Childrens Mercy Hospitals & Clinics

Kansas City, MO, United States

Childrens Mercy Hospitals & Clinics

Kansas City, MO, United States
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PubMed | Seattle Childrens Hospital, Arkansas Childrens Hospital, University of Wisconsin - Madison, CS Mott Childrens Hospital and 14 more.
Type: | Journal: The Journal of pediatrics | Year: 2016

To determine safety and pharmacodynamics/efficacy of teduglutide in children with intestinal failure associated with short bowel syndrome (SBS-IF).This 12-week, open-label study enrolled patients aged 1-17 years with SBS-IF who required parenteral nutrition (PN) and showed minimal or no advance in enteral nutrition (EN) feeds. Patients enrolled sequentially into 3 teduglutide cohorts (0.0125mg/kg/d [n=8], 0.025mg/kg/d [n=14], 0.05mg/kg/d [n=15]) or received standard of care (SOC, n=5). Descriptive summary statistics were used.All patients experienced 1 treatment-emergent adverse event; most were mild or moderate. No serious teduglutide-related treatment-emergent adverse events occurred. Between baseline and week 12, prescribed PN volume and calories (kcal/kg/d) changed by a median of -41% and -45%, respectively, with 0.025mg/kg/d teduglutide and by -25% and -52% with 0.05mg/kg/d teduglutide. In contrast, PN volume and calories changed by 0% and -6%, respectively, with 0.0125mg/kg/d teduglutide and by 0% and -1% with SOC. Per patient diary data, EN volume increased by a median of 22%, 32%, and 40% in the 0.0125, 0.025, and 0.05 mg/kg/d cohorts, respectively, and by 11% with SOC. Four patients achieved independence from PN, 3 in the 0.05 mg/kg/d cohort and 1 in the 0.025 mg/kg/d cohort. Study limitations included its short-term, open-label design, and small sample size.Teduglutide was well tolerated in pediatric patients with SBS-IF. Teduglutide 0.025 or 0.05mg/kg/d was associated with trends toward reductions in PN requirements and advancements in EN feeding in children with; EudraCT: 2013-004588-30.

Herd T.J.,Childrens Mercy Hospitals & Clinics | Nopper A.J.,Childrens Mercy Hospitals & Clinics | Horii K.A.,Childrens Mercy Hospitals & Clinics
Pediatric Dermatology | Year: 2017

Decreasing wait time for pediatric dermatology appointments is important to patients. We retrospectively examined the effect of a referral-only policy on patient wait time for an initial appointment in an academic pediatric dermatology clinic and found a statistically significant decrease in wait time after the policy was instituted. © 2017 Wiley Periodicals, Inc.

Rudert A.,Childrens Mercy Hospitals & Clinics | Portnoy J.,Childrens Mercy Hospitals & Clinics
Expert Review of Clinical Immunology | Year: 2017

Introduction: fungi produce substances that contain pathogen-associated molecular patterns (pamps) and damage-associated molecular patterns (damps) which bind to pattern recognition receptors, stimulating innate immune responses in humans. they also produce allergens that induce production of specific ige. Areas covered: In this review we cover both innate and adaptive immune responses to fungi. Some fungal products can activate both innate and adaptive responses and in doing so, cause an intense and complex health effects. Methods of testing for fungal allergy and evidence for clinical treatment including environmental control are also discussed. In addition, we describe controversial issues including the role of Stachybotrys and mycotoxins in adverse health effects. Expert commentary: Concerns about long-term exposure to fungi have led some patients, attorneys and fungus advocates to promote fears about a condition that has been termed toxic mold syndrome. This syndrome is associated with vague symptoms and is believed to be due to exposure to mycotoxins, though this connection has not been proven. Ultimately, more precise methods are needed to measure both fungal exposure and the resulting health effects. Once that such methods become available, much of the speculation will be replaced by knowledge. © 2017 Informa UK Limited, trading as Taylor & Francis Group.

PubMed | University of Manchester, Montpellier University, Imperial College London, Mayo Medical School and 8 more.
Type: Journal Article | Journal: Allergy | Year: 2015

The global allergy community strongly believes that the 11th revision of the International Classification of Diseases (ICD-11) offers a unique opportunity to improve the classification and coding of hypersensitivity/allergic diseases via inclusion of a specific chapter dedicated to this disease area to facilitate epidemiological studies, as well as to evaluate the true size of the allergy epidemic. In this context, an international collaboration has decided to revise the classification of hypersensitivity/allergic diseases and to validate it for ICD-11 by crowdsourcing the allergist community. After careful comparison between ICD-10 and 11 beta phase linearization codes, we identified gaps and trade-offs allowing us to construct a classification proposal, which was sent to the European Academy of Allergy and Clinical Immunology (EAACI) sections, interest groups, executive committee as well as the World Allergy Organization (WAO), and American Academy of Allergy Asthma and Immunology (AAAAI) leaderships. The crowdsourcing process produced comments from 50 of 171 members contacted by e-mail. The classification proposal has also been discussed at face-to-face meetings with experts of EAACI sections and interest groups and presented in a number of business meetings during the 2014 EAACI annual congress in Copenhagen. As a result, a high-level complex structure of classification for hypersensitivity/allergic diseases has been constructed. The model proposed has been presented to the WHO groups in charge of the ICD revision. The international collaboration of allergy experts appreciates bilateral discussion and aims to get endorsement of their proposals for the final ICD-11.

PubMed | Seattle Childrens Hospital, Johns Hopkins University, Childrens Mercy Hospitals & Clinics, Harvard University and Florida College
Type: Journal Article | Journal: Journal of pediatric surgery | Year: 2015

Despite rigorous data from adult literature demonstrating that oral antibiotics (OA) reduce infectious complications and mechanical bowel preparation (MBP) alone does not, MBP alone remains the preferred approach among pediatric surgeons. We aimed to explore the nature of this discrepancy through a survey of the American Pediatric Surgical Association membership.Surgeons were queried for their choice of bowel preparation, factors influencing their practice, and their impression of the strength and relevance of the adult literature to pediatric practice.Surgeons who used MBP alone (31%) cited a reduction in stool burden and infectious complications as important factors, whereas surgeons choosing not to use OA (70%) reported a lack of benefit in reducing infectious complications as the primary reason. Although 53% of surgeons reported that evidence from adult literature was the most important influence, 73% of surgeons reported there was poor evidence supporting the use of OA (MBP), and only 25% used a preparation supported by adult randomized data.Wide variation exists among pediatric surgeons in the perceived utility of MBP and OA. Although the majority of pediatric surgeons cited the adult literature as the strongest influence on their practice, this is not consistent with stated perceptions or practice.

PubMed | University of Kansas, Kansas State University and Childrens Mercy Hospitals & Clinics
Type: | Journal: Physiology & behavior | Year: 2015

Combined physical and psychological stress events have been associated with exacerbated endocrine responses and increased alterations in immune cell trafficking when compared to exercise stress alone. Military training programs are rigorous in nature and often purposefully delivered in environments combining high levels of both physical and mental stress. The objective of this study was to assess physiological and cognitive changes following U.S. Marine Corps Martial Arts training. Seven active-duty, male Marines were observed during a typical Marine Corps Martial Arts training session. Immune parameters, including immunomodulatory cytokines, and hormone concentrations were determined from blood samples obtained at baseline, immediately post training (IP) and at 15min intervals post-training to 1h (R15, R30, R45, R60). Assessments of cognitive moral functioning (moral judgment and intent) were recorded at intervals during recovery. There were significant fluctuations in immunoendocrine parameters. Peak endocrine measures were observed within the IP-R15 time interval. Distributions of circulating immune cells were significantly altered with neutrophils and all lymphocyte subsets elevated at IP. IFN- and IL-17a exhibited small, non-significant, parallel increases over the recovery period. Moral functioning was informed by different social identities during the recovery resulting in changes in moral decision-making. These data demonstrate that the Marine Corps Martial Arts Program induces significant alterations in lymphocyte and leukocyte distributions, but does not shift the balance of Th1/Th2 cytokines or induce a systemic inflammatory response. The program does, however, induce alterations in moral decision-making ability associated with the observed endocrine responses, even suggesting a potential interaction between ones social identities and endocrine responses upon moral decision-making.

PubMed | University of Kansas Medical Center and Childrens Mercy Hospitals & Clinics
Type: | Journal: BMC nursing | Year: 2016

Time trends and seasonal patterns have been observed in nurse staffing and nursing-sensitive patient outcomes in recent years. It is unknown whether these changes were associated.Quarterly unit-level nursing data in 2004-2012 were extracted from the National Database of Nursing Quality Indicators (NDNQI). Units were divided into groups based on patterns of missing data. All variables were aggregated across units within these groups and analyses were conducted at the group level. Patient outcomes included rates of inpatient falls and hospital-acquired pressure ulcers. Staffing variables included total nursing hours per patient days (HPPD) and percent of nursing hours provided by registered nurses (RN skill-mix). Weighted linear mixed models were used to examine the associations between nurse staffing and patient outcomes at trend and seasonal levels.At trend level, both staffing variables were inversely associated with all outcomes (By aggregating data across units we were able to detect associations between nurse staffing and patient outcomes at both trend and seasonal levels. More rigorous research is needed to study the underlying mechanism of these associations.

van Haandel L.,Childrens Mercy Hospitals & Clinics
Bioanalysis | Year: 2011

Low-dose methotrexate is used for the treatment of rheumatoid arthritis and juvenile idiopathic arthritis, but its effectiveness greatly varies between individuals. Therapeutic drug monitoring of intracellular methotrexate metabolites, the γ-polyglutamates (MTXGlu(n)), in human erythrocytes has shown promise in providing a basis for individualization of therapy. This work presents expedient methodology for the analysis of MTXGlu(1-7) in human erythrocytes by ion-pair UPLC with detection by tandem MS (UPLC-ESI-MS/MS). The use of N,N-dimethylheptylamine as an ion-pair agent was found to be favorable over others. Thermal extraction of erythrocyte lysates provides a simple one-step extraction procedure. The entire chromatographic run time is 6 min and the assay was validated within the therapeutic range of these metabolites The developed sample preparation procedure in combination with ion-pair UPLC-ESI-MS/MS analysis allowed for expedient quantitation of MTXGlu(1-7) in human erythrocytes. The rapid analysis time would enable therapeutic drug monitoring of MTXGlu(1-7) in the clinic.

Clinkenbeard E.L.,Indiana University | Farrow E.G.,Childrens Mercy Hospitals Clinics | Summers L.J.,Indiana University | Cass T.A.,Indiana University | And 7 more authors.
Journal of Bone and Mineral Research | Year: 2014

Fibroblast growth factor 23 (FGF23) gain of function mutations can lead to autosomal dominant hypophosphatemic rickets (ADHR) disease onset at birth, or delayed onset following puberty or pregnancy. We previously demonstrated that the combination of iron deficiency and a knock-in R176Q FGF23 mutation in mature mice induced FGF23 expression and hypophosphatemia that paralleled the late-onset ADHR phenotype. Because anemia in pregnancy and in premature infants is common, the goal of this study was to test whether iron deficiency alters phosphate handling in neonatal life. Wild-type (WT) and ADHR female breeder mice were provided control or iron-deficient diets during pregnancy and nursing. Iron-deficient breeders were also made iron replete. Iron-deficient WT and ADHR pups were hypophosphatemic, with ADHR pups having significantly lower serum phosphate (p < 0.01) and widened growth plates. Both genotypes increased bone FGF23 mRNA (>50 fold; p < 0.01). WT and ADHR pups receiving low iron had elevated intact serum FGF23; ADHR mice were affected to a greater degree (p < 0.01). Iron-deficient mice also showed increased Cyp24a1 and reduced Cyp27b1, and low serum 1,25-dihydroxyvitamin D (1,25D). Iron repletion normalized most abnormalities. Because iron deficiency can induce tissue hypoxia, oxygen deprivation was tested as a regulator of FGF23, and was shown to stimulate FGF23 mRNA in vitro and serum C-terminal FGF23 in normal rats in vivo. These studies demonstrate that FGF23 is modulated by iron status in young WT and ADHR mice and that hypoxia independently controls FGF23 expression in situations of normal iron. Therefore, disturbed iron and oxygen metabolism in neonatal life may have important effects on skeletal function and structure through FGF23 activity on phosphate regulation. © 2014 American Society for Bone and Mineral Research. © 2014 American Society for Bone and Mineral Research.

PubMed | Childrens Mercy Hospitals & Clinics
Type: Journal Article | Journal: Pediatric emergency care | Year: 2016

Sudden cardiac arrest (SCA) in the pediatric population is a rare and potentially devastating occurrence. An understanding of the differential diagnosis for the etiology of the cardiac arrest allows for the most effective emergency care and provides the patient with the best possible outcome. Pediatric SCA can occur with or without prodromal symptoms and may occur during exercise or rest. The most common cause is arrhythmia secondary to an underlying channelopathy, cardiomyopathy, or myocarditis. After stabilization, evaluation should include electrocardiogram, chest radiograph, and echocardiogram. Management should focus on decreasing the potential for recurring arrhythmia, maintaining cardiac preload, and thoughtful medication use to prevent exacerbation of the underlying condition. The purpose of this review was to provide the emergency physician with a concise and current review of the incidence, differential diagnosis, and management of pediatric patients presenting with SCA.

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