• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.1018-1023
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• 2007

Hereditary hemorrhagic telagiectasia (HHT), which is characterized by the classic triad of mucocutaneous telangiectases, arteriovenous malformations (AVMs) and inheritance, is an autosomal dominant disorder. The characteristic manifestations of HHT are all due to abnormalities of the vascular structure. This report deals with the case of a 14-year-old girl with typical features of HHT that include recurrent epistaxis, mucocutanous telangiectases, pulmonary and cerebral AVMs and a familial occurrence.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1996.04a
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• pp.81-81
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• 1996

선천성 후두열은 매우 드문 선천성 기형으로 신생아에게 심각한 호흡곤란 및 수유장애를 초래할 수 있다. 해부학적 결손정도와 관련된 기형에 따라 다양한 증상이 나타난다. 가장 흔한 특징적 증상으로는 흡기시 천명, 간헐적으로 나타나는 수유시 호홉곤란을 들 수 있다. 이 증상들은 후두연화증, 성대마비, 후비공폐색 둥과 같은 다른 선천성 기형에서도 나타나기 때문에 미리 의심하지 못하면 진단이 어렵다. 따라서 진단을 위해서는 세심한 주의와 정확한 내시경 검사가 필요하다. 저자들은 선천성 후두열 2례를 경험하였다. 첫 증례는 제 II형이었고 두번째는 III형이었으며, 두 증례 모두 전방 후두열 접근법으로 치료하였다. 제 III형 환아는 술전 흡기시 천명을 동반한 호흡곤란과 여러번의 흡인성 폐렴을 겪었으며 술후 캐놀라발거는 가능하였으나 경구를 통한 수유시 흡인을 동반하여 현재까지 위루술을 통해 음식을 섭취하고 있는 상태이다. 저자들은 최근 경험한 선천성 후두열 2례를 video presentation과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• v.35 no.11
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• pp.817-821
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• 2002

There are various surgical techniques in repairing Ebstein anomaly, but residual tricuspid regurgitation and compromized right heart function may ensue in some cases. We report our clinical experience of Ebstein anomaly and atrial flutter in a 19-year-old male patient who underwent simple modified tricuspid annuloplasty, hi-directional cavopulmonary shunt and cryoablation of cavotricuspid isthmus.

• Journal of the Korean Society of Radiology
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• v.85 no.1
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• pp.204-209
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• 2024

Mediastinal vascular malformations are rare and their diagnosis can be challenging. Imaging is vital for diagnosing mediastinal vascular malformations and can help avoid unnecessary invasive procedures. Herein, we report the detailed CT and MRI findings of a rare low-flow mediastinal vascular malformation in an asymptomatic 63-year-old male.

• Korean Journal of Environmental Biology
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• v.25 no.1
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• pp.66-71
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• 2007

The fourth instar larvae of C. riparius were treated with potential endocrine disruption chemicals (EDCs) such as bisphenol A (BPA) and 4-nonylphenol and the effects of morphological abnormalities were observed. The deformities of the mentum following exposure to EDCs showed the smooth tooth, the loss of tooth and deformed tooth. The incidence rates of the mentum deformity were associated with chemicals: BPA $31{\sim}90%$, and 4-nonlyphenol was $40{\sim}80%$. As the concentration of BPA increased, the incidence of deformed mentum was dose dependent. While the incidences of deformed mentum following exposure to 4-nonlyphenol was not associated with their concentrations. The deformed MLT observed smooth or round tooth and the deformity of LT showed loss of one or more than tooth. Also, the MIX type was usually smooth or loss tooth. The abundance of deformity type for the mentum showed MIX (MLT+LT) > LT (lateral teeth) > MLT (median lateral teeth).

• Proceedings of the KOR-BRONCHOESO Conference
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• 1978.06a
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• pp.7.2-7
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• 1978

There are over 60 types of hereditary hearing loss in man and most of these are distinguished from one another by outstanding associated defects caused the same gene producing hearing loss. However, there are at least 12 types of hereditary hearing loss with no known associated defects. The authors experienced two similar families with hereditary hearing loss. The affected offsprings had inevitably an affected parent in type of transmissin (autosomal dominant) and the hearing loss developed in childhood and progressed. Audiologic findings showed bilateral symmetrical moderate to severe sensori-neural hearing loss with flattened configuration on the audiogram. Vestibular function tests showed no abnormal findings and the associated anomalies could'nt be observed in history or clinical examinations.

• Proceedings of the Korean Society of Environment and Ecology Conference
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• 2005.10a
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• pp.150-153
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• 2005

• Journal of Chest Surgery
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• v.34 no.11
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• pp.858-860
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• 2001

VATER is a complex anomaly of multiple organs and often combined with cardiac anomalies. However, it can be cured with active surgical intervention. We achieved successful result with aggressive surgery in the patient with VATER and report it with references.

• Korean Journal of Environment and Ecology
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• v.18 no.3
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• pp.340-345
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• 2004

The embryotoxic effects of PCB (Aroclor 1248) on a Korean frog, Rana dybowskii was deter-mined by using the FETAX (Frog Embryo Teratogenesis Assay-Xenopus) protocol. The rates of mortality and malformed larvae were investigated by probit analysis. The results showed that PCB is highly embryolethal. From LC$_{50}$ of 1.48ppb and from EC$_{50}$ of 0.25ppb and TI of 5.7 were derived, which indicates PCB is to be considered a teratogenic compound. Specific malformations occurred in 62.0% as edema at the 0.1ppb, in 32.0% as tail deformations at the 1ppb, and in 68.0% as profound deformations at the 5ppb of PCB concentration which living embryos were exposed to. PCB suppressed the growth of head-tail length at a relatively low concentration (1.0ppb), and therefore growth inhibition as assessed by embryo length can be used as a sensitive indicator to evaluate the toxicity of pollutants in the environment. In conclusion, PCB must be considered highly embryotoxic to Rana dybowskii.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.21 no.4
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• pp.407-413
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• 1999

Maxillary duplication is a kind of proliferative neurocristopathy and considered to arise from bifurcation of neural crest elements soon after migration into mandibular arch. Sometimes this malformation is accompanied with oblique facial cleft. Usually this type of maxillofacial malformation requires multiple surgical intervention and the results are far from ideal. It became more troublesome if it had not been properly corrected on time, because secondary deformities could be developed from growth and development of abnormal tissues. This is a case of a 25-year-old-female patient who showed severe facial asymmetry thought to secondary deformity of maxillary duplication and masticatory disturbance due to multiple supernumerary teeth on posterior part right maxilla. We successully treated these deformities through four times of surgery of bone resection, orthodontic treatment, zygomatic and orbital reconstruction, orthognathic surgery and scar revision... ect. So we reported this rare case with review of literatures.