Cell-Free DNA Tumor Fraction in the Aqueous Humor Is Associated With Therapeutic Response in Retinoblastoma Patients.

Purpose: The aqueous humor (AH) liquid biopsy enables in vivo evaluation of tumor-derived cell-free DNA (cfDNA) from retinoblastoma (RB) eyes. Herein, we test our hypothesis that longitudinal dynamics of AH cfDNA-including tumor fraction (TFx) and somatic copy number alteration (SCNA) amplitude-correspond to therapeutic response.

Methods: Eyes with ≥3 AH extractions during intravitreal chemotherapy (IVM) or at secondary enucleation between 2015 to 2019 were included.

Increased Hepatic Stiffness in Young Adults After Biventricular Repair of Congenital Heart Disease.

Background: This study evaluated hepatic stiffness by shear wave elastography to investigate subclinical hepatic changes in a cohort of patients with congenital biventricular heart disease (BHD).

Methods: The BHD patients and age-matched healthy controls were prospectively recruited for hepatic ultrasonography and shear wave elastography. Real-time B-mode imaging with Doppler was performed for celiac axis, superior mesenteric artery, and main portal vein, and hepatic shear wave elastography was assessed.

Vincristine-induced peripheral neuropathy: A mini-review.

Vincristine (VCR), an alkaloid extracted from vinca, is often used in combination with other chemotherapeutic drugs to treat a variety of cancers, such as acute lymphoblastic leukaemia (ALL), malignant lymphoma, and neuroblastoma. However, VCR possesses dose-dependent neurotoxicity, which is the main factor restricting its application. Vincristine-induced peripheral neuropathy (VIPN) not only limits the dose of VCR and leads to the discontinuation of treatment but also triggers serious damage to the physical and mental health of patients.

Global Retinoblastoma Treatment Outcomes: Association with National Income Level.

Purpose: To compare metastasis-related mortality, local treatment failure, and globe salvage after retinoblastoma in countries with different national income levels.

Design: International, multicenter, registry-based retrospective case series.

Participants: Two thousand one hundred ninety patients, 18 ophthalmic oncology centers, and 13 countries on 6 continents.

Evaluation of real-time video from the digital indirect ophthalmoscope for telemedicine consultations in retinopathy of prematurity.

Introduction: This study aimed to evaluate the validity of using real-time video indirect ophthalmoscopy for telemedicine consultations for paediatric retinal findings, using retinopathy of prematurity (ROP) as a model disease.

Methods: An ophthalmologist simultaneously performed and recorded routine ROP screenings on enrolled premature infants (aged ≤30 weeks and weighing <1500 g) using the Keeler digital indirect ophthalmoscope. Examinations were graded as no ROP, mild, type 1 or type 2.

Four Risk Factors for Arthrofibrosis in Tibial Spine Fractures: A National 10-Site Multicenter Study.

Background: Tibial spine fractures (TSFs) are relatively rare pediatric injuries. Postoperative arthrofibrosis remains the most common complication, with few studies having examined factors associated with its development.

Purpose: To identify risk factors for arthrofibrosis and required MUA or lysis of adhesions in the largest known cohort of patients with TSFs.

A robust Fourier-based method to measure pulse pressure variability.

Objective: To propose a new method to estimate pulse pressure variability (PPV) in the arterial blood pressure waveform.

Methods: Traditional techniques of calculating PPV using peak finding have a fundamental flaw that prevents them from accurately resolving PPV for small tidal volumes, limiting the use of PPV to only mechanical ventilated patients. The improved method described here addresses this limitation using Fourier analysis of an oscillatory signal that exhibits a time-varying modulation of its amplitude.

Improving Human Milk and Breastfeeding Rates in a Perinatal Hospital in Japan: A Quality Improvement Project.

The leadership team at the Center for Maternal, Fetal and Neonatal Medicine, Saitama Medical Center, Saitama Medical University in Tokyo, Japan sought to improve our human milk (HM) and breastfeeding rates for vulnerable infants. This article describes the quality improvement (QI) initiative, which involved the implementation of the first three steps of the Spatz 10-step model for HM and breastfeeding in vulnerable infants. Our main objectives were to ensure that: (1) families were making informed feeding decisions about HM and breastfeeding as a medical intervention; (2) mothers were pumping early and often; (3) the staff implemented HM management; and (4) the proportion of infants who received HM at 1 month of life increased.

Methamphetamine-associated pulseless electrical activity in a young child.

This is a case report of a 19-month-old female who presented to the emergency department in cardiac arrest after methamphetamine exposure. Prior to presentation, she had seizure-like activity and then became unresponsive. On arrival, she had dilated pupils, intermittent clonus, and pulseless electrical activity.

Electronic Documentation in Pediatrics: The Rationale and Functionality Requirements.

Clinical documentation is a fundamental component of the practice of medicine. It has significantly evolved over the past decade, largely because of the growth of health information technology and electronic health records. Although government agencies and other professional organizations have published position statements on the structure and use of electronic documentation, few have specifically addressed the documentation needs for the care of children.

Electronic Documentation in Pediatrics: The Rationale and Functionality Requirements.

Clinical documentation has dramatically changed since the implementation and use of electronic health records and electronic provider documentation. The purpose of this report is to review these changes and promote the development of standards and best practices for electronic documentation for pediatric patients. In this report, we evaluate the unique aspects of clinical documentation for pediatric care, including specialized information needs and stakeholders specific to the care of children.

Grading TESI: Crypt and villus formation in tissue-engineered small intestine alters with stem/progenitor cell source.

The small intestine has a remarkable ability to enhance its absorptive and digestive surface area through the formation of villi, a process known as villification. We sought to learn whether developing mouse and human tissue-engineered small intestine (TESI) followed known developmental biology routes to villification, such as Sonic hedgehog ()/Indian hedgehog () and bone morphogenetic protein 4 ()/forkhead box F1 () signaling to identify targets to enhance the development of TESI. After generating TESI from prenatal and postnatal stem cell sources, we evaluated the effect of cell source derivation on villification with a grading scheme to approximate developmental stage.

Examining relations between neuropsychological and clinical epilepsy-specific factors with psychopathology and adaptive skills outcomes in youth with intractable epilepsy.

Pediatric epilepsy is a prevalent childhood neurological disorder. Youth with a diagnosis of medically uncontrollable or intractable epilepsy are at increased risk for poor neurocognitive and psychosocial functioning. To date, there is a paucity of clinical research examining and/or characterizing the relations of neuropsychological, clinical, and epilepsy-specific medical factors in clinical outcomes among youth with diagnosed intractable epilepsy.

Thermal Injection Measurement (TIM) Adds Accuracy to Injections and Can Assess Cold Chain Management.

Background: Inaccurate assessment of injected drug delivery may increase cost and morbidity or reduce efficacy. Yet currently most injections are evaluated solely by the formation of a visible wheal that might not truly estimate the actual area of effect. We hypothesized that thermal injection measurement (TIM) might verify appropriate temperature at the time of injection, as required for some temperature-sensitive vaccines and provide more accurate information about the area of delivery.

SMARCB1 loss induces druggable cyclin D1 deficiency via upregulation of MIR17HG in atypical teratoid rhabdoid tumors.

Atypical teratoid rhabdoid tumor (ATRT) is a fatal pediatric malignancy of the central neural system lacking effective treatment options. It belongs to the rhabdoid tumor family and is usually caused by biallelic inactivation of SMARCB1, encoding a key subunit of SWI/SNF chromatin remodeling complexes. Previous studies proposed that SMARCB1 loss drives rhabdoid tumor by promoting cell cycle through activating transcription of cyclin D1 while suppressing p16.

Lessons Learned: Identifying Items Felt To Be Critical to Leading a Pediatric Palliative Care Program in the Current Era of Program Development.

The experience of starting and growing a pediatric palliative care program (PPCP) has changed over the last 10 years as rapid increases of patient volume have amplified challenges related to staffing, funding, standards of practice, team resilience, moral injury, and burnout. These challenges have stretched new directors' leadership skills, yet, guidance in the literature on identifying and managing these challenges is limited. A convenience sample of 15 PPCP directors who assumed their duties within the last 10 years were first asked the following open-ended question: What do you wish you had known before starting or taking over leadership of a PPCP? Responses were grouped into themes based on similarity of content.

A Multicenter, International Collaborative Study for American Joint Committee on Cancer Staging of Retinoblastoma: Part II: Treatment Success and Globe Salvage.

Purpose: To evaluate the ability of the American Joint Committee on Cancer (AJCC) 8th edition to predict local tumor control and globe salvage for children with retinoblastoma (RB).

Design: International, multicenter, registry-based retrospective case series.

Participants: A total of 2854 eyes of 2097 patients from 18 ophthalmic oncology centers from 13 countries over 6 continents.

A Multicenter, International Collaborative Study for American Joint Committee on Cancer Staging of Retinoblastoma: Part I: Metastasis-Associated Mortality.

Purpose: To evaluate the ability of the 8th edition of the American Joint Committee on Cancer (AJCC) Cancer Staging Manual to estimate metastatic and mortality rates for children with retinoblastoma (RB).

Design: International, multicenter, registry-based retrospective case series.

Participants: A total of 2190 patients from 18 ophthalmic oncology centers from 13 countries over 6 continents.

Compartmental Strabismus.

Recent reports confirm innervational compartments of select rectus extraocular muscles as well as the superior oblique. Histopathological and orbital imaging studies demonstrate well defined compartmental innervation of the horizontal rectus muscles with less differentiation in the vertical rectus muscles. Acquired vertical misalignment not associated with cyclovertical muscle dysfunction has been associated with horizontal rectus muscle compartment dysfunction.

Chromosome 6p Amplification in Aqueous Humor Cell-Free DNA Is a Prognostic Biomarker for Retinoblastoma Ocular Survival.

Aqueous humor contains tumor-derived cell-free DNA (cfDNA) and can serve as a liquid biopsy for retinoblastoma. We previously associated somatic copy-number alteration (SCNA) 6p gain with a 10-fold increased risk of enucleation. Here we provide a 2-year update to further explore 6p gain as a prognostic biomarker for ocular survival.

Handoff Communication between Remote Healthcare Facilities.

Introduction: Handoffs and transitions of care are common weak points in healthcare provider communication as patients move between sites. With no consistent pattern of communication between St. Jude Children's Research Hospital (St.

Variability in retinoblastoma genome stability is driven by age and not heritability.

Retinoblastoma (RB) is a childhood intraocular cancer initiated by biallelic inactivation of the RB tumor suppressor gene (RB1 ). RB can be hereditary (germline RB1 pathogenic allele is present) or non-hereditary. Somatic copy number alterations (SCNAs) contribute to subsequent tumorigenesis.