35.6, 31.9 and 25.1 weeks, respectively (P < 0.001 by anova). Birthweights were 2886 ± 425 g, 2117 ± 623 g, 1430 ± 373 g, and 633 ± 77 g (mean ± SD), respectively (P < 0.001). The average inpatient medical care cost for mother and child(ren), from maternal admissions after 12 weeks' gestation to the discharge of all family members from hospital, was ¥703 279 yen (∼US$5861), ¥4 903 270 (∼US$40 861), ¥11 810 327 (∼US$98 419), and ¥44 961 000 (∼US$374 675), respectively (P < 0.001). Conclusion: The present study outlined the high costs of medical care for HOM pregnancies. Not only from a medical viewpoint, but also from the viewpoint of medical costs, it is important to avoid HOM pregnancies as a result of infertility treatment. (Reprod Med Biol 2004; 3: 159-164).