We studied 90 patients(179 femoral heads) with avascular necrosis of femoral head, who had been performed X-ray, bone scan and MRI to compare of the findings of AVN on bone scan between each other, retrospectively. The patients were 82 males and 9 females, their mean age was 45 years. Radiographic stages were classified by Steinberg modification, radionuclide stages were classified as followed; stage o(or type 0) : normal, stage 1 : faint ring like uptake around the femoral head, stage 2: intense ring like uptake, stage 3: irregular increased uptake with central photon defect, stage 4 : Intense diffuse increased uptake at femoral head and stage 5 : hip joint deformity with relatively mild increased uptake. The findings of MRI were classified according to extent, location, early or advanced lesion, signal intensity of the lesion and joint effusion. 156(87%) of 179 femoral heads had avascular necrosis, 68(75.5%) of 90 patients had bilateral AVN, 35 femoral heads had early stage and 120 had advanced stage. The detection rate of AVN by X-ray and bone scan were 85% (134), 91.6% (143), respectively. Early AVN with atypical types of bone scan showed larger extent, moderate to large amount of joint effusion, soft tissue hypertrophy within joint, and secondary degenerative changes. Bone scan had relatively high detection rate in the diagnosis of AVN of femoral head, and demonstrated various types depending on the disease stage.
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.
Purpose: Simple X-ray study and bone scan have limitations for early diagnosis of bone or bone marrow lesions in multiple myeloma. The purpose of this study was to evaluate the diagnostic usefulness of bone marrow immunoscintigraphy using anti-granulocyte monoclonal antibody for the evaluation of bone involvement in multiple myeloma. Materials and Methods: In 22 patients (Male: 15, Female: 7) with multiple myeloma, we performed whole-body immunoscintigraphy using $^{99m}Tc$-labelled antigranulocyte antibody (BW 250/183, Scintimum $Granulozyt^{(R)}$ CIS, France) and compared the findings with those of simple bone radiography and $^{99m}Tc$-MDP bone scan. Abnormal findings in bone marrow scintigraphy were, considered to be present in case of expansion of peripheral bone marrow or focal photon defect in axial bones. Results: Marrow expansion was noted in 15 of 22 patients (68%). Focal photon defects were found in 18 patients (82%). While one (33%) of 3 patients with Stage II disease showed focal defects in bone marrow scan, abnormal focal defects were observed in 17 of 19 (90%) patients with Stage III. Among 124 focal abnormal sites which were observed in bone marrow scan, bone scan or simple bone radiography, bone marrow scan detected 92 sites (74%), whereas 82 sites (66%) were observed in simple bone radiography(58 sites, 47%) or bone scan(40 sites, 32%). Fifty-one (41%) out of 124 bone lesions were detected by bone marrow scan only, and located mostly in thoracolumbar spine. Conclusion: Bone marrow scan using $^{99m}Tc$-labelled antigranulocyte antibody seems to be a more sensitive procedure for the detection of pathologic bone lesions than simple bone X-ray or bone scan in patients with multiple myeloma.
Cheong, Hee Jeong;Kim, Hye Rim;Lee, Seong Soo;Bae, Eun Joo;Park, Won Il;Lee, Hong Jin;Choi, Hui Chul
Clinical and Experimental Pediatrics
/
v.52
no.2
/
pp.199-204
/
2009
Purpose : Seizure associated with fever may indicate the presence of underlying inherited metabolic diseases. The present study was performed to investigate the presence of underlying metabolic diseases in patients with complex febrile seizures, using analyses of urine organic acids. Method : We retrospectively analyzed and compared the results of urine organic acid analysis with routine laboratory findings in 278 patients referred for complex febrile seizure. Results : Of 278 patients, 132 had no abnormal laboratory findings, and 146 patients had at least one of the following abnormal laboratory findings: acidosis (n=58), hyperammonemia (n=55), hypoglycemia (n=21), ketosis (n=12). Twenty-six (19.7 %) of the 132 patients with no abnormal findings and 104 (71.2%) of the 146 patients with statistically significant abnormalities showed abnormalities on the organic acid analysis (P<0.05). Mitochondrial respiratory chain disorders (n=23) were the most common diseases found in the normal routine laboratory group, followed by PDH deficiency (n=2) and ketolytic defect (n=1). In the abnormal routine laboratory group, mitochondrial respiratory chain disorder (n=29) was the most common disease, followed by ketolytic defects (n=27), PDH deficiency (n=9), glutaric aciduria type II (n=9), 3-methylglutaconic aciduria type III (n=6), biotinidase deficiency (n=5), propionic acidemia (n=4), methylmalonic acidemia (n=2), 3-hydroxyisobutyric aciduria (n=2), orotic aciduria (n=2), fatty acid oxidation disorders (n=2), 2-methylbranched chain acyl CoA dehydrogenase deficiency (n=2), 3-methylglutaconic aciduria type I (n=1), maple syrup urine disease (n=1), isovaleric acidemia (n=1), HMG-CoA lyase deficiency (n=1), L-2-hydroxyglutaric aciduria (n=1), and pyruvate carboxylase deficiency (n=1). Conclusion : These findings suggest that urine organic acid analysis should be performed in all patients with complex febrile seizure and other risk factors for early detection of inherited metabolic diseases.
Lee, Ji Eun;Moon, Kwang Bin;Hwang, Jong Hee;Kwon, Eun Kyung;Kim, Sun Hee;Kim, Jong Won;Jin, Dong Kyu
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.
Seo, Jeong Il;Yoo, Si Uk;Gong, Sung Hyeon;Hwang, Gwang Su;Lee, Hyeon Jung;Kim, Joong Pyo;Choi, Hyeon;Lee, Bo Young;Mok, Ji Sun
Clinical and Experimental Pediatrics
/
v.48
no.7
/
pp.706-710
/
2005
Purpose : Early diagnosis of congenital hearing loss through the neonatal hearing screening test minimizes language defect. This research intends to identify frequency of congenital hearing loss in infants through neonatal hearing screening test with the aim of communicating the importance of hearing test for infants. Methods : From May 20, 2003 to May 19, 2004, infants were subjected to Automated Auditory Brainstem Response test during one month of birth to conduct the test with 35 dB sound. Infants who passed the 1st round of hearing test, were classified into 'pass' group whereas those who did not were classified into 'refer' group. Infants who did not 'pass' in the hearing test conducted within one month of birth were subjected to re-test one month later, and if classified as 'refer' during the re-test, they were subjected to the diagnosis for validation of hearing loss by requesting test to the hearing loss clinic. Results : There was no difference among the 'pass' and 'refer' group in terms of form of childbirth, weight at birth and gestational age. In the 1st test, total of 45 infants were classified into 'refer' group. Six among 35 who were subjected to re-test(17%) did not pass the re-test, and all were diagnosed with congenital hearing loss. This corresponds to 0.35%(3.5 per 1,000) among total number of 1,718 subjects. Conclusion : In our study the congenital hearing loss tends to be considerably more frequently than congenital metabolic disorder. Accordingly, newly born infants are strongly recommended to undergo neonatal hearing screening test.
Kim, So Youn;Chung, Hae Yul;Back, Hee Jo;Choi, Ic Sun;Cho, Chang Yee;Choi, Young Youn
Clinical and Experimental Pediatrics
/
v.45
no.12
/
pp.1512-1518
/
2002
Purpose : Twins have a higher mortality and morbidity than singletons. Co-twin with one fetal death is particularly at risk. We investigated the neonatal outcome of live co-twins when one fetus had died after the 20th gestational week, and associated risk factors. Methods : A retrospective study was performed in fifteen cases of twin pregnancy with single intrauterine fetal deaths after the 20th gestational week during the period from January 1996 to December 2000 at Chonnam University Hospital. Results : Gestational age was $33.7{\pm}3.2weeks$, birth weight was $1,992{\pm}592g$. Interval between one fetal death being detected and the delivery of a live co-twin was $32.4{\pm}29.5days$. There were 11 cases(73.3%) of premature babies less than 37 gestational weeks. Main causes of preterm delivery were preterm labor and premature rupture of membranes. Hematologic findings suggesting disseminated intravascular coagulopathy(DIC) were not found in all mothers before delivery, and was not associated with DIC and encephalomalacia of the live co-twin. Perinatal outcome of fifteen live co-twins was as follows : six were normal(40%), three were DIC(20.0%), three were encephalomalacia(20.0%), one suffered intrauterine growth retardation, there was one case of twin to twin transfusion syndrome, and one of congenital heart disease(atrial septal defect with pulmonary stenosis). The occurrence of DIC and encephalomalacia in live co-twins was not related to placental chorionicity, birth weight, gestational week, and the interval between the detection one fetal death and the delivery of a live co-twin. Conclusion : We could not find any maternal hematologic problems in twin pregnancies complicated by one fetal death. Twenty percent of live co-twins showed DIC and encephalomalacia. However, its associated risk factors were not found. We need to investigate more closely the cases of live co-twins with one intrauterine fetal death.
Background: The late results of repair of tetralogy of Fallot(TOF) are favorable in most patients. Some portion of the patients with tetralogy, however, require reoperation for residual lesions or result in late death. The outcome of patients after tetralogy repair performed during the past 13 years was studied, with an emphasis on postrepair survival and problems including reoperations. Material and Method: A retrospective review of clinical, echocardiographic and catheterization data was performed in 569 of 775 patients with TOF who underwent corrective repair between 1983 and 1995 at Sejong General Hospital, Buchon, Korea. Result: Of 28(4.9%) early deaths(defined as 30 days postrepair), 12 deaths(42.9%) were <1 year of age, with an operative mortality of 15.4%. The surviving 541 patients(age 2.8 months to 43.4 years, median 23 months) have been followed up from 1 month to 12.6 years(median 35 months) postoperatively. Most patients were in good functional class and had normal right ventricular(RV) function. Postrepair results were compared between the transatrial-transpulmonary approach and the conventional right ventriculotomy. The former technique provided a lesser incidence of significant pulmonary regurgitation(P<0.001) and alesser degree of RV dysfunction(P<0.05) compared with those in the latter. There were 10(1.8%) late deaths during the follow-up period and 6 of the deaths were directly related to reoperation or ventricular dysfunction. The 10-year actuarial survival rate was 96.7%. There were 44 reoperations(8.1%) in 39 patients(7.2%), with an operative mortality of 10.3%. The main indications or reoperation included residual ventricular septal defect(VSD) (n=6), pulmonary stenosis(PS) (n=11), VSD with PS(n=17), pulmonary regurgitation(n=7), and tricuspid regurgitation(n=2). The 5- and 10-year freedom from reoperation were 89.4% and 76.1%, respectively. Conclusion: Although the majority of patients with repaired TOF are clinically well, with a high rate of survival, approximately 7% of patients have residual lesions that require surgical therapy. Therefore, the timely and meticulous corrective repair is mandatory to avoid reoperation, and continued close surveillance is also needed for the early detection of residual problems.
To assess contribution of T1-201 rest-24 hour delay redistribution in detection of viable myocardium, we studied the predictive value of this redistribution in 17 patients who peformed rest-24 hour delay perfusion SPECT before bypass surgery. Regional wall motion was compared with gated SPECT in 10 patients and echocardiography in 7 patients before and after bypass surgery. Rest and 24 hour delayed uptakes were scored from 0 (normal perfusion) to 3 (defect). In rest SPECT, 56 segments showed perfusion decrease. Thirty four segments(61%) improved after surgery and were defined as viable Nineteen(34%) segments had more uptake of T1-201 at 24 hour delay, and the other 37 segments did not. In 81%(25/31) of segments with mildly decreased perfusion, wall motion after bypass surgery improved, 57% (8/14) of segments with severely decreased perfusion improved, and 9%(1/11) of segments with defects improved. In 14 among 19 segments which had more T1-201 uptakes at 24 hour delay, wall motion was improved(positive predictive value of redistribution: 74%). 20 among 37 segments which had persistent decreases in rest-24 hour redistribution improved and 17 did not(negative predictive value: 46%). Segments having severe perfusion decrease or defects showed improved wall motion after surgery in 64%(7/11), if it had redistribution at delay. Segments with either mildly decreased uptake in resting or rest-delayed redistribution showed improved wall motion in 76%(32/42). Among the 14 segments which showed improvement in wall motion, 10 had partial reversibility in stress-rest images and the other 4 had persistent perfusion defects in stress-rest images. These 4 segments were found viable only with rest-24 hour delayed perfusion SPECT. We concluded that rest T1-201 uptake or redistribution at 24 hour delay should be referred as an evidence to warrant postoperative improvement of abnormal wall motion and we could predict myocardial viability with preoperative rest-24 hour delay perfusion SPECT in the segments with rest perfusion decreases.
Purpose : To evaluate the usefulness of functional MRI (fMRI) of visual cortex in patients with ischemic infarction in the occipital lobe. Materials and Methods : Four patients with the symptoms and signs of visual cortical ischemia were included. Functional MRI was performed by 2D-FLASH technique with the parameter of 90/56msec TR/TE, $40^{\circ}$ flip angle, $240{\times}240{\;}FOV,{\;}64{\times}128$ matrix number, 8.32 seconds acquisition time, 8mm slice thickness. An axial slice including both visual cortices was selected and alternative activation and resting of the visual cortex was performed using red color photostimulator. all patients undertook visual field test, and vascular abnormality was examined by MRA (n=4) and DSA (n=2). fMRI results were compared with the results of a visual field test, conventional MRI and cerebral angiography. Results : On fMRI, decreased activity of the visual cortex was found in the occipital lobe corresponding to stenosis of the posterior cerebral artery or its branch noted on angiogram. However, 2 of 4 patients showed no abnormal findings on conventional MRI. Visual field defect was noted in 3 patients, one and of whom showed no abnormality on conventional MRI and diffusion-weighted image, but revealed decreased activity in the corresponding visual cortex on fMRI. Conclusion : fMRI may be a sensitive method for detection of the status of decreased blood flow or vascular reserve which other methods can not.
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