• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.23-29
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• 2018

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is a rare autosomal recessive urea cycle disorder characterized by hyperammonemia. CPS1D is caused by mutations in the CPS1 gene on chromosome 2q35. Based on the age of onset, there are two phenotypes: the neonatal type and the delayed-onset type. The severity of clinical manifestation depends on the degree of CPS1 residual enzymatic activity, and can result in hyperammonemia and neurological dysfunction. We report a case of CPS1D in a neonate who developed vomiting, decreased consciousness and hyperammonemia at 25th day after birth. She showed excellent response to treatment including hydration, ammonia-lowering drugs and a low-protein diet without hemodialysis. Her growth, development and neurological outcomes were fair at the last follow-up at 17 months of age.

• Journal of Radiation Protection and Research
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• v.23 no.3
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• pp.149-157
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• 1998

An analysis has been performed to estimate the additional number of workers and the additional collective dose in man-cSv which would be required, nuclear industry-wide as a result of reducing individual dose limit. This analysis can be extended to the reduction in the dose limits recommended by ICRP Publ.60 and BEIR V report as well as the proposed dose limits by regulatory authorities. An industry-wide database was employed in the analysis based on a summary of industry-wide occupational radiation exposure compiled by the Korea Radioisotope Association. Correlation model was employed to compute the affects of setting specific annual individual dose limits. In this study, we have addressed worker non-productivity while in the radiation environment on a parametric or 'sensitivity analysis' basis. This alleviates the need for developing such data underlying a summation of many individual tasks at many nuclear facilities. It has the advantage that very low non-productivity assumptions can readily be defended as conservative, in that it is difficult to approach such low worker non-productivity factors even in the best of environments in any industry. On a per facility basis, for calendar year 1997, the number of workers required would be increased from 231 workers to 269 workers and collective man-cSv dose would be also increased by approximately fourteen percent if the individual dose limit was reduced to 2 cSv/y and an individual worker non-productivity fraction of 0.1 is assumed.

• Tuberculosis and Respiratory Diseases
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• v.53 no.3
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• pp.337-343
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• 2002

A tracheal bronchus, an aberrant bronchus arising directly from the trachea, is an infrequent congenital anomaly. The incidence of this anomaly ranges from 0.5 to 5%. It usually originates from the right lateral wall of the trachea at the level <2 cm above the tracheal bifurcation. These patients usually are asymptomatic, but some patients may experience recurrent pneumonia, chronic bronchitis, bronchiectasis, or asthmatic episodes. A tracheal bronchus may be associated with other anomalies such as a tracheal stenosis, pulmonary agenesis, pulmonary sequestration, congenital heart disease, a pulmonary venous anomaly and Down's syndrome. This anomaly is usually diagnosed incidentally during bronchoscopy in patients with respiratory problems. Here we report a case of a 20-year-old man with a past history of bronchial asthma, which was incidentally diagnosed as a tracheal bronchus during a medical examination prior to military service, and was associated with a bilateral superior vena cava anomaly.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.805-811
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• 1996

Congenital cystic adenomatoid malformation of the Lung(CCAM) is characterized by anomalous fetal development of terminal respiratory structures, resulting in an adenomatoid proliferation of bronchiolar elements and cystic formation. CCAM was first described and differentiated from other cystic lung disease in the English literature by Ch'in and Tang in 1949. CCAN is a rare, potentially lethal form of congenital pulmonary cystic disease and the salient features of lesion are an irregular network of terminal respiratory bronchiole-like structures and macrocysts variably lined by pseudostratified ciliated columnar epithelium and simple cuboidal epithelium. Adult presentation of CCAM of the lung is so rare that only 9 cases have been reported in the literature of date. The pathogenesis of CCAM remains disputed and reseachers have variously proposed that the lesion represents a developmental anomaly, hamartoma, or a fonn of pulmonary dysplasia. Van Dijk and Wagenvoort divided CCAM into three subtypes : cystic, intermediated, and solid. These correspond to types I, II, and III of Stocker. In adults, the evaluation of cystic or multi cystic lung disease requires consideration of a differential diagnosis including the acquired lesions of lung abscess, cavitary neoplasm or inflammatory mass, bullous disease, bronchiectasis, and postionflammatory pneumatocele. Congenital lesions such as sequestration, bronchopulmonary-foregut anomalies, and bronchogenic cyst are also encounted. The definitive treatment for CCAM is complele removal of the involved lobe. Panial lobectomy leads to multiple complications, including severe post-operative infection. We report a case of CCAM in a 14-year-old female presentated with a pneumothorax and large bullae, who was treated by surgical remove of the involved lobe.

• The Journal of Pediatrics of Korean Medicine
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• v.15 no.1
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• pp.117-129
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• 2001

Objective : Hyperlipidemia and hypertension are well recognized risk factors of cerebrovascular disease. So it's very important to evaluate blood pressure and s-cholesterol in adolescence. Material and Methods : April 1999, we were requested health examinaion for high school students of a boys high school and a girls high school in Jeonbuk, Korea. The subjects were 360 students, boys were 317 and girls were 343. They were checked their height, body weight, blood pressure, and s-cholesterol. Results and Conclusion : The mean of systolic blood pressure of study subjects was $111.89{\pm}12.43mmHg$. In boys, it was $116.25{\pm}11.78mmHg$, and in girls, it was $107.87{\pm}11.64mmHg$. The mean of diastolic blood pressure of study subjects was $68.45{\pm}9.40mmHg$. In boys, it was $66.92{\pm}10.00mmHg$, and in girls it was $69.85{\pm}9.40mmHg$. Hypertensives were 18(5.68%) in boys, 10(2.92%) in girls. The mean of s-cholesterol of study subjects was $174.95{\pm}32.28mg/dL$. In boys, it was $178.91{\pm}34.51mg/dL$, and in girls, it was $171.29{\pm}29.66mg/dL$. Hypercholesterolemias were 91(28.71%) in boys, 39(11.37%) in girls. The mean of BMI of study subjects was $21.12{\pm}3.27kg/m2$. In boys, it was $21.05{\pm}3.26kg/m2$, and in girls, it was $21.18{\pm}3.29kg/m2$. Obese boys were 18(5.68%), obese girls were 13(3.79%). The mean of diastolic blood pressure in obese boys was significantly higher than that of normal boys(p=0.001, Mann-Whitney test). The mean of s-cholesterol in obese boys($BMI{\ge}274$) was significantly higher than that of normal boys.(P=0.26, Mann-Whitney test).

• Journal of Korean Neurosurgical Society
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• v.49 no.5
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• pp.267-272
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• 2011

Objective : Although iliac crest autograft is the gold standard for lumbar fusion, the morbidity of donor site leads us to find an alternatives to replace autologous bone graft. Ceramic-based synthetic bone grafts such as hydroxyapatite (HA) and b-tricalcium phosphate (b-TCP) provide scaffolds similar to those of autologous bone, are plentiful and inexpensive, and are not associated with donor morbidity. The present report describes the use of Polybone$^{(R)}$ (Kyungwon Medical, Korea), a beta-tricalcium phosphate, for lumbar posterolateral fusion and assesses clinical and radiological efficacy as a graft material. Methods : This study retrospectively analyzed data from 32 patients (11 men, 21 women) who underwent posterolateral fusion (PLF) using PolyBone$^{(R)}$ from January to August, 2008. Back and leg pain were assessed using a Numeric Rating Scale (NRS), and clinical outcome was assessed using the Oswestry Disability Index (ODI). Serial radiological X-ray follow up were done at 1, 3, 6 12 month. A computed tomography (CT) scan was done in 12 month. Radiological fusion was assessed using simple anterior-posterior (AP) X-rays and computed tomography (CT). The changes of radiodensity of fusion mass showed on the X-ray image were analyzed into 4 stages to assess PLF status. Results : The mean NRS scores for leg pain and back pain decreased over 12 months postoperatively, from 8.0 to 1.0 and from 6.7 to 1.7, respectively. The mean ODI score also decreased from 60.5 to 17.7. X-rays and CT showed that 25 cases had stage IV fusion bridges at 12 months postoperatively (83.3% success). The radiodensity of fusion mass on X-ray AP image significantly changed at 1 and 6 months. Conclusion: The present results indicate that the use of a mixture of local autologous bone and PolyBone$^{(R)}$ results in fusion rates comparable to those using autologous bone and has the advantage of reduced morbidity. In addition, the graft radiodensity ratio significantly changed at postoperative 1 and 6 months, possibly reflecting the inflammatory response and stabilization.

• Journal of Acupuncture Research
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• v.17 no.3
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• pp.265-276
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• 2000

Objectives : The purpose of this study is to observe the clinical characteristics of 6 patients who were not improved after admitted conservative treatment in this 311 patients. Methods : We retrospectivety reviewed 311 patients of Herniated Lumbar Intervertebral Disc which undenvent admitted conservative treatment from November 1997 to June 2000. 311 patients were diagnosed by computed tomography or magnetic resonance imaging in addition to physical examination. Other complications were not detected in 311 patients with Hemiated Lumbar Intervertebral Disc. In case that the scoliosis appeared to caused by severe pain, we included this scoliosis patient with the object of survey. Results : 1. In 6 patients who were not improved, 4 patients were large herniation and 2 patients were medium herniation. 2. In all of 6 patients, the angle of straight leg raising test was low and was not imporved unril discharge. 3. X-ray findings showed straightening in all of 6 patients, disc space narrowing in 3 of 6 patients. 4. X-ray findings showed scoliosis in 3 of 4 patients who were severe pain. 5. The onset was relarively long from 30 days to 7 years. 6. The age was relatively young from 10's to 30's. 7. The adimission date was ralatively long from 32 days to 105 days. Conclusions : In 6 patients, there were observed much volume of disc herniation, low angle of straight leg raising test, young people in an age group, straightening of x-ray finding.

• Tuberculosis and Respiratory Diseases
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• v.47 no.6
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• pp.843-849
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• 1999

The prevalence of nonspecific interstitial pneumonitis(NSIP) in HIV-positive patients with pulmonary disease has varied from 11 to 38%. But NSIP in HIV-positive patients is indistinguishable from Pneumocystis carinii pneumonia(PCP) clinically and radiologically. The number of HIV-positive patients is less in Korea than in western developed countries, so little attention has been paid to the differential diagnosis between NSIP and PCP. We report a case of NSIP in HIV-positive, 61-year-old man which mimicked PCP. He presented with cough, sputum and mild exertional dyspnea. Lung sound was clear. But, chest X-ray and HRCT demonstrated diffuse patch and bilateral ground-glass opacities in central and perihilar area of both lung. Microbial pathogens were not found on sputum, BAL fluid and tissues taken by TBLB. In transbronchial lung biopsy specimen, interstitial infiltrates with lymphocytes were distributed on peribronchiolar regions. A pathologic diagnosis of NSIP was suggested, he received symptomatic treatment. The follow-up chest X-ray showed near complete resolution.

• Tuberculosis and Respiratory Diseases
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• v.63 no.2
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• pp.183-187
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• 2007

A glomus tumor is composed of modified smooth muscle cells that are similar to those of the glomus body. Its occurrence in the trachea is quite rare. To our knowledge, only 20 cases of glomus tumor of the trachea have been reported worldwide and there has been only 1 case in Korea. We report one case of a 58-year-old man with dyspnea who had a glomus tumor in the upper trachea with a review of literatures. The chest CT scan and bronchoscopy demonstrated a 2.5 cm sized lobulated tumor at the posterior wall of the upper trachea. It had an elongated shape with a broad base and with a highly vascularity. A simple resection of 3 levels of the trachearing was with a tumor and end-to-end anastomosis performed. Microscopic and immunohistochemical staining of the tumor revealed the characteristics of a glomus tumor.

• Tuberculosis and Respiratory Diseases
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• v.63 no.5
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• pp.449-453
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• 2007

Totally implanted central venous access devices for chemotherapy (chemoport) are being used increasingly in lung cancer patients. Vascular catheters are associated with various complications including infection, thrombosis as well as spontaneous fractures and embolization of the catheter, which is known as 'pinch-off syndrome'. 'Pinch-off syndrome' refers to the compression of a subclavian central venous catheter between the clavicle and first rib resulting in an intermittent or permanent obstruction, which can lead to tears, transection, or embolization. We report two cases of fractured and embolized implanted subclavian venous catheters in which the fragments were removed percutaneously. A 62-year-old man presented with back pain with a duration of a few weeks. The chest radiograph revealed complete transsection and embolization of the catheter into the right atrium. In addition, a 47-year-old woman with a chemoport had a grade 3 pinch-off sign in a chest radiograph demonstrating complete transsection and embolization of the catheter into the pulmonary artery. Both cases were managed by retrieving the embolized distal fragment percutaneously and removing the proximal section of the catheter.