516 results match your criteria: "Shriners Hospital for Children Portland & Oregon Health Science University[Affiliation]"

New insights into the structural role of EMILINs within the human skin microenvironment.

Sci Rep

December 2024

Department of Pediatrics and Adolescent Medicine, Faculty of Medicine and University Hospital Cologne, University of Cologne, Joseph-Stelzmann-Str. 52, 50931, Cologne, Germany.

Article Synopsis
  • Supramolecular ECM networks, crucial for skin structure and function, include EMILIN proteins that interact with elastin and influence cellular signaling.
  • Our study mapped the distribution of EMILIN-1, -2, and -3 in human skin, revealing their role in elastic fibers and their connections with basal keratinocytes.
  • We found that in conditions like Marfan syndrome and scleroderma, the presence and localization of EMILINs change, indicating they can be used as markers for monitoring dermal ECM rearrangements due to aging and disease.
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Article Synopsis
  • The paper discusses the importance of structured guidelines for returning to activity after spinal deformity surgery, emphasizing that a consistent approach can improve patient recovery and health outcomes.
  • It categorizes diverse patient groups, including early onset scoliosis, adolescent idiopathic scoliosis, young adults, adult spinal deformity, elite athletes, and general sports participants, offering tailored recommendations for each.
  • The document stems from an educational webinar and aims to standardize postoperative protocols, enhancing both surgeon practice and overall patient care.
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Transiting to dancing en pointe is an important milestone for young dancers who wish to progress in ballet training. Various criteria exist regarding pointe readiness, including age, range-of-motion, endurance, strength, balance, and technique. However, awareness and use of these criteria by dance instructors is currently unknown.

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Purpose: Arthrogryposis multiplex congenita (AMC) describes a heterogeneous group of rare congenital conditions. Health-related quality of life (HRQL) may be reduced in AMC due to broadly heterogeneous physical impairments and participation limitations. This study described HRQL in children and youth with AMC, compared HRQL between child self- and parent-proxy reports, and identified factors associated with better/worse HRQL.

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Purpose: This study aims to measure the impact of the Scoliosis Research Society's travel fellowship on a spinal surgeon's career.

Methods: A non-incentivized survey was sent to 78 previous SRS junior travel fellows from 1993 to 2021. The questionnaire assessed fellowship influence on academic and administrative positions, professional society memberships, and commercial relationships.

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Article Synopsis
  • Charcot-Marie-Tooth disease (CMT) can lead to a foot deformity in children called cavovarus, which is traditionally thought to occur due to the first metatarsal being positioned lower than the fifth, creating what is known as the tripod effect.* -
  • The study reviewed dynamic pedobarographic data from children with CMT to see if the first metatarsal always contacted the ground before the fifth metatarsal during walking, as previously suggested.* -
  • Results showed that in a significant number of cases, the first metatarsal did not consistently contact the ground before the fifth, suggesting that many feet were already positioned in a varus state before contact, indicating a need for
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Setrusumab for the treatment of osteogenesis imperfecta: 12-month results from the phase 2b asteroid study.

J Bone Miner Res

September 2024

Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences (NDORMS), University of Oxford, Wellington Square, Oxford OX1 2JD, United Kingdom.

Article Synopsis
  • * The phase 2b Asteroid study tested the efficacy and safety of the drug setrusumab in adults with OI, providing different doses of the drug or a placebo over 12 months.
  • * Results showed significant improvements in bone strength measures like failure load and stiffness for certain doses, and although there were some serious adverse reactions, the drug shows promise for further research in phase 3 trials.
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Osteogenesis imperfecta (OI)is a rare genetically heterogeneous disorder caused by changes in the expression or processing of type I collagen. Clinical manifestations include bone fragility, decreased linear growth, and skeletal deformities that vary in severity. In typically growing children, skeletal maturation proceeds in a predictable pattern of changes in the size, shape, and mineralization on the hand and wrist bones that can be followed radiographically known at the bone age.

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Study Design: Retrospective, Multicenter.

Objective: Assess curve progression and occurrence of revision surgery following tether breakage after vertebral body tethering (VBT).

Summary Of Background Data: Tether breakage after VBT is common with rates up to 50% reported.

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Recessive dystrophic epidermolysis bullosa is a rare genodermatosis caused by a mutation of the Col7a1 gene. The Col7a1 gene codes for collagen type VII protein, a major component of anchoring fibrils. Mutations of the Col7a1 gene can cause aberrant collagen type VII formation, causing an associated lack or absence of anchoring fibrils.

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Scoliosis is a common complication of neuromuscular disorders. These patients are frequently recalcitrant to nonoperative treatment. When treated surgically, they have the highest risk of complications of all forms of scoliosis.

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Analysis of three-dimensional spine growth for vertebral body tethering patients at 2 and 5 years post operatively.

Spine Deform

July 2024

Department of Orthopaedic Surgery, Orthopaedic Clinic, IWK Health Centre, 5850 University Avenue, PO Box 9700, Halifax, NS, B3K 6R8, Canada.

Purpose: Scoliosis can be treated with vertebral body tethering (VBT) as a motion-sparing procedure. However, the knowledge of how growth is affected by a tether spanning multiple levels is unclear in the literature. Three-dimensional true spine length (3D-TSL) is a validated assessment technique that accounts for the shape of the spine in both the coronal and sagittal planes.

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Purpose: Post-operative coronal decompensation (CD) continues to be a challenge in the treatment of adolescent idiopathic scoliosis (AIS). CD following selective spinal fusion has been studied. However, there is currently little information regarding CD following Vertebral Body Tethering (VBT).

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Background: Severe spastic wrist contractures secondary to cerebral palsy (CP) or alike can have significant implications for patient hand function, hygiene, skin breakdown, and cosmesis. When these contractures become rigid, soft tissue procedures alone are unable to obtain or maintain the desired correction. In these patients' wrist arthrodesis is an option-enabling the hand to be stabilized in a more functional position for hygiene, dressing, and general cosmesis, though are patients satisfied?

Methods: All children who had undergone a wrist arthrodesis for the management of a severe wrist contracture at Shriners Hospital, Portland between January 2016 and January 2021 were identified (n=23).

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Post-lumbar puncture headache occurs in 5% to 12% of children. The purpose of this study was to determine the frequency and predictors of post-lumbar puncture headache in children with hypertonia undergoing lumbar puncture for intrathecal baclofen trial. This was a retrospective single-center review of all 43 children (<18 years) with hypertonia and/or dyskinesia undergoing intrathecal baclofen trial from 2013-2022.

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There are some syndromes that present with unique manifestations pertaining to the spinal column. A good working understanding of these common syndromes is useful for the spinal deformity surgeons and related healthcare providers. This review attempts to encompass these unique features and discuss them in three broad groups: hypermobility syndromes, muscle pathology-related syndromes, and syndromes related to poor bone quality.

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Promoting Firearm Injury Prevention and Advocacy as Pediatric Surgeons: A Call to Action From the APSA/AAP Advocacy Committee.

J Pediatr Surg

June 2024

Division of Pediatric Surgery, University of Texas Medical Branch, 301 University Blvd, Research Building 6, Suite 3.220, Galveston, TX 77555, United States.

Firearm injuries have become the leading cause of death among American children. Here we review the scope of the problem, and the pivotal role pediatric surgeons have in preventing pediatric firearm injury. Specific methods for screening and counseling are reviewed, as well as how to overcome barriers.

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Study Design: Prospective multicenter study data were used for model derivation and externally validated using retrospective cohort data.

Objective: Derive and validate a prognostic model of benefit from bracing for adolescent idiopathic scoliosis (AIS).

Summary Of Background Data: The Bracing in Adolescent Idiopathic Scoliosis Trial (BrAIST) demonstrated the superiority of bracing over observation to prevent curve progression to the surgical threshold; 42% of untreated subjects had a good outcome, and 28% progressed to the surgical threshold despite bracing, likely due to poor adherence.

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Collagen IV of basement membranes: II. Emergence of collagen IV enabled the assembly of a compact GBM as an ultrafilter in mammalian kidneys.

J Biol Chem

December 2023

Division of Nephrology and Hypertension, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA; Aspirnaut, Vanderbilt University Medical Center, Nashville, Tennessee, USA; Center for Matrix Biology, Vanderbilt University Medical Center, Nashville, Tennessee, USA; Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA; Department of Biochemistry, Vanderbilt University, Nashville, Tennessee, USA; Vanderbilt-Ingram Cancer Center, Vanderbilt University, Nashville, Tennessee, USA; Vanderbilt Institute of Chemical Biology, Vanderbilt University, Nashville, Tennessee, USA; Department of Biological Sciences, Vanderbilt University, Nashville, Tennessee, USA; Department of Cell and Developmental Biology, Vanderbilt University, Nashville, Tennessee, USA.

The collagen IV (Col-IV) scaffold, the major constituent of the glomerular basement membrane (GBM), is a critical component of the kidney glomerular filtration barrier. In Alport syndrome, affecting millions of people worldwide, over two thousand genetic variants occur in the COL4A3, COL4A4, and COL4A5 genes that encode the Col-IV scaffold. Variants cause loss of scaffold, a suprastructure that tethers macromolecules, from the GBM or assembly of a defective scaffold, causing hematuria in nearly all cases, proteinuria, and often progressive kidney failure.

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Unraveling the role of TGFβ signaling in thoracic aortic aneurysm and dissection using Fbn1 mutant mouse models.

Matrix Biol

November 2023

Department of Molecular & Medical Genetics, Oregon Health & Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, United States. Electronic address:

Although abnormal TGFβ signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, its precise role in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to elucidate the role of TGFβ signaling in three Fbn1 mutant mouse models representing a range of aortic disease from microdissection (without aneurysm) to aneurysm (without rupture) to aneurysm and rupture. Results indicated that reduced TGFβ signaling and increased mast cell proteases were associated with microdissection.

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Article Synopsis
  • Collagen X biomarker (CXM) has been studied as an indicator of growth velocity in children with impaired kidney function, focusing on its correlation with actual growth measurements.
  • The study utilized data from children aged 2 to 16 enrolled in the Chronic Kidney Disease in Children (CKiD) study, employing various statistical models to assess the relationship between CXM levels and growth velocity.
  • Results showed a stronger correlation of CXM with growth in females than in males, with specific increases in CXM levels correlating to higher growth rates, suggesting its potential role in monitoring growth in pediatric chronic kidney disease while indicating the need for further research.
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Article Synopsis
  • Diastrophic dysplasia (DTD) is caused by mutations in the SLC26A2 gene, affecting sulfate uptake and glycosaminoglycan (GAG) sulfation, and it may benefit from pharmacological treatments as studied in an animal model.
  • In identifying non-invasive biomarkers for DTD, research focused on urinary GAG sulfation and blood levels of collagen X marker (CXM), which are linked to the severity of the condition and growth metrics.
  • Results indicated that DTD patients exhibit undersulfated urinary GAGs and lower than normal CXM levels, suggesting both biomarkers can help evaluate treatment effectiveness and the disorder's metabolism and ossification processes.
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Background: Nonsurgical interventions such as bracing with ankle foot orthoses (AFOs) aim to assist, restore, and redirect weightbearing forces to address difficulty with mobilization. We identified a custom carbon fiber passive dynamic ankle foot orthosis (PDAFO) that was designed to meet the needs of military combat veterans. We sought to evaluate the off-loading properties of one model of PDAFO (ExoSym) in a civilian population.

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Study Design: Retrospective review of a prospective database.

Objectives: The purpose of this study is to evaluate changes in the thoracic and thoracolumbar/lumbar curves and truncal balance in patients treated with selective thoracic anterior vertebral body tethering (AVBT) with Lenke 1A vs 1C curves at a minimum of 2 years follow-up. Lenke 1C curves treated with selective thoracic AVBT demonstrate equivalent thoracic curve correction and reduced thoracolumbar/lumbar curve correction compared to Lenke 1A curves.

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Orthopaedic Diagnoses in the Black Pediatric Population.

J Am Acad Orthop Surg

March 2023

From the Pediatric Orthopaedic Fellow, Phoenix Children's Hospital, Phoenix, AZ (Bridges), the Department of Orthopaedic Surgery, Baylor Scott & White Health, Temple, TX, (Agarwal), the College of Medicine, Texas A&M University, Temple, TX, (Agarwal), and the Shriners Hospital for Children Portland (Raney), Affiliate Professor Orthopaedics and Rehabilitation, Oregon Health Sciences University, Portland, OR.

Article Synopsis
  • The Black pediatric population faces historical underservice and ongoing unmet healthcare needs.
  • Lack of diversity in medical studies can lead to inadequate care for conditions like slipped capital femoral epiphysis and Blount disease.
  • Increased awareness of systemic issues and racial disparities in access to care can help practitioners offer more equitable treatment.
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