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A Follow-Up Study after Discontinuation of Antiepileptic Drug Therapy in Children with Well-Controlled Epilepsy : The Factors that Influence Recurrence (항전간제로 조절된 간질 환아에서 약물 중지 후 추적 관찰 - 재발위험인자)

  • Chung, Sa Jun;Chung, Hye Jeon;Choi, Young Mi;Cho, Eu Hyun
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1559-1570
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    • 2002
  • Purpose : There has been no exact answer to the question of when to discontinue antiepileptic drugs(AEDs) in children with well-controlled epilepsy for a long period. This study is about the risk factors of relapse after withdrawal of AEDs in seizure(Sz)-free patients to show a guideline for discontinuation of AEDs. Methods : One hundred and sixty-nine children were diagnosed as epileptic at the Pediatric Dept. of Kyung-Hee Univ. between 1993 to 1998, in whom AEDs had been withdrawn after at least two years of Sz-free period. Univariate analysis using Kaplan-Meier survival analysis and multivariate analysis using Cox-proportional hazard model were performed for sixteen risk factors. Results : Forty-nine of the 169 patients(28.9%) had recurrence of Szs. The mean follow-up after withdrawal of AEDs was 4.1 years, mean treatment period was 4.1 years, and the mean Sz-free period was 3.3 years. Factors associated with an increased risk of relapse were young age at onset, symptomatic Sz, Sz type in West and Lennox-Gastaut syndrome, neurologic deficit, longer Sz-controlling period, shorter total treatment period, number of AEDs used(more than one drug), age at withdrawal of AEDs, and Sz-free period less than two years in univariate analysis using Kaplan-Meier mothod. From multivariate analysis, the factors indicating a significantly higher relapse risk were pre-treatment period after first Sz attack, Sz-controlling period, Sz-free period, number of AEDs used, neurologic abnormalities. Conclusion : For epileptic children who were Sz-free for more than two years, and were more than six-years-old, the discontinuation of AEDs should be considered positively, according to age of onset, Sz type, age at withdrawal of AEDs, total treatment period, Sz-controlling period, number of AEDs used, etiology, neurologic deficit, and the wishes of the patients and the their parents.

Clinical Features of Isolated Noncompaction of the Ventricular Myocardium (심근의 단독 비경화증(Isolated Noncompaction of Ventricular Myocardium)의 임상 양상)

  • Moon, Eun-Kyoung;Lee, Hoon-Young;Chang, Mea-Young;Kil, Hong-Ryang;Chung, Yong-Hun
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1528-1533
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    • 2002
  • Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.

A Five-year Epidemiologic Study of Childhood Leukemia in Busan City, 1996 to 2000 (최근 5년간(1996-2000) 부산지역 소아 백혈병 환자에 대한 역학적 연구)

  • Moon, Jae Hoon;Lee, Soon Yong;Sinn, Jong Beom;Park, Jae Sun;Lee, Young Ho;Lim, Young Tak;Park, Su Eun
    • Clinical and Experimental Pediatrics
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    • v.46 no.10
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    • pp.972-976
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    • 2003
  • Purpose : For the control of childhood leukemia, of which the mortality is still high, the basic data for the incidence has a great importance. The authors analyzed the data from 133 new patients with childhood leukemia between 1996-2000 in Busan, Korea. Methods : The data were obtained from 133 new cases(87 males and 46 females from 0 to 15 years old) of childhood leukemia who were residents of Busan and who were admitted to the 4 university hospitals and 11 general hospitals from 1996 to 2000. Results : The total number of the new childhood leukemia patients was 133 between 1996-2000; the average annual number of new patients was 26.6. The age-and-sex adjusted annual incidence rate (/100,000) was in the range of 2.37-4.53(male 2.47-5.29, female 0.76-3.36) with an average of 3.29 (male 4.05, female 2.43). Age-specific annual incidence rate(/100,000) was 3.78 in the 0-4 year age group, 3.51 in the 5-9 year age group and 3.08 in the 10-14 year age group. Of the major types of childhood leukemia, the distribution of ALL was average 71.4%, of AML 23.3%, and of CML 4.5%. Of the major types of leukemia by age range, ALL showed highest in the 5-9 year age group, while AML in 0-4 and 10-14 year age groups. Sex-ratio(male to female) of major type of leukemia was 1.97 : 1 and 1.21 : 1, in ALL and AML groups, respectively, while all were male in CML. Conclusion : The average age-and-sex adjusted annual incidence rate(/100,000) of childhood leukemia in Busan from 1996 to 2000 was 3.29. Compared to data in related articles, this data suggests a steady increase in the incidence of childhood leukemia in the Busan area over the last 20 years since 1981.

Changes of Neutrophil Count in Peripheral Blood of the Neonate with Periventricular Leukomalacia (신생아 백질연화증 환아 말초혈의 중성구 변화)

  • Lee, Hwan Seok;Park, Kyung Pil;Kim, Heng Mi
    • Clinical and Experimental Pediatrics
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    • v.46 no.10
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    • pp.966-971
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    • 2003
  • Purpose : It is now well established that infection and inflammation play an important role in the pathogenesis of ischemic brain damage. The loss of neutrophils from systemic circulation is an associated finding in injury mediated by granulocyte. Periventricular leukomalacia(PVL) caused by ischemia is the principal form of brain injury in premature infants. This study was conducted to evaluate whether the low neutrophil count is associated with periventricular leukomalacia(PVL) in premature infants. Methods : Retrospective review of medical records was undertaken. Subjects were premature infants with a birth weight of less than 1,500 gm, admitted to the Neonatal Intensive Care Unit of Kyungpook University Hospital. A complete blood count of peripheral blood was done within the 1st hour of life. Neutropenia was defined as absolute neutrophil count < $1,500/mm^3$, PVL as increased periventricular echodensities followed by cyst formation on ultrasonography or corresponding signs on brain MRI. Results : Thirteen infants out of a total population of 37 revealed neutropenia. Respiratory distress syndrome and requirement for respiratory support were not different between infants with neutropenia( neutropenia group) and infants without neutropenia(control group). Intraventricular hemorrhage (IVH) and grade 3 and 4 IVH were more frequent in neutropenia group(P<0.05). There was no statistically significant increase of PVL in neutropenia group. The neutrophil count was $18,760.0{\pm}10,266.1/mm^3$, $7,272.0{\pm}7,435.0/mm^3$ infants with PVL and $11,131.7{\pm}3,386.5/mm^3$, $2,407.5{\pm}1,933.1/mm^3$ in infants without PVL, respectively. The frequency of mechanical ventilation and artificial surfactant therapy was higher in infants with PVL compared with infants without PVL, but statistical analysis was not performed due to small number of subjects. Conclusion : A low number of neutrophils in the systemic circulation was not associated with an increased risk of PVL in premature infants.

Early postoperative arrhythmias after open heart surgery of pediatric congenital heart disease (소아 선천성 심장병 개심술 후 발생한 조기 부정맥)

  • Choi, Hee-Joung;Kim, Yeo-Hyang;Cho, Joon-Yong;Hyun, Myung-Chul;Lee, Sang-Bum;Kim, Kyu-Tae
    • Clinical and Experimental Pediatrics
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    • v.53 no.4
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    • pp.532-537
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    • 2010
  • Purpose : Early postoperative arrhythmias are a major cause of mortality and morbidity after open heart surgery in the pediatric population. We evaluated the incidence and risk factors of early postoperative arrhythmias after surgery of congenital heart disease. Methods : From January 2002 to December 2008, we retrospectively reviewed the medical records of the 561 patients who underwent cardiac surgery in Kyungpook National University Hospital. We analyzed patients' age and weight, occurrence and type of arrhythmia, cardiopulmonary bypass (CPB) time, aortic cross clamp (ACC) time, and postoperative electrolyte levels. Results : Arrhythmias occurred in 42 of 578 (7.3%) cases of the pediatric cardiac surgery. The most common types of arrhythmia were junctional ectopic tachycardia (JET) and accelerated idioventricular rhythm (AIVR), which occurred in 17 and 13 cases, respectively. The arterial switch operation (ASO) of transposition of the great arteries (TGA) had the highest incidence of arrhythmia (36.4%). Most cases of cardiac arrhythmia showed good response to management. Patients with early postoperative arrhythmias had significantly lower body weight, younger age, and prolonged CPB and ACC times ($P$<0.05) than patients without arrhythmia. Although the mean duration of ventilator care and intensive care unit stay were significantly longer ($P$<0.05), the mortality rate was not significantly different among the 2 groups. Conclusion : Early postoperative arrhythmias are a major complication after pediatric cardiac surgery; however, aggressive and immediate management can reduce mortality and morbidity.

Clinical characteristics and progress of Kawasaki disease patients who had early treatment with intravenous immune globulin (가와사끼병에서 면역글로불린 조기 투여군의 임상적 특성 및 치료 경과)

  • Park, So-Yoon;Lee, Young Hwan
    • Clinical and Experimental Pediatrics
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    • v.50 no.10
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    • pp.1005-1010
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    • 2007
  • Purpose : To determine the optimal time of high dose intravenous immune globulin (IVIG) treatment, we analysed the clinical characteristics and progress of a group of Kawasaki disease patients who had early treatment with IVIG. Method : A retrospective study was conducted of 188 patients with Kawasaki disease who were admitted to Yeungnam University Medical Center from January 2000 to December 2005. All patients were treated with a high dose IVIG and high dose aspirin for the initial acute phase treatment. The early treatment group consisted of 94 patients who received treatment before 5 days of fever, and the conventional group consisted of 94 patients who were treated on or after day 5. The patients' sex, age, laboratory findings, total duration of fever, duration of fever after initial IVIG, need for additional IVIG and coronary artery status were noted. Result : There were no significant differences between the two groups in sex ratio and age. No significant differences were noted in the level of WBC count, ESR, CRP, serum albumin, LDH, total duration of fever and coronary abnormality. But the value of ALT($151.8{\pm}17.3$ vs. $81.9{\pm}13.4$, P=0.002), duration of fever after initial IVIG ($3.8{\pm}0.5days$ vs. $2.1{\pm}0.2days$, P=0.003), and rate of additional IVIG (15.9% vs. 6.3%, P=0.037) were significantly higher in the early treatment group. There was no significant difference in initial dose of IVIG, but dosage of aspirin was lower in early treatment group (P=0.037). Conclusion : There is no evidence that early treatment of IVIG has greater efficacy in preventing cardiac sequelae than conventional treatment. In addition, early treatment is likely to result in a greater requirement for additional IVIG treatment.

Clinical features of congenital muscular torticollis (선천성 근성 사경의 임상적 특징)

  • Jun, Ji Eun;Ryu, Hye Kyeong;Shim, Jae Won;Shim, Jung Yeon;Jung, Hye Lim;Park, Moon Soo;Kim, Deok-Soo
    • Clinical and Experimental Pediatrics
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    • v.50 no.3
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    • pp.241-247
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    • 2007
  • Purpose : Congenital muscular torticollis (CMT) is a common and benign congenital disorder of the musculoskeletal system in neonates and infants. The pathophysiology is that the sternocleidomastoid muscle (SCM) is shortened on the involved side by fibrosis, leading to ipsilateral tilt and contralateral rotation of the face and chin. In this study, we investigated the clinical features of CMT, the role of ultrasonography (USG) in prediction of prognoses and the clinical significance of early detection and treatment. Methods : Forty seven patients (M:F=31:16) were diagnosed as a CMT between March 2003 and May 2006. We reviewed age at diagnosis, physical findings, USG findings, treatment and therapeutic outcome from their medical records. Results : The median age at diagnosis was 90 days (18 days-9 years, 7 months) and the right side of neck was affected in more patients (right : left=26:21). Of 24 patients with a palpable neck mass, 21 had USG; 19 cases showed sternocleidomastoid tumor (SMT). In cases with no neck mass, USG was performed in 11 patients; seven had postural torticollis (POST), three had SMT and one had muscular torticollis (MT). Among 40 patients with follow-up, 36 had total resolution. There was negative correlation between the age at diagnosis and the recovery time, whereas the final outcome was not correlated with USG findings. However, the patients without positive findings in USG had earlier resolution (1 month vs 2.6 months, P=0.0008). The patients with SMT had earlier diagnosis and excellent outcomes. The patients with MT were delayed to diagnosis and had the longest time to resolve. Lastly, the patients with POST had delayed diagnoses, but they had excellent outcomes. Conclusion : Since the patients with delayed diagnoses, in despite of benign courses, may take a long time to resolve and rarely need surgical treatment, it is important to diagnose and treat early. This study showed that USG findings of the SCM may be used as predictive factors.

A clinical study of child bacterial meningitis in Daejeon and Chungcheong area : 2001-2005 (대전·충청 지역의 소아 세균성 수막염에 대한 임상적 연구 : 2001-2005)

  • Lee, Yong Joo;Lee, Soo Jin;Park, Ho Jin;Lee, Young Hyuk;Kang, So Young;Kim, Young Chang;Lee, In Kyu;Lee, Kyung Yeon;Lee, Keon Su;Kim, Won Seop
    • Clinical and Experimental Pediatrics
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    • v.50 no.2
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    • pp.157-162
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    • 2007
  • Purpose : The purpose of this study was to analyze the epidemiology, causative organism, clinical manifestation and prognosis of bacterial meningitis for children after the introduction of Haemophilus influenzae type b (Hib) vaccine in Daejeon and Chungcheong area. Methods : We analyzed retrospectively 53 medical records who had been diagnosed with bacterial meningitis at 10 general or university hospitals in Daejeon and Chungcheong area. All patients aged 1 month-14 years admitted between January 2001 through December 2005. Results : During the 5-year study period, 40 of all cases were positive for bacterial growth. Of the 40 cases that were CSF culture-proven bacterial meningitis, Streptococcus peumoniae was the most common bacteria for 17 (32.1%) of all cases, followed by H. influenzae for 10 (18.9%), Neisseria meningitidis for 3 (5.7%). In this study, the most common clinical manifestation is fever, accompanied by all cases. CSF leukocyte count was more than $100/mm^3$ in 45 (84.1%) cases. CSF glucose concentration was less than 50 mg/dL in 42 (79.2%) cases and protein concentration was more than 45 mg/dL in 49 (92.5%) cases. 45 of all cases made a recovery after treatment and were discharged. Most common complication after treatment is subdural effusion (19.0%) and hearing disturbance (9.4%). Conclusion : The most common organism of culture-proven bacterial meningitis in the children beyond neonatal period was S. pneumoniae. Continued surveillance studies were demanded to know the altered incidence of bacterial meningitis, because we expect the incidence of S. pneumoniae meninigitis will be on the decrease after more active innoculation of pneumococcal protein conjugate vaccine.

Analysis of disease mechanism of subacute necrotizing lymphadenitis in children (소아 아급성 괴사성 림프절염의 임상적, 방사선학적, 면역조직화학적 소견)

  • Kim, Hyun Jung;Yeom, Jung Suk;Park, Ji Suk;Park, Eun Sil;Seo, Ji Hyun;Lim, Jae Young;Park, Chan Hoo;Woo, Hyang Ok;Cho, Jae Min;Lee, Jeong Hee;Youn, Hee Shang
    • Clinical and Experimental Pediatrics
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    • v.51 no.11
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    • pp.1198-1204
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    • 2008
  • Purpose : The cause of subacute necrotizing lymphadenitis, a rare disease in children, has not been completely clarified. This study was aimed to investigate the disease mechanism by examining clinical, radiologic, and immunohistochemical findings in children diagnosed with subacute necrotizing lymphadenitis after an excisional biopsy. Methods : We examined 19 lymph node tissue specimens from 17 children diagnosed with subacute necrotizing lymphadenitis at Gyeongsang National University Hospital from March, 1998 to July, 2006. A retrospective survey of the medical records was performed. CT findings were analyzed. Immunohistochemical staining was done on tissues obtained by excisional biopsy from all patients. Results : The patient's age ranged from 5 to 19 years (average age :11.8 years). The main symptoms included a neck mass (17/19), pain in the mass (6/17), and fever (12/19). The palpable lymph nodes were mostly cervical in location; the maximum diameter, which was measured radiologically, was less than 3 cm in all 10 cases. The masses were pathologically divided into proliferative, necrotic, and xanthomatous types. With immunohistochemical staining the masses were divided into lesion (L), perilesion (PL), and necrosis (N). The CD8 staining was stronger than the CD4 staining for all regions in three types. The CD4 staining intensity was mainly increased in the perilesion, and CD8 was mainly increased in the lesion. Conclusion : We compared the radiologic findings, clinical symptoms, and pathology to help understand the cause of disease in patients with subacute necrotizing lymphadenitis.

Clinical significance of acanthosis nigricans in children and adolescents with obesity induced metabolic complications (비만으로 인한 대사적 합병증을 가진 소아 및 청소년에서 흑색가시세포증의 임상적 의의)

  • Chueh, Hee Won;Cho, Gyu Rang;Yoo, Jaeho
    • Clinical and Experimental Pediatrics
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    • v.50 no.10
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    • pp.987-994
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    • 2007
  • Purpose : This study investigated the clinical significance of AN in children and adolescents with obesity induced metabolic complications. Methods : Forty-nine patients who had obesity induced metabolic complications were participated in this cross-sectional study. Obesity induced metabolic complications are as follows: hypertension, dyslipidemia, impaired fasting glucose (IFG), impaired glucose tolerance (IGT), nonalcoholic steatohepatitis (NASH), homeostasis model assessment of insulin resistance (HOMA-IR)>3.16. Clinical characteristics, such as, age, percentage-weight-for-height (PWH), pubertal status, blood pressure (BP), fasting plasma insulin level, fasting and post-oral glucose tolerance test 2-hour glucose levels, liver function test, lipid profile, HOMA-IR were compared according to the presence of AN. Results : Sixty-five percent of patients had AN, 57.1% NASH, 57.1% dyslipidemia, 55.1% hypertension, 46.9% IFG, 24.5% HOMA-IR>3.16 and 16.2% IGT. The patients who were moderately to severely obese with AN had higher incidence of IGT and HOMA-IR>3.16. The patients with AN had significantly higher diastolic BP ($79.4{\pm}6.9$ vs $75.4{\pm}5.6mmHg$), fasting levels of plasma insulin ($10.6{\pm}6.0$ vs $6.2{\pm}5.4{\mu}IU/mL$), HOMA-IR index ($2.6{\pm}1.4$ vs $1.4{\pm}1.3$) and PWH ($42.4{\pm}13.0$ vs $34.3{\pm}1.8%$). The increasing tendency for the presence of AN was significantly related to the cumulative number of obesity induced metabolic complications. Binary logistic regression analysis revealed that the presence of AN was significantly associated with fasting plasma insulin level, PWH and IFG. Conclusion : AN could be useful as a clinical surrogate of obesity induced metabolic complications.