Abstract:
Sagliker syndrome (SS) is a rare but distinctive form of renal osteodystrophy associated with poorly managed secondary hyperparathyroidism (SHPT) in patients with chronic kidney disease (CKD). We present a case of a 28-year-old male with end-stage CKD on hemodialysis for 10 years, who exhibited progressive facial deformities and maxillofacial bone pain. Physical examination revealed bilateral expansion of the maxillary and mandibular bones and facial asymmetry. Radiological findings included diffuse bone thickening and multilocular cysts in the maxillofacial bones, while laboratory tests showed decreased levels of calcium and elevated parathyroid hormone, confirming SHPT. Despite multidisciplinary management involving nephrology, endocrinology, and maxillofacial surgery, the patient\'s condition deteriorated and he manifested community-acquired pneumonia leading to cardiopulmonary arrest and death. This case underscores the challenges in managing severe HPT in CKD and emphasizes the importance of early assessment and comprehensive multidisciplinary care to prevent irreversible complications.
摘要:
Sagliker综合征(SS)是一种罕见但独特的肾性骨营养不良形式,与慢性肾脏疾病(CKD)患者管理不善的继发性甲状旁腺功能亢进(SHPT)相关。我们介绍了一个28岁的男性,患有终末期CKD的血液透析10年,表现出进行性面部畸形和颌面部骨痛。体格检查显示上颌骨和下颌骨的双侧扩张以及面部不对称。放射学发现包括颌面部骨弥漫性骨增厚和多房性囊肿,虽然实验室检查显示钙水平下降和甲状旁腺激素升高,确认SHPT。尽管涉及肾病学的多学科管理,内分泌学,和颌面外科,患者病情恶化,表现为社区获得性肺炎,导致心肺骤停和死亡。该病例强调了治疗CKD严重HPT的挑战,并强调了早期评估和综合多学科护理对预防不可逆并发症的重要性。