• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Journal of Chest Surgery
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• v.39 no.5 s.262
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• pp.359-365
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• 2006

Background: Arterial grafts have been used to achieve better long-term results for coronary revascularization. Bilateral internal thoracic artery (ITA) grafts have a better results, but it may be not used in some situations such as diabetes and chronic obstructive pulmonary disease (COPD). We evaluated the clinical and angiographic results of composite left internal thoracic artery-radial artery (LITA-RA) Y graft. Material and Method: Between April 2002 and September 2004, 119 patients were enrolled in composite Y graft for coronary bypass surgery. The mean age was $62.6{\pm}8.8$ years old and female was 34.5%. Preoperative cardiac risk factors were as follows: hypertension 43.7%, diabetes 33.6%, smoker 41.2%, and hyperlipidemia 22.7%, There were emergency operation (14), cardiogenic shock (6), left ventricle ejection fraction (LVEF) less than 40% (17), and 17 cases of left main disease. Coronary angiography was done in 35 patients before the hospital discharge. Result: The number of distal anastomoses was $3.1{\pm}0.91$ and three patients (2.52%) died during hospital stay. The off-pump coronary artery bypass (OPCAB) was applied to 79 patients (66.4%). The LITA was anastomosed to left anterior descending system except three cases which was to lateral wall. The radial Y grafts were anastomosed to diagonal branches (4), ramus intermedius (21), obtuse marginal branches (109), posterolateral branches (12), and posterior descending coronary artery (8). Postoperative coronary angiography in 35 patients showed excellent patency rates (LITA 100%, and RA 88.5%; 3 RA grafts which anastomosed to coronary arteries <70% stenosed showed string sign with competitive flow). Conclusion: The LITA-RA Y composite graft provided good early clinical and angiographic results in multivessel coronary revascularization. But it should be cautiously used in selected patients.

• The Korean Journal of Nuclear Medicine Technology
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• v.14 no.1
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• pp.94-100
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• 2010

Purpose: We can evaluate function of kidney by Glomerular Filtration Rate (GFR) test using $^{99m}Tc$-DTPA which is simple. This test is influenced by several parameter such as net syringe count, kidney depth, corrected kidney count, acquisition time and characters of gamma camera. In this study, we evaluated predose count according to matrix size in the GFR test using $^{99m}Tc$-DTPA. Materials and Methods: Gamma camera of Infinia in GE was used, and LEGP collimator, three types of matrix size ($64{\times}64$, $128{\times}128$, $256{\times}256$) and 1.0 of zoom factor were applied. We increased radioactivity concentration from 222 (6), 296 (8), 370 (10), 444 (12) up to 518 MBq (14 mCi) respectively and acquired images according to matrix size at 30 cm distance from detector. Lastly, we evaluated these values and then substituted them for GFR formula. Results: In $64{\times}64$, $128{\times}128$ and $256{\times}256$ of matrix size, counts per second was 26.8, 34.5, 41.5, 49.1 and 55.3 kcps, 25.3, 33.4, 41.0, 48.4 and 54.3 kcps and 25.5, 33.7, 40.8, 48.1 and 54.7 kcps respectively. Total counts for 5 second were 134, 172, 208, 245 and 276 kcounts from $64{\times}64$, 127, 172, 205, 242, 271 kcounts from $128{\times}128$, and 137, 168, 204, 240 and 273 kcounts from $256{\times}256$, and total counts for 60 seconds were 1,503, 1,866, 2,093, 2,280, 2,321 kcounts, 1,511, 1,994, 2,453, 2,890 and 3,244 kcounts, and 1,524, 2,011, 2,439, 2,869 and 3,268 kcounts respectively. It is different from 0 to 30.02 % of percentage difference in $64{\times}64$ of matrix size. But in $128{\times}128$ and $256{\times}256$, it is showed 0.60 and 0.69 % of maximum value each. GFR of percentage difference in $64{\times}64$ represented 6.77% of 222 MBq (6 mCi), 42.89 % of 518 MBq (14 mCi) at 60 seconds respectively. However it is represented 0.60 and 0.63 % each in $128{\times}128$ and $256{\times}256$. Conclusion: There was no big difference in total counts of percentage difference and GFR values acquiring from $128{\times}128$ and $256{\times}256$ of matrix size. But in $64{\times}64$ of matrix size when the total count exceeded 1,500 kcounts, the overflow phenomenon was appeared differently according to predose radioactivity of concentration and acquisition time. Therefore, we must optimize matrix size and net syringe count considering the total count of predose to get accurate GFR results.