Isolated right ventricular hypoplasia, unassociated with severe pulmonary or tricuspid valvar malformations, is a rare primary congenital cardiac anomaly in which of the trabecular portion of right ventricle fails to develop. An atrial septal defect or a patent foramen ovale serves as an escape valve. We observed a 2-day-old neonate with this disorder who suffered from cyanosis. The diagnosis was made by Doppler-echocardiography which revealed marked reduction in right ventricular size and right-to-left shunt through the atrial septal defect. There was no other cardiac malformation such as pulmonary atresia or tricuspid atresia.
Cyanosis ; Diagnosis ; Foramen Ovale, Patent ; Heart Defects, Congenital ; Heart Septal Defects, Atrial ; Heart Ventricles ; Humans ; Infant, Newborn ; Pulmonary Atresia* ; Tricuspid Atresia* ; United Nations

Lymphangioleiomyomatosis, a rare disease in women of childbearing age, is the result of benign nodular hypertrophy of the smooth muscle of the lypmhatics and other tissues of the abdomen and thorax. We report a 36-years-old woman with pulmonary and retroperitoneal lymphangioleiomyomatosis who responded with hormone treatment. She developed vaginal pruritis and a pelvic ultraound was done given her significant past medical history. Ultrasound examination demonstrated a large mass in the right side of her pelvis. Therefore she was admitted to St. Michael's Hospital in Toronto for laparoscopy. Result of cytology was to be consistent with the diagnosis of retroperitoneal lymphangioleiomyomatosis. High resolution CT sacn of the thorax demonstrated multiple small cystic lesions, without associated nodularity compatible with a diagnosis of pulmonary lymphangioleiomyomatosis. She has been taking Provera tablets l00mg po tid since Dec. 15, 1993. We have given her a prescription for Depo provera 500mg IM monthly since she came back to Korea, and made arrangements for regular follow up monthly. We performed chest X-ray, CT of chest(high resolution), abdomen and pelvis, pulmonary function tests and arterial blood gas analysis. Chest X-ray and CT findings showed no significant change since July. 20, 1993.
Abdomen ; Blood Gas Analysis ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Hypertrophy ; Korea ; Laparoscopy ; Lymphangioleiomyomatosis* ; Medroxyprogesterone Acetate ; Muscle, Smooth ; Pelvis ; Prescriptions ; Pruritus ; Rare Diseases ; Respiratory Function Tests ; Tablets ; Thorax ; Ultrasonography

Isolated deficiency of 3-methylcrotonyl CoA carboxylase is a rare disorder of the catabolic pathway for leucine and many patients have mild symptoms or no symptom. However, the introduction of tandem mass spectrometry in newborn screening has revealed an unexpectedly high incidence of this disorder. We report an asymptomatic premature infant with isolated 3-methylcrotonyl CoA carboxylase deficiency detected by newborn screening program using tandem mass spectrometry. She was born at preterm, 36 weeks of gestation and her birth weight was 1,912 gm. She was delivered by Cesarian section due to maternal preeclampsia and oligohydramnios. An elevation of 3-hydroxyisovalerylcarnitine in a blood sample obtained at Seven days was detected by tandem mass screening. Massively elevated excretion of 3-hydroxyisovalerate and 3-methylcrotonylglycine was detected in the urine collected at 15 days. L-carnitine(100 mg/kg/day) was administrated orally to correct sencondary carnitine deficiency. Carnitine is conjugated with metabolites, to decrease the potential toxic effects. She is asymptomatic to date, and her growth and development are within normal limits.
Birth Weight ; Carnitine ; Female ; Growth and Development ; Humans ; Incidence ; Infant, Newborn* ; Infant, Premature ; Leucine ; Mass Screening* ; Oligohydramnios ; Pre-Eclampsia ; Pregnancy ; Tandem Mass Spectrometry*

Objective: To evaluate the impact of surgical simulation training using a three-dimensional (3D)-printed model of tetralogy of Fallot (TOF) on surgical skill development. Materials and Methods: A life-size congenital heart disease model was printed using a Stratasys Object500 Connex2 printer from preoperative electrocardiography-gated CT scans of a 6-month-old patient with TOF with complex pulmonary stenosis.Eleven cardiothoracic surgeons independently evaluated the suitability of four 3D-printed models using composite Tango 27, 40, 50, and 60 in terms of palpation, resistance, extensibility, gap, cut-through ability, and reusability of. Among these, Tango 27 was selected as the final model. Six attendees (two junior cardiothoracic surgery residents, two senior residents, and two clinical fellows) independently performed simulation surgeries three times each. Surgical proficiency was evaluated by an experienced cardiothoracic surgeon on a 1–10 scale for each of the 10 surgical procedures. The times required for each surgical procedure were also measured. Results: In the simulation surgeries, six surgeons required a median of 34.4 (range 32.5–43.5) and 21.4 (17.9–192.7) minutes to apply the ventricular septal defect (VSD) and right ventricular outflow tract (RVOT) patches, respectively, on their first simulation surgery. These times had significantly reduced to 17.3 (16.2–29.5) and 13.6 (10.3–30.0) minutes, respectively, in the third simulation surgery (p = 0.03 and p = 0.01, respectively). The decreases in the median patch appliance time among the six surgeons were 16.2 (range 13.6–17.7) and 8.0 (1.8–170.3) minutes for the VSD and RVOT patches, respectively. Summing the scores for the 10 procedures showed that the attendees scored an average of 28.58 ± 7.89 points on the first simulation surgery and improved their average score to 67.33 ± 15.10 on the third simulation surgery (p = 0.008). Conclusion Inexperienced cardiothoracic surgeons improved their performance in terms of surgical proficiency and operation time during the experience of three simulation surgeries using a 3D-printed TOF model using Tango 27 composite.

Objective: To evaluate the impact of surgical simulation training using a three-dimensional (3D)-printed model of tetralogy of Fallot (TOF) on surgical skill development. Materials and Methods: A life-size congenital heart disease model was printed using a Stratasys Object500 Connex2 printer from preoperative electrocardiography-gated CT scans of a 6-month-old patient with TOF with complex pulmonary stenosis.Eleven cardiothoracic surgeons independently evaluated the suitability of four 3D-printed models using composite Tango 27, 40, 50, and 60 in terms of palpation, resistance, extensibility, gap, cut-through ability, and reusability of. Among these, Tango 27 was selected as the final model. Six attendees (two junior cardiothoracic surgery residents, two senior residents, and two clinical fellows) independently performed simulation surgeries three times each. Surgical proficiency was evaluated by an experienced cardiothoracic surgeon on a 1–10 scale for each of the 10 surgical procedures. The times required for each surgical procedure were also measured. Results: In the simulation surgeries, six surgeons required a median of 34.4 (range 32.5–43.5) and 21.4 (17.9–192.7) minutes to apply the ventricular septal defect (VSD) and right ventricular outflow tract (RVOT) patches, respectively, on their first simulation surgery. These times had significantly reduced to 17.3 (16.2–29.5) and 13.6 (10.3–30.0) minutes, respectively, in the third simulation surgery (p = 0.03 and p = 0.01, respectively). The decreases in the median patch appliance time among the six surgeons were 16.2 (range 13.6–17.7) and 8.0 (1.8–170.3) minutes for the VSD and RVOT patches, respectively. Summing the scores for the 10 procedures showed that the attendees scored an average of 28.58 ± 7.89 points on the first simulation surgery and improved their average score to 67.33 ± 15.10 on the third simulation surgery (p = 0.008). Conclusion Inexperienced cardiothoracic surgeons improved their performance in terms of surgical proficiency and operation time during the experience of three simulation surgeries using a 3D-printed TOF model using Tango 27 composite.

BACKGROUND: In mitral stenosis with atial fibrillation, it is known that there is a significant linear correlation between end-diastolic mitral pressure gradient(EDMG) in cardiac catheterization and the corresponding RR interval. And using this, the equations to calculate the mitral valve area(MVA) in several heart retes has been inducing. However, as cardiac catheterization has a limitation not to be practiced repeatedly in all patients due to invasive procedure. In this study, using transthoracic doppler echocardiography, we tried to investigate the correlation of EDMG, RR interval and MVA and we tried to get the nomogram to predict MVA in different heart rates using it. METHODS: We made 9 patients an abject of this study whose measured MVA is from 0.5cm2 to 1.55cm2 by pressure half time(PHT) method among patients who have no any valvular heart disease except mitral stenosis with atrial fibrillation and whose condition is stable. We investigated the linear correlation between EDMG and RR interval, RR interval=a×EDMG+b(a ; slope, b ; intercept), using doppler echocardiography. We got the equation and nomogram to make an estimate of MVA with multiple regression analysis using the relation of measured MVA, slope and intercept. RESULTS: There was a linear correlation between EDMG and RR interval in all the patients. There was a mutual correlation of slope=53.0×(measured MVA)−101.2 between measured MVA and slope. There was a negative correlation of intercept=1,497−470×(measured MVA) between measured MVA and intercept. Using these, we could get multiple regression analysis equation, estimated MVA=0.0113×slope−0.0007×intercept+2.2497 and nomogram to estimate MVA. CONCLUSION: We could get the nomogram to estimate MVA easily using doppler echocardiography in mitral stenosis with atrial fibrillation.
Atrial Fibrillation ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography, Doppler ; Heart ; Heart Rate ; Heart Valve Diseases ; Humans ; Methods ; Mitral Valve Stenosis ; Mitral Valve ; Nomograms

BACKGROUND: Bronchial reactivity is known to be a component of airway hyperresponsiveness, a cardinal feature of asthma, with bronchial sensitivity, and is increments in response to induced doses of bronchoconstric tors as manifested by the steepest slope of the dose-response curve. However, there is some controversy regarding methods of measuring bronchial reactivity and clinical impact of such measurements. The purpose of this study was to evaluate the clinical significance and assess the clinical use by analyzing the relationship of the bronchial sensitivity, the clinical severity and the changes in pulmonary function with bronchial reactivity. METHOD: A total of 116 subjects underwent a methacholine bronchial provocation test. They were divided into 3 groups : mild intermittent, mild persistent, moderate and cough asthma. Severe patients were excluded. Methacholine PC20 was determined from the log dose-response curve and PC40 was determined by one more dose inhalation after PC20. The steepest slope of log dose-response curve, connecting PC20 with PC40, was used to calculate the bronchial reactivity. Body plethysmography and a single breath for the DLCO were done in 43 subjects before and after methacholine test. RESULTS: The average bronchial reactivity was 38.0 in the mild intermittent group, 49.8 in the mild persistent group, 61.0 in the moderate group, and 41.1 in the cough asthma group. There was a weak negative correlation between PC20 and bronchial reactivity. A heightened bronchial reactivity tends to produce an increased clinical severity in patients with a similar bronchial sensitivity and basal spirometric pulmonary function. There were significant correlations between the bronchial reactivity and the initial pulmonary function before the methacholine test in the order of sGaw, Raw, FEV1/FVC, MMFR. There were no correlations between the bronchial sensitivity and the % change in the pulmonary function parameters after the methacholine test. However, there were significant correlations between the bronchial reactivity and the PEF, FEV1, DLCO. CONCLUSION: There was weak significant negative correlation between the bronchial reactivity and the bronchial sensitivity, and the bronchial reactivity closely reflected the severity of the asthma. Accordingly, measuring both the bronchial sensitivity and the bronchial reactivity can be of assistance in assessing of the ongoing disease severity and in monitoring the effect of therapy.
Asthma ; Bronchial Provocation Tests ; Cough ; Humans ; Inhalation ; Maximal Midexpiratory Flow Rate ; Methacholine Chloride* ; Plethysmography

BACKGROUND: Low back pain (LBP) is known to be the significant reason for absenteeism and can develop long-term disability. So the airline authorities have made an effort to reduce the incidence of LBP by applying their physical standards. As a LBP Prevention Program, Airline A has provided health education, counseling and physical examinations. The purpose of this study is to analyze the relationship between LBP and physical characteristics, age and work duration, and this result will provide basic information for improving the LBP Prevention Program. METHOD: The subjects were the 585 flight attendants who had undergone regular physical check-ups with Airline A Medical Center from October 2000 to September 2001. We reviewed their absence records and physical characteristics, age and work duration. These data were analyzed statistically by the t-test and Lositic regression. RESULT: The LBP history group accounted for 18% of the study group. The male LBP history group had the more thin waist than the no history group. There were no differences (P<0.05) between LBP and other male and female physical characteristics. The age and work duration of male LBP history group was lower than the no history group (P<0.05). But the female comparison revealed that only the work experience in the LBP history group was lower than the No history group (P<0.05). Waist, age, work duration was analyzed statistically by logistic regression. Only female's work experience was significant. The fewer work experience was the more LBP. CONCLUSION: The results of this study suggest LBP is associated with work experience rather than physical characteristics. This is the first step toward improving a LBP Prevention Program for the cabin crew. It will be useful to conduct another review to assess various other reasons, such as psychosocial job factors, duration of work disability and muscle strength, related to LBP.
Absenteeism ; Counseling ; Female ; Health Education ; Humans ; Incidence ; Logistic Models ; Low Back Pain* ; Male ; Muscle Strength ; Physical Examination

Tuberculous aneurysm of the aorta is exceedingly rare. To date, the standard therapy for mycotic aneurysm of the abdominal aorta has been surgery involving in-situ graft placement or extra-anatomic bypass surgery followed by effective anti-tuberculous medication. Only recently has the use of a stent graft in the treat-ment of tuberculous aortic aneurysm been described in the literature. We report two cases in which a tuberculous aneurysm of the abdominal aorta was success-fully repaired using endovascular stent grafts. One case involved is a 42-year-old woman with a large suprarenal abdominal aortic aneurysm and a right psoas abscess, and the other, a 41-year-old man in whom an abdominal aortic aneurysm ruptured during surgical drainage of a psoas abscess.
Adult ; Aneurysm, Infected/drug therapy/radiography/*surgery ; Antitubercular Agents/therapeutic use ; Aortic Aneurysm, Abdominal/drug therapy/radiography/*surgery ; *Blood Vessel Prosthesis Implantation ; Case Report ; Female ; Human ; Male ; Psoas Abscess/surgery ; *Stents ; Tuberculosis, Cardiovascular/drug therapy/radiography/*surgery

Small cell carcinoma (SCC) primarily arises in the lung. Cases of primary extrapulmonary small cell carcinoma are uncommon, and may develop in various different organs. In particular, small cell carcinoma of the urinary bladder is very rare in female nonsmokers, and its prognosis is poor. The main part of treatment in SCC is a chemotherapy, which is a critical factor in its prognosis. In the present report, we describe the case of an 82-year-old female non-smoker who underwent transurethral resection of bladder tumor (TURBT) without chemotherapy for small-cell carcinoma of the bladder, and experienced a relapse at a same area to the primary tumor site.
Aged* ; Aged, 80 and over ; Carcinoma, Small Cell* ; Drug Therapy ; Female* ; Humans ; Lung ; Prognosis ; Recurrence ; Urinary Bladder Neoplasms ; Urinary Bladder*