• Journal of Yeungnam Medical Science
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• v.27 no.1
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• pp.63-68
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• 2010

Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that is associated with mutation in the tuberous sclerosis genes, renal angiomyolipomas, lymphatic spread and a remarkable female gender predilection. The pathology of LAM is represented by the proliferation of immature smooth muscle cells in the walls of airways, and venules and lymphatic vessels in the lung. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax and collections of chylous fluid. The diagnosis of pulmonary LAM can be made on chest X-ray, a high-resolution CT scan and lung biopsy. We experienced a case of pulmonary lymphangioleiomyomatosis in a 28-years-old female patient who had suffered from progressive dyspnea on exertion, so we report on it along with a brief review of the relevant literature.

• Journal of Korean Neurosurgical Society
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• v.30 no.10
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• pp.1237-1240
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• 2001

Spontaneous herniation of the spinal cord is a very rare. It's clinical symptom presents with progressive myelopathy. A 42-year old male patient who presented the progressive left leg weakness and Brown-Seqaurd syndrome is presented. MRI showed a typical finding of dural defect and herniation of the cord on the level of T3-4. Repair of dural defect using an artificial dura and reposition of cord herniation were undertaken after three level laminectomies with SSEP monitoring. Postoperatively, symptoms were improved rapidly. In our knowledgement, this is first case being reported in Korea. This entity, although rare, should be considered in the differential diagnosis of myelopathy in the absence of a mass lesion.

• Journal of Genetic Medicine
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• v.15 no.2
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• pp.110-114
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• 2018

Ataxia-telangiectasia (AT; OMIM 208900) is a rare autosomal recessive inherited progressive neurodegenerative disorder, with onset in early childhood. AT is caused by homozygous or compound heterozygous mutations in ATM (OMIM 607585) on chromosome 11q22. The average prevalence of the disease is estimated at 1 of 100,000 children worldwide. The prevalence of AT in the Republic of Korea is suggested to be extremely low, with only a few cases genetically confirmed thus far. Herein, we report a 5-year-old Korean boy with clinical features such as progressive gait and truncal ataxia, both ankle spasticity, dysarthria, and mild intellectual disability. The patient was identified as a compound heterozygote with two novel genetic variants: a paternally derived c.5288_5289insGA p.(Tyr1763*) nonsense variant and a maternally derived c.8363A>C p.(His2788Pro) missense variant, as revealed by next-generation sequencing and confirmed by Sanger sequencing. Based on claims data from the Health Insurance Review and Assessment Service Republic of Korea, we calculated the prevalence of AT in the Republic of Korea to be about 0.9 per million individuals, which is similar to the worldwide average. Therefore, we suggest that multi-gene panel sequencing including ATM should be considered early diagnosis.

• Journal of Electrodiagnosis and Neuromuscular Diseases
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• v.20 no.2
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• pp.112-118
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• 2018

We describe a case of a 71-year-old male patient who experienced progressive bilateral proximal upper limb weakness and atrophy without sensory symptoms and signs over 5 years. Electromyography demonstrated denervation potentials and neuropathic motor unit action potentials on C5-C7 myotome muscles bilaterally. Cervical spine magnetic resonance imaging revealed engorged anterior epidural venous plexus, T2 hyperintensity localized to grey matter ("snake-eye" appearance) at C2-C6 vertebral level, and ventral epidural fluid collection from C6 to T8 vertebral level. This case indicates that bibrachial amyotrophy associated with epidural fluid leak should be suspected in patients presenting with progressive bilateral upper limb weakness and atrophy without sensory involvement.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.1
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• pp.75-80
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• 2021

Purpose: Appendicitis is the most frequent urgency in pediatric age; the aim of this study was to investigate the association of quarantine for severe acute respiratory syndrome coronavirus 2 pandemic and the incidence of pediatric appendicitis in a specific macro area. Methods: We retrospectively analyzed the medical records of consecutive patients who underwent surgical exploration for acute appendicitis in the period March-April since 2014. This specific quarantine period was divided into two phases as indicated by National government. Patient data, demographics, characteristics and outcomes were studied and evaluated comparing patients treated during quarantine especially phase 1 vs. phase 2 (March-April 2020). Results: After reviewing medical charts following the inclusion and exclusion criteria, 155 patients were studied; focusing on the final outcome, it is possible to show a decreased amount of appendicitis during phase 1 and a progressive increase during phase 2; respect to previous years, there was a statistical increase in severity of appendicitis during quarantine (gangrenous vs. phlegmonous appendicitis). Conclusion: During this specific quarantine there was a reduction in appendicitis and a progressive increase during phase 2. These results offer new perspective among disease incidences during lockdown.

• Journal of Yeungnam Medical Science
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• v.39 no.2
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• pp.153-160
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• 2022

Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

• Journal of Korean Neurosurgical Society
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• v.46 no.1
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• pp.77-80
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• 2009

Orbital solitary fibrous tumor (SFT) is a rare tumor originating from the mesenchyme. We describe the clinical presentations, radiological and operative findings, and pathological features of a patient with orbital SFT. The patient was a 46-year-old female who presented with progressive proptosis advanced for 20 months. On ophthalmological examination, no visual impairment was detected, but left eye was found to be obviously protruded on exophthalmometry. Orbital magnetic resonance imaging showed a 2.5 ${\times}$ 2 ${\times}$ 2 cm, intensely enhanced mass in the left orbit, which compressed the eyeball forward and the optic nerve downward. The patient underwent frontal craniotomy with superior orbitotomy and gross total resection was performed for the tumor. The histopathological diagnosis including immunohistochemistry was a SFT. After the surgery, proptosis was markedly relieved without visual impairment. Although orbital SFT is extremely rare, it should be considered in the differential diagnosis of orbital tumors. Clinical presentations such as painless proptosis and CD34 immunoreactivity play a significant role in differentiating orbital SFT from other spindle-cell neoplasms of the orbit.

• Korean Journal of Head & Neck Oncology
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• v.16 no.1
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• pp.87-91
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• 2000

Neurofibromatosis type I or Von Recklinghausen's disease can occur at any site in the body. It is characterized by multiple $c\'{a}fe\;\'{a}u\;l\'{a}it$ spots on the skin-more than six spots greater than 1.5cm-, neurofibromas of the peripheral and centarl nervous system, and variety of other dysplastic abnormalities of the skin, bones, endocrine organs, nervous systems, and blood vessels. It is an autosomal dominant trait disease with a frequency of 1 of 3000. Neurofibromatosis is known to be complicated by malignancies. Neurofibromatosis is progressive disease and shows a marked variations in expression in affected individuals. In this report we describe a male patient with neurofibromatosis type I developed in the parapharyngeal space. The patient had huge mass at left parapharyngeal space and inguinal area. We successfully treated the patient with surgery without complication.

• Journal of Korean Public Health Nursing
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• v.11 no.2
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• pp.106-120
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• 1997

The Purpose of this study was to get grasp the law intelligence related to nursing duty of nurse in order to estimate a degree of mangement duty, patient mangement duty, injection duty, medication duty that nurse have. This paper made a survey of 270 nurses who were employed in general hospital of Chon-ju city. Questionnaire were composed 32 items through four dimension. The result of this survey showed that as follow : 1. Law intelligence related to nursing duty was expressed mean score 17.24 score in maxim score 32 score, mangement duty mean score 5.01 score in maxim score 11 score, patient mangement duty mean score 2.72 score in maxim score 11 score, injection duty mean score 3.84 score in maxim score 7 score, medication duty mean score 3.55 in maxim score 7 score of each domain 2. Relationships of mangement duty. patient mangement duty, injection duty. medication duty were expressed to progressive relationship of all. 3. Correlation between general features and law intelligence related to my duty.

• Journal of Pharmacopuncture
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• v.10 no.3
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• pp.143-147
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• 2007

Objective To derive further studies evaluating the effectiveness of Cultured Wild Ginseng Pharmacopuncture (CWGP) Therapy on squamous cell carcinoma as a first line. Methods Three cycles (4 weeks/cycle) of CWGP were administered as a dosage of 10 ml per day. Patient was diagnosed with stage IIIB squamous cell carcinoma and refused all therapy of conventional medicine because of old age and cardiac invasion of tumor. Intensive treatment of CWGP for 3 cycles was done on the patient. Computed Topography (CT) was performed to evaluate the therapeutic efficacy. Results After the intravenous infusion of 2 cycles of CWGP, chest CT revealed the mass size and pleural invasion sustained stable disease. After the point injection of 1 cycle of CWGP, chest CT revealed progressive disease. The disease free survival rate was 1 month. Conclusion This case may provide us the possibility that CWGP offers potential benefits for patients with squamous cell lung carcinoma. But this is a single case study and further case-series research should be compensated.