Bibik E E, Eremkina A K, Knyazeva O A, Mokrysheva N G
Endocrinology Research Centre.
"Altamed plus".
Probl Endokrinol (Mosk). 2021 Nov 17;67(6):31-38. doi: 10.14341/probl12798.
Multiple lesions of the parathyroid glands (PTG) in primary hyperparathyroidism (PHPT) can be sporadic or develop as part of hereditary syndromes, manifesting at young age. There the description of a severe sporadic PHPT with big parathyroid neoplasms in the young patient is presented. Clinical data made it possible to suspect MEN-1 syndrome or parathyroid carcinomas; however, mutations CDKN, CDC73, MEN1 were excluded. The patient underwent removal of three identified tumors: benign adenomas of the left PTG and hyperplasia of the right one. Postoperative hypocalcemia and severe hungry bone syndrome required the administration of vitamin D and calcium carbonate preparations. However, a year after the operation, a «mild» recurrent disease was confirmed. Taking into account the patient's refusal to reoperation and a significant improvement of the target organs state, active observation was continued. The patient needs further careful dynamic monitoring by specialists in order to timely identify indications for repeated surgical treatment to improve the life quality and span.
原发性甲状旁腺功能亢进症(PHPT)中甲状旁腺(PTG)的多发性病变可以是散发性的,也可以作为遗传性综合征的一部分出现,在年轻时发病。本文介绍了一名年轻患者患有严重散发性PHPT且伴有大的甲状旁腺肿瘤的情况。临床资料使人怀疑为MEN-1综合征或甲状旁腺癌;然而,排除了CDKN、CDC73、MEN1的突变。患者接受了切除三个已确定肿瘤的手术:左侧PTG的良性腺瘤和右侧PTG的增生。术后低钙血症和严重的饥饿骨综合征需要给予维生素D和碳酸钙制剂。然而,术后一年证实出现了“轻度”复发性疾病。考虑到患者拒绝再次手术且靶器官状态有显著改善,继续进行积极观察。患者需要专科医生进一步仔细的动态监测,以便及时确定再次手术治疗的指征,从而提高生活质量和延长寿命。