• Childhood Kidney Diseases
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• v.14 no.2
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• pp.203-209
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• 2010

Purpose : It has been a common medical practice to use prophylactic antibiotics to prevent recurrent urinary tract infections (UTI) in high risk situations such as urinary tract obstruction, vesicoureteral reflux, neurogenic bladder, or urinary stones. But sometimes, we meet difficult situation of breakthrough infections (BI) which might cause new or progressive renal scarring. The clinical characteristics of children contracting breakthrough UTI experienced in a single center were studied. Methods : The study was done retrospectively through medical records of 150 pediatric patients who had been cared in pediatric and urologic clinics of Pusan National University Hospital from Jan. 2001 till June 2006 and had prophylactic antibiotics to prevent recurrent UTI. Results : The starting age of prophylactic antibiotics of 150 patient was 1-76 months, and median age was 5 months. The BI developed 61 times in 43 patients (28.7%), 1.5 times per 100 patient-months. The BI occurred more frequently in patients with higher grade of VUR, and in the cases with abnormal DMSA scan. Co-trimoxazole was more effective than 2nd and 3rd generation cephalosporins to prevent UTI. The distribution of causative organisms was more diverse than usual UTI. The causative organisms were sensitive to the antibiotics used for prophylaxis in 29.5%, and resistant in 59.1%. After experience of BI, 40 percents of patients went to the surgical treatment including endoscopic injection of Deflux, 35% to new antibiotics for prophylaxis, 26% remain on the same antibiotics as the previous one. Conclusion : Based on our study results, preexisting renal scar might be one of the factors which should be considered in favor of early surgical interventions of VUR. Poor compliance and wrong selection of antibiotics such as cephalosporins are important underlying causes of breakthrough UTIs.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.24 no.1
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• pp.26-36
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• 2024

Lesch-Nyhan syndrome (LNS) is an Clinical symptoms can range from mild to severe depending on residual enzyme activity and genetic mutations. In Korea, 27 cases of LNS have been reported. We report the results of an 11-year comparative follow-up of two cases of children who visited because of pink diapers, one who died from LNS with no residual enzymes and one case with partial residual enzymes. Case 1: During follow-up, seizures, developmental delay, and regression were observed. The boy experienced insomnia and severe constipation. He exhibited self-mutilating behavior, a grand mal seizure, scoliosis with severe spasticity, truncal hypotonia, choreoathetoid movement, and ataxia. After prolonged emaciation, staghorn calculi, and recurrent pneumonia, the patient died suddenly at the age of 11 years. Genetic testing revealed a hemizygous HPRT1 variant (c.151C>T (p.Arg51Ter)). Uric acid level was 10.5 mg/dL (normal range: ~3.5-7.9) and HPRT activity 0.02 nmol/hr/spot (10-23.8 nmol/hr/spot). Case 2: During follow-up, the patient remained underweight. He has normal intelligence attending primary school. Self-mutilation symptoms were not observed. Regular renal ultrasonography did not reveal urolithiasis. The patient had a hemizygous HPRT1 variant (c.35A>C (p.Asp12Ala)). Uric acid level and HPRT activity were 11 mg/dL and 0.56 nmol/hr/spot. Pink diapers after the neonatal period and severe protein aversion, neurological problems, and kidney stones, differentiation for LNS is necessary. When suspected, serum uric acid levels, HPRT enzyme activity, and molecular biological tests may be helpful in predicting the prognosis of LNS.