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

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*

Tuberculosis mainly develops in the lung, but may also rarely invade other parts of the abdominal region. Abdominal tuberculosis is associated with pulmonary tuberculosis in approximately 15% of cases, and abdominal tuberculosis primarily develops in the terminal ileum and lymphatic gland. Moreover, hepatic tuberculosis is uncommon and is usually accompanied with active pulmonary or miliary tuberculosis. Hence, the development of primary hepatic tuberculoma as a single liver mass is very unusual. In the present report, we describe the case of a 63-year-old man with a solitary hepatic tuberculoma; the 6.4 cm mass was incidentally detected during abdominal computed tomography in the asymptomatic patient, and the diagnosis was confirmed by liver biopsy through ultrasonography-guided fine-needle aspiration.
Biopsy ; Biopsy, Fine-Needle ; Diagnosis ; Humans ; Ileum ; Liver ; Lung ; Middle Aged ; Tuberculoma* ; Tuberculosis ; Tuberculosis, Hepatic ; Tuberculosis, Miliary ; Tuberculosis, Pulmonary

With increasing life expectance and improvement of the overall health of the elderly, the understanding of geriatric disease becomes an important aspect of medical services. In the elderly, the frequencies of hypertension, DM, & anemia are increased according to aging and renal function is decreased progressively. As screening test for cervical cancer Papanicolaou smear is recommended due to its cost-effective benefits in females. In age-adjusted elderly male and female study populations, authors investigated the alterations of the above diseases, hypercholesterolemia, liver disease, obesity, & proteinuria distributions. METHODS: This study included 1,181 aged 40 years (600 males, 581 females) visitors between January 1, 1997 and December 31, 1997 in National Medical Center, Seoul, South Korea. Authors divided these populations into two large groups such as group A and B. Group A was composed of 40-59 years, group B 60 years and older. Group A and B were subdivided into 4 subgroups each other, such as A-1, A-2, A-3 and A-4, B-1, B-2, B-3 and B-4 by means of age-adjusted dividing scale. We used our inclusion criteria to define each disease. RESULTS: Hypertension was the most common disease in males of group B, but obesity was in females. The age-adjusted frequencies of hypertension, anemia, obesity & proteinuria were increased according to aging in the elderly aged 60 years and older. Though hypercholesterolemia revealed non-specific distribution in each group, increased frequency was observed in females compared to males in group B. With increasing their age, abnormal findings of Papanicolaou smear were found in females of group B. CONCLUSIONS: As the frequencies of hypertension, anemia, obestiy, proteinuria & abnormal findings of Papanicolaou smear were increased in the elderly aged 60 years and older as increasing their age. Much more attentions and follow up plans for these disease should be needed in the elderly.
Aged* ; Aging ; Anemia ; Attention ; Female ; Humans ; Hypercholesterolemia ; Hypertension ; Korea ; Liver Diseases ; Male ; Mass Screening ; Obesity ; Papanicolaou Test ; Proteinuria ; Seoul ; Uterine Cervical Neoplasms