• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.64-71
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• 1999

Purpose : The protective effects of dietary protein on the progression of renal failure were studied in subtotally nephrectomized rats. Methods : Treatment groups were as follows; 5/6 nephrectomy and a normal protein ($18.5\%$) diet (NP); 5/6 nephrectomy and a low protein ($6\%$) diet (LP): 5/6 nephrectomy, a normal protein diet and converting enzyme inhibitor, enalapril (NPE): 5/6 nephrectomy, a low protein diet and enalapril (LPE). Both diets were isocaloric and had the same phosphorus content. Proteinuria, remnant kidney weight, mesangial matrix expansion score and glomerular volume were assessed at 4, 12 and 16 weeks after renal ablation. Results : LP and NP developed progressive hypertension. Eight weeks after surgery, LPE and NPE controlled hypertension. LP, LPE, and NPE had significantly less proteinuria than NP at 16 weeks (P<0.05). Kidney weight in LP were markedly less enlarged than NP (P<0.05). There was no difference in kidney weight between LPE and NPE. At 12 and 16 weeks the mesangial matrix expansion score was significantly less in LP, LPE, and NPE compared to NP (P<0.05). At 12 and 16 weeks mean glomerular volume was significantly less in LP compared to NP (P<0.05). At 12 and 16 weeks mean glomerular volume in LPE was significantly less compared to NPE. Conclusion : Dietary protein restriction afforded considerable protection from renal injury in the rat remnant kidney model. During the enalapril treatment, there was no additional protective effect of dietary protein restriction against the development of renal lesions.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.27 no.3
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• pp.239-249
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• 2001

The purpose of this study is to evaluate the relationship of the factors which could be influenced by orthognathic surgery especillay SSRO. We measured the amounts of the maximum opening, lateral movements, maximum velocity and pattern of mandibular path during the opening and closing of mandible at the following times ; preoperative, 1 month after operation, 6 months after operation respectively using MKG. And the results were compared according to the categorized subgroups. Following results were obtained : 1. The change of the amounts of mandibular lateral movement and maximum opening velocity were statistically different between male and female (p<0.05), but the others were not. 2. According to the method of operation, there was no difference in the change of the mandibular movements between the group of SSRO and SSRO plus LeFort I osteotomy (p>0.05). 3. According to the amounts of mandibular movement, the recovery of left lateral movement of the group of $6{\sim}10mm$ was better than the other groups (p<0.05). 4. In the frontal pattern of the opening and closing of the mandible, the complex deflected type (F5), simple deflected type (F4), complex deviated type (F3), simple deviated type (F2), straight type (F1) were obtained in order at the time of preoperative, simple deflected type, simple deviated type, complex deviated type, straight type, complex deflected type in order at the time of 1 month after surgery, and the result at the time of 6 months after surgery was the same with that of the time of preoperative. In the sagittal pattern, non-coincident type (S2) was predominant at the time of preoperative, and coincident type (S1) was predominant at the time of 1 month after surgery. After 6 months, the result was also the same with that of the preoperative in sagittal pattern. 5. There was not a statistical difference in the change of the mandibular movement between group of presence of the preoperative TMJ symptoms and non-presence group (p>0.05). 6. There was not a statistical difference in the change of the mandibular movement between repositioning device applied group and non-applied group (p>0.05). 7. Sixty three percents of the patients who had preoperative TMJ symptoms were improved after surgery and preoperative TMJ symptoms were more improved after operation in the repositioning device non-applied group statistically (p<0.05).

• Pediatric Infection and Vaccine
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• v.5 no.1
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• pp.128-135
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• 1998

Purpose : This study was intended to measure seropositivities and the level of mumps- and rubella-specific IgG of MMR vaccinees from 12 to 17 years of age in Korea. Materials and Methods : From May 1996 to July 1996 we obtained sera from students of 1 middle and 2 high schools in Seoul, who were MMR vaccinees from 12 to 17 years of age and had no evidence of immunodeficiency. These 216 study population include 110 males and 106 females. Mumps- and rubella-specific IgG antibody levels were measured by ELISA. Cut-off values for seropositivity were 20 U(Gamma Unit) in mumps and over 0.17 in rubella. Results : 1) As age increased, seropositivities to mumps increased, being 68.4% in 12 year, 79.3% in 13 year, 72.2% in 14 year, 82.0% in 15 year, 87.5% in 16 year, 87.0% in 17 year, which however has no statistical significance. 2) As age increased, the level of mumps-specific IgG antibody(mean+standard deviation, GU) increased, being $52.0{\pm}49.2$ in 12 year, $65.9{\pm}51.4$ in 13 year, $71.1{\pm}66.0$ in 14 year, $67.8{\pm}53.6$ in 15 year, $82.8{\pm}67.8$ in 16 year, $92.0{\pm}68.9$ in 17 year, which however has no statistical significance. 3) As age increased, seropositivities of rubella-specific IgG increased significantly, being 26.3% in 12 year, 20.7% in 13 year, 50.0% in 14 year, 67.2% in 15 year, 66.7% in 16 year, 65.2% in 17 year(P<0.001). 4) As age increased, rubella-specific IgG increased significantly, being $0.13{\pm}0.145$ in 12 year, $0.087{\pm}0.101$ in 13 year, $0.194{\pm}0.168$ in 14 year, $0.260{\pm}0.187$ in 15 year, $0.305{\pm}0.213$ in 16 year, $0.325{\pm}0.221$ in 17 year(P<0.001). There was positive correlation between age and rubella-specific IgG titer(rubella-specific $IgG=0.0517{\times}age-0.5586$, r=0.3752, P<0.001). Conclusion : In adolescent, seropositivities and the level of mumps-specific IgG remained relatively high, but approximately 20% of study population showed seronegativity. Seropositivities and the level of rubella-specific IgG showed the lowest level at 13 years of age and were increased with age after 14 years of age. Further evaluation may be needed to elucidate the cause of these changes of rubella-specific IgG.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.65-73
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• 1999

Purpose: Regurgitation, vomiting and feeding intolerance are frequent in the neonates. Esophageal function and gastric peristalsis are not fully developed in the neonates, so we should give attention to reduce the incidence of regurgitation and vomiting after feeding. It is necessary to shorten the gastric emptying by change of feeding types and postprandial postures. Gastric emptying time was measured by ultrasound in the neonates to evaluate the effect of feeding types and postprandial postures. Method: We measured gastric antral cross sectional area along the abdominal aorta at the level of the superior mesenteric artery in longitudinal section at NPO state (4 hours after feeding), 0 and every 30 min. after feeding until the value goes below or back to the NPO state. Fifteen neonates were examined in each breast-fed and formula-fed group in supine position. Eighteen and 15 neonates were examined in supine and prone posture after formula feeding, respectively. We used 5 MHz convex prove with Aloka Echo Camera SSD-650. Result: 1) Gastric emptying time of breast-fed infants was $76.0{\pm}20.02$ min. which was significantly shorter than $96.0{\pm}20.28$ min. of formula-fed infants. 2) Gastric emptying time on postprandial prone posture was $85.0{\pm}22.43$ min. which was not significantly different from $96.0{\pm}20.28$ min. on postprandial supine posture. Conclusion: Breast feeding is strongly recommended to the neonates to shorten gastric emptying time. So we can expect to reduce the incidence of regurgitation, vomiting and feeding intolerance. The postprandial posture depends on the traditional trend which is safe and comfortable to the mothers.

• Pediatric Infection and Vaccine
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• v.9 no.2
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• pp.163-174
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• 2002

Background : To delineate the changes in the causative agents of neonatal sepsis and their antimicrobial susceptibilities in the neonatal intensive care unit and nursery of Hanyang University Hospital during the past 10 years. Methods : Hospital records of 15,144 patients hospitalized at the NICU and nursery of Hanyang University Hospital from 1989 to 1998 were reviewed and neonates diagnosed of neonatal sepsis were sorted and included in the study. The study period was divided into Period A(the first 5 years) and Period B(the second 5 years) to analyse causative agents and their antimicrobial susceptibilities. Results : Neonatal sepsis was diagnosed in 170 patients(1.1%{Period A 1.2%, Period B 1.0%}) among the total of 15,144 inpatients. Two hundred isolates(Period A 109 isolates, Period B 91 isolates) were identified in 186 blood cultures(Period A 99 cultures, Period B 87 cultures) from 170 patients(Period A 91 patients, Period B 79 patients). The average age at the onset of the disease, when the initial blood culture was drawn, was 12.3 days old(Period A 8.8 days, Period B 16.3 days), and the proportion of the early onset disease was 34.7% in Period A and 23.0% in Period B, indicating that neonatal sepsis developed earlier during Period A. Among the isolated organisms including Gram positive bacteria[132(66.0%)], Gram negative bacteria [60(30.0%)], and fungi[8(4.0%)], coagulase negative Staphylococcus(CNS) was the most common organism(69/34.5%), followed by Staphylococcus aureus(36/18.0%), Klebsiella pneumoniae(17/8.5%), Enterococcus(12/6.0%), Enterobacter cloacae(8/4.0%), Escherichia coli(6/3.0%), and Pseudomonas aeruginosae(5/2.5%). The isolated fungi were Candida parapsilosis, Candida albicans, and Trichosporon pullulans. CNS, S. aureus and Acinetobacter baumannii were isolated more frequently in Period A compared to Period B. Antimicrobial susceptibilities of CNS and S. aureus to methicillin and the first generation cephalo sporins were decreased in Period B compare to Period A, those to aminoglycosides were increased in Period B, and vancomycin resistant strains were not identified. K. pneumoniae, Enterococcus, E. coli, and P. aeruginosa were isolated less frequently in Period B, compared to Period A. For K. pneumoniae, antimicrobial susceptibilities to the first generation cephalosporins were low in both Periods A and B, those to tobramycin and gentamicin were increased in Period B, and those to amikacin, ceftriaxone, and trimethoprim-sulfamethoxazole were high in both Periods A and B. Antimicrobial susceptibilities of Enterococcous to ampicillin, penicillin, and the first generation cephalosporins were decreased in Period B, but vancomycin resistant strains were not identified. Conclusion : The occurrence rate of neonatal sepsis during the past 10 years in the NICU and nursery of the Hanyang University hospital was 1.1%, and the most common causitive agents were CNS and S. aureus, to which the antimicrobial susceptibilities to the first line drugs decreased in the later half of the study period with no vancomycin resistant isolates identified. Group B Streptococcus known to be the most common agent causing neonatal sepsis was not identified, and K. pneumoniae was isolated more commonly during the later half of the study period without decreased antimicrobial susceptibilities.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.1
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• pp.21-30
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• 2005

Purpose: The aim of this study was to identify the clinical features and natural history of dietary protein induced proctocolitis (DPIPC) and to detect the causative foods of DPIPC, and to evaluate the effect of elimination of the foods on the course of the disease. Methods: Between March 2003 and July 2004, data from 30 consecutive patients with DPIPC who were followed for over 6 months, was reviewed. The diagnostic criterion used for DPIPC was an increase in the number of eosinophils in the lamina propria (${\geq}60per$ 10 high-power fields). In breast feeding mothers, 5 highly allergenic foods were eliminated from the maternal diet for 7 days, namely, allergenic food groups such as dairy products, eggs, nuts and soybean, fish and shellfish, and wheat and buckwheat. We observed the disappearance or appearance of hematochezia after elimination or challenge with the offending foods. Results: Before diagnosis infants were breast-fed (93.3%) or formula-fed (6.7%). Mean age at symptom onset was $11.5{\pm}5.1$ (5~24) weeks, and mean age at diagnosis was $17.8{\pm}9.5$ (8~56) weeks. Duration from symptom onset to diagnosis was $6.3{\pm}6.7$ (0~36) weeks. Mean peripheral blood eosinophil count was $478{\pm}320$ (40~1,790)/$mm^3$ and eosinophilia (> $250/mm^3$) was observedin 90.0% of patients. None of patients were found to have an increased serum IgE level. Of 15 patients that received sigmoidoscopy, nodular hyperplasia with erosion was observed in 93.3%. Of 27 patients whose mother ate the diet eliminated the 5 food groups, hematochezia diappeared in 74.1% of patients. Offending foods were identified as dairy products (37.5%), wheat and buckwheat (27.5%), fish and shellfish (20.0%), nuts and soybean (7.5%) and eggs (7.5%). A free maternal diet without patient's clinical symptoms was achieved at $29.4{\pm}8.7$ (9~44) weeks of patient's age, and a free baby diet without blood in stools was achieved at $37.5{\pm}9.7$ (12~56) weeks of age. Conclusion: DPIPC commonly occurs in exclusively breast-fed babies. Elimination of the above-mentioned 5 hyper-allergenic food groups from the maternal diet for 7days enables the detection of the offending foods. DPIPC is a transient disorder and 96.0% of patients can tolerate the offending foods at 12 months of age.

• Pediatric Infection and Vaccine
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• v.9 no.1
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• pp.67-73
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• 2002

Purpose : Despite of the appropriate measles vaccination programs, epidemics occur every 2~3 years and especially occurred in large group in late of 2000 and early of 2001. To evaluate the effect of the vaccination, needs for revaccination and to determine the optimal age for revaccination, we examined measles specific IgG and IgM in mealses patients and investigated different antibody appearance according to vaccination history. Methods : Anti-measles antibodies were checked in sera of 201 patients(male : 117, female : 84) that are responsible for Criteria for Disease Control among 298 patients that are suspicious of measles including inpatients and outpatients in Wonju Christian Hospital from June in 2000 to June in 2001. They were checked by immunofluorescent assay. Then we classified them according to sex, month, distribution of age due to vaccination and appearance of measles antibody. Results : The ratio of male and female was 1.4 : 1. The maximum incidence was 38 cases(18.9%) in May in 2001. Incidence was increased from November in 2000 to January in 2001 and decreased in February and March in 2001. Thereafter it was increased from April in 2001 again and decreased from June. There were 93 cases(46.3%) in vaccinated group and 108 cases(53.7%) in unvaccinated group. In the distribution according to age in vaccinated group, there were 54 cases(58.1%) in more than 10 years old, 15 cases(16.0%) between 7 and 10 years old, 12 cases(12.9%) between 15 months and 3 years old, 6 cases (6.5%) between 4 and 6 years old and 6 cases(6.5%) between 6 months and 14 months old. In the distribution according to age in unvaccinated group, there were 88 cases(81.5%) between 6 months and 14 months old, 9 cases(8.3%) between 15 months and 3 years old, 7 cases(6.5%) less than 6 months old, 3 cases(2.8%) more than 10 years old and 1 case(0.9%) between 7 and 10 years old. In the distribution of measles specific IgG and IgM, 78 cas (87.6%) were IgG(+), IgM(+) and 11 cases(12.4%) are IgG(+), IgM(-) in vaccinated group. In unvaccinated group, there were 69 cases(63.9%) of IgG(+), IgM(+) and 39 cases (36.1%) of IgG(-), IgM(+). Con c lu s i on s : We thought that measles incidence was peaked between 6 months and 14 months old in unvaccinated group because of maximum decrement of transplacental matenal antibody and was peaked in more than 10 years old in vaccinated group because of maximum decrement of measles specific IgG. We think that measles revaccination as well as vaccination and especially optimal age for revaccination is very important to prevent measles successfully.