Craniofacial Microsomia, Associated Congenital Anomalies, and Risk Factors in 63 Cases from the Alberta Congenital Anomalies Surveillance System.

OBJECTIVE: To report associated congenital anomalies with unexplained craniofacial microsomia (CFM) and the phenotypic overlap with other recurrent constellations of embryonic malformations (RCEM), and to assess prenatal and perinatal risk factors. STUDY DESIGN: This is a retrospective cross-sectional study. Cases with CFM, delivered between January 1, 1997, and December 31, 2019, were abstracted from the population-based Alberta Congenital Anomalies Surveillance System. Livebirths, stillbirths, and early fetal losses were reviewed to include all types of pregnancy outcomes along the spectrum of this condition. Prenatal and perinatal risk factors were compared with the Alberta birth population to assess differences between the 2 groups. RESULTS: There were 63 cases with CFM, yielding a frequency of 1 per 16 949. There was a high rate of cases (65%) with anomalies outside the craniofacial and vertebral regions. Congenital heart defects were the most common (33.3%). A single umbilical artery was found in 12.7% of cases. The twin/triplet rate of 12.7% was significantly higher than the Alberta rate of 3.3% (P < .0001). There was an overlap with a second RCEM condition in 9.5% of cases. CONCLUSIONS: Although CFM is primarily a craniofacial condition, the majority of cases have congenital anomalies affecting other systems requiring additional assessments, including an echocardiogram, renal ultrasound examination, and a complete vertebral radiograph. The high rate of an associated single umbilical artery raises the possibility of a related etiological mechanism. Our findings support the proposed concept of RCEM conditions.

The multi-faceted dynamics of HIV-1 transmission in Northern Alberta: A combined analysis of virus genetic and public health data.

Molecular epidemiology has become a key tool for tracking infectious disease epidemics. Here, the spread of the most prevalent HIV-1 subtypes in Northern Alberta, Canada, was characterized with a Bayesian phylogenetic approach using 1146 HIV-1 pol sequences collected between 2007 and 2013 for routine clinical management purposes. Available patient metadata were qualitatively interpreted and correlated with onwards transmission using Fisher exact tests and logistic regression. Most infections were from subtypes A (n=36), B (n=815) and C (n=211). Africa is the dominant origin location for subtypes A and C while the subtype B epidemic was seeded from the USA and Middle America and, from the early 1990s onwards, mostly by interprovincial spread. Subtypes A (77.8%) and C (74.0%) were usually heterosexually transmitted and circulate predominantly among Blacks (61.1% and 85% respectively). Subtype B was mostly found among Caucasians (48.6%) and First Nations (36.8%), and its modes of transmission were stratified by ethnic origin. Compared to subtypes A (5.6%) and C (3.8-10.0%), a larger portion of subtype B patients were found within putative provincial transmission networks (20.3-29.5%), and this almost doubled when focusing on nationwide transmission clusters (37.9-57.5%). No clear association between cluster membership and particular patient characteristics was found. This study reveals complex and multi-faceted transmission dynamics of the HIV-1 epidemic in this otherwise low HIV prevalence population in Northern Alberta, Canada. These findings can aid public health planning.

Gestational age at birth and risk of autism spectrum disorders in Alberta, Canada.

OBJECTIVE: To examine the association between autism spectrum disorders (ASD) and each completed week of gestation using a graphical method of presenting results at all possible categorizations of gestational age (GA). STUDY DESIGN: The risk of ASD in a total of 218110 singleton live births with complete data from Alberta, Canada between 1998 and 2004 was examined through linkage to health insurance records. The relative risk of developing ASD according to the 21 dichotomizations of shorter gestation (GA ≤ 23 weeks vs >23 weeks to ≤ 43 weeks vs >43 weeks, in 1-week increments) was calculated using log-binomial regression and adjusted for fetal sex, socioeconomic status, and birth year. RESULTS: We observed a gradual increased risk of ASD with shorter gestation. Cutoffs only between 29 and 40 weeks clearly denoted an elevated risk of developing ASD compared with longer gestation, and the risk increased with earlier GA cutoff. The results were not affected by sex or measures of fetal growth. CONCLUSION: Our data confirm the role of shortened gestation in ASD risk. We warn against the use of prespecified or a data-driven GA cutoff, however; instead, we recommend systematically examining all plausible cutoffs for GA to avoid overstating the homogeneity of risk in children on either side of a given cutoff, as well as to increase the comparability of studies.

Neonatal and neurodevelopmental outcomes of very low birth weight infants with histologic chorioamnionitis.

OBJECTIVE: To determine survival and neurodevelopmental outcomes at 18 months corrected age among very low birth weight infants ≤ 32 weeks gestation with histologic chorioamnionitis. STUDY DESIGN: Observational, regionalized, single-center cohort study with prospective follow-up. RESULTS: Of the 628 infants meeting the selection criteria, 303 (48%) were born to mothers with evidence of histologic chorioamnonitis. Neonates with histologic chorioamnonitis were of lower gestational age and birth weight. On univariate analysis, they were more likely to have hypotension, bronchopulmonary dysplasia, severe intraventricular hemorrhage, severe retinopathy of prematurity, early-onset sepsis, and death. Infants with histologic chorioamnonitis were more likely to have any neurodevelopmental impairment, specifically, mental delay with a lower mental developmental index. When adjusting for perinatal variables, histologic chorioamnonitis had a protective effect on mortality rates (adjusted OR = 0.44, 95% CI: 0.24-0.8; P = .01; n = 619), had a nonsignificant effect on neurodevelopmental impairment (adjusted odds ratio = 1.33, 95% CI: 0.82-2.17; P = .25; n = 496), and was associated with a 4-point lower mental developmental index at 18-months follow-up (adjusted difference -3.93, 95% CI: -7.52 to -0.33; P = .03; n = 496). CONCLUSIONS: Although infants with histologic chorioamnonitis were at an increased risk for death and neurodevelopmental impairment, after multivariate analyses, histologic chorioamnonitis was not associated with adverse long-term outcomes. Results suggest fetal protection from treatment-responsive maternal infection and inflammation.

Travelers' knowledge of prevention and treatment of travelers' diarrhea.

BACKGROUND: Information regarding the prevention and treatment of travelers' diarrhea (TD) is available to the public from various sources, such as medical personnel, travel clinics, personal contacts, and the Internet. This type of information may help travelers avoid this illness or help those afflicted minimize its duration. METHODS: We collected questionnaire data from 104 travelers at departure gates for flights to Mexico from Calgary, Alberta on their knowledge of symptoms and treatment of TD and food risks associated with this illness and sources of information used. RESULTS: Almost half reported they received some information on travel-related diseases and on TD prior to the flight. When education level was controlled for, the mean score for people who had obtained information on TD was significantly higher than that for those who did not have such information. College or university-educated travelers scored better than did other travelers. A high proportion of travelers correctly identified risk levels associated with specific foods consumed during travel, and many recognize that they are at an increased risk of acquiring diarrheal illness while traveling in a developing country. CONCLUSIONS: Information on TD appears to improve the level of knowledge on its prevention and treatment among travelers from southern Alberta.

Effects of in vitro fertilization on low birth weight, preterm delivery, and multiple birth.

PURPOSE: To quantify the contribution of in vitro fertilization (IVF) on changes in the rates of low birth weight (LBW), preterm delivery, very low birth weight, and multiple births during the past 3 years. METHODS: Data on IVF pregnancies from 1994 to 1996 within Alberta were reviewed. Population data were obtained from the Provincial notice of a live or stillbirth. RESULTS: The IVF component of increased LBW rate in the province was 17.8% for infants <2500 g and 43.5% for those born <1500 g. IVF accounted for 10.5% of the provincial rate increase in deliveries <37 weeks' gestation and 66.2% of those <30 weeks' gestation. IVF accounted for 21.4% of the twins and all of the sets of triplets in the province. CONCLUSION: During a 3-year period IVF has affected the incidence of LBW, preterm delivery, and multiple birth. IVF is a substantial contributor to changes in very low birth weight and delivery before 30 weeks, which is partly related to multiple births.

Prolonged use of pancuronium bromide and sensorineural hearing loss in childhood survivors of congenital diaphragmatic hernia.

Sensorineural hearing loss (SNHL) is a significant neurologic morbidity in survivors of neonatal congenital diaphragmatic hernia (CDH), with a reported incidence of up to 60%. In a historical cohort study of 37 neonates with CDH, we investigated the use of pancuronium bromide (PB) and common ototoxic drugs during the neonatal period and their relationship to SNHL in childhood survivors. Survivors with SNHL (n = 23) had significantly higher cumulative dose of PB administered during the neonatal illness than survivors without SNHL (n = 14). The cumulative dose and duration of PB use significantly correlated (r = 0.66-0.81) and independently predicted (adjusted r (2) = 0.42-0.64) the greatest intensity (in decibels) and the widest band (lowest frequency in hertz) loss of SNHL. No differences were identified between survivors with and without SNHL regarding demographic and neonatal characteristics (including oxygenation and ventilation variables and the cumulative dose and duration of therapy with aminoglycosides, vancomycin, and furosemide), although survivors with SNHL had received a modestly higher cumulative dose of ethacrynic acid than survivors without SNHL. Although we show that prolonged administration of PB during the neonatal period is associated with SNHL in childhood survivors of CDH, further multicenter studies are required to investigate the possible etiologies of SNHL in this high-risk population.

Risk of hemolytic uremic syndrome after sporadic Escherichia coli O157:H7 infection: results of a Canadian collaborative study. Investigators of the Canadian Pediatric Kidney Disease Research Center.

OBJECTIVES: The objectives of this study were to better estimate the age-specific risks of hemolytic uremic syndrome (HUS) and hemolytic anemia after Escherichia coli O157:H7 infection among a representative cohort of both referred and nonreferred children with documented illness from the province of Alberta and to compare this with the rates in children evaluated at referral centers in the rest of Canada. STUDY DESIGN: Children with HUS or E. coli O157:H7 gastroenteritis were eligible if they were < 15 years of age. Hemoglobin, blood smear, urinalysis, and serum creatinine were obtained 8 to 10 days after the onset of diarrhea to ascertain for hemolysis, anemia, thrombocytopenia, and renal injury. Subjects were monitored for 1 month. RESULTS: From June 1991 to March 1994, HUS was diagnosed in 205 children. Of these 77% had evidence of E. coli O157:H7 infection. A further 582 children had E. coli O157:H7 gastroenteritis, of whom 18 had hemolytic anemia. The risk of HUS after E. coli O157:H7 infection in Alberta was 8.1% (95% confidence interval, 5.3 to 11.6) compared with 31.4% in referral centers in the rest of Canada. In Alberta the highest age-specific risk of HUS/hemolytic anemia was 12.9% in those < 5 years of age. CONCLUSIONS: These data will help guide clinical care and provide a basis for estimating the sample sizes required in future treatment trials for the secondary prevention of HUS.

No resuscitation and withdrawal of therapy in a neonatal and a pediatric intensive care unit in Canada.

STUDY OBJECTIVE: To compare and contrast the modes of death in a neonatal (NICU) and a pediatric (PICU) intensive care unit. DESIGN: Retrospective analysis of patient records. SUBJECTS: All newborn infants and children (< 17 years of age) who died in the NICU and PICU at the University of Alberta Hospitals, Edmonton, between Jan. 1, 1990, to Dec. 31, 1991. RESULTS: The mortality rate in the PICU was 8.7% (73/839) compared with 5.6% (75/1333) in the NICU (p = 0.007). Withdrawal of therapy was the most common cause of death in both units and occurred more commonly in the NICU (NICU = 69% vs PICU = 34%; p = 0.01). There were significantly more deaths as a result of failed cardiopulmonary resuscitation (CPR) in the PICU than in the NICU (29% vs 13%; p = 0.046). Death after no-CPR orders occurred with equal frequency in both units (NICU 17%; PICU 15%). Brain death accounted for 22% (16/87) of PICU deaths; no infant in the NICU was declared brain dead (p < 0.05). When deaths resulting from brain death and failed CPR were excluded, there was no significant difference between the two units regarding withdrawal of therapy (NICU 80% vs PICU 69%) and no-CPR orders (NICU 20% vs PICU 30%). CONCLUSIONS: This study confirms that both withdrawal of therapy and no-CPR orders are part of current clinical practice in both the NICU and PICU settings. The ethical foundations and implications of these practices need further elaboration.