Earticle

The rapid dissemination of extended-spectrum ${\beta}$-lactamase (ESBL)-producing Escherichia coli has significantly contributed to public health hazard globally. A total of 281 E. coli strains recovered from pigs and chickens between 2009 and 2015 in South Korea were analyzed for ESBL production. ESBL phenotypes were recognized in 14 E. coli isolates; ten and three ESBL-producing isolates carried only $bla_{CTX-M}$ and $bla_{CMY}$ genes, respectively, and one isolate harbored both genes. The predominant CTX-M and CMY types were CTX-M-15 (n = 8) and CMY-2 (n = 3). We also detected ESBL-producing isolates harboring $bla_{CTX-M-65}$, $bla_{CTX-M-14}$, $bla_{CMY-6}$, $bla_{DHA-1}$, and $bla_{TEM-1}$ genes. All ESBL-producing isolates showed resistance to the extent of the fourth generation cephalosporins, along with multidrug resistance. CTX-M-15-producing isolates showed higher MIC values than CTX-M-14- and CTX-M-65-producing isolates. The $bla_{CTX-M}$ and $bla_{CMY}$ genes have the potential to be transferable. The spreading of $bla_{CMY}$ and $bla_{CTX-M}$ genes was arbitrated mainly via Frep and IncI1 plasmids. Our isolates showed clonal diversity in PFGE analysis. This is the first report of E. coli isolates carrying $bla_{CMY-6}$ in chicken from South Korea. The emergence of CMY-6 ESBLs in a population of poultry suggests that extensive screening with long-term surveillance is necessary to prevent the dissemination of ESBL from chicken to human.

Purpose: Duodenal atresia (DA) and atrioventricular septal defect (AVSD) are well known ultrasonographic findings associated with Down syndrome. The risk of Down syndrome in fetuses with these anomalies has been reported as 30% to 40%. However, on the basis of our clinical experience, the risk of Down syndrome of DA may be lower in Korean population. To clarify this issue, we compared the risk of Down syndrome between cases with DA and AVSD. Materials and Methods: The study population consisted of neonates who were confirmed as DA or AVSD by postnatal diagnosis. Postnatal diagnosis was made by surgery, postnatal echocardiography, or autopsy. Medical record was reviewed retrospectively. Results: A total of 213 neonates with DA or AVSD were included: 67 cases with DA and 146 cases with AVSD. The risk of Down syndrome was 4.5% (3/67) in DA vs. 29.5% (43/146) in AVSD. When confining analysis to those whose karyotyping were not performed during antenatal period, the risk of Down syndrome were 7.9% (3/38) in DA and 35.4% (35/99) in AVSD. Conclusion: The risk of Down syndrome in cases with DA was much lower in Korean population than previously reported risk in the literature. The significance of some antenatal sonographic markers for Down syndrome may be different according to ethnicity.

Harlequin ichthyosis (HI, OMIM #242500) is one of the most severe skin diseases among the autosomal recessive congenital ichthyoses, with high morbidity and mortality, particularly in newborns. Clinically, it is characterized by a typical appearance of generalized, thick, yellowish, hyperkeratotic plates with deep erythematous fissures on the skin. Herein, we present the case of a newborn girl with HI that was genetically confirmed by targeted gene panel analysis. The premature baby was encased in an opaque white membrane with erosion covering the skin of the entire body except the lips, with her hands and feet restricted by the membrane. Humidification, emollient, and retinoic acid treatment were started; the thick ichthyosis gradually peeled off and the underlying skin was only covered with thin scales. Targeted gene panel analysis using next-generation sequencing and validation with Sanger sequencing and quantitative polymerase chain reaction analyses confirmed compound heterozygous mutations of the ABCA12 gene (p.N1380S and a partial gene deletion encompassing exon 9). The parents were carriers for each of the identified mutations. Early recognition of the genetic etiology of congenital ichthyosis can, thus, facilitate genetic counseling for patients and their families.

Purpose: The purpose of this study was to evaluate the efficacy and safety of Montelukast sodium in the prevention of bronchopulmonarydysplasia (BPD). Methods: The Interventional study was designed as a multicenter, prospective, and randomized trial, with open labeled and parallel-experimental groups, 66 infants were enrolled and allocated to either the case group (n=30) or the control group (n=36) based on gestational age (GA). Infants in the case group were given Montelukast sodium (Singulair) based on their body weight (BW). Zero week was defined as the start time of the study. Results: The incidence of moderate to severe BPD was not different between the groups (case group: 13 of 30 [43.3%] vs. control group: 19 of 36 [52.8%], P=0.912). Additionally, secondary outcomes such as ventilation index, mean airway pressure and resort to systemic steroids were not significantly different. There were no serious adverse drug reactions in either group, and furthermore the rate of occurrence of mild drug related-events were not significantly different (case group: 10 of 42 [23.8%] vs. control group: 6 of 48 (15.8%), P=0.414). Conclusion: Montelukast was not effective in reducing moderate or severe BPD. There were no significant adverse drug events associated with Montelukast treatment.

Mycobacterium avium subsp. paratuberculosis (MAP) is the causative agent of Johne's disease, a chronic debilitating disease affecting ruminants worldwide. In the present study, we aimed to determine the major gene networks and pathways underlying the immune response to MAP infection using whole-blood cells, as well as provide the potential transcriptional markers for identifying the status of MAP infection. We analyzed the transcriptional profiles of whole-blood cells of cattle identified and grouped according to the presence of MAP-specific antibodies and the MAP shed by them. The grouping was based on the results obtained by ELISA and PCR analyses as follows: i) Test1 group: MAP-negative results obtained by ELISA and positive results obtained by PCR; ii) Test2 group: MAP-positive results obtained by ELISA and negative results obtained by PCR; iii) Test3 group: MAP-positive results obtained by ELISA and positive results obtained by PCR; iv) uninfected control: MAP-negative results obtained both by ELISA and PCR analysis. The results showed down-regulated production and metabolism of reactive oxygen species in the Test1 group, activation of pathways related to the host-defense response against MAP (LXR/RXR activation and complement system) in the Test2 and Test3 groups, and anti-inflammatory response (activation of IL-10 signaling pathway) only in the Test3 group. Our data indicate a balanced response that serves the immune-limiting mechanism while the host-defense responses are progressing.

Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at $37^{+1}$ weeks of gestation with a birth weight of 1,690 g (<3rd percentile). Prenatal ultrasonographic findings revealed intrauterine growth retardation and skeletal dysplasia. At birth, the patient had characteristic features of ML II, and skeletal radiographs revealed dysostosis multiplex, similar to rickets. In addition, the patient had high levels of alkaline phosphatase and parathyroid hormone, consistent with severe secondary neonatal hyperparathyroidism. The activities of ${\beta}$-D-hexosaminidase and ${\alpha}$-N-acetylglucosaminidase were moderately decreased in the leukocytes but were 5- to 10-fold higher in the plasma. Examination of a placental biopsy specimen showed foamy vacuolar changes in trophoblasts and syncytiotrophoblasts. The diagnosis of ML II was confirmed via GNPTAB genetic testing, which revealed compound heterozygosity of c.3091C>T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.

목 적 : 신생아기와 조기 영아기에 발생한 기계환기요법이 필요했던 중증 respiratory syncytial virus (RSV) 감염의 임상적 특성을 고찰하여 그 임상적 특성을 파악함으로써 이 질환의 진료에 도움이 되는 임상 정보를 제공하고자 하였다. 방 법 : 2005년 3월부터 2007년 7월까지 분당서울대학교병원 중환자실에 RSV 감염으로 입원한 6개월 미만의 신생아와 영아들 중 기계환기요법을 필요로 했던 12명의 의무기록을 열람하여 임상적 특성을 분석하였다. RSV 감염은 환아의 비인두흡인물 또는 경기관지흡인액 내에서 RSV 항원이 효소면역법 또는 PCR 방법으로 검출된 경우로 하였다. 결 과 : 입원 시 환아들의 나이는 $35{\pm}15$일(범위, 14-59일)이었고 모두 RSV 유행기인 9월부터 3월 사이에 입원하였다. 환아들의 출생체중은 $2.8{\pm}0.6kg$, 재태연령은 $37{\pm}2$주였다. 12명의 환아들 중 4명은 미숙아였으며 이중 3명은 재태연령 34주에서 36주 사이의 준만삭아였다. 10명의 환아들이 입원 $4{\pm}2$일 전부터 경미한 상기도 감염 증상(비 폐쇄, 기침, 콧물)을 보였고 입원 $0{\pm}1$일 후에는 모든 환아들이 뚜렷한 호흡기 이상 징후(빈호흡, 흉곽함몰, 청색증, 비익확장)를 보였다. 9명의 환아들이 무호흡을 보였고, 이 중에 5명은 무호흡 자체가 기계환기요법의 사유가 되었다. 무호흡이 의료진에 의해 확인된 시점부터 뚜렷한 호흡기 이상 징후 또는 청진 상 나음 또는 천명 소견이 나타난 시점까지 $1{\pm}2$일이 걸렸으며, 이 중에 3명은 무호흡이 의료진에 의해 확인되었을 때 뚜렷한 호흡기 이상 징후 또는 진찰소견을 보이지 않았고 1-3일이 지난 후에야 그러한 징후 또는 진찰소견을 보였다. 환아들의 기계환기요법 기간은 $3{\pm}2$일, 중환자실 체류기간은 $6{\pm}2$일이었고 입원 중 사망한 환아는 없었다. 결 론 : RSV는 미숙아로 태어난 경우가 아니라도 생후 2개월 미만의 신생아 및 조기 영아들에게 중증의 호흡기 감염을 초래할 수 있으며 뚜렷한 호흡기 이상 징후가 나타나기 전에 치명적인 무호흡이 발생할 수 있으므로 이 연령의 영아들이 RSV 유행기에 경미한 상기도 감염 증상을 보이거나 RSV 감염에 노출될 위험이 높은 상황에서는 매우 조심스러운 추적관찰이 필요할 것으로 생각된다.

Purpose : To identify clinical characteristics of severe respiratory syncytial virus (RSV) in neonatal period and early infancy and provide information in clinical practice. Methods : Twelve neonates and young infants (<6 months) who were infected by respiratory syncytial virus and required mechanical ventilation between March 2005 and July 2007 were enrolled. Diagnosis of RSV infection was made based on the positive results by rapid antigen immunoassay or polymerase chain reaction. Results : There were four premature infants, of whom three were near-term. Birth weight of subject patients was $2.8{\pm}0.6kg$, gestational age was $37{\pm}2weeks$ and the age at the time of admission was $35{\pm}15days$. Nine of them showed apnea and in five patients, apnea itself was an indication for mechanical ventilation. In seven of the apneic patients, apnea was the first manifestation of RSV infection. In three of these seven apneic patients, apnea preceded definite respiratory distress signs or typical stethoscopic findings by 1-3 days. Mean duration of mechanical ventilation was $3{\pm}2days$, and mean duration of stay in intensive care unit was $6{\pm}2days$. Conclusion : RSV is a major cause of severe respiratory tract infection in term or near-term infant younger than 2 months. For apnea could be the first manifestation of the RSV infection, high level of suspicion is required in practice of neonates or young infants who show any upper respiratory infection symptoms during RSV season.

후두개 낭종은 신생아 및 영아에서 협착음을 일으킬 수 있는 드문 질환이지만, 이 질환에 이환된 환아는 갑작스런 기도 협착을 일으켜 사망할 수도 있다. 따라서, 협착음을 보이는 환아에서 후두개 낭종을 감별해 냄으로써 갑작스런 기도 협착으로 인한 사망을 방지할 수 있다. 후두개 낭종은 협착음, 쉰목소리, 흉골 함몰, 무호흡, 수유시 청색증 및 성장 장애를 일으킬 수 있는 질환으로 이 질환이 의심되면 굴곡성 후두경으로 진단해야 한다. 이 질환의 치료는 $CO_2$ 레이저를 이용한 후두미세수술이며, 적절한 치료를 통해 재발을 방지할 수 있다. 저자들은 협착음, 쉰목소리, 흉골 함몰, 수유시 청색증 및 성장 장애를 주소로 전원된 1개월 남아에서 굴곡성 후두경을 통해 후두개 낭종을 진단하였고, $CO_2$ 레이저를 통한 후두미세수술 후 증상이 호전된 증례 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Vallecular cyst is an uncommon but potentially dangerous condition causing stridor and has been associated with sudden airway obstruction resulting in death due to its anatomical location in neonates. It may also present with various degrees of feeding problems resulting in failure to thrive. When a vallecular cyst is suspected clinically, endoscopic laryngoscopy is necessary to visualize it. Other conditions leading to neonatal stridor such as laryngomalacia and other laryngotracheal abnormalities should be ruled out. Marsupialization with a $CO_2$ laser is the most effective and safest treatment to prevent recurrence. We report a case of a 1-month-old male infant with a vallecular cyst synchronous with gastroesophageal reflux, and failure to thrive. He was referred to our hospital because of hoarseness, inspiratory stridor, feeding-cyanosis, chest retraction and failure to thrive. Diagnostic workup revealed a cyst at the tongue base, suggesting a vallecular cyst. The cyst was removed by laryngomicrosurgery with $CO_2$ laser. After the surgery, the symptoms improved and the body weight increased steadily. We report a successfully treated case of neonatal vallecular cyst with symptoms of upper respiratory obstruction, gastroesophageal reflux, and failure to thrive.

Purpose: Blow-out fracture is one of the most common fractures in facial trauma. It is diagnosed by Computed Tomography(CT) scan, which is considered as the most effective diagnostic tool. Since, the Picture Archiving Communication System(PACS) has been provided recently to many hospitals, doctors are more familiar with imaging software of PACS. Because this software has many useful measuring tools, doctors can measure orbital structure easily and make a plan for treatment with its data. Therefore, authors intended to analyze the data of orbital structure measured with PACS imaging software and evaluate its usefulness. Methods: The charts and CT images of 100 patients, which were 50 patients with medial wall fracture and 50 patients with floor fracture, were reviewed. Patients were selected by pre-determined criteria and their CT images were measured with image software of PACS. 'Extraocular muscle thickness', 'Defect ratio'(ratio of defect area to normal area) and 'Globe position index' were measured and analyzed statistically. Results: The thickness of inferior rectus muscle and medial rectus muscle was simultaneously increased in acute-stage of blow-out fracture. The medial rectus muscle was more thickened in medial wall fracture and inferior rectus was more thickened in floor fracture, respectively. In acute blow-out fracture, globe position is exophthalmic rather than enophthalmic. Especially in floor fracture, numerical value summed up thickness of all extraocular muscle is correlated to the defect ratio and globe position index. Conclusion: Clinicians can decide globe position or presume defect ratio in inferior wall fracture by measurement of CT image in acute blow-out fracture using PACS.

It was assumed that the effect of estrogen on wound healing would be variable according to patient's gender and age since estrogen is a sex steroid. This study was designed to determine the variability of the effect of estrogen on proliferation of human dermal fibroblasts and collagen synthesis which are most important in wound healing considering patient's gender and age. Fibroblasts were isolated from the dermis of female patients in premenstrual, menstrual, or postmenopausal age group and that of male patients. The isolated fibroblasts were cultivated in the presence of estrogen($1.0{\mu}g/ml$). The cells were seeded at $5.0{\times}10^3cell/well$ in Dulbecco's Modified Eagle's Medium/Ham's F-12 nutrient including 5% fetal bovine serum in 96-well plates. The cells were incubated for 3 days. For fibroblast proliferation MTT assay method was used. To measure the production of collagen, the collagen type I carboxy- terminal propeptide enzyme immunoassay was carried out. Estrogen stimulated the proliferation of fibroblasts in female patients, but not in male patients. The greatest cell proliferation and collagen synthesis was seen at women in menstrual and postmenopausal age. These results demonstrated that effects of estrogen on dermal fibroblast proliferation and collagen synthesis were variable with gender and age.