• Journal of Music and Human Behavior
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• v.2 no.2
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• pp.15-31
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• 2005

Terminal pediatric patient include congenital metabolic abnormalities, chromosome aberrations, congenital anomalies, neuromuscular diseases and other incurable conditions as well as malignant tumors. One third of these diseases are cancers, and two thirds of cancers are lymphadenoma and leukemia. Terminal pediatric patient may feel fear, anger and frustration against treatment process in hospitals, lose control of themselves and stay in helplessness due to restrictions within controlled hospital environment. This study examined the relationship between hospitalized childrens behavior and music using Contextual Support Model of Music Therapya theory stating therapeutic music environments possess three elements of Structure, Autonomy support and Involvement, and increase childrens active engagement. Focused on these three therapeutic elements, this study analyzed music therapy cases of terminally ill children to examine the relationship between childrens behavior and music as environmental supportive medium in the environment. This study is on a single case case subject, a 10-year-old girl with acute lymphocytic leukemia. Nine sessions of music therapy activities were conducted and analyzed using qualitative method. Focusing on three therapeutic elements of Contextual Support Model, analysis of sessions was made on the basis of activities specifically designed for this study. Main music therapy activities included singing while playing musical instruments, singing while listening to music through CD player, making up songs, and searching for song lines. The findings of this study are as follows: a) in terms of Structure, music therapy suggested a direction for effective musical activities for terminally ill children by providing environments where child can sing, play musical instruments and make up songs; b) in terms of Autonomy support, music therapy encouraged childs voluntary participation by giving her chances to make choice while singing or playing musical instruments; c) in terms of Involvement, child could gain positive approval of the therapist and conduct musical activities in cooperation with the therapist. Music therapy serves as supportive medium in the environment, develops spontaneity and self-reliance in emotionally depressed children, and promotes voluntary attitudes in the restricted hospital environment. Based on the positive effects of music therapy, more studies need to be conducted with an approach to more various terminal pediatric patients.

• Pediatric Infection and Vaccine
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• v.23 no.1
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• pp.72-76
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• 2016

Infantile osteopetrosis is a rare congenital disorder caused by abnormal bone resorption. Patients with osteopetrosis can have severe anemia, thrombocytopenia, hepatosplenomegaly, rickets, visual impairment, and deafness. Cytomegalovirus also can cause a congenital infection with anemia, thrombocytopenia, hepatosplenomegaly, and calcifications in the brain. We report a 38-day-old infant with severe hepatosplenomegaly, thrombocytopenia, hypocalcemia, and growth failure. Real time polymerase chain reaction detected cytomegalovirus in the plasma. Skeletal radiography revealed generalized bone sclerosis. He was diagnosed with osteopetrosis along with cytomegalovirus infection. Only the test for mutation of the CLCN7 gene, representing the most common and heterogeneous form of osteopetrosis, was available, and the result was negative. With supportive care and antiviral treatment, severe thrombocytopenia due to the cytomegalovirus infection almost normalized despite the possible immunosuppression caused by osteopetrosis. We present the first report of an infant who suffered from osteopetrosis and CMV infection which was successfully treated by long term antiviral agent therapy.

• Neonatal Medicine
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• v.18 no.2
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• pp.365-369
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• 2011

Tetralogy of Fallot (TOF) assumes its' most severe form when accompanied by pulmonary atresia (PA). Preserving the patent ductus arteriosus to maintain pulmonary blood flow is life-saving for patients with this congenital heart disease. Milrinone, a selective phosphodiesterase III inhibitor, is a potent vasodilator. Here, we report the successful use of milrinone for a newborn infant with TOF and PA for keeping the ductus arteriosus open and thereby maintaining pulmonary circulation. Milrinone is a useful drug because of its inotropic, lusitropic, and pulmonary vasodilating effects, in addition to its ability to keep the ductus arteriosus open and its relatively mild side-effects. Case series and comparative studies will be needed in the future to verify the effectiveness of this drug.

• Neonatal Medicine
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• v.16 no.1
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• pp.64-70
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• 2009

Congenital diaphragmatic hernia (CDR) with severe pulmonary hypoplasia is associated with significant mortality. Recently, several new therapeutic methods have been suggested, such as high-frequency oscillatory ventilation and inhaled nitric oxide. For hypoxemic respiratory failure unresponsive to these advanced medical treatment options, extracorporeal membrane oxygenation (ECMO) serves as the last potentially effective treatment. An understanding of the pathophysiology of pulmonary hypertension associated with CDH led to a strategy involving preoperative stabilization and delayed surgical intervention with ECMO. We describe four cases of ECMO, including the first report of ECMO for neonatal CDH in Korea.

• Proceedings of the Korea Water Resources Association Conference
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• 2004.05b
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• pp.753-758
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• 2004

건천화란 하천이 건천이 되어가는 과정이라고 할 수 있다. 그러나 이의 정량적 접근이 어려운 이유는 건천이 되어가는 과정에 대한 현상을 지속적으로 관찰하기 어렵고, 순수한 자연현상이라기보다 인위적 요소가 간섭한 결과이기 때문이다. 본 연구는 건천이 되어가는 하천 중에서도 도시화에 의한 건천화의 영향이 큰 것으로 보이는 우이천 유역에 대해 건천화의 원인을 분석하여 몇 가지 결론과 대책을 제시하였다. 우이천은 유역면적 $27.67km^2$, 유로연장 11.75km의 비교적 좁은 면적 내에 인구 461,351명이 거주하고 주변 행정구역 총 인구가 604,282명인 지방2급하천이다. 유역 내 북부와 북서부의 산악지역을 통과하는 상류는 대부분 계류(溪流)로서 자연상태를 유지하고 있으나, 중${\cdot}$하류는 양안의 셋방, 복개, 직강화, 보 및 낙차공 등 인공구조물로 인해 하천형상이 크게 왜곡되어 있는 전형적인 도시하천유역이다. 본 연구는 우이천에 대한 건천화 원인분석을 위해 크게 4가지 방봅으로 접근하였는데 반분포형 유출모형인 TOPMODEL에 의한 장기유출분석, 수문${\cdot}$기상자료에 의한 분석, 인문${\cdot}$사회자료의 분석, 지하수위 분석 등이었다. 분석결과 우이천 유역과 같이 장기간에 걸쳐 도시화가 진행된 유역은 수문순환계의 왜곡이 돌이킬 수 없는 수준이므로, 적절한 건천화 방지대책의 방향은 건천화의 원인이 되는 인자를 제거함으로써 줄어든 유량을 회복하는 방안보다는 인위적이고 강제적인 방식으로 추가적인 하천수량을 확보하는 것이 타당하다 사료된다. 하천의 건천화는 하나의 주된 원인이 지배적일 수도 있지만 간접적인 영향이 많고, 하나의 원인이 다른 인자에 영향을 주어 연쇄적인 영향을 미칠 수도 있는 복잡한 현상이다. 따라서 다양한 각도에서 다양할 접근방식으로 방지대책을 수립하여야 할 것이다.적에 따른 임계지속기간과 유출량의 변화를 검토해 보았다.이를 각각의 경우의 해석해 결과와 비교${\cdot}$분석하였다. 후방추적 퍼프모형은 전방추적 퍼프모형에 비하여 사용된 퍼프수와 관계없이 작은 오차를 발생하였으며, 전체적으로 퍼프 모형이 입자모형보다는 훨씬 적은 수의 계산을 통해서도 작은 오차를 나타낼 수 있다는 것을 알 수 있었다. 그러나 Gaussian 분포를 갖는 퍼프모형은 전단흐름에서의 긴 유선형 농도분포를 모의할 수 없었고, 이에 관한 오차는 전단계수가 증가함에 따라 비선형적으로 증가하였다. 향후, 보다 다양한 흐름영역에서 장${\cdot}$단점 분석 및 오차해석을 수행한 후에 각각의 Lagrangian 모형의 장점만을 갖는 모형결합 방법을 제시할 수 있을 것으로 판단된다.mm/$m^{2}$로 감소한 소견을 보였다. 승모판 성형술은 전 승모판엽 탈출증이 있는 두 환아에서 동시에 시행하였다. 수술 후 1년 내 시행한 심초음파에서 모든 환아에서 단지 경등도 이하의 승모판 폐쇄 부전 소견을 보였다. 수술 후 조기 사망은 없었으며, 합병증으로는 유미흉이 한 명에서 있었다. 술 후 10개월째 허혈성 확장성 심근증이 호전되지 않아 Dor 술식을 시행한 후 사망한 예를 제외한 나머지 6명은 특이 증상 없이 정상 생활 중이다 결론: 좌관상동맥 페동맥이상 기시증은 드물기는 하나, 영유아기에 심근경색 및 허혈성 심근증 또는 선천성 승모판 폐쇄 부전등을 초래하는 심각한 선천성 심질환이다. 그러나 진단 즉시 직접 좌관상동맥-대동맥 이식술로 수술적 교정을 해줌으로써 좋은 성적을 기대할 수 있음을 보여주었다.특히 교사들이 중요하게 인식하는 해방적 행동에 대한 목표를 강조하여 적용할 필요가 있음을 시사하고 있다.교하여 유의한 차이가 관찰되지 않았다. 또한 HSP 환자군에서도 $IL1RN^{*}2$ allele 빈도와 carria

• Journal of Chest Surgery
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• v.33 no.12
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• pp.963-967
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• 2000

정상심실중격의 폐동맥협착은 흔한 선천성 심장질환이지만 신생아기에 심한 증세를 보이는 위기적 폐동맥 협착은 드물며 예후도 대단히 나쁘다. 경피적 풍선 판막성형술의 발달과 함께 폐동맥협착의 외과적 치료는 더욱 줄어드는 추세이다. 본 증례는 정상심실중격의 위기적 폐동맥협착증으로 진단받은 생후 2일된 남자 신생아로 심한 청색증과 저산소증을 보여 응급실로 내원하였다. 환아는 산소공급 및 Prostaglandin E$_1$을 투여 후 동맥혈 산소 분압이 19 mmHg에서 54mmHg로 증가하였다. 경피적 풍선 판막성형술을 시도하였으나 유도도관(Guide wire)이 판막의 개구부를 통과하지 못하였고 시술도중 심낭내로 조영제가 고이는 소견을 보여 우심실 천공이 의심되었으므로 응급으로 정상체온의 체외순환하에서 폐동맥 절개후 폐동맥 판막절개술을 시행하였다. 수술 후 중환자실에서의 수술경과는 양호하였고 현재 수술 6개월째 외래 추적관찰중이다.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.4
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• pp.545-550
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• 2010

Freeman-Sheldon Syndrome (FSS, also known as "Whistling Face Syndrome") is a rare genetic condition which characteristically includes a small "whistling" mouth, a flat mask-like face, club feet, joint contractures usually involving the fingers and hands, and under-development of the cartilage of the nose. Intelligence is usually normal. Most of the features of this syndrome are due to muscle weakness. The patient, 11 years old boy was consulted from pediatrics to pediatric dentistry due to dental management. After clinical & radiographic examinations, severe multiple problems were found. Dental problems were microsomia(whistling mouth) & micrognathia, perioral muscle contracture, restricted mouth opening, poor oral hygiene & care, generalized dental caries, high palatal vault, severe malocclusion & crowding. And Orthopedic problems, ophthalmic & respiratory, anesthetic problems were found. Then He also had psychiatric problem, hospital(dental) phobia due to previous medical history(frequent hospitalization). And he had genital problem, cryptochidism, too. Due to these intricate problems, he suffered with feeding, swallowing difficulties and showed growth retardation. For enhancing patient's oral health, pediatric dentist, orthodontist, oral surgeon, pediatrician, psychiatrist, orthopedist, they all agree with early, cautious intervention and treatment. So, he has been treated by multidisciplinary care, now he is recovering general health maintenance.

• Journal of the korean academy of Pediatric Dentistry
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• v.39 no.2
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• pp.161-165
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• 2012

Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare genetic disorder involving eyes, kidney, brain and musculoskeletal system, and occurs predominantly in males. The patient with Lowe syndrome is characterized with congenital cataracts, glaucoma, prominent forehead, thin and sparse hair, mental and growth retardation, muscular hypotonia, renal dysfunction, and metabolic bone disease. We have experienced a 10-year-old boy with Lowe syndrome who had poor oral hygiene and trouble for teeth brushing. To manage his behavior and systemic metabolic disorder, sedation was performed during dental care. Excessive calculus formation in this patient is caused by both medication and lack of ability to maintain oral hygiene. The dental management of those patients has to be focused on prevention due to difficulties in dental treatment and dangers of general anesthesia for the Lowe syndrome.

• Journal of Chest Surgery
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• v.39 no.8 s.265
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• pp.633-636
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• 2006

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.31-37
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• 1997

The DMSA scan is a useful radiologic study in diagnosis of morphologic and functional diseases of kidney. We evaluated the distribution of sex and age, clinical manifestations, diagnosis, combined diseases, treatment and prognosis of the 61 patients with non-functioning kidney(no isotope uptake or uptake below 5% in DMSA scan) who admitted in our hospital from 1980 to 1995. The proportion of patients under 1 year old age was 46%. Sex ratio was 1.4:1 with male predominance. Most diagnosis of non-functioning kidneys were congenital such as multicystic dysplastic kidney, hydronephrosis due to ureteropelvic junction obstruction, renal agenesis and renal hypoplasia. In order of frequency thirty one percent of them were previously detected on antenatal ultrasonogram. Treatment consisted of operation in 47.5%, mostly nephrectomy and 32.8% of patients were followed up at OPD base without definite treatment. The most common combined diseases was hydronephrosis, in 4patients who had both kidneys inveloved progressed to chronic renal failure, but the prognosis in most cases were good. It is important to evaluate renal diseases in perinatal periods, and we believe that highly sensitive diagnostic study contribute to early treatment plan and thus to good prognosis.