Inherited Defects in Distal Tubular Acidification

Editorials1 April 1975Inherited Defects in Distal Tubular AcidificationTERRY G. FEEST, M.B., OLIVER M. WRONG, D.M., F.R.C.P.TERRY G. FEEST, M.B.Search for more papers by this author, OLIVER M. WRONG, D.M., F.R.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-82-4-584 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe pathognomonic defect of classic, or type I, renal tubular acidosis is an inability of the distal tubule to maintain the normal hydrogen ion gradient between tubular urine and plasma, and so secrete a maximally acid urine (1, 2). The full syndrome includes, by definition, a systemic acidosis, but the development of acidosis depends on many factors, including the severity of the acidification defect, urinary excretion of hydrogen ion acceptors such as ammonia and phosphate, and the load of endogenously produced hydrogen ion requiring excretion. As might be expected, some patients do not have systemic acidosis despite the presence of...References1. MORRISSEBASTIANMCSHERRY RAE: Renal acidosis. Kidney Int 1:322-340, 1972 CrossrefMedlineGoogle Scholar2. SELDINWILSON DJ: Renal tubular acidosis, in The Metabolic Basis of Inherited Disease, 3rd ed., edited by STANBURY JB, WYNGAARDEN JB, FREDERICKSON DS. New York, McGraw-Hill, 1972, pp. 1548-1566 Google Scholar3. WRONGDAVIES OH: The excretion of acid in renal disease. Q J Med, 28:259-313, 1959 MedlineGoogle Scholar4. DEDMONWRONG RO: The excretion of organic anion in renal tubular acidosis, with particular reference to citrate. Clin Sci 22:19-32, 1962 MedlineGoogle Scholar5. RODRIGUEZ-SORIANOEDELMANN JC: Renal tubular acidosis. Ann Rev Med 20:363-380, 1969 CrossrefMedlineGoogle Scholar6. BUCKALEWPURVISSHULMAN VMM: Hereditary renal tubular acidosis. Medicine (Baltimore) 53:229-254, 1974 CrossrefMedlineGoogle Scholar7. HAYSLETTPERILLIEFINCH JPS: Urinary muramidase and renal disease. N Engl J Med 279:506-512, 1968 CrossrefMedlineGoogle Scholar8. PARFITTHIGGINSNASSIM ABJ: Metabolic studies in patients with hypercalciuria. Clin Sci 27:463-482, 1964 MedlineGoogle Scholar9. HARRISONCHISHOLMHARRISON HJH: Congenital renal tubular acidosis. AMA J Dis Child 96:588, 1958 Google Scholar10. ROSEHARRISON GA: The incidence, investigation and treatment of idiopathic hypercalciuria. Br J Urol 46:261-274, 1974 CrossrefMedlineGoogle Scholar11. COCHRANPEACOCKSMITH MMD: Renal tubular acidosis of pyelonephritis with renal stone disease. Br Med J 2:721-729, 1968 CrossrefMedlineGoogle Scholar12. BERLYNE G: Distal tubular function in chronic hydronephrosis. Q J Med 30:339-355, 1961 MedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: TERRY G. FEEST, M.B.; OLIVER M. WRONG, D.M., F.R.C.P.Affiliations: Medical Unit University College Hospital Medical School London England PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byCalcium oxalate nephrolithiasis: Defective oxalate transportDisorders of Proton Secretion by the KidneyDisorders of Proton Secretion by the KidneyFamily History of Renal Stones in Recurrent Stone PatientsThe kidneys in health and disease: renal physiology and pathophysiologyUrinary citrate excretion in the diagnosis of distal renal tubular acidosisCellular Defects in Urinary Acidification and Renal Tubular Acidosis 1 April 1975Volume 82, Issue 4Page: 584-585KeywordsAmmoniaBlood plasmaExcretionPhosphatesUrine ePublished: 1 December 2008 Issue Published: 1 April 1975 PDF downloadLoading ...