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  • ISSN: 2373-9312
    Volume 2, Issue 2
    Velinov M*
    Neuronal Ceroid Lipofuscinoses (NCL) are a group of progressive neurodegenerative disorders leading to early death. NCL as a group is considered to be the most common neurodegenerative disorder with childhood onset [1,2].
    Joseph Gigante*
    Feedback has been defined as “an informed, non-evaluative, and objective appraisal of past performance that is aimed at improving future performance” [1]. It is a critical element in the acquisition and development of clinical skills.
    Clinical Image
    Tatyana Vayngortin1* and Susanna Felsenstein2
    Our patient is a 5 year old male with ectodermal dysplasia and no past history of infectious complications, who presented with 5 weeks of daily fevers and bilateral knee pain. His absence of sweat glands resulting in frequent episodes of hyperthermia had contributed to the delayed diagnosis of fevers. His presentation had been preceded by two episodes of sore throat, which had been treated with two 10 day courses of Amoxicillin. Subsequently he developed an intermittent rash appearing on his arms, trunk, and abdomen, bilateral knee swelling, and multiple palpable lesions on his occiput, lower back, elbows, and hands.
    Case Report
    Tatyana Vayngortin1*#, Anita K. Pai1#, and Daniel W. Thomas2
    Abstract: Adenovirus is a common viral infection with variable clinical presentations, including respiratory tract infection, conjunctivitis, and gastroenteritis. In immunocompromised patients, it can present more severely with liver failure, renal failure, encephalitis, pancreatitis, myocarditis, and even disseminated disease. We report a case of acute liver failure caused by adenovirus in a previously healthy two-year-old child, whose illness spontaneously resolved. Although adenovirus is a rare cause of fulminant liver failure, it should be considered in the differential diagnosis, even in presumably immunocompetent children.
    Research Article
    Yao-Te Hsieh1, Colleen Garvey1, Andrea Osborne1, Stephanie Shishido1, Eun Ji Gang1, Halvard Bönig2 and Yong-Mi Kim1*
    Abstract: Objective: We showed previously that alpha4 blockade using a humanized monoclonal antibody against integrin alpha4, Natalizumab, antagonizes stromal adhesion of pre-B ALL cells and in combination with chemotherapy eliminates resistant pre-B-ALL in an MRD setting. Here we determined the effect of Natalizumab on adherence of patient- derived T-ALL cells in vitro and on survival prolongation of murine recipients of T-ALL cells, to explore the potential of Natalizumab as a novel T-ALL treatment strategy.
    Methods: Adhesion to stromal matrix proteins in the presence/absence of Natalizumab and survival of murine recipients of primary T-ALL cells treated with/without Natalizumab were assessed, using previously described methods.
    Results: Natalizumab inhibited adhesion of patient-derived T-ALL cells and attenuated leukemia progression, resulting in prolonged survival of recipient NOD/SCID IL2R gamma-/- mice of patient-derived T-ALL.
    Conclusion: Alpha4 blockade interferes with adhesion of T-ALL cells to its counter receptor and thus merits evaluation as a novel adjuvant strategy for T-ALL. Further study is needed to explore at the molecular level the effect of alpha4 blockade in T-ALL.
    Mathew George1*, Lindsey Korbel2, Maya Haasz1, Alvaro Coronado1 and Joseph Kitzmiller2
    Abstract: Asthma is common in pediatric patient populations, and acute asthma exacerbations are one of the leading causes for emergency department visits in pediatric hospitals. Several methods are used to evaluate the severity of asthma exacerbations, including the pediatric respiratory assessment measure and peak flow measurements. This study aims to determine which method is superior for advancing treatment intervals in a pediatric patient population. Fifty-six asthmatic patients between the ages of 12 and 16 were included in the study. Treatment intervals for those in Groups 1 and 2 were advanced based on peak flow measurements and pediatric respiratory assessment measure scores, respectively. The total number of treatments and the number of q2h treatments required before discharge were significantly lower for the group for which treatment intervals were advanced based on peak flow measurements. Protocols utilizing peak flow measurements may be more cost effective than protocols based on pediatric respiratory assessment scores. This may be especially relevant in resource-poor settings, and further investigation is warranted.
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