지주막하 출혈을 동반한 용혈성 요독 증후군 1례

김웅흠,김우택,이창연,김동욱,박성기,장무환 동국대학교 경주대학 1993 東國論集 Vol.12 No.-

Hemolytic uremic syndrome is characterized by Coombs-negative microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure in infant and children. At present, E. coli 0157 : H7 and other verotoxin-producing E. coli(VTEC) seem to be a major cause of this illness. The majority of patients have a prodrome of bloody diarrhea, and acute renal failure develops several days to weeks later. Although an optimal treatment remains unknown, the general management of hypertention and renal failure with early and frequent dialysis is the mainatay of treatment, and intravenous administration of immunoglobulin G is like to be effective. We experienced a case composed of bloody diarrhea, seizures, coma, and anuria in 1 year old girl who was diagnosed as HUS and recovered renal function completely with slight impairment of cognitive ability and behavioral function. We presented this case with a review of literatures.

Reagent Strip Method를 이용한 정상만삭아에서의 생후 첫 72시간의 혈장 혈당치의 변화

김웅흠 ( Woong Heum Kim ),최중환 ( Jung Hwan Choi ) 대한주산의학회 1997 大韓周産醫學會雜誌 Vol.8 No.3

대동맥 판상부 협착증에 동반된 폐동맥 및 우심실 유출로 협착

김태우,김웅흠,고재곤 世宗醫學硏究所 1993 世宗醫學 Vol.10 No.2

Congenital Diaphragmatic Hernia with Ipsilateral Cystic Adenomatoid Malformation of Lung and Hypoplastic Left Heart Syndrome(SNU CPC 95-4)

Chi, Je G.,Kim, Woong Heum Seoul National University 1995 The seoul journal of medicine Vol.36 No.3

에드워즈 증후군 2례

김우택,김웅흠 동국대학교 경주대학 1993 東國論集 Vol.12 No.-

We have seen two cases of Edwards syndrome, one is the female neonate who was born to a 22-year-old mother and a 30-year-old father at Dong-guk university Kyung-ju hospital and the other is the male neonate who was born beteeen a 38-year-old mother and a 40-year-old father at Dong-guk university Kyung-ju hospital and had a healthy 7-year-old sister. They had the multiple anomalies that were charaterized by intrauterine growth retardation, microcephalic skull with flat occiput, microphthalmia, low-set and malformed ears, micrognathia, shield chest & webbed neck, flexion deformity of fingers, simian crease, narrow pelvis, and rocker-bottom feet with short and big toes. Chromosomal analysis revealed 47,XX,+18 and 47,XX, + 18 respectively in these cases. A brief review of relevant literatures is made briefly.

소아 비만의 위험 인자와 비만한 자녀를 가진 가족의 특성에 관한 조사 연구

이창연,김웅흠 대한비만학회 1994 The Korean journal of obesity Vol.3 No.1

To study the risk factors of childhood obesity and the characteristics of the parents with obese child, We surveyed 676 primary-school children of 4-5-6th grade living in the isolated residential area for only the one large company employee and their farnilies, located in the suburb, at March 1990. The results were as follows; 1) The prevalence rate of childhood obesity is 19.2%. 2) The obesity index is significantly increased with the increase of birth weight(p$lt;0.05). But the percentage of obese children is not significantly different according to the duration of breast milk feeding(p$gt;0.05). 3) The percentage of obese children in the group who walk to school, or who exercise regularly, is not lower than in the group who do not.(p$gt;0.05) 4) The parental body mass index of the obese children are higher than those of the non-obese children(p$lt;0.05). 5) There is no significant difference of the percentage of obese children, according to the number of siblings, or to the order of birth(p$gt;0.05). 6) Both systolic blood pressure and diastolic blood pressure in the obese children are higher than those in tbe non-obese children(p$lt;0.05). 7) The recognition rate of childhood obesity by the parents with obese child is 74.1%, and most parents with obese child do not know the true medical Problems of childhood obesity exactly.

사립체 유전자 tRNA^(Leu(UUR))의 점돌연변이가 확인된 인슐린의존성당뇨병 1 예

박혜원,김희주,이홍규,신찬수,김원배,정해일,김숙경,김웅흠 대한내과학회 1997 대한내과학회지 Vol.53 No.1

We describe a case of insulin-dependent diabetes mellitus(lDDM) with a mutation at nucleotide 3243 of mitochondrial DNA. A 24-years-old female presented with recurrent episodes of generalised tonic clonic seizures, cognitive decline, short stature, bilateral sensory neural hearing loss, bilateral optic neuropathy, lactic acidosis, and basal ganglia calcifications in addition to IDDM. Maternal transmission of the disease was suggested, by the fact that her mother have died of diabetic complications of the age of 50. Heteroplasmy of wild type and mutant mitochondrial DNA derived from peripheral leucocytes was detected by Apa I digestion of the polymerase chain reaction products amplified with a set of primer for tBNA^(LUR(UUR)) Adenosin-to guanidine substitution, occurring at nucleotide position 3243 in tRNA^(LUR(UUR)) gene in comparison with reference sequences was confirmed.

영아기 이후 진단 된 1형 가성 저알도스테론증 1례

엄미령,한승범,임창환,이경연,김정수,김웅흠 대한소아내분비학회 2007 Annals of Pediatirc Endocrinology & Metabolism Vol.12 No.1

1형 가성 저알도스테론증은 신생아기에 염분소실 및 탈수를 유발하는 질환으로, 경구 염분 투여만으로도 증상이 호전되나, 적절한 치료가 이루어지지 않을 경우 심각한 탈수 및 사망을 초래할 수 있는 질환이다. 저자들은 반복적으로 구토 및 발한 증상과 함께 저나트륨혈증, 고칼륨혈증 및 대사성 산혈증을 보이며 혈장레닌활성도 및 혈중 알도스테론 수치의 상승이 동반되었던 생후 19개월 환아에서 1형 가성 저알도스테론증 진단 후 경구 탄산수소나트륨 투여로 증상이 호전되었던 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Pseudohypoaldosteronism (PHA) typeⅠ is a rare neonatal disease characterized by salt wasting, dehydration, hyperkalemia and metabolic acidosis. It is unresponsive to mineralocorticoid treatment with elevated aldosterone concentration. The three different modes of inheritance has been described. The autosomal dominant form has a mild clinical course and gradually improves with age. In this form, resistance to aldosterone seems to be restricted to the kidney. The autosomal recessive form displays generalized aldosterone resistance including kidney, colon, lung, sweat and salivary gland system. This form is more severe and requires life-long supplement with high-dose salt. The sporadic form is mild and resembles the autosomal dominant form. In this paper, we describe a male patient diagnosed as PHA typeⅠat the age of 19 months. He presented with recurrent vomiting, diaphoresis accompanying hyponatremia, hyperkalemia, metabolic acidosis, elevated plasma renin activity and aldosterone level. Ultimately he has improved with treatment by oral sodium bicarbonate.

생애 첫 발열성 요로 감염 환아에 대한 평가에서 배뇨성 방광 요도 조영술을 대체하기 위한 검사로서 $^{99m}technetium$ dimercaptosuccinic acid(DMSA) scan의 의의

한승범,고용민,이수영,정대철,강진한,이경연,엄미령,김웅흠,김정수,Han, Seung-Beom,Ko, Yong-Min,Lee, Sue-Young,Jeong, Dae-Chul,Kang, Jin-Han,Lee, Kyung-Yeon,Uhm, Mee-Ryung,Kim, Woong-Heum,Kim, Jung-Sue 대한소아신장학회 2007 Childhood kidney diseases Vol.11 No.2

목 적: 환아의 임상 양상과 검사실 소견 및 DMSA scan 검사 결과를 이용하여 방광 요관 역류에 대한 예측성을 연구하였다. 방 법 : 생애 첫 요로 감염이 진단되어 입원 치료를 받았던 환아 중, 요로 감염 급성기에 DMSA scan 및 배뇨성 방광 요도 조영술을 모두 시행한 84명의 환아를 대상으로 후향적 연구를 하였다. 환아들은 배뇨성 방광 요도 조영술 결과에 따라 역류가 없는 집단, 경증 역류가 있는 집단(grade I-II), 중증 역류가 있는 집단(grade III-V)으로 분류되었고, 환아의 성별, 나이, 발열 기간, 혈중백혈구 수치, 혈장 CRP수치, 원인 균주 및 DMSA scan 결과와 방광 요관 역류 사이의 연관성을 조사하였다. 결 과 : 84명의 환아 중, 6명은 경증, 17명은 중증 역류를 보였다. DMSA scan 결과 급성 신장손상 소견은 38명에서 관찰되었다. 방광 요관 역류를 갖는 환아는 역류가 없는 환아에 비해 유의하게 나이가 많은 결과를 보였다(P<0.01). 요로감염 원인균주로서의 E. coli 감염의 빈도는 중증역류 환아에서 나머지 두 집단에 비해 유의하게 낮았다(P<0.01). DMSA scan에서의 급성 신장손상 소견은 역류 현상 및 역류의 중증도와 유의한 연관성을 보였다(P<0.05). DMSA scan에서 정상 소견을 보였던 환아 중 10.9%는 중증의 방광요관 역류를 보였다. 방광 요관 역류를 진단하는데 있어서 DMSA scan은 민감도 69.6%, 특이도 63.9%, 양성예측도 42.1%, 음성예측도 84.8%의 결과를 보였다. 결 론 : 방광 요관 역류 및 그 중증도는 DMSA scan에서의 급성 신장 손상 소견과 연관성이 있으나 낮은 민감도, 특이도, 양성 예측도를 볼 때, 급성기 DMSA scan에서 이상 소견을 보인 환아에서는 배뇨성 방광 요도 조영술을 시행하여 방광 요관 역류의 정도를 진단하여야 하며, DMSA scan에서 이상 소견이 없더라도 10.9%에서 중증의 방광 요관 역류가 동반되므로 배뇨성 방광 요도 조영술을 시행해야 할 것으로 생각한다. Purpose : We studied the value of clinical signs, laboratory findings and $^{99m}technetium$ dime-rcaptosuccinic acid(DMSA) scan in predicting the presence of vesicoureteral reflux(VUR) in children with first febrile urinary tract infection(UTI). Methods : A retrospective analysis of 84 hospitalized children with first febrile UTI was performed. They underwent DMSA scan and voiding cystourethrography(VCUG) during the acute phase, and were divided into three groups according to the results of the VCUG: absence of VUR, mild(grade I-II) and severe VUR(grade III-V). We studied the relation of VUR to age, gender, fever duration, causative organism, white blood cell count, serum C-reactive protein and result of DMSA scan. Results : Among 84 patients, 6 had mild and 17 had severe VUR. Thirty-eight had abnormal DMSA scan. results Patients with VUR were older than those without VUR(P<0.01). There was a lower probability of infection with Escherichia coli in patients with severe VUR than in those with mild and absent VUR(P<0.01). An abnormal DMSA scan correlated with the presence and severity of VUR(P<0.05). Severe VUR was present in 10.9% of patients with normal DMSA scan. The sensitivity, specificity, positive and negative predictive values of the DMSA scan in predicting the presence of VUR were 69.6%, 63.9%, 42.1%, and 84.8%, respectively. Conclusion : An abnormal DMSA scan correlated to the presence and severity of VUR, but the sensitivity, specificity and positive predictive value of the DMSA scan were low. There-fore, patient with an abnormal DMSA scan requires a VCUG. In order to prevent missing the 10.9% of patients with severe VUR but normal DMSA scans, a VCUG should be performed in patient with normal DMSA scan.

MDBK 세포 배양에서 Eimeria tenella 발육 상황 및 닭 비장세포에 의한 발육 항진 효과

채종일(Jong-Yil Chai),이순형(Soon-Hyung Lee),김웅흠(Woong-Heum Kim),윤종구(Chong-Ku Yun) 대한기생충학열대의학회 1989 The Korean Journal of Parasitology Vol.27 No.2