Strategies reducing risk of surgical-site infection following pediatric spinal deformity surgery.

Background: Identifying beneficial preventive strategies for surgical-site infection (SSI) in individual patients with different clinical and surgical characteristics is challenging. The purpose of this study was to investigate the association between preventive strategies and patient risk of SSI taking into consideration baseline risks and estimating the reduction of SSI probability in individual patients attributed to these strategies.

Methods: Pediatric patients who underwent primary, revision, or final fusion for their spinal deformity at 7 institutions between 2004 and 2018 were included.

Intermittent boluses of balanced salt solution for post-operative intravenous hydration following elective major abdominal and thoracic surgery in children.

Purpose: Maintenance fluids following major operations in children are typically administered with a continuous rate. We hypothesized that administering fluids as intermittent boluses is more physiologic and could limit post-operative fluid volume, thereby avoiding harmful effects of excess fluid.

Methods: We retrospectively reviewed children aged 1-21 admitted after an elective major abdominal or thoracic operation from 2015 to 2021.

Surgical considerations for neuroblastoma-associated opsoclonus-myoclonus-ataxia syndrome: a series of 14 patients from a single institution.

Introduction: Neuroblastoma is a childhood cancer of neural crest cells occasionally associated with opsoclonus-myoclonus-ataxia syndrome (OMAS), a paraneoplastic process characterized by ataxia, rapid eye movements, and muscle twitching. OMAS treatment and outcomes are well studied, but prior reports do not detail how the presence of OMAS should impact surgical approach, particularly for tumors with image defined risk factors (IDRF).

Methods: We reviewed patients with neuroblastoma and OMAS at our institution from January 2009 to December 2020 and recorded tumor characteristics, operative details, OMAS therapies, and outcomes.

Purification of Lumbricus terrestris erythrocruorin (LtEc) with anion exchange chromatography.

The naturally extracellular hemoglobin (erythrocruorin) of the Canadian nightcrawler, Lumbricus terrestris (LtEc), is a unique oxygen transport protein that may be an effective substitute for donated human blood. Indeed, this ultra-high molecular weight (~3.6 MDa) hemoglobin has already been shown to avoid the side effects associated with previous hemoglobin-based oxygen carriers and its high thermal stability (T = 56°C) and resistance to heme oxidation (k = 0.

Echocardiographic Screening of Cardiovascular Status in Pediatric Sickle Cell Disease.

Although elevated right ventricular pressure and left ventricular diastolic dysfunction measured by echocardiogram are independent predictors of death in adults with sickle cell disease (SCD), the utility of routine echocardiographic screening in the pediatric population is controversial. We performed a 3-year retrospective review of children ≥ 10 years of age with SCD who underwent an outpatient transthoracic echocardiogram as part of a screening program. Of 172 patients referred for screening, 105 (61%) had a measurable tricuspid regurgitation jet velocity (TRV): median 2.

Patient-proxy agreement on health-related quality of life in juvenile fibromyalgia syndrome.

Background: Health-related quality of life (HRQoL) measures serve as important indicators of pain-related physical and psychosocial function in youth with juvenile fibromyalgia syndrome (JFMS). While the administration of parent-proxy reported HRQoL measures in the assessment of JFMS is common, its added clinical value to patient self-reports is unclear. We aimed to determine the level of agreement on HRQoL among patients with JFMS as well as their parent-proxies and to determine factors associated with this agreement.

What Is New in Prenatal Skeletal Dysplasias?

Objective: The purpose of this article is to discuss advances in imaging and diagnosis of skeletal dysplasias.

Conclusion: Skeletal dysplasias are a heterogeneous group of disorders affecting bone and cartilage and characterized by abnormal shape, growth, and integrity of the skeleton. These disorders may be inherited in a multitude of genetic patterns-autosomal dominant, autosomal recessive, somatic mosaic, imprinting errors of metabolism, X-linked, and teratogenic exposure.

Preventing Distal Junctional Kyphosis by Applying the Stable Sagittal Vertebra Concept to Selective Thoracic Fusion in Adolescent Idiopathic Scoliosis.

Study Design: Multicenter retrospective review.

Objective: To assess the effectiveness of using the stable sagittal vertebra (SSV) for selecting the lowest instrumented vertebrae (LIV) to prevent distal junctional kyphosis (DJK) in selective thoracic fusions.

Summary Of Background Data: Cho et al.

MRI of Native Knee Cartilage Delamination Injuries.

Objective: The purpose of this article is to describe the normal imaging appearance of cartilage and the pathophysiologic findings, imaging appearance, and surgical management of cartilage delamination.

Conclusion: Delamination injuries of knee cartilage signify surgical lesions that can lead to significant morbidity without treatment. These injuries may present with clinical symptoms identical to those associated with meniscal injury, and arthroscopic identification can be difficult, thereby creating a role for imaging diagnosis.

Epileptic Encephalopathies as Neurodegenerative Disorders.

The epileptic encephalopathies are severe and often treatment-resistant conditions that are associated with a progressive disturbance of brain function, resulting in a broad range of neurological and non-neurological comorbidities. The concept of epileptic encephalopathies entails that the encephalopathy aspect of the overall condition is primarily driven by the epileptic activity of the disease, which often manifests as specific and pathological features on the electroencephalogram. Genetic factors in epileptic encephalopathies are increasingly recognized.

Tracheobronchomalacia Is Associated with Increased Morbidity in Bronchopulmonary Dysplasia.

Rationale: Tracheobronchomalacia is a common comorbidity in neonates with bronchopulmonary dysplasia. However, the effect of tracheobronchomalacia on the clinical course of bronchopulmonary dysplasia is not well-understood.

Objective: We sought to assess the impact of tracheobronchomalacia on outcomes in neonates with bronchopulmonary dysplasia in a large, multi-center cohort.

Up-To-Date Practical Imaging Evaluation of Neonatal Soft-Tissue Tumors: What Radiologists Need to Know.

Objective: The purposes of this article are to provide an up-to-date overview of neonatal soft-tissue tumors, including information regarding their unique nature, and to present practical imaging techniques and characteristic imaging findings.

Conclusion: Neonatal soft-tissue tumors are a unique set of neoplasms that often have characteristic clinical and imaging findings. Imaging evaluation, mainly with ultrasound and MRI, plays an important role in the initial diagnosis, staging, preoperative assessment, and follow-up evaluation.

Lysosomal Biology in Cancer.

Cells depend on the lysosome for sequestration and degradation of macromolecules in order to maintain metabolic homeostasis. These membrane-enclosed organelles can receive intracellular and extracellular cargo through endocytosis, phagocytosis, and autophagy. Lysosomes establish acidic environments to activate enzymes that are able to break down biomolecules engulfed through these various pathways.

Epileptic Encephalopathies-Clinical Syndromes and Pathophysiological Concepts.

Epileptic encephalopathies account for a large proportion of the intractable early-onset epilepsies and are characterized by frequent seizures and poor developmental outcome. The epileptic encephalopathies can be loosely divided into two related groups of named syndromes. The first comprises epilepsies where continuous EEG changes directly result in cognitive and developmental dysfunction.

Medical Mimics of Child Abuse.

Objective: Physicians have an ethical and legal mandate to identify abused children so that they may be protected from further harm and are simultaneously required to think broadly and objectively about differential diagnoses. The medical literature is replete with examples of medical diseases that mimic abuse, potentially leading to misdiagnoses and subsequent harm to children and families.

Conclusion: This review highlights some of the common and uncommon diseases that mimic physical and sexual abuse of children.

Surgical management of aortic coarctation in adolescents and adults.

Objectives: Coarctation of the aorta (CoA) in adolescents and adults is often complicated by other cardiac or aortic problems and may carry additional surgical difficulties. Limited studies have reported the surgical outcomes of CoA repair in this particular patient population. We reviewed our contemporary experience of open surgical management of CoA in adolescents and adults.

Imaging the Injured Pediatric Athlete: Upper Extremity.

Increased physical activity in childhood has resulted in a large number of sports-related injuries. Although there is overlap between the sports-related injuries seen in pediatric and adult patients, important differences exist in the injury patterns of pediatric patients. These differences are related to the continuous changes in the developing skeleton and its relationship with adjacent soft tissues.

SPATA5 mutations cause a distinct autosomal recessive phenotype of intellectual disability, hypotonia and hearing loss.

We examined an extended, consanguineous family with seven individuals with severe intellectual disability and microcephaly. Further symptoms were hearing loss, vision impairment, gastrointestinal disturbances, and slow and asymmetric waves in the EEG. Linkage analysis followed by exome sequencing revealed a homozygous variant in SPATA5 (c.

Pulmonary alveolar proteinosis and Niemann Pick disease type B: An unexpected combination.

Background: Pulmonary involvement in Niemann-Pick disease (NPD) is a common finding, especially in type B. It usually manifests with symptoms of restrictive lung disease appearing in adulthood but showing gradual deterioration over time. Treatment options have been dramatically limited, with whole lung lavage offering only temporary improvement.

Methods for Studying Autophagy Within the Tumor Microenvironment.

Defective autophagy has been linked with many pathologies, including cancer, diabetes, infectious disease, myopathies, heart, liver, lung, and neurodegenerative disease. Autophagy has therefore become an important target in drug discovery. Recent advances have identified new ways to monitor autophagy in vitro and in vivo.

Ultrasound Tutorials in Under 10 Minutes: Experience and Results.

Objective: The objective of the present study was to validate the use of web-based tutorials to teach technical skills in pediatric ultrasound.

Materials And Methods: A series of 10 video tutorials, each of which was less than 10 minutes long, was created to improve the education of radiology trainees in terms of their ultrasound technique. Pediatric radiology fellow trainees from our institution in the United States and radiology resident trainees from our partner institution in Ethiopia were invited to participate in the study.

The complement system, neuronal injury, and cognitive function in horizontally-acquired HIV-infected youth.

The complement system (C1q/C3) is a key mediator of synaptic pruning during normal development. HIV inappropriately induces C1q and C3 production in the brain, and reduces neuronal complement inhibition. HIV may thus alter neural connectivity in the developing brain by excessively targeting synapses for elimination.

Persistent Left Superior Vena Cava Connected to the Coronary Sinus in the Fetus: Effects on Cardiac Structure and Flow Dynamics.

Ventricular size discrepancy may be due to a persistent left superior vena cava (PLSVC) in utero. We sought to investigate for differences in cardiac structure measures and hemodynamics between fetuses with isolated PLSVC connected to the coronary sinus (CS) and normal. Fetuses diagnosed with isolated PLSVC in the second and third trimester were enrolled.