• Childhood Kidney Diseases
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• 제26권2호
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• pp.86-90
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• 2022

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis requires prompt diagnosis and treatment, since renal function at the time of diagnosis is significantly associated with renal outcomes. Here, we report two pediatric patients with ANCA-positive glomerulonephritis initially presenting with hematuria, mild proteinuria, and normal renal function. The first patient with a high myeloperoxidase-ANCA titer (>134 IU/mL) was diagnosed with rapidly progressive glomerulonephritis based on renal biopsy and treated with immunosuppressive therapy after 10 months of follow-up. The second patient with a low myeloperoxidase-ANCA titer (11 IU/mL) maintained normal kidney function without medication. Two cases showed different clinical course according to ANCA titer.

• 대한청각학회지
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• 제23권1호
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• pp.59-62
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• 2019

Primary tumors arising from the external auditory canal (EAC) are rare. We describe two cases of mass lesions within the EAC causing slowly progressive hearing loss without otorrhea or otalgia. Otoendoscopic examination demonstrated total obstruction of the EAC, and pure tone audiometry revealed conductive hearing loss. Based on the findings of the histopathologic examination, one patient was diagnosed with venous hemangioma that was treated using surgical resection, and the other patient was diagnosed with diffuse large B-cell lymphoma (DLBCL) that was treated using external-beam radiation therapy. Although primary tumors in the EAC are rare, both benign tumors such as venous hemangiomas and malignant lesions such as DLBCL should be considered as possible differential diagnoses of mass lesions in the EAC.

• Journal of Audiology & Otology
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• 제23권1호
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• pp.59-62
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• 2019

Primary tumors arising from the external auditory canal (EAC) are rare. We describe two cases of mass lesions within the EAC causing slowly progressive hearing loss without otorrhea or otalgia. Otoendoscopic examination demonstrated total obstruction of the EAC, and pure tone audiometry revealed conductive hearing loss. Based on the findings of the histopathologic examination, one patient was diagnosed with venous hemangioma that was treated using surgical resection, and the other patient was diagnosed with diffuse large B-cell lymphoma (DLBCL) that was treated using external-beam radiation therapy. Although primary tumors in the EAC are rare, both benign tumors such as venous hemangiomas and malignant lesions such as DLBCL should be considered as possible differential diagnoses of mass lesions in the EAC.

• Journal of Chest Surgery
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• 제21권6호
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• pp.1117-1123
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• 1988

Prolapse of the aortic valve is the main cause of insufficiency of the aortic valve as a complication of ventricular septal defect. Aortic insufficiency gets worse by the progress of prolapse of aortic valve due to lack of support of the valve and the hemodynamic effect of blood flow through the ventricular septal defect. This produces typical clinical picture, that may be serious and threatening when it is untreated. Type and timing for the surgical treatment of the ventricular septal defect with aortic insufficiency is considered. Among 113 ventricular septal defect, 9 patients of ventricular septal defect with associated aortic insufficiency were experienced from June. 1983 to June 1988 at the Department of Thoracic and Cardiovascular Surgery, Chon-Buk University Hospital. Male was 6 patients and female was 3 patients. Ages were from 7 years to 24years. 5 patients were from 10 to 19 years age. 3 patients were below 10 years age. The ratio of pulmonary blood flow to systemic f low [Qp/Qs] was 1.53 and in pulmonary vascular resistance, normal or slight increase was 7 patients, moderate 1 patient, and severe 1 patient. Ventricular septal defect was subpulmonic in 5 patients and infracristal in 4 patients. Prolapse of right coronary cusp was 7 patients, right and non coronary cusp 1 patient and non coronary cusp 1 patient. Teflon patch closure of ventricular septal defect was undertaken in 3 patients and primary closure in 1 patient. Among the 4 patients of defect closure alone, one patient performed valve replacement 7 months later due to progressive regurgitation and cardiac failure and the result was good. The other 3 patients were good result. Closure of ventricular septal defect and aortic valvuloplasty performed in 4 patients. 2 patients of these required valve replacement for the sudden intractable cardiac failure and died due to low cardiac output. The cause of intractable cardiac failure was tearing of repaired valve at the fixed site. The other 2 patients were good result. Closure of ventricular septal defect and valve replacement performed in 1 patient with good result.

• Archives of Plastic Surgery
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• 제35권5호
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• pp.615-618
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• 2008

Purpose: Pyoderma gangrenosum is a rare cutaneous ulcerative disease. First described in 1930, the condition is characterized by progressive ulceration with deeply undermined purple-red edge. The lower extremities are most commonly affected but other parts of the skin and mucous membranes may also be involved. Although medical treatments with topical wound therapy are commonly used, surgical intervention is still controversial. In this paper, we report an atypical case of pyoderma gangrenosum which was characterized by extensive soft tissue breakdown. Methods: A 27-year-old male patient was referred to our institution with a $7{\times}8cm$ sized deeply undermined ulceration with pus-like discharge and fever. Incision and drainage was performed at another clinic 3 days prior to admission to our institution. After a thorough physical examination and the MRI review, a diagnosis of necrotizing faciitis was made. Accordingly, fasciotomy and debridement was performed. However, the wound enlarged progressively and the patient remained highly febrile for 9 days after the treatment. Septic screening did not reveal any occult infection. After a secondary review of the case, the initial diagnosis of necrotizing fasciitis was rejected and changed to pyoderma gangrenosum. With the use of dexamethasone intravenously, the wound improved dramatically and the fever was eliminated. Steroid mediation was tapered with duration of 1 month. The wound was stabilized and subsequently covered with split-thickness skin graft. Results: Split-thickness skin grafting with 1 : 1.5 mesh was successfully taken. Conclusion: Initial clinical features of pyoderma gangrenosum are very similar to that of necrotizing fasciitis. High fever and progressive ulceration with severe pain could invite earlier surgical approach. The advancing wound margins (the well defined violaceous, undermined border and necrotic ulcer base) and lack of isolation of pathogenic organism was used to make the correct diagnosis of pyoderma gangrenosum. We achieved a good result with proper medication and split-thickness skin graft.

• 한국의학물리학회지:의학물리
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• 제26권4호
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• pp.229-233
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• 2015

정위 방사선치료를 받은 폐암환자에서 결과를 후향적으로 분석하고자 하였다. 연속된 흉부 컴퓨터단층촬영(CT)의 종양의 크기변화 분석을 통해 치료 반응 평가를 조사하였다. 11명의 초기 비소세포폐암환자를 대상으로 정위 방사선치료 선량의 중앙값은 6,000 cGy이고 분할 조사 회수의 중앙값은 5회였다. 경과 관찰기간의 중앙값은 28개월로 치료후 종양의 크기 변화는 총 51회의 CT를 통해 분석하였고, 각 환자당 중앙값 3회의 CT 촬영이 시행되었다. 본 연구에서 치료의 총 반응률은 90.9%로 5명의 환자에서 완전관해와 5명에서 부분관해가 관찰되었다. 부분관해와 완전관해까지의 기간의 중앙 값은 각각 3개월과 5개월이었다. 경과관찰에서 3명의 환자가 병의 진행양상을 나타내었다. 본 연구에서 초기 폐암의 정위방사선치료후 CT 분석을 통해 반응 평가와 함께 치료 반응 시점을 확인할 수 있었다.

• Clinical and Experimental Pediatrics
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• 제57권9호
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• pp.416-419
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• 2014

X-linked adrenoleukodystrophy (X-ALD) is a rare peroxisomal disorder, that is rapidly progressive, neurodegenerative, and recessive, and characteristically primary affects the central nervous system white matter and the adrenal cortex. X-ALD is diagnosed basaed on clinical, radiological, and serological parameters, including elevated plasma levels of very long chain fatty acids (VLCFA), such as C24:0 and C26:0, and high C24:0/C22:0 and C26:0/C22:0 ratios. These tests are complemented with genetic analyses. A 7.5-year-old boy was admitted to Department of Pediatrics, Chungnam National University Hospital with progressive weakness of the bilateral lower extremities. Brain magnetic resonance imaging confirmed clinically suspected ALD. A low dose adrenocorticotropic hormone stimulation test revealed parital adrenal insufficiency. His fasting plasma levels of VLCFA showed that his C24:0/C22:0 and C26:0/C22:0 ratios were significantly elevated to 1.609 (normal, 0-1.390) and 0.075 (normal, 0-0.023), respectively. Genomic DNA was extracted from peripheral whole blood samples collected from the patient and his family. All exons of ABCD1 gene were amplified by polymerase chain reaction (PCR) using specific primers. Amplified PCR products were sequenced using the same primer pairs according to the manufacturer's instructions. We identified a missense mutation (p.Arg163Leu) in the ABCD1 gene of the proband caused by the nucleotide change 488G>T in exon 1. His asymptomatic mother carried the same mutation. We have reported an unpublished mutation in the ABCD1 gene in a patient with X-ALD, who showed increased ratio of C24:0/C22:0 and C26:0/C22:0, despite a normal VLCFA concentrations.

• Journal of Korean Neurosurgical Society
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• 제56권5호
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• pp.419-422
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• 2014

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset headache with focal neurologic deficit and prolonged but reversible multifocal narrowing of the distal cerebral arteries. Stroke, either hemorrhagic or ischemic, is a relatively frequent presentation in RCVS, but progressive manifestations of subarachnoid hemorrhage, intracerebral hemorrhage, cerebral infarction in a patient is seldom described. We report a rare case of a 56-year-old woman with reversible cerebral vasoconstriction syndrome consecutively presenting as cortical subarachnoid hemorrhage, intracerebral hemorrhage, and cerebral infarction. When she complained of severe headache with subtle cortical subarachnoid hemorrhage, her angiography was non-specific. But, computed tomographic angiography showed typical angiographic features of this syndrome after four days. Day 12, she suffered mental deterioration and hemiplegia due to contralateral intracerebral hematoma, and she was surgically treated. For recurrent attacks of headache, medical management with calcium channel blockers has been instituted. Normalized angiographic features were documented after 8 weeks. Reversible cerebral vasoconstriction syndrome should be considered as differential diagnosis of non-aneurysmal subarachnoid hemorrhage, and repeated angiography is recommended for the diagnosis of this under-recognized syndrome.

• Journal of Korean Neurosurgical Society
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• 제56권6호
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• pp.517-520
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• 2014

Cauda equina syndrome (CES) associated with dural ectasia is a rare neurologic complication in patients with longstanding ankylosing spondylitis (AS). We report a 68-year-old male with a 30-year history of AS who presented a typical symptom and signs of progressive CES, urinary incontinence and neuropathic pain of the lumbosacral radiculopathy. Computed tomography (CT) and magnetic resonance imaging (MRI) findings showed the unique appearances of dural ectasia, multiple dural diverticula, erosion of posterior element of the lumbar spine, tethering of the conus medullaris and adhesion of the lumbosacral nerve roots to the posterior aspect of the dural ectasia. Considering the progressive worsening of the clinical signs, detethering of the conus medullaris through resection of the filum terminale was performed through a limited laminectomy. However, the urinary incontinence did not improve and there was a partial relief of the neuropathic leg pain only. The possible pathogenetic mechanism of CES-AS and the dural ectasia in this patient with longstanding AS are discussed with a literature review.

• Journal of Korean Neurosurgical Society
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• 제38권4호
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• pp.316-319
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• 2005

A 42-year-old female was admitted with an 11-month history of progressive spastic paraparesis and ataxic gait. Magnetic resonance imaging showed intraspinal space occupying lesion compressing the spinal cord posteriorly, located from C5 to T2 with iso to high signal intensity at T2-weighted images and high signal intensity at T1-weighted images. The patient underwent surgery for decompression of the affected spinal cord because of the progressive neurological deficit. At surgery, the lesion was intradural extramedullary lipoma composed with mature adipose tissue. Partial tumor removal to decompress the neural structures and laminoplasty to avoid postoperative instability and deformity were performed. Postoperatively, she demonstrated improvement in paraparesis and was able to walk without assistance. Though attempts to decrease the size of or even to totally remove a lipoma are not required to achieve satisfactory results and carry considerable risks of surgical morbidity, a careful and limited decompression of the affected spinal cord through a partial removal of the tumor and laminoplasty could result in a significant neurological improvement.