• Maxillofacial Plastic and Reconstructive Surgery
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• v.36 no.6
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• pp.292-297
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• 2014

Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal genetic disease caused by a PTCH mutation. The disease is characterized by multiple basal cell carcinomas of the skin, multiple keratocystic odontogenic tumors (KCOTs) in the jaw, palmar and/or plantar pits, bifid ribs, ectopic calcification of the falx cerebri, and skeletal abnormalities. Early diagnosis is difficult in many cases because there may be a number of systemic symptoms. The purpose of this study is to report the case of a 12-year-old girl who was hospitalized with multiple KCOTs that occurred in the upper and lower jaws. Through characteristic clinical symptoms and radiologic findings, she was finally diagnosed as having NBCCS. This study also aims to organize the symptoms often observed in Korea using previously published case reports to provide useful information for the early diagnosis of NBCCS.

• The Journal of Internal Korean Medicine
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• v.21 no.3
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• pp.529-533
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• 2000

Intracranial tuberculoma is very rare nowadays. It is very difficult to differentiate tuberculoma from other space-occupying lesions. CT and MRI and intensive systemic review is very useful to diagnose intracranial tuberculoma. This case of patient had several symptoms of hemiparesis, headache. And we supposed that his symptoms were due to cerebrovascular accident at entry. But he was diagnosed as tuberculoma with brain edema in cerebrum. After treating him with mannitolization, acupuncture and herb medicine, his symptoms were remarkably improved. So we report this case of tuberculoma in cerebrum.

• Journal of Chest Surgery
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• v.25 no.12
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• pp.1563-1569
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• 1992

From Jan. 1981 to Dec. 1991, we had treated 25 patients with congenital coronary art-eriovenous fistulas [CAVF] in Seoul National University Hospital. A retrospective review was made to delineate the course and the management of CAVF and to clarify the role of surgical treatment. Fifteen patients were male and 10 were female with The mean age of 17.4 years[from 3 months to 58 years]. The most frequent symptom was dyspnea on exertion[56%]. Other symptoms were angina and palpitation. Sixty-eight percent of the patients were symptomatic. Fifty-three percent of patients less than 20 years old were symptomatic and 100% of patients over 20 years old were symptomatic. Three patients had multiple CAVFs. The fistula drained into the right ventricle in 13, pulmonary artery in 9, left ventricle in 4, right atrium in 2, and left atrium in 1. Thirteen patients had other associated cardiac lesions. The mean pulmonary-to-systemic blood flow[Qp /Qs] in the isolate CAVF group was 2.19. All patients were operated on to correct the fistulas and other associated cardiac lesions. All patients were followed from 1 month to 11 years without late death. Postoperative complication rate was 24% -significant arrhythmia [3], recurred CAVF[1], psychosis[1], pneumonia [1]. Symptomatic improvement was evident postoperatively. Below 20 years old, 94% of patients were asymptomatic, but above 20 years old, symptoms persisted in 25%. In summary, early elective repair of CAVF is indicated in all patients because of higher complication rate and frequent persistent symptoms in older patients.

• Molecules and Cells
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• v.25 no.1
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• pp.64-69
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• 2008

Although the arthritis symptoms observed in the K/BxN model have been shown to be dependent on the functions of T and B cells specific to the self antigen glucose-6-phosphate isomerase, less is known about the in vivo roles of $CD4^{+}CD25^{+}$ regulatory T($T_{reg}$) cells in the pathology of K/BxN mice. We determined the quantitative and functional characteristics of the $T_{reg}$ cells in K/BxN mice. These mice contained a higher percentage of $Foxp3^+\;T_{reg}$ cells among the $CD4^+$ T cells than their BxN littermates. These $T_{reg}$ cells were anergic and efficiently suppressed the proliferation of $na\ddot{i}ve$ $CD4^+$ T cells and cytokine production by effector $CD4^+$ T cells in vitro. Antibody-mediated depletion of $CD25^+$ cells caused K/BxN mice to develop multi-organ inflammation and autoantibody production, while the symptoms of arthritis were not affected. These results demonstrate that despite the inability of the $T_{reg}$ cells to suppress arthritis development, they play a critical role protecting the arthritic mice from systemic expansion of autoimmunity.

• The Journal of the Korean bone and joint tumor society
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• v.13 no.1
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• pp.43-47
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• 2007

Sj$\"{o}$gren's syndrome is a systemic, autoimmune disorder of the exocrine glands with associated lymphocytic infiltrates of the affected glands and characteristic of dry eye and mouth. We report a case of Sj$\"{o}$gren's syndrome in which the patient presented systemic symptoms without sicca symptoms and had radiologic findings mimicking multiple skeletal metastasis and was finally confirmed with Sj$\"{o}$gren's syndrome with renal tubualr acidosis.

• Journal of Acupuncture Research
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• v.30 no.4
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• pp.203-210
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• 2013

Objectives : The purpose of this study is to report clinical effect of oriental medical for a not improved ITP patient, who developed complication from DVT, after intravenous immunoglobulin treatment. Methods : The patient was treated using Saam acupuncture, herbal medication, moxibustion and physical treatment. And we measured of systemic symptoms and platelet counts. Results : After treatment, systemic symptoms and platelet counts were improved in case. Conclusions : Oriental medical treatment showed significant effect on ITP patient that not improved platelet counts after intravenous immunoglobulin treatment.

• Journal of The Korean Society of Clinical Toxicology
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• v.7 no.1
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• pp.10-18
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• 2009

Purpose: Carbon Monoxide Poisoning is an important medical emergency in Korea, but the factors leading to its serious manifestation are not well studied. Methods: We conducted a 5-year retrospective study of 98 carbon monoxide poisoned patients who visited the emergency departments of the Medical Center between December 2004 and March 2009. We categorized the patients into those exhibiting only local symptoms (group 1) and those showing systemic symptoms and complications (group 2). We compared the general characteristics as well as the clinical and laboratory findings between both groups. Results: The male to female ratio was 1.18. The most common systemic symptom was a mental change (43.9%), while the most common complication was rhabdomyolysis (31.6%). Poisoned area closed private room in group 2 were 23 (41.8%) cases and burning region in group 2 were 16(29.1%) cases (p=0.956). Individuals who were accidentally poisoned comprised of 43 (78.2%) cases while those that attempted suicidal poisoning comprised 12 (21.8%) cases (p=0.016). The most common symptom at arrival was mental change 33 (60.0%) cases in group 2. The mean time exposed to carbon monoxide was 43$\pm$3.97 hours in group 1 and 55$\pm$10.11 in group 2 (p=0.012). The patient's age, context of poisoning, symptom at arrival, and time exposed to the poison were found to be significant risk factors for complications by logistic regression analysis. Conclusion: Carbon monoxide poisoning is an emergency medical condition and the risk factors involved in the development of serious complications must be evaluated.

• Tuberculosis and Respiratory Diseases
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• v.82 no.4
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• pp.285-297
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• 2019

Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the cooccurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjogren's syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.

• Korean Journal of Head & Neck Oncology
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• v.38 no.2
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• pp.23-27
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• 2022

Systemic lupus erythematosus(SLE) is a multisystemic disorder of autoimmune etiology. SLE can occur commonly in young women, and the early symptoms include fever, myalgia, arthralgia, weight loss, lymphadenopathy and these nonspecific symptoms develop into skin rash, splenomegaly, serositis and encephalopathy. Diagnosis of SLE requires clinical and serologic criteria, and treatment choices are hydroxyquinolone and NSAIDs for mild disease, corticosteroids and immunosuppressant for severe disease. In lupus patient, the prevalence of lymphadenopathy is 12~59%. Although lymphadenopathy is common finding in SLE, it is hard to distinguish in early phase of SLE. A 38-year-old woman visited our hospital for cervical lymphadenopathy with polyarthritis and malaise. Multiple cervical lymph nodes enlargement was found on Neck CT, and serologic laboratory test including ANA, antiphospholipid antibody, and anti-dsDNA was positive. For excluding lymphoma, PET-CT and excisional biopsy were performed. The patient finally diagnosed with SLE, and got regular follow-up without complication.

• Pediatric Infection and Vaccine
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• v.29 no.3
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• pp.166-172
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• 2022

Blau syndrome is a systemic autoinflammatory disease presenting with non-caseating granulomatous dermatitis, chronic uveitis, and arthritis. It is caused by a gain-of-function variant of the nucleotide-binding oligomerization domain protein 2 gene, which leads to the overactivation of inflammatory cytokines and eventually causes autoinflammation. Since the symptoms of Blau syndrome are nonspecific and usually do not appear simultaneously, it is challenging to differentiate Blau syndrome from other inflammatory disorders. This is a case report of a 13-month-old boy who had suffered from recurrent skin rash and fever. The patient was previously misdiagnosed as refractory Kawasaki disease twice and was treated with intravenous immunoglobulin and systemic glucocorticoid, which only resulted in transient improvement of the symptoms. He was eventually diagnosed with Blau syndrome.