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Uric Acid Nephrolithiasis

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161. Metabolic Acidosis (Diagnosis)

has to pump protons (intracellular pH 7.5 to luminal pH 5) becomes too steep. A maximally acidified urine, even with a volume of 3 L, would thus contain a mere 30 µEq of free H + . Instead, more than 99.9% of the H + load is excreted buffered by the weak bases NH 3 or phosphate. Titratable acidity The amount of secreted H + that is buffered by filtered weak acids is called titratable acidity. Phosphate as HPO 4 2- is the main buffer in this system, but other urine buffers include uric acid (...) , 2018 Author: Christie P Thomas, MBBS, FRCP, FASN, FAHA; Chief Editor: Vecihi Batuman, MD, FASN Share Email Print Feedback Close Sections Sections Metabolic Acidosis Overview Practice Essentials Metabolic acidosis is a clinical disturbance characterized by an increase in plasma acidity. Metabolic acidosis should be considered a sign of an underlying disease process. Identification of this underlying condition is essential to initiate appropriate therapy. (See Etiology, , , and .) Understanding

2014 eMedicine.com

162. Medullary Sponge Kidney (Diagnosis)

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

163. Lesch-Nyhan Syndrome (Diagnosis)

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

164. Extracorporeal Shockwave Lithotripsy (Diagnosis)

for detecting and tracking calcified and otherwise radio-opaque stones, both in the kidney and the ureter. Conversely, it is usually poor for localizing radiolucent stones (eg, uric acid stones). To compensate for this shortcoming, intravenous contrast can be introduced or (more commonly) cannulation of the ureter with a catheter and retrograde instillation of contrast (ie retrograde pyelography) can be performed. Ultrasonographic localization allows for visualization of both radiopaque and radiolucent (...) , Gutierrez-Aceves J, et al. Lower pole I: a prospective randomized trial of extracorporeal shock wave lithotripsy and percutaneous nephrostolithotomy for lower pole nephrolithiasis-initial results. J Urol . 2001 Dec. 166(6):2072-80. . Anagnostou T, Tolley D. Management of ureteric stones. Eur Urol . 2004 Jun. 45(6):714-21. . Auge BK, Preminger GM. Update on shock wave lithotripsy technology. Curr Opin Urol . 2002 Jul. 12(4):287-90. . Chacko J, Moore M, Sankey N, Chandhoke PS. Does a slower treatment rate

2014 eMedicine.com

165. Hyperoxaluria (Treatment)

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

166. Pregnancy and Urolithiasis (Treatment)

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

167. Hyperuricosuria and Gouty Diathesis (Treatment)

are urinary alkalinization (ie, pH 6.5-7.0), hydration (ie, urinary output 1500-2000 mL/d), and allopurinol (ie, patients with hyperuricosuric calcium nephrolithiasis) to decrease serum and urinary uric acid levels. The authors have observed impressive dissolution of large uric acid stones (staghorn) with oral alkalinization (see the images below). These measures are effective for dissolving existing uric acid stones and for stone prophylaxis. CT scan demonstrating right partial staghorn uric acid (...) . Metabolic syndrome and uric acid nephrolithiasis. Semin Nephrol . 2008 Mar. 28(2):174-80. . Mehta TH, Goldfarb DS. Uric acid stones and hyperuricosuria. Adv Chronic Kidney Dis . 2012 Nov. 19(6):413-8. . Wan KS, Liu CK, Ko MC, Lee WK, Huang CS. Nephrolithiasis among male patients with newly diagnosed gout. Hong Kong Med J . 2016 Dec. 22 (6):534-7. . . Abu-Ghanem Y, Kleinmann N, Winkler HZ, Zilberman DE. Nephrolithiasis in Israel: Epidemiological Characteristics of Return Patients in a Tertiary Care

2014 eMedicine.com

168. Hyperuricemia (Treatment)

-hour urine uric acid excretion of less than 800 mg in 24 hours, no history of nephrolithiasis, and good renal function (creatinine clearance >80 mL/min). The starting dose for probenecid is 250 mg twice a day, which can be increased gradually to a maximum daily dose of 3 g/d. Some degree of gastrointestinal irritation is experienced by approximately 2% of patients. Allopurinol is the most widely used antihyperuricemic agent. The major metabolite of allopurinol is oxypurinol, and both allopurinol (...) and oxypurinol are competitive inhibitors of the enzyme xanthine oxidase. The ideal candidates for allopurinol treatment are as follows: Uric acid overproducers (24-h urinary uric acid excretion >800 mg on general diet or >600 mg on a purine-restricted diet) Patients with renal insufficiency, nephrolithiasis, or tophaceous gout Patients at risk for developing uric acid nephropathy Although allopurinol can be used in almost any hyperuricemic state, the above-mentioned conditions are more specific indications

2014 eMedicine.com

169. Ureteroscopy (Treatment)

, Champion PC, Wollin TA, Denstedt JD. Holmium:YAG lithotripsy of uric acid calculi. J Urol . 1998 Dec. 160(6 Pt 1):2130-2. . Wollin TA, Teichman JM, Rogenes VJ, Razvi HA, Denstedt JD, Grasso M. Holmium:YAG lithotripsy in children. J Urol . 1999 Nov. 162(5):1717-20. . Bus MT, de Bruin DM, Faber DJ, Kamphuis GM, Zondervan PJ, Laguna Pes MP, et al. Optical diagnostics for upper urinary tract urothelial cancer: technology, thresholds, and clinical applications. J Endourol . 2015 Feb. 29 (2):113-23. . Hao YC (...) , Assimos DG, Dretler SP, Kahn RI, Lingeman JE, et al. Nephrolithiasis Clinical Guidelines Panel summary report on the management of staghorn calculi. The American Urological Association Nephrolithiasis Clinical Guidelines Panel. J Urol . 1994 Jun. 151(6):1648-51. . Segura JW, Preminger GM, Assimos DG, Dretler SP, Kahn RI, Lingeman JE, et al. Ureteral Stones Clinical Guidelines Panel summary report on the management of ureteral calculi. The American Urological Association. J Urol . 1997 Nov. 158(5):1915

2014 eMedicine.com

170. Polycystic Kidney Disease (Overview)

ADPKD Nodular hepatomegaly: In severe polycystic liver disease Rarely, symptoms related to renal failure (eg, pallor, uremic fetor, dry skin, edema) Testing Routine laboratory studies include the following: Serum chemistry profile, including calcium and phosphorus CBC count from cysts Urinalysis Urine culture Uric acid determination Intact PTH assay Genetic testing may be performed, in which the major indication is for genetic screening in young adults with negative ultrasonographic findings who (...) pyelonephritis, infected cysts, perinephric abscess) Nephrolithiasis and renal colic Rarely, a coincidental hypernephroma See for more detail. Diagnosis Examination in patients with ADPKD may demonstrate the following: Hypertension: One of the most common early manifestations of ADPKD, [ , ] in which increased diastolic BP is the rule; clinical course in ADPKD is usually more severe early on, then becomes less problematic as the renal insufficiency progresses Palpable, bilateral flank masses: In advanced

2014 eMedicine.com

171. Papillary Necrosis (Overview)

diagnoses of flank pain or hematuria includes a plethora of conditions with infectious, neoplastic, congenital, metabolic, and hematogenous sources. Intraluminal and extraluminal lesions may result in ureteral obstruction. If acute ureteral obstruction manifests as hematuria, colic, and flank pain, consider that nephrogenic calculi may have embolized to the ureter. Approximately 15% of all calculi, particularly uric acid stones, are not radiopaque (ie, visible on radiographs); therefore, the examining (...) clinician needs to perform further imaging studies for an accurate identification. CT scanning is an excellent imaging modality for identifying all calculi (including uric acid stones), [ ] except for the rare indinavir (Crixivan) crystal concretions that form in the ureters of patients with HIV infection who are taking this protease inhibitor. If calculi are not seen on a CT scan, consider the possibility of an obstructing urothelial tumor; cytology and endoscopic procedures help to determine

2014 eMedicine.com

172. Pregnancy and Urolithiasis (Overview)

glycoproteins inhibit oxalate stone formation (eg, nephrocalcin). This explains why pregnancy is not associated with a net increase in the rate of stone formation relative to nonpregnant patients. The net effect of these physiologic changes is a stable relative supersaturation of important ions such as calcium oxalate, urate, and phosphate. Uric acid stone formation The formation of uric acid stones requires continued and excessive oversaturation of urine with uric acid or extreme aciduria. Dehydration (...) , , and significantly acidic urine contribute to uric acid supersaturation and stone formation. However, during gestation, urine tends to be more alkaline, probably because of greater intrinsic purine use and increased urinary citrate excretion. Thus, renal units are generally protected against uric acid stone formation during pregnancy. Calcium oxalate and calcium phosphate stone formation Stone composition in pregnant women is predominantly (74%) calcium phosphate, with only 26% being calcium oxalate

2014 eMedicine.com

173. Percutaneous Endourology (Overview)

are needed to demonstrate the clear clinical benefit of tubeless PCNL. Perfusion chemolysis to dissolve and clear certain renal stones This therapy is now largely adjunctive in nature owing to the advent of new lithotripsy techniques and oral chemolytic agents (eg, potassium citrate). Chemolysis of uric acid and cystine stones is particularly effective. Bicarbonate solution produces adequate alkalinization to dissolve uric acid debris while a combination of tris- (hydroxymethylene)-aminomethane (THAM (...) dissolution depends on the stone burden and composition. Irrigations may dissolve in a few days (ie, with uric acid) or may require weeks (ie, with cystine or struvite). As a rule, 1 cm per month can be expected. The irrigants that may be used include potassium or sodium bicarbonate for uric acid stones; D-penicillamine, N -acetylcysteine, or tromethamine-E solution for cystine stones; or Suby solution (G or M) or hemiacidrin (Renacidin) for struvite or apatite stones. Renacidin irrigant is used

2014 eMedicine.com

174. Gout (Overview)

(11):692-4. . Puig JG, Michan AD, Jimenez ML, et al. Female gout. 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 (...) , blurred vision, anterior uveitis (rare), scleritis Complications of gout include the following: Severe degenerative arthritis Secondary infections Urate or uric acid nephropathy Increased susceptibility to infection Urate nephropathy Renal stones Nerve or spinal cord impingement Fractures in joints with tophaceous gout See for more detail. Diagnosis Studies that may be helpful include the following: Joint aspiration and synovial fluid analysis Serum uric acid measurement (though hyperuricemia

2014 eMedicine.com

175. Gout (Overview)

(11):692-4. . Puig JG, Michan AD, Jimenez ML, et al. Female gout. 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 (...) , blurred vision, anterior uveitis (rare), scleritis Complications of gout include the following: Severe degenerative arthritis Secondary infections Urate or uric acid nephropathy Increased susceptibility to infection Urate nephropathy Renal stones Nerve or spinal cord impingement Fractures in joints with tophaceous gout See for more detail. Diagnosis Studies that may be helpful include the following: Joint aspiration and synovial fluid analysis Serum uric acid measurement (though hyperuricemia

2014 eMedicine.com

176. Ureteroscopy (Overview)

treatment of ureteropelvic junction obstruction. J Urol . 1998 Nov. 160(5):1643-6; discussion 1646-7. . Teichman JM, Champion PC, Wollin TA, Denstedt JD. Holmium:YAG lithotripsy of uric acid calculi. J Urol . 1998 Dec. 160(6 Pt 1):2130-2. . Wollin TA, Teichman JM, Rogenes VJ, Razvi HA, Denstedt JD, Grasso M. Holmium:YAG lithotripsy in children. J Urol . 1999 Nov. 162(5):1717-20. . Bus MT, de Bruin DM, Faber DJ, Kamphuis GM, Zondervan PJ, Laguna Pes MP, et al. Optical diagnostics for upper urinary tract (...) patterns in urologic management of upper-tract transitional-cell carcinoma. J Endourol . 2005 Apr. 19(3):366-71. . Segura JW, Preminger GM, Assimos DG, Dretler SP, Kahn RI, Lingeman JE, et al. Nephrolithiasis Clinical Guidelines Panel summary report on the management of staghorn calculi. The American Urological Association Nephrolithiasis Clinical Guidelines Panel. J Urol . 1994 Jun. 151(6):1648-51. . Segura JW, Preminger GM, Assimos DG, Dretler SP, Kahn RI, Lingeman JE, et al. Ureteral Stones Clinical

2014 eMedicine.com

177. Struvite and Staghorn Calculi (Overview)

of complete staghorn calculi were metabolic in origin, consisting of calcium phosphate (55%), uric acid (21%), calcium oxalate (14%), or cystine (10%). [ ] Unlike other urinary stones that commonly produce symptoms (eg, renal colic) that necessitate intervention, treatment of struvite stones often occurs in patients without classic signs of nephrolithiasis; this is because large staghorn calculi may not cause acute renal or ureteral dilatation and resultant pain. Previous Next: Relevant Anatomy (...) cause significant morbidity and mortality if left untreated; therefore, large struvite stones must typically be removed. Interestingly, an article investigating the structural analysis of renal calculi in northern India reported that over 90% of staghorn stones were composed of oxalates. [ ] In a study from southern Thailand, the most common component of staghorn calculi was uric acid; struvite was found in only 11.6% of cases. [ ] A US study, published in 2011, reported that slightly over half

2014 eMedicine.com

178. 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

179. Metabolic Acidosis (Overview)

has to pump protons (intracellular pH 7.5 to luminal pH 5) becomes too steep. A maximally acidified urine, even with a volume of 3 L, would thus contain a mere 30 µEq of free H + . Instead, more than 99.9% of the H + load is excreted buffered by the weak bases NH 3 or phosphate. Titratable acidity The amount of secreted H + that is buffered by filtered weak acids is called titratable acidity. Phosphate as HPO 4 2- is the main buffer in this system, but other urine buffers include uric acid (...) Author: Christie P Thomas, MBBS, FRCP, FASN, FAHA; Chief Editor: Vecihi Batuman, MD, FASN Share Email Print Feedback Close Sections Sections Metabolic Acidosis Overview Practice Essentials Metabolic acidosis is a clinical disturbance characterized by an increase in plasma acidity. Metabolic acidosis should be considered a sign of an underlying disease process. Identification of this underlying condition is essential to initiate appropriate therapy. (See Etiology, , , and .) Understanding

2014 eMedicine.com

180. 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

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