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Uric Acid Nephrolithiasis

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181. Medullary Sponge Kidney (Overview)

or cuboidal epithelium and rarely by transitional epithelium, which is caused by the effects of calculi. Closed cysts are lined with atrophic epithelium. The rest of the kidney usually is normal, unless or renal obstruction complicates the course of the disease. Etiology Most cases of medullary sponge kidney are sporadic. Theories suggest that dilatation of a collecting duct may occur, caused by occlusion by uric acid during fetal life or resulting from tubular obstruction due to calcium oxalate calculi (...) , and the prevalence may be as much as 1 case per 1000 population in urology clinics. In addition, medullary sponge kidney has been identified in 12-20% of patients who form calcium stones. [ ] Approximately 0.5% of patients undergoing intravenous urography are estimated to have medullary sponge kidney, while another 1% have papillary blush. No autopsy series have examined the prevalence of medullary sponge kidney specifically. In patients with nephrolithiasis, up to 20% may have mild degrees of medullary sponge

2014 eMedicine.com

182. Hyperchloremic Acidosis (Overview)

, urinary loss of glucose, amino acids, phosphate, uric acid, and other organic anions, such as citrate, can also occur (Fanconi syndrome). A distinctive feature of type II pRTA is that it is nonprogressing, and when the serum bicarbonate is reduced to approximately 15 mEq/L, a new transport maximum for bicarbonate is established and the proximal tubule is able to reabsorb all of the filtered bicarbonate. A fractional excretion of bicarbonate (FE[HCO 3 - ]) greater than 15% when the plasma bicarbonate (...) , metabolic acidosis, which may be primary or secondary to a respiratory alkalosis. Loss of bicarbonate stores through diarrhea or renal tubular wasting leads to a metabolic acidosis state characterized by increased plasma chloride concentration and decreased plasma bicarbonate concentration. Primary metabolic acidoses that occur as a result of a marked increase in endogenous acid production (eg, lactic or keto acids) or progressive accumulation of endogenous acids when excretion is impaired by renal

2014 eMedicine.com

183. Hypercalciuria (Overview)

in calcium-stone disease. The reason for this is unclear but may reflect urinary volume and optimal levels of other urinary metabolites, such as oxalate, uric acid, sodium, phosphate, citrate, urinary volume, and serum vitamin D-3. In some cases, the vitamin D-3 level has been suggested to be responsible for determining which patients with hyperparathyroidism actually develop kidney stones. This apparently reasonable hypothesis remains unproved, however, and the current evidence suggests that vitamin-D (...) or less per day, whereas in women the usual daily limit is only 250 mg. These reference values were created using large numbers of people (not calcium kidney-stone formers) to establish a reference range. The most likely reason for the discrepancy is that men are generally larger physically than women and have a correspondingly larger amount of material, such as calcium and uric acid, to excrete. Clearly, stone development occurs when the chemical conditions are favorable, regardless of what any

2014 eMedicine.com

184. Hyperoxaluria (Overview)

to have no substantially beneficial role and acts as a metabolic end-product, much like uric acid. If not for oxalate’s high affinity for calcium and the low solubility of calcium oxalate, oxalate and oxalate metabolism would be of little interest. Urinary oxalate is the single strongest chemical promoter of kidney stone formation. Ounce for ounce, it is roughly 15-20 times more potent than excess urinary calcium. Daily oxalate intake in humans is usually 80-120 mg/d; it can range from 44-350 mg/d (...) excretion levels or concentration between geriatric and younger cohorts of individuals with calcium oxalate stones have been found. [ , ] Primary hyperoxaluria type I presents primarily as a pediatric disease, with symptoms first appearing before age 4 in nearly half of the patients. [ ] Kidney stones are 3 times more common in men than in women, but the reason for this difference is not always clear. Different reference ranges of several urinary metabolites (eg, uric acid, calcium, oxalate) illustrate

2014 eMedicine.com

185. Inflammatory Bowel Disease (Overview)

to malabsorbed fatty acids, oxalate combines with sodium to form sodium oxalate, which is soluble and is absorbed in the colon (enteric hyperoxaluria). The development of calcium oxalate stones in Crohn disease requires an intact colon to absorb oxalate. Patients with ileostomies generally do not develop calcium oxalate stones, but they may develop uric acid or mixed stones. [ ] Previous Next: Etiology Three characteristics define the etiology of inflammatory bowel disease (IBD): (1) genetic predisposition (...) , or vice versa) Pharmacotherapy The following medications may be used in patients with IBD: 5-Aminosalicylic acid derivatives (eg, sulfasalazine, mesalamine, balsalazide, olsalazine) Antibiotics (eg, metronidazole, ciprofloxacin, rifaximin) Corticosteroid agents (eg, hydrocortisone, prednisone, methylprednisolone, prednisolone, budesonide, dexamethasone) Immunosuppressant agents (eg, azathioprine, 6-mercaptopurine, methotrexate, cyclosporine) Tumor necrosis factor inhibitors (eg, infliximab, adalimumab

2014 eMedicine.com

186. Hypocitraturia (Overview)

crystallization inhibition, increasing the risk of calcium nephrolithiasis. It also may play a role in uric acid solubility and uric acid stone formation. Urinary calculi secondary to hypocitraturia are typically composed of some hydroxyapatite (calcium phosphate), along with calcium oxalate. The following are causes of hypocitraturic calcium nephrolithiasis: Distal renal tubular acidosis (RTA) Chronic diarrheal syndrome Thiazide diuretic or acetazolamide administration Diet high in animal protein Strenuous (...) and gouty diathesis are conditions that involve excessive serum uric acid, which often is associated with nephrolithiasis. Controlling the uric acid problem and its potential contribution to stone formation may involve limiting purine intake, controlling hepatic uric acid production, monitoring urinary uric acid levels, and checking or altering urinary acidity. Active urinary tract infection UTI with bacteria that degrade citrate lowers urinary citrate levels. Chronic Kidney Disease (CKD) As glomerular

2014 eMedicine.com

187. Hyperuricosuria and Gouty Diathesis (Overview)

of uric acid calculi. Such high levels may be due to either excess dietary intake of purine-rich foods or endogenous uric acid overproduction. Hyperuricosuria may be associated with . In contrast, the term gouty diathesis describes the formation of urinary stones in persons with primary . These patients may present with other manifestations of gout (eg, gouty arthritis). Uric acid–related nephrolithiasis may involve pure calcium stones, uric acid stones, or a combination of both. Furthermore, uric (...) acid stones may develop in persons with normal urinary and serum levels of uric acid. Unlike most other forms of urolithiasis, medical therapy is an integral part of management of uric acid stones. Therefore, an understanding of the pathophysiology of uric acid–related nephrolithiasis is important for a cost-effective treatment approach. In patients with hyperuricosuria, dietary purine restriction is the initial treatment. If that proves insufficient, pharmacologic intervention with allopurinol may

2014 eMedicine.com

188. Lesch-Nyhan Syndrome (Overview)

was cloned and sequenced by Friedmann and colleagues in 1985. Lesch-Nyhan disease is a genetic disorder associated with 3 major clinical elements: overproduction of uric acid, neurologic disability, and behavioral problems. [ ] The overproduction of uric acid is associated with . If left untreated, it can produce nephrolithiasis with renal failure, gouty arthritis, and solid subcutaneous deposits known as tophi. The neurologic disability is dominated by dystonia but may include choreoathetosis, ballismus (...) have overproduction of uric acid and its consequences alone. These patients are identified by demonstrating HPRT deficiency or a mutation in the HPRT gene. Collectively, they are referred to as Lesch-Nyhan variants. Treatment of the condition is limited. Allopurinol is useful to control the overproduction of uric acid and reduces the risk of nephrolithiasis and gouty arthritis. Few treatments have proven consistently helpful for the neurologic or behavioral difficulties. Motor disability is managed

2014 eMedicine.com

189. Diabetic Nephropathy (Overview)

for uric acid stones. J Am Soc Nephrol . 2006 Jul. 17 (7):2026-33. . . Zelnick LR, Weiss NS, Kestenbaum BR, et al. Diabetes and CKD in the United States Population, 2009-2014. Clin J Am Soc Nephrol . 2017 Dec 7. 12 (12):1984-90. . Cheung CY, Ma MKM, Chak WL, Tang SCW. Cancer risk in patients with diabetic nephropathy: a retrospective cohort study in Hong Kong. Medicine (Baltimore) . 2017 Sep. 96 (38):e8077. . . Fan JZ, Wang R. Non-diabetic renal diseases in patients with type 2 diabetes: a single (...) to accumulation of extracellular matrix. The image below is a simple schema for the pathogenesis of diabetic nephropathy. Simple schema for the pathogenesis of diabetic nephropathy. Light microscopy findings show an increase in the solid spaces of the tuft, most frequently observed as coarse branching of solid (positive periodic-acid Schiff reaction) material (diffuse diabetic glomerulopathy). Large acellular accumulations also may be observed within these areas. These are circular on section and are known

2014 eMedicine.com

190. Diabetic Nephropathy (Treatment)

:CD006763. . Daudon M, Jungers P. Diabetes and nephrolithiasis. Curr Diab Rep . 2007 Dec. 7 (6):443-8. . Daudon M, Traxer O, Conort P, Lacour B, Jungers P. Type 2 diabetes increases the risk for uric acid stones. J Am Soc Nephrol . 2006 Jul. 17 (7):2026-33. . . Zelnick LR, Weiss NS, Kestenbaum BR, et al. Diabetes and CKD in the United States Population, 2009-2014. Clin J Am Soc Nephrol . 2017 Dec 7. 12 (12):1984-90. . Cheung CY, Ma MKM, Chak WL, Tang SCW. Cancer risk in patients with diabetic (...) acidosis is more common in patients with type 2 DM, especially those with moderate renal insufficiency, and is associated with decreased ammoniagenesis. It has also been noted that kidney stones may be more common in patients with type 2 DM, as well as metabolic syndrome. [ ] The increased risk of stone disease is linked to insulin resistance, which, as a result of impaired ammoniagenesis, leads to a reduced urine pH. A low urine pH primarily favors uric acid stone formation; studies have found

2014 eMedicine.com

191. Medullary Sponge Kidney (Treatment)

in excess of 2L. A 24-hour urine collection for potential kidney stone risk factors (eg, calcium, citrate, uric acid, magnesium, sodium, oxalate, phosphate) can be very helpful in treating the metabolic factors contributing to nephrolithiasis. [ , ] Patients with medullary sponge kidney and distal RTA Adjust the dosage and timing of potassium citrate supplementation to increase the urinary pH to a maximum of 7.0-7.2. Overalkalinization can lead to calcium phosphate precipitation and stone formation (...) . Obtaining periodic urinalysis and abdominal radiographs is recommended, although guidelines for the frequency of radiologic surveillance in asymptomatic adults are unclear. Asymptomatic children with medullary sponge kidney In asymptomatic children with medullary sponge kidney, conduct regular surveillance for Wilms tumor and other abdominal tumors. Patients with medullary sponge kidney and recurrent nephrolithiasis Patients are advised to drink plenty of fluids in order to have a daily urinary output

2014 eMedicine.com

192. Gout (Treatment)

, with its uricosuric effect, may reduce the serum concentration of uric acid. In one study, 500 mg/day for 2 months reduced uric acid by a mean of 0.5 mg/dL in patients without gout. [ ] However, gout patients appear to be less responsive to such a low dose of ascorbate. Vitamin C treatment should be avoided in patients with nephrolithiasis, urate nephropathy, or cystinuria. Kobylecki et al reported that each 10 µmol/L higher plasma vitamin C level was associated with a 2.3 µmol/L lower plasma urate (...) . Clinical spectrum and uric acid metabolism. Arch Intern Med . 1991 Apr. 151(4):726-32. . Meyers OL, Monteagudo FS. Gout in females: an analysis of 92 patients. Clin Exp Rheumatol . 1985 Apr-Jun. 3(2):105-9. . Macfarlane DG, Dieppe PA. Diuretic-induced gout in elderly women. Br J Rheumatol . 1985 May. 24(2):155-7. . Kramer HM, Curhan G. The association between gout and nephrolithiasis: the National Health and Nutrition Examination Survey III, 1988-1994. Am J Kidney Dis . 2002 Jul. 40(1):37-42

2014 eMedicine.com

193. Gout (Treatment)

, with its uricosuric effect, may reduce the serum concentration of uric acid. In one study, 500 mg/day for 2 months reduced uric acid by a mean of 0.5 mg/dL in patients without gout. [ ] However, gout patients appear to be less responsive to such a low dose of ascorbate. Vitamin C treatment should be avoided in patients with nephrolithiasis, urate nephropathy, or cystinuria. Kobylecki et al reported that each 10 µmol/L higher plasma vitamin C level was associated with a 2.3 µmol/L lower plasma urate (...) . Clinical spectrum and uric acid metabolism. Arch Intern Med . 1991 Apr. 151(4):726-32. . Meyers OL, Monteagudo FS. Gout in females: an analysis of 92 patients. Clin Exp Rheumatol . 1985 Apr-Jun. 3(2):105-9. . Macfarlane DG, Dieppe PA. Diuretic-induced gout in elderly women. Br J Rheumatol . 1985 May. 24(2):155-7. . Kramer HM, Curhan G. The association between gout and nephrolithiasis: the National Health and Nutrition Examination Survey III, 1988-1994. Am J Kidney Dis . 2002 Jul. 40(1):37-42

2014 eMedicine.com

194. Extracorporeal Shockwave Lithotripsy (Treatment)

as the cutoff. [ ] Stone composition The density and ability of a stone to resist ESWL is based in part on the composition of the stone. Stones composed of calcium oxalate dihydrate, magnesium ammonium phosphate, or uric acid tend to be softer and to fragment more easily with ESWL. Stones composed of calcium oxalate monohydrate or cystine, on the other hand, are less susceptible to ESWL. To a degree, this can be predicted with CT scanning by measuring the radio-opacity of stones. A recent retrospective (...) study showed that ESWL monotherapy is more likely to be effective against stones with a Hounsfield units [HU] < 815 Hounsfield units [HU]) than those with a higher radio-opacity. [ ] In addition, certain radiolucent stones (uric acid, indinavir [Crixivan]) are difficult to visualize on fluoroscopy and therefore require either ultrasonography-guided localization or the addition of retrograde or intravenous contrast to localize a calculus. Stone location See the list below: Lower-pole calculi

2014 eMedicine.com

195. Lesch-Nyhan Syndrome (Treatment)

=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTE4MTM1Ni10cmVhdG1lbnQ= processing > Lesch-Nyhan Disease Treatment & Management Updated: Dec 14, 2015 Author: H A Jinnah, MD, PhD; Chief Editor: Amy Kao, MD Share Email Print Feedback Close Sections Sections Lesch-Nyhan Disease Treatment Approach Considerations In Lesch-Nyhan disease, the gross overproduction of uric acid must be controlled to prevent the development of urologic or articular complications. The goal is to maintain uric acid levels in the normal range. A common error is to attempt to suppress uric (...) acid below normal levels, but this approach risks the development of oxypurine stones instead. The control of uric acid requires 2 important components: (1) inhibiting the metabolism of hypoxanthine and xanthine to uric acid with allopurinol and (2) generous hydration. Generous hydration at all times is essential. Hydration should be increased during periods of increased fluid loss, such as a febrile illness or recurrent emesis. In patients with Lesch-Nyhan disease, hospital admissions should

2014 eMedicine.com

196. Light Chain-Associated Renal Disorders (Treatment)

Uric acid released following chemotherapy may precipitate in the tubules and may precipitate acute renal failure. Hence, pretreating patients undergoing chemotherapy with allopurinol and diuresis is important. Nephrolithiasis and urinary tract infections Treat nephrolithiasis and urinary tract infections promptly. Hyperviscosity This can be treated with plasmapheresis. Myeloma cell infiltration Treat the underlying process. Previous Next: Treatment of Complications Renal failure Institute dialysis

2014 eMedicine.com

197. Hypercalciuria (Treatment)

in calcium-stone disease. The reason for this is unclear but may reflect urinary volume and optimal levels of other urinary metabolites, such as oxalate, uric acid, sodium, phosphate, citrate, urinary volume, and serum vitamin D-3. In some cases, the vitamin D-3 level has been suggested to be responsible for determining which patients with hyperparathyroidism actually develop kidney stones. This apparently reasonable hypothesis remains unproved, however, and the current evidence suggests that vitamin-D (...) or less per day, whereas in women the usual daily limit is only 250 mg. These reference values were created using large numbers of people (not calcium kidney-stone formers) to establish a reference range. The most likely reason for the discrepancy is that men are generally larger physically than women and have a correspondingly larger amount of material, such as calcium and uric acid, to excrete. Clearly, stone development occurs when the chemical conditions are favorable, regardless of what any

2014 eMedicine.com

198. Acute Renal Failure (Overview)

-abdominal hypertension - Tense ascites Renal vein thrombosis Diseases causing urinary obstruction from the level of the renal tubules to the urethra include the following: Tubular obstruction from crystals - Eg, uric acid, calcium oxalate, acyclovir, sulfonamide, methotrexate, myeloma light chains Ureteral obstruction - Retroperitoneal tumor, retroperitoneal fibrosis (methysergide, propranolol, hydralazine), urolithiasis, or papillary necrosis Urethral obstruction - Benign prostatic hypertrophy (...) Pericardial friction rub: Uremic pericarditis Increased jugulovenous distention, rales, S 3 : Heart failure Abdomen The following signs of AKI may be discovered during an abdominal examination: Pulsatile mass or bruit: Atheroemboli Abdominal or costovertebral angle tenderness: Nephrolithiasis, papillary necrosis, renal artery thrombosis, renal vein thrombosis Pelvic, rectal masses; prostatic hypertrophy; distended bladder: Urinary obstruction Limb ischemia, edema: Rhabdomyolysis Pulmonary system Pulmonary

2014 eMedicine.com

199. Medullary Sponge Kidney (Follow-up)

in excess of 2L. A 24-hour urine collection for potential kidney stone risk factors (eg, calcium, citrate, uric acid, magnesium, sodium, oxalate, phosphate) can be very helpful in treating the metabolic factors contributing to nephrolithiasis. [ , ] Patients with medullary sponge kidney and distal RTA Adjust the dosage and timing of potassium citrate supplementation to increase the urinary pH to a maximum of 7.0-7.2. Overalkalinization can lead to calcium phosphate precipitation and stone formation (...) . Obtaining periodic urinalysis and abdominal radiographs is recommended, although guidelines for the frequency of radiologic surveillance in asymptomatic adults are unclear. Asymptomatic children with medullary sponge kidney In asymptomatic children with medullary sponge kidney, conduct regular surveillance for Wilms tumor and other abdominal tumors. Patients with medullary sponge kidney and recurrent nephrolithiasis Patients are advised to drink plenty of fluids in order to have a daily urinary output

2014 eMedicine.com

200. Pregnancy and Urolithiasis (Follow-up)

with the normal extracellular volume expansion of pregnancy. The safest method of medically managing calcium stone disease during pregnancy is to increase fluid intake and to avoid excessive calcium intake (including calcium-fortified prenatal vitamins). Typically, calcium intake should not exceed 1000-1200 mg/d during pregnancy. This treatment may prevent or reduce the risk of urolithiasis during pregnancy. Sodium intake and protein consumption should also be curtailed. Uric acid disease In uric acid stone (...) disease, xanthine oxidase inhibitors (eg, allopurinol) prevent uric acid stone formation by inhibiting the final step in human purine metabolism, thereby decreasing both serum and urinary uric acid levels. However, use of xanthine oxidase inhibitors is contraindicated during pregnancy because the effects of the drug on the fetus are unknown. Alternative treatment modalities for uric acid stones during pregnancy include increasing fluid intake, limiting dietary purine intake, and increasing urinary

2014 eMedicine.com

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