Are current cut-off values of 11-DOC in children useful for assessing suspected nonclassical congenital adrenal hyperplasia due to 11ß-hydroxylase deficiency?

OBJECTIVE: A nonclassic form of 11ß-hydroxylase deficiency (NC11ß-OHD) has been reported to cause mild androgen excess symptoms. Currently, the gold standard for biochemical diagnosis is elevated 11-deoxycortisol (11-DOC) levels after corticotropin stimulation test (ACTHstimT). However, there are no clear 11-DOC level cutoffs. One of the accepted references for 11-DOC levels for the paediatric population was published in 1991 by Lashansky et al. AIM: To determine the correlation between 11-DOC levels measured during ACTHstimT and clinical symptoms attributed to NC11ß-OHD. DESIGN: A retrospective study including all paediatric patients who underwent ACTHstimT at Shamir Medical Center between 2007 and 2015. Clinical data were collected from the patients' medical files. Outcome measures included the number of patients with hyperandrogenism signs and predefined elevated 11-DOC cut-off levels according to Lashansky for sex and age, and according to commercial kit cut-offs. RESULTS: Data were complete at presentation for 136 patients. Long-term clinical data were documented for 98 patients, mean follow-up duration of 3.1 years (1.37-5.09). There was no statistically significant difference in the number of cases with elevated 11-DOC according to both cut-offs and early puberty, premature adrenarche nor acne. Follow-up data demonstrated no statistically significant difference in the number of cases with elevated 11-DOC levels among patients with compromised final adult height, polycystic ovarian syndrome or hyperandrogenism. CONCLUSIONS: Basal and corticotropin stimulated 11-DOC levels were not significantly elevated above the 1.5 times cut-offs according to paediatric-specific norms or the commercial assay in paediatric individuals with possible clinical suspicion of NC11ß-OHD.

Precipitous delivery complicated by uterine artery laceration and uterine rupture in an unscarred uterus: A case report.

Precipitous delivery is associated with rapid cervical dilation and fetal descent. Complications of precipitous delivery can include vascular trauma, uterine rupture, and uterine artery laceration. Uterine artery laceration is a rare complication that can lead to significant postpartum hemorrhage and injury. Careful evaluation for trauma and aggressive resuscitation are critical to prevent maternal morbidity and mortality. This is a case report of a 39-year-old woman, gravida 2 para 1, at 39 weeks of gestation who delivered after induction of labor due to chronic hypertension. Her labor course was precipitous and complicated by uterine rupture and uterine artery laceration with postpartum hemorrhage that required massive transfusion, exploratory laparotomy with a supracervical hysterectomy, and interventional radiology for uterine and cervical artery embolization. This seems to be the first published case report of precipitous delivery associated with uterine artery laceration and uterine rupture. Thorough evaluation after precipitous delivery is critical to decrease maternal morbidity and mortality secondary to uterine artery injury.