나팔꽃증후군에서 동반된 모야모야병 1예

변준철,이세엽,조순영 대한안과학회 2024 대한안과학회지 Vol.65 No.3

Purpose: We report a diagnosis of Moyamoya disease based on brain magnetic resonance imaging (MRI) and brain magnetic resonance angiography (MRA) performed in a child diagnosed with morning glory syndrome. Case summary: A 6-year-old boy visited the hospital with a chief complaint of exodeviation of his left eye that started at 1 year of age. The corrected visual acuity was 20/30 in the right eye and 20/400 in the left. The Krimsky test showed exotropia of 35 prism diopters (PD) for near vision and 30 PD for distance. Fundus photography showed an enlarged optic disc with funnel-shaped excavation in the left eye. With a diagnosis of morning glory syndrome accompanied by exotropia, the patient underwent brain MRI and brain MRA. The patient had no neurological symptoms such as headache and muscle weakness. Moyamoya disease was diagnosed, as brain MRI and MRA showed tiny tortuous vessels forming collateral vessels from the lenticulostriatal arteries, left posterior cerebral artery, and left middle meningeal artery, which originated from the external carotid artery. Conclusions: Moyamoya disease can occur in children with morning glory disease. Brain MRI and MRA can help to diagnose Moyamoya disease. Caution is needed because transient ischemic attack, cerebral infarction, and cerebral hemorrhage may occur in patients with Moyamoya disease. 목적: 나팔꽃증후군으로 진단받은 환아에서 시행한 뇌 자기공명영상 및 뇌 자기공명혈관조영술상 모야모야병을 진단하였기에 이를보고하고자 한다. 증례요약: 6세 남아가 만 1세경부터 시작된 좌안이 바깥으로 나가는 증상을 주소로 내원하였다. 교정시력 우안 0.6 좌안 0.02로 측정되었으며, 크림스키검사상 근거리 35프리즘디옵터(prism diopter, PD), 원거리 30 PD의 외사시 소견을 보였다. 안저검사상 좌안에깔때기 모양의 함몰과 함께 커져 있는 시신경유두가 관찰되었다. 나팔꽃증후군과 동반된 외사시로 진단하였다. 환아는 두통, 근력약화 등의 증상이 없었으며 신경학적 이상 소견은 보이지 않았다. 동반된 뇌기형을 알아보기 위해 뇌 자기공명영상 및 뇌 자기공명혈관조영술을 시행하였다. 작고 구불거리는 혈관들이 좌측 렌즈핵선조체동맥, 후대뇌동맥, 외내경동맥으로부터 중경막동맥에 이르는부분까지 측부 혈관을 이루는 모습이 관찰되어 모야모야병으로 진단하였다. 결론: 나팔꽃증후군 환아에서 모야모야병이 동반될 수 있으며, 뇌 자기공명영상 및 뇌 자기공명혈관조영술이 진단에 도움이 될 수있다. 모야모야병에서는 일과성 허혈 발작, 뇌경색, 뇌출혈 등이 발생할 수 있어 주의하여야 한다.

Weaver 증후군 1례

변준철,김천수,이상락,권태찬,이희정 대한소아청소년과학회 2004 Clinical and Experimental Pediatrics (CEP) Vol.47 No.11

Weaver syndrome is a very rare overgrowth syndrome with accelerated skeletal maturation, unusual facies and clinodactyly. We experienced an extremely rare case of Weaver syndrome in Korea. The clinical manifestations were macrocephaly, unusal facies, simian crease, clinodactyly, decreased muscle tone, accelerated bone age and congenital heart diseases. We report this with a brief review of related literature 저자들은 대두증과 특이한 얼굴모습, 원선, 수지 만곡증 등의 신체 기형과 근긴장도 저하, 빠른 골 성숙, 선천성 심장질환 등을 동반한 Weaver 증후군 1례를 경험하였기에 문헌 고찰과 보고하는 바이다.

소아 경부 림프절 종대의세침 흡인 세포검사의 진단적 유용성

변준철,최병규,황진복,김흥식,이상숙 대한소아청소년과학회 2006 Clinical and Experimental Pediatrics (CEP) Vol.49 No.2

Purpose : There are few reports in Korean literature on the diagnostic efficacy of fine needle aspiration cytology(FNAC) for pediatric cervical lymphadenopathy. This study examined the diagnostic value of FNAC on cervical lymphadenopathy in children. Methods : Data from 57 pediatric patients(aged 0.3 to 14 years) who underwent FNAC due to cervical lymphadenopathy between January 2001 and March 2005 was reviewed retrospectively. Results : Reactive cervical lymphadenitis was the most common result of the FNAC(52.5 percent). Malignant disease were revealed in 14 percent of all cases. The sensitivity, specificity was 86 percent and 96 percent, respectively. The positive and negative predictive values for malignant disease was 75 percent and 98 percent, respectively. There were two false-positive cases on FNAC; one case of Langerhans cell histiocytosis, and another with infectious mononucleosis. A false-negative case on FNAC was found to be acute lymphocytic leukemia. In seven cases(12 percent), the final diagnosis was confirmed by an open biopsy after the FNAC. There were four FNAC cases where the specimen was not satisfactory for making a diagnosis(7 percent). There were no serious complications of the FNAC procedure. Conclusion : FNAC had a high diagnostic efficacy for evaluating children with cervical lymphadenopathy. 목 적 : 소아의 경부 림프절 종대에서 세침 흡인 세포검사의 진단적 가치에 대한 자료를 분석한 보고는 국내에서 알려져 있지 않아, 이에 대한 분석을 보고하는 바이다.방 법 : 2001년에서 2005년까지 계명대학교 동산의료원 소아과에 내원하여 경부 림프절 종대가 있어 세침 흡인 세포검사를 시행하였던 환자 57례를 의무 기록을 통해 후향적으로 분석하였다. 세침 흡인 세포검사의 결과와 임상적 최종 진단 결과를 비교 관찰하였다.결 과 : 대상 환자 57례 중 비특이성 경부 림프절염이 52.5%로 가장 많았다. 악성질환은 모두 8례로 전체의 14.0%를 차지하였다. 세침 흡인 세포검사의 악성질환에 대한 진단적 효용성을 보았을 때 민감도 86%, 특이도 96%, 양성 예측률 75%, 음성 예측률 98%였다. 위양성인 예는 랑게르한스 조직구증식증 1례와 전염성 단핵구증 1례이었고, 위음성이었던 예는 급성 림프구성백혈병 1례이었다. 세침 흡인 세포검사 후 개방 생검을 한 경우가 7례가 있었다. 검체가 충분하지 못했던 경우가 4례로 7.0%를 차지하였다.

폐렴사슬알균 뇌척수막염: 소아 측두골 골절의 드문 합병증

변준철,김엘 계명대학교 의과대학 2023 계명의대학술지 Vol.42 No.2

Meningitis is a rare and potentially serious complication in children with temporal bone fractures. We present an unusual case of a 7-year-old girl with pneumococcal meningitis complicated by cerebrospinal fluid (CSF) leakage following transverse fracture in the left temporal bone. She had an otorrhea resolved spontaneously in the early stage of hospitalization. At 4 days post-discharge, the patient returned with headache, fever, neck stiffness, voiding difficulty, and bilateral abducens nerve palsy. Magnetic resonance images demonstrated an intense uniform contrast enhancement in the cerebral cisterns and the sacral nerve roots. Laboratory analysis and culture diagnosed meningitis caused by <i>Streptococcus pneumonia</i>. She was discharged home after getting intravenous ceftriaxone for 5 weeks. Follow-up for the patient required constant vigilance and included a multidisciplinary approach. At 7 months after head trauma, the child was well with no neurological and auditory deficits. This case illustrates a previously unreported complication in pediatric patient of temporal bone fracture associated with CSF otorrhea.

원발성 대뇌 뇌실내 농양의 치료: 이중 배액관 및 오마야 장치의 이용

변준철,김엘 계명대학교 의과대학 2023 계명의대학술지 Vol.42 No.2

An isolated ventricular abscess is a rare event, and its treatment is a real challenge. We report such case in a 52-year-old man that was successfully managed with aid of occipital approach and ventricular access device. This patient presented with the chief complaint of headache and fever of 3-day duration. Magnetic resonance imaging showed fluid-filled layering within the posterior horns of the lateral ventricle without contrast enhancement. Blood test and cerebrospinal fluid analysis was consistent with acute bacterial ventriculitis. Using stereotactic technique guided by electromagnetic navigation, the occipital horns were bilaterally targeted and catheterized, and then the abscess and debris was evacuated. The Ommaya reservoir implanted at the left entry was intermittently punctured for preventing the recollection. The pus culture was positive for Streptococcus pneumoniae. He received an antibiotic therapy and the reservoir aspiration leading to rapid recovery and remission of clinical manifestations. There was no evidence of the recurrence within the ventricles on follow-up scans after discharging. This modification, occipital approach and reservoir placement, is effective treatment for improving the cure rate in the selected cases with intraventricular cerebral empyema.

신생아 뇌졸중의 임상적 양상에 관한 연구

변준철(Jun Chul Byun),서혜은(Hye Eun Seo),김행미(Heng Mi Kim),권순학(Soonhak Kwon) 대한소아신경학회 2010 대한소아신경학회지 Vol.18 No.2

Purpose : Neonatal strokes are common and may be associated with various com- plications. However, few studies have been conducted on the clinical spectrum in Korea. This study aimed at investigating the clinical presentation and neurological outcome of neonatal strokes. Methods : Twenty-seven neonates with neonatal stroke were enrolled in the neo- natal intensive care unit at Kyungpook National University Hospital from January 2000 to December 2009. Their medical records and neuroradiological findings were retrospectively reviewed. Results : The mean age of the subjects was 4 5.6 days. Sixteen patients were full ± term, nine were prematurite and six had low birth weights. The onset of symptoms was mostly within first week (85.2%) of life, especially in the first day of life (51.9 %). The most common symptom was seizure (40.7%), which were focal clonic (38.5 %) or multifocal clonic (38.5%). Nine patients showed abnormal EEG findings. Thirteen patients had subdural hemorrhage, seven showed intraventricular hemor- rhage, and three revealed cerebral infarction. Among 12 patients who followed-up for one year, four had mild neurologic dysfunction and two had severe impairment. Conclusion : We found that the onset of symptom in neonatal strokes was mostly within the first day of life, and the most common symptom was focal seizure. We, therefore recommend that neuroimaging be done when newborns have seizures within their first week of life. However, further studies are needed to elucidate this further.

茵蔯蒿湯의 랫드에서의 單回投與毒性試驗

김상찬,변준철,박종현,지선영,변성희,이형식,Kim, S.C.,Byun, J.S.,Park, J.H.,Jee, S.Y,Byun, S.H.,Lee, H.S. 대한한방안이비인후피부과학회 2001 한방안이비인후피부과학회지 Vol.14 No.2

The single dose toxicity of Injinhotang, a herbal drug for treatment of hepatic injuries. was evaluated in Sprague-Dawley rats. Injinhotang was once administered to both sexes of rats at the dose levels of 2000, 1000, 500, 250 and 125 mg/kg for oral route. After single administration, clinical signs were observed every day for 14 days and body weights were measured 5 times including initial measurement on day 0 (the days of administration). When observation period was over, the animals were sacrificed and macroscopic examination of major organs was conducted. In addition, the histopathological profiles of these major organs were also conducted. Neither significant clinical signs nor death after administration was observed during the observation periods except for soft feces or diarrhea. In addition, no abnormal necropsy findings, changes of body weight and histopathological profiles were observed at terminal necropsy in both sexes. From these results, it is considered that $LD_{50}$ of Injinhotang is over 2000 mg/kg in oral administration in both sexes of rats.

선천성 부신피질 과형성증(Congenital Adrenal Hyperplasia)에 동반된 신생아 담즙정체증 1례

최선윤,변준철,최원정,김흥식,강유나,황진복 대한소아소화기영양학회 2005 Pediatric gastroenterology, hepatology & nutrition Vol.8 No.1

It has been suggested that cortisol deficiency may be responsible for the cholestasis. Although congenital hypopituitarism was reported as a possible cause of neonatal cholestasis, congenital adrenal hyperplasia with neonatal cholestasis is a very rare report in the literature. We experienced a case presenting with neonatal cholestasis associated with congenital adrenal hyperplasia. This case provides a clinical evidence supporting the notion that cortisol has an important physiological role in bile acid synthesis and transport 저자들은 색소침착과 기력저하, 담즙정체증을 보인 2개월 여아에서 호르몬 검사와 복부 MRI 검사로 선천성 부신피질 과형성증으로 진단하고 히드로코르티손과 광물부신겉질호르몬을 투여하여 담즙정체증과 색소 침착의 완전한 회복을 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Clinical Characteristics of Symptomatic Rotavirus Infection in Newborn Infants and Genotype Analysis

김동석,변준철,류남희,김천수 대한주산의학회 2022 Perinatology Vol.33 No.3

Objective: Clinical data on rotavirus infection in the neonatal period are still limited. In this study, we investigated the incidence and monthly distribution, clinical features, and genotypes of symptomatic rotavirus infection in newborn infants. Methods: Medical records of newborn infants with rotavirus infection in a university hospital of Korea over a 4-year period (2011–2014) were retrospectively analyzed. These enrolled cases included hospital-acquired (HA) and community-acquired (CA) infections according to where the infection occurred. Infants included full-term and preterm infants according to the gestational age. Results: Among 135 finally enrolled patients with symptomatic rotavirus infections, 80 (59.3%) and 55 (40.7%) cases had HA and CA infections, respectively. There were 85 (63%) and 50 (37%) full-term and preterm infants, respectively. HA infections were more common in preterm infants, whereas CA infections were more common in full-term infants (P<0.001). In the comparison between the HA group and the CA group, clinical symptoms of rotavirus infection were similar between the 2 groups. On the other hand, in comparisons between preterm and full-term groups, abdominal distension, feeding intolerance and bloody stool were more common in preterm infants, while fever was more common in full-term infants (P<0.005). The genotype of rotavirus was determined for 121 (89.6%) cases, all of which had the G4[P6] strain. Conclusion: The type and clinical findings of rotavirus infection in preterm infants are different from those of full-term infants. G4[P6] was the only strain detected in neonatal infections regardless of where the infection occurred or the gestational age of patient.

위공장 경관식이(Gastrojejunal Tube Feeding) 환아에서 동반된 덤핑 증후군(Dumping Syndrome) 1례

이성혁,변준철,최원정,최순옥,황진복,Lee, Sung Hyuk,Byun, Jun Chul,Choi, Won Joung,Choi, Soon-Ok,Hwang, Jin-Bok 대한소아소화기영양학회 2005 Pediatric gastroenterology, hepatology & nutrition Vol.8 No.1

덤핑 증후군은 소아의 위공장 경관식이의 합병증으로 발생할 수 있는 질환으로, 19개월된 여아에서 식후 나타나는 특징적인 임상증상과 당부하 검사상 조기 고혈당 후 나타나는 후기 저혈당으로 진단할 수 있었으며, 생옥수수 전분을 포함한 식이요법만으로도 증상의 호전과 영양상태의 호전을 보일 수 있었다. Dumping syndrome is a known complication of gastric surgery in adults, but a very rare disease in the pediatric population. We report on a case of dumping syndrome in a 19-month-old child, who underwent gastrojejunal feeding tube insertion for the treatment and prevention of gastroesophageal reflux and frequent aspiration pneumonia. At 17 months of age, 2 months after the beginning of gastrojejunal tube feeding, postprandial diaphoresis, palpitation, lethargy, bloating, and diarrhea occurred, and a single episode of convulsion with hypoglycemia were noted. Early and late dumping syndrome was confirmed by an abnormal oral glucose tolerance test with early onset hyperglycemia followed by delayed onset hypoglycemia. Diet therapy including uncooked corn starch then improved the postprandial diaphoresis, abnormal glucose levels, and her nutritional status. We conclude that dumping syndrome may be considered as a complication of gastrojejunal tube feeding in a child.