García Romero José Manuel, Guerrero Morales Pedro Hugo, Rico Razo Maria Fernanda, Córdova Argueta José Macario, Olaya Niebla Erick
Transplant and Donation Department, Regional General Hospital 1 of the Mexican Social Security Institute, Querétaro, MEX.
Cureus. 2024 Jul 12;16(7):e64399. doi: 10.7759/cureus.64399. eCollection 2024 Jul.
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.
萨利克综合征(SS)是一种罕见但独特的肾性骨营养不良形式,与慢性肾脏病(CKD)患者继发性甲状旁腺功能亢进(SHPT)管理不善有关。我们报告一例28岁男性,终末期CKD,已接受血液透析10年,出现进行性面部畸形和颌面骨疼痛。体格检查发现上颌骨和下颌骨双侧膨隆及面部不对称。影像学检查结果包括颌面骨弥漫性骨质增厚和多房囊肿,而实验室检查显示钙水平降低和甲状旁腺激素升高,确诊为SHPT。尽管进行了包括肾脏病学、内分泌学和颌面外科的多学科管理,但患者病情仍恶化,出现社区获得性肺炎,导致心肺骤停并死亡。该病例突显了CKD患者严重甲状旁腺功能亢进管理中的挑战,并强调了早期评估和全面多学科护理以预防不可逆并发症的重要性。