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Caring for Gender-Diverse Children and Youth

Journal of Pediatric Health Care - Fri, 03/01/2019 - 00:00
Youth who identify themselves as transgender and gender-diverse (TGD) are traditionally underserved by our current health care system and, as a result, encounter multiple associated risks as well as mental health issues. While society at large has been grappling with issues such as proper pronouns and restroom guidelines, the health care system has also been lacking in promoting gender-affirming clinical environments. Many providers acknowledge a lack of preparation and experience to adequately care for children and youth who identify as TGD, and have called for more formal training, standardized treatment approaches, and the funding of research to further examine approaches to care and health outcomes.
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NAPNAP Position Statement on Health Risks and Needs of Lesbian, Gay, Bisexual, Transgender, and Questioning Youth

Journal of Pediatric Health Care - Fri, 03/01/2019 - 00:00
The overall goal in caring for all youth, including those who are lesbian, gay, bisexual, gender nonconforming, transgender, or questioning (LGBTQ), is to promote normal adolescent development, social and emotional well-being, physical health, and reduce any associated physical and mental health risks (Adelson, Stroeh, & Ng, 2016; American Academy of Pediatrics [AAP], 2013; AAP, 2018). While many LGBTQ youth navigate adolescence as well as their heterosexual peers, others are exposed to social stigma, discrimination, prejudice, and victimization (both mental and physical; Earnshaw et al., 2017).
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It's Time to Lead, Before Someone Else Does

Journal of Pediatric Health Care - Fri, 03/01/2019 - 00:00
As you read this, we will be getting ready to meet in New Orleans for our annual meeting. One of my absolute favorite things about being a NAPNAP member is the opportunity to attend our annual conference. I always walk away with new knowledge that helps me develop clinically and professionally, and the networking opportunities are the best I have ever found. Through the years, I have met and made friends with people from all over the country, and most meetings feel like a family reunion as we all reconnect and share updates about the important things in our lives.
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NAPNAP Position Statement on Age Parameters for Pediatric Nurse Practitioner Practice

Journal of Pediatric Health Care - Fri, 03/01/2019 - 00:00
Creation of exclusive age limits for the provision of health care in pediatric, adolescent, and young adult patients may unnecessarily create barriers and limit access to care continuity for this population (American Nurses Association, National Association of Pediatric Nurse Practitioners [NAPNAP], & Society of Pediatric Nurses, 2015; Hardin, Hacknell, & Committee on Practice and Ambulatory Medicine, 2017; Licensure, Accreditation, Certification and Education [LACE] APRN Network, 2012). Pediatric nurse practitioners (PNPs) have the education, certification, and licensure to provide comprehensive care to all children from birth through young adulthood.
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Implementing an Evidence-Based, Asthma Decision Support Tool for Children Younger Than 5 Years Old

Journal of Pediatric Health Care - Wed, 02/27/2019 - 00:00
Asthma is underdiagnosed, particularly for children younger than 5 years old. Clinical practice guidelines have been shown to improve asthma diagnosis and management, but are underutilized. This evidence-based practice project aimed to develop, implement, and evaluate a three-page decision support tool (DST) to improve the asthma diagnosis process among children younger than 5 years old.
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Catheter-associated bloodstream infections

Paediatrics and Child Health - Wed, 02/27/2019 - 00:00
Each year an estimated quarter million central venous catheters (CVCs) are inserted in the UK for monitoring, delivery of drugs or intravenous feeding and blood sampling in patients receiving critical care. CVCs are indispensable, but the reported rate of catheter associated bloodstream infection (CA-BSI) is high with an incidence varying between 0.46 and 26.5 per 1000 CVC days. CA-BSI is an important cause of adverse clinical outcome, with associated morbidity and mortality, and an additional increased economic cost to the National Health Service.
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Management of meningococcal disease

Paediatrics and Child Health - Wed, 02/27/2019 - 00:00
In recent years remarkable progress has been made in the development of vaccines against the different disease-causing serotypes of Neisseria meningitidis. Despite this, invasive meningococcal disease (IMD) remains a life-threatening illness with significant mortality, morbidity and long term sequelae. Prompt recognition and early treatment with antibiotics are the first steps in its management. Professionals looking after children with suspected IMD should be familiar with its clinical course, so that progression of the disease can be identified early, and its complications including septic shock, coagulopathy and raised intracranial pressure managed aggressively.
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When is difficult asthma severe?

Paediatrics and Child Health - Wed, 02/27/2019 - 00:00
Asthma is the commonest childhood chronic disease in the UK. Most children with asthma are classed as having mild to moderate disease. Regular treatment with inhaled steroids and bronchodilators are enough to successfully control the symptoms. A small proportion of asthmatic children continue to have sub-optimal control despite apparent appropriate therapy (problematic asthma). Most of these children have modifiable factors resulting in poor asthma control (difficult asthma). True therapy-resistant asthma is rare in children and the paediatric community should focus on ensuring the correct diagnosis, identifying and managing modifiable risk factors before using the label severe asthma.
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Newborn screening for inborn errors of metabolism

Paediatrics and Child Health - Sat, 02/23/2019 - 00:00
The aim of screening is to identify newborns who appear healthy but could be at risk of developing rare conditions that can lead to serious complications and if left untreated even death (table 1). The need for prompt and effective intervention in screen positive patients is particularly important in cases of inherited metabolic diseases (IMD). These conditions often have complex or urgent needs and evidence suggests that outcome may be strongly influenced by referral and treatment pathways. Referrals of all IMD screen positive patients are undertaken in co-ordination with a paediatric IMD centres across the UK.
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Self-assessment

Paediatrics and Child Health - Thu, 02/21/2019 - 00:00
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How to use abdominal X-rays in preterm infants suspected of developing necrotising enterocolitis

Roopali Soni
Feb 18, 2019; 0:archdischild-2018-315252v1-edpract-2018-315252
Interpretations
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Phenylketonuria

Paediatrics and Child Health - Mon, 02/18/2019 - 00:00
Phenylketonuria remains one of the most common inborn errors of metabolism. In the UK it is detected on the newborn heel-prick screening sample allowing early treatment with a strict low phenylalanine diet supplemented with artificial amino acids and appropriate vitamin and minerals. Although the long-term prognosis is good, there is an increasing body of evidence highlighting subtle problems in neuropsychological function with slower reaction times and poorer executive function than peers. White matter changes clearly seen on brain magnetic resonance imaging may have some relationship to these neuropsychological difficulties but their significance is not clearly understood.
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A general paediatrician's guide to managing chest drains in children and young people

Paediatrics and Child Health - Fri, 02/15/2019 - 00:00
Chest drains are inserted in to the pleural cavity to evacuate abnormal collections of air or fluid and to maintain a negative intrathoracic pressure. They are usually inserted under general anaesthesia but can be performed under local anaesthesia especially in co-operative older children. Wider availability of small bore pigtail catheters which are inserted using the Seldinger technique under ultrasound guidance has made this procedure relatively simpler and safer.
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Genomic medicine for the paediatrician

Paediatrics and Child Health - Fri, 02/15/2019 - 00:00
Genomic medicine is the use of sequence data from an individual's entire genetic code to aid diagnosis and personalise therapeutic options. An important goal of the 100,000 genomes project in the UK is to embed the infrastructure for the widespread use of whole genome sequencing into the National Health Service. Paediatricians will be at the forefront in using this new technology in the diagnosis of children with multiple congenital anomalies, developmental delay and other suspected genetic conditions.
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Inherited mitochondrial disease

Paediatrics and Child Health - Fri, 02/15/2019 - 00:00
Inherited disorders that affect mitochondrial function are a diverse group of conditions with different pathophysiological mechanisms and highly variable clinical phenotypes. Affected children may present with a defined clinical syndrome or, more commonly, with non-specific signs of mitochondrial disease. Recognition of mitochondrial disease relies on having a high index of suspicion when faced with a multisystem disorder of unclear aetiology, in addition to knowledge of the various defined mitochondrial clinical syndromes.
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Acute presentations of inherited metabolic disorders: investigation and initial management

Paediatrics and Child Health - Thu, 02/14/2019 - 00:00
Inborn errors of metabolism are individually rare, but so many have now been described that the general paediatrician will encounter one from time to time. For many, early treatment is important. Unfortunately, most that present acutely do so with non-specific symptoms and signs. It is therefore necessary to identify and investigate those at high risk. The most common problems are neurological (including coma, seizures and stroke-like episodes), hypoglycaemia, disorders of acid-base regulation, acute liver disease, rhabdomyolysis, cardiomyopathy and sudden collapse.
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Familial hypercholesterolaemia: what’s new?

Paediatrics and Child Health - Thu, 02/14/2019 - 00:00
Autosomal Dominant Familial Hypercholesterolaemia (FH) is the commonest inherited disorder of lipoprotein metabolism. Untreated monogenic FH caused by mutations in the LDLR, APOB or PCSK9 genes result in early onset cardiovascular death (below the age of 60 years). In the UK the prevalence of heterozygous FH is 1 in 270 and homozygous FH is 160,000 approximately.The introduction of statins nearly three decades ago has altered the natural history of FH, with a significant reduction of cardiovascular related morbidity and mortality.
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