Distal toxic acral erythema and mucositis secondary to 6-mercaptopurine in a thiopurine methyltransferase "super shunter".

Toxic erythema of chemotherapy is a broad but useful diagnosis used to summate the non-infectious, non-allergic, and reproducible reaction of certain chemotherapeutics. Due to overlapping chemotherapy side effects and often multiple drug exposures, identification of a singular culprit drug is challenging for dermatologists. Herein, we report a patient with 6-mercaptopurine (6-MP) toxic erythema confirmed via toxic metabolite markers secondary to increased levels of thiopurine methyltransferase activity, or so called "super shunting.

Simultaneous Home-Based Pediatric Hospice and Palliative Care: Characterizing the Population.

Children and adolescents with serious conditions may benefit from simultaneous hospice and palliative care. Although the Affordable Care Act covers concurrent care, uptake has been limited. Limited descriptive data exist on receiving simultaneous community hospice and pediatric palliative care.

Clinical and molecular characterization of a large primary hyperoxaluria cohort from Saudi Arabia: a retrospective study.

Background: Primary hyperoxalurias (PHs) constitute rare disorders resulting in abnormal glyoxalate metabolism. PH-associated phenotypes range from progressive nephrocalcinosis and/or recurrent urolithiasis to early kidney failure.

Methods: A retrospective study was conducted for patients with confirmed PH diagnoses from three tertiary centers in Saudi Arabia.

The Impact of Organ Procurement Injury on Transplant Organ Availability.

Patients on the transplant waiting list continue to have a significant wait time as organ supply remains low. Many initiatives have been undertaken in the last few years to attempt to increase the organ allograft supply. As organ procurement organizations have attempted to increase their procurement of organs from deceased donors, emphasis has been placed on avoidance of injury to organs during procurement.

Renal Interstitial Invasion by Group A : A Rare Presentation.

We report the case of a 5-year-old who had interstitial invasion of his kidneys with group A (GAS). Glomeruli and tubules were relatively preserved. He recovered from this event and was admitted a couple of months later with dilated cardiomyopathy needing a heart transplant.

Papilledema as the Sole Manifestation of Neuroborreliosis.

Lyme disease is reportable, and approximately 30,000 cases/year are notified to the local and state health departments. However, other estimates based on insurance records suggest there are close to 476,000 cases/year that are diagnosed and treated. In addition to a large burden of illness, areas where Lyme disease is common are expanding.

Relapsed Symptoms in a Patient Treated for Pubic Symphysis Septic Arthritis/Adjacent Osteomyelitis: Diagnostic Challenges.

We describe the case of a 14-year-old girl with relapsed pain following adequate treatment of pubic symphysis septic arthritis with adjacent osteomyelitis. Evaluation of her symptoms was challenging, because magnetic resonance imaging (MRI) was not helpful and repeat surgical exploration was not favored. She was treated with a combination of prolonged antimicrobial therapy and local steroid injection.

Perianesthesia Nurses Pain Management Practices: Findings and Recommendations From a National Descriptive Study of Members of the American Society of Perianesthesia Nurses.

Purpose: The purpose of this study was to identify current individual practice of perianesthesia nurses regarding assessment and documentation of pain.

Design: Descriptive cross-sectional design using vignette technique.

Methods: Vignettes with questions available via electronic survey offered to attendees of the 2017 American Society of PeriAnesthesia Nurses National Conference.

Cranial Suture Evaluation on Routine Pediatric MRI.

Purpose: The efficacy of magnetic resonance imaging (MRI) for evaluating sutures has not been well studied. CT with 3-dimensional reformats is currently the preferred modality for imaging the major cranial sutures. The role of MRI is primarily is for evaluating the brain for any concurrent malformations.

Aberrant epigenetic silencing of neuronatin is a frequent event in human osteosarcoma.

The paternally imprinted neuronatin () gene has been identified as a target of aberrant epigenetic silencing in diverse cancers, but no association with pediatric bone cancers has been reported to date. In screening childhood cancers, we identified aberrant CpG island hypermethylation in a majority of osteosarcoma (OS) samples and in 5 of 6 human OS cell lines studied but not in normal bone-derived tissue samples. CpG island hypermethylation was associated with transcriptional silencing in human OS cells, and silencing was reversible upon treatment with 5-aza-2'-deoxycytidine.

Genetic susceptibility to aminoglycoside ototoxicity.

Introduction: Aminoglycosides are a well-known clinically relevant antibiotic family used to treat bacterial infections in humans and animals and can produce toxic side effects. Aminoglycoside-induced hearing loss (HL) has been shown to have a genetic susceptibility. Mitochondrial DNA mutations have been implicated in inherited and acquired hearing impairment.

Clinical Guidelines in Pediatric Hearing Loss: Systemic Review Using the Appraisal of Guidelines for Research and Evaluation II Instrument.

Objectives: Despite the importance, impact, and prevalence of pediatric hearing loss (HL), there are very few published clinical practice guidelines (CPG) supporting the evaluation and management of pediatric patients with HL. Our objective was to appraise existing CPGs to ensure safe and effective practices.

Methods: A literature search was conducted in PubMed, Google Scholar, EBSCO, as well as a manual Google search.

Congenital intestinal hypoganglionosis: A radiologic mimic of Hirschsprung's disease.

Intestinal hypoganglionosis or isolated hypoganglionosis is a rare entity with a clinical and radiologic presentation that can mimic Hirschsprung's disease in the neonatal period. The diagnosis of this entity can be challenging with suction rectal biopsies that are standard for diagnosing Hirschsprung's disease. We present this case of congenital intestinal hypoganglionosis detailing the neonatal course, due to its rarity and the conundrums faced before an eventual diagnosis could be rendered.

Educational implications for surgical telementoring: a current review with recommendations for future practice, policy, and research.

Background: Surgical telementoring (ST) was introduced in the sixties, promoting videoconferencing to enhance surgical education across large distances. Widespread use of ST in the surgical community is lacking. Despite numerous surveys assessing ST, there remains a lack of high-level scientific evidence demonstrating its impact on mentorship and surgical education.

Unusual case of recurrent SMART (stroke-like migraine attacks after radiation therapy) syndrome.

Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare delayed complication of cerebral radiation therapy. A 53-year-old female initially presented with headache, confusion and left homonymous hemianopia. Her medical history was notable for cerebellar hemangioblastoma, which was treated with radiation in 1987.

Headache and Chiari I Malformation in Children and Adolescents.

Headache is a common problem in children and adolescents. Its recurrent and disabling nature may lead to use of neuroimaging to exclude secondary causes of headache such as Chiari I malformation (CM I). CM I has a variety of presentation with headache being the most common symptom.

Dedicated pediatric behavioral health unit: serving the unique and individual needs of children in behavioral health crisis.

Pediatric mental health emergencies are an increasing part of emergency medical practice because emergency departments have become the safety net for a fragmented mental health infrastructure that is experiencing critical shortages in services in all sectors. The emergency services for behavioral health unit at Akron Children's Hospital is an innovative model for delivering care to pediatric patients with mental health emergencies. A multidisciplinary team using the expertise of emergency services, psychiatry, social work, parent advisory counsel, security services, and engineering/architecture developed the emergency services for behavioral health unit blueprint, process, and staffing model.