尿毒症:终末期慢性肾脏疾病的挑战。关于一个案例]。

Valentina Burckhardt-Bravo, Antonia Bañados-Alarcón, Rodrigo Funes-Ferrada, César Caviedes-Rivera
{"title":"尿毒症:终末期慢性肾脏疾病的挑战。关于一个案例]。","authors":"Valentina Burckhardt-Bravo, Antonia Bañados-Alarcón, Rodrigo Funes-Ferrada, César Caviedes-Rivera","doi":"10.4067/s0034-98872024000500634","DOIUrl":null,"url":null,"abstract":"<p><p>Uremic leontiasis ossia (ULO) is a rare manifestation of renal osteodystrophy in) patients with end-stage chronic kidney disease (CKD) and secondary hyperparathyroidism (SHPTH). It occurs due to increased osteoclastic activity secondary to high plasmatic parathyroid hormone (PTH) levels. This leads to bone deformation with thickening and massive enlargement of the cranial vault, resulting in a leonine face appearance. Imaging such as CT scans of the head and neck can reveal bone remodeling, which aids in diagnosing ULO in patients with elevated PTH. Treatment requires a multidisciplinary approach, including medical management of SHPTH, parathyroidectomy, and osteoplasty under a maxillofacial surgery specialist to correct anatomical abnormalities.</p><p><strong>Aim: </strong>Herein, we present a case report of a patient with ULO, demonstrating valuable insights into early recognition and multidisciplinary management of the disease.</p><p><strong>Case report: </strong>A 39-year-old female patient with a past medical history of CKD on hemodialysis, hypertension, and SHPTH. The patient was admitted for total parathyroidectomy due to non-toxic multinodular goiter. Physical exam shows a nonpainful increasing bilateral growth of maxillary and mandibular bone, loss of nasolabial folds, and widening of the nares, resulting in a leonine appearance. Relevant laboratory exams showed a plasmatic PTH level of 4557 pg/ml. CT scan of the head and neck shows tunnel-like bones in the cranial vault consistent with ULO. The patient underwent total thyroidectomy and subtotal parathyroidectomy without complications. Laboratory exams on the first day post-surgery showed improvement in PTH plasmatic levels.</p><p><strong>Conclusion: </strong>ULO is a rare disease and poses a diagnostic challenge. Early recognition of clinical features of ULO and elevated PTH levels in end-stage CKD patients is essential for a prompt diagnosis and appropriate treatment to avoid the consequences of ULO.</p>","PeriodicalId":101370,"journal":{"name":"Revista medica de Chile","volume":"152 5","pages":"634-639"},"PeriodicalIF":0.0000,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Uremic Leontiasis Ossea: A Challenge in End-Stage Chronic Kidney Disease. About a Case].\",\"authors\":\"Valentina Burckhardt-Bravo, Antonia Bañados-Alarcón, Rodrigo Funes-Ferrada, César Caviedes-Rivera\",\"doi\":\"10.4067/s0034-98872024000500634\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Uremic leontiasis ossia (ULO) is a rare manifestation of renal osteodystrophy in) patients with end-stage chronic kidney disease (CKD) and secondary hyperparathyroidism (SHPTH). It occurs due to increased osteoclastic activity secondary to high plasmatic parathyroid hormone (PTH) levels. This leads to bone deformation with thickening and massive enlargement of the cranial vault, resulting in a leonine face appearance. Imaging such as CT scans of the head and neck can reveal bone remodeling, which aids in diagnosing ULO in patients with elevated PTH. Treatment requires a multidisciplinary approach, including medical management of SHPTH, parathyroidectomy, and osteoplasty under a maxillofacial surgery specialist to correct anatomical abnormalities.</p><p><strong>Aim: </strong>Herein, we present a case report of a patient with ULO, demonstrating valuable insights into early recognition and multidisciplinary management of the disease.</p><p><strong>Case report: </strong>A 39-year-old female patient with a past medical history of CKD on hemodialysis, hypertension, and SHPTH. The patient was admitted for total parathyroidectomy due to non-toxic multinodular goiter. Physical exam shows a nonpainful increasing bilateral growth of maxillary and mandibular bone, loss of nasolabial folds, and widening of the nares, resulting in a leonine appearance. Relevant laboratory exams showed a plasmatic PTH level of 4557 pg/ml. CT scan of the head and neck shows tunnel-like bones in the cranial vault consistent with ULO. The patient underwent total thyroidectomy and subtotal parathyroidectomy without complications. Laboratory exams on the first day post-surgery showed improvement in PTH plasmatic levels.</p><p><strong>Conclusion: </strong>ULO is a rare disease and poses a diagnostic challenge. Early recognition of clinical features of ULO and elevated PTH levels in end-stage CKD patients is essential for a prompt diagnosis and appropriate treatment to avoid the consequences of ULO.</p>\",\"PeriodicalId\":101370,\"journal\":{\"name\":\"Revista medica de Chile\",\"volume\":\"152 5\",\"pages\":\"634-639\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-05-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Revista medica de Chile\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4067/s0034-98872024000500634\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Revista medica de Chile","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4067/s0034-98872024000500634","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

摘要

骨性尿毒症(ULO)是终末期慢性肾病(CKD)和继发性甲状旁腺功能亢进(SHPTH)患者肾性骨营养不良的一种罕见表现。它的发生是由于高血浆甲状旁腺激素(PTH)水平继发的破骨细胞活性增加。这导致骨变形,增厚和颅骨拱顶的大量扩大,导致狮子脸的外观。头部和颈部的CT扫描等成像可以显示骨重塑,这有助于诊断PTH升高患者的ULO。治疗需要多学科的方法,包括SHPTH的医疗管理、甲状旁腺切除术和颌骨外科专家的骨成形术来纠正解剖异常。目的:在此,我们提出了一例ULO患者的病例报告,展示了对该疾病的早期识别和多学科管理的有价值的见解。病例报告:一名39岁女性患者,既往有血液透析、高血压和SHPTH的CKD病史。患者因无毒性多结节性甲状腺肿入院行甲状旁腺全切除术。体格检查显示双侧上颌骨和下颌骨无痛性增长,鼻唇沟缺失,鼻孔变宽,呈狮形外观。相关实验室检查显示血浆甲状旁腺素4557 pg/ml。头部和颈部的CT扫描显示颅顶有隧道状骨,符合ULO。患者行甲状腺全切除术及甲状旁腺次全切除术,无并发症。术后第一天的实验室检查显示PTH血浆水平有所改善。结论:ULO是一种罕见的疾病,对诊断具有挑战性。早期认识终末期CKD患者ULO的临床特征和PTH水平升高对于及时诊断和适当治疗以避免ULO的后果至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
[Uremic Leontiasis Ossea: A Challenge in End-Stage Chronic Kidney Disease. About a Case].

Uremic leontiasis ossia (ULO) is a rare manifestation of renal osteodystrophy in) patients with end-stage chronic kidney disease (CKD) and secondary hyperparathyroidism (SHPTH). It occurs due to increased osteoclastic activity secondary to high plasmatic parathyroid hormone (PTH) levels. This leads to bone deformation with thickening and massive enlargement of the cranial vault, resulting in a leonine face appearance. Imaging such as CT scans of the head and neck can reveal bone remodeling, which aids in diagnosing ULO in patients with elevated PTH. Treatment requires a multidisciplinary approach, including medical management of SHPTH, parathyroidectomy, and osteoplasty under a maxillofacial surgery specialist to correct anatomical abnormalities.

Aim: Herein, we present a case report of a patient with ULO, demonstrating valuable insights into early recognition and multidisciplinary management of the disease.

Case report: A 39-year-old female patient with a past medical history of CKD on hemodialysis, hypertension, and SHPTH. The patient was admitted for total parathyroidectomy due to non-toxic multinodular goiter. Physical exam shows a nonpainful increasing bilateral growth of maxillary and mandibular bone, loss of nasolabial folds, and widening of the nares, resulting in a leonine appearance. Relevant laboratory exams showed a plasmatic PTH level of 4557 pg/ml. CT scan of the head and neck shows tunnel-like bones in the cranial vault consistent with ULO. The patient underwent total thyroidectomy and subtotal parathyroidectomy without complications. Laboratory exams on the first day post-surgery showed improvement in PTH plasmatic levels.

Conclusion: ULO is a rare disease and poses a diagnostic challenge. Early recognition of clinical features of ULO and elevated PTH levels in end-stage CKD patients is essential for a prompt diagnosis and appropriate treatment to avoid the consequences of ULO.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
自引率
0.00%
发文量
0
期刊最新文献
[Another Lesson from the Pandemic: Supervisor Social Support for Mental Health Protection in Healthcare Workers, Chile]. [Chronic Insomnia: A Public Health Problem]. [Coronary Angiography in Clinical Practice: Update on Indications, Complications, and Contraindications]. [Diversity and Institution: Scopes for Health Sciences Education]. [Duties of the Physician in Relation to the Use of Alternative and Complementary Medicines by Their Patients].
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1