No abstract

Arthrogryposis multiplex congenita (AMC) refers either to a syndromic or to a nonsyndromic group of conditions with varied etiology and complex clinical features, including multiple congenital contractures in different body areas. Its etiology still remains unclear but generally any cause that leads to reduced fetal movement may lead to congenital contractures and in severe cases to fetal akinesia deformation sequence (FADS). It affects approximately 1 in 2-3000 live births with an approximately equal gender ratio. There are many known subgroups of AMC differing in signs, symptoms, and causes. The primary diagnosis is made when a lack of mobility and an abnormal position is noted in routine ultrasound scanning. Early diagnosis, prenatal evaluation, and further surveillance via image scanning (ultrasound and MRI) give the opportunity for family counseling concerning neonatal morbidity and mortality and labor or delivery planning. Better understanding of the ultrasound findings and the etiology of this clinical situation offers the opportunity for careful prenatal assessment.

Objective: To assess the association between endogenous sex hormones and risk factors for atherosclerosis in healthy postmenopausal women. Design: Cross-sectional study in a university menopause clinic. Methods: Serum sex hormones and lipid-lipoprotein profile, arterial pressure, homocysteine and insulin resistance, measured by the homeostasis model assessment of insulin resistance (HOMA-IR), were assessed in 598 healthy postmenopausal women not on hormone therapy. Results: Compared with women in the lowest testosterone quartile (Q), women in the highest testosterone quartile had higher total cholesterol (Q1: 225.2G41.3 vs Q4: 246.2G38.4 mg/dl, P!0.01), low-density lipoprotein (LDL)-cholesterol (Q1: 146.9G37.2 vs Q4: 171.8G35.3 mg/dl, P!0.001), atherogenic index of plasma (AIP) (Q1: -0.224G0.238 vs Q4: -0.087G0.254, P!0.01), apolipoprotein B (ApoB) (Q1: 100.7G23.1 vs Q4: 113.9G23.8 mg/dl, P!0.001) and higher highdensity lipoprotein (HDL)-cholesterol (Q1: 60.7G14.5 vs Q4: 52.9G13.0 mg/dl, P!0.01). Accordingly, women in the highest free androgen index (FAI) quartile had higher AIP (Q1: -0.232G0.254 vs Q4: -0.078G0.243, P!0.001) and ApoB (Q1: 102.4G25.5 vs Q4: 114.2G 25.8 mg/dl, P!0.01) and lower HDL-cholesterol (Q1: 62.0G15.7 vs Q4: 51.9G11.6 mg/dl, P!0.001) and apolipoprotein A (Q1: 159.6G25.6 vs Q4: 147.9G24.1 mg/dl, P!0.01) compared with women in the lowest FAI quartile. These differences remained significant after adjustment for age, body mass index (BMI), insulin resistance and social habits. The free estrogen index (FEI) exhibited similar associations to the FAI. HOMA-IR showed an independent positive association with total testosterone (Q1: 2.00G1.36 vs Q4: 2.66G1.60, P!0.01), FAI (Q1: 1.70G1.12 vs Q4: 3.04G1.66, P!0.001) and FEI (Q1: 1.70G0.91 vs Q4: 3.08G1.77, P!0.001). Conclusions: Increased androgenicity in healthy postmenopausal women is associated with an unfavorable cardiovascular risk profile. High endogenous estradiol is related to a pro-atherogenic lipid profile, an association which may, in part, be mediated by insulin resistance. 154 907-916 European Journal of Endocrinology

The needs of the uterus and the fetus for the provision of nutrients and oxygen, supplied by the blood flow, are understandably extremely high, with the circulatory system playing the most important role in this action. Abnormal vascular growth and transformation that create a high vessel resistance network have been associated with various pregnancy pathologies, including miscarriage, small for gestational age (SGA) fetuses with or without preeclampsia and intrauterine growth restriction (IUGR). Placental growth factor (PlGF) has a major role in vasculogenesis and angiogenesis in human placenta. Low concentrations of PlGF and high concentrations of its inhibitor-soluble Fms-like tyrosine kinase-1 (sFlt-1) are linked with impaired angiogenesis and placental development, leading to the above pregnancy complications. The activity of vascular endothelial growth factor (VEGF), which is the most potent of all angiogenic mediators, is partly modulated by PlGF. Although the mechanisms via which PlGF exerts its various effects are still under investigation, we herein discuss the known actions exerted by this major mediator together with its results on fetal growth.