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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 19  |  Issue : 4  |  Page : 619-623
Hyperuricosuria, an Often Overlooked Cause of Recurrent Oliguria in Children


Department of Pediatrics, Jordan University Hospital, Amman, Jordan

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   Abstract 

The association of oliguria with hyperuricosuria is often overlooked. Herein, we report an infant who since the age of five months had recurrent oliguria secondary to hyperuricosuria documented several times in the hospital. The decreased urine output co­incided with the presence of increased excretion of uric acid, which fluctuated within 24 hours as well as every few weeks. The child responded to treatment with increase in the fluid fluid intake along with the xanthine oxidase inhibitor, allopurinol. Being alert for hyperuricosuria in cases of oliguria, especially if there is history of gout or stones, may avoid performing many unnecessary investigations

Keywords: Hyperuricosuria, Recurrent oliguria, Uric acid

How to cite this article:
Akl K. Hyperuricosuria, an Often Overlooked Cause of Recurrent Oliguria in Children. Saudi J Kidney Dis Transpl 2008;19:619-23

How to cite this URL:
Akl K. Hyperuricosuria, an Often Overlooked Cause of Recurrent Oliguria in Children. Saudi J Kidney Dis Transpl [serial online] 2008 [cited 2019 Oct 20];19:619-23. Available from: http://www.sjkdt.org/text.asp?2008/19/4/619/41324

   Introduction Top


The association of oliguria with hyperuri­cosuria is often overlooked. In 2006, seven patients with idiopathic hyperuricosuria and oliguria were seen in the pediatric nephrology service at the Jordan University Hospital. Herein, we report one child that had oligu­ria documented several times in the hospital.


   Case Report Top


The patient was born after a smooth full­term pregnancy and normal vaginal deli­very. Birth weight was 2,700 grams. Her parents were not consanguinous. At the age of four months, the patient presented to the Jordan University Hospital with a one­month history of decreased urine output des­pite adequate feedings. On physical exami­nation at five months, the head circum­ference was 41 cm (25 th percentile), length 56 cm (50 th percentile), and weight was 5,300 grams (25 th percentile).

Laboratory investigations showed the fol­lowing: serum creatinine, 8.84-27.4 µmol/L (normal is 17.68-35.36 µmol/L), uric acid 77.32-285.5 µmol/L (normal is 142.75­380.67 µmol/L), urine analysis was nega­tive for protein, red and white blood cells and the urine culture was negative. Several spot urine samples and 24-hour urine col­lections for uric acid were done. Spot urine uric acid concentration corrected for glome­rular filtration rate was 2.54 mg/dl (consi­dered abnormal if > 0.53 mg/dl). 24-hour urine uric acid excretion on two occasions was 15084.7 µmol/kg and 30169.4 µmol/kg. The normal value for age is < 2203.38 µmol/kg. There was no family history of similar problems. Renal ultrasound was sus­picious of presence of a stone in the kidney but spiral computed tomography was nor­mal. The infant was admitted to the hos­pital several times because of decreased urine output, which improved after good hydration. The urine color was reddish- orange on some occasions, and this color was found to change during different hours of the same day. The patient responded to orange juice and increased water intake. However, due to non compliance, she was finally put on allopurinol 2.5 mg/kg/day. The improvement was dramatic, and she stopped having the recurrent oliguria.


   Discussion Top


Hyperuricosuria in children may be idio­pathic or secondary to gout. In addition to dietary factors, [1] conditions that lead to hyperuricemia and concomitant hyperurico­suria include malignancies such as leuke­mia and lymphoma, where there is increased purine catabolism. Tumour lysis syndrome may occur before or after chemotherapy. Hereditary disorders of purine metabolism with complete deficiency of hypoxanthine phosphoribosyl transferase (HPRT) result in the Lesch Nyhan syndrome, which consists of hyperuricemia, mental retardation, cho­reoathetosis, and self mutilation. [2] The en­zyme deficiency may be partial, when it is not associated with neurologic manifesta­tions. [3] Other enzyme deficiencies include adenine phosphoribosyl transferase (APRT) and genetic overproduction of purine as seen in patients with phosphoribosyl pyro­phosphate synthetase overactivity. [4]

Cystic fibrosis, certain drugs such as cy­closporine and tacrolimus, sickle cell di­sease, congenital heart disease with poly­cythemia and neonates may have associa­ted hyperuricosuria.

During infancy and childhood, there is increased uric acid production. [5],[6] Fractio­nal excretion of uric acid in early child­hood is higher than that in adults. [7] Before puberty, hyperuricosuria rather than hyper­uricemia may be the only clue to purine overproduction due to the high uric acid clearance. [4] When hyperuricosuria is secon­dary to hyperuricemia, the renal manifesta­tions may include acute uric acid nephro­pathy, chronic uric acid nephropathy, and nephrolithiasis. [8] Our patient had hyperuri­cosuria without hyperuricemia. The main manifestation was recurrent episodes of oliguria, which resolved with hydration, and allopurinol. The latter is a xanthine oxidase inhibitor that results in decreased serum and urinary uric acid by blocking the con­version of hypoxanthine and xanthine to uric acid. In a large series of hyperuri­cosuria in children, La Manna and asso­ciates mentioned a spectrum of signs and symptoms in 102 children with idiopathic hyperuricosuria. These included abdominal or flank pain, dysuria, gross or microsco­pic hematuria, and passage of gravel. Normal urinalysis was found in 15 patients. How­ever, there was no mention of oliguria. [9] Acute uric acid nephropathy may occur in the newborn, especially in the first few days of life due to breakdown of hema­poietic cells, or secondary to HPRT enzyme deficiency. [10]

The entity of neonatal transient renal failure with renal medullary hyperechoge­nicity is believed to be secondary to over production of uric acid. [11],[12],[13],[14] Most of the reported neonatal cases had associated hy­peruricemia. [15] Complete or partial HPRT deficiency may cause acute renal failure in infancy. [1] Batch et al, described a three month old infant with acute renal failure secondary to over-production of urate. The infant was found to have excessive urinary excretion of uric acid and partial deficien­cy of HPRT and responded to allopurinol and the renal failure resolved. [17] Our patient responded to daily orange juice because of the presence of citrate, which provided an alkali load. Odvina found out that amongst the citrus fruits, only orange juice preven­ted crystallization of calcium oxalate and uric acid. [18] One interesting aspect of our patient was the diurnal variation in uric acid excretion which was observed in the hospital and reflected as recurrent oliguria within a period of 24-hours in addition to that observed every few days to weeks. It is known that uric acid excretion may be intermittent as demonstrated in humans, [19],[20] and in Cebus monkeys where the highest plasma uric acid was before bedtime at night, and the lowest, early in the morning. [21] The 24-hour urine excretion of uric acid in our patient was always highly elevated against the random spot specimens, which were sometimes normal. Because of the circadian rhythm, random urine uric acid testing may not be accurate. [22] Furthermore, in patients with normal enzyme activity, uric acid to creatinine ratios in random urine samples do not correlate with 24-hour urine collections. [23],[24] Spot samples correc­ted for creatinine clearance (mg/dL GFR) are more accurate than the uric acid to creatinine ratio. [25],[26]

The cause of hyperuricosuria in our pa­tient remains unknown. It unlikely that she has familial juvenile hyperuricemic nephro­pathy because the serum uric acid was normal. [27] HPRT deficiency is usually asso­ciated with an elevated serum uric acid. In adenine phosphoribosyltransferase deficien­cy, the serum and urine uric acid is not elevated, but patients may present with acute renal failure. [28] Our patient could have an isolated defect in postsecretory reabsorption of uric acid, or idiopathic hyperuricosuria. Unfortunately, enzyme assays were not avai­lable. La Manna and associates emphasize the importance of family history of gout or stones in cases of hyperuricosuria. [29]


   Conclusion Top


A child with recurrent oliguria secondary to hyperuricosuria is presented. One should be on the lookout for such cases especially if there is family history of gout or uric acid stones. This will avoid doing unnece­ssary investigations.[30]

 
   References Top

1.Schlesinger N. Dietary factors and hyper­uricaemia. Curr Pharm Des 2005;11(32): 4133-8.  Back to cited text no. 1    
2.Lesch M, Nyhan WL. A familial disorder of uric acid metabolism and central ner­vous system function. Am J Med 1964; 36:561-70.  Back to cited text no. 2  [PUBMED]  
3.Cherian S, Crompton CH. Partial hypoxan­thine-guanine phosphoribosyltransferase deficiency presenting as acute renal failure. Pediatr Nephrol 2005;20(12):1811-4.  Back to cited text no. 3    
4.Cameron JS, Moro F, Simmonds HA. Gout, uric acid and purine metabolism in 4 paediatric nephrology. Pediatr Nephrol 1993;7(1):105-18.  Back to cited text no. 4    
5.Berry HK, Granger M. Uric acid excretion in infants and children. Clin Chim Acta 1971;32(3):377-83 .  Back to cited text no. 5    
6.Baldree LA, Stapleton FB. Uric acid metabolism in children. Pediatr Clin North Am 1990;37(2):391-418.  Back to cited text no. 6    
7.Stapleton FB, Linshaw M, Hassanein KH, Gruskin A. Uric acid excretion in normal children. J Pediatr 1978;92(6):911-4.  Back to cited text no. 7    
8.Conger JD. Acute uric acid nephropathy. Med Clin North Am 1990;74(4):859-71.  Back to cited text no. 8    
9.La Manna A, Polito C, Marte A, Iovene A, Di Toro R. Hyperuricosuria in children: clinical presentation and natural history. Pediatrics 2001;107(1):86-90 .  Back to cited text no. 9    
10.Wingen AM, Loffler W, Waldherr R, Scharer K. Acute renal failure in an infant with partial deficiency of hypoxanthine- guanine phosphoribosyltransferase. Proc Eur Dial Transplant Assoc Eur Ren Assoc 1985;21:751-5.  Back to cited text no. 10    
11.Makhoul IR, Soudack M, Smolkin T, et al. Neonatal transient renal failure with renal medullary hyperechogenicity: clinical and laboratory features. Pediatr Nephrol 2005; 20(7):904-9.  Back to cited text no. 11    
12.Ahmadian Y, Lewy PR. Possible urate nephropathy of the newborn infant as a12 cause of transient renal insufficiency. J Pediatr 1977;91(1):96-100.  Back to cited text no. 12    
13.Gottlieb RP, Roeloffs S, Galler-Rimm G, Gross GW. Transient renal insufficiency in the neonate related to hyperuricemia and hyperuricosuria. Chil Nephrol Urol 1991; 11(2):111-4.  Back to cited text no. 13    
14.Talosi G, Steitman K, Suranyi A, Pinter S, Horvath I, Mulugeta Z. The possible role of uric acid in renal hyperchogenicity in neonatal hypoxic acute shock. J Perinat Med 1996;24(6):693-7.  Back to cited text no. 14    
15.Shenoi A, Phadke KD. Uric acid nephro­pathy as an unusual cause of acute renal failure in a neonate. Indian Pediatr 2000; 37(3):322-4.  Back to cited text no. 15    
16.Lorentz WB, Burton BK, Trillo A, Browning MC. Failure to thrive, hyperuricemia and renal insufficiency in early infancy secondary to partial hypoxanthine-guanine phosphoribosyl transferase deficiency. J Pediatr 1984;104(1):94-7.  Back to cited text no. 16    
17.Batch JA, Riek RP, Gordon RB, Burke JR, Emmerson BT. Renal failure in infancy due to over-production of urate. Aust NZJ Med 1984;14(6):852-4.  Back to cited text no. 17    
18.Odvina CV. Comparative value of orange juice versus lemonade in reducing stone­forming risk. Clin J Am Soc Nephrol 2006;1(6):1269-74.  Back to cited text no. 18    
19.Yu KH, Luo SF, Tsai WP, Huang YY. Intermittent elevation of serum urate and 24-hour urinary uric acid excretion. Rheumatlogy 2004;43(12):1541-5.  Back to cited text no. 19    
20.Singh RK, Bansal A. Studies on circadian periodicity of serum and urinary urate in healthy Indians and renal stone formers. Prog Clin Biol Res 1987;227B:305-13.  Back to cited text no. 20  [PUBMED]  
21.Shinosaki T, Inagaki H, Nakai T, Yamashita T, Yonetani Y. Circadian rhythm of plasma uric acid and handling stress induced hyperuricemia in conscious cebus monkeys. Jpn J Pharmacol 1992;58(4): 443-50 .  Back to cited text no. 21    
22.Moriwaki Y, Yamamoto T, Takahashi S, Yamakita J, Tsutsumi Z, Hada T. Spot urine uric acid to creatinine ratio used in the estimation of uric acid excretion in primary gout. J Rheumatol 2001;28(6): 1306-10.  Back to cited text no. 22    
23.Wortmann RL, Fox IH. Limited value of uric acid to creatinine ratios in estimating uric acid excretion. Ann Intern Med 1980; 93(6):822-5.  Back to cited text no. 23    
24.Penido MG, Diniz JS, Guimaraes MM, Cardosa RB, Souto MF, Penido MG. Urinary excretion of calcium, uric acid and citrate in healthy children and adolescents. J Pediatr (Rio J) 2002;78(2):153-60.  Back to cited text no. 24    
25.Stapleton FB, Nash DA. A screening test for hyperuricosuria. J Pediatr 1983;102(1): 88-90.  Back to cited text no. 25    
26.Simkin PA. When, why, and how should we quantify 24-hour urinary uric acid excretion. J Rheumatol 2001;28(6):1207-10.  Back to cited text no. 26    
27.McBride MB, Rigden S, Haycock GB, et al. Presymptomatic detection of familial juvenile hyperuricaemic nephropathy in children. Pediatr Nephrol 1998;12(5):357-64.  Back to cited text no. 27    
28.Simmonds HA, Cameron JS, Barrat TM, Dillon MJ, Meadow SR, Trompeter RS  Back to cited text no. 28    
29.Purine enzyme defects as a cause of acute renal failure in childhood. Pediatr Nephrol 1989;3(4):433-7.  Back to cited text no. 29    
30.Polito C, La Manna A, Nappi B, Villani J, Di Toro. Ideopathic hypercalciuria and hyperuricosuria: family prevalence of nephrolithiasis. Pediatr Nephrol 2000;14 (12):1102-4.  Back to cited text no. 30    

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Correspondence Address:
Kamal Akl
Department of Pediatrics, Jordan University Hospital, P.O. Box 831373, Amman 11183
Jordan
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PMID: 18580023

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    Abstract
    Introduction
    Case Report
    Discussion
    Conclusion
    References
 

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