• Childhood Kidney Diseases
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• v.3 no.1
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• pp.48-56
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• 1999

Purpose : This multicenter collaboratory study was conducted to see the therapeutic efficacy and side effect of cyclosporine A (Cipol-$N^{(R)}$, Chong Kun Dang) on children with idiopathic nephrotic syndrome who experienced frequently relapsing (FR), steroid dependent (SD), or steroid resistant (SR) pattern. Patients and methods : Thirty-nine children with SD/FR NS and 3 children with SR NS were enrolled in the study. After induction of remission (SD/FR NS) with steroid or after 4 weeks of steroid therapy (SR NS), cyclosporine A was started in a dose of 4-5 mg/Kg/day in two divided dose and steroid (prednisolone or equivalent dose of deflazacort) was tapered slowly. During 16 weeks of study period, monthly check up of physical examination and various laboratory tests including BUN, creatinine, Ccr and cyclosporine blood level were done. Results : Out of 39 children with SD/FR NS, 35($89.7\%$) maintained sustained remission and at 4 weeks after therapy, values of serum protein, albumin, cholesterol, and 24 hours urinary protein excretion showed normal values. Two out of 3 children with SR NS showed and sustained remission with cyclosporine A therapy. Side reaction to cyclosporine A therapy showed hypertrichosis in 8 cases and hyperuricemia in 5 cases. However, other laboratory tests including CBC, liver profile, BUN, creatinine and GFR (creatinine clearance utilizing 24 hour urine) did not show any abnormalities during the 16 weeks of study period. Conclusion : Cyclosporine A (Cipoi-$N^{(R)}$ Chong Kun Dang) can be utilized quite effectively on children with SD/FR or SR NS and further trial of cyclosporine A on long-term basis (1-2 year period) is needed to determine it's efficacy and side effect (especially nephrotoxicity) of long-term administration of cyclosporine A.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.182-191
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• 2006

Purpose : We studied the degree of changes in antibiotic sensitivity toward causative organisms, prevalence and clinical manifestations of extended-spectrum $\beta$-lactamase(ESBL)-producers of urinary tract infection(UTI) for a period of three years. This serves to provide useful information in selecting adequate drugs for the treatment of UTI. Methods : We recruited 137 patients who grew more than $10^5$ CFU/mL in their urine culture among 250 patients who visited and were admitted to Handong University's Sunlin Hospital for UTI treatment from January 2003 to December 2005. We retrospectively analyzed the data from the medical records. Results : The common pathogenic organisms were Escherichia coli(65.0%), Klepsiella pneumoniae(14.0%), Enterococcus faecalis(5.8%) and Proteus vulgaris(2.9%) in consecutive order. The prevalence of ESBL-producers among isolated E. coli and K. pneumoniae was 4.5%(4 cases) and 14.3%(2 cases), respectively. The antibiotic sensitivity rates of E. coli were relatively high to amikacin(100%), imipenem(100%), ceftriaxone(95.5%) and tobramycin(91.4%) while relatively low to TMP/SMZ(55.4%), ampicillin/sulbactam(29.4%) and ampicillin(24.2%). Conclusion : The use of ampicillin, ampicillin/sulbactam and TMP/SMZ, which have been the first choices in the treatment of UTI, should be reconsidered due to the low sensitivity rates towards these antibiotics. Due to the high incidence and antibiotic tolerance of ESBL that might have risen from the development of new antibiotics and increased antibiotic use, it is necessary to consider changing the standard antibiotics that have been used in the treatment of UTI.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.174-177
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• 2014

Isolated methylmalonic acidemia (MMA) is a group of autosomal recessive inborn errors of metabolism caused by impaired activity of methylmalonyl-coenzyme A mutase (MCM). Mutations in the gene encoding MCM (MUT ) is the most common cause of isolated MMA. In this report, we identify an asymptomatic 15 days old female who had elevated C3-acylcarnitine (C3) in the newborn screening. Her serum homocysteine was normal and urine methylmalonic acid was increased that suggested isolated MMA. She was maintained on a low-protein diet and carnitine supplementation. At 3 months of age, she was still asymptomatic and had normal growth. We analyzed MUT gene mutations. Two heterozygote mutations in the MUT gene were identified including c.323G>A and c.1672+2T>C (IVS8(+2)T>C. Among these, c.1672+2T>C (IVS8(+2)T>C) have not been described previously.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.24-33
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• 2016

Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.

• Clinical and Experimental Pediatrics
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• v.48 no.5
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• pp.527-533
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• 2005

Purpose : Long-term administration of anticonvulsants in children with epilepsy may cause short stature, hypocalcemia and low bone mineral density. This study was performed for the early detection of abnormal bone metabolism in children with epilepsy on taking anticonvulsants. Methods : Thirty children aged 5 to 16 years who were diagnosed with epilepsy were enrolled in this study. All had taken anticonvulsants for more than one year. Bone mineral density of lumbar vertebra was measured by dual-energy X-ray absorptiometry. Serum calcium, phosphorous, alkaline phosphatase, 25-hydroxycholecalciferol[$25(OH)D_3$], parathyroid hormone, and urine deoxypyridinoline were measured as biochemical bone markers. Bone age and body mass index were also calculated. Results : Bone minreal density, body mass index, bone age, and height were significantly decreased in two female patients who had taken two antiepileptic drugs for more than four years and they also had chronic diseases such as cerebral palsy with microcephaly, encephalomalacia, and microcephaly with atrial septal defect. Bone mineral density had significant positive correlations with body mass index(P<0.01) and bone age(P<0.01). Conclusion : This study showed chronic medication of anticonvulsants in children may cause low bone mineral density and short stature. Bone age and body mass index could be the important surrogate markers to find the population at risk. More studies, including a large study population and long term cohort study, will be required.

• Clinical and Experimental Pediatrics
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• v.48 no.12
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• pp.1354-1361
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• 2005

Purpose : Rotavirus is an enteric pathogen that affects millions of children globally each year. But no specific therapy is available for the management of rotavirus diarrhea. Due to the clear need to define improved modality for treatment of rotavirus diarrhea, we evaluated the efficacy of antirotavirus IgY in the treatment of infants and children with gastroenteritis. Methods : First, the amount of viral particle in the stools of thirteen patients(seven were given IgY, 6 placebo) infected by rotavirus were evaluated for 3 days with the quantitative RT-PCR method. Second, 36 children with known rotavirus infection identified by ELISA or semi-quantitative RT-PCR were evaluated. We gave 5 g anti-rotavirus egg yolk daily in two equally divided doses for 3 days to two groups(an 18 IgY group and an 18 placebo group), respectively after parenteral consent. Daily vomiting frequency, stool frequency, oral intake and urine output were monitored for 3 days, and electrolyte and blood chemistry were checked at the first and third days. Results : First, in the placebo group, the amount of virus particles increased daily, but in the IgY group it decreased daily. Second, when IgY and placebos were given to children infected with rotavirus, diarrhea on the third day decreased significantly in the IgY group, compared with the placebo group. Conclusion : Treatment with antirotavirus immunoglobulin from immunized chicken's egg resulted in a decrease in the amount of viral particles in stools and diarrhea frequency in children. These results suggest that anti-rotavirus IgY is effective in the treatment of rotavirus gastroenteritis.

• Pediatric Infection and Vaccine
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• v.5 no.2
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• pp.230-238
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• 1998

Purpose : Worldwidely, the incidence of the hepatitis A has been markedly decreased in the past years. Also in Korea, there has been only a few sporadic cases without evidence of epidemics since the mid 1980s. However, the incidence of the hepatitis A in young adults and childen has been in trends of increasing since 1996. So, we retrospectively performed the study on the epidemiolgical changes and the clinical feature of hepatitis A in childen, living in Kyung-gi province, since 1988 to 1998. Methods : The four affiliated hospitals of the Catholic University of Korea, Our lady of Mercy's hospital, Holy family hospital, Eui-Jung-bu St. Mary's hospital, and St. Vincent hospital, were enrolled in this study. We conducted statistical analysis on the incidence of hepatitis A since 1988 to 1998, concerning with the period (monthly, annually), age, sex and related epidemiological property, by reviewing the hospital records of the patients with hepatitis A. We simultaneously performed the study concerning with the clinical characteristics of hepatitis A. Results : 1) During the study period, 46 children was diagnosed as hepatitis A, and among them 28 children (60.1%) developed sporadically in the 1998. And sex ratio was 1.2(male):1(female). 2) Most of the patients developed between March and July, and hepatitits A were mainly developed in the patients above the 10 year old ages(37 patients; 80.4%). 3) The socioeconomic and educational status of the patient's parents was almostly below the middle and low class. Although, we could not find the etiological factors in most cases, but we found the evidence of interfamilial transmission in one family. 4) The patients complained the symptoms of nausea and vomiting, jaundice, poor appetite, mild fever, fatigueness, abdominal pain, URI symptom, dyspepsia, dark urine, headache and diarrhea in order, and findings of jaundice, hepatomegaly, RUQ tenderness, splenomegaly and LLQ tenderness in order were seen. 5) All patients showed abnormal findings of the liver function tests, and the pattern of cholestatic hepatitis were seen in most cases, but these findings were normalized within the 2~4 weeks. The findings of the gall bladder wall thickening, hepatomegaly, splenomegaly and ascites were seen on abodominal sonogram. 6) The prognosis of all patients were good without complications, except in one case who had the episode of transient recuurence. And the mean hospitalization dates were 11.1 days. Conclusion : We found that the incidence of hepatitis A showed the increasing trend, and peaked in the 1998 in Kyung-gi province children. And hepatitis A mainly developed in children above the 10 year old ages. The scioeconomic and educational status of patient's family was almostly below the middle class. All of them showed abnormal liver function, and clinically cholestatic hepatitis features were seen in most cases. But, the prognosis was excellent without complication in all cases.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.23-30
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• 2006

Purpose: The estimation of fluid deficit is crucial to the proper management of dehydrated children. Without well-documented serial weights on the same scale, the estimation of any given child's fluid deficit is imprecise and dependent largely on subjective clinical criteria. Despite the abundance of literature on clinical and laboratory evaluation of dehydration, few studies have focused on serum uric acid. So, we examined the usefulness of scrum uric acid in gastroenteritis patients with dehydration. Methods: Medical records of 90 gastroenteritis patients were retrospectively reviewed. By the body weight loss, we classified patients with mild, moderate, and severe dehydration groups. We studied the relevance of laboratory data (BUN, creatinine, serum bicarbonate, glucose, urine specific gravity, and uric acid) with dehydration. Results: 54 children (60%) were dehydrated mildly, 24 (26%) dehydrated and moderately, and 12 (14%) dehydrated severely. Statistically significant differences in BUN, creatinine, serum bicarbonate, glucose, and urine specific gravity could not be observed. But there was significant relationship between uric acid and the degree of dehydration. Data analysis suggested that the level of 7.0 mg/dL is the best cut-off value for predicting the development of moderate or severe dehydration. At this cut-off value, the sensitivity and specificity were 66.6% and 87.1%. Conclusion: Our study supports that the measurement of serum uric acid with traditional scale is useful for predicting the development of dehydration. But, in order 10 be used as the indicator for proper treatment at an earlier stage, further validation about serum uric acid is necessary.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.18-26
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• 2006

Purpose : Empirical antimicrobial treatment is indicated before bacteriological results are available for young children with febrile UTI to minimize renal scarring. To ensure appropriate therapy, knowledge of the prevalence of causative organisms and their susceptibility patterns to antimicrobials is mandatory. We performed a retrospective analysis investigating the local prevalence and resistance patterns of uropathogens, primarily E. coli, isolated from community-acquired UTIs. Methods : A total of 103 positive urine cultures from children with febrile UTI collected at Bundang CHA General Hospital from February 2004 to February 2005 were analyzed. Inclusion criteria were fever higher than $37.5^{\circ}C$, significant bacteriuria with single strain growth of at least 10s colony forming units/mL urine, and leukocyturia >5/HPF. Results : E. coli(89.3%) was the leading uropathogen followed by Enterococcus spp.(3.9%) Klebsiella spp.(2.9%), Citrobctcter spp.(1.9%) and Enterobacter spp.(1.9%). E. coli strains revealed a low proportion of antimicrobial susceptibility to ampicillin(AMP; 27.2%) ampicillinsulbactam(AMS; 34.8%) and trimethoprim-sulfamethoxazole(SXT; 65.2%). Susceptibility patterns to cephalosporins were as follows; cefazolin(1st generation; 91.3%), cefoxitin(2nd; 100%), ceftriaxone(3rd; 97.8%) and cefepime(4th; 97.8%). Three E. coli isolates produced ex tended - spectrum beta-lactamase(ESBL). Conclusion : Empirical treatment with AMP, AMS and SXT, which are commonly used in pediatric clinics, is not recommended for childhood UTI due to high incidence of resistance. The high level of susceptibility to cephalosporins makes these drugs reasonable alternatives. However the emergence of ESBL-producers, even though they are quite few, may have an impact on cephalosporin treatment in the future. (J Korean Soc Pediatr Nephrol 2006;10:18-26)

• Journal of Nutrition and Health
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• v.28 no.8
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• pp.749-758
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• 1995

In this study, the food intake, feces and urine of 16 primary school age boys and girls were collected and intake and excretion of sodium and calcium were measured. The boys and girls were 8-12 years old and measurement continued for four weeks during which they maintained their normal living pattern and body weight. Each boy's and girl's daily intake and excretion of sodium and calcium were measured and apparent digestibility and balance were also studied. The results were as follows. 1) Mean daily intake of sodium was 8.52$\pm$0.38g for the boys and 7.31$\pm$0.44g for the girls. The mean value in males was significantly higher than that in females(p<0.05). Mean daily in take of calcium was 411.0$\pm$16.0mg for the boys and 356.5$\pm$15.4mg for the girls. The mean value in males was significantly higher than that in females(p<0.01). 2) Mean daily fecal loss and apparent digestibility of sodium was 0.32$\pm$0.04g and 96% for the boys and 0.52$\pm$0.07g and 93% for the girls. The fecal loss mean value in males was significantly lower than that in females(p<0.05). Mean daily fecal loss and apparent digestibility of calcium was 299.8$\pm$8.3mg and 29% for the boys and 194.1$\pm$14.3mg and 46% for the girls. The fecal loss mean value in males was significantly higer than that in females(p<0.01). 3) Mean daily urinary loss of sodium was 6.55$\pm$0.50g and showed the positive balance of 1.65g for the boys and 5.67$\pm$0.20g and showed the positive balance of 1.12g for the girls. The urinary loss mean values of the two groups were not significantly different. Mean daily urinary loss of calcium was 42.8$\pm$5.1mg and showed the positive balance of 79.4mg for the boys and 25.0$\pm$1.64mg and showed the positive balance of 137.4mg for the girls. The urinary loss mean value in males was significantly higer than that in females(p<0.01).