Utilization Patterns of Coagulation Factor Consumption for Patients with Hemophilia

Hemophilia is a serious rare disease that requires continuous management and treatment for which the medicine is costly at the annual average of 100 million KRW for an individual. The aim of this study was to investigate trends in the utilization of coagulation factor (CF) used for hemophilia treatment using the National Health Insurance database from 2010 to 2013 in Korea and compare the utilization of CF with other countries. The consumption of CF per capita (IU) in Korea was not more than other countries with similar income to Korea. However, CF usage per patient IU was higher because the prevalence rate of hemophilia in Korea was lower than in other countries while the number of serious patients was much more. Therefore, it is difficult to say that the consumption of hemophilia medicine in Korea is higher than that in other countries. The consumption and cost of hemophilia medicine in Korea is likely to increase due to the increased utilization of expensive bypassing agents and the widespread use of prophylaxis for severe hemophilia. Even during the research period, it increased slightly and other countries show a similar trend. Thus, hemophilia patient management should accompany active monitoring on the health and cost outcomes of pharmaceutical treatment in the future. This study is expected to contribute to further insight into drug policies for other countries that face similar challenges with high price pharmaceuticals.

Putative association of RUNX1 polymorphisms with IgE levels in a Korean population

RUNX1, a member of the runt domain gene family of transcription factors, encodes a heterodimeric transcription factor and regulates the expression of various genes related to hematopoiesis and myeloid differentiation. RUNX1 has been one of the target genes for research into various autoimmune diseases due to its properties as a transcription factor and functional distribution for chromosomal translocation. In an effort to identify additional gene polymorphisms in which variants have been implicated in asthma, we investigated the genetic polymorphisms in RUNX1 to evaluate it as a potential candidate gene for a host genetic study of asthma and IgE production. We identified 19 sequence variants by direct DNA sequencing in 24 individuals of which four common variants were selected for genotyping in our asthma cohort (1,055 asthmatic patients, 384 normal controls). Using logistic regression analysis for association with the risk of asthma, while controlling for age, gender, and smoking status as covariates, no significant associations with the risk of asthma were detected. However, two polymorphisms in the promoter region (-2084G>C and -1282G>A) showed a marginal association with total IgE levels (0.03 and 0.03 in recessive models, respectively). Our findings suggest that polymorphisms in RUNX1 might be one of the genetic factors for the regulation of IgE production.

Ventilatory Dynamics according to Bronchial Stenosis in Bronchial Anthracofibrosis / 결핵및호흡기질환

BACKGROUND: Bronchial anthracofibrosis usually manifest as a form of obstructive airway disease, and can be accompanied by parenchymal diseases such as pneumonia, and pulmonary tuberculosis. This study investigated the ventilatory dynamics according to the severity of bronchial stenosis in patients with bronchial anthracofibrosis. Method : One hundred and thirteen patients with bronchial anthracofibrosis that was confirmed by bronchoscopy and who had undergone a pulmonary function test were enrolled in this study group. The correlation coefficients between the pulmonary functional parameters and the number of lobes with bronchial stenosis were investigated. RESULTS: The incidence of ventilatory dysfunction was 56(49.6%) for obstructive, 8(7.1%) for restrictive, 2(1.8%) for mixed, and 47(41.6%) for a normal pattern. The FEV1/FVC, FEF25~75%, FEF25%, FEF50%, FEF75%, and PEF showed a significant negative correlation (p<0.05) and the Raw had a significant positive correlation with the number of lobes with bronchial stenosis(p<0.001). CONCLUSION: These findings suggest that the most common abnormality of the ventilatory function in bronchial anthracofibrosis is an obstructive pattern with a small airway dysfunction according to the severity of bronchial stenosis.

The Clinical Evaluation of Anomalous Bronchi Arising From The Trachea and Main Bronchi / 결핵및호흡기질환

OBJECTIVES: To evaluate the clinical significance of abnormal bronchi originating from the trachea or main bronchi. METHODS: 21 patients (male:female ratio, 13:8; mean age, 58.2 years, range 34-77), who were diagnosed with major tracheobronchial anomalies by bronchoscopy from January 2001 to March 2005, were enrolled in this study. The anomalous bronchi consisted of 13 tracheal bronchi and 8 cardiac accessory bronchus. The clinical features, bronchoscopic findings, and outcomes were analyzed retrospectively. RESULTS: Common symptoms, including hemoptysis, cough and dyspnea, resulted from the underlying lung disease regardless of the bronchial anomalies. In this series of 13 tracheal bronchi, 7 cases originated from the trachea within 1cm of the carina (carinal type) and 6 cases originated at a higher level(tracheal type). Most patients had favorable outcome with conservative treatment for the underlying lung disease. CONCLUSION: Most tracheobronchial anomalies are found incidentally in the process of diagnosing lung disease. The clinical outcome of patients with a bronchial anomaly depends on the underlying lung disease.

A Case of Staphylococcal Scalded Skin Syndrome

No abstract available.

A Case of Diabetes Insipidus associated with Suprasellar Germinoma

We report I case of diabetes insipidus associated with suprasellar germinoma, obstructive hydrocephalus and both optic nerve atrophy. In this patient, symptoms of increased intracranial pressure was preceded by characteristic polyuria and polydipsia. The water restriction and pitressin test resulted in central originated diabetes insipidus. Computerized tomography in the brain revealed smooth marginated rounded mass density measuring 3.9 cm obliterating 3rd ventricle and extending to suprasellar region. On neurosurgical operation, egg sized tumor mass was noted in suprasellar area with complete obstruction of 3rd ventricle. The cytological and histopathological findings revealed malignant atypical teratoma(Ectopic pinealoma) which is extremely rare in pediatric age. The patient was received partial excision of tumor mass and ventriculo-peritoneal shunt. After operation, the patient was exhibited progressive renal failure and discharged against doctors order without improvement. We also briefly reviewed with related literatures.

A Clinical Study on Intussusception in Infancy and Children

A retrospective clinical study on 63 cases of intussusception in infants and children, who were admitted to the department of pediatrics and surgery from May, 1974 to Feb. 1978, was done. The prognosis of this disease is dependent upon early diagnosis and treatment but the causatio of this disease is not clear yet. It is a relatively common disease and we think it is worthwhile to analyze our won cases. The results were : 1. Among 63 cases, 37 were admitted to the department of pediatrics and 36 were admitted to the department of GS. 2. In age distribution, 85% of the patients were under 1 year of age and the peak incidence was 3~9 months of age. 3. Male was predominant than female. Male to female ratio was 2.8:1 4. The 58.8% of the patients were above 50 percentile of body weight comparing with Korean standard data. 5. In the aspect of the etiology, 96.9% of the cases were idiopathic and only 3.1% of the cases had organic causes. 6. The 4 cardinal symptoms were abdominal pain(93.7%), vomiting(90.%), bloodymucous stool(80.1%) and abdominal mass(74.6%) 7. Among the cases, colocolic type was 33.3%, ileocolic type was 22.2% and ileocecal type was 20.6% 8. The 71.4% of the cases were reduced successfully by barium enema. 9. Recurrence rate was 4.8%.