• Tuberculosis and Respiratory Diseases
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• v.45 no.5
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• pp.1058-1066
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• 1998

Background: Actinomycotic infection is uncommon and primary actinomycosis of the lung and chest wall has been less frequently reported. This disease may present as chronic debilitating illness with radiologic manifestation simulating lung tumor, pulmonary infiltrating lesion, or chronic suppuration. Diagnosis of choice was not definded yet and role of bronchoscopy on diagnosis was not described yet. Methods: From 1989 to 1998, we experienced 17 cases of thoracic actinomycosis. We have reviewed the case notes of 17 patients with thoracic actinomycosis. The mean age at presentation was $53{\pm}13$ years, 11 were male. Results: Cough, hemoptysis, sputum production, chest pain and weight loss were the commonest symptoms. The mean delay between presentation and diagnosis was $6.6{\pm}7.8$ months. There were six patients who presented with a clinical picture of a suppurative lesion and eleven patients were suspected of having primary lung tumor initially. In no cases was made an accurate diagnosis at the time of hospital admission. Associated diseases were emphysema (1 case), bronchiectasis (2 cases) and tuberculosis (2 cases). Bronchoscopic findings were mucosal swelling and stenosis(n=4), mucosal swelling, stenosis and necrotic covering (n=2), mass (n=3), mass and necrotic covering (n=1) and normal(n=6). Radiologic findings were mass lesion(n=8), pneumonitis(n=3), atelectasis(n=3), pleural effusion(n=2), and normal(n=3). Final diagnosis was based on percutaneous needle aspiration and biopsy (n=3), bronchoscopic biopsy specimens (n=9), mediastinoscopic biopsy (n=1) and histologic examination of resected tissue in the remaining patients(n=4) who received surgical excision. Among 17 patients, 13 were treated medically and the other 4 received surgical intervention followed by antibiotic treatment. Regarding the surgically treated patients, suspected malignancy is the most common indication for operation. However. both medically and surgically treated patients achieved good clinical results. Conclusion: Thoracic actinomycosis is rare. but should still be considered in the differential diagnosis of a chrinic, localized pulmonary lesion. Thoracic actinomycosis may co-exist with pulmonary tuberculosis or lung cancer. If the lesion is located in the central of the lung. the bronchoscopy is recommanded for the diagnosis.

• Research in Community and Public Health Nursing
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• v.10 no.1
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• pp.80-92
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• 1999

The purpose of this study was to survey appropriateness of admission and days of care for the cerebral ischemia patients as a basis to provide an effectiveness of hospitalization. The authors retrospectively reviewed the medical records of cerebral ischemia patients in two hospitals from November 1997 to February 1998. Of 194 medical records reviewed, there were 2108 medical days. I t is used a 'Appropriateness Evaluation Protocol' previously developed by Gertman and Restuccia (1981) and translated by Department of Health Management, Seoul National University and Korea Institute for Health Services Management (1993), It was found that the 'Appropriateness Evaluation Protocol' had a high inter-rater reliability(k=.92), Statistical significant was tested by using the percentage, mean, and logistic regression by SAS 6.12. The results were as follows; 1. The appropriate admissions were 87.6%, days of care 63.4%, and the average length of stay $10.9{\pm}6.7$ days. 2. The reasons of inappropriate admissions were for work-up(75.0%) and conservative care (25.0%). Major reasons of inappropriate days of care were 'cases in which the medical purpose of hospitalization has been accomplish or can be addressed in a less setting(45.0%)', and 'cases in which there is a delay in performing the work-up or treatment which required patients is hospitalized (44.4%)'. 3. Appropriate days of care were higher as ageing. Appropriate days of care were higher in patients with lower accademic back ground than those of upper college graduates, and in the patients who enter a hospital via emergency room than out-patients department. Appropriate days of care were higher in the patient with MCA infarction, and lower in the patient with cerebellar infarction than the patient with lacunar infarction. Appropriate days of care were higher in attack first than attack above second, in nomortension patients than hypertensive, and lower in groups who engaged in semi-private room and public room than private room in hospital. Appropriate days of care were higher in shorter length of stay than longer length of stay. 4. Diagnosis, admission path, and appropriate days of care explained appropriate admissions. Diagnosis, appropriate admissions, hypertension explained appropriate days of care. According to the above results, author confirms the substantial amount of inappropriate hospital bed utilization. To reduce inappropriateness, it is necessary to develop some alternative services such as home care services or nursing home with which can be replaced inpatient services and to introduce policy such as case management which includes Critical Pathway for consistent management. And, it should be followed the further study for the effectiveness.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.1
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• pp.29-35
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• 2020

Joubert syndrome (JS) is a rare genetic disorder that is characterized by ataxia, hypotonia, developmental delay, respiratory abnormalities such as apnea-hyperpnea, and abnormal eye movements. The pathognomonic diagnostic finding is the "molar tooth sign" (MTS) on brain magnetic resonance imaging (MRI), described as cerebellar vermis hypoplasia or dysplasia, thick and horizontally oriented superior cerebellar peduncles, and an abnormally deep interpeduncular fossa. JS is characterized by genetic heterogeneity: pathogenic variants in over 30 genes have been identified to date. The CEP290 protein, which is on chromosome 12q21.3, is most frequently mutated in patients with JS, especially with renal involvement. Here, we report a case of JS in a 14-year-old male patient with end-stage renal disease. To the best of our knowledge, this is the first Korean report of a patient with JS due to CEP290 mutation (c.6012-12T> A) whose diagnosis was confirmed after repetitive MRI. We suggest consultation with an experienced neuro-radiologist and follow-up MRI studies to detect a "hidden" MTS if clinical findings suggest a diagnosis of JS. Furthermore, even in the absence of an MTS, whole exome sequencing should be considered.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.2
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• pp.925-928
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• 2014

Background: Gestational trophoblastic neoplasia (GTN) is a spectrum of disease with abnormal trophoblastic proliferation. Treatment is based on FIGO stage and WHO risk factor scores. Patients whose score is 12 or more are considered as at extremely high risk with a high likelihood of resistance to first line treatment. Optimal therapy is therefore controversial. Objective: This study was conducted in order to summarize the regimen used for extremely high risk or resistant GTN patients in our institution the in past 10 years. Materials and Methods: All the charts of GTN patients classified as extremely high risk, recurrent or resistant during 1 January 2002 to 31 December 2011 were reviewed. Criteria for diagnosis of GTN were also assessed to confirm the diagnosis. FIGO stage and WHO risk prognostic score were also re-calculated to ensure the accuracy of the information. Patient characteristics were reviewed in the aspects of age, weight, height, BMI, presenting symptoms, metastatic area, lesions, FIGO stage, WHO risk factor score, serum hCG level, treatment regimen, adjuvant treatments, side effects and response to treatment, including disease free survival. Results: Eight patients meeting the criteria of extremely high risk or resistant GTN were included in this review. Mean age was 33.6 years (SD=13.5, range 17-53). Of the total, 3 were stage III (37.5%) and 5 were stage IV (62.5%). Mean duration from previous pregnancies to GTN was 17.6 months (SD 9.9). Mean serum hCG level was 864,589 mIU/ml (SD 98,151). Presenting symptoms of the patients were various such as hemoptysis, abdominal pain, headache, heavy vaginal bleeding and stroke. The most commonly used first line chemotherapeutic regimen in our institution was the VAC regimen which was given to 4 of 8 patients in this study. The most common second line chemotherapy was EMACO. Adjuvant radiation was given to most of the patients who had brain metastasis. Most of the patients have to delay chemotherapy for 1-2 weeks due to grade 2-3 leukopenia and require G-CSF to rescue from neutropenia. Five form 8 patients were still survived. Mean of disease free survival was 20.4 months. Two patients died of the disease, while another one patient died from sepsis of pressure sore wound. None of surviving patients developed recurrence of disease after complete treatment. Conclusions: In extremely high risk GTN patients, main treatment is multi-agent chemotherapy. In our institution, we usually use VAC as a first line treatment of high risk GTN, but since resistance is quite common, this may not suitable for extremely high risk GTN patients. The most commonly used second line multi-agent chemotherapy in our institution is EMA-CO. Adjuvant brain radiation was administered to most of the patients with brain metastasis in our institution. The survival rate is comparable to previous reviews. Our treatment demonstrated differences from other institutions but the survival is comparable. The limitation of this review is the number of cases is small due to rarity of the disease. Further trials or multicenter analyses may be considered.

• Journal of the Korean Society of Radiology
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• v.6 no.1
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• pp.19-26
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• 2012

It is very important for early diagnosis and therapy with ischamic cerebral infarction patients. This study was to know the ischemic penumbra lesion which compared CT-perfusion and diffusion weighted MRI(DWMRI) with acute cerebral infarction patients. 12 acute cerebral infarction patients had performed perfusion CT and performed DWMRI. Perfusion images including cerebral blood volume(CBV), cerebral blood flow(CBF), time to peak(TTP) and mean transit time(MTT) maps obtained the values with defect lesion and contralateral normal cerebral hemisphere and DWMRI was measured by signal intensity and compared of lesion size between each perfusion map. All perfusion CT maps showed the perfusion defect lesions in all patients. There were remarkable TTP and MTT delay in perfusion defect lesions. The lesions on CBF map was the most closely correlated with the lesions on DWMRI. The size of perfusion defect lesions on TTP and MTT map was larger than that of lesions on DWMRI, suggesting that MTT map can evaluate the ischemic penumbra. Perfusion CT maps make it possible to evaluate not only ischemic core and ischemic penumbra, but also hemodynamic status in the perfusion defect area. These results demonstrate that perfusion CT can be useful to the diagnosis and treatment in the patients with acute cerebral ischemic infarction.

• Korean Journal of Radiology
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• v.23 no.4
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• pp.413-425
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• 2022

Objective: We compared appendiceal visualization on 2-mSv CT vs. conventional-dose CT (median 7 mSv) in adolescents and young adults and analyzed the undesirable clinical and diagnostic outcomes that followed appendiceal nonvisualization. Materials and Methods: A total of 3074 patients aged 15-44 years (mean ± standard deviation, 28 ± 9 years; 1672 female) from 20 hospitals were randomized to the 2-mSv CT or conventional-dose CT group (1535 vs. 1539) from December 2013 through August 2016. A total of 161 radiologists from 20 institutions prospectively rated appendiceal visualization (grade 0, not identified; grade 1, unsure or partly visualized; and grade 2, clearly and entirely visualized) and the presence of appendicitis in these patients. The final diagnosis was based on CT imaging and surgical, pathologic, and clinical findings. We analyzed undesirable clinical or diagnostic outcomes, such as negative appendectomy, perforated appendicitis, more extensive than simple appendectomy, delay in patient management, or incorrect CT diagnosis, which followed appendiceal nonvisualization (defined as grade 0 or 1) and compared the outcomes between the two groups. Results: In the 2-mSv CT and conventional-dose CT groups, appendiceal visualization was rated as grade 0 in 41 (2.7%) and 18 (1.2%) patients, respectively; grade 1 in 181 (11.8%) and 81 (5.3%) patients, respectively; and grade 2 in 1304 (85.0%) and 1421 (92.3%) patients, respectively (p < 0.001). Overall, undesirable outcomes were rare in both groups. Compared to the conventional-dose CT group, the 2-mSv CT group had slightly higher rates of perforated appendicitis (1.1% [17] vs. 0.5% [7], p = 0.06) and false-negative diagnoses (0.4% [6] vs. 0.0% [0], p = 0.01) following appendiceal nonvisualization. Otherwise, these two groups were comparable. Conclusion: The use of 2-mSv CT instead of conventional-dose CT impairs appendiceal visualization in more patients. However, appendiceal nonvisualization on 2-mSv CT rarely leads to undesirable clinical or diagnostic outcomes.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.2
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• pp.39-44
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• 2023

Hereditary tyrosinemia type 1 (HT-1) is a metabolic disorder caused by biallelic pathogenic variants in the fumarylacetoacetate hydrolase (FAH) gene, which impairs the function of the FAH enzyme, resulting in the accumulation of tyrosine's toxic metabolites in hepatocytes and renal tubular cells. As a consequence, individuals with HT-1 exhibit symptomatic manifestations. Rapid diagnosis and treatment of HT-1 can prevent short-term death and long-term complications. A 15-day-old boy presented to the outpatient department with elevated levels of tyrosine on his newborn screening tests conducted at the age of 3 and 10 days, respectively. Further blood tests revealed increased levels of alpha-fetoprotein and amino acids including tyrosine and threonine. Urine organic acid tests indicated a significant elevation in tyrosine metabolites, as well as the presence of succinylacetone (SA), which led to the diagnosis of HT-1. Two pathogenic and likely pathogenic variants of FAH compatible with HT-1 were also detected. He began a tyrosine-restricted diet at one month old and received nitisinone (NTBC) at two months old. With continued treatment, the patient's initially elevated AFP level, detection of SA in the urine, and mild hepatomegaly showed improvement. During four years and seven months of treatment, there were no exceptional complications apart from an increase in tyrosine levels and a delay in speech. We report a case of tyrosinemia type 1 detected through newborn screening, treated with dietary restriction and NTBC, with a good prognosis.

• Clinical and Experimental Reproductive Medicine
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• v.44 no.4
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• pp.181-186
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• 2017

With advances in the methods of cancer treatment used in modern medicine, the number of breast cancer survivors has been consistently rising. As the number of women who wish to become pregnant after being diagnosed with breast cancer increases, it is necessary to consider fertility preservation in these patients. However, medical doctors may be unaware of the importance of fertility preservation among cancer patients because most patients do not share their concerns about fertility with their doctors. Considering the time spent choosing and undergoing treatment, an early referral to a reproductive specialist is the best way to prevent a delay in cancer treatment. Since it is not easy to make decisions on matters related to cancer diagnosis and fertility, patients should be provided with enough time for decision-making, and to allow for this, an early referral will provide patients with sufficient time to choose an appropriate method of fertility preservation. The currently available options of fertility preservation for patients with breast cancer include cryopreservation of embryos, oocytes, and ovarian tissue and gonadotropin-releasing hormone agonist treatment before and during chemotherapy. An appropriate method of fertility preservation must be selected through consultations between individual patients and health professionals and analyses of the pros and cons of different options.

• Tuberculosis and Respiratory Diseases
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• v.76 no.5
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• pp.245-248
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• 2014

Miliary tuberculosis (TB) is a rare extrapulmonary form of TB, and there have been only two reports of miliary TB associated with infection with multidrug-resistant (MDR)-TB pathogen in an immunocompetent host. A 32-year-old woman was referred to our hospital because of abnormal findings on chest X-ray. The patient was diagnosed with MDR-TB by a line probe assay and was administered proper antituberculous drugs. After eight weeks, a solid-media drug sensitivity test revealed that the pathogen was resistant to ethambutol and streptomycin in addition to isoniazid and rifampicin. The patient was then treated with effective antituberculous drugs without delay after diagnosis of MDR-TB. To the best of our knowledge, this is the first case of miliary TB caused by MDR-TB pathogen in Korea.

• Korean Journal of Head & Neck Oncology
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• v.11 no.1
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• pp.24-29
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• 1995

Melanoma of the mucous membrane is a rare condition and has the worse prognosis than that of any other sites. It is usually asymptomatic in early stage and difficult to find out the primary lesion by visual examination, and these facts lead to delay in diagnosis and reduce the curability. The major factor of failure in treatment of melanoma is local recurrence rather than regional spread. Because the radical procedure is difficult in head and neck lesion due to anatomical limitation, radiation therapy and chemotherapy can be used in the treatment of local recurrence. We experienced two cases of nasopharyngeal melanoma in advanced stage and treated them with radiation therapy and/or chemotherapy. And both patients showed relatively favorable response to these palliative treatments.