| ObjectiveHypokalemic periodic paralysis is a clinical syndrome characterized by periodic sudden attacks of muscle weakness associated with a decrease in serum potassium.The complex pathogenesis has not been fully elucidated.The treatment is symptomatic treatment of potassium supplementation which dose not prevent the recurrence of paralysis.So we summarized the clinical characteristics of patients with HOKPP and observed morphological changes in skeletal muscle, expression of TNF-α,IL-6 and deposition of immune complexes to study the the role of inflammatory and immune factors in the development of HOKPP, and discuss the pathogenesis of HOKPP from the angle of inflammation - immune, providing a theoretical basis for the prevention and treatment.MethodsPatients diagnosed as HOKPP collected from the Department of Endocrinology, General Hospital of Tianjin Medical University. Male 33, female3.Summarized the clinical characteristics of 36 patients.Control group consisted of fracture patients collected from Orthopedics, General Hospital of Tianjin Medical University,male 12, female 2. All patients underwent muscle biopsy. The pathological changes were observed under light microscope. Using the method of immunohistochemistry staining to observe the expression of TNF-αand IL-6, and the immunofluorescence staining to observe the deposition of IgA, IgG, IgM, C3, C1q and FRA. Applying the Image-Pro Plus 6.0 for the image analysis, and SPSS 17.0 for statistical analysis.Results1. Age of onset (25.94±10.29) years, male to female 33:3,27 cases complained of limb weakness and flaccid paralysis, while 17 cases with muscle soreness.24 cases presented the symptoms in the early morning.19 cases had significant inducement before the onset.6 cases had family history.Serum potassium (2.21±0.58) mmol/L,24h urinary potassium (65.92±30.14) mmol/24h; 1 case with DM, 5 cases with IGT,32 cases with hyperinsulinemia. AST(46.67±33.69) U/L, CK (118.22±77.39) U/L. ACTH (48.71±27.70) pg/ml, COR (21.88±8.85) ug/ml, 24h urinary COR (77.36±37.36) ug/24h, PRA (1.28±1.43)ng/ml/h, ALD (12.64±3.95)ng/dl, ATⅡ(50.55±14.68)pg/ml, 24h urinary ALD (2.57±1.79) ug/24h.2.HOKPP group:part of the skeletal muscle fibers atrophy, vacuolar degeneration, cytoplasmic absorption, fibrosis,muscle striations fuzzy, light band and dark band disappeared,nuclear proliferation and ingression, stromal hyperplasia with scattered inflammatory cells. Expression of TNF-a in HOKPP was significantly positive to the control group (p<0.01). Expression of IL-6 in HOKPP was significantly positive to the control group (p<0.01).The deposition of IgA, IgG, IgM, C3, Clq, and FRA of HOKPP were significantly positive to the control group (p <0 .01).Conclusion1. Multi-factors participate in the course of HOKPP.2. The clinical features of HOKPP included mostly of the cases were sporadic presenting on the age fromlO to 30, most of the cases were male,significant inducement before the onset, attack occurred usually on the early morning involving both lower extremities. Serum potassium levels are of severe low-grade, completely normal during the interictal period. Most cases associated with hyperinsulinemia and IGT.High corticosteroid levels and activity of RAAS. The level of the AST and CK elevated, the lower of the serum potassium level, the more remarkable the creatase elevated.3. There were histopathological changes, inflammatory factors and immune complex deposition in skeletal muscle of HOKPP, which inferred that inflammatory factors-mediated immunological reaction may participate in the course of HOKPP. |
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