• Childhood Kidney Diseases
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• v.25 no.2
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• pp.78-83
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• 2021

Background: Uric acid levels in urine are measured using urine specimens 24 hours or by uric acid glomerular filtration rate (UAGFR) with spot urine, which additionally requires a blood sample. This study aimed to investigate whether urinary uric acid creatinine ratio (UUACr) obtained by spot urine alone could be recognized as a substitute for UAGFR value, and hyperuricosuria can be screened by UUACr. UUACr is known to vary with age and regional differences. This study focused on the reference value of each value in Korean young populations. Method: We enrolled Korean subjects 1-20 years with normal kidney function, from a single hospital, classified into 5 age groups, 1-5 years, 6-8 years, 9-12 years, 13-15 years, and 16-20 years. We checked spot urine uric acid, creatinine and serum uric acid, creatinine levels on the same day from February 2014 to December 2018. We measured the average of UAGFR and UUACr in each groups. The UUACr cut-off value of the upper 2 standard deviation (SD) of UAGFR were taken. Results: The upper 2 SD of UUACr (mg/mg) and UAGFR (mg/dL) were determined in all age groups. UUACr decreased with grown up (P=0.000), but UAGFR were not statistically different among the groups. UUACr and UAGFR were not significantly different by gender. UUACr and UAGFR were positively correlated; UUACr cut-off value of upper 2 SD UAGFR (0.54 mg/dL) was 0.65 mg/mg in total age. Conclusions: UUACr could potentially be used to screen for hyperuricosuria.

• The Korean Journal of Food And Nutrition
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• v.7 no.4
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• pp.307-317
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• 1994

The study analyzes relationship between Increasing tendency of urinary stone patients and changes of food intake through statistical data during recent 10 years(1982∼1991) in Korea. In four university hospitals, the number of urinary stone patients increased 2.5 times during the period. Study statistics showed sex ratio of male and female patient as 2.1 : 1.The types of the urinary stone of each patient were broad and mostly Ca and others(85.8%), and Ca oxalate stones were 65.2%. The incidence of patients with hypercalciuria and hyperuricosuria was observed in the Ca stone patients than in normal person. The same period showed increase of total monthly household expenditure as 3.2 times, food as 2.5 times and medical expenses as 2.4 times : The food expenditure decreased relatively In the expenditure composition. Daily hood consumption (g/person) increased as 3 times in meat, 4 times in milk, 2.4 times in seaweeds. Daily total Ca nutrition consumption was undulant and became 1.1 times during the same period. Daily food supply (g/person) was increased 1.9 times in meat, 1.4 times in spinach. However, anchovy, laver, brown seaweed, herring and makerel showed undulant tendency. Relationship (correlation coefficient) between the increase of urinary stone patients and of food intake were studied as follows. In the case of food intake, the relative index was 0.923 in seaweeds, 0.865 in meat and 0.대27 In milk And In food supply, the relative index was 0.855 in purine group which constitute meat, anchovy, herring and makerel. The relative index of Ca components which constitute anchovy, laver, brown seaweed and shrimp was 0.270. In the case of nutrition intake, the relative index was medium in protein(r: 0.443) and In animal protein (r: 0.488) but was negative in Ca(r: -0.028).

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.35 no.6
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• pp.459-461
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• 2009

Polycythemia vera is one type of myeloproliferative disorder which occurs due to the clonal proliferation of hematopoietic stem cell related to the production of leukocyte and megakaryocyte which produces a little less than erythrocyte. Polycythemia vera has a peak incidence in the sixth decade of life with males affected slightly more frequently than females. Vasquez first described polycythemia vera as an autonomous erythrocytosis in 1892, and a further description, delineation of the disease process and a complete course outline were made in 1899, 1903 and 1938, respectively. Symptoms include pruritus, tinnitus, vertigo, gastrointestinal (GI) pain, and bleeding gums. Hyperuricemia and hyperuricosuria are present in about 40% of these patients. Complications are hemorrhage, thrombosis, post-polycythemic myeloid metaplasia, and leukemic transformation. In case of surgery, complications such as hemorrahge and thrombosis are highly likable to happen. We report a case of preoperative and postoperative of a 63-year-old male, who was diagnosed as oral cavity cancer in the mouth floor, with known history of hypertension and polycythemia vera. We considered that conservative management would be an advisable treatment for patients with uncontrolled systemic disease.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.505-509
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• 2003

Lesch-Nyhan syndrome is an X-linked recessive disorder characterized by hyperuricemia, choreoathetosis, spasticity, mental retardation, and compulsive, self-injurious behavior. This disorder results from a complete deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyl transferase(HPRT). We report here on a case of Lesch-Nyhan syndrome in a 1-year, 7-month-old male who presented with frequent vomiting, failure to thrive, and developmental delay. The diagnostic work-up revealed hyperuricemia, hyperuricosuria, and medullary nephrolithiasis. The HPRT activity in the erythrocytes was undetectable with a biochemical assay. We also identified de novo mutation which was a deletion of the 649th base, adenosine, in HPRT gene(649delA) by analysis of cDNA using RT-PCR technique coupled with direct sequencing.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.86-90
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• 2003

An 8-month-old male infant presented with persistent, gross, orange-colored crystals in his urine. His physical and neurological development was normal. Laboratory study showed hyperuricemia, hyperuricosuria and urate crystaluria. He was determined to have partial hypoxanthine-guanine phosphoribosyl transferase(HPRT) deficiency. The molecular genetic analysis revealed a missense mutation in the patient's HPRT gene. By sequencing the patient's cDNA, we identified an A-to-G transition at nucleotide 239, resulting in the replacement of Aspartate with Glycine at amino acid 80 in the HPRT. To our knowledge, this mutation has not previously been reported. Our patient is now being placed on allopurinol therapy, and has had no problem since. Partial HPRT deficiency has been known to cause recurrent acute renal failure without the phenotypic features of Lesch-Nyhan syndrome. Therefore, we think that early diagnosis and treatment are very crucial in preventing acute renal failure.

• The Korean Journal of Food And Nutrition
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• v.10 no.1
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• pp.44-52
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• 1997

Nephrolithiasis is the most common disorder of the urinary tract in hospitalized patients, more frequently increased in 30~50 years of age, more common in males than in females, prior right stone to left side, and than upper ureteral stone is found in cultural country, while lower ureteral stone is increased in uncultural country. Stone components are classified as calcium oxalate, calcium phosphate, magnesium ammonium phosphate, uric acid, cystine, and their mixed stone, respectively. According to the pathophysiology of urinary stones, supersaturation/crystalization of inorganic salt concentration in urine, organic matrix, inhibitor deficiency, and epitaxy theory could be based on the stone formation. Not only hypercalciuria, hyperparathyroidism, hyperoxaluria, hyperuricosuria, and cystinuria, but also renal tubular acidosis, hypervitaminosis D, and peptic ulcer, are significantly associated with nephrolithiasis. In this study upper ureteral stone component were analyzed with chemical analysis, infrared spectrum, and image analyzer from K43 patient wit erosive gastritis. As the results, mixed stone of calcium oxalate dihydrate and calcium phosphate apatite was identified, the values of clinical test in blood and urine maintained normal revels. The relapsing urinary stone from K43 have no correlation between factors for stone formation reported early, also have no evidence for risk from erosive gastritis.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.3
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• pp.532-538
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• 2008

Lesch-Nyhan syndrome is a rare X-linked recessively inherited disorder, caused by complete absence or decrease in activity of hypoxanthine guanine phosphoribosyl transferase(HPRT), an enzyme involved in purine metabolism. This enzyme deficiency gives rise to nephropathy symptoms, such as hyperuricosuria and hyperuricemia by excessive uric acid production and neuropathy symptoms, such as mental retardation, choreoathetosis and self mutilation behavior. Patients with Lesch-Nyhan syndrome have tendency to bite their lip, tongue and finger. In severe cases, partial or even total amputation of tongue or finger occur. Self-inflicted bites are often complicated by secondary infection to the injured site as well as pain. Furthermore tissue loss by biting results in esthetic problems. The dental management of self mutilation includes treatment with appliances such as soft mouth guard or lip bumper, extraction of all the teeth, and orthognathic surgery. We report a case of a 13 year-old boy with Lesch-Nyhan syndrome, who severely injured himself on his tongue. At first, conservative treatment using soft mouth guard was considered, but it could not prevent trauma on his tongue. Therefore, extraction of the lower anterior and posterior teeth was carried out.