• Clinical and Experimental Pediatrics
• /
• v.53 no.2
• /
• pp.158-162
• /
• 2010

Purpose : Turner syndrome (TS) is a disorder in which various anomalies can be accompanied, especially cardiovascular, renal, thyroid and auditory problems. The aim of this study is to identify the incidence of these disorders in patients with TS according to karyotype. Methods : We reviewed medical records of 90 patients with TS diagnosed by chromosomal analysis in 4 hospitals from Jan 1998 to Dec 2007. We evaluated these cases by prepared protocol of 4 medical problems.Results : The distribution of karyotype was 45,X (47.8%), mosaic pattern (34.4%) and structural aberration group (17.8%). Renal anomalies, cardiovascular anomalies, thyroid disorders and auditory problems are accompanied in 4.4%, 10.0 %, 11.1% and 5.6%, respectively. 45,X group had renal anomalies (7.0%), cardiovascular anomalies (18.6%), thyroid disorders (9.3%) and auditory problems (11.6%). Mosaic group had renal anomalies (3.2%), thyroid disorders (12.9%), no cardiovascular anomalies and auditory problems. Structural aberration group had cardiovascular anomalies (6.3%), thyroid disorders (12.5%) and no other 2 problems. Patients with 45,X group had a significant higher incidence of cardiovascular anomalies (P =0.025). Conclusion : Our results indicate that there are differences clinically according to karyotype of TS, especially in incidence of cardiovascular anomalies.

• Clinical and Experimental Pediatrics
• /
• v.53 no.2
• /
• pp.163-166
• /
• 2010

Purpose : Leigh syndrome is a typical type of mitochondrial disease. This study was conducted to analyze the types of ophthalmologic symptoms and results of funduscopy conducted in the ophthalmologic examination of patients with Leigh syndrome. Methods : Funduscopy was conducted on 24 subjects, who were chosen among those diagnosed as having mitochondrial respiratory chain complex defect and who were clinically suitable for the criteria of Leigh syndrome. Their clinical features, ophthalmologic symptoms, and ophthalmologic examination results were retrospectively analyzed. Results : Of the 24 patients with Leigh syndrome, 11 developed ophthalmologic symptoms and no abnormal finding was observed in 13. The most frequent abnormal finding was visual disturbance in 5 patients. Funduscopy revealed abnormal findings in 17 patients; retinal pigmentation was the most frequent abnormality and was seen in 9 patients. Conclusion : Funduscopy can be an important screening test to find ophthalmologic abnormalities among patients with mitochondrial disease (MD), including those patients whose ophthalmologic symptoms are inconspicuous. It is predicted that an improved screening test can be made in the future that will identify risk factors related to ophthalmologic symptoms.

• Journal of Korean Medical classics
• /
• v.18 no.2 s.29
• /
• pp.1-19
• /
• 2005

통과대명대저명의가(通過對明代著名醫家), 온보파창시자설기(溫補派創始者薛己)$(1487{\sim}1559)$적생애화의학사상(的生涯和醫學思想), 득출여하결론(得出如下結論): 설기생여의학세가(薛己生於醫學世家), 종소학습유학화의학(從小學習儒學和醫學), 기부사후(其父死後), 체부진입태의원성료의사(替父進入太醫院成了醫士), 지후선후역임태의원이목(之後先後歷任太醫院吏目), 남경태의원원판(南京太醫院院判), 원사등직(院使等職), 병어1540년(44세)기관회향(幷於1540年(44歲)棄官回鄕), 전심여치료환자여저술(專心於治療患者與著述). 설기적저작대부분내(薛己的著作大部分內) 용이의안형식서사(容以醫案形式書寫), 기내용포함내과(其內容包含內科), 외과(外科), 부산과(婦産科), 소아과(小兒科), 침구과(鍼灸科), 치과(齒科), 안과(眼科), 골과(骨科), 본초등(本草等). 기본인친(其本人親) 자저술적유이(自著述的有以)${\ulcorner}$내과적요(內科摘要)${\lrcorner}$위시약12종(爲始約12種), 교주적유이(校注的有以)${\ulcorner}$부인대전양방(婦人大全良方)${\lrcorner}$위시8종(爲始8種), 교감적의서약유6종(校勘的醫書約有6種), 공계대약(共計大約) 유26종지다(有26種之多). 설기수(薛己受)${\ulcorner}$내경(內經)${\lrcorner}$, ${\ulcorner}$난경(難經)${\lrcorner}$급장원소화금원사대가적영향교심(及張元素和金元四大家的影響較深). 재사대가중(在四大家中), 우기수동원화단계적영향최심(尤其受東垣和丹溪的影響最深). 차외(此外), 가지기수교주의서적영향역교대(可知其受校注醫書的影響亦較大), 기통과박람군서(其通過博覽群書), 대담실천(大膽實踐), 광수자료(廣收資料), 선우총결(善于總結), 최종성료일명의가(最終成了一名醫家). 설기수이동원(薛己受李東垣)${\ulcorner}$비위론(脾胃論)${\lrcorner}$적영향(的影響), 강조비위적중요성(强調脾胃的重要性), 병수차제출비통혈적이론(幷首次提出脾統血的理論) 기여이동원적학설부동지처시(其與李東垣的學說不同之處是), 재치료화쇠토약형허한증시(在治療火衰土弱型虛寒證時), 주장이보화생토지법내치료(主張以補火生土之法來治療), 인위비여신적생리작용호위관련(認爲脾與腎的生理作用互爲關聯), 비토화생정미(脾土化生精微), 이자양신여명문(以滋養腎與命門) 반과래(反過來), 신여명문역대위토기온후작용(腎與命門亦對緯土起溫煦作用); 이재병리방면(而在病理方面), 비토구허(脾土久虛), 가치신허(可致腎虛), 신허즉불능생토(腎虛則不能生土), 역가치비허(亦可致脾虛). 설기적극반대명대의가수금원지유풍(薛己積極反對明代醫家受金元之遺風), 이한양약공하(以寒凉藥攻下), 혹남용지백이강화(或濫用知柏以降火), 극벌생기적작법(克伐生氣的作法), 이주장용편온적약물진행조보(而主張用偏溫的藥物進行調補), 기상용적전인처방유보중익기탕(其常用的前人處方有補中益氣湯), 사물탕(四物湯), 사군자탕(四君子湯), 육군자탕(六君子湯), 독삼탕(獨蔘湯), 삼부탕(蔘附湯), 육미환(六味丸), 팔미환(八味丸), 신기환(腎氣丸), 회양탕(回陽湯), 당귀보혈탕등(當歸補血湯等).

• The Korean Nurse
• /
• v.36 no.2
• /
• pp.73-83
• /
• 1997

Bed rest is recommended to prevent postlumbar puncture headaches(PLPHA), but the period of bed rest varies in the literature from 6 hours to 24 hours. In clinical practice the period of bed rest varies but nursing methods for adults and children have little difference. In Seoul National University Hospital, children have been given at least 6 hours bed rest after a lumbar puncture. Pediatric oncology patients require a lumbar puncture for an initial diagnosis, follow up treatment or administration of chemotherapeutic agent. But it is difficult for young children to lie supine or to refrain from their usual activities in any way, and unpleasant problems related to a shortage of beds often occurs during discharge or in an outpatient setting. The purpose of this study is to substantiate the preventive effect of PLPHA by the period of bed rest, to identify the other factors that influence PLPHA, and to use the nursing methods proper to children. The subjects were 65 children, ages 1-17, undergoing treatment in the children's cancer center at SNUCH during the period June 1, 1995, to Aug. 31, 1995. The team nurses asked questions about PLPHA of the parents and children in order to fill out a questionnaire. The data were evaluated by percent, t-test, Chi-square test and Mann-Whitney U test. Result; 1. There was no significant difference relating the bed rest time spent to the occurrence of postspinal headaches (t-test). 2. There was a significant risk of PLPHA in the children who were irritable before procedure and/or had experienced previous PLPHA(p<0.05, ${x^2}-test$). 3. The following factors were not found to be associated with increased risk of PLPHA: previous puncture experience, giving analgesics, the choice of puncturist, inpatient/outpatient status, gauge of needle, purpose, the amount of CSF removed, gender, diagnosis, the number of peripheral WBCs, previous lumbago experience after LP, position after bed rest, age, the number of aural puncture at the time. A longer period of bed rest is unlikely to be more effective to prevent PLPHA and seems impractical. A shorter period will save time and effort. Perhaps it will also allay some of the fears which surround LP. So 1 hour bed rest after LP is suggested and nursing methods for emotional support should be investigated to reduce PLPH.

• Clinical and Experimental Pediatrics
• /
• v.50 no.10
• /
• pp.982-986
• /
• 2007

Purpose : Intravenous immunoglobulin (IVIG) is effective for the treatment of idiopathic thrombocytopenic purpura (ITP) in children. Recently, several reports have been published that show its impact on the absolute neutrophil count. The present study was performed to confirm these findings. Methods : Data on 26 ITP patients were analyzed. Patients with febrile illness or increased C-reactive protein levels at presentation, which would influence the neutrophil counts, were excluded to determine the sole impact of IVIG. In addition, patients who received steroid treatment were also excluded. Results : Sixteen boys and ten girls were analyzed. For patients who received an IVIG dose of 0.4 g/kg/day (n=17), the absolute neutrophil count (ANC) measured next day was significantly decreased. For patients who received an IVIG dose of 1 g/kg/day (n=9), the ANC measured the next day was also significantly decreased. However, the decrease was more profound in the high-dose group compared to the low-dose group. Among six cases with profoundly decreased ANC greater than $1,000/mm^3$, four patients (67%) received IVIG at a dose of 1 g/kg/day. All four cases with increased ANC were treated with IVIG dose of 0.4 g/kg/day, and three cases (75%) among them had a febrile reaction during IVIG administration. None of the cases with decreased ANC had a febrile reaction. No cases had infectious complications reported. Conclusion : IVIG treatment for ITP patients appears to suppress the ANC. This decrease of ANC was more pronounced when a higher dose of IVIG was used. Some cases with increased ANC counts after IVIG use were found only in low-dose IVIG group, and was associated with febrile reactions during IVIG use.

• The Academic Congress of Korean Shoulder and Elbow Society
• /
• 1995.05a
• /
• pp.7-7
• /
• 1995

최근에 스키가 동계스포츠로 각광받으면서 스키인구가 폭발적으로 증가함에 따라 스키손상도 많이 발생하게 되어 스키손상은 스포츠외상에서 중요한 비중을 차지하게 되었다. 스키인구의 증가에 따라 스키손상에 대한 정확한 분석과 이에 대한 예방대책이 절실히 요구되며 이에 경희대학교 의과대학 정형외과학 교실에서는 1982년 12월부터 1995년 3월까지( 1986년과 1987년을 제외 ) 11년간 용평스키장내에 동계스포츠클리닉을 개설하여 상기 기간 중 내장한 총 스키인구 3,093,667명중 스키손상으로 동계스포츠클리닉에 내원한 7,172명의 환자$(0.23\%)$에 대하여 스키손상의 년도별 발생빈도, 스키손상의 특징 스키골절과 탈구의 발생빈도 및 특징 등에 대하여 11년 간의 변화추세를 분석하였다. 11년 간의 스키장의 내장객수는 1983년을 기준 (83,100)으로 하였을 때 1995년에 $977\%로 국부 큰 증가를 보였으며, 스키인구 1000명당 손상율은 1983년의 3.4에서 1995년의 1.4(평균 2.3)로 감소하는 추세를 보였다. 스키손상의 형태는 단순열상 및 타박상등을 제외하고 슬관절 인대손상이 $22.6\%로 가장 많이 발생하였으며, 조사기간동안 수치상의 의미 있는 변화는 없었다. 스키골절 및 탈구는 스키인구 1000명당 평균 0.35였으며, 1983년의 0.90으로부터 점차 감소하여 1995년에는 0.21에 이르렀다. 연령별 스키골절 및 탈구는 20세 이하에서 평균 $41.6\%로 전체적으로 성인에서 더 많이 발생하였으며, 이는 처음 3년 간 20세 이하 골절 및 탈구가 $70.5\%로 높은 발생빈도를 보였으나 1995년도의 20세 이하 골절 및 탈구가$30.3\%까지 감소하게되어 년도가 증가함에 따라 청장년 층에 비하여 소아골절 및 탈구가 전체적으로 감소하는 경향을 보였다. 스키골절의 부위별 발생빈도는 1990년 이전까지 하지골절 및 탈구가 많았으나 이후 점차 상지의 골절 탈구가 증가하였다 하지에서 가장 많은 골절은 경골 골절이었으며, 경골골절은 회전력에 의한 나선형골절이 $76.5\%로 가장 많았고 년도에 따른 변화는 보이지 않았다. 스키손상의 발생빈도는 초기에 비하여 점차 감소하는 경향을 보였으며, 손상의 특성도 부위별, 연령별로 다양한 변화를 나타내었다.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.13 no.1
• /
• pp.86-91
• /
• 2010

Retroperitoneal cystic lymphangiomas are benign, extremely rare tumors. Although surgical resection is the treatment of choice, sclerotherapy should be considered initially. A 9-year-old boy was admitted due do worsening abdominal pain of 4 days duration. Serial complete blood counts revealed a hemoglobin level of 12.8 g/dL on admission to 10.6 g/dL on hospital day 3. An abdominal computed tomography (CT) scan showed a large, lobulated, septated, retroperitoneal cystic mass ($10{\times}9.5{\times}5cm$) in the left anterior pararenal space with intracystic hemorrhage surrounding the inferior mesenteric vein (IMV). Because of the high operative risk, we performed a tubogram of the cystic mass, percutaneous catheter drainage (PCD), and ethanol sclerotherapy. The follow-up abdominal CT scan showed that the cystic mass had decreased in size. He is well without relapse of the retroperitoneal cystic mass for 13 months after discharge. Sclerotherapy with PCD should be considered as initial therapy for patients with retroperitoneal cystic lymphangiomas at high surgical risk.

• Childhood Kidney Diseases
• /
• v.11 no.2
• /
• pp.294-298
• /
• 2007

Moyamoya disease is a progressive cerebrovascular disorder with stenosis or occlusion of the bilateral internal carotid arteries with abnormal vascular networks at the base of the brain. Previous reports have shown that there are extracranial vascular involvements in Moyamoya disease, especially in the renal artery. We report a 7-year-old patient with Moyamoya disease associated with renovascular hyper tension, who presented in infancy with seizures and hemiparesis. Renal angiography showed multiple stenoses of the right renal artery. Although renal artery stenosis in Moyamoya disease has been effectively treated with balloon angioplasty, stent implantation, or surgery, bat-loon angioplasty could not be done in this patient due to multiple stenoses. His blood pressure was successfully controlled with medical treatment, and remained normotensive during the follow up period of 6 months.

• Childhood Kidney Diseases
• /
• v.18 no.2
• /
• pp.128-131
• /
• 2014

Histopathologic evidence of "full-house" immune complex deposits is a pathognomonic feature of lupus nephritis. This report presents the case of a 12-year-old boy with persistent microscopic hematuria and proteinuria. He was diagnosed with "full-house" nephropathy based on a renal biopsy. However, there was no other clinical or biological evidence of systemic lupus erythematosus (SLE). Although the potential for isolated "full-house" nephropathy preceding SLE is unclear, such patients should be followed for clinical signs and autoantibodies of SLE. In most cases, microscopic hematuria has a good prognosis, and follow-up usually requires only regular urinalysis. However, we should be aware of isolated "full-house" nephropathy that remains asymptomatic for a long time, as few patients with no clinical signs and negative serology ultimately develop SLE.

• Journal of Life Science
• /
• v.21 no.6
• /
• pp.845-850
• /
• 2011

Norovirus (NoV) causes major acute non-bacterial gastroenteritis in humans. NoV genus is a member of the family Caliciviridae, which is transmitted by contaminated food and water or from human to human. Many genotypes of genogroups I and II have been reported because of their high genetic diversity. To obtain molecular epidemiological information on gastroenteritis sporadic cases in Busan, Korea, we analyzed the nucleotide sequences of NoV strains detected during 2008~2010. We performed one step RT-PCR amplifying the open reading frame (ORF) 2 (capsid region) followed by semi-nested PCR. Fecal samples were collected from 4,071 acute gastroenteritis, and genotypes of the 421 positive samples were determined by sequence analysis. Based on partial sequence of capsid region, 7 NoV were categorized into genogroup I and 13 into genogroup II. Prevalent genotypes among gastroenteritis patients within Busan were GII.4, GI.6, GII.5 in 2008~2010. The results of this study will contribute to the currently available epidemiological data and improve public health and hygiene via development of diagnostic methods and sustainable surveillance.