Leigh Disease, or subacute necrotizing encephalopathy (SNE), is a degenerative disorder characterized by lesions of the gray and white matter in the bran and spinal cord. The pathogenesis was known as mitochondrial enzyme defect of the respiratory chain system. We experienced 2 cases of Leigh disease. The first case, a seven-month old girl who was presented with weak respiration and failure to thrive, showed lactic acidemia and increased lactic acid in CSF fluid, high signal intensity in the bilateral putamen and head of caudate of nucleus at T2 weighted MR imaging. The second case, a 3-year-old girl with ataxic gait and bilateral ptosis also showed lactic acidemia, increased lactic acid in CSF fluid and high signal intensity in the bilateral basal ganglia. Respiratory difficuly developed in both cases and died within 1 month after visiting our hospital. The diagnosis was made by lactic acidosis and specific MRI finding. We report these cases with a brief review of its related literature.
Acidosis, Lactic ; Basal Ganglia ; Child, Preschool ; Diagnosis ; Electron Transport ; Failure to Thrive ; Female ; Gait ; Head ; Humans ; Lactic Acid ; Leigh Disease* ; Magnetic Resonance Imaging ; Putamen ; Respiration ; Spinal Cord

BACKGROUND AND OBJECTIVE: There have been several reports suggesting close relationships bet- ween problem behavior syndrome scale and/or social competence scale and pediatric allergic disorders such as bronchial asthma and atopic dermatitis. The aim of the present study was to confirm these relationships. We compared the behavioral symptoms and social competence scale between allergic and non-allergic patient groups using Korean-Child Behavior Checklist (K- CBCL). MATERIALS AND METHOD: One hundred and seventy-one subjects(aged 4-11 years) were enro- lled in this study. Allergy patient group(N=84) was divided into 4 subgroups, according to the allergic diseases such as atopic asthma(Group 1, BA), allergic rhinitis(Group 2, AR), atopic dermatitis(Group 3, AD), and chronic urticaria(Group 4, CU). Eighty-seven non-allergic patients (Group 5, NA) included those with pediatric general disorders, except psychomotor retardation, neurologic deficit, malignancy or other chronic disorders. K-CBCL which was filled in by parents or guardian, included eight dimensions of problem behavior: withdrawal, somatic problems, anxiety/ depression, social problems, thought problems, attention problems, delinquent behavior, and aggressive behavior. The first three scales were classified as an Internalizing broadband group and the last two scales as Externalizing group. In addition, the questionnaire included social scale, school scale, and total competence scale. RESULTS: There were no significant differences in age, sex, social economic status between 5 subgroups of allergic and non-allergic patients(p>0.05). The mean problem behavior scores tended to be higher in allergic patient group, but no statistical significance was noted(p>0.05). The mean school scale, social scale, total competence scale of allergic patient group were not significantly higher than those of non-allergic patient group(p>0.05). Combined disease group with more than 2 allergic disorders showed problem behaviors compared to non-allergic group or asthma only group. Total social competence scale was lower in allergic patient group with 1-3 years of disease duration than for shorter(less than 1 year) and longer(more than 3 years) durations. CONCLUSION: Taken together, combined disease group and allergic disease group with 1-3 years of duration showed more problem behaviors and the lowest total social competence scales, respectively.
Asthma ; Behavioral Symptoms ; Checklist* ; Child ; Child Behavior* ; Child* ; Depression ; Dermatitis, Atopic ; Humans ; Hypersensitivity ; Mental Competency ; Neurologic Manifestations ; Parents ; Social Problems ; Weights and Measures ; Surveys and Questionnaires

OBJECTIVE: This study is aimed to estimate the equality of two diagnostic modalities of computed tomography(CT) and magnetic resonance image in herniated intervertebral disc and other degenerative cervical diseases. METHODS: Retrospective study was carried out on 37 patients who have undergone surgery for degenerative cervical diseases from December 1996 to February 2001. Mean age was 49.7 years. Twenty eight patients were male(mean age : 48.4 years) and 9 patients female(mean age : 53.7 years). Magnetic resonance image and CT of the patients were evaluated blindly about 5 items as bony spur, existence of ossification of posterior longitudinal ligament(OPLL), intradiscal calcification, herniated intervertebral disc and spinal stenosis. RESULTS: While comparing radiologic results, bony spur was found in CT of 25 patients and magnetic resonance image of 20 patients, OPLL was seen in CT of six and magnetic resonance image of four and herniated intervertebral disc was seen in CT of 32 patients and MRI of 33 patients. These three diagnosis were considered to be concordant between CT and magnetic resonance image(p<0.01). Intradiscal calcification was seen in CT of 6 patients and magnetic resonance imge of one patient that the diagnosis was not considered statistically concordant(p=0.023). Although spinal stenosis was positive in CT of 16 patients and magnetic resonance image of 13 patients, diagnostic concordance could not be stated(p=0.345). CONCLUSION: Bony spur, OPLL and the herniated intervertebral disc may be diagnosed by any of the radiologic method because both magnetic resonance image and CT have shown statistically significant diagnostic concordance. Since intradiscal calcification and spinal stenosis have not shown diagnostic concordance, it is thought that either diagnostic method alone may not be sufficient confirmative diagnosis.
Diagnosis ; Humans ; Intervertebral Disc ; Magnetic Resonance Imaging ; Retrospective Studies ; Spinal Stenosis

PURPOSE: This study was designed to aid the diagnosis and predict the prognosis of Nutcracker syndrome in adults by understanding its clinical course. METHODS: We reviewed the records of 22 adult patients diagnosed with Nutcracker syndrome from August 2003 to August 2006 at Soonchunhyang university Bucheon hospital. RESULTS: The mean age was 24.0+/-12.0 years (15-59) including 13 males and 9 females. Twenty of 22 patients visited our hospital due to hematuria. Combined symptoms were flank pain (7 patients), back pain (2), abdominal pain (2), foamy urine (3), weight loss (1) and fatigue (1). Urine analysis showed hematuria (18 patients), isolated proteinuria (1), combined form (6), and normal findings (3). The mean level of proteinuria was 496.3+/-387.5 mg/d. Patients were diagnosed by doppler ultrasonography (17), abdominal computed tomography (8), and renal angiography (1). The mean ratio of the anterioposterior diameter of the left renal vein (LRV) between the hilar portion and the aortomesenteric (AM) portion was 8.25+/-3.05. The mean peak velocity ratio in the LRV between the AM and hilar portion was 6.09+/-1.47. Renal biopsy was done in the 3 patients with proteinuria >500 mg/day, and revealed membranous glomerulonephritis, IgA nephropathy and normal finding, respectively. Eight patients were followed up in 9.9+/-7.9 months. Only 1 patient showed improvement of hematuria. CONCLUSION: In the adults with Nutcracker syndrome, other symptoms beyond hematuria occurs. And when proteinuria is presented, the possibility of combined glomerulonephritis must be avoided.
Abdominal Pain ; Adult* ; Angiography ; Back Pain ; Biopsy ; Diagnosis ; Fatigue ; Female ; Flank Pain ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranous ; Gyeonggi-do ; Hematuria ; Humans ; Male ; Prognosis ; Proteinuria ; Renal Veins ; Ultrasonography, Doppler ; Weight Loss

OBJECTIVE: The purpose of this study was to evaluate the clinical usefulness of CD44v6 in patients with histologically confirmed endometrial cancer. METHODS: Immunohistochemistry was used to determine the expression of CD44v6 in 39 hysterectomy specimens with the diagnosis of endometrial cancer. RESULTS: CD44v6 expression was detected in 8 specimens with no myometrial invasion of endometrial cancer, one specimen with <50% myometrial invasion, and none with >50% myometrial invasion. There was a significant association between CD44v6 expression and no myometrial invasion of endometrial cancer. There was a statistically significant association between CD44v6 expression and lymph node metastasis, lymphovascular involvement. And all cases of CD44v6 expression were FIGO stage I with histological grade 1 or 2. CD44v6 expression was statistically related with estrogen receptor expression. Although statistical significance was not revealed, it was likely that CD44v6 expression was related with progesterone receptor expression with positive predictive value of 6/9 (66.7%). CONCLUSION: Significant relationships between myometrial invasion, lymph node metastasis, lymphovascular involvement, surgical stage and expression of estrogen receptor and CD44v6 expression suggest that CD44v6 expression could be a good prognostic factor. CD44v6 expression may have potential clinical usefulness if this expression is demonstrated in a further study with prehysterectomy sampling specimens containing endometrial cancer.
Diagnosis ; Endometrial Neoplasms* ; Estrogens ; Female ; Humans ; Hysterectomy ; Immunohistochemistry ; Lymph Nodes ; Neoplasm Metastasis ; Receptors, Progesterone

BACKGROUND: It is known that long-term survival rate in patients underwent bronchial sleeve lobectomy for primary lung cancer is at least equal to that in patients underwent pneumonectomy, and bronchial sleeve lobectomy is performed in patients with suitable tumor location even in patients have adequate pulmonary function. Sleeve pneumonectomy is performed when carina was invaded by tumor or tumor location was near to the carina. We performed this study to know our results of sleeve resection for primary lung cancer. MATERIAL AND METHOD: We analyzed retrospectively the medical records of 45 patients who underwent sleeve lobectomy or sleeve pneumonectomy for primary lung cancer by one thoracic surgeon from May 1990 to July 2003 in Department of Thoracic & Cardiovascular Surgery, College of Medicine, Kyung Hee University. Follow-up loss was absent and last follow-up was performed in April 5, 2005. Kaplan-Meyer method and log-lank test were used to know long-term survival rate and p-value. RESULT: Mean age was 60 years old and male to female ratio 41:1. Histologic types were squamous cell carcinoma were 39, adenocarcinoma were 4, and others were 2 patients. Pathologic stages were I 14, II 14, and III 17 patients. Nodal stages were N0 23, N1 13, and N2 9 patients. Types of operation were sleeve lobectomy 40 and sleeve pneumonectomy 5 patients. Operative mortality was 3 patients and its cause was respiratory complications. Early complications were pneumonia 4, atelectasis 8, air leakage more than 7 days 6, and atrial fibrillation 4 patients. In 19 patients tumor was recurred. Local recurrence was 10 and systemic metastasis was 9 patients. Overall 5, 10-year survival rate were 54.2%, 42.5%. The 5, 10-year survival rates according to the pathologic stage were 83.9%, 67.1% in stage I, 55%, 47.1% in II, 33.3%, 25% in III, and significance difference was present between stage I and III. The 5, 10-year survival rate according to the lymph node involvement were 63.9%, 54.6% in N0, 53.8%, 46.5% in N1, 28.5%, 14.2% in N2, and significance difference was present between N0 and N2. CONCLUSION: Because bronchial sleeve lobectomy for primary lung cancer could be performed safely and shows acceptable long-term survival rate, it could be considered primary in case of suitable tumor location if complete resection is possible. Although sleeve pneumonectomy for primary lung cancer shows somewhat high operative mortality rate, it could be considered in view of curative treatment.
Adenocarcinoma ; Atrial Fibrillation ; Carcinoma, Squamous Cell ; Female ; Follow-Up Studies ; Humans ; Lung Neoplasms* ; Lung* ; Lymph Nodes ; Male ; Medical Records ; Middle Aged ; Mortality ; Neoplasm Metastasis ; Pneumonectomy ; Pneumonia ; Pulmonary Atelectasis ; Recurrence ; Retrospective Studies ; Survival Rate

The treatment of choice for post-intubation tracheal stenosis is partial tracheal resection and end-to-end anastomosis. The surgical treatment of tracheal restenosis that results from unsuccessful repair of post-intubation tracheal stenosis is not easy. Failed reoperation results in permanent tracheostomy and loss of voice. If the first operation fails, about 4~6 months of period for resolution of inflammatory reaction, edema, and fibrosis is needed. The exact evaluation of the patient's status is necessary and success rate of reoperation for the appropriate candidates is over 90%. We report the results of treatment in two cases of tracheal restenosis that resulted from unsuccessful repair of post-intubation tracheal stenosis with review of literatures.
Edema ; Fibrosis ; Reoperation ; Tracheal Stenosis* ; Tracheostomy ; Voice

BACKGROUND AND OBJECTIVES: The proportion of mitral regurgitation caused by chordae rupture has recently been seen to be increasing, as has the role of mitral valve repair in the treatment of chordae rupture. This study evaluated the clinical characteristics and trends of surgical treatment of chordae rupture. Additionally, we attempted to discern the usefulness of transthoracic echocardiography (TTE) in the preoperative diagnosis of chordae rupture. SUBJECTS AND METHODS: Forty patients (20 men, mean age:49+/-14) presenting with chordae rupture confirmed during surgery between January 1994 and April 2001 were included in this study. Clinical, TTE and surgical data were analyzed retrospectively. RESULTS: The cause of chordae rupture was idiopathic degeneration in 28 cases, rheumatic heart disease in 5, infective endocarditis in 6, and trauma in 1 case. The sites of rupture were the anterior leaflet (14), posterior leaflet (23), and anterior and posterior leaflets (3). Mitral valve repair was performed in 20 cases and mitral valve replacement was performed in 20 cases. Most mitral valve repairs were performed beginning in 1998 (17/20). Chordae rupture diagnosed by TTE numbered 14 cases (35%). CONCLUSION: Regurgitation caused by chordae rupture was primarily associated with idiopathic degenerative change. Posterior leaflet rupture was more frequent than anterior leaflet rupture. The use of mitral valve repair has been increasing since 1998.
Chordae Tendineae ; Diagnosis ; Echocardiography ; Endocarditis ; Humans ; Male ; Mitral Valve ; Mitral Valve Insufficiency ; Retrospective Studies ; Rheumatic Heart Disease ; Rupture*

PURPOSE: Diesel exhaust particles (DEPs) can induce and trigger airway hyperresponsiveness (AHR) and inflammation. The aim of this study was to investigate the effect of long-term DEP exposure on AHR, inflammation, lung fibrosis, and goblet cell hyperplasia in a mouse model. METHODS: BALB/c mice were exposed to DEPs 1 hour a day for 5 days a week for 3 months in a closed-system chamber attached to a ultrasonic nebulizer (low dose: 100 microg/m3 DEPs, high dose: 3 mg/m3 DEPs). The control group was exposed to saline. Enhanced pause was measured as an indicator of AHR. Animals were subjected to whole-body plethysmography and then sacrificed to determine the performance of bronchoalveolar lavage and histology. RESULTS: AHR was higher in the DEP group than in the control group, and higher in the high-dose DEP than in the low-dose DEP groups at 4, 8, and 12 weeks. The numbers of neutrophils and lymphocytes were higher in the high-dose DEP group than in the low-dose DEP group and control group at 4, 8, and 12 weeks. The levels of interleukin (IL)-5, IL-13, and interferon-gamma were higher in the low-dose DEP group than in the control group at 12 weeks. The level of IL-10 was higher in the high-dose DEP group than in the control group at 12 weeks. The level of vascular endothelial growth factor was higher in the low-dose and high-dose DEP groups than in the control group at 12 weeks. The level of IL-6 was higher in the low-dose DEP group than in the control group at 12 weeks. The level of transforming growth factor-beta was higher in the high-dose DEP group than in the control group at 4, 8, and 12 weeks. The collagen content and lung fibrosis in lung tissue was higher in the high-dose DEP group at 8 and 12 weeks. CONCLUSIONS: These results suggest that long-term DEP exposure may increase AHR, inflammation, lung fibrosis, and goblet cell hyperplasia in a mouse model.
Airway Remodeling ; Animals ; Bronchoalveolar Lavage ; Collagen ; Fibrosis ; Goblet Cells ; Hyperplasia ; Inflammation* ; Interferon-gamma ; Interleukin-10 ; Interleukin-13 ; Interleukin-6 ; Interleukins ; Lung ; Lymphocytes ; Mice* ; Nebulizers and Vaporizers ; Neutrophils ; Plethysmography ; Pneumonia ; Ultrasonics ; Vascular Endothelial Growth Factor A ; Vehicle Emissions*

PURPOSE: Allergic diseases have been increased in Korea. The aim of this study was to investigate the changes of sensitization rate to inhalant allergens over 10 years in children with allergic diseases from a tertiary referral hospital. METHODS: Skin prick tests were done in total 2,225 children in 2000, 2005, and 2010. Changes of skin reactivity to 12 major inhalant allergens, including Tree pollen mixture I (Alder, Elm, Hazel, Poplar, Willow), Tree pollen mixture II (Beech, Birch, Oak, Plane tree), Dermatophagoides pteronyssinus, Dermatophagoides farina, Alternaria, cat dander, and dog dander were investigated. RESULTS: The sensitization rates to Tree pollen mixture I and II were increased as 5.0%, 6.8% in 2000, 8.5%, 9.8% in 2005, and 14.8%, 17.3% in 2010, respectively (trend P<0.001, P<0.001, respectively). And the sensitization rates to D. pteronyssinus cat dander and dog dander were changed as 55%, 4.8%, 4.8% in 2000, 62.2%, 11.9%, 28% in 2005, and 63.1%, 12.7%, 16.7% in 2010, respectively. The distribution of sensitized pollens was oak (12.8%), beech (12.4%), pine (10.3%), birch (10%), hazel (9.6%), alder (8%), elder (6.9%), elm (5.4%) in the study of 2010. When allergen/histamine ratio was compared, rates of strong sensitization over 4+ to tree pollen were increased. CONCLUSION: The sensitization rates to inhalant allergens were increased in children with asthma or allergic rhinitis from a tertiary referral hospital in Seoul over last 10 years. Especially, an increase in the degree of skin reactivity was observed in cat dander, dog dander, and tree pollens. The studies to investigate the etiology of this increase and prevention will be needed.
Allergens ; Alnus ; Alternaria ; Animals ; Asthma* ; Betula ; Cats ; Child* ; Dander ; Dermatophagoides pteronyssinus ; Dogs ; Fagus ; Humans ; Hypersensitivity ; Korea ; Pollen ; Pyroglyphidae ; Rhinitis* ; Seoul ; Skin ; Tertiary Care Centers*