Alport Syndrome Medication

  • Author: Ramesh Saxena, MD, PhD; Chief Editor: Vecihi Batuman, MD, FACP, FASN   more...
 
Updated: Nov 21, 2011
 

Medication Summary

ACE inhibitors or angiotensin-receptor blockers (ARBs) should be administered to patients with Alport syndrome who have proteinuria with or without hypertension.

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ACE inhibitors

Class Summary

Help to reduce proteinuria by decreasing intraglomerular pressure; moreover, angiotensin II is a growth factor that is implicated in glomerular sclerosis. By inhibiting angiotensin II, these drugs have a potential role in slowing down glomerular sclerosis.

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Enalapril (Vasotec)

 

Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.

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Fosinopril (Monopril)

 

Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.

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Lisinopril (Zestril, Prinivil)

 

Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.

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Quinapril (Accupril)

 

Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.

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Angiotensin-receptor blockers

Class Summary

Help reduce proteinuria by decreasing the intraglomerular pressure. By inhibiting angiotensin II, these drugs have a potential role in slowing down glomerular sclerosis, as with ACE inhibitors. Unlike ACE inhibitors, ARBs do not activate bradykinin and are not associated with cough and angioedema.

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Losartan (Cozaar)

 

Nonpeptide angiotensin II receptor antagonist that blocks the vasoconstrictor and aldosterone-secreting effects of angiotensin II. May induce a more complete inhibition of the renin-angiotensin system than ACE inhibitors and do not affect the response to bradykinin and are less likely to be associated with cough and angioedema. For patients unable to tolerate ACE inhibitors.

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Candesartan (Atacand)

 

Nonpeptide angiotensin II receptor antagonist that blocks the vasoconstrictor and aldosterone-secreting effects of angiotensin II. May induce a more complete inhibition of the renin-angiotensin system than ACE inhibitors and do not affect the response to bradykinin and are less likely to be associated with cough and angioedema. For patients unable to tolerate ACE inhibitors.

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Contributor Information and Disclosures
Author

Ramesh Saxena, MD, PhD  Associate Professor, Department of Internal Medicine, Division of Nephrology, University of Texas Southwestern Medical Center

Ramesh Saxena, MD, PhD is a member of the following medical societies: American Medical Association, American Society of Nephrology, International Society of Nephrology, and National Kidney Foundation

Disclosure: e-medicine Honoraria authoring review articles

Specialty Editor Board

Frank C Brosius III, MD  Nephrology Program Director, Professor of Internal Medicine and Physiology, Department of Internal Medicine, Division of Nephrology, University of Michigan School of Medicine

Frank C Brosius III, MD is a member of the following medical societies: Alpha Omega Alpha, American Diabetes Association, American Society of Nephrology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Eleanor Lederer, MD  Professor of Medicine, Chief, Nephrology Division, Director, Nephrology Training Program, Director, Metabolic Stone Clinic, Kidney Disease Program, University of Louisville School of Medicine; Consulting Staff, Louisville Veterans Affairs Hospital

Eleanor Lederer, MD is a member of the following medical societies: American Association for the Advancement of Science, American Federation for Medical Research, American Society for Biochemistry and Molecular Biology, American Society for Bone and Mineral Research, American Society of Nephrology, American Society of Transplantation, International Society of Nephrology, Kentucky Medical Association, National Kidney Foundation, and Phi Beta Kappa

Disclosure: Dept of Veterans Affairs Grant/research funds Research

Rebecca J Schmidt, DO, FACP, FASN  Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine

Rebecca J Schmidt, DO, FACP, FASN is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation, Renal Physicians Association, and West Virginia State Medical Association

Disclosure: Renal Ventures Ownership interest Other

Chief Editor

Vecihi Batuman, MD, FACP, FASN  Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Medicine Service, Southeast Louisiana Veterans Health Care System

Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, and International Society of Nephrology

Disclosure: Nothing to disclose.

References

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Electron micrograph of kidney biopsy from a patient with Alport syndrome. Note the splitting and lamellation of the glomerular basement membrane (see arrows).
Table 1. Location and Mutations of the Genes Coding for Alpha (IV) Chains of Type IV Collagen in Alport Syndrome
Alpha (IV) ChainGenesChromosomal LocationMutation
Alpha-1 (IV)COL4A113Unknown
Alpha-2 (IV)COL4A213Unknown
Alpha-3 (IV)COL4A32ARAS*
Alpha-4 (IV)COL4A42ARAS
Alpha-5 (IV)COL4A5xXLAS †
Alpha-6 (IV)COL4A6xLeiomyomatosis ‡
* Autosomal recessive Alport syndrome (mutations spanning 5' regions of COL4A5 and COL4A6 genes)



† X-linked Alport syndrome



‡ Autosomal recessive Alport syndrome



Table 2. Tissue Distribution of Alpha (IV) Chains
Alpha (IV) ChainTissue Distribution
Alpha-1 (IV)Ubiquitous
Alpha-2 (IV)Ubiquitous
Alpha-3 (IV)GBM, distal TBM*, Descemet membrane, Bruch membrane, anterior lens capsule, lungs, cochlea
Alpha-4 (IV)GBM, distal TBM, Descemet membrane, Bruch membrane, anterior lens capsule, lungs, cochlea
Alpha-5 (IV)GBM, distal TBM, Descemet membrane, Bruch membrane, anterior lens capsule, lungs, cochlea
Alpha-6 (IV)Distal TBM, epidermal basement membrane
* Tubular basement membrane
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