Literature DB >> 22715234

Primary Sjögren's disease and its complications presenting with progressive paralysis.

Matthew Julian1, Trishna Chakravorty, Philip Dyer.   

Abstract

A 24-year-old female presented with generalised weakness, lethargy and aches in legs. She was subsequently found to be markedly hypokalaemic and have a metabolic acidosis. A diagnosis of distal renal tubular acidosis (RTA) was made. In addition to this failure to alkalinise her urine, she was unable to concentrate it and so a diagnosis of nephrogenic diabetes insipidus was reached. Further questioning revealed previous investigation of a connective tissue disorder thought to be primary Sjögren's syndrome. RTA is a recognised but rare complication of Sjögren's syndrome. Urinary alkalinisation using potassium bicarbonate was instituted; the patient responded well to treatment and is having outpatient follow-up.

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Year:  2011        PMID: 22715234      PMCID: PMC3029323          DOI: 10.1136/bcr.09.2010.3347

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  10 in total

1.  Altered expression of renal NHE3, TSC, BSC-1, and ENaC subunits in potassium-depleted rats.

Authors:  Marie-Louise Elkjaer; Tae-Hwan Kwon; Weidong Wang; Jakob Nielsen; Mark A Knepper; Jørgen Frøkiaer; Søren Nielsen
Journal:  Am J Physiol Renal Physiol       Date:  2002-07-30

Review 2.  Renal tubular acidosis: the clinical entity.

Authors:  Juan Rodríguez Soriano
Journal:  J Am Soc Nephrol       Date:  2002-08       Impact factor: 10.121

3.  Renal tubular acidosis in primary Sjögren's syndrome.

Authors:  K C Siamopoulos; M Elisaf; A A Drosos; A A Mavridis; H M Moutsopoulos
Journal:  Clin Rheumatol       Date:  1992-06       Impact factor: 2.980

4.  Hypokalemia-induced downregulation of aquaporin-2 water channel expression in rat kidney medulla and cortex.

Authors:  D Marples; J Frøkiaer; J Dørup; M A Knepper; S Nielsen
Journal:  J Clin Invest       Date:  1996-04-15       Impact factor: 14.808

5.  Absence of vacuolar H(+)-ATPase pump in the collecting duct of a patient with hypokalemic distal renal tubular acidosis and Sjögren's syndrome.

Authors:  P E DeFranco; L Haragsim; P G Schmitz; B Bastani
Journal:  J Am Soc Nephrol       Date:  1995-08       Impact factor: 10.121

Review 6.  Hypokalaemic paralysis.

Authors:  S K Ahlawat; A Sachdev
Journal:  Postgrad Med J       Date:  1999-04       Impact factor: 2.401

7.  Amphotericin B decreases adenylyl cyclase activity and aquaporin-2 expression in rat kidney.

Authors:  S W Kim; C H Yeum; S Kim; Y Oh; K C Choi; J Lee
Journal:  J Lab Clin Med       Date:  2001-10

8.  Cellular localization of type 5 and type 6 ACs in collecting duct and regulation of cAMP synthesis.

Authors:  C Héliès-Toussaint; L Aarab; J M Gasc; J M Verbavatz; D Chabardès
Journal:  Am J Physiol Renal Physiol       Date:  2000-07

9.  Hypokalaemia and paralysis.

Authors:  S-H Lin; M R Davids; M L Halperin
Journal:  QJM       Date:  2003-02

10.  Combined renal tubular acidosis and diabetes insipidus in hematological disease.

Authors:  Ewout J Hoorn; Robert Zietse
Journal:  Nat Clin Pract Nephrol       Date:  2007-03
  10 in total

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