Ultrasound prediction of birthweight and growth restriction in fetal gastroschisis.

OBJECTIVE: Most ultrasound estimated fetal weight (EFW) formulas incorporate abdominal circumference, which may overstimate growth restriction in fetal gastroschisis. The aim of this study was to determine the optimal ultrasound formula for prediction of birthweight and fetal growth restriction (FGR) in gastroschisis. STUDY DESIGN: We conducted a retrospective cohort analysis of singleton fetuses with gastroschisis. Percentage of error between ultrasound EFW (performed within 2 weeks of delivery) and birthweight was calculated. Agreement between EFW by ultrasound formulas and birthweight was determined by Bland-Altman limits of agreement; concordance between ultrasound and birthweight diagnosis of FGR was evaluated with McNemar's test. RESULTS: Birthweight was best predicted by the formulas of Shepard et al and Siemer et al. Only these formulas demonstrated significant agreement with birthweight for prediction of FGR at the 5th and 10th percentiles. CONCLUSION: The formulas of Shepard et al and Siemer et al best estimate birthweight, and their use has the potential to reduce rates of overdiagnosis of FGR.

Invasive squamous cell carcinoma of the vagina in the setting of true hermaphroditism.

BACKGROUND: Primary vaginal cancer constitutes less than 2% of all malignancies of the female genital tract and is even rarer in true hermaphrodites. A unique case report of the occurrence of both of these developments in the same patient is presented. CASE: A 31-year-old 46,XX hermaphrodite presented with a history of vague abdominal symptoms and weight loss. Surgery was performed, and pathology revealed invasive squamous cell cancer arising in an epithelial-lined tissue consistent with vagina. The patient subsequently completed whole pelvic external-beam radiation therapy with cisplatin chemosensitization. CONCLUSION: This is a case of primary vaginal cancer in a patient with true hermaphroditism, which is exceedingly rare. It is a reminder that malignant changes in residual mullerian tissue should be considered in these patients.

Differential effects of Drosophila mastermind on asymmetric cell fate specification and neuroblast formation.

During neurogenesis in the ventral nerve cord of the Drosophila embryo, Notch signaling participates in the pathway that mediates asymmetric fate specification to daughters of secondary neuronal precursor cells. In the NB4-2 --> GMC-1 --> RP2/sib lineage, a well-studied neuronal lineage in the ventral nerve cord, Notch signaling specifies sib fate to one of the daughter cells of GMC-1. Notch mediates this process via Mastermind (Mam). Loss of function for mam, similar to loss of function for Notch, results in GMC-1 symmetrically dividing to generate two RP2 neurons. Loss of function for mam also results in a severe neurogenic phenotype. In this study, we have undertaken a functional analysis of the Mam protein. We show that while ectopic expression of a truncated Mam protein induces a dominant-negative neurogenic phenotype, it has no effect on asymmetric fate specification. This truncated Mam protein rescues the loss of asymmetric specification phenotype in mam in an allele-specific manner. We also show an interallelic complementation of loss-of-asymmetry defect. Our results suggest that Mam proteins might associate during the asymmetric specification of cell fates and that the N-terminal region of the protein plays a role in this process.