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The Covid-19 pandemic challenged health care delivery systems worldwide. Many acute care hospitals in communities that experienced surges in cases and hospitalizations had to make decisions such as rationing scarce resources. Hospitals serving low-income communities, communities of color, and those in other historically marginalized or vulnerable groups reported the greatest operational impacts of surges. However, cross-institutional collaborations within jurisdictions offer unique opportunities to prevent or mitigate health disparities in resource utilization and access to care. In January 2020, in response to the emerging coronavirus epidemic, the San Francisco Department of Public Health (SFDPH) and local hospital and health systems partners convened to align and coordinate medical surge planning and response. Adopting a governance structure of mutual accountability and transparency, the San Francisco Health Systems Collaborative guided local medical and public health response in the areas of medical surge, vaccination administration, testing, and therapeutics. Four principles guided the collaborative response: (1) shared priorities, (2) clear governance and accountability, (3) data transparency, and (4) operational coordination. High-level priorities established included protecting vulnerable people, protecting health care workers, and maintaining health system capacity. The governance structure consisted of three layers: local hospital and health systems CEOs coordinating with SFDPH executives; hospital chief medical and nursing officers coordinating high-level surge capacity assessments and mitigation plans; and local clinical operational managers working with public health response operational leaders to coordinate scarce resource utilization. Fluctuating with the tempo of the disease indicators and medical surge, governance and coordination were maintained through a tiered meeting and reporting system. Data visibility and transparency were key principles facilitating operational decision-making and executive-level coordination of resources, including identifying additional surge bed capacity for use systemwide, as well as ensuring efficient and equitable vaccine distribution through implementation of five mass-vaccination sites with prioritized access for vulnerable communities. Applying these four principles of shared priorities, accountability, transparency, and operational coordination and pragmatism helped the public health and individual hospital systems make contributions to the overall response that were aligned with their unique strengths and resources. Publication here represents the first official public use of the name San Francisco Health Systems Collaborative (which had served as the term used internally to refer to the group) and the first time codifying this structure. Through this coordination, San Francisco achieved one of the lowest Covid-19 death rates and had one of the highest vaccination and booster rates, compared with rates across California or the United States. Similar principles and implementation methods can be adopted by other health jurisdictions for future emergency outbreak response.

Background:This project is an effort to understand how orders for IV immunoglobulin (IVIg) are documented and prescribed by physicians, and subsequently, how they are reviewed by insurance companies for the treatment of immune neuropathies. Methods:A panel of neuromuscular specialists reviewed case records from 248 IVIg-naive patients whose in-home IVIg infusion treatment was submitted to insurance for authorization. After reviewing a case record, 1 panelist was asked to make a diagnosis and to answer several questions about the treatment. A second panelist reviewed the original record and follow-up records that were obtained for reauthorization of additional treatments and was asked to determine whether the patient had responded to the treatment. Results:Our specialists believed that only 32.2% of 248 patients had an immune neuropathy and were appropriate candidates for IVIg therapy, whereas 46.4% had neuropathies that were not immune mediated. Only 15.3% of cases met electrodiagnostic criteria for a demyelinating neuropathy. Our specialists believed that 36.7% of 128 cases with follow-up records had responded to therapy. In cases in which the initial reviewer had predicted that there would be a response to IVIg, the second reviewer found that 54% had responded. This is compared with a 27% response rate when the first reviewer predicted that there would be no response (p = 0.019). Conclusions:Our expert review finds that the diagnosis of immune neuropathies made by providers, and subsequently approved for IVIg therapy by payers, is incorrect in a large percentage of cases. If payers include an expert in their review process, it would improve patient selection, appropriate use, and continuation of treatment with this expensive therapeutic agent.

Duvoglustat HCl (AT2220, 1-deoxynojirimycin) is an investigational pharmacological chaperone for the treatment of acid α-glucosidase (GAA) deficiency, which leads to the lysosomal storage disorder Pompe disease, which is characterized by progressive accumulation of lysosomal glycogen primarily in heart and skeletal muscles. The current standard of care is enzyme replacement therapy with recombinant human GAA (alglucosidase alfa [AA], Genzyme). Based on preclinical data, oral co-administration of duvoglustat HCl with AA increases exposure of active levels in plasma and skeletal muscles, leading to greater substrate reduction in muscle. This phase 2a study consisted of an open-label, fixed-treatment sequence that evaluated the effect of single oral doses of 50 mg, 100 mg, 250 mg, or 600 mg duvoglustat HCl on the pharmacokinetics and tissue levels of intravenously infused AA (20 mg/kg) in Pompe patients. AA alone resulted in increases in total GAA activity and protein in plasma compared to baseline. Following co-administration with duvoglustat HCl, total GAA activity and protein in plasma were further increased 1.2- to 2.8-fold compared to AA alone in all 25 Pompe patients; importantly, muscle GAA activity was increased for all co-administration treatments from day 3 biopsy specimens. No duvoglustat-related adverse events or drug-related tolerability issues were identified.

This study investigates parental perceptions of cries of 1-month-old infants later diagnosed with autism spectrum disorder (ASD) and non-ASD controls. Parents of children with and without ASD listened to cry recordings of infants later diagnosed with ASD and comparison infants and rated them on cry perception scales. Parents completed the Broad Autism Phenotype Questionnaire (BAPQ) to assess the potential relations between traits associated with autism and cry perception. Across parents, ASD infant cries were rated as more distressed, less typical, and reflecting greater pain, with no significant differences between parent groups. Parents of children with ASD scored higher on the BAPQ compared to parents of children without ASD. Follow up analyses explored the relations between BAPQ score and cry ratings.

The current study investigated healthcare satisfaction and factors related to satisfaction in 92 adults with Autism Spectrum Disorder (ASD). Participants or their caregiver completed a survey about their experiences with primary care and specialty physicians. Respondents reported a high level of satisfaction with their healthcare. The only factor significantly associated with satisfaction was age, with participants under age 26 reporting significantly higher levels of satisfaction than participants above age 26. Participants under age 26 also were significantly more likely to live at home, have private health insurance, and have others making their healthcare decisions than participants above age 26. Results indicate that healthcare satisfaction can be high for adults with ASD that have good family and community support.

Little is known about arousal to socially stressful situations in children with Autism Spectrum Disorders. This preliminary study investigates physiologic arousal in children with high functioning autism (HFA, n = 19) compared to a comparison group (n = 11) before, during, and after the Trier Social Stress Test. The HFA group was more likely to have a decrease in salivary cortisol following the stressor, while the comparison group was more likely to have an increase (p = 0.02). However, there was no difference in electrodermal activity, a measure of sympathetic arousal, or vagal tone, a measure of parasympathetic activity, between groups. These findings implicate a differential neuroendocrine response to social stress in children with HFA despite similar sympathetic and parasympathetic responses during a stressor. Further studies are required to substantiate this finding. (Contains 3 figures and 3 tables.)

The objective of this study was to establish a large, densely sampled, U.S. population‐based cohort of people with autism spectrum disorder (ASD). The Rhode Island Consortium for Autism Research and Treatment (RI‐CART) represents a unique public‐private‐academic collaboration involving all major points of service for families in Rhode Island affected by ASD. Diagnosis was based on direct behavioral observation via the Autism Diagnostic Observation Schedule, Second Edition. For the first 1,000 participants, ages ranged from 21 months to 64 years. Using Geographic Information System and published prevalence rates, the overall cohort is estimated to represent between 20% and 49% of pediatric age persons in Rhode Island with ASD, with demographics representative of U.S. Census. We observed a high rate of co‐occurring medical and psychiatric conditions in affected individuals. Among the most prominent findings of immediate clinical importance, we found that females received a first diagnosis of ASD at a later age than males, potentially due to more advanced language abilities in females with ASD. In summary, this is the first analysis of a large, population‐based U.S. cohort with ASD. Given the depth of sampling, the RI‐CART study reflects an important new resource for studying ASD in a representative U.S. population. Psychiatric and medical comorbidities in ASD constitute a substantial burden and warrant adequate attention as part of overall treatment. Our study also suggests that new strategies for earlier diagnosis of ASD in females may be warranted. Autism Res 2020, 13: 474–488. © 2020 International Society for Autism Research, Wiley Periodicals, Inc. Lay Summary The Rhode Island Consortium for Autism Research and Treatment (RI‐CART) represents a unique public‐private‐academic collaboration involving all major points of service for families in Rhode Island affected by autism spectrum disorder (ASD). In this article, we provide results from the first 1,000 participants, estimated to represent >20% of affected families in the state. Importantly, we find a later age at first diagnosis of ASD in females, which potentially calls attention to the need for improved early diagnosis in girls. Also, we report a high rate of co‐occurring medical and psychiatric conditions in affected individuals. [ABSTRACT FROM AUTHOR]

The objective of this study was to establish a large, densely sampled, U.S. population-based cohort of people with autism spectrum disorder (ASD). The Rhode Island Consortium for Autism Research and Treatment (RI-CART) represents a unique public-private-academic collaboration involving all major points of service for families in Rhode Island affected by ASD. Diagnosis was based on direct behavioral observation via the Autism Diagnostic Observation Schedule, Second Edition. For the first 1,000 participants, ages ranged from 21 months to 64 years. Using Geographic Information System and published prevalence rates, the overall cohort is estimated to represent between 20% and 49% of pediatric age persons in Rhode Island with ASD, with demographics representative of U.S. Census. We observed a high rate of co-occurring medical and psychiatric conditions in affected individuals. Among the most prominent findings of immediate clinical importance, we found that females received a first diagnosis of ASD at a later age than males, potentially due to more advanced language abilities in females with ASD. In summary, this is the first analysis of a large, population-based U.S. cohort with ASD. Given the depth of sampling, the RI-CART study reflects an important new resource for studying ASD in a representative U.S. population. Psychiatric and medical comorbidities in ASD constitute a substantial burden and warrant adequate attention as part of overall treatment. Our study also suggests that new strategies for earlier diagnosis of ASD in females may be warranted. Autism Res 2020, 13: 474-488. © 2020 International Society for Autism Research, Wiley Periodicals, Inc. LAY SUMMARY: The Rhode Island Consortium for Autism Research and Treatment (RI-CART) represents a unique public-private-academic collaboration involving all major points of service for families in Rhode Island affected by autism spectrum disorder (ASD). In this article, we provide results from the first 1,000 participants, estimated to represent >20% of affected families in the state. Importantly, we find a later age at first diagnosis of ASD in females, which potentially calls attention to the need for improved early diagnosis in girls. Also, we report a high rate of co-occurring medical and psychiatric conditions in affected individuals., (© 2020 International Society for Autism Research, Wiley Periodicals, Inc.)

Objective: The objective of this study was to evaluate the effects of prenatal cocaine exposure on special education at age 7 with adjustment for covariates., Methods: As part of the prospective, longitudinal, multisite study of children with prenatal cocaine exposure (Maternal Lifestyle Study), school records were reviewed for 943 children at 7 years to determine involvement in special education outcomes: (1) individualized education plan; (2) special education conditions; (3) support services; (4) special education classes; and (5) speech and language services. Logistic regression was used to examine the effect of prenatal cocaine exposure on these outcomes with environmental, maternal, and infant medical variables as covariates, as well as with and without low child IQ., Results: Complete data for each analysis model were available for 737 to 916 children. When controlling for covariates including low child IQ, prenatal cocaine exposure had a significant effect on individualized education plan. When low child IQ was not included in the model, prenatal cocaine exposure had a significant effect on support services. Male gender, low birth weight, white race, and low child IQ also predicted individualized education plan. Low birth weight and low child IQ were significant in all models. White race was also significant in speech and language services. Other covariate effects were model specific. When included in the models, low child IQ accounted for more of the variance and changed the significance of other covariates., Conclusions: Prenatal cocaine exposure increased the likelihood of receiving an individualized education plan and support services, with adjustment for covariates. Low birth weight and low child IQ increased the likelihood of all outcomes. The finding that white children were more likely to get an individualized education plan and speech and language services could indicate a greater advantage in getting educational resources for this population.