Ultrasonography in Pediatric Rheumatology Vega-Fernandez, Patricia; Ting, Tracy V; Pratt, Laura ...
Rheumatic diseases clinics of North America,
02/2022, Letnik:
48, Številka:
1
Journal Article
Recenzirano
This review highlights the increasing evidence from the last few years supporting the use of musculoskeletal ultrasonography (MSUS) in the evaluation and management of patients with pediatric ...rheumatic diseases, particularly focusing on juvenile idiopathic arthritis. Recently developed definitions for the sonographic appearance of healthy and pathologic joints in children are discussed. Further topics explored include how MSUS enhances the diagnosis of inflammatory joint disease (synovitis, enthesitis, tenosynovitis), including the detection of subclinical synovitis. There is a brief summary on the use of ultrasonography in the evaluations of myositis, Sjögren syndrome, and scleroderma.
Background Binding of adenosine to the anti-inflammatory Gs-coupled adenosine 2A receptor (A2A R) inhibits the activity of most inflammatory cells. Extensive preclinical evidence exists for the use ...of A2A R agonists in the prevention of acute ischemia-reperfusion injury. Activation of A2A Rs on lymphocytes and antigen-presenting cells also attenuates the alloimmune response. Because ischemia-reperfusion injury and alloimmunity are risk factors for the development of bronchiolitis obliterans (BO), the objective of this study was to determine the effect of A2A R signaling on tracheal rejection in a mouse model of BO. Methods A non-revascularized heterotopic tracheal model across a total alloantigenic mismatch was used to study A2A R signaling in a mouse model of BO. Tracheal transplants were performed using Balb/c donors into wild-type or A2A R knockout C57BL/6 recipient mice. Another group of Balb/c transplants into C57BL/6 recipients were also treated with a selective A2A R agonist. Tracheas were assessed at 3, 7, 12, 21, and 28 days after transplantation by hematoxylin and eosin staining, immunohistochemical staining, and collagen staining. Results Compared with allograft tracheas in wild-type recipients, allografts in A2A R knockout recipients had increased inflammation and more severe BO development. Recipient wild-type mice treated with a selective A2A R agonist were significantly protected from lymphocyte infiltration and luminal occlusion, but fibro-obliteration still developed by 28 days after transplantation. Conclusions Endogenous adenosine signals through the A2A R to attenuate inflammatory and immune factors involved in BO development. Synthetic A2A R agonists may provide a novel treatment strategy to prevent BO.
This summary from the American Heart Association provides guidance for the provision of primary and subspecialty palliative care in pediatric congenital and acquired heart disease.
A comprehensive ...literature search was conducted from January 2010 to December 2021. Seminal articles published before January 2010 were also included in the review. Human subject studies and systematic reviews published in English in PubMed, ClinicalTrials.gov, and the Cochrane Collaboration were included. Structure: Although survival for pediatric congenital and acquired heart disease has tremendously improved in recent decades, morbidity and mortality risks remain for a subset of young people with heart disease, necessitating a role for palliative care. This scientific statement provides an evidence-based approach to the provision of primary and specialty palliative care for children with heart disease. Primary and specialty palliative care specific to pediatric heart disease is defined, and triggers for palliative care are outlined. Palliative care training in pediatric cardiology; diversity, equity, and inclusion considerations; and future research directions are discussed.
Abstract A full-term male prenatally diagnosed with left congenital diaphragmatic hernia (CDH) was intubated at birth and scheduled for elective repair. The pre-operative chest radiograph ...incidentally demonstrated tracheal narrowing and an intraoperative flexible bronchoscopy was performed, suggestive of impending airway occlusion secondary to complete tracheal rings. Given these findings, he was electively cannulated post-operatively to venoarterial extracorporeal membrane oxygenation (ECMO) for stabilization and tracheal repair. Post-operative day one, he underwent further characterization by computed tomography and was taken for slide tracheoplasty on ECMO support. Immediately following tracheal repair, he was decannulated from ECMO and was extubated to nasal CPAP two days after the slide tracheoplasty. This case highlights the rare association of tracheal anomalies associated with CDH and options for perioperative management, including the role of ECMO.
Obese youth without diabetes with monophasic oral glucose tolerance test (OGTT) glucose response curves have lower insulin sensitivity and impaired β-cell function compared with those with biphasic ...curves. The OGTT glucose response curve has not been studied in youth-onset type 2 diabetes. Here we test the hypothesis that the OGTT glucose response curve at randomization in youth in the TODAY (Treatment Options for Type 2 Diabetes in Adolescents and Youth) study forecasts heightened glycemic failure rates and accelerated decline in β-cell function.
OGTTs (
= 662) performed at randomization were categorized as monophasic, biphasic, or incessant increase. Demographics, insulin sensitivity (1/fasting insulin), C-peptide index (△C
/△G
), and β-cell function relative to insulin sensitivity (oral disposition index oDI) were compared among the three groups.
At randomization, 21.7% had incessant increase, 68.6% monophasic, and 9.7% biphasic glucose response curves. The incessant increase group had similar insulin sensitivity but significantly lower C-peptide index and lower oDI, despite similar diabetes duration, compared with the other two groups. Glycemic failure rates were higher in the incessant increase group (58.3%) versus the monophasic group (42.3%) versus the biphasic group (39.1%) (
< 0.0001). The 6-month decline in C-peptide index (32.8% vs. 18.1% vs. 13.2%) and oDI (32.2% vs. 11.6% vs. 9.1%) was greatest in incessant increase versus monophasic and biphasic with no difference in insulin sensitivity.
In the TODAY study cohort, an incessant increase in the OGTT glucose response curve at randomization reflects reduced β-cell function and foretells increased glycemic failure rates with accelerated deterioration in β-cell function independent of diabetes duration and treatment assignment compared with monophasic and biphasic curves. The shape of the OGTT glucose response curve could be a metabolic biomarker prognosticating the response to therapy in youth with type 2 diabetes.
Mutations in the RASA-1 gene underlie several related disorders of vasculogenesis. Capillary malformation-arteriovenous malformation (CM-AVM) is one such entity and was recently encountered in a ...neonate who demonstrated its clinical and radiologic features. A single mutation in the RASA-1 gene was detected.A novel flow reduction strategy was employed to a large AVM affecting the patient's upper limb. The imaging findings, surgical procedure and patient's improved post-operative state are described.
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