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Author

Christie P Thomas, MBBS, FRCP, FASN, FAHA Professor, Department of Internal Medicine, Division of Nephrology, Departments of Pediatrics and Obstetrics and Gynecology, Medical Director, Kidney and Kidney/Pancreas Transplant Program, University of Iowa Hospitals and Clinics

Christie P Thomas, MBBS, FRCP, FASN, FAHA is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Nephrology, Royal College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

Khaled Hamawi, MD, MHA Director, Multi Organ Transplant Center, King Fahad Specialist Hospital, Dammam

Khaled Hamawi, MD, MHA is a member of the following medical societies: American Society of Transplantation, American Society of Nephrology

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Eleanor Lederer, MD, FASN 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, FASN is a member of the following medical societies: American Association for the Advancement of Science, 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

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Society of Nephrology<br/>Received income in an amount equal to or greater than $250 from: Healthcare Quality Strategies, Inc.

Chief Editor

Vecihi Batuman, MD, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Interim Chair, Deming Department of Medicine, Tulane University School of Medicine

Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, International Society of Nephrology, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Additional Contributors

James W Lohr, MD Professor, Department of Internal Medicine, Division of Nephrology, Fellowship Program Director, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

James W Lohr, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Nephrology, Central Society for Clinical and Translational Research

Disclosure: Received research grant from: GSK<br/>Partner received salary from Alexion for employment.

Characteristics	Proximal (Type 2)	Distal (Type 1)	Type 4
Primary defect	Proximal HCO₃ - reabsorption	Diminished distal H+ secretion	Diminished ammoniagenesis
Urine pH	< 5.5 when serum HCO₃ - is low	>5.5	< 5.5
Serum HCO₃ -	>15 mEq/L	Can be < 10 mEq/L	>15 mEq/L
Fractional excretion of HCO₃ - (FEHCO₃)	>15-20% during HCO₃ - load	< 5% (can be as high as 10% in children)	< 5%
Serum K₊	Normal or mild decrease	Mild-to-severe decrease*	High
Associated features	Fanconi syndrome	...	Diabetes mellitus, renal insufficiency
Alkali therapy	High doses	Low doses	Low doses
Complications	Osteomalacia or rickets	Nephrocalcinosis, nephrolithiasis	...
*K⁺ may be high if RTA is due to volume depletion.

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