• Pediatric Infection and Vaccine
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• v.24 no.2
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• pp.112-116
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• 2017

Kawasaki disease (KD) is an acute febrile mucocutaneous lymph node syndrome that commonly presents with stable hemodynamic status during the acute phase. An 8-year-old boy initially presented with severe hypotension and acute kidney injury. He was placed in the intensive care unit and was diagnosed with KD. Observed clinical features were defined as KD shock syndrome. His coronary artery was dilated during the subacute phase. Furthermore, he was given anti-hypertensive medications, owing to hypertension as an unusual complication of KD. We knew the importance of monitoring for blood pressure considering vasculitis as an aspect of the main pathogenesis of KD.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.164-175
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• 2001

Purpose : Alport syndrome is a hereditary nephrotic disease characterized by progressive nephrotic symptom, sensorineural hearing loss, ophthalmic abnormality, typical microscopic findings, and familial occurrence. In this study, we tried to find the risk factors related with its prognosis by taking a close observation on clinical symptoms of children with Alport syndrome reviewing retrospectively. Materials & methods : We chose children diagnosed as Alport syndrome in renal biopsy during 20 years(from 1980, Jan. until 1999, Dec.) who could receive follow up studies in tile department of pediatrics. They were divided into two groups by comparing renal function at the time of diagnosis and at current status. We compared several clinical aspects in them, and applied nonparametric test for statistical analysis. Results : The sex ratio(male:female) of 24 children was 3:1. The most common clinical symptom presented at their first visit was gross hematuria. Among those 24 children, 11 cases($46\%$) of progressing into chronic renal failure(Group II) were observed. Hypertension, proteinuria and edema were seen much frequently in group II. The level of serum protein, albumin, and creatinine clearance were decreased while BUN, creatinine were relatively increased. All the results were statistically significant. Conclusion Clinically significant risk factors related to prognosis in Alport syndrome were the presence of hypertension, edema, and proteinuria at the time of diagnosis. Also, the level of serum protein, albumin, BUN, creatinine, and glomerular filtration rate were proved to be important factors in predicting prognosis. We believe that studies on these possible risk factors would be of great help in treating and predicting prognosis of children suffering with Alport syndrome. (J Korean Soc Pediatr Nephrol 2001;5 : 164-75)

• Tuberculosis and Respiratory Diseases
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• v.65 no.1
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• pp.37-40
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• 2008

Nephrotic syndrome is a relatively rare complication of malignancy. A few cases of nephrotic syndrome accompanying Hodgkin's disease, non-Hodgkin lymphoma, leukemia and other malignancies have been reported since the first case of the nephrotic syndrome associated with extrarenal malignancy was reported in 1922. Hodgkin's disease and solid tumors are known to be the most common malignancies accompanying nephrotic syndrome. The pathologic findings of kidney in patients with Hodgkin's disease commonly show minimal change nephropathy. Membranous glomerulonephropathy is the most common pathologic feature in patients with solid tumors. Although membranous glomerulonephropathy related to small cell lung cancer has rarely been reported in Korea, minimal change nephropathy accompanying small cell lung cancer has never been reported. We present here a case of a 70 year-old male with minimal change nephropathy that was related to small cell lung cancer. We detected small cell lung cancer during the diagnosis work-up of nephrotic syndrome. We suggest that nephrotic syndrome can be a manifestation of underlying malignancy.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.13-16
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• 1997

Steroid induced bone loss is a serious problem in frequently relapsing nephrotic syndrome especially in growing children. In order to evaluate whether $1-(OH)D_3$ (IL Sung Pharma.Co.) is effective in preventing steroid induced bone loss, we gave $0.5{\mu}g$ of $1-(OH)D_3$ for one year to forty patients with frequently relapsing nephrotic syndrome, receiving longterm prednisolone therapy (mean duration $50.12{\pm}29.40$ months). We checked the following markers before and after $1-(OH)D_3$ therapy. i.e. bone mineral density(BMD) using dual energy X-ray absorptiometry(DEXA) at the 2nd to 4th lumbar spine, serum calcium, phosphorus, parathyroid hormone(PTH), osteocalcin and urine pyridinoline(U-PYD). BMD($g/cm^2$) was increased even steroid therapy from $0.71{\pm}0.0\;to\;0.73{\pm}0.0$ (p<0.05). Lumbar spine BMD is a sensitive marker for evaluating steroid induced bone loss in children receiving longterm corticosteroid therapy and that $1-(OH)D_3$ appears to be effective in treating and preventing steroid induced bone loss.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.209-217
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• 2002

Purpose : Corticosteroid has been used as the mainstay therapy of childhood NS. But SIO is one of the serious complications of long-term steroid therapy, especially in growing children. Recently calcium, calcitonin, PTH, vitamin D and bisphosphonate has been used to treat or prevent SIO in adult, which is rare in children with NS. We studied the effect of $1{\alpha}-(OH)D_3$ and Pamidronate on SIO using dual energy X-ray absorptiometry (DEXA). Patients and methods : We studied thirty patients who admitted in the Dept. of Pediatrics of Kyung Hee Medical Hospital with NS. All patients was received longterm steroid therapy. There was no history of bone, liver, or endocrine disease. The samples, serum protein, albumin, BUN, creatinine, calcium, phosphorus, and BMD were obtained before and the six months after the dose of $1{\alpha}-(OH)D_3$ and Pamidronate, respectively Results : The mean age was $6.9{\pm}3.3\;and\;6.5{\pm}2.5$ years old. The mean duration of steroid therapy was $28.8{\pm}1.8\;and\;27.6{\pm}1.0$ months. The changes of serum protein, albumin, BUN, creatinine, calcium and phosphorus level between pre-treatment and post-treatment did not show statistical significance in both $1{\alpha}-(OH)D_3$ and Pamidronate treatment group. However, BMD was increased in both from $0.472{\pm}0.12\;and\;0.457{\pm}0.10\;g/cm^2\;to\;0.533{\pm}0.12$ and $0.529{\pm}0.09\;g/cm^2$ after treatment. (P<0.05) Conclusion : Both $1{\alpha}-(OH)D_3$ and Pamidronate appears to be effective in treating and preventing SIO in children with nephrotic syndrome requiring long-term steroid therapy.

• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.45 no.1
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• pp.109-114
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• 2018

Chronic infantile neurological cutaneous articular (CINCA) syndrome periodically causes fever along with inflammation in multiple organs. Patients with this condition are vulnerable to dental problems due to systemic inflammation. For uncooperative patients, general anesthesia has been widely used to control negative behavior. However, caution should be exercised when administering general anesthesia in these patients because this syndrome is pro-inflammatory. The present case report describes the clinical considerations of the dental treatment of an uncooperative child with CINCA syndrome who was treated under general anesthesia.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.7 no.2
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• pp.103-106
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• 2011

다운 증후군(Down syndrome, Trisomy 21)은 가장 흔하게 나타나는 증후군으로 대략 1/1000의 확률로 태어난다. 세 개의 유전적 유형이 있지만, 95% 정도가 3개의 21번 염색체를 갖는 비분리 염색체 유형이다. 주된 구강 증상으로는 거대설, 균열설, 과잉치, 결손치, 왜소치, 유치와 영구치의 맹출지연 그리고 이에 따른 부정교합 등이 있다. 75%의 환자에서는 어린 나이에 치주질환에 이환되기 쉬우며, 치아우식증에는 낮은 이환율을 보인다는 보고가 있다. 급성 림프구성 백혈병(Acute Lymphoblastic Leukemia)은 주로 어린이에서 나타나며, 특히 다운 증후군 환자에서의 발병율은 정상인에 비해 20배 높다. 치은비대와 출혈의 양상이 주로 나타나며, 간혹 상악골과 구개골에 종괴가 관찰 되기도 한다. 본 증례에서는 서울대학교치과병원 소아치과에 내원한 환자 중 급성림프구성 백혈병을 앓고 있는 다운 증후군 환아가 있어 이를 보고하고자 한다.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.126-131
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• 2007

Flank Pain is a leading indicator of renal and upper urinary tract disease or trauma, and rarely results from pelvic congestion syndrome. Although pelvic congestion syndrome occurs commonly in multi-parous women, pelvic congestion syndrome should also be considered as the cause of flank pain in an adolescent girl. We report the first case of pelvic congestion syndrome presenting with chronic left flank pain in an adolescent girl.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.206-211
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• 2002

Wilson's disease is a treatable autosomal recessive inherited disorder of copper metabolism due to mutation of the copper transporting gene. The basic strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering both a low copper diet and copper-chelating agents. D-penicillamine is the first choice as a copper-chelating agent. Some serious side effects could occur in 3~5% of all patients following D-penicillamine therapy. We report a 19 year-old male with Wilson's disease who developed nephrotic syndrome 6 months after the initiation of D-penicillamine therapy. Prednisolone was administered to control nephrotic syndrome and D-penicillamine was switched to trientine. Urinary remission was achieved within a week and maintained thereafter. Nephrotic syndrome was proven to be MCNS by kidney biopsy.