20例低血钾型周期性麻痹患者临床和病理分析

CLINICAL AND PATHOLOGICAL STUDY ON 20 PATIENTS OF HYPOKALEMIC PERIODIC PARALYSIS

目的探讨低血钾型周期性麻痹(hypokalemic period paralysis,HOPP)患者的临床及骨骼肌病理特点。方法回顾性分析2005年9月—2012年12月在河北医科大学第三医院做骨骼肌活检的HOPP患者20例,均行血钾、肌酸激酶、心电图及甲状腺功能检查。结果 20例患者中,原发性HOPP组16例,甲状腺功能亢进合并HOPP组(TPP组)4例。TPP组的起病年龄较原发性HOPP组晚,(42.75±9.36)岁vs(24.81±5.49)岁(P<0.01);原发性HOPP组与TPP组分别有43.75%(7/16)和25.00%(1/4)的患者存在血清肌酸激酶水平异常增高;2组患者血钾水平差异无统计学意义,(2.82±0.30)mmol/L vs(2.58±0.13)mmol/L);20例患者中,有8例(40.00%)骨骼肌活检见肌纤维胞浆中存在典型"管聚集"现象,其中原发性HOPP组7例,TPP组1例。结论低血钾型周期性麻痹"管聚集"的发病率为40.00%,8例患者存在肌纤维网结构紊乱、"管聚集"现象,原发性HOPP组"管聚集"现象较TPP组更常见;原发性HOPP组发病年龄较早。

Objective To explore the clinical and skeletal muscle pathological features of hypokalemic period paralysis （HOPP） patients. Methods The clinical data of 20 patients with hypokalemic periodic paralysis, who received skeletal muscle biopsy, were retrospectively analyzed from September 2005 to December 2012 at the hospital, all patients were performed serum potassium, serum creatinine, electrocardiogram （ECG）, and thyroid function. Results Sixteen patients were diagnosed as primary HOPP, and four as thyrotoxic periodic paralysis （TPP）. The average age of primary HOPP group was （ 24.81± 5.49 ） years old,and （42.75 ± 9.36 ） years old in TPP group （P 〈 0.01 ）. About 43.75% （7/16） patients with primary HOPP and 25.00% （1/4） patients with TPP had elevated levels of serum creatinine. The average level of serum potassium was （2.58 ± 0.13 ） mmol/L in HOPP group and （2.82 ±0.30） mmolfL in TPP group （P 〉0.05）. There were 8 （8/20,40.00%） patients had tubular aggregate phenomenon in muscle fibers cytoplasm according to pathological examination, 7 patients with primary HOPP, and 1 with TPP. Conclusion Incidence of hypokalemie periodic paralysis tubular aggregate about 40.00% , 8 patients with muscle fibers network structure disorder and tubular aggregate phenomenon, which are more common in primary HOPP group than TPP group. The age at onset is earlier in primary HOPP group than TPP group.