• Clinical and Experimental Pediatrics
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• 제56권7호
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• pp.286-290
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• 2013

Purpose: Although chronic and recurrent rhinosinusitis is prevalent in children, little is known about its causes. Here, we investigated the humoral immunity in children with chronic or recurrent rhinosinusitis. Methods: We examined 16 children attending the outpatient clinic at the CHA Bundang Medical Center including 11 boys and 5 girls, aged 3-11 years (mean age, 5.6 years), who had rhinosinusitis for >3 months or >3 times per year. The complete blood count with differential and total serum concentrations of Immunoglobulin (Ig) E, IgA, IgD, IgM, IgG, and IgG subclasses ($IgG_1$, $IgG_2$, $IgG_3$, and $IgG_4$) of all children were measured. All subjects received 23-polysaccharide pneumococcal vaccination (PPV), and the levels of antibodies to 5 serologic types (4, 6B, 14, 18C, and 23F) of pneumococcal capsular polysaccharide antigens were measured before and after vaccination. Post-PPV antibody titers ${\geq}0.35{\mu}g/mL$ or with a ${\geq}4$-fold increase were considered as positive responses. Results: The titers of IgG, IgA, IgD, and IgM were within normal range in all 16 children, whereas the total IgE concentration was higher than normal in 2 children. $IgG_1$ deficiency was observed in 1 patient and $IgG_3$ deficiency in 3. After PPV, 1 patient failed to respond to all 5 serologic types, 2 failed to respond to 4 serologic types, and 2 failed to respond to 3 serologic types. Conclusion: Clinicians should consider the evaluation of humoral immune functions in children with chronic or recurrent rhinosinusitis who do not respond to prolonged antibiotic treatment.

• 대한소아치과학회지
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• 제36권4호
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• pp.575-579
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• 2009

베체트병은 전신적 혈관염으로 여러 장기에 걸쳐 나타날 수 있으며, 현재로선 확진을 위한 검사 방법이 없으므로 병의 진단은 임상적 증상에 기초하여 내려진다. 구강 및 외음부의 재발성 궤양, 피부 병소, 안구 병소, 및 이상초과민검사(pathergy test)에 대한 반응을 기초로 진단을 내리며, 이 외에도 위장관과 중추신경계에 증상이 나타날 수 있다. 이 중 재발성 구강 궤양은 대부분의 베체트병 환자에서 나타나며, 많은 경우 베체트병의 첫번째 임상 증상으로 발현되기 때문에 치과 영역은 베체트병의 조기 진단에 있어서 중요한 위치에 있다. 본 증례에서 6세 여환은 반복되어 나타나는 재발성 구강 궤양으로 인한 동통 및 섭식 장애를 주소로 본원 소아치과에 내원하였다. 문진 및 병력 채취, 그리고 임상 검사를 통해 치과 영역에서 베체트병을 조기 진단할 수 있었고, 추후 환자는 소아과, 피부과, 소화기내과, 안과와의 협의 진료 하에 베체트병으로 확진되었다. 치과에선 환자의 동통을 완화시키고 섭식을 돕기 위해 도포마취제를 처방하였으며, 추후 베체트병의 확진 하에 그에 따른 적절한 의과적 치료를 시행할 수 있었기에 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제49권3호
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• pp.298-304
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• 2006

목 적 : 소아 천식이 급격히 증가하고 있다. 영유아 시기의 천명을 보이는 환아들에서 천식 발생을 예측할 수 있는 고위험군을 선별하는 지표들이 알려져 있다. 저자들은 Tucson Children's Respiratory Study Group에서 제시한 천식 예측 지표 API와 변형한 천식 예측 지표 mAPI를 한국 영유아 천명 환아들에게 적용하여 의미있는 예측 지표를 규명하고자 하였다. 방 법 : 반복성 천명을 주소로 서울아산병원 소아과를 방문한 262명의 환아들을 대상으로 API와 mAPI를 적용하여 고위험군과 저위험군으로 나누었다. 각 군에서 알레르기 지표 뿐만 아니라 환아와 가족들의 알레르기 질환의 병력을 조사하였다. 결 과 : API 고위험군 환아(n=131)는 저위험군 환아(n=131) 보다 성별은 차이가 없었고 연령, 총호산구수, 호산구 비율, 혈중 ECP, 총 IgE가 높았으며, 흔한 항원에 대한 감작 여부가 포함되지 않았지만 흡입항원과 식품항원에 대한 감작률이 더 높게 나타났다. mAPI 고위험군 환아(n=141)는 저위험군 환아(n=121) 보다 총호산구수, 호산구 비율, 혈중 ECP, 총 IgE, egg, milk, D.p-, D.f-특이 IgE 농도가 높았으며 성별과 연령의 차이는 없었다. 위험군별 다변량 분석에서는 API군에서는 흡입항원에 대한 감작 여부가 고위험군을 결정하는 유의한 지표로 나타났으며 mAPI군에서는 혈중 ECP 농도가 유의한 지표로 나타났다. 대상군 중 6세 이상 추적 관찰된 환아는 모두 29명이었으며 최근 1년간 천명의 병력이 확인되고 임상적으로 천식으로 진단된 환아는 15명이었다. API의 민감도, 특이도, 양성예측률, 음성예측률은 66.7%, 50.0%, 58.8%, 58.3%이었으며, mAPI는 93.3%, 57.1%, 70.0%, 88.9%로서 mAPI가 더 높았다. 결 론 : API와 mAPI를 적용한 고위험군 환아에서 흔한 흡입 항원과 식품항원에 대한 감작률이 높아서, 영유아 천명 환아에서 주관적인 증상에 의한 진단보다는 객관적인 흡입항원과 식품항원에 대한 감작 여부를 지표로 사용하는 것이 더 의미 있을 것으로 생각한다. 또한 한국 영유아 천명 환아에서는 mAPI가 소아 천식의 발생을 예측하는데 더 도움이 될 것으로 생각한다. 그러나 본 연구는 29명의 소수의 환아들만 추적 관찰된 결과이므로 이후로 보다 더 많은 환아들을 추적 관찰하는 것이 필요할 것이다.

• BMB Reports
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• 제53권4호
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• pp.234-239
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• 2020

Epilepsy is a chronic neurological disease characterized by spontaneous recurrent seizures and caused by various factors and mechanisms. Malfunction of the olfactory bulb is frequently observed in patients with epilepsy. However, the morphological changes in the olfactory bulb during epilepsy-induced neuropathology have not been elucidated. Therefore, in the present study, we investigated the expression of parvalbumin (PV), one of the calcium-binding proteins, and morphological changes in the rat main olfactory bulb (MOB) following pilo-carpine-induced status epilepticus (SE). Pilocarpine-induced SE resulted in neuronal degeneration in the external plexiform layer (EPL) and glomerular layer (GL) of the MOB. PV immunoreactivity was observed in the neuronal somas and processes in the EPL and GL of the control group. However, six hours after pilocarpine administration, PV expression was remarkably decreased in the neuronal processes compared to the somas and the average number of PV-positive interneurons was significantly decreased. Three months after pilocarpine treatment, the number of PV-positive interneurons was also significantly decreased compared to the 6 hour group in both layers. In addition, the number of NeuN-positive neurons was also significantly decreased in the EPL and GL following pilocarpine treatment. In double immunofluorescence staining for PV and MAP2, the immunoreactivity for MAP2 around the PV-positive neurons was significantly decreased three months after pilocarpine treatment. Therefore, the present findings suggest that decreases in PV-positive GABAergic interneurons and dendritic density in the MOB induced impaired calcium buffering and reciprocal synaptic transmission. Thus, these alterations may be considered key factors aggravating olfactory function in patients with epilepsy.

• Anatomy and Cell Biology
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• 제51권4호
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• pp.266-273
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• 2018

The ganglion cardiacum or juxtaductal body is situated along the left recurrent laryngeal nerve in the aortic window and is an extremely large component of the cardiac nerve plexus. This study was performed to describe the morphologies of the ganglion cardiacum or juxtaductal body in human fetuses and to compare characteristics with intracardiac ganglion. Ganglia were immunostained in specimens from five fetuses of gestational age 12-16 weeks and seven fetuses of gestational age 28-34 weeks. Many ganglion cells in the ganglia were positive for tyrosine hydroxylase (TH; sympathetic nerve marker) and chromogranin A, while a few neurons were positive for neuronal nitric oxide synthase (NOS; parasympathetic nerve marker) or calretinin. Another ganglion at the base of the ascending aorta carried almost the same neuronal populations, whereas a ganglion along the left common cardinal vein contained neurons positive for chromogranin A and NOS but no or few TH-positive neurons, suggesting a site-dependent difference in composite neurons. Mixtures of sympathetic and parasympathetic neurons within a single ganglion are consistent with the morphology of the cranial base and pelvic ganglia. Most of the intracardiac neurons are likely to have a non-adrenergic non-cholinergic phenotype, whereas fewer neurons have a dual cholinergic/noradrenergic phenotype. However, there was no evidence showing that chromogranin A- and/or calretinin-positive cardiac neurons corresponded to these specific phenotypes. The present study suggested that the ganglion cardiacum was composed of a mixture of sympathetic and parasympathetic neurons, which were characterized the site-dependent differences in and near the heart.

• Asian Pacific Journal of Cancer Prevention
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• 제16권15호
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• pp.6663-6668
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• 2015

Background: Treatment failure in leukemia is due to either pharmacokinetic resistance or cell resistance to drugs. Materials and Methods: Gene expression of multiple drug resistance protein (MDR-1), multidrug resistance-related protein (MRP) and low resistance protein (LRP) was assessed in 45 pediatric ALL cases and 7 healthy controls by real time PCR. The expression was scored as negative, weak, moderate and strong. Results: The male female ratio of cases was 2.75:1 and the mean age was 5.2 years. Some 26/45 (58%) were in standard risk, 17/45(38%) intermediate and 2/45 (4%) in high risk categorie, 42/45 (93%) being B-ALL and recurrent translocations being noted in 5/45 (11.0%). Rapid early response (RER) at day 14 was seen in 37/45 (82.3%) and slow early response (SER) in 8/45 (17.7%) cases. Positive expression of MDR-1, LRP and MRP was noted in 14/45 (31%), 15/45 (33%) and 27/45 (60%) cases and strong expression in 3/14 (21%), 11/27 (40.7%) and 8/15 (53.3%) cases respectively. Dual or more gene positivity was noted in 17/45 (38%) cases. 46.5 % (7/15) of LRP positive cases at day 14 were in RER as compared to 100% (30/30) of LRP negative cases (p<0.05). All 8 (100%) LRP positive cases in SER had strong LRP expression (p=<0.05). Moreover, only 53.3% of LRP positive cases were in haematological remission at day 30 as compared to 100% of LRP negative cases (p=<0.05). Conclusions: Our study indicated that increased LRP expression at diagnosis in pediatric ALL predicts poor response to early treatment and hence can be used as a prognostic marker. However, larger prospective studies with longer follow up are needed, to understand the clinical relevance of drug resistance proteins.

• 대한정형외과스포츠의학회지
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• 제9권2호
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• pp.98-103
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• 2010

목적: 슬개골 재발성 탈구 환자에 있어 관절경 보조하의 외측 유리술 및 내측부 중첩술의 결과를 보고하고자 한다. 대상 및 방법: 재발성 슬개골 탈구로 수술적 치료를 받은 20 명의 환자, 21례를 대상으로 하였다. 남자가 4 명, 여자가 16명이고 평균연령 20.2세였다. 모든 환자가 외상의 병력이 있었고 평균 추시 기간은 19개월이었다. 수술 결과의 평가는 Lysholm점수와 Kujala슬관절 점수를 이용하였고, 방사선 사진상 congruence angle, lateral patellofemoral angle을 측정하였고, 전산화 단층촬영에서 tibial tuberosity-trochlear groove distance를 측정하였다. 결과: 단순 방사선 사진상 congruence angle은 술전 중위값 $16.5^{\circ}$ (범위, $0.0{\sim}+34^{\circ}$) 에서 추시 시 $-6.4^{\circ}$ (범위, $-19{\sim}10^{\circ}$), lateral patellofemoral angle은 술전 중간값 -4.5도(범위, $-8{\sim}+6^{\circ}$)에서 $4.0^{\circ}$ (범위, $2.0{\sim}10^{\circ}$)로 호전되었다(p=0.034). Lysholm 점수는 수술 전 중위값 70점(범위, 63~81점)에서 최종 추시 시 중위값 88점(범위, 80~95점)이었다(p=0.341). Kujala 슬관절 점수는 수술 전 중위값 72점(범위,65~80점)에서 수술 후 중위값 87점(범위, 80~92점)으로 호전되었다. (p=0.024). 재발성 탈구는 총 2례에서 발생하였으며 1례는 thumb to forearm검사 양성이었던 경우로 재탈구 되어 내측 슬개대퇴인대 재건술을 시행하였다. 결론: 관절경적 외측 유리술 및 내측부 중첩술은 대퇴슬개관절의 골성 부정정렬이 없는 경우에서 시행하여 좋은 결과를 얻을 수 있었지만, 관절 이완이 심한 환자에서는 좀 더 주의가 필요하다고 사료된다.

• 대한후두음성언어의학회지
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• 제20권2호
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• pp.136-140
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• 2009

Background and Objectives: Recurrent respiratory papillomatosis (RRP) is difficult to treat because of its tendency to recur and spread throughout the aerodigestive tract. We aimed to estimate the effect of intralesional injections of cidofovir in patients with RRP. Materials and Method: Within the period from January 2003 to July 2007, 13 patients aged 2 to 61 years were treated with intralesional injections of cidofovir combined with surgical excision of RRP. Cidofovir was injected intralesionaly at a concentration of 5 mg/cc after complete removal of the papilloma with $CO_2$ laser or microdebrider. We evaluated the effect of intralesional cidofovir therapy by comparing pre-treatment mean interval of recurrence with post-treatment interval of recurrence. Results: Of 13 patients, two patients showed complete response during follow up period and four patients showed partial response. Seven patients did not respond to cidofovir at all. Mean pre-treatment mean interval of recurrence was 9 months and mean post-treatment interval of recurrence was 13.1 months (p=0.039). There was a statistical significance between the injected dose of cidofovir and post-treatment interval of recurrence (p=0.009). There were no local or systemic side effects caused by cidofovir. Conculsion : Intralesional injection of cidofovir seems to have a potential of a safe and effective adjuvant therapy of RRP. There was a positive correlation between the injected dose of cidofovir and patient clinical outcomes so that administration of higher doses and more frequency of injections should be needed to reduce recurrence. Further study regarding injection therapy regimen for RRP is required.

• Journal of Chest Surgery
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• 제9권2호
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• pp.265-270
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• 1976

Implantation of a permanent pacemaker is a widely accepted procedure for the patient with complete heart block.As a result of these device, the prognosis for patients with Adams-Stokes syndrome caused by complete A-V block and other cardiac arrhythmia have become much more optimistic. Permanent pacemaker implantation by means of a transvenous approach has made the operative risk much less and the procedure simpler. However, a number of complications have been reported in the literature regarding transvenous endocardial pacemaker implantation during the last a decade. The patient presented in this paper is a 26-year old girl who was implanted with a permanent pacemaker at 14 years of age because of a congenital A-V block. Following first exchange of pulse generator, the electrode (lead) was fractured, so that by the pulse generator, a change to the transvenous technique of implantation was made, After this, there were episodes of recurrent wound infection on three occasions, even though the site of pulse generator implantation was exchanged to the contralateral side of chest wall, massive doses of antibiotics were administered and sensitivity tests for coagulase positive staphylococcal infection were performed. Though there was no definite evidence of blood stream infection by blood culture, we decided not to use the transvenous technique and not to implant the pulse generator in the chest wall because the venous system and the entire anterior chest wall appeared to be diseased or contaminated by virulent pyogenic organisms. Finally this intractable systemic and local wound infection was successfully controlled by myocardial lead implantation via a subxiphoid approach and implantation of the pulse generator far down in the abdominal wall. The causes and routes of recurrent wound infection and possible blood born infection in this particular patient are still obscure. We strongly believe that myocardial pacemaker implantation is much safer than transvenous endocardial pacemaker implantation & myocardial pacemaker implantation is a definite method for controlling such an intractable wound infection. following transvenous pacemaker implantation.

• Clinical and Experimental Pediatrics
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• 제49권10호
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• pp.1111-1115
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• 2006

유전성 췌장염은 비교적 젊은 연령에서 다른 이유 없이 반복적으로 급성 췌장염으로 나타나는데 나이가 들면서 만성 췌장염으로 이행된다. 동일 가계 내에 2세대 이상에 걸쳐서 3명 이상의 췌장염 환자가 있을 때 진단이 가능하며 이와 관련된 유전자로 trypsinogen을 만드는 PRSS1 유전자 변이(R122H, N29I)가 가장 대표적으로 알려져 있다. 저자들은 3세부터 반복적인 췌장염으로 입원 치료를 했던 15세 환아와 반복적 췌장염을 앓은 환아모, 환아 동생을 대상으로 CT 유전자의 exon 2, 3의 염기 서열을 분석하여 환아와 환아모에서 국내 처음으로 N29I 변이를 경험하였기에 보고하는 바이다.