Fetal inheritance of GP*Mur causing severe HDFN in an unrecognized case of maternal alloimmunization.

BACKGROUND: The clinical and laboratory features of hemolytic disease of the newborn can be challenging to diagnose during pregnancy in the apparent absence of a blood group antibody. Low-frequency antibodies go undetected due to the lack of appropriate antigen-positive reagent red blood cells (RBCs). CASE REPORT: A pregnant woman of Southeast Asian descent was referred to a maternal-fetal medicine outpatient clinic due to a complicated obstetric history and a negative antibody screen. This initial visit at 29 weeks and 0 days' gestational age (GA) was unremarkable. A hydropic infant, born at 29 weeks and 5 days' GA, succumbed on the seventh day of life. Comprehensive laboratory testing was performed after birth. The hospital blood bank performed a maternal antibody identification. Direct antiglobulin test was performed on the cord blood. A reference laboratory confirmed an anti-Mia , performed paternal Mia phenotyping, and identified a hybrid glycophorin B-A-B GP*Mur allele. DISCUSSION: Maternal alloimmunization to low-frequency antigens remains a challenge. Southeast Asians make up a significant percentage in some US locations. Worldwide reports on the frequency of maternal alloimmunization of the MNS system can be used to guide the use of specific reagent RBCs for testing. Such strategies rely on the identification of blood donor units for reagent manufacture and use in perinatal antibody screens. CONCLUSION: The incidence of Mia and related antibodies is significant among Southeast Asians. In North America, prenatal antibody screening cells are not routinely chosen to match this population. The clinical and societal implications are discussed.

Genetic Risk of Alzheimer's Disease: Three Wishes Now That the Genie is Out of the Bottle.

The availability and increasing popularity of direct-to-consumer genetic testing for the presence of an APOE4 allelle led the Alzheimer's Foundation of America Medical, Scientific and Memory Screening Advisory Board to identify three critical areas for attention: 1) ensure consumer understanding of test results; 2) address and limit potential negative consequences of acquiring this information; and 3) support linking results with positive health behaviors, including potential clinical trial participation. Improving access to appropriate sources of genetic counseling as part of the testing process is critical and requires action from clinicians and the genetic testing industry. Standardizing information and resources across the industry should start now, with the input of consumers and experts in genetic risk and health information disclosure. Direct-to-consumer testing companies and clinicians should assist consumers by facilitating consultation with genetic counselors and facilitating pursuit of accurate information about testing.

The Relationship of Nosocomial Infection Reduction to Changes in Neonatal Intensive Care Unit Rates of Bronchopulmonary Dysplasia.

OBJECTIVE: To examine whether recent reductions in rates of nosocomial infection have contributed to changes in rates of bronchopulmonary dysplasia (BPD) in a population-based cohort. STUDY DESIGN: This was a retrospective, population-based cohort study that used the California Perinatal Quality Care Collaborative database from 2006 to 2013. Eligible infants included those less than 30 weeks' gestational age and less than 1500 g who survived to 3 days of life. Primary variables of interest were rates of nosocomial infections and BPD. Adjusted rates of nosocomial infections and BPD from a baseline period (2006-2010) were compared with a later period (2011-2013). The correlation of changes in rates across periods for both variables was assessed by hospital of care. RESULTS: A total of 22 967 infants from 129 hospitals were included in the study. From the first to second time period, the incidence of nosocomial infections declined from 24.7% to 15% and BPD declined from 35% to 30%. Adjusted hospital rates of BPD and nosocomial infections were correlated positively with a calculated 8% reduction of BPD rates attributable to reductions in nosocomial infections. CONCLUSIONS: Successful interventions to reduce rates of nosocomial infections may have a positive impact on other comorbidities such as BPD. The prevention of nosocomial infections should be viewed as a significant component in avoiding long-term neonatal morbidities.

Beyond Necrotizing Enterocolitis Prevention: Improving Outcomes with an Exclusive Human Milk-Based Diet.

OBJECTIVE: The aim of this study was to compare outcomes of infants pre and post initiation of a feeding protocol providing an exclusive human milk-based diet (HUM). MATERIALS AND METHODS: In a multicenter retrospective cohort study, infants with a birth weight <1,250 g who received a bovine-based diet (BOV) of mother's own milk fortified with bovine fortifier and/or preterm formula were compared to infants who received a newly introduced HUM feeding protocol. Infants were excluded if they had major congenital anomalies or died in the first 12 hours of life. Data were collected 2-3 years prior to and after introduction of an exclusive HUM diet. Primary outcomes were necrotizing enterocolitis (NEC) and mortality. Secondary outcomes included late-onset sepsis, retinopathy of prematurity (ROP), and bronchopulmonary dysplasia (BPD). RESULTS: A total of 1,587 infants were included from four centers in Texas, Illinois, Florida, and California. There were no differences in baseline demographics or growth of infants. The HUM group had significantly lower incidence of proven NEC (16.7% versus 6.9%, p < 0.00001), mortality (17.2% versus 13.6%, p = 0.04), late-onset sepsis (30.3% versus 19.0%, p < 0.00001), ROP (9% versus 5.2%, p = 0.003), and BPD (56.3% versus 47.7%, p = 0.0015) compared with the BOV group. CONCLUSIONS: Extremely premature infants who received an exclusive HUM diet had a significantly lower incidence of NEC and mortality. The HUM group also had a reduction in late-onset sepsis, BPD, and ROP. This multicenter study further emphasizes the many benefits of an exclusive HUM diet, and demonstrates multiple improved outcomes after implementation of such a feeding protocol.

Platelet count and associated morbidities in VLBW infants with pharmacologically treated patent ductus arteriosus.

OBJECTIVE: Characterize the diagnosis of PDA and the distribution of pretreatment platelet count in pharmacologically managed PDA in infants ≤1500 g and assess the relationship of platelet count to serious morbidities. STUDY DESIGN: This is a retrospective, observational study. In 40 hospitals, data were collected on PDA, including pretreatment platelet count. Distribution of platelet count was examined. The association of platelet count and clinical outcomes of IVH, NEC and PDA closure prior to discharge were examined. Chi-square test was used to compare outcomes by platelet count groups. RESULTS: There were 311 patients treated with medically treated PDA. Pretreatment platelet counts were categorized as 0-119 K, 120-199 K, 200-299 K, >300 K. Incidence and grade of IVH were not significantly different by platelet group. Across all groups: No IVH 62-83%, Grades 1-2 IVH 13-25%, Grades 3-4 IVH 2-13%. NEC occurred in 0-11% of all patients studied. PDA closure rate was 33-45%. CONCLUSION: PDA closure was not significantly affected by platelet count. Platelet count was not a statistically significant factor for development of IVH and NEC in infants born <1500 g with pharmacologically treated PDA.

Aerosol particulate matter in the Baltimore metropolitan area: Temporal variation over a six-year period.

UNLABELLED: This study investigates the sources of fine particulate matter (aerodynamic diameter ≤2.5 µm; PM(2.5)) composition for the Baltimore, Maryland, metropolitan area, covering a 6-year period (2008-2013). Data obtained from the U.S. Environmental Protection Agency (EPA) Air Quality System (AQS) were used for the identification of eight chemical speciation clusters (factors), which, as a percentage of the average concentration, were identified as secondary sulfate (31.9%), secondary nitrate (14.3%), gasoline (17.4%), diesel (10.1%), soil (4.0%), biomass burning (11%), marine aerosol (4.1%), and industrial processing (7.2%). The results show predominant influence from vehicle emissions transiting major highways I-695 and I-95 located in the vicinity of the sampling site. Strong influence on PM2.5 mass from biomass burning was found in the first 2 years (2008-2009) due to particulate matter remnants from forest fire events in North Carolina and a strong contribution in 2013 that was due mainly to wood burning during winter. Sulfate, nitrate, soil, and marine aerosol fractions registered very low variability over the 6-year period analyzed. In addition, this study shows a significant reduction in particulate matter from industrial origins after a major industrial source in Baltimore shut down. The results obtained from Baltimore were compared with those from the Beltsville, Maryland, sampling station located 25 miles south of Baltimore for 2011 and 2012, where good agreement was found for most of the factors. IMPLICATIONS: This paper presents the first long-term aerosol speciation analysis in a Mid-Atlantic United States metropolitan area, which is essential for the air quality management agencies in order to revise regulations and reduce human exposure to adverse air quality conditions. The results suggest that although a declining trend in the overall PM2.5 was observed, no significant tendency was observed in the identified sources besides exceptional events such as the impact of wildfires on local air quality and downward contribution from industrial fraction of PM(2.5) after the Steel Mill at Sparrows Point closure in 2012.

Hospital variation in medical and surgical treatment of patent ductus arteriosus.

OBJECTIVE: This study aims to characterize population risks for diagnosis, medical treatment, and surgical ligation of patent ductus arteriosus (PDA) in very low-birth-weight infants. STUDY DESIGN: Maternal and neonatal data were collected in 40 hospitals in California during 2011 for infants with birth weight ≤ 1,500 g without any congenital malformation, with a diagnosis of PDA. Multivariable logistic regression was used to determine independent risks for PDA diagnosis and for surgical ligation. RESULTS: There were 770/1,902 (40.4%) infants diagnosed with PDA. Low birth weight, gestational age, respiratory distress syndrome, and surfactant administration were associated with PDA diagnosis. Ligation occurred in 43% of patients with birth weight ≤ 750 g, in 24% of patients weighing between 715 and 1,000 g, and in 12% of patients weighing from 1,001 to 1,500 g. Older gestational age (1 week, odds ratio 0.55, 95% confidence interval 0.48-0.63) and absence of respiratory distress syndrome (odds ratio 0.14, 95% confidence interval 0.03-0.59) were associated with lower ligation risk. The median hospital ligation rate was 14% (interquartile range 0-38%). CONCLUSION: Most patients with PDA receive treatment for closure. Practice variation may set the stage for further exploration of experimental trials.

Implementation methods for delivery room management: a quality improvement comparison study.

BACKGROUND: There is little evidence to compare the effectiveness of large collaborative quality improvement versus individual local projects. METHODS: This was a prospective pre-post intervention study of neonatal resuscitation practice, comparing 3 groups of nonrandomized hospitals in the California Perinatal Quality Care Collaborative: (1) collaborative, hospitals working together through face-to-face meetings, webcasts, electronic mailing list, and data sharing; (2) individual, hospitals working independently; and (3) nonparticipant hospitals. The collaborative and individual arms participated in improvement activities, focusing on reducing hypothermia and invasive ventilatory support. RESULTS: There were 20 collaborative, 31 individual, and 44 nonparticipant hospitals caring for 12,528 eligible infants. Each group had reduced hypothermia from baseline to postintervention. The collaborative group had the most significant decrease in hypothermia, from 39% to 21%, compared with individual hospital efforts of 38% to 33%, and nonparticipants of 42% to 34%. After risk adjustment, the collaborative group had twice the magnitude of decrease in rates of newborns with hypothermia compared with the other groups. Collaborative improvement also led to greater decreases in delivery room intubation (53% to 40%) and surfactant administration (37% to 20%). CONCLUSIONS: Collaborative efforts resulted in larger improvements in delivery room outcomes and processes than individual efforts or nonparticipation. These findings have implications for planning quality improvement projects for implementation of evidence-based practices.

The local forces acting on the mechanotransduction channel in hair cell stereocilia.

In hair cells, mechanotransduction channels are located in the membrane of stereocilia tips, where the base of the tip link is attached. The tip-link force determines the system of other forces in the immediate channel environment, which change the channel open probability. This system of forces includes components that are out of plane and in plane relative to the membrane; the magnitude and direction of these components depend on the channel environment and arrangement. Using a computational model, we obtained the major forces involved as functions of the force applied via the tip link at the center of the membrane. We simulated factors related to channels and the membrane, including finite-sized channels located centrally or acentrally, stiffness of the hypothesized channel-cytoskeleton tether, and bending modulus of the membrane. Membrane forces are perpendicular to the directions of the principal curvatures of the deformed membrane. Our approach allows for a fine vectorial picture of the local forces gating the channel; membrane forces change with the membrane curvature and are themselves sufficient to affect the open probability of the channel.

A Lean Six Sigma quality improvement project to increase discharge paperwork completeness for admission to a comprehensive integrated inpatient rehabilitation program.

Lean Six Sigma (LSS) process analysis can be used to increase completeness of discharge summary reports used as a critical communication tool when a patient transitions between levels of care. The authors used the LSS methodology as an intervention to improve systems process. Over the course of the project, 8 required elements were analyzed in the discharge paperwork. The authors analyzed the discharge paperwork of patients (42 patients preintervention and 143 patients postintervention) of a comprehensive integrated inpatient rehabilitation program (CIIRP). Prior to this LSS project, 61.8% of required discharge elements were present. The intervention improved the completeness to 94.2% of the required elements. The percentage of charts that were 100% complete increased from 11.9% to 67.8%. LSS is a well-established process improvement methodology that can be used to make significant improvements in complex health care workflow issues. Specifically, the completeness of discharge documentation required for transition of care to CIIRP can be improved.

Stereocilia membrane deformation: implications for the gating spring and mechanotransduction channel.

In hair cells, although mechanotransduction channels have been localized to tips of shorter stereocilia of the mechanically sensitive hair bundle, little is known about how force is transmitted to the channel. Here, we use a biophysical model of the membrane-channel complex to analyze the nature of the gating spring compliance and channel arrangement. We use a triangulated surface model and Monte Carlo simulation to compute the deformation of the membrane under the action of tip link force. We show that depending on the gating spring stiffness, the compliant component of the gating spring arises from either the membrane alone or a combination of the membrane and a tether that connects the channel to the actin cytoskeleton. If a bundle is characterized by relatively soft gating springs, such as those of the bullfrog sacculus, the need for membrane reinforcement by channel tethering then depends on membrane parameters. With stiffer gating springs, such as those from rat outer hair cells, the channel must be tethered for all biophysically realistic parameters of the membrane. We compute the membrane forces (resultants), which depend on membrane tension, bending modulus, and curvature, and show that they can determine the fate of the channel.

Disaster preparedness: emergency planning in the NICU.

Hospital emergency management has evolved beyond satisfying regulatory requirements. Although tools and resources have been developed to support hospitals in emergency planning, there appears to be a scarcity of resources to guide hospital departments. To ensure that standards of care are maintained and to minimize the impact on the hospital and/ or a nursing unit, Good Samaritan Hospital has developed a mobile emergency system and an Emergency Operations Plan (EOP) template to assist other nursing units in their planning efforts. This article focuses on the development of emergency bedside backpacks, mobile disaster boxes, disaster documentation and forms go-kits, and guidelines for creating such a plan. The ongoing equipment testing, inventory rotation, staff training, and exercising response protocols are all crucial to test the effectiveness of the program in place. All these activities require a multidisciplinary approach to ensure integration with hospital-wide emergency planning efforts.

Nosocomial infection reduction in VLBW infants with a statewide quality-improvement model.

OBJECTIVE: To evaluate the effectiveness of the California Perinatal Quality Care Collaborative quality-improvement model using a toolkit supplemented by workshops and Web casts in decreasing nosocomial infections in very low birth weight infants. DESIGN: This was a retrospective cohort study of continuous California Perinatal Quality Care Collaborative members' data during the years 2002-2006. The primary dependent variable was nosocomial infection, defined as a late bacterial or coagulase-negative staphylococcal infection diagnosed after the age of 3 days by positive blood/cerebro-spinal fluid culture(s) and clinical criteria. The primary independent variable of interest was voluntary attendance at the toolkit's introductory event, a direct indicator that at least 1 member of an NICU team had been personally exposed to the toolkit's features rather than being only notified of its availability. The intervention's effects were assessed using a multivariable logistic regression model that risk adjusted for selected demographic and clinical factors. RESULTS: During the study period, 7733 eligible very low birth weight infants were born in 27 quality-improvement participant hospitals and 4512 very low birth weight infants were born in 27 non-quality-improvement participant hospitals. For the entire cohort, the rate of nosocomial infection decreased from 16.9% in 2002 to 14.5% in 2006. For infants admitted to NICUs participating in at least 1 quality-improvement event, there was an associated decreased risk of nosocomial infection (odds ratio: 0.81 [95% confidence interval: 0.68-0.96]) compared with those admitted to nonparticipating hospitals. CONCLUSIONS: The structured intervention approach to quality improvement in the NICU setting, using a toolkit along with attendance at a workshop and/or Web cast, is an effective means by which to improve care outcomes.

Decreasing central line associated bloodstream infection in neonatal intensive care.

Central Line Associated Bloodstream Infections (CLABSIs) have come to be recognized as preventable adverse events that result from lapses in technique at multiple levels of care. CLABSIs are associated with increased mortality and adverse outcomes that may have lifelong consequences. This review provides a summary of evidence-based strategies to reduce CLABSI in the newborn intensive care unit that have been described in the literature over the past decades. Implementation of these strategies in "bundles" is also discussed, citing examples of successful quality improvement collaboratives. The methods of implementation require an understanding of the scientific data and technical developments, as well as knowledge of how to influence change within the unique and complicated milieu of the newborn intensive care unit.

Neurocognitive predictors of financial capacity across the dementia spectrum: Normal aging, mild cognitive impairment, and Alzheimer's disease.

Financial capacity is a complex instrumental activity of daily living critical to independent functioning of older adults and sensitive to impairment in patients with amnestic mild cognitive impairment (MCI) and Alzheimer's disease (AD). However, little is known about the neurocognitive basis of financial impairment in dementia. We developed cognitive models of financial capacity in cognitively healthy older adults (n = 85) and patients with MCI (n = 113) and mild AD (n = 43). All participants were administered the Financial Capacity Instrument (FCI) and a neuropsychological test battery. Univariate correlation and multiple regression procedures were used to develop cognitive models of overall FCI performance across groups. The control model (R2 = .38) comprised (in order of entry) written arithmetic skills, delayed story recall, and simple visuomotor sequencing. The MCI model (R2 = .69) comprised written arithmetic skills, visuomotor sequencing and set alternation, and race. The AD model (R2 = .65) comprised written arithmetic skills, simple visuomotor sequencing, and immediate story recall. Written arithmetic skills (WRAT-3 Arithmetic) was the primary predictor across models, accounting for 27% (control model), 46% (AD model), and 55% (MCI model) of variance. Executive function and verbal memory were secondary model predictors. The results offer insight into the cognitive basis of financial capacity across the dementia spectrum of cognitive aging, MCI, and AD.

A review of empirical evidence of somatic treatment options for the MI/DD population.

This article reviews recent evidence in the literature for the use of psychotropic and other somatic treatments in the management of patients with mental retardation (MR). The search methodology included peer-reviewed English language publications in PubMed and PsychINFO with the words Mental Retardation, Intellectual Disability, Developmental Disability and Mental Disorders/Drug Therapy or Antipsychotic medications, Psychiatric Somatic therapies, Neuroleptic Drugs, Antidepressants and Electroconvulsive Therapy from 1998 to 2008. The review revealed few randomized controlled trials on the medications frequently prescribed for patients with MR. Three RCTs of Risperidone in children, one combining adults and children and one with adults only are discussed. There was one RCT involving Quetiapine and one on Citalopram. There is little evidence to support the scope of psychotropic medication use in the MR population though the field is advancing. The contribution of psychiatric illness to challenging behaviors is not systematically addressed in the literature.

Disruption of existing mental health treatments and failure to initiate new treatment after Hurricane Katrina.

OBJECTIVE: The authors examined the disruption of ongoing treatments among individuals with preexisting mental disorders and the failure to initiate treatment among individuals with new-onset mental disorders in the aftermath of Hurricane Katrina. METHODS: English-speaking adult Katrina survivors (N=1,043) responded to a telephone survey administered between January and March of 2006. The survey assessed posthurricane treatment of emotional problems and barriers to treatment among respondents with preexisting mental disorders as well as those with new-onset disorders posthurricane. RESULTS: Among respondents with preexisting mental disorders who reported using mental health services in the year before the hurricane, 22.9% experienced reduction in or termination of treatment after Katrina. Among those respondents without preexisting mental disorders who developed new-onset disorders after the hurricane, 18.5% received some form of treatment for emotional problems. Reasons for failing to continue treatment among preexisting cases primarily involved structural barriers to treatment, while reasons for failing to seek treatment among new-onset cases primarily involved low perceived need for treatment. The majority (64.5%) of respondents receiving treatment post-Katrina were treated by general medical providers and received medication but no psychotherapy. Treatment of new-onset cases was positively related to age and income, while continued treatment of preexisting cases was positively related to race/ethnicity (non-Hispanic whites) and having health insurance. CONCLUSIONS: Many Hurricane Katrina survivors with mental disorders experienced unmet treatment needs, including frequent disruptions of existing care and widespread failure to initiate treatment for new-onset disorders. Future disaster management plans should anticipate both types of treatment needs.

Mental health service use among hurricane Katrina survivors in the eight months after the disaster.

OBJECTIVE: This study examined use of mental health services among adult survivors of Hurricane Katrina in order to improve understanding of the impact of disasters on persons with mental disorders. METHODS: A geographically representative telephone survey was conducted between January 19 and March 31, 2006, with 1,043 displaced and nondisplaced English-speaking Katrina survivors aged 18 and older. Survivors who reported serious and mild-moderate mood and anxiety disorders in the past 30 days and those with no such disorders were identified by using the K6 scale of nonspecific psychological distress. Use of services, system sectors, and treatments and reasons for not seeking treatment or dropping out were recorded. Correlates of using services and dropping out were examined. RESULTS: An estimated 31% of respondents (N=319) had evidence of a mood or anxiety disorder at the time of the interview. Among these only 32% had used any mental health services since the disaster, including 46% of those with serious disorders. Of those who used services, 60% had stopped using them. The general medical sector and pharmacotherapy were most commonly used, although the mental health specialty sector and psychotherapy played important roles, especially for respondents with serious disorders. Many treatments were of low intensity and frequency. Undertreatment was greatest among respondents who were younger, older, never married, members of racial or ethnic minority groups, uninsured, and of moderate means. Structural, financial, and attitudinal barriers were frequent reasons for not obtaining care. CONCLUSIONS: Few Katrina survivors with mental disorders received adequate care; future disaster responses will require timely provision of services to address the barriers faced by survivors.