Division of Endocrinology and Metabolism, Department of Internal Medicine, Korea University College of Medicine, Seoul, Republic of Korea.
Department of Biostatistics, Korea University College of Medicine, Seoul, Republic of Korea.
Front Endocrinol (Lausanne). 2023 Aug 10;14:1229750. doi: 10.3389/fendo.2023.1229750. eCollection 2023.
Chronic idiopathic hypophosphatemia (CIH) induced by X-linked hypophosphatemic rickets or tumor-induced osteomalacia is a rare inherited or acquired disorder. However, due to its rarity, little is known about the epidemiology and natural course of CIH. Therefore, we aimed to identify the prevalence and long-term health outcomes of CIH patients.
Using the Korean Health Insurance Review and Assessment claims database, we evaluated the incidence of hypophosphatemia initially diagnosed from 2003 to 2018. After excluding secondary conditions that could change serum phosphorus levels, we identified 154 patients (76 men and 78 women) with non-secondary and non-renal hypophosphatemia. These hypophosphatemic patients were compared at a ratio of 1:10 with age-, sex-, and index-year-matched controls (n = 1,540).
In the distribution of age at diagnosis, a large peak was observed in patients aged 1-4 years and small peaks were observed in ages from 40-70 years. The age-standardized incidence rate showed non-statistically significant trend from 0.24 per 1,000,000 persons in 2003 to 0.30 in 2018. Hypophosphatemic patients had a higher risk of any complication (adjusted hazard ratio [aHR], 2.17; 95% confidence interval [CI], 1.67-2.69) including cardiovascular outcomes, chronic kidney disease, hyperparathyroidism, osteoporotic fractures, periodontitis, and depression. Hypophosphatemic patients also had higher risks of mortality and hospitalization than the controls (aHR, 3.26; 95% CI, 1.83-5.81; and aHR, 2.49; 95% CI, 1.97-3.16, respectively).
This first nationwide study of CIH in South Korea found a bimodal age distribution and no sex differences among patients. Hypophosphatemic patients had higher risks of complications, mortality, and hospitalization compared to age- and sex-matched controls.
X 连锁低磷性佝偻病或肿瘤诱导性骨软化症引起的慢性特发性低磷血症（CIH）是一种罕见的遗传性或获得性疾病。然而，由于其罕见性，人们对 CIH 的流行病学和自然病程知之甚少。因此，我们旨在确定 CIH 患者的患病率和长期健康结果。
我们使用韩国健康保险审查和评估索赔数据库，评估了 2003 年至 2018 年首次诊断出的低磷血症的发病率。排除可能改变血清磷水平的继发性疾病后，我们确定了 154 名（76 名男性和 78 名女性）非继发性和非肾性低磷血症患者。这些低磷血症患者与年龄、性别和指数年匹配的对照（n = 1,540）按 1:10 的比例进行比较。
在诊断时的年龄分布中，1-4 岁的患者中有一个大高峰，40-70 岁的患者中有一个小高峰。年龄标准化发病率显示，2003 年为 0.24/100 万人，2018 年无统计学意义趋势为 0.30/100 万人。低磷血症患者发生任何并发症的风险较高（调整后的危险比[aHR]，2.17；95%置信区间[CI]，1.67-2.69），包括心血管结局、慢性肾脏病、甲状旁腺功能亢进、骨质疏松性骨折、牙周炎和抑郁症。低磷血症患者的死亡率和住院率也高于对照组（aHR，3.26；95%CI，1.83-5.81；aHR，2.49；95%CI，1.97-3.16）。
这是韩国首例全国性 CIH 研究，发现患者的年龄分布呈双峰分布，无性别差异。与年龄和性别匹配的对照组相比，低磷血症患者发生并发症、死亡和住院的风险更高。
