정상아에서 Lymphoma로 혼동된 Cryptococcal Lymphadenitis 1례

김보미,민기식,김종완,김광남,유기양,Kim, Bo Mi,Min, Ki Sik,Kim, Jong Wan,Kim, Kwang Nam,Ryoo, Ki Yang 대한소아감염학회 1996 Pediatric Infection and Vaccine Vol.3 No.2

Cryptococcosis is a rare in normal child and the majority of cases usually occur in patients with defective cell-mediated immunity. Infection is acquired by inhalation of organisms from the environment and disseminated via the blood stream to any organ of the body. We experienced a 7 year old girl who presented with fever, both cervical lymphadenopathy, hepatomegaly under the impression of lymphoma. However lymph node biopsy revealed cryptococcal budding and culture of lymph nodes yielded cryptococcus neoformans. The radiologic finding showed huge, multiple cervical lymph node enlargement spreading to mediastinum and abdomen. The immune fuction in term of T cell, B cell, serum immunoglobulin, complement and neutrophil function tests was normal. The patient was treated with amphotericin B and flucytosine for 6 weeks and responded to the treatment well. We report this case with brief review of the related literatures.

호중구 감소된 급성 림프구성 백혈병환아에서 발생한 Pneumocystis carinii 폐렴 1례

최영환,민기식,김종완,김광남,유기양,Choi, Young Hwan,Min, Ki Sik,Kim, Jong Wan,Kim, Kwang Nam,Ryoo, Ki Yang 대한소아감염학회 1997 Pediatric Infection and Vaccine Vol.4 No.1

Pneumocystis carinii pneumonia mainly occurs in immunocompromised patients and it is also known of major cause of death in children with acute lymphoblastic leukemia. After consolidation chemotherapy, acute lymphoblastic leukemia children is developed Pneumocystis carinii pneumonia frequently no an opportunistic infection but there were no controlled studies which have been performed to evaluate the usefulness of corticosteroid in Pneumocystis carinii pneumonia with acute lymphoblastic leukemia. We experienced a case of Pneumocystis carinii pneumonia in acute lymphoblastic leukemia with febrile neutropenic 6 years old girl. She was treated with trimethoprim-sulfamethoxazole and prednisone. We report this case with brief review of related literature.

3회 재발한 폐렴 구균성 뇌막염 1례

최승은,민기식,김종완,김광남,유기양,Choi, Seung Eun,Min, Ki Sik,Kim, Jong Wan,Kim, Kwang Nam,Ryoo, Ki Yang 대한소아감염학회 1996 Pediatric Infection and Vaccine Vol.3 No.1

Streptococcal pneumoniae is the second most coomon cause of meningitis in infancy and a major respiratory pathogen. It is the one of the most common cause of acquired pneumonia and otitis media in childhood. Intracranial extesion of acute otitis media occur somewhat more often from poorly pneumatized than from well-pneumatized temporal bones and in ears with a history of previous attacks of otitis media. We experienced a case of pneumococcal meningitis with 3 time recurrences in a 6 year-old male patient who had hospitalized with high fever, headache, vomiting and stupor mental state. He was diagnosed as CSF study and brain CT. Brain CT showed poorly pneumatized right mastoid region of temporal bone at that time. We report a case of pneumococcal meningitis with 3 time recurrences due to poorly pnematized mastoid region of temporal bone. A review of literatures was also presented briefly.

종 식별 분자 마커 개발을 위한 섬모충류 Euplotes의 small subunit ribosomal RNA 변이성 분석

김선영,김세주,민기식,양은진,유만호,최중기,Kim, Sun-Young,Kim, Se-Joo,Min, Gi-Sik,Yang, Eun-Jin,Yoo, Man-Ho,Choi, Joong-Ki 한국해양학회 2007 바다 Vol.12 No.3

Small subunit ribosomal RNA (18S rRNA)의 loop 부위들의 변이를 분석하여 해양 섬모충류의 종 특이 유전적 마커로써 이용 가능성을 확인하고자 9종의 Euplotes (Hypotrichia : Ciliophora)에 대하여 18S rRNA 유전자의 염기서열 변이성을 조사하였다. 연구 결과에 의하면 V1, V3 그리고 V5 부위는 종간 변이가 없었고, V7과 V8은 종간변이는 높으나 염기서열의 길이가 각각 44 bp와 79 bp로 길이가 짧아서 충분한 유전 정보를 가지기 어렵기 때문에이 부위들은 종특이 분자마커로 적합하지 않았다. 그러나 V2와 V4부위는 $1.75{\sim}20.61%$로 높은 변이성을 보여주었고 종간 계통 관계도 잘 나타내었다. 또한 염기서열의 길이도 각각 123 bp와 306 bp로 마커 개발에 충분한 길이를 가지고 있었다. 따라서 18S rRNA의 V2와 V4부위는 섬모충류의 종 특이 분자 마커 개발에 가장 적합한 부위라는 결론을 얻었다. To verify which loop regions of 18S rRNA gene are suitable as species-specific genetic markers in ciliates, we analyzed the genetic variation of 18S rRNA gene among 9 Euplotes species (Hypotrichia : Ciliophora). In our result, no inter-specific variation was detected from V1, V3 and V5 regions, and the length of V7 and V8 are 44 bp and 79 bp, respectively, which are too short to make genetic marker. In contrast, V2 and V4 may be good candidate segments of species-specific diagnostic molecular markers because these two regions are most variable ($1.75{\sim}20.61%$) and showed good inter-specific phylogeny. Furthermore, the sequences of V2 and V4 are 123 bp and 306 bp, respectively in length which are enough to make species-specific marker.

소아에서 사고에 의하지 않은 두개내 출혈의 임상적 고찰

허권회,송금호,민기식,유기양,Huh, Kwon Hoe,Song, Keum Ho,Min, Ki Sik,Yoo, Ki Yang 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.11

목 적 : 소아에서 사고에 의하지 않은(non-accidental) 두개내출혈은 빈도가 아주 드물지 않으며 성인과 다른 임상 양상을 보이고 높은 사망률과 심각한 후유증을 남긴다. 특히 흔들린 아이증후군은 소아에서만 있는 두개내출혈로 최근 본원에서 여려 사례를 경험하였다. 이에 저자들은 사고에 의하지 않은 두개내 출혈로 한림대학교성심병원에 입원한 소아 환아들을 대상으로 성별, 연령 분포, 원인, 내원 당시 임상 증상, 출혈 유형, 사망률과 후유증에 대해 조사하였다. 방 법 : 1999년 1월부터 2002년 6월까지 3년 6개월간 한림대학교 성심병원 소아과와 신경외과에 사고에 의하지 않은 두개내출혈로 입원한 15세 이하의 환아 중 신생아의 두개내출혈 환아를 제외한 20례를 대상으로 병력지를 검토하여 후향적으로 분석하였다. 결 과 : 1) 남녀비는 1 : 0.8이었고 연령 분포는 1세 미만 9례(45%), 1-5세 2례(10%), 6-10세 3례(15%), 11-15세 6례(30%)로 1세 미만과 11-15세에서 가장 많이 분포하였다. 2) 원인은 흔들린 아이 증후군 5례(25%), 동정맥 기형 5례(25%), 해면상 혈관종 2례(10%), 뇌종양 2례(10%), 동맥류 1례(5%), 원인 질환이 밝혀지지 않은 경우가 5례(25%)로 흔들린 아이 증후군과 동정맥 기형이 가장 많았다. 3) 내원 당시 임상 증상으로 경련 11례(55%), 의식 변화 6례(30%), 구토 5례(25%), 두통 3례(15%), 편측 부전마비 1례(5%), 보챔 1례(5%), 안면 경축 1례(5%)로 경련이 가장 많았다. 4) 출혈 유형은 경막하 출혈 8례(40%), 뇌내 출혈 5례(25%), 복합 두개내 출혈 5례(25%), 지주막하 출혈 2례(10%)였다. 5) 대상 환아 20례 중 사망률은 3례로 15%였고 생존율은 17례로 85%였다. 생존아 중 추적 관찰이 되지 않은 5례를 제외한 12례에서 완전 회복된 경우가 6례였으며 후유증이 남은 경우가 6례였다. 후유증이 남은 6례 중 편측 부전마비 2례, 동측성 반맹 2례, 발달 장애 2례, 사지 마비 2례, 단마비 1례, 학습 장애 1례, 시력 장애 1례였다. 결 론 : 소아에서 사고에 의하지 않은 두개내 출혈의 발생률은 아주 낮지 않으며 사망률과 후유증 발생 가능성이 높기 때문에 조기 진단과 적절한 치료가 필요하다. 또한 최근 의학의 발달로 생존율이 높아지면서 신경학적 후유증을 가진 소아들이 늘어남에 따라 이들에 대한 적극적인 재활 관리가 필요하다고 생각된다. Purpose : Non-accidental intracranial hemorrhage in children is not low in incidence and results in high mortality and serious sequelae. So, the authors have researched the distribution of sex and age, causes, symptoms and signs, hemorrhagic types, mortality rate and sequelae of the patients hospitalized with non-accidental intracranial hemorrhage at Hallym University Sacred Heart Hospital. Methods : The medical records of twenty patients, aged 15 or younger, and excluding neonatal patients, were analyzed retrospectively. The patients in this study were admitted with non-accidental intracranial hemorrhage from January 1999 to June 2002. Results : Of the twenty cases, the ratio of male to female was 1 : 0.8. The patients aged one or less and between 11 and 15 were discovered to be the most frequent cases. Shaken baby syndrome and arteriovenous malformation were found to be the most frequent causes. Seizure was most frequently found to be a symptom and a sign. Hemorrhagic type was classified into subdural hemorrhage eight, intracerebral hemorrhage five. There were three mortal cases. Twelve surviving patients, excluding five not-followed ones, were reclassified into six cases of complete recovery and six of sequalae. Conclusion : Non-accidental intracranial hemorrhage in children is not low in incidence, with a high mortality rate and a high incidence of serious sequelae after survival. Consequently, early diagnosis and appropriate treatment are required. In addition, appropriate rehabilitation after treatment is needed because the high survival rate due to advanced medical treatment results in an increasing number of neurologic sequelae.

발작을 주소로 입원한 환아의 임상적 연구

박경민(Gyoung Min Park),민기식(Ki Sik Min),이혜란(Hae Ran Lee),조도준(Do Jun Cho),이대형(Dae Hyoung Lee),심은정(Eun Jung Shim),이소연(So Yeon Lee) 대한소아신경학회 2010 대한소아신경학회지 Vol.18 No.2

Purpose : The purpose of this study was to evaluate childhood seizures to provide appropriate medical services. Methods : We retrospectively reviewed the medical records of 221 chidren under 18 years of age with seizures (excluding febrile convulsion), who were admitted to the pediatric department of Sacred Heart Hospital, Hallym University from 2007 to 2009. Results : The male to female was 1.3:1 and the peak age was 6 years or less, accounting for 63%. The most common causes of seizures according to age were listed as follows; hypocalcemia (41%) and hypoxic ischemic encephalopathies (41%) in the neonatal period, benign convulsion with mild gastroenteritis (BCwMG, 47%) in preschool children (1month and 5 years), and unprovoked seizure (80%) more than 6 years. Electroencephalogram and neuroimaging abnormalities were found in the ratio from 20% and 14% respectively. Conclusion : Seizure is the most common neurologic disease in the childhood. The above results reveal that the causes of childhood seizures in the different age group are different. Therefore, the exact diagnosis of disease according to age is needed. We hope that further clinical studies on this topic will be performed.

영아형 알렉산더 병 1례

박경민(Gyoung Min Park),고정희(Jeung Hee Ko),민기식(Ki Sik Min) 대한소아신경학회 2009 대한소아신경학회지 Vol.17 No.2

저자들은 경련을 일으킨 후 시행한 뇌 자기공명영상에서 광범위한 뇌백질의 변화가 발견된 알렉산더 병 환자를 관찰하여 문헌 고찰과 함께 보고하는 바이다. 이 환자는 로젠탄 섬유의 형성에 관여하는 GFAP 유전자에 돌연변이가 있었고 이것은 알렉산더 병의 진단에 이용되었다. 첫 내원 당시 발달은 정상이었으나 3개월 후 시행한 발달 검사에서 퇴행하는 양상을 보였다. 환아는 항경련제로 경련을 조절하면서 신경운동치료 중으로 경과관찰이 필요하다. Alexander disease(AD) is a rare fatal demyelinating disorder, caused by the mutation of glial fibrillary acidic protein(GFAP) gene. It is characterized by progressive demyelination of central nervous system, and the accumulation of Rosenthal fibers within astrocytes. It is divided into three group: infantile, juvenile, and adult. The infantile type is most common, has onset during the first 2 years of life. It shows macrocephaly and psychomotor delay, spastic paraparesis, seizure, and feeding problems, and usually dies within the first decade. The severity of the pathological changes depend on the age of onset. Radiological study revealed white matter loss, usually with frontal predominance. It is diagnosed by DNA analysis. We present case of a 10-month-old male patient with AD. He had focal seizures, demyelination in the frontal lobe in MRI, and the presence of a K86E mutaion in the GFAP gene, involving the replacement of adenosine with guanine.

4세 여아에서 자연 완해된 담즙 마개 증후군 1례

지금봉,송준영,유기양,민기식,김덕하,이관섭,Jee, Keum-Bong,Song, Jun-Young,You, Ki-Yang,Min, Ki-Sik,Kim, Deok-Ha,Lee, Kwan-Seop 대한소아소화기영양학회 1999 Pediatric gastroenterology, hepatology & nutrition Vol.2 No.2

Obstruction of the extrahepatic bile ducts is the most common cause of conjugated hyperbilirubinemia in early infancy. More than 90% of such obstructive lesions are accounted for by extrahepatic biliary atresia. A rare lesion is obstruction of the common duct by impacted, thickened secretions and bile. Bile plug syndrome is defined as extrahepatic obstruction of the bile ducts by bile sludge in term infants without anatomic abnormalities, congenital chemical defects of bile, or hepatocellular lesions. Obstruction of extrahepatic ducts by plugs of biliary material apperas to be due to the inspissation and precipitation of bile and mucus within the lumen of the ducts. Cholestasis and precipitation of bile develop in association with abnormal composition of bile in cystic fibrosis, hepatocellular damage, prolonged erythroblastic jaundice, altered biliary dynamics with total parenteral nutrition, gut dysfunction, diuretic therapy, exchange transfusions and perinatal hemolysis. In those cases, the term inspissated bile syndrome is used. The clinical and laboratory findings in bile plug syndrome are identical to those observed in biliary atresia and choledochal cyst. The diagnosis can be suspected based on the findings of clinical and laboratory examinations together with hepatobiliary imaging, ultrasonography, radionuclide scan and liver biopsy. We experienced a case of spontaneous resolution of bile plug syndrome in a 4-year-old girl. We report this case with brief review related literatures.

흔들린 아이 증후군 5례

조옥연,허권회,조도준,김덕하,민기식,유기양,이열,Cho, Ok Yeon,Huh, Kwon Hoe,Cho, Do Jun,Kim, Dug Ha,Min, Ki Sik,Yoo, Ki Yang,Lee, Yul 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.4

저자들은 경련, 구토를 주소로 입원한 환아에서 만성 경막하출혈과 망막출혈을 보여 흔들린 아이 증후군으로 진단한 5례를 경험하였다. 현재까지 국내에서는 보고와 연구가 드물지만 의료인이 흔들린 아이 증후군에 대한 가능성을 염두에 두고 좀 더 적극적으로 방사선 검사나 안과 검사를 시행하면 올바른 진단과 함께 치명적인 합병증의 발생을 감소시킬 수 있다고 사료되는 바 문헌 고찰과 함께 보고하는 바이다. Shaken baby syndrome is a serious form of child abuse, mostly involving children younger than 2 years. It results from extreme rotational cranial acceleration induced by violent shaking. The characteristic injuries include subdural hemorrhage, retinal hemorrhage, and fracture of ribs or long bones. If physicians have no recognition of, or suspicion about, shaken baby syndrome, this syndome is difficult to diagnosis because of a lack of obvious external signs and failure of the abuser to admit his or her actions. In addition to the high mortality, 60% of survivors have significant long term neurologic and developmental abnormality. The authors experienced five cases of shaken baby syndrome presented with seizures or vomiting, without external signs of trauma. All of these cases had subdural hemorrhages, and four cases had retinal hemorrahges. In our follow up, two children were found to have sequelae such as quadriplegia, monoplegia, and developmental delay. We emphasize that early recognition and prompt treament are key to overall success of case management. The incidence of shaken baby syndrome can be reduced through public awareness and education of parents not to shake a babies.

동일 열성질환에서 반복성 열성경련의 예방을 위한 Diazepam의 효과

조도준(Do Jun Cho),민기식(Ki Sik Min ),김종완(Jong Wan Kim),김광남(Kwang Nam Kim),유기양(Ki Yang Ryoo) 대한소아신경학회 1993 대한소아신경학회지 Vol.1 No.2

The efficacy of short-term diazepam prophylaxis in repeated febrile seizures was evaluated in a prospective controlled study. A total 172 consecutive children admitted with their febrile seizure were participated in our study from May,1991 to June,1992. The children of control group were admitted with their febrile seizure from January,1987 to December,1990. Intravenous Diazepam(0.1mg/kg) is administered to patients with febrile seizures at admission. And oral Diazepam(0.5mg/kg/day) is administered at first hospitalization day. At second hospitalization day, consecutive oral Diazepam (0.6 mg/kg/day) is administered. The results were as follows: 1) The incidence of repeated febrile seizure was 8.1% in study group and 17.0%in control group. 2) In hospital, repeated febrile convulsion was 0.6% in study group and 4.3% in control group. 3) The repeat rate of febrile seizure with fever less than 39℃ was 6.3% in study group and 20.5% in control group. 4) The repeat rate of febrile seizure with serum sodium concentration under 135mEq/L was 10.9% in study group and 47.4% in control group. 5) There was no serious side effects of diazepam.