Publications by authors named "David Biko"

Background: The Paediatric Rheumatology International Trials Organisation (PRINTO) recently undertook an effort to better harmonize the pediatric and adult arthritis criteria. These provisional criteria are being refined for optimal performance. We aimed to investigate differences between patients who did and did not fulfill these PRINTO criteria amongst youth diagnosed with juvenile spondyloarthritis (SpA) that met axial juvenile SpA (axJSpA) classification criteria.

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Dynamic contrast-enhanced magnetic resonance lymphangiography is a high-resolution imaging technique that has emerged as the preferred method for evaluating lymphatic anatomy and flow dynamics due to its precise anatomical detail. The lymphatic system has a complex anatomical distribution, and variability is common among individuals with cardiac abnormalities, particularly congenital heart disease. Lymphatic imaging has recently been revolutionized by the introduction of MR lymphangiography.

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Fetal brain size is decreased in some children with complex CHDs, and the distribution of blood and accompanying oxygen and nutrients is regionally skewed from early fetal life dependent on the CHD. In transposition of the great arteries, deoxygenated blood preferentially runs to the brain, whereas the more oxygenated blood is directed towards the lungs and the abdomen. Knowledge of whether this impacts intrauterine organ development is limited.

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Childhood interstitial lung disease (chILD) encompasses a diverse group of genetic, infectious, and inflammatory conditions affecting infants and children. The recognition and understanding of these entities have highlighted the necessity for more accurate classification. This group of rare heterogeneous diseases comprises more than 200 different conditions and has a combined estimated prevalence of less than one patient per 100 000 children.

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Article Synopsis
  • The study focuses on using dynamic magnetic resonance imaging (dMRI) to analyze diaphragm motion in patients with thoracic insufficiency syndrome (TIS), providing insights into the severity of respiratory disorders without exposing patients to radiation.
  • The paper outlines a three-step approach to segment the left and right hemi-diaphragm from dMRI images, overcoming challenges like low resolution and motion blur by employing advanced deep learning techniques for accurate recognition and delineation.
  • Results showed a mean-Hausdorff distance of approximately 3 mm for diaphragm delineation and a positional error of about 3 mm in identifying the mid-sagittal plane, validated using 100 test images of TIS patients.
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Friedreich's ataxia (FRDA) is a progressive disorder caused by insufficient expression of frataxin, which plays a critical role in assembly of iron-sulfur centers in mitochondria. Individuals are cognitively normal but display a loss of motor coordination and cardiac abnormalities. Many ultimately develop heart failure.

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Objective: The goal was to develop and validate classification criteria for axial juvenile spondyloarthritis (SpA; AxJSpA).

Methods: This international initiative consisted of four phases: (1) item generation, (2) item reduction, (3) criteria development, and (4) validation of the AxJSpA criteria by an independent team of experts in an internationally representative validation cohort.

Results: These criteria are intended to be used on youth with a physician diagnosis of juvenile SpA and for whom axial disease is suspected.

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Medicare enrollment is complex, particularly for low-income individuals who are dually eligible for Medicare and Medicaid, and the wrong plan choice can adversely impact beneficiaries' out-of-pocket costs and access to providers and medications. The State Health Insurance Assistance Program (SHIP) is a federal program that provides counseling on Medicare coverage, but the degree to which SHIP services are accessible to low-income beneficiaries is unknown. We interviewed SHIP counselors and coordinators to characterize factors affecting access to and quality of SHIP services for low-income beneficiaries.

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Article Synopsis
  • Lymphatic imaging helps doctors see how the lymphatic system works, which is important for new treatments.
  • Problems in the lymphatic system can happen in kids, and good imaging is needed to find and treat these issues correctly.
  • New, non-invasive methods for imaging are being developed to improve how doctors understand and use lymphatic imaging in children's surgery.
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Purpose To evaluate lymphatic abnormalities before and after Fontan completion using noncontrast lymphatic imaging and relate findings with postoperative outcomes. Materials and Methods This study is a retrospective review of noncontrast T2-weighted lymphatic imaging performed at The Children's Hospital of Philadelphia from June 2012 to February 2023 in patients with single ventricle physiology. All individuals with imaging at both pre-Fontan and Fontan stages were eligible.

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Article Synopsis
  • Thoracic insufficiency syndrome (TIS) restricts respiratory function due to spinal and thoracic deformities, making corrective orthopedic surgery a potential solution to improve lung space and diaphragm movement.
  • A study involving 149 TIS pediatric patients and 190 healthy controls used free-breathing dynamic MRI to analyze diaphragm motion and changes before and after surgery.
  • Results showed significant increases in diaphragm mobility, particularly in the posterior regions, with the surgery having a more pronounced effect on diaphragm function than spinal curve reductions.
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Purpose: There is a concern in pediatric surgery practice that rib-based fixation may limit chest wall motion in early onset scoliosis (EOS). The purpose of this study is to address the above concern by assessing the contribution of chest wall excursion to respiration before and after surgery.

Methods: Quantitative dynamic magnetic resonance imaging (QdMRI) is performed on EOS patients (before and after surgery) and normal children in this retrospective study.

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Magnetic resonance imaging (MRI) is now an indispensable diagnostic tool in medicine due to its outstanding contrast resolution and absence of radiation exposure, enabling detailed tissue characterization and three-dimensional anatomical representation. This is especially important when evaluating individuals with congenital heart disease (CHD) who frequently require cardiac implantable electrical devices (CIEDs). While MRI safety issues have previously limited its use in patients with CIEDs, new advances have called these limitations into question.

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Objectives: Counseling and education on Medicare coverage options are available through the federal State Health Insurance Assistance Program (SHIP), but little is known about the population that SHIP reaches.

Study Design: Cross-sectional study.

Methods: Using a novel data source on SHIP counseling site locations, we characterized the availability of in-person SHIP counseling by zip code tabulation area (ZCTA) and used linear regression and t tests to evaluate whether SHIP counseling sites are disproportionately located in higher-income communities.

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Introduction: Tracheomalacia (TM) is an important cause of respiratory morbidity. Dynamic flexible bronchoscopy is considered the gold standard for diagnosis. Dynamic airway computed tomography (DACT) is a low radiation, noninvasive diagnostic tool utilizing images obtained continuously over several respiratory cycles.

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Patients with a single ventricle develop aortopulmonary collaterals (APCs) whose flow has been shown to be inversely proportional to cerebral blood flow (CBF) in a previous cross-sectional study. Longitudinal CBF and APC flow in patients with Fontan physiology adjusting for brain injury (BI) has never been reported. Decreased CBF and BI may adversely impact neurodevelopment.

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Primary cardiac and pericardial neoplasms are rare in the pediatric population and can include both benign and malignant lesions. Rhabdomyomas, teratomas, fibromas, and hemangiomas are the most common benign tumors. The most common primary cardiac malignancies are soft-tissue sarcomas, including undifferentiated sarcomas, rhabdomyosarcomas, and fibrosarcomas.

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Background: Patients with unbalanced common atrioventricular canal can be difficult to manage. Surgical planning often depends on pre-operative echocardiographic measurements. We aimed to determine the added utility of cardiac MRI in predicting successful biventricular repair in common atrioventricular canal.

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Dual-energy CT has expanded the potential of thoracic imaging in both children and adults. Data processing allows material- and energy-specific reconstructions, which improve material differentiation and tissue characterization compared with single-energy CT. Material-specific reconstructions include iodine, virtual unenhanced, perfusion blood volume, and lung vessel images, which can improve assessment of vascular, mediastinal, and parenchymal abnormalities.

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Article Synopsis
  • - Central conducting lymphatic anomaly (CCLA) is a serious condition caused by improper development of the lymphatic system, and this study focused on individuals with CCLA linked to KRAS gene mutations.
  • - Researchers used human cells and zebrafish to explore how these KRAS variants lead to lymphatic issues, finding that the mutations activate a specific signaling pathway (RAS/MAPK) that contributes to lymphatic malformation.
  • - Treatment with MEK inhibitors showed promise in reducing symptoms in lab models, suggesting this approach should be further investigated for treating CCLA in patients with KRAS mutations.
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Objective: Radiography is still used worldwide for the detection of sacroiliitis in juvenile spondyloarthritis (JSpA), despite its low sensitivity and reliability. We aimed to define unequivocal evidence of sacroiliitis on pelvic radiography in skeletally immature youth for use in classification criteria when magnetic resonance imaging (MRI) is unavailable.

Methods: Subjects were a retrospective cohort of juvenile patients with spondyloarthritis with a radiograph and MRI as part of a diagnostic evaluation for axial disease.

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There have been reports of myocarditis following vaccination against COVID-19. We sought to describe cardiac magnetic resonance (CMR) findings among pediatric patients. Retrospective review at a large academic center of patients clinically diagnosed with post-vaccine myocarditis (PVM) undergoing CMR.

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Purpose: The contribution of thoracic duct obstruction to lymphatic flow disorders has not been well-characterized. We describe imaging findings, interventions, and outcomes in patients with suspected duct obstruction by imaging or a lympho-venous pressure gradient (LVPG).

Materials And Methods: Clinical, imaging, and interventional data, including the LVPG, of patients with flow disorders and imaging features of duct obstruction who underwent lymphatic intervention were retrospectively reviewed, collated, and analyzed with descriptive statistics.

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