중심측두엽 극파를 동반한 양성 소아 뇌전증에서의 저용량 Toporamate 1일 1회 투약 효과

최영얼,장해인,배시영,손영준,이영진,김영옥,우영종 대한소아신경학회 2012 대한소아신경학회지 Vol.20 No.2

목 적 : 중심측두엽 극파를 동반한 양성 소아 뇌전증(benign childhood epilepsy with centrotempo-ral spikes, BECTS)은 예후가 양호하여, 약물 투여의 부작용을 고려한 최소한의 치료가 요구되는 질환이다. 본 연구에서는 새로 진단받은 BECTS 환자에게 저용량 topiramate (TPM) 1일 1회 저녁 투약을하여 그 효과를 기존의 oxcarbazepine (OXC) 투약군과 비교해보고자 하였다. 방 법 : 2003년 1월부터 2010년 11월까지 전남대학교병원 소아청소년과에서 BECTS로 진단하여 OXC를 투여하였던 58명의 기록을 토대로 진단 시 나이, 항경련제 투여 용량과 기간, 임상 양상 및 경과 등에 대하여 조사하였다. 2009년 8월부터 2011년 4월까지 새로 진단된 BECTS 환자에게 저용량(25mg) TPM을 저녁 1회 투여하였고 12개월 이상 외래에서 추적 관찰된 14명 의 경과를 검토하여 OXC 투여군과 비교하였다. 결 과 :OXC 투여군 58명 중 발작이 조절되었던 환자는 45명 (77.6%), 발작이 이전과 같은 빈도이거나 오히려 악화된 경우는 13명 (22.4%)이었다. 13명에게는 다른 항경련제 (TPM 9명, lamotrigine 4명) 추가하여 발작이 조절되었다. 초기 단일요법으로 저용량 TPM를 투여하였던 14명 중 발작이 재발하지 않았던 환자는 9명 (64.3%) 이었고, 2명은 약물용량을 각각 100mg/day, 75mg/day까지 종량하였다. 3명은 발작이 조절되지 않아 OXC로 바꾸었으며, 이들 모두 발작이 조절되었다. 결 론 : BECTS 환자에게서 저용량 TPM 단일요법(1일 1회 25mg 투약)의 효용성은 OXC 치료와 비교했을 때 큰 차이가 없었으며, 특별한 부작용의 사례는 없었다. Purpose : Benign childhood epilepsy with centrotemporal spikes (BECTS) is known to have a good prognosis, and is easily controlled with antiepileptic drugs. In this study, we evaluate the efficacy of low-dose topiramate (TPM), once at night time in children with BECTS, and compare with that of oxcarbazepine (OXC). Methods : OXC was used as a first drug in 58 patients with BECTS at the department of pediatrics, Chonnam National University hospital from Jan. 2003 to Nov. 2010. Their medical records were reviewed, focusing on the onset age of seizure, the dosage of drugs, response to treatment and other clinical features. Since Aug. 2008 through Apr. 2011, some newly diagnosed patients with BECTS were given a low-dose (25mg) of TPM, once at nighttime. The efficacy was reviewed in 14 children, who had been followed up in the outpatient clinic for longer than twelve months. Results : Forty five out of 58 (77.6%) patients were well controlled with OXC. Thirteen out of 58 (22.4%), seizures recurred, on whom TPM (n=9) or lamotrigine (n=4) was added. Nine (64.3%) of the 14 patients who were started with low dose TPM became seizure-free for at least 12 months. However, Two out of 14, the dose of TPM had to be increased up to 100 mg/day and 75 mg/day, respectively. In three, seizure was controlled with OXC. Conclusion : A single low-dose TPM would be as effective as the conventional OXC without any adverse effects in children with BECTS.

Clinical Utility of Echocardiography for the Diagnosis and Prognosis in Children with Bronchopulmonary Dsyplasia

최영얼,조화진,송은송,정인석,윤남식,최영연,마재숙,조영국 한국심초음파학회 2016 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.24 No.4

Background: Bronchopulmonary dysplasia (BPD) may result in chronic pulmonary artery hypertension and right ventricular(RV) dysfunction. Various echocardiographic assessments of RV dysfunction have been used to determine whether echocardiographicmeasurements of premature infants with BPD could provide sensitive measures of RV function that correlates with BPDseverity. Methods: Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderateBPD, and six patients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction byM-mode, tricuspid regurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and tissueDoppler imaging (TDI) measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, and sixpatients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction by M-mode, tricuspidregurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and TDI measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. Results: None of the standard echocardiographic findings was significantly different between the control group and BPDgroups. However, mean septal TDI-MPI of the severe BPD group (0.68 ± 0.06) was significantly (p < 0.01) higher than that of thenon-BPD (0.58 ± 0.10) or the mild BPD group (0.59 ± 0.12). In addition, mean RV TDI-MPI of the severe BPD group (0.71 ±0.13) was significantly (p < 0.05) higher than that of the non-BPD group (0.56 ± 0.08) or the mild BPD group (0.60 ± 0.125). Linear regression showed a good correlation between the severity of BPD and RV TDI-MPI (p = 0.01, R = 0.30) or septal TDIMPI(p = 0.04, R = 0.24). Conclusion: Echocardiographic evaluation of RV function based on an assessment of RV TDI-MPI can provide RV dysfunctionparameter in premature infants with BPD.

조기 신생아기에 진단된 Hallermann-Streiff 증후군 2례

최영얼,최준식,김영남,송은송,최영륜 대한신생아학회 2012 Neonatal medicine Vol.19 No.3

Hallermann-Streiff syndrome is a very rare congenital disorder, which is primarily characterized by the head and face abnormalities. Approximately 180 cases have been reported worldwide, including 8 cases in Korea since it was first described by Hallermann in 1893. Patients exhibit a bird-like face, hypotrichosis, atrophy of skin, dental abnormalities, proportionate nanism, and various ophthalmic disorders, including congenital cataracts and bilateral micropthalmia. As a result of many life-threatening complications,such as respiratory and cardiac difficulties, many patients die during infancy. We report here two cases of HSS diagnosed immediately after birth with literature reviews. They showed two additional characteristics, including chubby cheeks and antenatal ultrasonographic findings, which have not been mentioned in previous reports.

Transvenous proximal closure of large congenital coronary arteriovenous fistula using the single Amplatzer vascular plug in a 3-year-old girl

장해인,최영얼,조화진,조영국,마재숙 대한소아청소년과학회 2013 Clinical and Experimental Pediatrics (CEP) Vol.56 No.2

Congenital coronary arteriovenous fistulas (CCAFs) are rare coronary artery abnormalities in which blood is shunted into a cardiac chamber or great vessel. If the fistula itself is large and tortuous, it is generally recommended to occlude the fistula to prevent several complications. In approaches of transcatheter occlusion, the transvenous approach is preferred over the transarterial approach. The transvenous approach would enable the cannulation of a relatively larger catheter or sheath without potential damage to the femoral vessels or normal coronary arteries, which can occur in the transarterial approach. The transvenous approach may also minimize the blind pouch after releasing the devices. Herein, we report the success of transvenous proximal closure of a CCAF using an Amplatzer vascular plug (AVP) in a 3-year-old patient with cardiomegaly. Complete occlusion was achieved by a single AVP and thrombus formation of the distal aneurysmal portion of the fistula. We suggest that this strategy of closing the proximal end with a dilated fistula using a single AVP by the transvenous approach may be a good option in treating CCAFs in a young child.

양성 소아 부분 뇌전증의 치료:중심측두엽 극파를 동반한 양성 소아 부분뇌전증에서의 저용량 Topiramate 저녁 1회 요법 투약 효과

김병영,오하진,최영얼,박수민,조화진,김영옥,우영종 대한소아신경학회 2014 대한소아신경학회지 Vol.22 No.4

목적:BECTS는 예후가 양호하여,약물투여의 부작용을 고려한 최소한의 치료가 요구되는 질환이다 . 본 연구에서는 TPM1회 요법의 치료효과와 유용성을 알아보고자 하였다 . 방법:2009년 8월부터 2014년 7월까지 BECTS로 진단되어 저용량(25 mg) TPM을 저녁 1회 투여하여 18개월 이상 외래에서 추적 관찰하였던 23명 (남 13, 여 10)의 경과와 2003년 1월부터 2010년 11월까지 전남대학교병원 소아청소년과에서 BECTS로 진단하여 OXC를 투여하였던 63명(남34,여29)의 기록을 검토하였다 . 결과:저용량 TPM 1회 요법을 시행하였던 23명 중 18명 (78.2%)이 발작이 조절되었고 5명(21.8%)은 OXC을 추가하여 발작이 조절되었다.OXC 투여군 63명중 46명(73.1%)은발작이 조절되었으며,이중 5 명은 투약기간이 길어져 저용량 TPM으로 교체하였다 . 17명 (26.9%) 은 다른 항경련제 (TPM 13명, LTG 4명)를 추가하여 발작이 조절되었다. 저용량 TPM 1회 요법에서 뇌파 검사가 정상화되는 기간이 더 짧았다 . 부작용은 OXC 투여군은 7명 (피부발적 3, 체중증가 3, 기억력감퇴1)에서,저용량 TPM 1회 요법군은 1명(땀감소1)에서 나타났다 . 결론:BECTS 환자에게서 저용량 TPM 저녁 1회 요법은 OXC 치료와 비슷한 치료효과를 보이며 부작용이 없고 복용의 간편성을 고려하여 추천할만한 치료방법의 하나로 사료된다 . Purpose: Benign epilepsy with centrotemporal spikes (BECTS) is a benign disease which requires treatment with minimal side effects. The purpose of this study is to evaluate the effectiveness and efficacy of low dose topiramate (TPM), single dose, at night therapy. Method: We have reviewed the results of low dose TPM, single dose, at night therapy in BECTS who were treated from Oct. 2009 to Jul. 2014, and compared that with those of the treatment with oxcarbazepine (OXC) from Jan. 2003 to Nov. 2010. Results: Among the patients with single dose of TPM (n=23), the symptoms of 18 patients (78.2%) were controlled with TPM only and 5 patients (21.8%) were controlled with TPM with OXC. Among the patients with OXC therapy (n=63), the symptoms of 46 patients (73.1%) were controlled. Among these patients, 5 patients were converted to TPM therapy for the continued electroencephalogram (EEG) abnormality. The symptoms of 17 patients (26.9%) were controlled with add on therapy (TPM in 13 patients and Lamotrigine in 4). The duration of nomalized EEG of TPM therapy was shoter than those of OXC therapy. The side effects of OXC were skin rash in 3, weight gain in 3, and decreased memory in 1 patient. In TPM patients, decreased sweating was reported in 1 patient. Conclusion: In BECTS patients, single, low dose of TPM is as effective as OXC therapy. Also, it has better compliance with lesser side effects.

Total Anomalous Pulmonary Venous Return in Siblings

김호성,정구미,조화진,최우연,최영얼,마재숙,조영국 한국심초음파학회 2014 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.22 No.4

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an earlyoperation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heartmay not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those ofrespiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, includingsiblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR havebeen reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery wasperformed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.