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Recent documents in eScholarship@UMMS
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Pseudohypoparathyroidism type 1a and insulin resistance in a child

Sun, 09/11/2016 - 7:23pm

Background. A 5-year-old white girl with a history of hypothyroidism in infancy presented to the endocrinology clinic of a tertiary hospital. Her physical examination noted a stocky physique, broad chest, short neck and short digits. Two years later, skin examination revealed subcutaneous nodules and acanthosis nigricans.

Investigations. Measurement of levels of serum phosphate, parathyroid hormone, ionized calcium and insulin; measurement of peak growth hormone by the arginine-levodopa stimulation test; calculation of homeostasis model assessment of insulin resistance; assessment of bone age; DNA analysis of the GNAS gene.

Diagnosis. Pseudohypoparathyroidism type 1a in a patient with Albright hereditary osteodystrophy, characterized by hypocalcemia, hypothyroidism, growth-hormone deficiency and insulin resistance.

Management. The child continued to take levothyroxine 25 microg once daily, and at 5 years of age she was started on 40 mg/kg elemental calcium as calcium carbonate daily, and calcitriol (active vitamin D) 0.25 microg twice daily. Lifestyle modifications were also recommended for weight control. At 6 years and 4 months of age, treatment with growth hormone was initiated at a dose of 0.3 mg/kg weekly.

Multifetal Pregnancy May Increase the Risk for Severe Maternal and Neonatal Vitamin D Deficiency

Sun, 09/11/2016 - 7:23pm

Vitamin D deficiency is prevalent in pregnant women. This deficiency could be exaggerated in multifetal pregnancies by the increased demand on maternal stores of vitamin D. We present 2 cases in which hypocalcemia and secondary hyperparathyroidism occurred in 2 sets of twins from mothers with vitamin D deficiency. The first mother had gastric bypass surgery and Crohn disease. The second mother had no apparent cause of vitamin D deficiency. Both women had iron deficiency anemia and lived in Northeastern United States. We speculate that in twins, the demand for 25-hydroxyvitamin D by 2 fetuses could deplete the 25-hydroxyvitamin D stores in a mother.

Do Atypical Antipsychotic Agents Trigger Autoimmune Diabetes?

Sun, 09/11/2016 - 7:23pm

Atypical or second-generation antipsychotic agents, such as aripiprazole and olanzapine, are increasingly used in the management of schizophrenia and bipolar disorders in children. The atypical antipsychotic agents have been associated with the development of hyperglycemia, ketoacidosis, and diabetes. The mechanism of atypical antipsychotic-mediated hyperglycemia is unclear. Most of the published reports have been on individuals at risk for type 2 diabetes. We present the first known cases of the development of diabetes and positive glutamic acid decarboxylase antibodies (suggestive of autoimmune diabetes) in adolescents while on treatment with atypical antipsychotics. The nature of their clinical presentations and the time course of antipsychotic therapy to clinical diagnosis of diabetes make us speculate that atypical antipsychotics may shorten the time course of the development of autoimmune diabetes in predisposed children.

Principles of Surgical Oncology

Tue, 09/06/2016 - 8:25am

Surgery is a branch of medicine that developed primarily around the management of wounds, infections, and bladder stones. Along the way, it also became the primary treatment modality for malignant solid tumors. For many cancers, surgical resection remains the foundation of curative treatment. This chapter in Cancer Concepts: A Guidebook for the Non-Oncologist aims to introduce the history of cancer surgery, to answer the question of “What is a surgical oncologist?”, and to discuss the different categories of cancer surgery.

PRogram In Support of Moms (PRISM) Research Study

Fri, 09/02/2016 - 7:49am

An ongoing pilot study of PRISM (PRogram In Support of Moms) suggests that while both MCPAP (Massachusetts Child Psychiatry Access Project) for Moms and PRISM improve maternal perinatal depression symptoms, there is a greater decrease in depression severity with the additional intervention components included in PRISM. Over the next 5 years, investigators will run a randomized control trial that compares a set of 6 Massachusetts practices using MCPAP for Moms to a set of 6 practices using MCPAP for Moms plus PRISM.

A perinatal health framework for women with physical disabilities

Thu, 09/01/2016 - 2:36pm

BACKGROUND: Studies suggest that women with disabilities experience health and health care disparities before, during, and after pregnancy. However, existing perinatal health and health care frameworks do not address the needs and barriers faced by women with physical disabilities around the time of pregnancy. A new framework that addresses perinatal disparities among women with physical disabilities is needed.

OBJECTIVE: To propose a framework for examining perinatal health and health care disparities among women with physical disabilities.

METHODS: We developed a perinatal health framework guided by the International Classification of Functioning, Disability and Health (ICF) and the integrated perinatal health framework by Misra et al.

RESULTS: The proposed framework uses a life span perspective in a manner that directly addresses the multiple determinants specific to women with physical disabilities around the time of pregnancy. The framework is based on longitudinal and integrated perspectives that take into account women's functional status and environment over their life course.

CONCLUSION: The perinatal health framework for women with physical disabilities was developed to inform the way researchers and health care professionals address disparities in perinatal health and health care among women with physical disabilities.

Prevalence and risk factors for postpartum depression symptoms among women with disabilities

Thu, 09/01/2016 - 2:35pm

The adverse consequences of postpartum depression on the health of the mother and her child are well documented. However, there is little information on postpartum depression among mothers with disabilities. This study examines the patterns of depression and depressive symptoms before, during and after pregnancy and the association between depression before and during pregnancy and postpartum depression symptomatology (PPD) among women with and without disabilities. Data from the 2009-2011 Rhode Island Pregnancy Risk Assessment Monitoring System (PRAMS) were analyzed in 2013. Almost 30% (28.9%; 95% CI 22.8-35.8) of mothers with disabilities reported often or always feeling down, depressed or sad after childbirth compared to 10% of those without disabilities (95% CI 8.9-11.3). Compared to other women in the study, women with disabilities had a greater likelihood for PPD symptoms (RR 1.6, 95% CI 1.1-2.2) after accounting for sociodemographics, maternal characteristics related to PPD, and depression before and during pregnancy. Adjusting for other covariates, self-reported prenatal diagnosis of depression was not associated with symptoms of PPD and depression during pregnancy was marginally associated with PPD symptomatology for women with disabilities. Women with disabilities are at a greater risk of experiencing symptoms of postpartum depression than other women. Screening for PPD among new mothers with disabilities and timely referral of those with PPD diagnosis are vital to the health of mothers with disabilities and their children.

Overdiagnosis and overtreatment over time

Thu, 09/01/2016 - 2:12pm

Overdiagnosis and overtreatment are often thought of as relatively recent phenomena, influenced by a contemporary combination of technology, specialization, payment models, marketing, and supply-related demand. Yet a quick glance at the historical record reveals that physicians and medical manufacturers have been accused of iatrogenic excess for centuries, if not millennia. Medicine has long had therapeutic solutions that search for ever-increasing diagnostic problems. Whether the intervention at hand has been leeches and lancets, calomel and cathartics, aspirins and amphetamines, or statins and SSRIs, medical history is replete with skeptical critiques of diagnostic and therapeutic enthusiasm. The opportunity cost of this profusion shapes the other side of the coin: chronic persistence of underdiagnosis and undertreatment. Drawing from key controversies of the 19th and 20th centuries, we chart the enduring challenges of inter-related diagnostic and therapeutic excess. As the present critique of overdiagnosis and overtreatment seeks to mobilize resources from inside and outside of medicine to rein in these impulses, we provide an instructive historical context from which to act.

Mild hypertension in people at low risk

Thu, 09/01/2016 - 2:12pm

Antihypertensive drugs have an important role in the treatment of malignant hypertension, secondary prevention of cardiovascular disease, and primary prevention for people at high risk: those with moderate to severe hypertension (≥160/100 mm Hg), diabetes, or chronic kidney disease. Debate continues, however, about the level at which treatment should begin and the appropriate targets for treatment. The greatest uncertainty surrounds mild hypertension (140-159/90-99 mm Hg), which accounts for over 60% of those with hypertension or 22% of the global adult population. Evidence suggests no net benefit from drug treatment of mild hypertension in people without the higher risks of diabetes or chronic kidney disease. Nevertheless, most people with mild hypertension are treated with drugs. In this article, we examine the overdiagnosis and overtreatment of mild hypertension.

Collaborative Family Healthcare Association commentary on the "joint principles: Integrating behavioral health care into the patient-centered medical home"

Tue, 08/30/2016 - 3:16pm

The Collaborative Family Healthcare Association (CFHA) welcomes the opportunity to comment on the complementary set of Joint Principles underscoring the Integration of Behavioral Health Care Into the Patient-Centered Medical Home (The Working Party Group on Integrated Behavioral Healthcare et al., 2014). CFHA is an organization that promotes comprehensive and cost-effective models of health care delivery that integrate mind and body, individual and family, patients, providers, and communities. CFHA appreciates that the Joint Principles do not explicitly endorse any single model of collaboration between behavioral health and medical practice. Rather, they broadly emphasize integration, affirming the only way to have a whole person orientation is to adopt a biopsychosocial-spiritual perspective. This commentary will highlight areas of notable strength within the Joint Principles, as well as challenge the language, if not perspective, on a few critical elements.

Intimate partner violence in the relationships of men with disabilities in the United States: relative prevalence and health correlates

Tue, 08/30/2016 - 3:16pm

Despite the growing literature on intimate partner violence (IPV) victimization against people with disabilities, few studies have examined IPV against men with disabilities. This study uses population-based data to examine the prevalence of past-year and lifetime IPV against men with disabilities in the United States in comparison with men without disabilities and women with and without disabilities, compare the demographic characteristics of men with disabilities who reported IPV to those of other men, and examine associations of IPV and disability status with mental and physical health and other health risks among men. Results indicate that, adjusting for demographic characteristics, men with disabilities were more likely to report lifetime IPV than men without disabilities and, among those reporting any lifetime IPV, men with disabilities were more likely to report past-year IPV than both nondisabled men and women. With few exceptions, comparisons of health indicators revealed that men with disabilities reporting lifetime IPV were more likely than other men to report poor health status and to report engaging in health risk behaviors. Directions for future research and programmatic and policy implications of these results are discussed.

Pregnancy outcomes among women with intellectual and developmental disabilities

Tue, 08/30/2016 - 3:16pm

BACKGROUND: There is currently no population-based research on the maternal characteristics or birth outcomes of U.S. women with intellectual and developmental disabilities (IDDs). Findings from small-sample studies among non-U.S. women indicate that women with IDDs and their infants are at higher risk of adverse health outcomes.

PURPOSE: To describe the maternal characteristics and outcomes among deliveries to women with IDDs and compare them to women with diabetes and the general obstetric population.

METHODS: Data from the 1998-2010 Massachusetts Pregnancy to Early Life Longitudinal database were analyzed between November 2013 and May 2014 to identify in-state deliveries to Massachusetts women with IDDs.

RESULTS: Of the 916,032 deliveries in Massachusetts between 1998 and 2009, 703 ( < 0.1%) were to women with IDDs. Deliveries to women with IDDs were to those who were younger, less educated, more likely to be black and Hispanic, and less likely to be married. They were less likely to identify the father on the infant's birth certificate, more likely to smoke during pregnancy, and less likely to receive prenatal care during the first trimester compared to deliveries to women in the control groups (p < 0.01). Deliveries to women with IDDs were associated with an increased risk of adverse outcomes, including preterm delivery, very low and low birth weight babies, and low Apgar scores.

CONCLUSIONS: Women with IDDs are at a heightened risk for adverse pregnancy outcomes. These findings highlight the need for a systematic investigation of the pregnancy-related risks, complications, costs, and outcomes of women with IDDs. Inc.

Pregnancy Outcomes Among U.S. Women With Intellectual and Developmental Disabilities

Tue, 08/30/2016 - 3:16pm

The existing research on pregnancy outcomes for women with intellectual and developmental disabilities (IDD) is sparse. This study analyzed the 2010 Healthcare Cost and Utilization Project Nationwide Inpatient Sample and compared deliveries among women with IDD (n = 340) to the general obstetric population. Women with IDD had longer hospital stays and were more likely to have Caesarean deliveries in contrast to other women. Rates of adverse pregnancy outcomes were elevated for women with IDD across a range of measures, including early labor, preterm birth, and preeclampsia, and their infants were more likely to have low birth weight, even after adjusting for age, race, ethnicity, and insurance type. Targeted interventions are needed to address these deleterious outcomes.

Changes in smoking-related symptoms during enforced abstinence of incarceration

Tue, 08/30/2016 - 3:16pm

BACKGROUND: Tobacco use among prisoners is much higher than among the general population. Little is known about changes in smoking-related symptoms during periods of incarceration. The objective of this study is to evaluate changes in smoking-related symptoms during incarceration.

METHODS: We recruited 262 inmates from a tobacco-free prison. At baseline, participants were asked about smoking-related symptoms prior to incarceration and then asked about recent symptoms.

RESULTS: All symptom scores on the American Thoracic Society Questionnaire (ATSQ) improved during incarceration. Higher ATSQ scores were associated with asthma, depressive symptoms, stress, higher addiction and more pack years of smoking. Greater improvement in symptoms was not associated with smoking status after release.

CONCLUSION: Forced tobacco abstinence leads to significant improvements in smoking-related symptoms. However, improvements in symptoms are not associated with smoking behavior changes. Addressing changes in symptoms during incarceration will require further evaluation in smoking cessation interventions for incarcerated populations.

Vision Stations: Addressing Corrective Vision Needs With Low-cost Technologies

Tue, 08/30/2016 - 3:16pm

Eyeglasses, required for functional vision by nearly half the world's population, are still needed by more than a billion people. There are a number of constraints on the provision of eyeglasses: product cost, durability, and appearance; traditional approaches to evaluating refraction; and sustainably scaling potential distribution methods. We offer our experience with an immigrant population in a US urban setting using a "Vision Station." The station allowed for immediate provision of adjustable glasses using self-refraction, ordering of custom lenses from a low-cost website, and referral to primary and eye care physicians for those with medical eye concerns. As with models in development by other groups, Vision Stations connect people with the life-changing provision of functional vision.

Frequent Emergency Department Visits and Hospitalizations Among Homeless People With Medicaid: Implications for Medicaid Expansion

Tue, 08/30/2016 - 3:16pm

OBJECTIVES: We examined factors associated with frequent hospitalizations and emergency department (ED) visits among Medicaid members who were homeless.

METHODS: We included 6494 Massachusetts Medicaid members who received services from a health care for the homeless program in 2010. We used negative binomial regression to examine variables associated with frequent utilization.

RESULTS: Approximately one third of the study population had at least 1 hospitalization and two thirds had 1 or more ED visits. More than 70% of hospitalizations and ED visits were incurred by only 12% and 21% of these members, respectively. Homeless individuals with co-occurring mental illness and substance use disorders were at greatest risk for frequent hospitalizations and ED visits (e.g., incidence rate ratios [IRRs] = 2.9-13.8 for hospitalizations). Individuals living on the streets also had significantly higher utilization (IRR = 1.5).

CONCLUSIONS: Despite having insurance coverage, homeless Medicaid members experienced frequent hospitalizations and ED visits. States could consider provisions under the Patient Protection and Affordable Care Act (e.g., Medicaid expansion and Health Homes) jointly with housing programs to meet the needs of homeless individuals, which may improve the quality and cost effectiveness of care.

Performance of the GRACE Risk Score 2.0 Simplified Algorithm for Predicting 1-Year Death After Hospitalization for an Acute Coronary Syndrome in a Contemporary Multiracial Cohort

Mon, 08/29/2016 - 9:07am

The GRACE Risk Score is a well-validated tool for estimating short- and long-term risk in acute coronary syndrome (ACS). GRACE Risk Score 2.0 substitutes several variables that may be unavailable to clinicians and, thus, limit use of the GRACE Risk Score. GRACE Risk Score 2.0 performed well in the original GRACE cohort. We sought to validate its performance in a contemporary multiracial ACS cohort, in particular in black patients with ACS. We evaluated the performance of the GRACE Risk Score 2.0 simplified algorithm for predicting 1-year mortality in 2,131 participants in Transitions, Risks, and Actions in Coronary Events Center for Outcomes Research and Education (TRACE-CORE), a multiracial cohort of patients discharged alive after an ACS in 2011 to 2013 from 6 hospitals in Massachusetts and Georgia. The median age of study participants was 61 years, 67% were men, and 16% were black. Half (51%) of the patients experienced a non-ST-segment elevation myocardial infarction (NSTEMI) and 18% STEMI. Eighty patients (3.8%) died within 12 months of discharge. The GRACE Risk Score 2.0 simplified algorithm demonstrated excellent model discrimination for predicting 1-year mortality after hospital discharge in the TRACE-CORE cohort (c-index = 0.77). The c-index was 0.94 in patients with STEMI, 0.78 in those with NSTEMI, and 0.87 in black patients with ACS. In conclusion, the GRACE Risk Score 2.0 simplified algorithm for predicting 1-year mortality exhibited excellent model discrimination across the spectrum of ACS types and racial/ethnic subgroups and, thus, may be a helpful tool to guide routine clinical care for patients with ACS.

Prognostic value of dynamic electrocardiographic T wave changes in non-ST elevation acute coronary syndrome

Mon, 08/29/2016 - 9:07am

OBJECTIVE: To assess the relationship between the evolution of T wave inversion (TWI) on the 24-48 h postadmission ECG and the patient characteristics, management and clinical outcomes among those with non-ST elevation acute coronary syndrome (NSTE-ACS).

METHODS: We evaluated admission and 24-48 h follow-up ECGs of 7201 patients with NSTE-ACS from the prospective, multicentre Global Registry of Acute Coronary Events (GRACE) and Canadian ACS Registry I. We performed multivariable analyses to determine the association between new TWI (on follow-up ECG only), resolved TWI (on admission ECG only) and persistent TWI (on both admission and follow-up ECG) and inhospital and cumulative 6-month all-cause mortality.

RESULTS: Patients with TWI were older, more likely to have cardiovascular risk factors, higher Killip class and GRACE risk scores. After adjustment for known prognostic factors, compared with patients presenting without TWI, new TWI was associated with significantly lower inhospital mortality (OR=0.60, 95% CI 0.38 to 0.95, p=0.029), whereas resolved (OR=1.06, 95% CI 0.65 to 1.75, p=0.81) and persistent (OR=0.73, 95% CI 0.48 to 1.11, p=0.14) TWI did not predict inhospital mortality. No TWI pattern independently predicted inhospital adverse cardiovascular events or cumulative 6-month mortality. In contrast, ST depression on the admission and follow-up ECG were independent predictors of inhospital and 6-month mortality.

CONCLUSIONS: Across the spectrum of NSTE-ACS, TWI within 48 h of presentation was associated with high-risk clinical features, but its presence or dynamic change did not provide additional prognostic value beyond other established clinical predictors.

Clinical Characteristics, Management, and Outcomes of Acute Coronary Syndrome in Patients With Right Bundle Branch Block on Presentation

Mon, 08/29/2016 - 9:07am

We examined the relations between right bundle branch block (RBBB) and clinical characteristics, management, and outcomes among a broad spectrum of patients with acute coronary syndrome (ACS). Admission electrocardiograms of patients enrolled in the Global Registry of Acute Coronary Events (GRACE) electrocardiogram substudy and the Canadian ACS Registry I were analyzed independently at a blinded core laboratory. We performed multivariable logistic regression analysis to assess the independent prognostic significance of admission RBBB on in-hospital and 6-month mortality. Of 11,830 eligible patients with ACS (mean age 65; 66% non-ST-elevation ACS), 5% had RBBB. RBBB on admission was associated with older age, male sex, more cardiovascular risk factors, worse Killip class, and higher GRACE risk score (all p < 0.01). Patients with RBBB less frequently received in-hospital cardiac catheterization, coronary revascularization, or reperfusion therapy (all p < 0.05). The RBBB group had higher unadjusted in-hospital (8.8% vs 3.8%, p < 0.001) and 6-month mortality rates (15.1% vs 7.6%, p < 0.001). After adjusting for established prognostic factors in the GRACE risk score, RBBB was a significant independent predictor of in-hospital death (odds ratio 1.45, 95% CI 1.02 to 2.07, p = 0.039), but not cumulative 6-month mortality (odds ratio 1.29, 95% CI 0.95 to 1.74, p = 0.098). There was no significant interaction between RBBB and the type of ACS for either in-hospital or 6-month mortality (both p > 0.50). In conclusion, across a spectrum of ACS, RBBB was associated with preexisting cardiovascular disease, high-risk clinical features, fewer cardiac interventions, and worse unadjusted outcomes. After adjusting for components of the GRACE risk score, RBBB was a significant independent predictor of early mortality.