급성 췌장염과 저혈량성 급성 신부전이 동반된 Henoch-Sch$\"{o}$nlein 자반증 1예

오기원,박상규,김준성,Oh, Ki-Won,Park, Sang-Kyu,Kim, Joon-Sung 대한소아소화기영양학회 2009 Pediatric gastroenterology, hepatology & nutrition Vol.12 No.2

저자들은 하지의 자반과 함께 발생한 복통, 혈변, 구토, 핍뇨 등을 주소로 내원한 7세 소아에서 급성 췌장염과 저혈량성 급성 신부전이 함께 동반된 Henoch-Sch$\"{o}$nlein 자반증으로 진단하고 치료한 증례를 경험하였기에 문헌고찰과 함께 보고한다. 급성 췌장염은 Henoch-Schonlein 자반증 환자에서 매우 드문 합병증이지만 불필요한 개복술을 피하기 위하여 급성 복증의 다른 원인들과 반드시 감별 진단해야 한다. Gastrointestinal involvement in Henoch-Sch$\"{o}$nlein purpura (HSP) is common. However, both acute pancreatitis and acute renal failure appear to be very rare complications of HSP. We describe a case of HSP with acute pancreatitis and hypovolemic acute renal failure in a 7-year-old girl who presented with a vasculitic purpuric rash involving the lower extremities, abdominal pain, hematochezia, vomiting, and oliguria. Laboratory findings showed increased serum levels of amylase, lipase, and creatinine. An abdominal CT scan revealed diffuse enlargement of the head and body of the pancreas. The patient was successfully managed with conservative treatment, including corticosteroids, and then her pancreatic enzymes and renal function returned to normal. Acute pancreatitis should be differentiated from other causes of acute abdomen in HSP to avoid unnecessary surgery.

윌슨병 환자에서 D-penicillamine 치료 중 발생한 미세변화형 신증후군 1례

오기원,김세영,이환석,최병호,고철우,구자훈,Oh, Ki-Won,Kim, Se-Young,Lee, Hwan-Suk,Choe, Byung-Ho,Ko, Cheol-Woo,Koo, Ja-Hoon 대한소아소화기영양학회 2002 Pediatric gastroenterology, hepatology & nutrition Vol.5 No.2

윌슨병의 치료제로 구리 흡착제인 D-penicillamine이 주로 사용되고 있으나, 심각한 부작용이 발생할 경우 투약을 중단하고 trientine 등을 대체약으로 사용할 수 있다. 저자들은 윌슨병으로 D-penicillamine 사용 도중 6개월만에 미세변화형 신증후군이 발생한 후 trientine으로 치료약을 대체 후 관해가 유도된 1례를 경험하였기에 보고하는 바이다. Wilson's disease is a treatable autosomal recessive inherited disorder of copper metabolism due to mutation of the copper transporting gene. The basic strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering both a low copper diet and copper-chelating agents. D-penicillamine is the first choice as a copper-chelating agent. Some serious side effects could occur in 3~5% of all patients following D-penicillamine therapy. We report a 19 year-old male with Wilson's disease who developed nephrotic syndrome 6 months after the initiation of D-penicillamine therapy. Prednisolone was administered to control nephrotic syndrome and D-penicillamine was switched to trientine. Urinary remission was achieved within a week and maintained thereafter. Nephrotic syndrome was proven to be MCNS by kidney biopsy.

언어발달장애에 있어서 청성뇌간반응의 임상적 의의

오기원,박우생,권순학,김진경,이준화,Oh, Ki Won,Park, Woo Saeng,Kwon, Soon Hak,Kim, Jin Kyung,Lee, Jun Hwa 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.10

목 적: 언어발달장애를 주소로 내원하는 소아들을 대상으로 청성뇌간반응(ABR)을 선별검사로 시행함으로써 그 원인을 조기 발견하고 조기 치료함으로써 이들의 장애를 최소화하고 언어발달 과정에 도움을 주고자 본 연구를 시행하였다. 방 법 : 1999년 1월부터 2001년 12월까지 3년 동안 경북대학교병원 소아과에 언어발달장애를 주소로 내원한 18개월 이상 소아 139례를 대상으로 하여 청성뇌간반응(ABR)을 시행하였으며 이들의 임상진단과 ABR 이상에 따라 분류하였다. 결 과 : 전체 139례 중 ABR 검사상 이상소견을 보인 환아는 15례(10.8%)였으며 전반적 발달장애 7례, 발달성 언어장애 4례, 기타 4례로 나타났으며 이상소견을 보인 환아 중 7례는 전음성 난청이었으며 8례는 감각신경성 난청이었다. 또한 ABR 검사상 정상을 보인 18개월에서 7세 사이의 I파 및 V파의 평균잠복기 및 표준편차는 각각 $1.40{\pm}0.13$, $5.57{\pm}0.26$이었으며, I-V의 정점간 잠복기는 $4.18{\pm}0.24$였다. 중등도 이상의 감각신경성 난청환아의 4례에서 보청기를 착용하였으며 이들을 추적 관찰하였다. 결 론: 언어발달장애를 주소로 내원하는 소아들에게 선별검사로서의 ABR은 반드시 필요한 것으로 생각되며 이것을 통해 감각신경성 난청을 조기 발견하고 감각 신경성 난청의 경우 보청기를 사용하여 조기 치료함으로써 이들의 신경학적 발달 및 사회적 적응에 도움을 줄 수 있을 것이다. Purpose : The study aimed to evaluate the efficacy of auditory brainstem response(ABR) as a screening tool in children with speech and language disorders. Methods : Between January 1, 1999 and December 31, 2001, 139 patients with chief complaints of speech and language delay were recruited from the pediatric neurology clinic, Kyungpook National University Hospital, Daegu, Korea. They had ABR on entry and the clinical data were then analyzed. Results : Fifteen out of 139 cases(10.8%) showed abnormal findings; seven had pervasive developmental disorders, four had developmental language disorders, and four were noted to have other conditions. Among them, seven cases were noted to have conductive hearing loss and eight had sensoryneuronal hearing loss. We also evaluated the normal values in children at the ages of 18 months to seven years. The mean latency of wave I and V were $1.40{\pm}0.13$ and $5.57{\pm}0.26$ respectively. Interpeak latency of I-V was $4.18{\pm}0.24$. Conclusion : Based on these findings, ABR has proved to be a highly sensitive and specific index of hearing impairment. It should be used as a screening tool in children with speech and language disorders.

COX-2 억제제 투여 후 호전을 보인 가족성 선종성 용종증 1례

오기원,김세영,이환석,이명훈,최병호,Oh, Ki Won,Kim, Se Young,Lee, Hwan Suk,Lee, Myung Hoon,Choe, Byung Ho 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.12

Familial adenomatous polyposis(FAP) is an autosomal dominant disease characterized by numerous adenomas in the colorectum. Patients with FAP are always at risk of malignant transformation, so that colectomy is unavoidable. NSAID, such as sulindac, and selective COX-2 inhibitor, such as celecoxib, have shown a positive effect on FAP by causing polyp regression in some patients. We report a case of FAP in a 9-year-old female whose polyposis regressed markedly after six months-treatment with celecoxib. 가족성 선종성 용종증은 전 대장에 수백개 이상의 용종들이 발생하는 유전성 질환으로, 예방적 대장절제술을 시행하지 않으면 거의 100%에서 대장암으로 악성화 한다. NSAID와 선택적 COX-2 억제제가 용종의 퇴행을 유발하는데 효과가 있는 것으로 알려져 있다. 저자들은 9년 5개월 여아에서 FAP로 진단받고 6개월간 선택적 COX-2 억제제인 celecoxib를 투여한 후 증상이 소실되고 양호한 성장을 보였을 뿐만 아니라 용종의 수와 크기도 현저하게 감소한 1례를 경험하였기에 보고하는 바이다.

정상 면역기능을 가진 소아에서 수두 감염 후에 발생한 급성 췌장염 1예

오기원,김준성,김자형,박상규,정진영,Oh, Ki-Won,Kim, Joon-Sung,Kim, Ja-Hyeong,Park, Sang-Kyu,Jeong, Jin-Young 대한소아소화기영양학회 2008 Pediatric gastroenterology, hepatology & nutrition Vol.11 No.1

저자들은 복통과 반복적인 구토를 주소로 내원한 정상적인 면역기능을 가진 6세 소아에서 수두-대상포진 바이러스 감염 후에 드문 합병증의 하나로 발생한 급성 췌장염 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Blunt trauma, drugs, infection, congenital anomalies of the pancreaticobiliary system, and multisystem diseases are the main causes of acute pancreatitis in children. Various viruses can cause acute pancreatitis, but varicella-induced pancreatitis is unusual and generally observed in adults or immunocompromised patients. We report a rare case of acute pancreatitis associated with varicella-zoster virus infection in a 6-year-old immunocompetent girl. The patient initially presented complaining of severe abdominal pain and repetitive vomiting. The patient had multiple cutaneous crusts that has been caused by preceding varicella infection and had elevated values of serum amylase and lipase. Abdominal ultrasonography demonstrated swelling of the pancreas and pancreatic duct dilatation, findings which were compatible with acute pancreatitis. The patient's clinical and laboratory abnormalities were completely normalized through conservative treatment consisting of fasting, total parenteral nutrition, and analgesic therapy.

한국인 성인 여성에서 osteoprotegerin 유전자 다형성과 대동맥 석회화의 관련성

오기원 ( Ki Won Oh ),윤은주 ( Eun Joo Yun ),이은정 ( Eun Jung Rhee ),이원영 ( Won Young Lee ),백기현 ( Ki Hyun Baek ),윤건호 ( Kun Ho Yoon ),강무일 ( Moo Il Kang ),오은숙 ( Eun Sook Oh ),박철영 ( Cheol Young Park ),임성희 ( Sung H 대한내과학회 2006 대한내과학회지 Vol.70 No.1

제대 정맥 카테터 삽입술 후에 발생한 심방조동 1례

오기원 ( Ki Won Oh ),이경연 ( Kyung Yeon Lee ),김자형 ( Ja Hyeong Kim ),김준성 ( Joon Sung Kim ) 대한주산의학회 2012 Perinatology Vol.23 No.1

Umbilical catheterization is one of the common procedures performed in the neonatal intensive care unit, but it is sometimes associated with significant complications. Atrial flutter (AF) is a rare cardiac complication following the insertion of an umbilical venous catheter. We report a newborn infant who developed AF after umbilical venous catheterization. The tachyarrhythmia was successfully managed with synchronized cardiover sion. There were no recurrent episodes of AF and antiarrhythmic medications were not needed on the follow-up.

만성간질환에서의 골대사이상

오기원 ( Ki Won Oh ) 대한간학회 2012 Postgraduate Courses (PG) Vol.2012 No.-

Metabolic bone diseases occurring in patients with chronic liver diseases, known as hepatic osteodystrophy, is an important complication of chronic liver diseases. Generally, there is a bone mineral density (BMD) deficit in chronic liver disease, but considerable patient heterogeneity exists. Vertebral and non-vertebral fracture rates are increased in chronic liver disease, especially in postmenopausal women. Risk factors of osteoporotic fracture include older age, hypogonadism, corticosteroid therapy, and established cirrhosis. Patients who have osteoporotic fractures, who are postmenopausal women, and who require long-term treatment with corticosteroids should undergo BMD testing. All patients require education regarding the lifestyle changes (weight-bearing exercise, smoking and alcohol cessation) as well as vitamin D and calcium supplementation. All patients should receive at least 800 IU of vitamin D daily. Vitamin D deficiency should be corrected by increasing serum 25(OH) vitamin D levels to at least 30ng/mL. Bisphosphonates should be considered in patients with osteoporosis, with osteoporotic fractures, with long-term treatment with corticosteroids.

주요 내분비질환의 진료 지침 : 골다공증

오기원 ( Ki Won Oh ) 대한내과학회 2008 대한내과학회지 Vol.75 No.3

Recent The Korean Society of Bone Metabolism guidelines recommended bone mineral density (BMD) in postmenopausal women; in women during the menopausal transition with clinical risk factors for osteoporosis; in premenopausal women with amenorrhea longer than 6 months; in men aged 70 and older; in men under age 70 with clinical risk factors for osteoporosis; in adults with a fragility fracture; in adults with diseases or taking medications associated with secondary osteoporosis; in anyone being considered for pharmacologic therapy; and in anyone being treated, to monitor treatment effects. Several interventions to reduce osteoporotic fracture risk can be recommended to the general population. A calcium intake of at least 1,200 mg per day and vitamin D supplementation of at least 800 IU per day should be recommended. Regular weight-bearing and muscle-strengthening exercise to reduce the risk of falls and fractures. Also, advice patients to avoid tobacco smoking and recognize and treat patients with excessive alcohol intake. The Korean Society of Bone Metabolism guidelines recommended pharmacologic therapy in postmenopausal women and men aged 50 and older with hip or vertebral fracture; with a T score below -2.5; and with a T score between -1.0 and -2.5 with either other prior fractures or secondary causes associated with high risk of fracture. (Korean J Med 75:267-273, 2008)

정상한국인에서 혈중 호모시스테인과 엽산 농도 및 Methylenetetrahydrofolate Reductase 유전자 다형성

오기원(Ki Won oh),이원영(Won Young Lee),안유배(Yoo Bae Ahn),송기호(Ki Ho Song),유순집(Soon Jib Yoo),윤건호(Kun Ho Yoon),강무일(Moo Il Kang),차봉연(Bong Youn Cha),이광우(Kwang Woo Lee),손호영(Ho Young Son),강성구(Sung Ku Kang) 대한내과학회 1999 대한내과학회지 Vol.57 No.6

N/A Background : Hyperhomocysteinemia is an independent risk factor for cardiovascualr disease. Recently, a mutation (677C→T) was identified in the methylenetetrahydrofolate reductase gene leading to the substitution of valine(V) for alanine(A). This mutation causes a reduced folate-dependent enzyme activity which leads to increased homocysteine. In this study, we examined the association between the V allele of the methylenetetrahydrofolate reductase gene and serum total homocysteine and folate concentrations in Korean healthy subjects. Methods : In 198 healthy subjects, the methylenetetrahydrofolate reductase genotypes were analyzed by polymerase chain reaction followed by HinfI digestion. Serum total homocysteine and folate concentrations were measured in age- and sex-matched 14 healthy subjects in each of three methylenetetrahydrofolate reductase genotypes. Results : Homozygosity for 677C→T mutation in the methylenetetrahydrofolate reductase gene was found in 31 (15.7%) of 198 healthy subjects. In healthy subjects, those bearing the VV genotype tend to have higher serum total homocysteine concentrations 1.5 μmol/L(18.6%) than AA genotype but this was not statistically significant. Correlation between serum total homocysteine concentrations and other clinical variables showed that serum folate and creatinine were significant. Conclusion : We conclude that although the frequency of VV genotype in Korean healthy subjects is higher than that of other reports, this mutation is not associated with increased serum total homocysteine concentrations in Korean healthy subjects. (Korean J Med 57:1030-1036, 1999)