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Hyperuricosuria and Gouty Diathesis Clinical Presentation

  • Author: Bijan Shekarriz, MD; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
 
Updated: Apr 20, 2015
 

History

Acute phase

Pain symptoms are as follows:

  • As with many other stone types, patients with uric acid–related urinary calculi may present with acute renal or ureteral colic.
  • A typical episode occurs suddenly during the night. The episode is abrupt and does not resolve with rest or change in position. In contrast to someone with an acute abdomen who is stationary, patients experiencing acute renal colic frequently move in unusual positions in an effort to relieve their severe pain.
  • Some patients may experience a gradual onset of pain, resulting in a dull chronic ache in the flank region. The location of pain varies depending on stone location and may shift from the flank to the anterior abdominal wall, groin, and, eventually, to the ipsilateral testicle or labia.
  • When the stone is lodged in the ureterovesical junction, patients frequently report marked urinary frequency and urgency.
  • The severity of pain is often inversely proportional to the size of the stone. Large renal staghorn calculi rarely result in acute renal colic, as is observed with smaller ureteral stones.
  • The degree and type of pain is not specific to any particular stone composition.

Gastrointestinal symptoms include the following:

  • Nausea and vomiting are frequently associated with urolithiasis.
  • Patients may report altered bowel habits, including diarrhea or constipation.

Urinary symptoms include the following:

  • Patients with ureteral stones near the bladder often report dysuria, frequency, and urgency. Furthermore, gross hematuria may be observed. Eighty-five percent of patients present with either gross or microscopic hematuria.
  • Some patients may present with an associated urinary tract infection. Rare, severe, gas-forming infections may result in pneumaturia.
  • The vast majority of ureteral stones that pass into the bladder transit the urethra uneventfully. A ureteral stone passed into the bladder rarely causes urinary obstruction; these persons usually report an interrupted stream. Unlike in cats, in whom stones frequently become inspissated in the urethra, resulting in renal failure and/or death, stones rarely become obstructed in the human urethra.

Chronic phase

A history of gout and associated joint disease are important clues in discerning the etiology. The use of probenecid (a known uricosuric agent) in patients with gout may result in hyperuricosuria and increase the risk of uric acid stone formation. Uricosuric agents such as probenecid specifically increase urinary uric acid levels to help decrease hyperuricemia and to reduce gout attacks. When they cause hyperuricosuria or uric acid calculi, alternate therapies for the hyperuricemia should be used.

Myeloproliferative disorders, especially in children, may result in hyperuricosuria and associated urinary stones. Furthermore, a massive increase in the endogenous purine pool due to tumor necrosis may result in severe hyperuricosuria, crystalluria, and acute urinary obstruction during chemotherapy for myeloproliferative disorders. In these patients, the rate of uric acid stone formation is approximately 40%, which is much higher than in patients with gout.

Obtaining a dietary history is important to help elucidate iatrogenic causes of hyperuricosuria, including an excessive intake of purine-rich foods and excessive weight loss, which result in a catabolic state and increased uric acid production.[3]

A hereditary form of hyperuricemia and hyperuricosuria is Lesch-Nyhan syndrome, which may be associated with urinary calculi. These patients have a deficiency in the enzyme hypoxanthine-guanine phosphoribosyl-transferase. This enzyme catalyzes the salvage pathway of purines and is responsible for the conversion of hypoxanthine to inosinic acid and guanine to guanylic acid. If the enzyme is deficient, low levels of guanylic and inosinic acid occur with a subsequent increase in de novo purine synthesis because these nucleotides modulate purine synthesis by feedback inhibition.

Long-standing malaise and lethargy may be an associated symptom of urinary obstruction with or without infection.

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Physical

Costovertebral angle tenderness is common during acute renal colic. Abdominal distention and tenderness secondary to ileus may also occur. In cases with associated urinary tract infection, fever is common.

After resolution of the acute stone event, patients may be asymptomatic or may present with physical findings related to the underlying disease. For example, patients with gout may present with chronic joint changes (ie, tophi) due to gouty arthritis.

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Causes

Hyperuricosuric calcium nephrolithiasis is characterized by calcium oxalate or calcium phosphate stones in persons with hyperuricosuria. The hyperuricosuria is most commonly due to an excessively purine-rich diet; however, hyperuricosuria may be related to overproduction of uric acid in as many as 30% of these patients. This may represent a latent form of gout. In contrast to calcium-based stones, uric acid stones form in an acidic environment with a urinary pH that is always below 5.5.

The solubility of uric acid depends on three factors: urinary pH, uric acid concentration, and urinary volume. The causes of uric acid stones can be categorized according to those three factors.

Acidic urine may result from any of the following:

  • Gouty diathesis
  • Chronic diarrhea
  • Inflammatory bowel disease
  • Exercise/dehydration
  • Familial
  • Obesity, diabetes, and the metabolic syndrome - Strong association with low urinary pH [4]

Hyperuricosuria may result from any of the following:

  • Gouty diathesis
  • An excessively purine-rich diet
  • Inborn errors of metabolism (ie, hypoxanthine-guanine phosphoribosyl-transferase deficiency, phosphoribosylpyrophosphate synthetase overactivity, glucose-6-phosphatase synthetase deficiency
  • Myeloproliferative disorders (eg, leukemia, hemolytic anemia, neoplasia)
  • Medications
  • Chemotherapy

Low urinary volume may result from any of the following:

  • GI disorders
  • Strenuous exercise/dehydration
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Contributor Information and Disclosures
Author

Bijan Shekarriz, MD Director, Laparoscopy and Minimally Invasive Surgery, Associate Professor of Urology, Department of Urology, State University of New York Upstate Medical University

Bijan Shekarriz, MD is a member of the following medical societies: American Urological Association, Endourological Society

Disclosure: Nothing to disclose.

Coauthor(s)

Marshall L Stoller, MD Professor and Vice Chairman, Medical Director of Urinary Stone Center, Department of Urology, University of California, San Francisco, School of Medicine

Marshall L Stoller, MD is a member of the following medical societies: American Urological Association

Disclosure: Nothing to disclose.

Brian H Eisner, MD Instructor in Surgery, Department of Urology, Massachusetts General Hospital-Harvard Medical School; Fellow in Endourology, Department of Urology, University of California

Brian H Eisner, MD is a member of the following medical societies: American Urological Association, Endourological Society

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.

Chief Editor

Bradley Fields Schwartz, DO, FACS Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine

Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, Society of Laparoendoscopic Surgeons, Society of University Urologists, Association of Military Osteopathic Physicians and Surgeons, American Urological Association, Endourological Society

Disclosure: Nothing to disclose.

Additional Contributors

Allen Donald Seftel, MD Professor of Urology, University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School; Head, Division of Urology, Director, Urology Residency Training Program, Cooper University Hospital

Allen Donald Seftel, MD is a member of the following medical societies: American Urological Association

Disclosure: Received consulting fee from lilly for consulting; Received consulting fee from abbott for consulting; Received consulting fee from auxilium for consulting; Received consulting fee from actient for consulting; Received honoraria from journal of urology for board membership; Received consulting fee from endo for consulting.

References
  1. Sakhaee K, Maalouf NM. Metabolic syndrome and uric acid nephrolithiasis. Semin Nephrol. 2008 Mar. 28(2):174-80. [Medline].

  2. Ferraro PM, Robertson WG, Johri N, Nair A, Gambaro G, Shavit L, et al. A London experience 1995-2012: demographic, dietary and biochemical characteristics of a large adult cohort of patients with renal stone disease. QJM. 2014 Dec 17. [Medline].

  3. Del Valle EE, Negri AL, Spivacow FR, Rosende G, Forrester M, Pinduli I. Metabolic diagnosis in stone formers in relation to body mass index. Urol Res. 2011 Jun 10. [Medline].

  4. Mehta TH, Goldfarb DS. Uric acid stones and hyperuricosuria. Adv Chronic Kidney Dis. 2012 Nov. 19(6):413-8. [Medline].

  5. Leng S, Shiung M, Ai S, Qu M, Vrtiska TJ, Grant KL, et al. Feasibility of discriminating uric acid from non-uric acid renal stones using consecutive spatially registered low- and high-energy scans obtained on a conventional CT scanner. AJR Am J Roentgenol. 2015 Jan. 204(1):92-7. [Medline]. [Full Text].

  6. Asplin JR. Uric acid stones. Semin Nephrol. 1996 Sep. 16(5):412-24. [Medline].

  7. Bernardo NO, Smith AD. Chemolysis of urinary calculi. Urol Clin North Am. 2000 May. 27(2):355-65. [Medline].

  8. Cameron MA, Sakhaee K. Uric acid nephrolithiasis. Urol Clin North Am. 2007 Aug. 34(3):335-46. [Medline].

  9. Coe FL, Kavalach AG. Hypercalciuria and hyperuricosuria in patients with calcium nephrolithiasis. N Engl J Med. 1974 Dec 19. 291(25):1344-50. [Medline].

  10. Low RK, Stoller ML. Uric acid-related nephrolithiasis. Urol Clin North Am. 1997 Feb. 24(1):135-48. [Medline].

  11. Moe OW, Abate N, Sakhaee K. Pathophysiology of uric acid nephrolithiasis. Endocrinol Metab Clin North Am. 2002 Dec. 31(4):895-914. [Medline].

  12. Pak CY. Medical management of urinary stone disease. Nephron Clin Pract. 2004. 98(2):c49-53. [Medline].

  13. Pak CY, Moe OW, Sakhaee K, Peterson RD, Poindexter JR. Physicochemical metabolic characteristics for calcium oxalate stone formation in patients with gouty diathesis. J Urol. 2005 May. 173(5):1606-9. [Medline].

  14. Pak CY, Poindexter JR, Peterson RD, Koska J, Sakhaee K. Biochemical distinction between hyperuricosuric calcium urolithiasis and gouty diathesis. Urology. 2002 Nov. 60(5):789-94. [Medline].

  15. Pak CY, Sakhaee K, Moe O, Preminger GM, Poindexter JR, Peterson RD, et al. Biochemical profile of stone-forming patients with diabetes mellitus. Urology. 2003 Mar. 61(3):523-7. [Medline].

  16. Shekarriz B, Stoller ML. Uric acid nephrolithiasis: current concepts and controversies. J Urol. 2002 Oct. 168(4 Pt 1):1307-14. [Medline].

  17. Steele TH. Hyperuricemic nephropathies. Nephron. 1999. 81 Suppl 1:45-9. [Medline].

  18. Stoller ML. Gout and stones or stones and gout?. J Urol. 1995 Nov. 154(5):1670. [Medline].

  19. Xu H, Zisman AL, Coe FL, Worcester EM. Kidney stones: an update on current pharmacological management and future directions. Expert Opin Pharmacother. 2013 Mar. 14(4):435-47. [Medline].

 
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CT scan demonstrating right partial staghorn uric acid calculus. Uric acid stones appear dense on CT scan and radiolucent on kidneys, ureters, and bladder (KUB) imaging (not shown).
Follow-up CT scan of patient in the image above (ie, with partial staghorn uric acid calculus) 1 year later. This patient was treated with oral urinary alkalinization with sodium bicarbonate. Note only a small residual fragment is present (right image).
 
 
 
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