Shakeri Sara, Zarehparvar Moghadam Soroush, Sadeghi Ramin, Ayati Narjess
Nuclear Medicine Research Centre, Mashhad University of Medical Sciences, Mashhad, Iran.
Asia Ocean J Nucl Med Biol. 2018 Spring;6(2):167-170. doi: 10.22038/aojnmb.2018.10567.
Sagliker syndrome is a rare form of renal osteodystrophy resulted from untreated secondary hyperparathyroidism. It is described by severe skeletal deformities, high level of PTH in patients with chronic renal failure, and deformed face. This paper reports a 44-year-old male patient with the mentioned characteristics. In addition to the unique clinical features, high levels of ALP and PTH hormones encouraged us to search for syndrome-like a disease, which clinically and paraclinically matched the Sagliker syndrome. This case highlights the importance of clinicians' attention for early monitoring and appropriate treatment as it is shown to be effective in preventing irreversible complications such as soft tissue and bone abnormalities and cardiovascular impairment in patients with Sagliker syndrome. Therefore, considering the syndrome is recommended as one of the diagnostic hypothesis in young patients with renal insufficiency, secondary hyperparathyroidism, and skeletal deformities.
萨利克综合征是一种由于继发性甲状旁腺功能亢进未得到治疗而导致的罕见肾性骨营养不良形式。它的特征是严重的骨骼畸形、慢性肾衰竭患者甲状旁腺激素水平升高以及面部畸形。本文报告了一名具有上述特征的44岁男性患者。除了独特的临床特征外,碱性磷酸酶(ALP)和甲状旁腺激素(PTH)水平升高促使我们寻找一种类似该综合征的疾病,其临床和副临床特征与萨利克综合征相符。该病例突出了临床医生早期监测和适当治疗的重要性,因为这已被证明可有效预防萨利克综合征患者出现软组织和骨骼异常以及心血管损害等不可逆并发症。因此,对于患有肾功能不全、继发性甲状旁腺功能亢进和骨骼畸形的年轻患者,建议将该综合征作为诊断假设之一。