Improvement in medication education in a pediatric subspecialty practice

<p>Abstract</p> <p>Background</p> <p>The purpose of this study was to measure the impact of an educational intervention on parents of children taking methotrexate (MTX) for juvenile idiopathic arthritis (JIA).</p> <p>Methods</p> <p>This study was conducted using a pre- and postsurvey design. The parents of 100 children with JIA taking MTX for at least 2 months were surveyed during a routine office visit. The parents completed an initial questionnaire regarding the safe use, adverse effects, and guidelines for monitoring the toxicity of MTX. An educational intervention was then administered, and an identical follow-up questionnaire was given during the next office visit. Statistical analysis using a paired <it>t</it>-test (critical <it>P </it>value < 0.05) was performed on individuals who answered both questionnaires.</p> <p>Results</p> <p>There were 100 responses to the initial questionnaire and 67 responses to the follow-up questionnaire. The mean length of time between surveys was 2.9 ± 0.9 months. In those who completed both questionnaires, the overall correct score increased significantly from 75.8% to 93.4%, respectively (<it>P </it>< 0.0001). Individuals scored the lowest (49%) on the question that addressed MTX's impact on pregnancy and fertility.</p> <p>Conclusions</p> <p>MTX knowledge may be less than expected in the parents of children with JIA. Brief educational interventions in the pediatric subspecialty practice can significantly affect a family's understanding of their child's medications.</p

Growing pains in children

We review the clinical manifestations of "growing pains", the most common form of episodic childhood musculoskeletal pain. Physicians should be careful to adhere to clear clinical criteria as described in this review before diagnosing a child with growing pain. We expand on current theories on possible causes of growing pains and describe the management of these pains and the generally good outcome in nearly all children

Constitutively active Lyn kinase causes a cutaneous small vessel vasculitis and liver fibrosis syndrome

Neutrophilic inflammation is a hallmark of many monogenic autoinflammatory diseases; pathomechanisms that regulate extravasation of damaging immune cells into surrounding tissues are poorly understood. Here we identified three unrelated boys with perinatal-onset of neutrophilic cutaneous small vessel vasculitis and systemic inflammation. Two patients developed liver fibrosis in their first year of life. Next-generation sequencing identified two de novo truncating variants in the Src-family tyrosine kinase, LYN, p.Y508*, p.Q507* and a de novo missense variant, p.Y508F, that result in constitutive activation of Lyn kinase. Functional studies revealed increased expression of ICAM-1 on induced patient-derived endothelial cells (iECs) and of β2-integrins on patient neutrophils that increase neutrophil adhesion and vascular transendothelial migration (TEM). Treatment with TNF inhibition improved systemic inflammation; and liver fibrosis resolved on treatment with the Src kinase inhibitor dasatinib. Our findings reveal a critical role for Lyn kinase in modulating inflammatory signals, regulating microvascular permeability and neutrophil recruitment, and in promoting hepatic fibrosis

Policy challenges for the pediatric rheumatology workforce: Part II. Health care system delivery and workforce supply

The United States pediatric population with chronic health conditions is expanding. Currently, this demographic comprises 12-18% of the American child and youth population. Affected children often receive fragmented, uncoordinated care. Overall, the American health care delivery system produces modest outcomes for this population. Poor, uninsured and minority children may be at increased risk for inferior coordination of services. Further, the United States health care delivery system is primarily organized for the diagnosis and treatment of acute conditions. For pediatric patients with chronic health conditions, the typical acute problem-oriented visit actually serves as a barrier to care. The biomedical model of patient education prevails, characterized by unilateral transfer of medical information. However, the evidence basis for improvement in disease outcomes supports the use of the chronic care model, initially proposed by Dr. Edward Wagner. Six inter-related elements distinguish the success of the chronic care model, which include self-management support and care coordination by a prepared, proactive team

Validation and clinical significance of the childhood myositis assessment scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies

Objective. To examine the measurement characteristics of the Childhood Myositis Assessment Scale (CMAS) in children with juvenile idiopathic inflammatory myopathy (juvenile IIM), and to obtain preliminary data on the clinical significance of CMAS scores. Methods. One hundred eight children with juvenile IIM were evaluated on 2 occasions, 7-9 months apart, using various measures of physical function, strength, and disease activity. Interrater reliability, construct validity, and responsiveness of the CMAS were examined. The minimum clinically important difference (MID) and CMAS scores corresponding to various degrees of physical disability were estimated. Results. The intraclass correlation coefficient for 26 patients assessed by 2 examiners was 0.89, indicating very good interrater reliability. The CMAS score correlated highly with the Childhood Health Assessment Questionnaire (C-HAQ) score and with findings on manual muscle testing (MMT) (r(s) = -0.73 and 0.73, respectively) and moderately with physician-assessed global disease activity and skin activity, parent-assessed global disease severity, and muscle magnetic resonance imaging (rs = -0.44 to -0.61), thereby demonstrating good construct validity. The standardized response mean was 0.81 (95% confidence interval 0.53, 1.09) in patients with at least 0.8 cm improvement on a 10-cm visual analog scale for physician-assessed global disease activity, indicating strong responsiveness. In bivariate regression models predicting physician-assessed global disease activity, MMT remained significant in models containing the CMAS (P = 0.03) while the C-HAQ did not (P = 0.4). Estimates of the MID ranged from 1.5 to 3.0 points on a 0-52-point scale. CMAS scores corresponding to no, mild, mild-to-moderate, and moderate physical disability, respectively, were 48, 45, 39, and 30. Conclusion. The CMAS exhibits good reliability, construct validity, and responsiveness, and is therefore a valid instrument for the assessment of physical function, muscle strength, and endurance in children with juvenile IIM. Preliminary data on MID and corresponding levels of disability should aid in the clinical interpretation of CMAS scores when assessing patients with juvenile IIM

Colite ulcerativa inespecífica em crianças e adolescentes: análise de casuística Non-specific ulcerative colitis in children and adolescents: casuistic analysis

OBJETIVOS: descrever uma série de casos de colite ulcerativa inespecífica, com ênfase nas manifestações iniciais, propedêutica e intervalo entre os primeiros sintomas e o diagnóstico. MÉTODOS: análise retrospectiva de casos de colite ulcerativa inespecífica, cujos sintomas iniciaram antes de 20 anos de idade. Os pacientes foram selecionados nos Serviços de Gastroenterologia Pediátrica do Hospital das Clínicas da Universidade Federal de Minas Gerais e do Instituto Materno Infantil de Pernambuco. RESULTADOS: foram encontrados 29 pacientes com diagnóstico de colite ulcerativa inespecífica, sendo 15 (51,7%) pacientes do sexo masculino. A idade ao início dos sintomas variou entre 0,3 a 15,8 anos. Os sintomas mais freqüentes ao diagnóstico foram diarréia (89,7%) e dor abdominal (82,8%). A manifestação extra-intestinal mais comum foi artralgia/artrite (41,4%). A relação peso para a idade estava abaixo do percentil 3 em 13,8% dos pacientes. O intervalo de tempo entre o início dos sintomas e o diagnóstico variou entre 0,1 e 10,9 anos. CONCLUSÕES: o intervalo entre os sintomas iniciais e o diagnóstico ainda continua elevado na nossa Região devido ao somatório de vários fatores.<br>OBJECTIVES: to describe a series of non-specific ulcerative colitis with focus on the initial manifestation of the disease, propedeutics and interval between the first symptoms and diagnosis. METHODS: retrospective analysis of non-specific ulcerative colitis with symptoms appearing before 20 years old. Patients were selected in the Pediatrics Gastroenterology Services of the Hospital das Clínicas da Universidade Federal de Minas Gerais and of the Instituto Materno Infantil de Pernambuco. RESULTS: 29 patients with the diagnosis of non-specific ulcerative colitis were identified with fifteen patients (51.7%) of the male sex. The age of the beginning of the symptoms varied between 0.3 to 15.8. The most frequent symptoms of the diagnosis were diarrhea (89.7%) and abdominal pain (82.8%). The most common extra-intestinal manifestation was arthralgia/arthritis (41.4%) The weight for age ratio was below the 3 percentile in 13.8% of the patients. Time interval between symptoms appearance and diagnosis varied between 01 and 10.9 years old. CONCLUSIONS: time interval between initial symptoms and diagnosis remains high in our Region due to many different factors

Yeast killer toxin-like candidacidal Ab6 antibodies elicited through the manipulation of the idiotypic cascade

A mouse anti-anti-anti-idiotypic (Id) IgM monoclonal antibody (mAb K20, Ab4), functionally mimicking a Wyckerhamomyces anomalus (Pichia anomala) killer toxin (KT) characterized by fungicidal activity against yeasts presenting specific cell wall receptors (KTR) mainly constituted by beta-1,3-glucan, was produced from animals presenting anti-KT Abs (Ab3) following immunization with a rat IgM anti-Id KT-like mAb (mAb K10, Ab2). MAb K10 was produced by immunization with a KTneutralizing mAb (mAb KT4, Ab1) bearing the internal image of KTR. MAb K20, likewise mAb K10, proved to be fungicidal in vitro against KT-sensitive Candida albicans cells, an activity neutralized by mAb KT4, and was capable of binding to beta-1,3- glucan. MAb K20 and mAb K10 competed with each other and with KT for binding to C. albicans KTR. MAb K20 was used to identify peptide mimics of KTR by the selection of phage clones from random peptide phage display libraries. Using this strategy, four peptides (TK 1-4) were selected and used as immunogen in mice in the form of either keyhole limpet hemocyanin (KLH) conjugates or peptide-encoding minigenes. Peptide and DNA immunization could induce serum Abs characterized by candidacidal activity, which was inhibited by laminarin, a soluble beta-1,3-glucan, but not by pustulan, a beta-1,6- glucan. These findings show that the idiotypic cascade can not only overcome the barrier of animal species but also the nature of immunogens and the type of technology adopted