Xanthinuria Follow-up

Author: Sahar Fathallah-Shaykh, MD; Chief Editor: Craig B Langman, MD more...

Updated: Jun 28, 2011

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Further Inpatient Care
Further Outpatient Care
Complications
Prognosis
Patient Education
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Further Inpatient Care

Inpatient care may be necessary for the secondary complications of pyelonephritis, obstructive urolithiasis, or acute renal failure.

Further Outpatient Care

Monitor frequency of symptoms, renal function, and passage of stones.

Ensure consistent high intake of fluid to maintain dilute urine.

Monitor purine intake.

Complications

Complications of xanthinuria may include the following:

Urolithiasis
Crystal nephropathy
Renal failure
Obstructive uropathy
Urinary tract infection
Hematuria
Myopathy
Arthropathy
Arthritis

Prognosis

Prognosis depends on the degree of renal injury from crystal nephropathy, urinary obstruction, and/or pyelonephritis.

Patient Education

Advise patients regarding the importance of the following:

Maintaining a dilute urine
Avoiding dehydration
Intervening early for conditions that may lead to dehydration
Avoiding high-purine foods
Providing proper home therapy for renal colic

Contributor Information and Disclosures

Author

Sahar Fathallah-Shaykh, MD Assistant Professor in Pediatric Nephrology, University of Alabama at Birmingham School of Medicine; Consulting Staff, Division of Pediatric Nephrology, Medical Director of Pediatric Dialysis Unit, Children's Health System

Sahar Fathallah-Shaykh, MD is a member of the following medical societies: American Society of Nephrology and American Society of Pediatric Nephrology

Disclosure: emedecine Honoraria Other

Coauthor(s)

Steven C Diven, MD Medical Director of Pediatric Dialysis Unit, Assistant Professor, Department of Pediatrics, University of Texas Medical Branch at Galveston

Steven C Diven, MD is a member of the following medical societies: National Kidney Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard Neiberger, MD, PhD Director of Pediatric Renal Stone Disease Clinic, Associate Professor, Department of Pediatrics, Division of Nephrology, University of Florida College of Medicine and Shands Hospital

Richard Neiberger, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Medical Association, American Society of Nephrology, American Society of Pediatric Nephrology, Christian Medical & Dental Society, Florida Medical Association, International Society for Peritoneal Dialysis, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Shock Society, Sigma Xi, Southern Medical Association, Southern Society for Pediatric Research, and Southwest Pediatric Nephrology Study Group

Disclosure: The Osler Institute Honoraria Speaking and teaching

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Luther Travis, MD Professor Emeritus, Departments of Pediatrics, Nephrology and Diabetes, University of Texas Medical Branch School of Medicine

Luther Travis, MD is a member of the following medical societies: Alpha Omega Alpha, American Federation for Medical Research, International Society of Nephrology, and Texas Pediatric Society

Disclosure: Nothing to disclose.

Howard Trachtman, MD Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine

Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, Children's Memorial Hospital

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology

Disclosure: Merck Grant/research funds None; NIH Grant/research funds None; Raptor Pharmaceuticals, Inc Grant/research funds None; Alexion Pharmaceuticals, Inc. Grant/research funds None

References

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Carpenter TO, Lebowitz RL, Nelson D, Bauer S. Hereditary xanthinuria presenting in infancy with nephrolithiasis. J Pediatr. Aug 1986;109(2):307-9. [Medline].
Fildes RD. Hereditary xanthinuria with severe urolithiasis occurring in infancy as renal tubular acidosis and hypercalciuria. J Pediatr. Aug 1989;115(2):277-80. [Medline].
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van Gennip AH, Mandel H, Stroomer LEM. Effects of allopurinol on the xanthinuria in a patient with molybdenum cofactor deficiency. In: Purine and Pyrimidine Metabolism in Man. Vol 8. 1995:375-8.
Zannolli R, Micheli V, Mazzei MA, et al. Hereditary xanthinuria type II associated with mental delay, autism, cortical renal cysts, nephrocalcinosis, osteopenia, and hair and teeth defects. J Med Genet. Nov 2003;40(11):e121. [Medline].

Purine metabolic pathway.

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