Shen, Yu Peng, Yun Huang, Pengcheng Zheng, Yilei Li, Shumeng Jiang, Kaiyan Zhou, Meihong Deng, Jianwen Zhu, Min Hong, Daojun
...
Published in
Frontiers in Genetics
Background: Primary serine deficiency disorders have a broad range of the phenotypic spectrum. As an inborn error of metabolism, individuals with severe phenotype may be easily recognized with Neu-Laxova syndrome. However, late-onset mild phenotypes may be underdiagnosed and will lead to disastrous consequences due to treatment delays. Materials an...
Gamal, Sherif M Fouad, Nermeen Yosry, Nora Badr, Wael Sobhy, Nesreen
Published in
Archives of rheumatology
This study aims to compare disease characteristics in patients with juvenile-onset systemic lupus erythematosus (JSLE) and adult-onset systemic lupus erythematosus (ASLE). Between June 2010 and March 2020, a total of 186 patients with JSLE (23 males, 163 females; median age: 25 years; range, 20 to 30.3 years) and 236 patients with ASLE (23 males, 2...
ZHANG, Liang MAN, Siliang LI, Hongchao BIAN, Tao GUO, Shaoyi ZHOU, Yixin
Published in
Chinese Journal of Reparative and Reconstructive Surgery
目的 评估幼年发病型强直性脊柱炎（juvenile-onset ankylosing spondylitis，JAS）接受人工全髋关节置换术（total hip arthroplasty，THA）的中期临床随访结果。 方法 回顾性分析2004年1月—2018年3月的81例（127髋）JAS患者（年龄≤16岁，JAS组）和267例（391髋）成年发病型强直性脊柱炎（adult-onset ankylosing spondylitis，AAS）患者（年龄>16岁，AAS组）行THA手术重建的临床随访结果。常规收集患者术前基线人口学数据、临床参数、影像学参数和实验室数据；术前及末次随访时评价指标包括疾病整体活动度 ［巴氏强直性脊柱炎疾病活动指数（Bath ankylosing spondyliti...
Wright, James R Jr McIntyre, Lynn
Published in
Journal of medical biography
Historians of diabetes have long claimed that physicians were aware of two distinct types of diabetes mellitus by the 1880s, and that these were the direct forerunners of type 1, juvenile-onset and type 2, adult-onset diabetes. French physician Étienne Lancereaux (1829-1910), based on autopsy and clinical studies, classified diabetes either as diab...
Chanakul, Ankanee Khunrattanaphon, Suriyaphon Deekajorndech, Tawatchai
Published in
Asian Biomedicine
Background Improvement of disease recognition and management has increased the survival of children with systemic lupus erythematosus (SLE), but has shifted the morbidity focus toward long-term complications, such as low bone mass and osteoporosis. Studies in adults with SLE show older age, chronic inflammation, and corticosteroid therapy are risk ...
Jin, Wenyuan Yang, Xinghui Lu, Meiping
Published in
Medicine
Osteonecrosis (ON) is a devastating illness that leads to bone ischemia and potential joint destruction. Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease, with multi-system involvement which is closely associated with occurrence of ON. Multifocal ON, with an estimated morbidity of 3% in SLE patients, is extremely rare in juvenile...
Kripps, Kimberly Kierstein, Janell Nicklas, Daniel Nelson, Julie Yang, Michele Collins, Abigail Troy, Elizabeth Escolar, Maria Maloney, John Neuberger, Ilana
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Published in
Journal of child neurology
Krabbe disease is a progressive neurologic disorder caused by deficiency of the lysosomal enzyme galactocerebrosidase. The disease commonly has an early-infantile onset, but can have late-infantile, juvenile, or adult-onset phenotypes. Classic computed tomography (CT) and magnetic resonance imaging (MRI) findings in Krabbe have been well described....
Obrynba, Kathryn S Indyk, Justin A Gandhi, Kajal K Buckingham, Don Kamboj, Manmohan K
Published in
Pediatric diabetes
The American Diabetes Association (ADA) and the International Society for Pediatric and Adolescent Diabetes (ISPAD) have outlined standards for best practices in providing optimal diabetes care to children with type 1 diabetes (T1D). Our objectives were to design a metric that evaluated delivery of optimal diabetes care and to use this metric to dr...
Lee, Victor Thurston, Travis Thurston, Chris
Published in
Methods of information in medicine
Type 1 diabetes requires frequent testing and monitoring of blood glucose levels in order to determine appropriate type and dosage of insulin administration. This can lead to thousands of individual measurements over the course of a lifetime of a single individual, of which very few are retained as part of a permanent record. The third author, aged...
