Dual diagnosis of trisomy 21 and lethal perinatal Gaucher disease as a cause of non-immune hydrops fetalis in a twin pregnancy for a consanguineous couple.

Non-immune hydrops is a prenatal finding which can occur due to an underlying genetic diagnosis such as common chromosomal aneuploidy (Trisomy 21, Turner syndrome etc.). It is extremely rare to have more than one genetic cause of hydrops fetalis in a single pregnancy. This report describes a dichorionic diamniotic pregnancy for a consanguineous couple where noninvasive prenatal testing was "high risk" for Trisomy 21. Family declined amniocentesis and opted for postnatal genetic testing. The pregnancy was later complicated with severe hydrops fetalis leading to demise for one of the twins, and a premature delivery of the other twin who had remarkable collodion not in keeping with Trisomy 21. Postnatal genetic investigations confirmed both Trisomy 21 and prenatal lethal Gaucher disease in the survivor twin. This case report highlights some of the prenatal diagnostic challenges for a consanguineous couple where a rare cause of fetal hydrops was concealed in a setting of a common chromosomal aneuploidy. The prompt postnatal diagnosis of perinatal lethal Gaucher disease, confirmed with undetectable glucocerebrosidase enzyme activity, assisted the family in the decision of providing palliative care for their infant who was quickly deteriorating. The importance of postnatal genetic evaluation and its impact on immediate patient management in an NICU setting is emphasized. This dual diagnosis was significant for the couple as it explained pervious pregnancy losses and has important future recurrence risk implications.

Atonic Postpartum Hemorrhage: Blood Loss, Risk Factors, and Third Stage Management.

OBJECTIVE: Atonic postpartum hemorrhage rates have increased in many industrialized countries in recent years. We examined the blood loss, risk factors, and management of the third stage of labour associated with atonic postpartum hemorrhage. METHODS: We carried out a case-control study of patients in eight tertiary care hospitals in Canada between January 2011 and December 2013. Cases were defined as women with a diagnosis of atonic postpartum hemorrhage, and controls (without postpartum hemorrhage) were matched with cases by hospital and date of delivery. Estimated blood loss, risk factors, and management of the third stage labour were compared between cases and controls. Conditional logistic regression was used to adjust for confounding. RESULTS: The study included 383 cases and 383 controls. Cases had significantly higher mean estimated blood loss than controls. However, 16.7% of cases who delivered vaginally and 34.1% of cases who delivered by Caesarean section (CS) had a blood loss of < 500 mL and < 1000 mL, respectively; 8.2% of controls who delivered vaginally and 6.7% of controls who delivered by CS had blood loss consistent with a diagnosis of postpartum hemorrhage. Factors associated with atonic postpartum hemorrhage included known protective factors (e.g., delivery by CS) and risk factors (e.g., nulliparity, vaginal birth after CS). Uterotonic use was more common in cases than in controls (97.6% vs. 92.9%, P < 0.001). Delayed cord clamping was only used among those who delivered vaginally (7.7% cases vs. 14.6% controls, P = 0.06). CONCLUSION: There is substantial misclassification in the diagnosis of atonic postpartum hemorrhage, and this could potentially explain the observed temporal increase in postpartum hemorrhage rates.

The Impact of Standardized Acuity Assessment and a Fast-Track on Length of Stay in Obstetric Triage: A Quality Improvement Study.

To prospectively assess the impact of a standardized 5-category Obstetrical Triage Acuity Scale (OTAS) and a fast-track for lower-acuity patients on patient flow. Length of stay (LOS) data of women presenting to obstetric triage were abstracted from the electronic medical record prior to (July 1, 2011, to March 30, 2012) and following OTAS implementation (April 1 to December 31, 2012). Following computerized simulation modeling, a fast-track for lower acuity women was implemented (January 1, 2013, to February 28, 2014). Prior to OTAS implementation (8085 visits), the median LOS was 105 (interquartile range [IQR] = 52-178) minutes. Following OTAS implementation (8131 visits), the median LOS decreased to 101 (IQR = 49-175) minutes (P = .04). The LOS did not correlate well with acuity. Simulation modeling predicted that a fast-track for OTAS 4 and 5 patients would reduce the LOS. The LOS for lower-acuity patients in the fast-track decreased to 73 (IQR = 40-140) minutes (P = .005). In addition, the overall LOS (12 576 visits) decreased to 98 (IQR = 47-172) minutes (6.9% reduction; P < .001). Standardized assessment of acuity and a fast-track for lower acuity pregnant women decreased the overall LOS and the LOS of lower-acuity patients.

Acuity Assessment in Obstetrical Triage.

OBJECTIVE: A five-category Obstetrical Triage Acuity Scale (OTAS) was developed with a comprehensive set of obstetrical determinants. The purposes of this study were: (1) to compare the inter-rater reliability (IRR) in tertiary and community hospital settings and measure the intra-rater reliability (ITR) of OTAS; (2) to establish the validity of OTAS; and (3) to present the first revision of OTAS from the National Obstetrical Triage Working Group. METHODS: To assess IRR, obstetrical triage nurses were randomly selected from London Health Sciences Centre (LHSC) (n = 8), Stratford General Hospital (n = 11), and Chatham General Hospital (n= 7) to assign acuity levels to clinical scenarios based on actual patient visits. At LHSC, a group of nurses were retested at nine months to measure ITR. To assess validity, OTAS acuity level was correlated with measures of resource utilization. RESULTS: OTAS has significant and comparable IRR in a tertiary care hospital and in two community hospitals. Repeat assessment in a cohort of nurses demonstrated significant ITR. Acuity level correlated significantly with performance of routine and second order laboratory investigations, point of care ultrasound, nursing work load, and health care provider attendance. A National Obstetrical Triage Working Group was formed and guided the first revision. Four acuity modifiers were added based on hemodynamics, respiratory distress, cervical dilatation, and fetal well-being. CONCLUSION: OTAS is the first obstetrical triage scale with established reliability and validity. OTAS enables standardized assessments of acuity within and across institutions. Further, it facilitates assessment of patient care and flow based on acuity.

Implementing an obstetric triage acuity scale: interrater reliability and patient flow analysis.

A 5-category Obstetric Triage Acuity Scale (OTAS) was developed with a comprehensive set of obstetrical determinants. The objectives of this study were as follows: (1) to test the interrater reliability of OTAS and (2) to determine the distribution of patient acuity and flow by OTAS level. To test the interrater reliability, 110 triage charts were used to generate vignettes and the consistency of the OTAS level assigned by 8 triage nurses was measured. OTAS performed with substantial (Kappa, 0.61 - 0.77, OTAS 1-4) and near perfect correlation (0.87, OTAS 5). To assess patient flow, the times to primary and secondary health care provider assessments and lengths of stay stratified by acuity were abstracted from the patient management system. Two-thirds of triage visits were low acuity (OTAS 4, 5). There was a decrease in length of stay (median [interquartile range], minutes) as acuity decreased from OTAS 1 (120.0 [156.0] minutes) to OTAS 3 (75.0 [120.8]). The major contributor to length of stay was time to secondary health care provider assessment and this did not change with acuity. The percentage of patients admitted to the antenatal or birthing unit decreased from 80% (OTAS 1) to 12% (OTAS 5). OTAS provides a reliable assessment of acuity and its implementation has allowed for triaging of obstetric patients based on acuity, and a more in-depth assessment of the patient flow. By standardizing assessment, OTAS allows for opportunities to improve performance and make comparisons of patient care and flow across organizations.

IL-6 and TNFalpha across the umbilical circulation in term pregnancies: relationship with labour events.

OBJECTIVE: We have determined venous and arterial cord blood levels for IL-6 and TNFalpha at the time of delivery to assess gestational tissue versus fetal sources in labouring and non-labouring patients at term, and the relationship to labour events. METHODS: Fifty-five patients were studied (elective cesarean section n=24, and labouring n=31) with blood sampling from a clamped segment of cord after delivery of the fetus and from the cord at its insertion into the placenta after delivery of the placenta, with subsequent measurement of blood gases, pH, IL-6 and TNFalpha. RESULTS: Umbilical cord levels for IL-6 were increased by 4 fold in low risk labouring patients, and a further 6 fold when showing histologic chorioamnionitis, but with no evident effect of nuchal cord with 'variable' fetal heart rate decelerations, fetal acidemia, nor of labour duration. IL-6 levels from the cord at its insertion into the placenta were generally higher than those from the respective umbilical levels indicating that placental release of IL-6 into cord blood must be occurring. However, a consistent venoarterial difference for IL-6 and thereby a net flux from the placenta could not be demonstrated. TNFalpha levels for both patient groups were uniformly low for all of the cord measurements with no significant differences noted. CONCLUSION: Umbilical cord levels for IL-6 are increased in low risk labouring patients at term in the absence of evident infection which likely involves both gestational tissue and fetal contributions. Cord levels for IL-6 are further increased in low risk labouring patients showing histologic chorioamnionitis which might then contribute to newborn morbidity in these pregnancies.

Risk of pregnancy-related hypertension within five years of exposure to bacteria-contaminated drinking water.

Exposure to Escherichia coli O157:H7 may result in subclinical kidney injury manifesting as hypertension during pregnancy. We evaluated the risk of pregnancy-related hypertension (PRH) among previously healthy females from the Walkerton Health Study, Canada (2002-6), who conceived within five years of exposure to bacteria-contaminated drinking water. Ontario Ministry of Health Antenatal forms were used to determine outcomes and risk factors. PRH was defined as any systolic or diastolic blood pressure (BP) > or =140 mm Hg and > or =90 mm Hg, respectively. Chronic and gestational hypertension were defined, respectively, as elevated BP observed prior to or at > or =20 weeks gestation. Risk of PRH was evaluated using a modified Poisson regression model that controlled for known risk factors. Of 148 eligible pregnancies, antenatal audits with blood pressure data were available for 135. PRH was detected in 20.7% pregnancies, of which 6.7% were chronic hypertension and 14.1% gestational hypertension. Although nonsignificant, we observed a consistent trend toward higher rates of PRH and mean arterial pressure, particularly prior to 20 weeks gestation, among women who reported symptomatic gastroenteritis compared to asymptomatic women. BP should be monitored closely in women after exposure to contaminated water.