• The Korean Journal of Nuclear Medicine
• /
• v.39 no.6
• /
• pp.473-480
• /
• 2005

Purpose: Adenosine myocardial perfusion SPECT has proven to be useful in the detection of coronary artery disease, in the follow up the success of various therapeutic regimens and in assessing the prognosis of coronary artery disease. The purpose of this study is to define the reproducibility of myocardial perfusion SPECT using adenosine stress testing between two consecutive Tc-99m sestaMIBI (MIBI) SPECT studies in the same subjects. Methods: Thirty patients suspected of coronary artery disease in stable condition underwent sequential Tc-99m MIBI SPECT studies using intravenous adenosine. Gamma camera, acquisition and processing protocols used for the two tests were identical and no invasive procedures were performed between two tests. Mean interval between two tests were 4.1 days (range: 2-11 days). The left ventricular wall was divided into na segments and the degree of myocardial tracer uptake was graded with four-point scoring system by visual analysis. Images were interpretated by two independent nuclear medicine physicians and consensus was taken for final decision, if segmental score was not agreeable. Results: Hemodynamic responses to adenosine were not different between two consecutive studies. There were no serious side effects to stop infusion of adenosine and side effects profile was not different. When myocardial uptake was divided into normal and abnormal uptake, 481 of 540 segments were concordant (agreement rate 89%, Kappa index 0.74). With four-grade storing system, exact agreement was 81.3% (439 of 540 segments, tau b=0.73). One and two-grade differences were observed in 97 segments (18%) and 4 segments (0.7%) respectively, but three-grade difference was not observed in any segment. Extent and severity scores were not different between two studios. The extent and severity scores of the perfusion defect revealed excellent positive correlation between two test (r value for percentage extent and severity score is 0.982 and 0.965, p<0.001) Conclusion: Hemodynamic responses and side effects profile were not different between two consecutive adenosine stress tests in the same subjects. Adenosine Tc-99m sestaMIBI SPECT is highly reproducible, and could be used to assess temporal changes in myocardial perfusion in individual patients.

• Childhood Kidney Diseases
• /
• v.3 no.2
• /
• pp.209-216
• /
• 1999

Purpose : Nephrogenic diabetes insipidus (NDI) is a rare X-linked disorder associated with renal tubule resistance to arginine vasopressin (AVP). The hypothesis that the defect underlying NDI might be a dysfunctional renal AVPR2 has recently been proven by the identification of mutations in the AVPR2 gene in NDT patients. To investigate the association of mutations in th AVPR2 gene with NDI, we analyzed the AVPR2 gene located on the X chromosome. Methods : We have analyzed the AVPR2 gene in a kindred with X-linked NDI. The proband and proband's mother were analyzed by polymerase chain reaction-single strand conformational polymorphism(PCR-SSCP) and DNA sequencing of the AVPR2 gene. We also have used restriction enzyme analysis of genomic PCR product to evaluate the AVPR2 gene. Results : C to T transition at codon 202, predictive of an exchange of tryptophan 202 by cysteine(R202C) in the third extracellular domain was identified. This mutation causes a loss of Hae III site within the gene. Conclusion : We found a R202C missense mutation in the AVPR2 gene causing X-linked NDI, and now direct mutational analysis is available for carrier screening and early diagnosis.

• Journal of Chest Surgery
• /
• v.35 no.12
• /
• pp.862-870
• /
• 2002

The surgical results of the patients with single ventricle(SV) associated with total anomalous pulmonary venous connection(TAPVC) has been reported with high mortality and morbidity due to their morphologic and hemodynamic complexity. A retrospective review was undertaken to report the outcome of the first-stage palliative surgery in our institution and to determine the factors influencing early death. Material and Method: Between January 1987 and June 2002, 39 patients with SV and TAPVC underwent surgical intervention with or without TAPVC repair. Age at operation ranged from 1day to 10.7months (median age, 2.4month), and 29 patients were male. Preoperative diagnosis included 20 right-dominant SV, 15 SV with endocardial cushion defect, 3 left-dominant SV, and 1 tricuspid atresia. The pulmonary venous connection was supracardiac in 22, cardiac in 5, infracardiac in 11, and mixed in 1, Obstructed TAPVC was present in 11. First-stage palliative surgery was performed in 37. Repair of TAPVC, either alone or in association with other procedures, was performed during the initial operation in 31. Univariate and multivariate analyses were performed to analyze the risk factors influencing the operative death. Result: A mean follow-up period of survivors was 34.3 $\pm$ 43.0(0.53 ~ 146.2)months. Overall early operative mortality was 43.6%(17/39). The causes were low cardiac output in 8, failure of weaning from cardiopulmonary bypass in 3, sepsis in 2, pulmonary hypertensive crisis in 1, pulmonary edema in 1, pneumonia in 1, and postoperative arrhythmia in 1. Risk factors influencing early death in univariate analysis were body weight, surgical intervention in neonate, obstructive TAPVC, preoperative conditions including metabolic acidosis, and need for inotropic support, TAPVC repair in initial operation, operative time, and cardiopulmonary bypass(CPB) time. In multivariable analysis, body weight, age at initial operation, surgical intervention in neonate, preoperative conditions including metabolic acidosis, need for inotropic support and CPB time were the risk factors. Conclusion: In this study, we demonstrated that the patients with SV and TAPVC had high perioperative mortality. Preoperative poor condition, young age, the length of operative and CPB time, the presence of obstructive TAPVC had been proven to be the risk factors. This fact suggests that the avoidance of unnecessarily additional procedures may improve the surgical outcomes of the first-stage palliative surgery. However further observation and collection of the data is mandatory to determine the ideal surgical strategy.

• Childhood Kidney Diseases
• /
• v.13 no.1
• /
• pp.40-48
• /
• 2009

Purpose : This study was performed to identify factors related to the resolution of primary vesicoureteral reflux (VUR) in infants. Methods : We reviewed 183 infants (M : F=149 : 34) diagnosed as urinary tract infection (UTI) between February 2002 and July 2007 at Sanggye Paik Hospital. The diagnosis of UTI was made by culture from a urine specimen obtained by suprapubic puncture (n=97), catheterization (n=83), or collection bag method (n=3, twice positive culture of same organism). All of the infants were performed renal ultrasonography, DMSA scan and voiding cystourethrography (VCUG) study. Follow-up imaging consisted of contrast VCUG or direct isotope VCUG at interval of 1 year. We evaluated the relationship of clinical and laboratory finding, radiologic finding in infants with VUR. Results : Among 51 VUR patients, 18 infants had grade I-II, 12 infants had grade III and the other 21 patients had grade IV-V. Abnormal findings including hydronephrosis on renal ultrasonography were not correlated with severity of VUR. However, the incidence of renal defect in the first DMSA scan showed a tendency of direct correlation with severity of VUR in female patients only (P<0.001). There was significant difference of resolution rate in three VUR groups (grade I-II, III, IV-V) in male patients only (P=0.025). Resolution rate was higher for male patients with unilateral VUR than bilateral (P<0.001). But unilaterality had not any affect on VUR resolution in female VUR patients (P=0.786). Resolution rate was higher for VUR patients without renal scar than VUR patients with renal scar (P<0.001). Conclusion : According to our findings, grade of VUR, laterality and renal scar are the factors that contribute to resolution of primary VUR in male and female infants differently.

• Journal of Chest Surgery
• /
• v.39 no.12 s.269
• /
• pp.900-905
• /
• 2006

Background: Homografts and bioprostheses are most commonly used for Rastelli operation in congenital heart disease, but the limited durability is responsible for multiple reoperations associated with increased morbidity This study evaluated long-term results after Rastelli operation with a mechanical valved conduit. Material and Method: A total of 20 patients underwent Rastelli operation with mechanical valved conduit from January 1990 to July 1992. Operative mortality was 1 of 20 patients, and a retrospective review of 19 patients(10 males, 9 females) was done. Initial diagnosis was congenitally corrected transposition of great arteries(cc-TGA, n=4), complete TGA (n=2), ventricular septal defect with pulmonary atresia(VSD with PA, n=9), truncus arteriosus(n=2), double outlet right ventricle with pulmonary stenosis(DORV with PS, n=2). The mean age at Rastelli operation was $4.6{\pm}3.4$ years, and mean follow-up period was $12.8{\pm}2.7$ years. Patients underwent Rastelli opearation using 16 CarboMedics mechanical valve, and 3 Bjork-Shiley mechanical valve($17{\pm}2$ mm). Result: There were 15 reoperations for failed mechanical valved conduit. The freedom from reoperation at 5 and 10 years was 53% and 37%. Most patients were received oral anticoagulation with warfarin, and maintained the international normalized ratio(INR) of 1.5 to 2.0. There was no anticoagulation or thromboembolism related complication. There was a significant difference in the causes of a conduit failure between early(within 3 years) and late(after 3 years) failure groups. The six patients reported early prosthetic valve failure, mainly due to valvular dysfunction by thrombosis or pannus formation. The other nine patients reported late prosthetic valve failure, mainly due to dacron conduit stenosis at anastomosis sites, whereas their valvar motion was normal except 1 patient. Conclusion: To avoid early prosthetic valve failure, strict anticoagulation therapy would be helpful. About the late development of obstructive intimal fibrocalcific peels within the Dacron conduit, an improvement of conduit material is necessary to reduce late prosthetic valve failure. In selected patients, the long term results were satisfactory.

• Clinical and Experimental Pediatrics
• /
• v.49 no.4
• /
• pp.410-416
• /
• 2006

Purpose : Ciliary abnormalities of the respiratory system usually accompany recurrent or persistent respiratory diseases such as paranasal sinusitis, bronchiectasis, rhinitis, and/or otitis media, since they cause certain derangements in ciliary cleaning activities. This disease is usually inherited by autosomal recessive trait, but may also be found to be acquired or transient in rare cases after heavy exposure to pollutants, cigarette smoking or severe infection. We performed this study in children with frequently recurrent or persistent respiratory diseases to clarify if the ciliary abnormalities are preceding factors. Methods : We enrolled 17 children with suspected respiratory ciliary abnormalities. The indications for evaluation of ciliary ultrastructure were recurrent or persistent respiratory infections. Children with immunologic abnormalities were excluded. From August 2000 to July 2003, we performed a biopsy on nasal mucosa and examined the structure of ciliary status by using an electron microscope. Results : Of the subjects, there were seven males and 10 females, aged 2 to 10 years. Out of the 17 subjects, 12 cases of chronic paranasal sinusitis, nine chronic coughs, nine frequent upper respiratory infections, seven cases of recurrent otitis media, four cases of recurrent pneumonia, and four cases of bronchial asthma were found. Out of the 17 cases on which histologic examinations were conducted, four cases showed pathologic findings, including one case of inner dynein arm defect, one of microtubular transposition, one of supernumerous tubules, and one singlet, respectively. Conclusion : It is essential for differential diagnosis and effective treatment to identify the abnormalities of ultrastructure of nasal cilia in children with symptoms of frequently recurrent or persistent respiratory diseases, if immunodeficiency or respiratory allergy could be excluded.

• Tuberculosis and Respiratory Diseases
• /
• v.54 no.3
• /
• pp.330-337
• /
• 2003

Background : Low spirometric forced vital capacity(FVC) in conjunction with a normal or high ratio of the forced expiratory volume at 1 second to the forced vital capacity($FEV_1$/FVC%) has traditionally been classified as a restrictive abnormality. However, the gold-standard diagnosis of a restrictive pulmonary impairment requires a measurement of the total lung capacity (TLC). This study was performed to determine the predictive value of spirometric measurements of the FVC for diagnosing a restrictive pulmonary abnormality. Methods : Test results from 1,371 adult patients who undertook both spirometry and lung volume measurements on the same visit from January 1999 to December 2000 were enrolled in this study. The test values for the FVC, the TLC that was below 80% of predicted value, and a $FEV_1$/FVC% that was below 70%, were classified as being abnormal. Results : Of the 1,371 patients, 353 patients had a reduced a FVC. Of these patients, 186 patients had a reduced TLC. Therefore, the positive predictive value was 52.7%. Of the 196 patients with a normal $FEV_1$/FVC% and a reduced FVC, 148(75.5%) patients had a lower TLC. Thirty eight (24.2%) patients out of 157 patients with a low $FEV_1$/FVC% and a low FVC showed a restrictive defect. Conclusion : Spirometry is useful to rule out a restrictive pulmonary abnormality, but a restrictive pattern on the spirometry dose not mean there is a true restrictive disease. For the patients with a low FVC, TLC measurements are essential for diagnosing a restrictive pulmonary impairment.

• Clinical and Experimental Pediatrics
• /
• v.45 no.9
• /
• pp.1126-1133
• /
• 2002

Purpose : Prader-Willi syndrome(PWS) is a complex disorder affecting multisystems with characteristic clinical features. Its genetic basis is an expression defect in the paternally derived chromosome 15q11-q13. We analyzed the clinical features and genetic basis of PWS patients for early detection and treatment. Methods : We retrospectively studied 24 patients with PWS in Department of Pediatrics, Samsung Medical Center, from September 1997 to September 2001. We performed cytogenetic and molecular genetic techniques using high resolution GTG banding techniques, fluorescent in situ hybridization and methylation-specific PCR for CpG island of SNRPN gene region. Results : The average birth weight of PWS patients was $2.67{\pm}0.47kg$ and median age at diagnosis was 1.3 years. The average height and weight of PWS patients under one year at diagnostic time were located in a 3-10 percentile relatively, and a rapid weight gain was seen between two and six years. Feeding problems in infancy and neonatal hypotonia were the two most consistently positive major criteria in over 95% of the patients. In 18 of the 24 cases(75%), deletion of chromosome 15q11-q13 was demonstrated and one case among 18 had an unbalanced 14;15 translocation. In four cases without any cytogenetic abnormality, it may be considered as maternal uniparental disomy and the rest showed another findings. Conclusion : We suggest diagnostic testing for PWS in all infants/neonates with unexplained feeding problems and hypotonia. It is necessary for clinically suspicious patients to undergo an early genetic test. As the genetic basis of PWS was heterogenous and complex, further study is required.

• Journal of Yeungnam Medical Science
• /
• v.16 no.2
• /
• pp.228-236
• /
• 1999

Background: Thyrotoxic periodic paralysis is an uncommon illness characterized by intermittent flaccid paralysis of skeletal muscle, usually accompanied by hypokalemia, in patient with hyperthyroidism. However, the pathophysiology of thyrotoxic periodic paralysis remains largely unexplained and controversial. This report describes the clinical and biochemical findings in 19 patients with thyrotoxic periodic paralysis who were examined at the Yeungnam University Medical Center(YUMC) during the past decade. Methods: The medical records of 997 YUMC patients, seen between 1986 and 1996, with diagnosis of hyperthyroidism were reviewed. Nineteen patients out of 997 hyperthyroidism patients were diagnosed, and examined by history, physical examination, serum electrolyte value, and thyroid function test during paralysis. On the basis of these results, comparisons were made on age, sex, precipitating factors, timing, affected limbs, prognosis, serum potassium and serum phosphate and thyroid hormone levels. Results: The prevalence of periodic paralysis in hyperthyroidism was 1.9 percent and the male to female prevalence ratio was 30:1 and in all patients, the development of perodic paralysis was correlated with hyperfunctional state of the thyroid gland. Eleven cases of periodic paralysis were associated with hypokalemia and their thyroid hormone levels were significantly more increased than those of the patients without hypokalemia. Interestingly, our study shows the recurrence of paralysis after treatment. Conclusion: Although the precise pathophysiology of the disease is as yet undefined and controversial, it occurs primarily in Asians with an overwhelming male preponderance and prevalence of 2 percent in hyperthyroidism. The interactive roles of thyroid hormone, Na-K pump, and genetically inherited defect in the cellular membrane potential of the skeletal muscle can be speculated. Further investigation will be needed to firmly establish the mechanism of thyrotoxic periodic paralysis.

• Journal of Chest Surgery
• /
• v.40 no.5 s.274
• /
• pp.351-355
• /
• 2007

Background: Postinfarction ventricular septal rupture is associated with mortality as high as $85\sim90%$, if it is treated medically. This report documents our experience with postinfarction ventricular septal rupture that was treated surgically, Material and Method: We retrospectively reviewed the medical records of 11 patients who were operated on due to postinfarction ventricular septal rupture between August 1996 and August 2006. There were 4 men and 7 women, with a mean age of $70{\pm}11$ years (age range: $50\sim84$ years). The location of the rupture was anterior in 7 cases and posterior in 4 cases. The interval between the onset of acute myocardial infarction and the occurrence of the ventricular septal rupture was $2.0{\pm}1.3$ days (range: $1\sim5$ days). Operation was performed at an average of $2.4{\pm}2.7$ days (range: $0\sim8$ days) after the diagnosis of septal rupture. Preoperative intraaortic balloon pump therapy was performed in 10 patients. Result: The infarct exclusion technique was used in all cases. Coronary artery bypass grafting was done in 8 cases, with the mean number of distal anastomosis being $1.0{\pm}0.8$. There was one operative death. In 2 patients, reoperation was performed due to a residual septal defect. The postoperative morbidities were transient atrial fibrillation (n=7), paroxysmal supraventricular tachycardia (n=1), low cardiac output syndrome (n=3), bleeding reoperation (n=2), delayed sternal closure (n=2), acute renal failure (n=2), pneumonia (n=1), intraaortic balloon pump-related thromboembolism (n=1), and transient delirium (n=2). Nine patients have been followed up for a mean of $38{\pm}40$ months except for one follow-up loss. There have been 3 late deaths. At the latest follow-up, all 6 survivors were in a good functional class. Conclusion: We demonstrated satisfactory operative and midterm results with our strategy of preoperative intraaortic balloon pump therapy, early repair of septal rupture by infarct exclusion and combined coronary revascularization.