Abstract：
Patients with familial hypokalemic periodic paralysis (HOKPP) experience episodes of reversible immobility and are at an increased risk of limited sunlight exposure, potentially leading to vitamin D deficiency. However, there is a lack of data on vitamin D levels in this population. We investigated serum vitamin D levels and their associated factors in children with HOKPP. This study included 170 genetically-confirmed children with HOKPP, aged 3-18 years, and 170 age-, sex-, and body mass index (BMI)-matched healthy controls from the Korean Channelopathy Study, a prospective controlled investigation. Anthropometric and clinical characteristics were recorded, and serum levels of calcium, ionized calcium, phosphorus, alkaline phosphatase, 25-hydroxyvitamin D, and intact parathyroid hormone (PTH) were analyzed. Vitamin D deficiency (< 20 ng/mL) was observed in 87.0% of the patients compared to 45.5% of the controls (P < 0.05) during the summer-fall season. During the winter-spring season, 91.7% of the patients and 73.4% of the controls were deficient (P < 0.05). A strong positive correlation was found between onset age of the first paralytic attack and vitamin D levels (r = 0.78, P < 0.01). Conversely, the frequency and duration of paralytic attacks were negatively correlated with vitamin D levels (r = -0.82 and r = -0.65, P < 0.01, respectively). Age, BMI, age at onset, frequency and duration of attacks, and PTH levels were independently associated with vitamin D levels (ß = -0.10, -0.12, 0.19, -0.27, -0.21, and -0.13, P < 0.05, respectively).
CONCLUSIONS: Vitamin D deficiency was highly prevalent in children with HOKPP, and vitamin D levels correlated with various disease characteristics. We recommend routine screening for vitamin D levels in these patients to address this prevalent deficiency. Considering the high prevalence of vitamin D deficiency observed, further research on other diseases characterized by reversible immobility is warranted.
BACKGROUND: • A correlation between immobility and low serum vitamin D levels has been established. However, the vitamin D status of patients with familial hypokalemic periodic paralysis (HOKPP) who experience periods of reversible immobility remains unknown.
BACKGROUND: • Vitamin D deficiency was highly prevalent in children with HOKPP, and vitamin D levels correlated with various disease characteristics.
CONCLUSIONS: Vitamin D deficiency was highly prevalent in children with HOKPP, and vitamin D levels correlated with various disease characteristics. We recommend routine screening for vitamin D levels in these patients to address this prevalent deficiency. Considering the high prevalence of vitamin D deficiency observed, further research on other diseases characterized by reversible immobility is warranted.
BACKGROUND: • A correlation between immobility and low serum vitamin D levels has been established. However, the vitamin D status of patients with familial hypokalemic periodic paralysis (HOKPP) who experience periods of reversible immobility remains unknown.
BACKGROUND: • Vitamin D deficiency was highly prevalent in children with HOKPP, and vitamin D levels correlated with various disease characteristics.
摘要：
家族性低钾性周期性麻痹（HOKPP）患者会出现可逆性不活动，并且有限的阳光照射风险增加。可能导致维生素D缺乏。然而,该人群缺乏维生素D水平的数据。我们调查了HOKPP患儿的血清维生素D水平及其相关因素。这项研究包括170名遗传证实的HOKPP儿童，3-18岁,170岁-，sex-,和体重指数（BMI）匹配的健康对照来自韩国信道病研究，前瞻性对照调查。记录人体测量和临床特征，和血清钙水平，离子钙，磷，碱性磷酸酶,25-羟基维生素D,和完整的甲状旁腺激素（PTH）进行分析。在夏秋季节，87.0%的患者出现维生素D缺乏（<20ng/mL），而对照组为45.5%（P<0.05）。在冬春季节，91.7％的患者和73.4％的对照组缺乏（P<0.05）。首次麻痹发作的发病年龄与维生素D水平呈显著正相关(r=0.78,P<0.01)。相反，瘫痪发作的频率和持续时间与维生素D水平呈负相关（分别为r=-0.82和r=-0.65，P<0.01）。年龄,BMI,发病年龄,攻击的频率和持续时间，和PTH水平与维生素D水平独立相关（β分别为-0.10、-0.12、0.19、-0.27、-0.21和-0.13，P<0.05）。
结论：维生素D缺乏在HOKPP儿童中非常普遍，维生素D水平与各种疾病特征相关。我们建议对这些患者进行常规的维生素D水平筛查，以解决这种普遍的缺乏。考虑到维生素D缺乏的高患病率，有必要对其他以可逆不动为特征的疾病进行进一步研究。
背景：•已经建立了不动与低血清维生素D水平之间的相关性。然而,家族性低钾性周期性麻痹(HOKPP)患者出现可逆性不活动期的维生素D状态尚不清楚.
背景：•维生素D缺乏在HOKPP儿童中非常普遍，维生素D水平与各种疾病特征相关。
结论：维生素D缺乏在HOKPP儿童中非常普遍，维生素D水平与各种疾病特征相关。我们建议对这些患者进行常规的维生素D水平筛查，以解决这种普遍的缺乏。考虑到维生素D缺乏的高患病率，有必要对其他以可逆不动为特征的疾病进行进一步研究。
背景：•已经建立了不动与低血清维生素D水平之间的相关性。然而,家族性低钾性周期性麻痹(HOKPP)患者出现可逆性不活动期的维生素D状态尚不清楚.
背景：•维生素D缺乏在HOKPP儿童中非常普遍，维生素D水平与各种疾病特征相关。
