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November 2015

Volume 1  Issue 1  

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Editorial

From Resilience to Empowerment: The Path of Childhood Cancer Survivors

Massimo LM* and Wiley TJ

Treatment outcomes in childhood cancer patients have dramatically improved over the last 40 years, achieving a survival rate above 80%. At the same time, with increased survival the delayed effects linked both to therapies and to the psychosocial implications of the disease itself have become manifest. Those most commonly identified, but in a low percentage, are an increased incidence of organ defects, growth retardation, sterility, second malignancies, and neuropsychological and cognitive disturbances. Published reports on the late health effects and quality of life in childhood cancer survivors focus principally on current perceptions of prominent indicators like social life, education, occupation, fertility and marriage. 

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Research Article

Alpha-1-Antitrypsin Deficiency in Children: Clinical Impact of the Liver Involvement

Rainer Ganschow*, Marijke Sornsakrin, Andrea Briem-Richter, and Enke Grabhorn

Alpha-1-antitrypsin deficiency (AATD) is one of the most common genetic disorders causing pulmonary and hepatic damage. In practice, diagnosis is often too late, when pulmonary destruction or liver damage has become irreversible. There is clear evidence in the literature that affected patients present with clinical and laboratory signs in the neonatal period. We analyzed a large cohort of AATD pediatric patients in order to asses signs and symptoms leading to diagnosis. Furthermore, the hepatological follow-up examination should be evaluated with regard to analyze predictive parameters for the prognosis of liver disease.

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Case Report

Infantile Growth Hormone Deficiency and X- Linked Adrenal Hypoplasia Congenita

Stephanie T. Chung*, Carolyn H. Chi, Morey W. Haymond, George S, Jeha

X-linked adrenal hypoplasia congenita (AHC) is a rare but important cause of primary adrenal insufficiency and can be associated with significant morbidity and mortality. AHC is caused by mutations within the NROB1 gene that codes for the DAX-1 protein, an orphan nuclear receptor essential for the development of the hypothalamic-pituitary-adrenal axis. Affected individuals typically present in early infancy with adrenal insufficiency and growth is usually normal once medical therapy is instituted. Here we report the first case of growth hormone deficiency in an infant with AHC and a novel NR0B1 missense mutation.     

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Research Article

Utility of Phase Angle to Identify Responders with Acute Airway Obstruction in the Emergency Room

Elena Rodriguez, MD, MSP*; Kathryn Giordano, DO; Nicole Green, MD; Milena Hirata Armani, MD, MBA; Magdy W. Attia, MD; and Thomas H. Shaffer, MSE, PhD

Asthma exacerbations represent a significant proportion of pediatric emergency department (ED) visits, with higher at-risk based emergency visits than adults. Management decisions are largely based on respiratory physical examination findings (wheezing, air entry, contraction of accessory respiratory muscles), vital signs [heart rate (HR),  respiratory rate (RR)], and pulse oximetry; currently these components are integrated completely or partially into an emergency severity assessment, creating different clinical scoring systems.   

 

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Research Article

Glucometer Manipulation in Adolescents with Type 1 Diabetes Mellitus

Holley Allen*,MD,MSPH, Rebecca G. Feinberg, MPH, Stacey M.Dipalma MD, Alexander B. Knee, MS

This study sought to determine the frequency of and reasons for manipulation of glucometers in adolescents with Type 1 diabetes. Using a cross sectional study design, adolescents with Type 1 diabetes who attended Baystate Pediatric Endocrinology Clinic, completed a brief, confidential questionnaire assessing intentional glucometer manipulation. Eligible subjects were aged 12-22 years with a Type 1 diabetes diagnosis for greater than two years.                  

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Review Article

Child Sexual Abuse Prevention: Are Safe Environment Training Programs Effective? A Topical Review of the Literature

Angelo P. Giardino MD, PhD, Kavita Desai Esq, Dawn Lew Esq., Mary Jane Doerr, BA, MA, Bernie Nojadera, BA, MSW

Child victims of sexual abuse face a number of short and long-term difficulties as a result of their victimization. Prevention of child sexual abuse is ideal since the victimization would be stopped prior to a child being harmed and suffering the consequences of such betrayal of trust and abuse. The literature surrounding child sexual abuse prevention programs, typically called “safe environment training” is examined to determine the evidence for their effectiveness. This topical review explores the evidence to support core elements in the curricular structure that may indicate effectiveness.                                                                 

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Brief Communication

Three Theories that Explain why Male Antisocial Behavior in Childhood Predicts Male Antisocial Behavior in Adulthood

Robert Eme*

There is strong evidence from prospective longitudinal studies that psychopathology in childhood robustly predicts psychopathology in adulthood. Lahey has recently reviewed three of the major theories for this strong predictive correlation. This article will present a brief overview of these theories as applied to male life-course-persistent (MLCP) antisocial behavior which is arguably the most important of all pediatric mental health problems. MLCP refers to the childhood onset of severe overt conduct problems such as physical aggression, opposition-defiance, and rule-breaking that emerge from early neurodevelopmental (e.g., Attention Deficit Hyperactivity Disorder) and environmental adversity risk factors (e.g., dysfunctional family) which greatly increases the risk for delinquency, adult criminality, and a host of other problems.                   

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