继发于原发性干燥综合征的症状性周期性麻痹

来源 :世界核心医学期刊文摘.眼科学分册 | 被引量 : 0次 | 上传用户:programmelove
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Introduction.Hypokalaemic periodic paralysis can be primitive or secondary to potassium deficiency which can arise from several causes.Primary Sjogren’ s syndrome is a rare cause related to kidney involvement.Case report.A 50-year-old woman has been admitted for hypotonic tetraparesis which had appeared a few days earlier.History taking revealed three previous similar episodes with a notion of oral and lacrimal dryness.Laboratory tests revealed severe hypokalaemia,hyperchloremia,alkaline urinary pH and a minima 24h proteinuria.Additional investigations led to the diagnosis of a primary Sjogren’ s syndrome defined on the basis of international criteria.Kidney biopsy revealed tubular-interstitial nephritis.Oral corticosteroid therapy and potassium supplementation led to symptom improvement.A recurrent episode also responded to treatment.Additional urinary alkalinisation has prevented further relapse.Discussion.Primary Sjogren’ s syndrome is an exocrine disease causing systemic disorders.Tubular-inters-titial nephropathy may occur in 25 percent of patients leading to distal tubular acidosis defined by the association of hypokalaemia,hyperchloremia and alkaline urinary pH.When hypokalaemia is severe,periodic paralysis may occur.Conclusion.Primary Sjogren’ s syndrome can lead to nephropathy and subsequent hypokalaemic periodic paralysis.Urinary alkalinisation is essential to prevent this catastrophic presentation from recurring. Introduction. Hypokalaemic periodic paralysis can be primitive or secondary to potassium deficiency which can arise from several causes. Primary Sjogren’s syndrome is a rare cause related to kidney involvement. Case report. A 50-year-old woman has been admitted for hypotonic tetraparesis which had occurred a few days earlier. History taken opened three previous similar episodes with a notion of oral and lacrimal dryness. Laboratory tests revealed severe hypokalaemia, hyperchloremia, alkaline urinary pH and a minima 24h proteinuria. Additional investigations led to the diagnosis of a primary Sjogren’s syndrome defined on the basis of international criteria. Kidney biopsy revealed tubular-interstitial nephritis. Oral corticosteroid therapy and potassium supplementation led to symptom improvement. A recurrent episode also responded to treatment. Additional urinary alkalinisation has prevented further relapse. Discussion. Primary Sjogren’s syndrome is an exocrine disease causing systemic disorders. Tubular-inters-titial nephropathy may occur in 25 percent of patients leading to distal tubular acidosis by the association of hypokalaemia, hyperchloremia and alkaline urinary pH. Whypokalaemia is severe, periodic paralysis may occur. Confluence. Primary Sjogren’s syndrome can lead to nephropathy and subsequent hypokalaemic periodic paralysis. Urinary alkalinisation is essential to prevent this catastrophic presentation from recurring.
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