• Clinical and Experimental Pediatrics
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• 제59권10호
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• pp.421-424
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• 2016

Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3. We were unable to identify the cause for the HLH. He remained symptom-free until the development of back pain and right ankle joint pain with swelling at 15 years of age. He was diagnosed with HLA B27-positive AS with bilateral active sacroiliitis. He showed symptom aggravation despite taking naproxen and methotrexate, and the symptoms improved with etanercept. On admission, his laboratory data showed leukopenia with high ferritin and triglyceride levels. Bone marrow biopsy examination showed histiocytic hyperplasia with hemophagocytosis. There was no evidence of infection. He received naproxen alone, and his symptoms and laboratory data improved without any other immunomodulatory medications. Genetic study revealed no primary HLH or inflammasome abnormalities. In this case, underlying autoimmune disease should have been considered as the cause of recurrent MAS in the young patient once primary HLH was excluded.

• 대한한방내과학회지
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• 제26권4호
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• pp.912-917
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• 2005

Caroli's disease is a non-obstructive dilatation of the intrahepatic bile ducts. It is a rare congenital disorder that classically causes saccular ductal dilatation, which usually it segmental dilatation. Caroli's disease is associated with recurrent bacterial cholangitis and stone formation. A 57 years-old female inpatient suffering from cholangitis due to Caroli's disease was admitted at Wonkwang University Jeonju Oriental Medicine Hospital. The patient was treated with herbal medicine such as Yongdamsagan-tang and acupuncture. As result, there was improvement of symptoms. The process and content of treatment and the patient's recovery over general symptoms is described.

• Journal of Korean Neurosurgical Society
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• 제50권4호
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• pp.381-384
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• 2011

The authors report a case of atypical extraventricular neurocytoma (EVN) transformed from EVN which had been initially diagnosed as an oligodendroglioma 15 years ago. An 8-year-old boy underwent a surgical resection for a right frontal mass which was initially diagnosed as oligodendroglioma. When the tumor recurred 15 years later, a secondary operation was performed, followed by salvage gamma knife treatment. The recurrent tumor was diagnosed as an atypical EVN. The initial specimen was reviewed and immunohistochemistry revealed a strong positivity for synaptophysin. The diagnosis of the initial tumor was revised as an EVN. The patient maintained a stable disease state for 15 years after the first operation, and was followed up for one year without any complications or disease progression after the second operation. We diagnosed an atypical extraventricular neurocytoma transformed from EVN which had been initially diagnosed as an oligodendroglioma 15 years earlier. We emphasize that EVN should be included in the differential diagnosis of oligodendroglioma.

• Clinics in Orthopedic Surgery
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• 제10권4호
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• pp.420-426
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• 2018

Background: The tibial tuberosity-trochlear groove (TT-TG) distance is used to determine the necessity of tibial tubercle osteotomy. We conducted this study to determine the extent to which each of the tibial tuberosity lateralization, trochlear groove medialization, and knee rotation angle affects the TT-TG distance in both normal and patella dislocated patients and thereby scrutinize the rationale for tuberosity transfer based on the TT-TG distance. Methods: Retrospective analysis of rotational profile computed tomography was done for patella dislocated and control group patients. Femoral anteversion, tibial torsion, knee rotation angle, tuberosity lateralization, and trochlear groove medialization were assessed in all patients. Relationship of these parameters with the TT-TG distance was investigated to evaluate their effects on the TT-TG distance. Results: We observed that the patellar dislocation group, compared to the control group, had increased TT-TG distance (mean, 19.05 mm vs. 9.02 mm) and greater tuberosity lateralization (mean, 64.1% vs. 60.7%) and tibial external rotation in relation to the femur (mean, $7.9^{\circ}$ vs. $-0.81^{\circ}$). Conclusions: Tuberosity lateralization and knee rotation were factors affecting patellar dislocation. These factors should be considered in addition to the TT-TG distance to determine the need for tibial tubercle osteotomy in patients with patellar dislocation.

• Journal of Korean Neurosurgical Society
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• 제52권5호
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• pp.488-490
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• 2012

Behçet's disease (BD) is an inflammatory systemic disorder with oral and genital ulcers, as well as ophthalmologic and cutaneous symptoms. Neurological manifestations in BD represent between 2.2% to 50% of the cases. The 25-year-old male patient, diagnosed with BD three years earlier, was admitted to our clinic with complaints of recurrent headaches. Tumor-like-parenchimal involvement was detected on a cranial magnetic resonance imaging. The lesion was removed surgically and then he suffered from right hemiparesis and epilepsy. Pathological examination of the lesion noted a demyelinating non-tumoural etiology. A neuro-Beh$\check{g}$et's case with parenchymal involvement has been examined in light of the literature, in terms of a tumor and a demyelinating disease differential diagnosis.

• Investigative Magnetic Resonance Imaging
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• 제22권3호
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• pp.187-193
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• 2018

Sweet's syndrome also known as acute neutrophilic dermatosis is a multisystem inflammatory disorder characterized by fever, malaise, leukocytosis, and skin lesions. Sweet's syndrome affects multiple organs though only rarely does it affect the central nervous system (CNS) when it does it is called Neuro-Sweet disease (NSD). We report on a case study of a biopsy-proven NSD in a 50 year old man. Serial magnetic resonance imaging (MRI) showed repeated CNS involvement of Sweet's syndrome after a respiratory tract infection preceded it. On the MRI, T2 hyperintense lesions occurred at multiple sites and disappeared after steroid therapy.

• 대한기관식도과학회지
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• 제19권1호
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• pp.11-14
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• 2013

Gastro-esophageal reflux disease (GERD) is a recurrent, long-term gastrointestinal condition characterised by the abnormal reflux of stomach contents into the esophagus. Heartburn is the most dominant symptom, which can be life long and can considerably reduce quality of life. The main goals of treatment are to alleviate symptoms and prevent relapses, esophageal stricture and adenocarcinoma. This paper presents a review comparing the effectiveness of omeprazole with Nissen fundoplication surgery for the treatment of GERD. Nissen fundoplication is more effective in controlling heartburn symptoms, healing esophagitis and preventing relapses than omeprazole. Quality of life, in terms of productivity and ability to work, is difficult to assess in the omeprazole group and, as a result, a comparison of this aspect is limited. Although fundoplication is expensive in the short term, it is more cost-effective than omeprazole. Nurses need to be aware of the effectiveness of omeprazole and fundoplication to provide patients with accurate information, which assists patients in decision making regarding treatment options.

• Clinical and Experimental Pediatrics
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• 제55권8호
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• pp.297-300
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• 2012

Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

• Radiation Oncology Journal
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• 제12권3호
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• pp.361-368
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• 1994

연구목적 :고환의 정상피종은 방사선치료에 매우 민감한 종양으로서 초기 정상피종에 있어서 근치적 고환절제술 후 보조적 방사선치료로 좋은 결과를 얻을 수 있는 것으로 알려져 있다. 10년간 서울대학교병원 치료방사선과에서 치료를 받은 28명의 환자를 대상으로 치료성적의 분석을 실시하였다. 대상 및 방법 : 1980년 8월에서 1990년 2월까지 서울대학교병원에서 치료를 받은 고환 정상피종 환자 32명을 대상으로 후향적 분석을 하였다. 27명의 환자는 근치적 고환절제술 후에 그리고 5명은 재발 후에 방사선치료를 받았는데 각군에서 2명씩은 불완전한 방사선치료로 연구대상에서 제외되었다. 고환절제술후 치료를 받은 환자들의 병기별 분포는 1기 18명, IIA기 5명, IIB기 1명 IIC기 1명이었다. 재발 후 방사선치료를 받은 3명은 첫 진단시 병기를 알 수 없었다. 전체 환자 중 6명 환자에서 동측 고환, 2명에서 반대편 고환, 그리고 1명은 양측에서 잠복고환이 관찰되었다. 후복막 및 골반 임파절에 대한 방사선치료 선략의 중앙치는 2900cGy (1550-4550cGy)이었다. 종격동조사를 시행한 환자는 1기 1명, IIA기 4명, IIB기 1명 그리고 재발된 환자가 1명이었다. 항암화학요법은 IIB기 1명, IIC기 1명, 재발된 1명에서 방사선치료 전에 시행되었다. 환자들의 중앙추적기간은 104개월 (3-144개월) 이다. 결과 : 전체 국소치유율은 5년에 100%를 보였고, 1기 환자 1명에서 종격동 전이로 인한 사망을 보여 생존율은 5년에 1기 94.4%, 11기 100%, 그리고 재발한 경우에서 100%를 보였다. 결론 : 고환 정상피종에 있어서 방사선치료는 근치적 고환절세술 후의 보조적 목적뿐 아니라 국소적으로 재발한 경우에 있어서도 우수한 치료방법으로 생각된다.

• Radiation Oncology Journal
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• 제28권3호
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• pp.133-140
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• 2010

목 적: 방사선 치료 후에 방사선조사범위내에서 재발한 골반암 환자들을 대상으로 나선형 토모테라피를 이용한 재 치료를 시행한 후 그 효과 및 안전성에 대하여 후향적 분석을 시도하였다. 대상 및 방법: 총 14명의 환자를 대상으로 하였으며, 남녀 비는 1 : 1이었고, 평균연령은 51세였다. 직장암이 8명, 자궁경부암이 5명, 원발부위를 알 수 없는 경우가 1명이었다. 13명의 환자들이 재발 전에 수술을 받은 적이 있고, 환자들의 골반부위에 조사된 방사선량의 중앙값은 50.4 Gy였다. 재발 당시 종양크기의 중앙값은 3.5 cm (2.0~7.5cm)였으며, 재발이 발견된 후 항암화학요법을 먼저 받은 경우가 9명, 고강도초음파치료를 받은 경우가 1명이었다. 나머지 4명은 재발 후에 다른 치료는 시행하지 않은 상태에서 토모테라피를 이용한 재치료를 받았다. 재치료 당시 종양으로 인한 통증을 호소한 환자는 7명이었으며, 종양 표지자가 증가해있던 경우는 8명이었다. 최초 방사선 치료와 재치료의 간격은 중앙값 19.9개월(5.0~75.4개월)이었다. 토모테라피를 이용한 재치료시 육안적종양체적, 임상적표적체적 및 계획용표적체적에 총4~5주간 20~25회에 걸쳐, 각각 중앙값 50 Gy, 47.8 Gy 그리고 45 Gy의 방사선이 조사되었다. 치료 전 매일 초고압전산화단층촬영(megavoltage computed tomography)을 통하여 X, Y, Z축 및 회전각도에 대한 online correction을 시행하였다. 치료 후 반응평가는 Response Evaluation Criteria in Solid Tumors ver. 1.0을 이용하였으며, 국소종양조절기간은 치료 종료 후 Kaplan-Meyer법으로 계산하였고, 치료 후 합병증의 평가는 Common Terminology Criteria for Adverse Event ver. 3.0을 이용하였다. 결 과: 추적기간의 중앙값은 17.3개월(3.0~38.3개월)이었으며, 영상학적반응율은 64.3%(완전관해, 7.1%; 부분관해, 57.2%)였다. 치료 전 통증이 있었던 7명의 환자 중 6명에서 통증이 완화되어 85.7%의 통증완화율을 보였으며, 종양 표지자가 증가되어 있던 8명 중 5명(62.5%)에서 감소가 일어났다. 국소종양조절기간은 중앙값 25.8개월(95% 신뢰구간, 6.12~45.5개월)이었으며, 치료 후 국소재발이 8명(57.1%), 원격전이가 3명(21.4%) 그리고 국소재발과 원격전이가 동시에 일어난 경우가 1명(7.1%)이었다. 합병증으로는 대부분 grade I 혹은 II의 급성 피부염, 직장염, 방광염 등이 나타났지만, 만성 합병증이나 grade IV 이상의 급성 합병증은 나타나지 않았다. 결 론: 방사선치료 후 방사선조사 범위 내에서 재발한 골반암에서 나선형 토모테라피를 이용한 재치료는 비교적 안전하고 효과적인 고식적 치료법이라고 생각된다. 그러나 치료 효과를 좀 더 높이기 위해서 혹은 적은 환자수, 다양한 질환 그리고 후향적 분석이라는 본 연구의 문제점을 극복하기 위해서는 많은 환자들을 대상으로 한 전향적 임상연구가 필요할 것이다.