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Chronic Thrombocytopenia as the Initial Manifestation of STIM1-Related Disorders

Pediatric thrombocytopenia has a wide differential diagnosis, and recently, genetic testing to identify its etiology has become more common. We present a case of a 16-year-old boy with a history of chronic moderate thrombocytopenia, who later developed constitutional symptoms and bilateral hand edema with cold exposure. Laboratory evaluation revealed evidence both of inflammation and elevated muscle enzymes. These abnormalities persisted over months. His thrombocytopenia was determined to be immune mediated. Imaging revealed lymphadenopathy and asplenia, and a muscle biopsy was consistent with tubular aggregate myopathy. Ophthalmology evaluation noted photosensitivity, pupillary miosis, and iris hypoplasia. Genetic testing demonstrated a pathogenic variant in STIM1 consistent with autosomal dominant Stormorken syndrome. Our case is novel because of the overlap of phenotypes ascribed to both gain-of-function and loss-of-function pathogenic variants in STIM1, thereby blurring the distinctions between these previously described syndromes. Pediatricians should consider checking muscle enzymes when patients present with thrombocytopenia and arthralgia, myalgia, and/or muscle weakness. Our case highlights the importance of both multidisciplinary care and genetic testing in cases of chronic unexplained thrombocytopenia. By understanding the underlying genetic mechanism to a patient’s thrombocytopenia, providers are better equipped to make more precise medical management recommendations.




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STEPP IN: Working Together to Keep Infants Warm in the Perioperative Period

OBJECTIVES:

Reduce postoperative hypothermia by up to 50% over a 12-month period in children’s hospital NICUs and identify specific clinical practices that impact success.

METHODS:

Literature review, expert opinion, and benchmarking were used to develop clinical practice recommendations for maintaining perioperative euthermia that included the following: established euthermia before transport to the operating room (OR), standardized practice for maintaining euthermia on transport to and from the OR, and standardized practice to prevent intraoperative heat loss. Process measures were focused on maintaining euthermia during these time points. The outcome measure was the proportion of patients with postoperative hypothermia (temperature ≤36°C within 30 minutes of a return to the NICU or at the completion of a procedure in the NICU). Balancing measures were the proportion of patients with postoperative temperature >38°C or the presence of thermal burns. Multivariable logistic regression was used to identify key practices that improved outcome.

RESULTS:

Postoperative hypothermia decreased by 48%, from a baseline of 20.3% (January 2011 to September 2013) to 10.5% by June 2015. Strategies associated with decreased hypothermia include >90% compliance with patient euthermia (36.1–37.9°C) at times of OR arrival (odds ratio: 0.58; 95% confidence interval [CI]: 0.43–0.79; P < .001) and OR departure (odds ratio: 0.0.73; 95% CI: 0.56–0.95; P = .017) and prewarming the OR ambient temperature to >74°F (odds ratio: 0.78; 95% CI: 0.62–0.999; P = .05). Hyperthermia increased from a baseline of 1.1% to 2.2% during the project. No thermal burns were reported.

CONCLUSIONS:

Reducing postoperative hypothermia is possible. Key practices include prewarming the OR and compliance with strategies to maintain euthermia at select time points throughout the perioperative period.




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Office-Based Screening for Sexually Transmitted Infections in Adolescents

Almost 1 in 4 adolescents have a sexually transmitted infection (STI). These infections are preventable through safe sexual practices and routine screening. Pediatricians are the first line of clinical care for adolescents and are well positioned to offer sexual and reproductive health care counseling and services to their patients; yet, there is a paucity of sexual health screening provided at routine health supervision visits. This article addresses the epidemiology of STIs in adolescents, reviews the evidence of current clinical practice, presents recommended STI screening from government and medical agencies, and offers strategies to address barriers to providing care for adolescents and for sexual health screening in primary care.




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Diagnosis and Management of Polycystic Ovary Syndrome in Adolescents

Polycystic ovary syndrome (PCOS) is a common female reproductive disorder that often manifests during adolescence and is associated with disruptions in health-related quality of life. Prompt evaluation and clinical support after diagnosis may prevent associated complications and optimize overall health management. This article incorporates the most recent evidence and consensus guidelines to provide an updated review of the pathogenesis, clinical presentation, diagnostic evaluation, and management strategies for adolescents with this complex condition. We will review the recent international guidelines on PCOS; because the diagnosis of PCOS remains controversial, management of this condition is inconsistent. In 2019, PCOS remains a common, yet neglected, condition, in part, because of the lack of agreement around both diagnosis and management.




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Primary and Secondary Prevention of Youth Suicide

Youth suicide is a national and global public health crisis. Pediatricians can use primary and secondary prevention strategies to intervene with youth before or after the onset of suicidal behaviors. Universal suicide risk screening programs can be used to identify youth in medical settings who may otherwise pass through the health care setting with undetected suicide risk. Pediatricians are uniquely positioned to help foster resilience in their young patients and equip families of at-risk youth with safety plans and lethal means safety counseling. Pediatricians on the frontlines of this critical public health crisis require education and training in detecting suicide risk, managing those who screen positive, and connecting their patients to much needed mental health interventions and treatments. Evidence-based suicide risk screening and assessment tools, paired with interventions, are feasible and potentially life-saving in the medical setting.




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Technology Use for Adolescent Health and Wellness

As avid users of technology, adolescents are a key demographic to engage when designing and developing technology applications for health. There are multiple opportunities for improving adolescent health, from promoting preventive behaviors to providing guidance for adolescents with chronic illness in supporting treatment adherence and transition to adult health care systems. This article will provide a brief overview of current technologies and then highlight new technologies being used specifically for adolescent health, such as artificial intelligence, virtual and augmented reality, and machine learning. Because there is paucity of evidence in this field, we will make recommendations for future research.




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Marijuana Legalization and Youth

Various states have legalized marijuana for medical purposes and/or decriminalized recreational marijuana use. These changes coincide with a decrease in perceived harmfulness of the drug and an increase in its use among youth. This change is of critical concern because of the potential harmful impact of marijuana exposure on adolescents. Marijuana use has been associated with several adverse mental health outcomes, including increased incidence of addiction and comorbid substance use, suicidality, and new-onset psychosis. Negative impacts on cognition and academic performance have also been observed. As the trend toward legalization continues, the pediatric community will be called on to navigate the subsequent challenges that arise with changing policies. Pediatricians are uniquely positioned to provide innovative care and educate youth and families on the ever-evolving issues pertaining to the impact of marijuana legalization on communities. In this article, we present and analyze the most up-to-date data on the effects of legalization on adolescent marijuana use, the effects of adolescent use on mental health and cognitive outcomes, and the current interventions being recommended for use in pediatric office settings.




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Management of Opioid Misuse and Opioid Use Disorders Among Youth

In response to the growing impact of the current opioid public health crisis in the United States on adolescents and young adults, pediatricians have an expanding role in identifying opioid use early, preventing escalation of risky use, reducing opioid-related harms, and delivering effective therapies. Research and expert consensus suggest the use of brief interventions focused on reducing risks associated with ongoing opioid use and using motivational interviewing strategies to engage youth in treatment. Because fatal opioid overdose remains a major cause of opioid-related mortality among youth, delivering overdose education as part of any visit in which a youth endorses opioid use is one evidence-based strategy to decrease the burden of opioid-related mortality. For youth that are injecting opioids, safe injection practices and linkage to needle or syringe exchanges should be considered to reduce complications from injection drug use. It is crucial that youth be offered treatment at the time of diagnosis of an opioid use disorder (OUD), including medications, behavioral interventions, and/or referral to mutual support groups. The 2 medications commonly used for office-based OUD treatment in adolescents are extended-release naltrexone (opioid antagonist) and buprenorphine (partial opioid agonist), although there is a significant treatment gap in prescribing these medications to youth, especially adolescents <18 years of age. Addiction is a pediatric disease that pediatricians and adolescent medicine physicians are uniquely poised to manage, given their expertise in longitudinal, preventive, and family- and patient-centered care. Growing evidence supports the need for integration of OUD treatment into primary care.




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A picture of transformation

A remarkable story which came to a climax on February 23 this year when OM Ireland took possession of the deeds to Lacken House as their new headquarters and ministry centre.




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Meet Anna with OM Ireland

Canadian by birth, but Papua New Guinean at heart, Anna Stanton shares how God led her to serve with OM Ireland.




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Donkey teaches Irish children true meaning of Christmas

The Creative Arts team perform their Christmas show for school children all over Ireland in the course of three weeks.




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Golf and the gospel

OM Ireland and FCA Golf hold a four-day golf camp in Co. Westmeath, Ireland, to promote golf and the gospel.




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Impacting Ireland

Participants from all over the world joined together in Ireland for two weeks of training and outreach in partnership with local churches.




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Camel and donkey preach the gospel to thousands

OM Ireland's two multi-media puppet shows perform over 75 times in schools around Ireland. The gospel message is expected to be heard by thousands.




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St. Patrick's message still relevant

St. Patrick's Day celebrates the patron saint of Ireland known for bringing Christianity to Ireland. Hundreds of years later, OM Ireland brings the same message.




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OM Sweden and OM Ireland team up over Easter

OM Sweden joined OM Ireland for outreach over the Easter holiday in Carlow town.




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Participants have a plan but rely on God to act

OM Ireland's biggest outreach of the year teaches participants to commit their ways to Him and trust Him to act.




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We ran out of Bibles

OM Ireland journalist Hannah Rueber volunteered for a school programme and witnessed something truly incredible.




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Transformed by love

One Irish family experienced the transforming love of God...one woman at a time.




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Trained and equipped in Ireland

Through training at OM, Rebecca became more confident sharing Jesus in her home country.




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Continuing to plant seeds

The opportunity to continue planting gospel seeds keeps drawing short-term outreach participants back to Ireland.




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The truth and rain

The Africa Trek team reaches out to the Damara people in Namibia.




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Standing on the Father's Rock

Young men from African nations find their identity and destiny through hiking in Namibia with the Wilderness Therapy Programme.




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The invisible woman

Walvis Bay, Namibia :: Local women attend a challenging discipleship event on board Logos Hope




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Life and light on the Kuiseb

Two native Namibians stand as the only Christians in their community




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The Namibian road to Emmaus

A new missions experience in Namibia is not as much a programme as it is a journey.




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Australian Floods

An update about the flood damage in Eastern Australia




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Time of growth and change at TeenStreet

God changes teens' lives during TeenStreet Queensland in Australia at the beginning of July.




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Life-changing moment

Peter* from MAP Australia has a Muslim friend who attended a church camp. His friend has never been the same since.




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Prayer and the Word of God changes lives

OM worker is amazed to see God change lives, as he peers over his book table on a busy Muslim street in Australia.




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More than coffee and sweets

MAP Australia hosts Bound for Paradise, a week-long outreach to Muslims from the Persian Gulf in Queensland, Australia, from 25-29 August.




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From Afghanistan to Australia

A former Afghan fighter discovers Jesus Christ in the Qur’an.




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The way I see it  - When it means what it says

It matters not if we are from 200 countries; we are one in Christ and shall be for eternity. OMNI-team member Greg Kernaghan about ‘globalisation’.




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A Sporting Infusion for Ukrainian Summer Camps

OM Ukraine's sports team equips and trains churches in using non-traditional sports in their summer camps.




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Lost and found

God restores a lost bag to the OM team during a short-term outreach to school children in northern Russia.




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Teaching spiritual and physical fitness

An OM Ukraine sports team member helps young Ukrainian women gain a healthier understanding and appreciation of the bodies God gave them.




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Biodiesel plant fuels relief efforts in Ukraine

A Ukrainian pastor responds to nearly 100 per cent unemployment in his village by starting biofuel and cash crop business enterprises.




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Visiting orphans

The OM team in Odessa, Ukraine, regularly visits orphanages in the province, bringing love and the good news to children.




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Can you find meaning in failure?

A Christian football coach experiences what he says is the best gift from God, something “so much more than winning”.




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Looking after orphans

OM Ukraine's team in Odessa heads up the annual All-Ukrainian Day of Prayer for Orphans in the Odessa province.




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Summer camp leads to heart change

During the summer months, OM Ukraine in Odessa facilitated a camp, during which the Lord touched the hearts of many children.




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War, winter and witnesses in the Ukraine

Ministering in a country at war is not fun – but a privilege. Ukraine is going through intense emotions and people are open to Kingdom conversations.




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OM lending a hand to start the healing

OM supported the Ukrainian Military Ministry in order to print trauma counselling books




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Church planting within the conflict zone

A report of results: OM has been supporting church planting efforts and training local missionary teams to plant churches nearby and within the conflict area.




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OM making an impact: A personal account of a refugee

A 29 year old recounts how his family fled the war zone and came to stay at OM Odessa centre




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Anya's story (stories from Ukrainian refugees)

A 17 year old high school student shares about how she fled from the war zone and came to stay at OM Odessa's centre.




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New Soccer and sports ministry in Odessa, Ukraine

Through the "Lighthouse" soccer team, Maksim Shlikov is reaching out to young orphanage graduates looking for meaning and hope.




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Failing business man finds hope and purpose

A welder's life is renewed in Kaharlyk, Ukraine through a new business opportunity with OM.




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Odessa orphans: his heart's home

Long-term follower of Jesus, Nikolay, stepped out in faith to help orphans in Ukraine, and partners with OM in this ministry to children.




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Bus4Life brings literature and life to Europe

Bus4Life is OM’s mobile missions centre to the unreached people of Central and Eastern Europe, bringing books and also participating in summer programmes, as it did in summer 2017.