• Clinical and Experimental Pediatrics
• /
• v.52 no.1
• /
• pp.50-55
• /
• 2009

Purpose : This study aimed to determine the influence of low-dose oxygen ($FiO_2$ <25%) therapy through nasal cannulae on the progress and prognosis of retinopathy of prematurity (ROP) as well as methods of preventing ROP. Methods : Our subjects comprised premature infants (gestation period <37 weeks; birth weight <1,750 g) born in Daegu Fatima Hospital between February 1, 2001 and January 31, 2006. We retrospectively reviewed and analyzed the medical records of 273 patients who were available for eye examination and follow up over 6 months. Results : The factors maximally influencing the occurrence of ROP were low gestation age and low birth weight. We observed that the incidence of ROP increased with the increasing duration of low-dose oxygen therapy. ROP onset was delayed during ongoing oxygen therapy; however, rapid progression of ROP occurred after the discontinuation of oxygen therapy among premature infants up to the prethreshold stage. Conclusion : To prevent of occurrence of severe ROP and its rapid progression, the period of low-dose oxygen therapy needs to be shortened. Moreover, frequent eye examinations should be performed after the discontinuation of oxygen therapy.

• Journal of Chest Surgery
• /
• v.36 no.7
• /
• pp.523-526
• /
• 2003

A 44-year-old man had been admitted for dyspnea on exertion and abdominal distension. The echocardiography revealed abnormal mass in right atrium and tricuspid valve stenosis with right ventricular obliteration. The operation was performed with mass removal, enlargement of tricuspid valve opening, and right ventricular endocardiectormy. And then, atrial septal defect was made due to inadequate right ventricular volume. The patient's symptom was improved and he discharged without events. The endomyocardial fibrosis was diagnosed with microscopic examination. Eighteen months later, the patient was readmitted due to aggravated dyspnea and cyanosis. The right ventricular obliteration was progressed and pulmonary blood flow was severely decreased in follow up echocardiography. Palliative bidirectional cave-pulmonary shunt was performed due to functional single ventricle. The dyspnea and cyanosis was markedly improved. Bidirectional cavo-pulmonary shunt for advanced and isolated right ventricular endomyocardial fibrosis provided effective palliation at early postoperative period, However, long-term follow up is mandatory.

• Clinical and Experimental Pediatrics
• /
• v.53 no.4
• /
• pp.598-602
• /
• 2010

Transient hyperammonemia in a newborn is an overwhelming disease manifested by hyperammonemic coma. The majority of affected newborns are premature and have mild respiratory syndrome. The diagnosis may be difficult to determine. This metabolic disorder is primarily characterized by severe hyperammonemia in the postnatal period, coma, absence of abnormal organic aciduria and normal activity of the enzymes of the urea cycle. Hyperammonemic coma may develop within 2-3 days of life, although its etiology is unknown. Laboratory studies reveal marked hyperammonemia (>$4,000{\mu}mol/L$). The degree of neurologic impairment and developmental delay in this disorder depends on the duration of hyperammonemic coma. Moreover, the infant may succumb to the disease if treatment is not started immediately and continued vigorously. Hyperammonemic coma as a medical emergency requires dialysis therapy. Here, we report a case of severe transient hyperammonemia in a preterm infant (35 week of gestation) presented with respiratory distress, seizure, and deep coma within 48 hours and required ventilatory assistance and marked elevated plasma ammonia levels. He survived with aggressive therapy including peritoneal dialysis, and was followed 2 years later without sequelae.

• Clinical and Experimental Pediatrics
• /
• v.50 no.3
• /
• pp.315-318
• /
• 2007

This report describes the long-term follow-up of a 10-year-old female patient with Joubert syndrome with short stature and brachydactyly. She presented with hyperpnea alternated with hypopnea, uncontrolled jerking eye movements, and hypotonia during early infancy. She was diagnosed with Joubert syndrome based on clinical symptoms and typical MRI findings at 5 months of age. Abnormal ventilation and eye movements disappeared at around 4 years of age. Head circumference kept within normal range for her age, but her height and weight growth were markedly retarded. Simple X-ray showed an enlarged skull with increased digital markings, hypoplasia of facial bones, and abnormal enchondral bone formations in hands and feet. This article is the first report of Joubert syndrome with peripheral dysostosis.

• Korean Journal of Head & Neck Oncology
• /
• v.29 no.2
• /
• pp.75-78
• /
• 2013

영아의 설근부에 발생하는 악성 종양은 매우 드물다. 저자들은 연하곤란과 폐쇄성 수면 무호흡증으로 의뢰된 17개월 남아의 설근부에 발생한 횡문근육종을 경험하였기에 문헌고찰과 함께 보고하는 바이다. 외래에서 실시한 이학적 검사 상 설근부 전체가 돌출되어 있었으며 단단한 종괴가 촉지 되었다. 조직검사와 기관절개술을 시행하였으며 컴퓨터단층촬영, 양전자 방출 단층 촬영 그리고 뼈 스캔과 함께 염색체 분석을 실시하였다. 조직검사 결과 폐포성 횡문근육종이 확인되었으며 전이의 증거는 없었다. 염색체 분석상 폐포성 횡문근육종에 상응하는 PAX7-FKHR 유전자 전좌가 발견되었다. 8 회의 항암화학요법과 방사선 치료 후 촉지되는 설근부의 종괴는 없었으며 환자가 호소하는 증상도 개선되었다. 추적 관찰 시 시행된 자기공명영상 결과 확연한 종괴 크기의 감소를 확인할 수 있었다. 횡문근육종은 매우 드문 악성 종양으로 수술과 함께 항암화학요법, 방사선치료 등 여러 치료 방법이 동원되지만 전이나 재발이 있을 시 예후는 매우 불량하다. 그러므로 영아에서 연하곤란, 호흡 곤란 등의 증상이 있을 시에는 설근부를 포함한 상부호흡소화관을 적극적으로 검사하여야 하며 악성 종양의 가능성을 염두에 두어야 하겠다.

• Clinical and Experimental Pediatrics
• /
• v.49 no.4
• /
• pp.455-459
• /
• 2006

The major pathogenesis of acute respiratory distress syndrome (ARDS) is an inflammatory process that results from a diversity of injuries to the body. Due to the various cytokines and vasoactive peptides released from the endothelium, the vascular permeability is increased; the migration of inflammatory cells and the leakage of plasma proteins then occur and edema develops in the alveolus. There is a hypothesis that the impairment of alveolar recruitment in ARDS is caused by a defect of the surfactant system and the resultant increase of alveolar surface tension. This has been studied in pediatric patients in ARDS; after the administration of surfactant, hypoxia, respiratory symptoms and survival chances were improved. To alleviate the major pathogenic mechanism in this disease, that is to say, inflammation of the lung, steroids have been used and studied as another treatment modality for ARDS, and it has been concluded that the administration of low dose methylprednisolone may improve patients' symptoms and survival rates. We report here on a case of a young infant admitted with ARDS, who, after the intratracheal administration of 120 mg/kg surfactant, on $PaO_2/FiO_2$ was elevated. Subsequent low doses of methylprednisolone were given, and the symptoms did not recur, and no fibrotic change was shown during the follow-up period of 2 months.

• Tuberculosis and Respiratory Diseases
• /
• v.59 no.6
• /
• pp.690-695
• /
• 2005

Nitric acid is an oxidizing agent used in metal refining and cleaning, electroplating, and other industrial applications. Its accidental spillage generates oxides of nitrogen, including nitric oxide (NO) and nitrogen dioxide ($NO_2$), which cause chemical pneumonitis when inhaled. The clinical presentation of a nitric acid inhalation injury depends on the duration and intensity of exposure. In mild cases, there may be no symptoms during the first few hours after exposure, or the typical symptoms of pulmonary edema can appear within 3-24 hours. However, in cases of prolonged exposure, progressive pulmonary edema develops instantaneously and patients may not survive for more than 24 hours. We report a case of a 44-year-old male who was presented with acute respiratory distress syndrome after nitric acid inhalation. He complained of cough and dyspnea of a sudden onset after inhaling nitric acid fumes at his workplace over a four-hour period. He required endotracheal intubation and mechanical ventilation due to fulminant respiratory failure. He was managed successfully with mechanical ventilation using positive end expiratory pressure and systemic corticosteroids, and recovered fully without any deterioration in his pulmonary function.

• Tuberculosis and Respiratory Diseases
• /
• v.45 no.2
• /
• pp.457-464
• /
• 1998

This report describes a 5-month follow-up of the efficacy of Herbst oral appliance(OA) in a obstructive sleep apnea syndrome patient who has failed with uvulopalatopharyngoplasty(UPPP) and was unable to tolerate to subsequent nasal continuous positive airway pressure(CPAP) because of mouth air leak. The obstructive sleep apnea and daytime performance were markedly improved by regular home use of OA, and the patient still continues to use OA without complications. It is suggested that OA can be a successful alternative therapeutic modality in patients who are unable to tolerate to nasal CP AP, especially after UPPP.

• Tuberculosis and Respiratory Diseases
• /
• v.60 no.4
• /
• pp.397-403
• /
• 2006

Background: Chronic obstructive pulmonary disease(COPD) is categorized by the percentage of the predicted $FEV_1$(Forced expiratory volume in 1 second) result which is highly correlated with disease severity(morbidity and mortality). In COPD patients, dyspnea seems to be different from disease severity. We investigated whether dyspnea is correlated with disease severity, as measured by $FEV_1$, quality of life(QoL), occupation, and supporting level of family members and neighbors. Method: Thirty-six clinically stable patients with chronically irreversible airflow limitation were enrolled. We used the Medical Research Council(MRC) dyspnea scale to assess the level of dyspnea and the Korean St. Goerge's respiratory questionnaire(SGRQ) as measure the QoL. Result: The mean percentage of the predicted $FEV_1$ was 32.0%. Dyspnea was not correlated with GOLD stage using $FEV_1$(p=0.114). With deteriorating level of dyspnea the scores of symptoms(p=0.041), activity(p=0.004), impact(p=0.001), and total SGRQ score(p<0.001) were significantly increased. Dyspnea was not correlated with the level of occupation(p=0.259). The supporting level of family members and neighbors was significantly negatively correlated with dyspnea scale(p=0.011). Conclusion: In the management of COPD patients, we have to remember that the level of subjective dyspnea is correlated with QoL(symptom, activity and impact on society) and social supporting level as well as GOLD stage($FEV_1$).

• Progress in Medical Physics
• /
• v.24 no.2
• /
• pp.127-132
• /
• 2013

The position of the internal organs can change continually and periodically inside the body due to the respiration. To reduce the respiration induced uncertainty of dose localization, one can use a respiratory gated radiotherapy where a radiation beam is exposed during the specific time of period. The main disadvantage of this method is that it usually requests a long treatment time, the massive effort during the treatment and the limitation of the patient selection. In this sense, the combination of the real-time position management (RPM) system and the volumetric intensity modulated radiotherapy (RapidArc) is promising since it provides a short treatment time compared with the conventional respiratory gated treatments. In this study, we evaluated the accuracy of the respiratory gated RapidArc treatment. Total sic patient cases were used for this study and each case was planned by RapidArc technique using varian ECLIPSE v8.6 planning machine. For the Quality Assurance (QA), a MatriXX detector and I'mRT software were used. The results show that more than 97% of area gives the gamma value less than one with 3% dose and 3 mm distance to agreement condition, which indicates the measured dose is well matched with the treatment plan's dose distribution for the gated RapidArc treatment cases.