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Gamis A.S.,Childrens Mercy Hospitals and Clinics | Smith F.O.,University of Cincinnati
British Journal of Haematology | Year: 2012

Children with trisomy 21 have a unique predisposition to develop a megakaryoblastic proliferative disease of varying severity during their first 3 months of life. This disorder exists in no other children or adults without the presence of trisomy 21 and only occurs in the fetal or neonatal period of life. Its spontaneous resolution in most cases further delineates it from otherwise indistinguishable neonatal leukaemias. The identification that GATA1 mutations are the leukaemogenic source along with three recently reported prospective clinical trials now provide a clearer understanding of this haematopoietic disorder. These recent advances in this enigmatic disorder, now known as Transient Myeloproliferative Disorder, are reviewed here in order to bring clarity to the breadth of organ involvement, the range of severity, the risk factors for mortality, the therapeutic options for severe manifestations, the natural course of spontaneous resolution regardless of therapy, and the elucidation of the subsequent risk for myeloid leukaemia. © 2012 Blackwell Publishing Ltd.


Johnson R.J.,Childrens Mercy Hospitals and Clinics | Warady B.A.,Childrens Mercy Hospitals and Clinics
Pediatric Nephrology | Year: 2013

Background: End-stage renal disease (ESRD) during infancy has been associated with poor short-term neurocognitive outcomes. Limited information exists regarding long-term outcomes. Methods: Neurocognitive outcomes for 12 patients diagnosed with ESRD during the first 16 months of life were assessed. Nine patients (mean age: 11 years) were compared to their healthy siblings (mean age: 10 years) on measures of intellectual and executive functioning, memory, and academic achievement using paired-samples t tests. Results: Patients' Full Scale IQ (FSIQ) scores (M = 78, SD = 16.1) were significantly lower than sibling controls (M = 94, SD = 18.9; p < 0.03). For patients, FSIQ negatively correlated with total months on dialysis (r = -0.6, p < 0.04), as did WISC-IV Processing Speed (r = -0.6, p < 0.05). Patients' scores on the Metacognition Index of the BRIEF (M = 61.4, SD = 16.3) were significantly higher (indicating greater risk for dysfunction) than siblings (M = 46.7, SD = 6.4; p < 0.04). Patients' scores (M = 84, SD = 19) on the WIAT-II-A Total Achievement were significantly lower than siblings (M = 103, SD = 20, p < 0.01). Younger age at transplant was associated with higher scores on measures of Processing Speed (r = -0.7, p < 0.05), as well as higher scores on measures of executive functioning, memory, and academic achievement. Conclusions: In summary, patients diagnosed with ESRD as infants had intellectual and metacognitive functioning significantly lower than sibling controls. Fewer months on dialysis and younger age at transplant were associated with better outcomes. © 2013 IPNA.


O'Donnell R.,Childrens Mercy Hospitals and Clinics
Pediatrics | Year: 2013

Children's hospitals play a central role in our child health care system. These hospitals face unique challenges under health care reform. They care for children with the most complex medical problems but often are not reimbursed for good preventive care, care coordination, or quality. We discuss a proposal by children's hospital leaders to create a network of Nationally Designated Children's Hospitals. These would be Centers of Excellence on which states and families could rely to care for a uniquely vulnerable and uniquely costly population of children. On a federal level, the proposal is focused on 3 provisions: (1) creating and delivering a national coordinated delivery model for children with chronic and complex conditions in Medicaid and the State Child Health Insurance Program; (2) developing pediatric-specific care coordination guidelines, quality metrics, and network adequacy standards to improve pediatric care delivery; and (3) producing cost savings by reducing fragmentation in care delivery, while providing a payment model that provides a significant measure of budget certainty for states and the federal government, either through a bundled payment or a shared savings payment method. We believe that this approach will ensure access to appropriate care without compromising the quality of care. It will also provide enhanced budget certainty for Medicaid and the State Child Health Insurance Program. Copyright © 2013 by the American Academy of Pediatrics.


Portnoy J.M.,Childrens Mercy Hospitals and Clinics
Current allergy and asthma reports | Year: 2014

Food allergies are estimated to affect as many as 8 % of children with 2.5 % being allergic to peanut products. Based on the results of recent surveys, this prevalence has been increasing over the last few decades for unknown reasons. As children with food allergies reach school age, the issue is becoming more common in schools. For that reason, schools are now required to be prepared to take responsibility for the safety of food-allergic students. This review discusses the common problems surrounding management of food allergies in the school setting along with reasonable recommendations for addressing those problems. The most important component of food allergy management is for the student to get an accurate diagnosis and to then discuss development of an anaphylaxis action plan with their health-care provider. Each school should insist that a copy of such a plan be provided for each student with food allergy and that epinephrine is readily available should a student have an anaphylactic reaction. In addition to epinephrine, it is essential that school personnel be properly trained to recognize and treat allergic reactions should they occur. Known deficiencies in school preparedness have been documented in previous literature, and consequently, both state and the federal government have begun to implement policies to help with school preparedness.


Mikat-Stevens N.A.,American Academy of Pediatrics | Larson I.A.,Childrens Mercy Hospitals and Clinics | Tarini B.A.,University of Michigan
Genetics in Medicine | Year: 2015

Purpose: We aimed to systematically review the literature to identify primary-care providers' perceived barriers against provision of genetics services. Methods: We systematically searched PubMed and ERIC using key and Boolean term combinations for articles published from 2001 to 2012 that met inclusion/exclusion criteria. Specific barriers were identified and aggregated into categories based on topic similarity. These categories were then grouped into themes. Results: Of the 4,174 citations identified by the search, 38 publications met inclusion criteria. There were 311 unique barriers that were classified into 38 categories across 4 themes: knowledge and skills; ethical, legal, and social implications; health-care systems; and scientific evidence. Barriers most frequently mentioned by primary-care providers included a lack of knowledge about genetics and genetic risk assessment, concern for patient anxiety, a lack of access to genetics, and a lack of time. Conclusion: Although studies reported that primary-care providers perceive genetics as being important, barriers to the integration of genetics medicine into routine patient care were identified. The promotion of practical guidelines, point-of-care risk assessment tools, tailored educational tools, and other systems-level strategies will assist primary-care providers in providing genetics services for their patients. © 2015 American College of Medical Genetics and Genomics.


Becker M.L.,Childrens Mercy Hospitals and Clinics
Clinical Pharmacology and Therapeutics | Year: 2012

Therapeutics used in pediatric rheumatology have evolved substantially over the past few decades; they currently target specific cytokines that are known to be involved in the pathophysiology of these complex diseases. The field is limited by a lack of full understanding of the etiology and pathophysiology of these conditions, as well as by the rarity of these diseases in the pediatric population. Advances in biomarkers, pharmacogenomics, and biologic therapies, along with a more unified effort from clinicians and investigators, have enabled continued growth in the field, fostering the hope for the most effective and appropriate therapeutics for pediatric patients. © 2012 American Society for Clinical Pharmacology and Therapeutics.


Olitsky S.E.,Childrens Mercy Hospitals and Clinics
American Family Physician | Year: 2010

Hereditary hemorrhagic telangiectasia is an uncommon autosomal dominant disease that occurs in approximately one in 5,000 to 8,000 persons. This multisystem disorder can affect the nose, skin, gastrointestinal tract, lungs, liver, and brain. Epistaxis is the most common presenting problem, occurring in 90 percent of affected patients. Approximately 15 to 30 percent of patients with hereditary hemorrhagic telangiectasia will have an arteriovenous malformation in the lungs and more than 10 percent will have one in the brain. The symptoms of hereditary hemorrhagic telangiectasia are often unrecognized. Many patients, even those with affected family members, may go undiagnosed. Hereditary hemorrhagic telangiectasia is a clinical diagnosis that is based on the presence of three of four criteria (i.e., epistaxis, telangiectasias, visceral arteriovenous malformations, or family history of the disease). Screening and treatment recommendations have been created in an attempt to limit the morbidity and mortality associated with this disease. Patients with confirmed or suspected hereditary hemorrhagic telangiectasia should be screened for brain and lung arteriovenous malformations using magnetic resonance imaging of the brain and contrast echocardiography. Pulmonary arteriovenous malformations can be treated with embolization. Patients with a history of pulmonary arteriovenous malformations or those who have not been screened should use antibiotic prophylaxis before dental treatment, endoscopy, or other procedures that could cause bacteremia because of the risk of paradoxical brain embolism or infection. © 2010 American Academy of Family Physicians.


O'Malley D.M.,Childrens Mercy Hospitals and Clinics
Nursing Administration Quarterly | Year: 2013

The Patient Protection and Affordable Care Act aims to increase access to many who were previously uninsured, to increase the quality of health care, and to decrease costs. Accountable Care Organizations are a manifestation of these new health care reforms. A proactive approach focused on primary prevention to address modifiable determinants of health holds the promise of a healthier population while being cost-effective. Exposure to toxic stress in childhood places many children on a trajectory for poor immediate and long-term health outcomes. A lifecourse approach to disease prevention offers opportunities at every age and stage to build resilience, which buffers and protects children from the effects of adversity and toxic stress. The American Academy of Pediatrics, recognizing that many adult diseases are rooted in experiences in early childhood, has proposed an ecobiodevelopmental framework that expands the role of pediatricians and embraces a preventive lifecourse approach to health and well-being. Exposure to adversity and toxic stress in childhood is a serious public health problem. A new kind of leadership is required to address these issues. Pediatric nurses and physicians are trusted healers and in a position to seek solutions skillfully and intentionally. Copyright © 2013 Wolters Kluwer Health | Lippincott Williams & Wilkins.


Kingsmore S.,Childrens Mercy Hospitals and Clinics
PLoS Currents | Year: 2012

Of 7,028 disorders with suspected Mendelian inheritance, 1,139 are recessive and have an established molecular basis. Although individually uncommon, Mendelian diseases collectively account for ~20% of infant mortality and ~18% of pediatric hospitalizations. Molecular diagnostic testing is currently available for only ~300 recessive disorders. Preconception screening, together with genetic counseling of carriers, has resulted in remarkable declines in the incidence of several severe recessive diseases including Tay-Sachs disease and cystic fibrosis. However, extension of preconception screening and molecular diagnostic testing to most recessive disease genes has hitherto been impractical. Recently, we reported a preconception carrier screen/molecular diagnostic test for 448 recessive childhood diseases. The current status of this test is reviewed here. Currently, this reports analytical validity of the comprehensive carrier test. As the clinical validity and clinical utility in the contexts described is ascertained, this article will be updated.


Nandyala S.V.,University of Missouri - Kansas City | Schwend R.M.,Childrens Mercy Hospitals and Clinics
Spine | Year: 2013

STUDY DESIGN.: Retrospective case control study. OBJECTIVE.: To determine prevalence and risk factors for intraoperative bacterial contamination in posterior spinal deformity surgery. SUMMARY OF BACKGROUND DATA.: The prevalence of deep surgical site infection in pediatric spinal deformity surgery varies from 1% to 14%. Little evidence exists about the incidence and role of intraoperative bacterial contamination. METHODS.: A total of 114 cases of pediatric posterior instrumented deformity surgery were retrospectively identified. All patients received preoperative and every 4-hour intraoperative antibiotics and 3M Ioban 2 Antimicrobial Incise Drape. Preoperative photographs of patients' backs were used to correlate presence of back acne with contaminant bacteria. Laboratory cultures were obtained from paraspinal muscle debrided before closure. RESULTS.: Of the 114 cultures obtained, 26 (23%) were positive in 21% of idiopathic, 37% of neuromuscular (P = 0.02), and 14% of congenital patients. Contaminant bacteria included Propionibacterium acnes (69%), Staphylococcus (23%), Coryneform (4%), and Clostridium (4%). P. acnes was seen only in children 11 years or older (P = 0.02) and only with back acne (P < 0.0001). Eight of 19 (42%) patients with pelvic fusion had positive cultures (P = 0.04) and all 8 were neuromuscular patients. Eighty-one percent of culture-positive patients were older than 11 years (P = 0.01). Three of 114 (2.7%) patients developed an early deep surgical site infection, all with positive cultures (P = 0.01). CONCLUSION.: Neuromuscular patients fused to the pelvis, children older than 11 years, and surgery duration greater than 6 hours were associated with positive cultures. Back acne is a preventable risk factor for P. acnes seeding. Intraoperative bacterial contamination indicates a need to consider the type of surgery and patient age to determine prophylactic antibiotics and other modalities to prevent infection.Level of Evidence: N/A © 2013, Lippincott Williams & Wilkins.

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