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地中海贫血患者颅骨中骨质疏松症和眶筛骨病变的大体及影像学表现。

Gross and radiographic appearance of porotic hyperostosis and cribra orbitalia in thalassemia affected skulls.

作者信息

Chaichun Amnart, Yurasakpong Laphatrada, Suwannakhan Athikhun, Iamsaard Sitthichai, Arun Supatcharee, Chaiyamoon Arada

机构信息

Department of Anatomy, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.

Department of Anatomy, Faculty of Science, Mahidol University, Bangkok, Thailand.

出版信息

Anat Cell Biol. 2021 Jun 30;54(2):280-284. doi: 10.5115/acb.20.323.

DOI:10.5115/acb.20.323
PMID:33731491
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8225484/
Abstract

Porotic hyperostosis (PH) is the expansion of the cranial diploë, generating 'hair-on-end' appearance on X-rays. This condition is extremely rare and had a prevalence of 0.08%. It is alternatively known as cribra orbitalia (CO) when the orbital roof is affected. In this study, we describe the gross morphology of two skulls affected by PH and CO. The first skull belonged to a 41-year-old female previously diagnosed with beta thalassemia. The skull was affected by excessive PH and CO. The second skull, belonging to a 35-year-old male diagnosed with unspecified thalassemia, showed PH without CO. The etiology of PH and CO is discussed. In conclusion, PH and CO are extremely rare, and are of significant importance for radiologists and anatomists when interpreting radiographs or encountering unusual gross morphology of the skull.

摘要

多孔性骨质增生(PH)是颅骨板障的扩张,在X射线上产生“头发直立”的外观。这种情况极为罕见,患病率为0.08%。当眶顶受影响时,它也被称为筛孔状眶骨炎(CO)。在本研究中,我们描述了两个受PH和CO影响的颅骨的大体形态。第一个颅骨属于一名先前被诊断为β地中海贫血的41岁女性。该颅骨受到过度的PH和CO影响。第二个颅骨属于一名被诊断为未明确类型地中海贫血的35岁男性,显示有PH但无CO。文中讨论了PH和CO的病因。总之,PH和CO极为罕见,对于放射科医生和解剖学家在解读X光片或遇到颅骨异常大体形态时具有重要意义。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f80/8225484/04ce8af825e1/acb-54-2-280-f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f80/8225484/2cf7eaee1a7a/acb-54-2-280-f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f80/8225484/04ce8af825e1/acb-54-2-280-f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f80/8225484/2cf7eaee1a7a/acb-54-2-280-f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f80/8225484/04ce8af825e1/acb-54-2-280-f2.jpg

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本文引用的文献

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Am J Phys Anthropol. 2017 Sep;164(1):76-96. doi: 10.1002/ajpa.23258. Epub 2017 Jun 8.
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IMAGES IN CLINICAL MEDICINE. Hair-on-End Sign.
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Bone disease and skeletal complications in patients with β thalassemia major.β 地中海贫血重型患者的骨骼疾病和骨骼并发症。
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古 DNA 表明,贫血症和低骨密度是导致古代个体出现多孔性骨质增生的原因。
Sci Rep. 2023 Apr 28;13(1):6968. doi: 10.1038/s41598-023-33405-7.
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Classification and Morphometric Features of Pterion in Thai Population with Potential Sex Prediction.泰国人群翼点的分类及形态计量学特征与潜在性别预测
Medicina (Kaunas). 2021 Nov 21;57(11):1282. doi: 10.3390/medicina57111282.
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The causes of porotic hyperostosis and cribra orbitalia: a reappraisal of the iron-deficiency-anemia hypothesis.多孔性骨质增生和眶筛状板病变的病因:对缺铁性贫血假说的重新评估。
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Prevalence and etiology of acquired anemia in Medieval York, England.
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Porotic hyperostosis: relationship between orbital and vault lesions.
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