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      • KCI등재

        Supraventricular tachycardia in a neonate with cardiac rhabdomyoma and tuberous sclerosis

        방인국,김천수,이상락,권태찬,김여향 대한소아청소년과학회 2008 Clinical and Experimental Pediatrics (CEP) Vol.51 No.7

        Primary tumors of the heart are uncommon among pediatric patients. Rhabdomyoma is the most frequent cardiac tumor in infants and children, which is commonly associated with tuberous sclerosis. Tuberous sclerosis is a neurocutaneous syndrome affecting the brain, heart, skin, and other organs. Cardiac rhadomyomas are reported in 50-64% of infants with tuberous sclerosis. Tuberous sclerosis involves multiple locations in the atrium, ventricle and septum, and may induce mechanical obstruction of the outflow tract and heart failure depending on the location, number, size, and degree of invasion of tumors. Arrhythmias may also develop in infants with cardiac rhadomyomas, but only a few of these patients require prolonged anti-arrhythmic therapy because arrhythmia often disappears with spontaneous regression of the tumors, and the ultimate prognosis may be decided by the cerebral manifestations.

      • KCI등재후보

        성장 클리닉을 방문한 아동들에 대한 대체요법 이용 실태

        방인국,최희철,신현정,최병규,김흥식 대한소아내분비학회 2008 Annals of Pediatirc Endocrinology & Metabolism Vol.13 No.1

        Purpose : Although growth hormone treatment is now widely used to treat some group of children with short stature, it is still expensive and many parents are seeking alternative therapies. We report the current status of alternative therapies for children visiting the 'growth clinic' in a single tertiary center. Methods : Questionnaire about the usage of alternative therapy was used. Parents were asked whether they used alternative therapies and where they achieved the information. Also, they were asked whether the treatment was effective, and whether they would continue to use the alternative therapy. Results : Two hundred and twenty nine children (127 boys, 102 girls) visiting the growth clinic in Dongsan Medical Center from February 2005 to January 2006 were analyzed. Their ages were between 6.1 years to 15.4 years (mean 10.4 years). Height Standard Deviation Score (SDS) were between -3.2 to 1.0 (mean -1.1). Twelve boys (9.4%) and 7 girls (6.9%) were more than zero in height SDS value. Among 145 children (63.3%) who used the alternative therapy, supplemental foods and oriental herbs were most widely used (43.3% each) followed by over-the-counter drugs. They attained the information most frequently from their neighbors followed by television, newspaper, and internet. More than half (57.9%) of the parents answered that the alternative therapies were not effective. However, 46.9% of the parents answered they would continuously use the therapy though it is ineffective. Conclusion : Many patients visiting the 'growth clinic' for short stature had experiences on the alternative therapies. Although these therapies seemed not quite effective, many parents answered that they would continue alternative therapy.

      • KCI등재

        특발성혈소판감소성자반증 환아에서 정주용 면역 글로불린 투여 후 절대 호중구 수치의 변화

        신현정,방인국,최병규,황진복,김준식,김흥식 대한소아청소년과학회 2007 Clinical and Experimental Pediatrics (CEP) Vol.50 No.10

        Purpose : Intravenous immunoglobulin (IVIG) is effective for the treatment of idiopathic thrombocytopenic purpura (ITP) in children. Recently, several reports have been published that show its impact on the absolute neutrophil count. The present study was performed to confirm these findings. Methods : Data on 26 ITP patients were analyzed. Patients with febrile illness or increased C-reactive protein levels at presentation, which would influence the neutrophil counts, were excluded to determine the sole impact of IVIG. In addition, patients who received steroid treatment were also excluded. Results : Sixteen boys and ten girls were analyzed. For patients who received an IVIG dose of 0.4 g/kg/day (n=17), the absolute neutrophil count (ANC) measured next day was significantly decreased. For patients who received an IVIG dose of 1 g/kg/day (n=9), the ANC measured the next day was also significantly decreased. However, the decrease was more profound in the high-dose group compared to the low-dose group. Among six cases with profoundly decreased ANC greater than 1,000/ mm3, four patients (67%) received IVIG at a dose of 1 g/kg/day. All four cases with increased ANC were treated with IVIG dose of 0.4 g/kg/day, and three cases (75%) among them had a febrile reaction during IVIG administration. None of the cases with decreased ANC had a febrile reaction. No cases had infectious complications reported. Conclusion : IVIG treatment for ITP patients appears to suppress the ANC. This decrease of ANC was more pronounced when a higher dose of IVIG was used. Some cases with increased ANC counts after IVIG use were found only in low-dose IVIG group, and was associated with febrile reactions during IVIG use. 목 적 : 특발성혈소판감소성자반증의 치료로 정주용 면역 글로불린을 사용한 후 절대 중성구치의 변화에 대하여 조사하였다. 방 법 : 내원 당시 발열이나 CRP 증가, 스테로이드 사용 등 백혈구수치에 영향을 받을 수 있는 인자를 가지지 않은 특발성혈소판감소성자반증 환아 26례를 대상으로 사용된 정주용 면역 글로불린의 용량과 사용중 발열 여부 등에 따라 정주용 면역 글로불린을 1일간 사용한 전후의 절대 중성구 수치의 변화를 후향적으로 관찰하였다. 결 과 : 특발성혈소판감소성자반증의 치료로 면역 글로불린을 사용한 후 1일이 지났을 때 절대 호중구 수치는 감소되었던 경우가 많았고 절대 호중구 수치의 감소 정도는 면역 글로불린의 용량과 관계가 있었다. 면역 글로불린 사용 후 절대 호중구 수치가 증가되었던 경우는 면역 글로불린 사용 중 발열을 동반하거나 저용량의 면역 글로불린을 사용하였던 경우들이었다. 절대 호중구 수치가 감소된 경우에도 감염성 합병증이 있었다고 기술되었던 경우는 찾을 수 없었다. 결 론 : 특발성혈소판감소성자반증의 치료에 면역 글로불린을 사용하고 1일 후 절대 호중구 수치가 감소되는 현상을 관찰할 수 있었다. 심각한 감염성 합병증이 있었다는 기록은 찾기 어려웠으나 이러한 현상이 환자에게 어떤 영향을 미치는지에 대해서는 앞으로 계속 관찰할 필요가 있겠다.

      • KCI등재후보

        Russell-Silver 증후군 환아에서 성장 호르몬의 치료효과

        김흥식,박상우,방인국,최병규 대한소아내분비학회 2007 Annals of Pediatirc Endocrinology & Metabolism Vol.12 No.1

        Russell-Silver syndrome is characterized by low birth weight, growth retardation, delayed bone age, asymmetry, abnormal sexual development, cranio-facial disproportion, short little finger and clinodactyly. Short stature is the major concern, so there have been interests treating Russell-Silver syndrome with human growth hormone. However, there are no large-scale controlled studies to confirm the effectiveness of growth hormone therapy until now because of the rarity of this disorder. In Korea, we cannot find reports dealing with growth hormone treatment in Russell-Silver syndrome. We present three children with Russell-Silver syndrome who showed accelerated growth with growth hormone treatment. 자궁내 성장 부전의 가장 심한 형태인 Russell-Silver 증후군에서 가장 문제가 될 수 있는 것이 저신장증이다. 저자들은 Russell-Silver 증후군으로 진단받은 세 명의 환아에게 성장 호르몬 치료 효과를 경험을 하였기에 보고하는 바이다.

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