• Tuberculosis and Respiratory Diseases
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• 제45권4호
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• pp.896-901
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• 1998

Pulmonary arteriovenous malformation(PAVM) is an uncommon congenital anomaly. As pulmonary arteriovenous malformation is a direct communication between the branches of pulmonary artery and vein which originated from the malformation of capillary development, major disturbances in gas exchange can result. This malformation results in the several symptoms such as dyspnea, hemopyssis, cyanosis, and severe neurologic complaints. However, the most of patients are usually asymptomatic. Selective pulmonary angiography is well known the helpful diagnostic method. Recently, therapeutic embolization has been advocated as the treatment of choice for pulmonary arteriovenous malformations. We report a case of multiple pulmonary arteriovenous malformation, which was detected on the simple chest X-ray and successfully treated with coil embolization in a 19-year-old asymptomatic woman.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권2호
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• pp.198-203
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• 2008

저자들은 만성적인 철결핍성 빈혈을 보이는 10세 여아에서, 캡슐내시경을 비롯한 내시경적 검사로 위장관계의 다발성 정맥기형을 발견하여 blue rubber bleb nevus syndrome으로 진단하였고, 광범위한 외과적 절제술로 성공적으로 치료하였기에 이에 보고하는 바이다.

• Imaging Science in Dentistry
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• 제44권1호
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• pp.67-73
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• 2014

Intraosseous vascular lesions of the maxillofacial region are rare, and the differential diagnosis of intraosseous vascular malformations from other jaw lesions can be challenging. In the present case, magnetic resonance imaging and three-dimensional computed tomographic angiography (CTA) was used for diagnosis, and the lesion was treated wih surgical excision. Diverse characteristics such as the "honeycomb" and "sunburst" radiographic appearances and the absence of major peripheral feeder vessels in the CTA were noted. Intraosseous vascular malformations have a varied radiographic appearance, and the nomenclature of these lesions is equally diverse, with several overlapping terms. Pathologists do not generally differentiate among intraosseous vascular lesions on the basis of histopathology, although these lesions may present with contrasting immunohistochemical and clinical behaviors requiring varied treatment strategies. This case report highlights the need for multiple imaging modalities to differentiate among vascular lesions, as well as to better understand the behaviors of these unique lesions.

• 대한두개안면성형외과학회지
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• 제24권5호
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• pp.236-239
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• 2023

Porocarcinoma (PC) and basal cell carcinoma (BCC) are distinct skin cancers. Few studies have documented the occurrence of two concurrent types of skin cancers, and to the best of our knowledge, this represents the inaugural report of such a coexisting lesion arising from a capillary malformation. Herein, we report a case of concurrent PC and BCC presenting with capillary malformation. A 93-year-old woman visited our hospital with a protruding mass in her right nasal ala that appeared as a capillary malformation. A biopsy was performed on the skin lesion, and BCC was diagnosed. A wide excision was performed. Permanent biopsy revealed that the skin lesion was a PC with basal cells and squamous differentiation. The safety margin of the deep tissue margin was < 0.1 cm; however, considering the advanced age of the patient, further excision was deemed to not possess any benefits. This case illustrates the importance of recognizing the possibility of multiple skin cancers, even in patients with benign lesions such as capillary malformations. The rarity of this presentation highlights the importance of thorough investigation and histopathological examination of skin lesions in guiding appropriate surgical excision.

• Tuberculosis and Respiratory Diseases
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• 제66권4호
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• pp.314-318
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• 2009

저자들은 반복적인 코출혈이 있는 가족에서 폐동정맥기형이 있는 동생과 점막의 모세혈관확장증을 가진 형을 경험하고 희귀한 유전질환인 유전출혈모세혈관확장증의 가계도를 확인하였기에 문헌고찰과 함께 보고하는 바이다.

• BMB Reports
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• 제49권5호
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• pp.255-262
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• 2016

The disease known as cerebral cavernous malformations mostly occurs in the central nervous system, and their typical histological presentations are multiple lumen formation and vascular leakage at the brain capillary level, resulting in disruption of the blood-brain barrier. These abnormalities result in severe neurological symptoms such as seizures, focal neurological deficits and hemorrhagic strokes. CCM research has identified 'loss of function' mutations of three ccm genes responsible for the disease and also complex regulation of multiple signaling pathways including the WNT/β-catenin pathway, TGF-β and Notch signaling by the ccm genes. Although CCM research is a relatively new and small scientific field, as CCM research has the potential to regulate systemic blood vessel permeability and angiogenesis including that of the blood-brain barrier, this field is growing rapidly. In this review, I will provide a brief overview of CCM pathogenesis and function of ccm genes based on recent progress in CCM research.

• Communications for Statistical Applications and Methods
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• 제29권2호
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• pp.239-250
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• 2022

In many applications, we frequently encounter correlated multiple outcomes measured on the same subject. Joint modeling of such multiple outcomes can improve efficiency of inference compared to independent modeling. For instance, in developmental toxicity studies, fetal weight and number of malformed pups are measured on the pregnant dams exposed to different levels of a toxic substance, in which the association between such outcomes should be taken into account in the model. The number of malformations may possibly have many zeros, which should be analyzed via zero-inflated count models. Motivated by applications in developmental toxicity studies, we propose a Bayesian joint modeling framework for continuous and count outcomes with excess zeros. In our model, zero-inflated Poisson (ZIP) regression model would be used to describe count data, and a subject-specific random effects would account for the correlation across the two outcomes. We implement a Bayesian approach using MCMC procedure with data augmentation method and adaptive rejection sampling. We apply our proposed model to dose-response analysis in a developmental toxicity study to estimate the benchmark dose in a risk assessment.

• Radiation Oncology Journal
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• 제9권2호
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• pp.351-359
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• 1991

LINAC 뇌정위적 방사선 수술은 주로 multiple noncoplanar arc를 이용한 single isocenter를 이용하고 있다. 이러한 방법에 의하면 구형선량 분포를 얻게 되는데, 뇌동정맥 기형이나 뇌종양의 경우 구형이 아닌 임의의 형태를 가진 경우도 많다. 본 논문에서는 임의의 병소형태에 대하여 multiple isocenters를 이용하여 병소모양과 같은 형태의 선량분포를 얻는 방법에 대하여 논하고져 한다. 적당한 조사변수들을 처음에 잘 선정하는 것은, 빠른 시간내에 최적선량 계획을 수행하는데 중요하다. 긴 병소모양에 대하여 같은 형태의 선량분포를 얻기 위한 isocenter 간격 및 조사면에 대한 guideline이 만들어졌다. 특히, 3차원 선량분포를 이용한 multiple isocenters의 응용에 대하여 논하여진다.

• Korean Journal of Radiology
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• 제23권8호
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• pp.794-802
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• 2022

Objective: To evaluate the feasibility of single-shot whole thoracic time-resolved MR angiography (TR-MRA) to identify the feeding arteries of pulmonary arteriovenous malformations (PAVMs) and reperfusion of the lesion after embolization in patients with multiple PAVMs. Materials and Methods: Nine patients (8 females and 1 male; age range, 23-65 years) with a total of 62 PAVMs who underwent percutaneous embolization for multiple PAVMs and were subsequently followed up using TR-MRA and CT obtained within 6 months from each other were retrospectively reviewed. All imaging analyses were performed by two independent readers blinded to clinical information. The visibility of the feeding arteries on maximum intensity projection (MIP) reconstruction and multiplanar reconstruction (MPR) TR-MRA images was evaluated by comparing them to CT as a reference. The accuracy of TR-MRA for diagnosing reperfusion of the PAVM after embolization was assessed in a subgroup with angiographic confirmation. The reliability between the readers in interpreting the TR-MRA results was analyzed using kappa (κ) statistics. Results: Feeding arteries were visible on the original MIP images of TR-MRA in 82.3% (51/62) and 85.5% (53/62) of readers 1 and 2, respectively. Using the MPR, the rates increased to 93.5% (58/62) and 95.2% (59/62), respectively (κ = 0.760 and 0.792, respectively). Factors for invisibility were the course of feeding arteries in the anteroposterior plane, proximity to large enhancing vessels, adjacency to the chest wall, pulsation of the heart, and small feeding arteries. Thirty-seven PAVMs in five patients had angiographic confirmation of reperfusion status after embolization (32 occlusions and 5 reperfusions). TR-MRA showed 100% (5/5) sensitivity and 100% (32/32, including three cases in which the feeding arteries were not visible on TR-MRA) specificity for both readers. Conclusion: Single-shot whole thoracic TR-MRA with MPR showed good visibility of the feeding arteries of PAVMs and high accuracy in diagnosing reperfusion after embolization. Single-shot whole thoracic TR-MRA may be a feasible method for the follow-up of patients with multiple PAVMs.

• 대한영상의학회지
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• 제82권6호
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• pp.1628-1633
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• 2021

척수 경막외 동정맥루는 매우 드문 질환으로 진단과 치료가 어렵다. 무증상 환자도 있으나 동정맥단락으로 척수주위정맥으로 역류되고 늘어난 정맥이 인접한 신경근을 압박하면서 증상이 생길 수 있다. 본 증례는 31세 남자 환자로 2년 동안 지속되는 요통, 방사성 허벅지통증, 하지 감각변화로 내원하였다. 컴퓨터단층촬영, 자가공명장치 및 척수혈관조영술에서 요추의 경막외 공간에서 동정맥단락에 의해 늘어난 정맥이 신경근을 압박하고 있는 척수 경막외 동정맥루로 진단하였다. 코일색전술을 시행하여 동정맥단락을 차단하였고 수일 내에 신경근 압박증상이 호전되었다. 이번 증례에서는 척수 경막외 동정맥루에서 외과적 수술의 대안으로 코일 색전술이 효과적인 치료가 될 수 있다는 것을 보여주었다.