Management of Adrenal Insufficiency Risk After Long-term Systemic Glucocorticoid Therapy in Duchenne Muscular Dystrophy: Clinical Practice Recommendations.

Long-term glucocorticoid therapy has improved outcomes in patients with Duchenne muscular dystrophy. However, the recommended glucocorticoid dosage suppresses the hypothalamic-pituitary-adrenal axis, leading to adrenal insufficiency that may develop during severe illness, trauma or surgery, and after discontinuation of glucocorticoid therapy. The purpose of this review is to highlight the risk of adrenal insufficiency in this patient population, and provide practical recommendations for management of adrenal insufficiency, glucocorticoid withdrawal, and adrenal function testing.

Health Utility Scores of Atopic Dermatitis in US Adults.

Background: The impact of atopic dermatitis (AD) on health-related quality of life and health utility in the US adult population is not well established.

Objective: To determine the health utilities and quality-adjusted life-years (QALYs) lost in adults with AD versus without AD in the US population.

Methods: A cross-sectional, population-based study of 3495 adults was performed.

Pediatric Adrenal Insufficiency: Diagnosis, Management, and New Therapies.

Adrenal insufficiency may result from a wide variety of congenital or acquired disorders of hypothalamus, pituitary, or adrenal cortex. Destruction or dysfunction of the adrenal cortex is the cause of primary adrenal insufficiency, while secondary adrenal insufficiency is a result of pituitary or hypothalamic disease. Timely diagnosis and clinical management of adrenal insufficiency are critical to prevent morbidity and mortality.

Post-viral atopic airway disease: pathogenesis and potential avenues for intervention.

: In early childhood, wheezing due to lower respiratory tract illness is often associated with infection by commonly known respiratory viruses such as respiratory syncytial virus (RSV) and human rhinovirus (RV). How respiratory viral infections lead to wheeze and/or asthma is an area of active research. : This review provides an updated summary of the published information on the development of post-viral induced atopy and asthma and the mechanisms involved.

Growth Hormone Deficiency and Nonalcoholic Fatty Liver Disease with Insights from Humans and Animals: Pediatric Implications.

In addition to its growth promoting role, growth hormone (GH) has a significant effect on intermediary metabolism in the well state. Despite the latter fact, pediatric practitioners are usually focused on the growth promoting aspects of GH as opposed to those metabolic. In recent years various animal and human studies (in adults mainly) and clinical reports in children have repeatedly shown the association of GH deficiency (GHD) and fatty liver disease.

Profile of asfotase alfa in the treatment of hypophosphatasia: design, development, and place in therapy.

Hypophosphatasia (HPP) is a multi-systemic metabolic disorder caused by loss-of-function mutations in the gene that encodes the mineralization-associated enzyme, tissue-nonspecific alkaline phosphatase (TNSALP). HPP is characterized by defective bone and dental mineralization, leading to skeletal abnormalities with complications resulting in significant morbidity and mortality. Management of HPP has been limited to supportive care until the introduction of a recently approved enzyme replacement therapy employing bone-targeted recombinant human TNSALP, asfotase alfa (AA).

Early Antibiotic Exposure and Adverse Outcomes in Preterm, Very Low Birth Weight Infants.

Objectives: To determine whether antibiotic use in the first 14 postnatal days in preterm, very low birth weight (birth weight of ≤1500 g) infants is associated with risk after 14 days of age for late-onset sepsis, necrotizing enterocolitis (NEC), or death after controlling for severity of illness using the Clinical Risk Index in Babies II score, and determine whether duration of antibiotic exposure was associated with risk of adverse outcomes.

Study Design: This retrospective cohort study included very low birth weight infants born at ≤32 weeks of gestation admitted to the neonatal intensive care unit from September 2010 to June 2014. Infants were excluded if they had major congenital anomalies or culture-proven sepsis, NEC, or death during the first 14 days of life.

Association of atopic dermatitis with allergic, autoimmune, and cardiovascular comorbidities in US adults.

Background: Atopic dermatitis (AD) has been associated with multiple comorbid extracutaneous and systemic disorders. The relation between AD severity and disease comorbidities is complex and not fully understood.

Objective: To determine the complex relation between AD severity and comorbidities.

Patient burden and quality of life in atopic dermatitis in US adults: A population-based cross-sectional study.

Background: The patient burden and quality of life (QOL) impact of atopic dermatitis (AD) in the United States population is not well established.

Objective: To elucidate the patient burden of AD in the US population.

Methods: A cross-sectional, population-based study of 602 adults was performed.

Heterogeneity and the origins of asthma.

Objective: To examine the roots of asthma across different ages, including atopy, the role of the microbiome and viral infections, and comorbidities and confounders, such as obesity, aspirin-exacerbated respiratory disease, neutrophilic asthma, cigarette smoking, and the possibility of an asthma-chronic obstructive pulmonary disease overlap syndrome.

Data Sources: Data were taken from various scientific search engines, including PubMed and Science Direct databases.

Study Selections: Articles that reviewed information on the origins of asthma in persons of all ages, including different phenotypes and genotypes of asthma, were used.

Exploring knowledge and perceptions of palliative care to inform integration of palliative care education into cystic fibrosis care.

Background: Individuals with cystic fibrosis (CF) face the challenges of managing a chronic, progressive disease. While palliative care is a standard of care in serious illnesses, there are no guidelines for its incorporation into CF care. Patients with CF, caregivers, and CF care providers may lack knowledge about palliative care and perceive barriers to integrated care.

Detection of cytomegalovirus in saliva from infants undergoing sepsis evaluation in the neonatal intensive care unit: the VIRIoN-C study.

Objective To determine the frequency of detection of cytomegalovirus (CMV) among infants evaluated for late-onset sepsis in the neonatal intensive care unit (NICU). Methods This study was a prospective cohort study. Results During the 13-month study, 84 infants underwent 116 sepsis evaluations, and CMV DNA was detected in saliva in three (4%) infants (median: gestational age 28 weeks, birth weight 950 g), representing 5% (n=6) of all sepsis evaluations.

A review of the conundrum of mild hypoxic-ischemic encephalopathy: Current challenges and moving forward.

A review of the conundrum called mild hypoxic-ischemic encephalopathy (HIE) is provided. During the past decades, the definition of HIE has evolved to accommodate the short window of time required for the initiation of therapeutic hypothermia. Also, neurological evaluations have changed with the use of simpler staging systems that can be applied within the first 6 h of life.

In iodine-induced thyrotoxicosis, steroid therapy today could keep the surgical knife at bay.

Background: Amiodarone-induced thyrotoxicosis (AIT) type 2, characterized as a destructive thyroiditis, is well described in the medical literature; however, iodine-induced thyrotoxicosis (IIT) is not, though the latter has similar features and can be managed similarly.

Case Presentation: We present a 17-year-old female who presented with a history of an intermittent goiter with thyroid function tests (TFTs): thyroid-stimulating hormone (TSH)<0.015 (0.

Dexmedetomidine as Single Continuous Sedative During Noninvasive Ventilation: Typical Usage, Hemodynamic Effects, and Withdrawal.

Objectives: Dexmedetomidine use in pediatric critical care is increasing. Its prolonged effects as a single continuous agent for sedation are not well described. The aim of the current study was to describe prolonged dexmedetomidine therapy without other continuous sedation, specifically the hemodynamic effects, discontinuation strategies, and risk factors for withdrawal.

Zoledronic acid in pediatric metabolic bone disorders.

Zoledronic acid (ZA), a highly potent intravenous bisphosphonate (BP), has been increasingly used in children with primary and secondary osteoporosis due to its convenience of shorter infusion time and less frequent dosing compared to pamidronate. Many studies have also demonstrated beneficial effects of ZA in other conditions such as hypercalcemia of malignancy, fibrous dysplasia (FD), chemotherapy-related osteonecrosis (ON) and metastatic bone disease. This review summarizes pharmacologic properties, mechanism of action, dosing regimen, and therapeutic outcomes of ZA in a variety of metabolic bone disorders in children.

The effect of body position on esophageal reflexes in cats: a possible mechanism of SIDS?

BackgroundIt has been hypothesized that life-threatening events are caused by supraesophageal reflux (SER) of gastric contents that activates laryngeal chemoreflex-stimulated apnea. Placing infants supine decreases the risk of sudden infant death syndrome (SIDS). The aim of this study was to determine whether body position affects esophageal reflexes that control SER.

Asfotase alfa treatment for 1 year in a 16 year-old male with severe childhood hypophosphatasia.

We describe the clinical outcome of asfotase alfa therapy in a 16-year-old boy with severe childhood hypophosphatasia (HPP), who began therapy at age 15 years. The patient was diagnosed with HPP at age 2 years when he presented with genu varum and premature loss of primary teeth. He had a history of multiple fractures requiring 16 orthopedic surgeries with rod and pin placement in his lower extremities.

Pulmonary sequelae and functional limitations in children and adults with bronchopulmonary dysplasia.

Preterm infants with bronchopulmonary dysplasia (BPD) often suffer from life-long pulmonary impairments in pulmonary physical function. This review summarizes our current understanding of the chronic pulmonary impairments and physical functional limitations associated with BPD from preterm birth to adulthood. It also identifies opportunities for intervention in children and adults living with chronic lung disease (CLD) after preterm birth.