• Journal of Korean Neurosurgical Society
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• v.50 no.6
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• pp.535-537
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• 2011

Recently, combination of surgery and radiation therapy (RT) has been recommended in the treatment of craniopharyngioma. RT could be associated with late complications, including vasculopathy. We report two cases of the moyamoya syndrome seen in children with craniopharyngioma who received RT after surgical resection. Thirty-five patients in pediatric age with craniopharyngioma were surgically treated. Fifteen out of 35 patients underwent surgical resection followed by RT or gamma knife surgery. Two of the 15 were found to have symptoms of transient ischemic attack and were diagnosed as moyamoya syndrome through the cerebral angiography. Age at RT was 4 and 13 years, respectively. The latent period for development of the moyamoya syndrome was 27 months and 3 years, respectively, after RT. The RT dose of both patients was 54 Gy. These two patients received bilateral encephaloduroarteriosynangiosis procedures. We report here these two cases of radiation-induced moyamoya syndrome in pediatric craniopharyngioma. Pediatric patients with craniopharyngioma who received RT should be reminded, during follow-up, about the risk of development of the moyamoya syndrome.

• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.408-414
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• 2015

Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.

• Korean Circulation Journal
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• v.52 no.5
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• pp.398-400
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• 2022

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.86-90
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• 2003

Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse vascular abnormalities, which appear to be a feature of Alagille syndrome, suggest Notch signaling pathway defects affect angiogenesis. The associations of Alagille syndrome with moyamoya disease, the chronic cerebrovascular occlusive disease, were reported and suggested as additional evidence of vasculopathy of Alagille syndrome. We report another 25 month-old Alagille syndrome girl who presented with acute left hemiparesis and was diagnosed with moyamoya disease through the cerebral angiographic study.

• Journal of the Korean neurological association
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• v.36 no.4
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• pp.337-340
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• 2018

Human immunodeficiency virus (HIV) infection can result in ischemic stroke via several mechanisms, including opportunistic infection, vasculopathy, cardioembolism, and coagulopathy. HIV-vasculopathy is related to endothelial dysfunction, stenosis and aneurysm formation, infectious vasculitis, dissection and accelerated atherosclerosis during highly active antiretroviral therapy (HAART). We represent a case of HIV infection manifested as an acute ischemic stroke attack. After 4 months during HAART, our patient experienced a recurrent ischemic stroke with progression of middle cerebral artery stenosis.

• Journal of Chest Surgery
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• v.42 no.2
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• pp.135-140
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• 2009

Background: Chronic rejection after a cardiac allograft usually occurs about six months after the operation. Vasculopathy due to chronic rejection causes atherosclerosis in the coronary artery of the transplanted heart and then this causes myocardial injury. We intended to discover and document those findings that occur in a transplanted ascending aorta. Material and Method: In rats weighting $200{\sim}300gm$ (Spraque-Dawley rat), we carried out heterotopic heart allo-transplantation with the modified Ono-Lindsey method and then the rats were administrated cyclosporine (10mg/kg/day). After three months survival, we acquired biopsy materials from the native ascending aorta and the allo-transplanted ascending aorta and we compared them. We classified each severity of 1) intimal thickening, 2) medial hyperplasia, 3) medial calcification, 4) medial inflammation and 5) chondroid metaplasia, which are specific biopsy findings for chronic rejection after a cardiac allograft. Each severity was classified, according to the opinion of one pathologist, in the native ascending aorta biopsies (n=9) and the allo-transplanted ascending aorta biopsies (n=13). The data of the control group and the study group were statistically analyzed with using the Mann-Whitney test (SPSS version 12.0 window). Result: The important changes of the allo-transplanted aorta were intimal thickening (p<0.0001), medial calcification (p=0.045), medial inflammation (p<0.0001) and chondroid metaplasia (p=0.045), but not medial hyperplasia (p=0.36). Conclusion: Cardiac allograft vasculopathy was seen in the transplanted ascending aorta, the same as was seen in the coronary artery, after allograft cardiac transplantation. We have reached the conclusion that chronic rejection also progresses in the aorta.

• Journal of The Korean Society of Clinical Toxicology
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• v.18 no.1
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• pp.47-50
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• 2020

A 21-year-old female patient complaining of hemiparesis was diagnosed with right middle cerebral artery infarction. No risk factor was found, despite an extensive young-age stroke work-up, except her history of marijuana use. The patient had smoked marijuana for treating depression for more than five years. Magnetic resonance angiography showed multifocal intra- and extracranial stenoses, suggesting cannabinoid-induced vasculopathy. Since the use of illicit drugs has increased nationwide, physicians should consider it as a possible cause of a stroke due to an unknown etiology.

• Archives of Plastic Surgery
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• v.37 no.2
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• pp.148-152
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• 2010

Purpose: In treating diabetic foot ulcers, satisfactory vascularity is an essential prerequisite. To improve vascularity, a bypass graft has long been carried out. Recently, however, percutaneous transluminal angioplasty (PTA) has also been tried since the PTA is less invasive than the bypass graft. However, publication demonstrating the improvement of vascularity after the PTA are lacking. Therefore, this study was designed to show usefulness of the PTA in treating vasculopathy of diabetic foot. Materials: and Methods This study included 30 feet of 24 ischemic diabetic foot patients. Inclusion criteria were diabetes (duration > 5 years) and a significant lower extremity ischemia, as determined by a transcutaneous oxygen pressure ($TcpO_2$) < 30 mmHg. The PTA was carried out in 61 arteries. PTA procedure was considered successful, when residual stenosis was less than 30%. The procedure was considered failed when residual stenosis was more than 50%. Residual stenosis between 30% and 50% was considered acceptable. For evaluation of PTA effect, foot $TcpO_2$ and infrared thermography were measured before and 7th day after PTA. Results: Immediately after PTA performed in 61 arteries, 58 and 3 arteries were evaluated as being successful and acceptable, respectively. Before PTA, average foot $TcpO_2$ was $12.6{\pm}8.8$ mmHg and its value was increased to $44.2{\pm}23.9$ on 7th day after PTA (p<0.01). Average skin temperature was $31.8{\pm}1.2^{\circ}C$ before PTA and it was increased to $33.5{\pm}1.1^{\circ}C$ on 7th day after PTA (p<0.01). Conclusion: PTA procedure increases tissue oxygenation of ischemic diabetic feet which do not have wound healing potential due to low tissue oxygenation, to the level of possible wound healing. In addition, PTA increases skin temperature of ischemic diabetic feet which can imply an improvement of peripheral circulation.

• Childhood Kidney Diseases
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• v.15 no.1
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• pp.38-48
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• 2011

Purpose: Focal segmental glomerulosclerosis (FSGS) is the most common glomerulopathy causing pediatric renal failure. Since specific treatment targeting the etiology and pathophysiology of primary FSGS is yet elusive, the authors explored the pathophysiology of FSGS by transcriptome analysis of the disease using an animal model. Methods: FGS/kist strain, a mouse model of primary FSGS, and RFM/kist strain, as control and the parent strain of FGS/kist, were used. Kidney tissues were harvested and isolated renal cortex was used to extract mRNA, which was run on AB 1700 mouse microarray chip after reverse transcription to get the transcriptome profile. Results: Sixty two genes were differentially expressed in FGS/kist kidney tissue compared to the control. Those genes were related to cell cycle/cell death, immune reaction, and lipid metabolism/vasculopathy, and the key molecules of their networks were TNF, IL-6/4, IFN${\gamma}$, TP53, and PPAR${\gamma}$. Conclusion: This study confirmed that renal cell death, immune system activation with subsequent fibrosis, and lipid metabolism-related early vasculopathy were involved in the pathophysiology of FSGS. In addition, the relevance of methodology used in this study, namely transcriptome profiling, and Korean animal model of FGS/kist was validated. Further study would reveal novel pathophysiology of FSGS for new therapeutic targets.

• Tuberculosis and Respiratory Diseases
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• v.50 no.1
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• pp.122-126
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• 2001

A non-traumatic, spontaneous hemothorax is rare. The most common causes are coagulopathy, due to anticoagulation treatment, and cancers with a metastasis to the pleural surface. Other unusual causes include thoracic endometriosis, ruptured aortic aneurysm, pulmonary arterio-venous malformation, coagulopathy, Osler-Rendeu-Weber syndrome, Ehlers-Danlos syndrome et cetera. A type 1 neurofibromatosis(Von Recklinghausen's disease) is an autosomal dominant disease that is characterized by multiple skin tumors(neurofibroma) and abnormal skin pigmentation(caf$\acute{e}$-au-lait spots). Some are accompanied by vasculopathy, and are present with a spontaneous hemothorax. Such cases are unusual but fatal. We have recently experienced a case where a young male patient with neurofibromatosis initially presented with hypovolemic shock due to a spontaneous hemothorax. Later, aortography revealed that the cause of the hemothorax was a rupture of a pseudoaneurysm of the right internal mammary artery and as a result, an embolization was performed. Here we report this case with a review of the appropriate literature.