• Investigative Magnetic Resonance Imaging
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• v.4 no.2
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• pp.107-112
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• 2000

Purpose : To evaluate the usefulness of comparison of the signal intensity of uterine septum in the differential diagnosis of bicornuate and septate uterus on magnetic resonance (MR) imaging. Materials and methods : Preoperative MR imaging findings of surgically proven 5 bicornuate and 6 septate uteri were retrospectively analyzed. Because preoperative differential diagnosis of both was possible in all cases in terms of the intercornual distance, external contour of uterine fungus, and divergent angle of two uterine cavities, these criteria were excluded in this study. The signal intensity of uterine septum in patients with bicornuate and septate uterus was analyzed on T1-weighted and fast spin echo T2-weighted images obtained in the axial and coronal planes, using a 1.5-T MR scanner. The signal intensity of uterine septum especially on T2-weighted images was compared with that of myometrium or junctional zone. Results : The signal intensity of uterine septum in patients with bicornuate uterus (n=5) and septate uterus (n=6) was similar to that of myometrium in all cases on T1-weighted images. The septum of bicornuate uterus (n=5) on fast spin echo T2-weighted images was isointense with myometrium in three and hypointense in two cases. The uterine septum of septate uterus (n=6) on T2-weighted images was isointense with myometrium in two, hypointense in two, and isointense with or more hypointense than junctional zone in two cases. No patient showed different signal intensity between upper and lower uterine septum. Conclusion : Because the MR signal intensity of the uterine septum in bicornuate or septate uterus is variable, it should not be used alone in the differential diagnosis of them. In these clinically important differentiation, therefore, comprehensive analysis of MR findings in terms of the external contour of uterine fundus, intercornual distance, divergent angle of two uterine cavities, in addition to the signal intensity of the uterine septum, should be considered.

• The Journal of the Korea Contents Association
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• v.8 no.11
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• pp.210-216
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• 2008

In this study, image comparisons were carried out using a MRI contrast medium which was derived by mixing a polyaminocarboxylic ligand and a gadolinium (III) transition metal which is paramagnetic and has good neutron absorbing capabilities with Gd-DTPA which is currently being used widely in the clinical setting. By using a 1.0T (Harmony, SIEMENS) MR equipment, phantoms of which 100cc of saline was diluted with a diethylenetriaminetriacetic acid derivative and Gd-DTPA were imaged. The amount of diethylenetriaminetriacetic acid and Gd-DTPA which was diluted into the 100cc of saline was 0.05mmol/L, 0.1mmol/L, 0.15mmol/L, 0.2mmol/L, 0.3mmol/L, 0.5mmol/L, 1.0mmol/L, 2.0mmol/L, 3.0mmol/L and 4.9mmol/L respectively. Head coils were used and while fixing the SE pulse sequence and image variable (as TE is 14ms, 1NEX with a 256x201 matrix), the signal intensity and simple contrast ratios according to changing concentrations and TR were compared with various TR at 300ms, 400ms, 500ms, 600ms, 700ms, 800ms, 900ms, 1000ms, 1200ms, 1400ms and 1600ms. According to the comparison results of the signal intensity of the image based on changes in contrast medium concentrations and TR, the differences in signal intensity between the two contrast mediums were found to be small at $1.0{\sim}2.0mmol/L$ when the highest signal intensity was achieved. However, at concentrations of 1.0mmol/L or less, the signal intensity was markedly higher in the Diethylenetriaminetriacetic acid derivative than in the Gd-DTPA complex. It was also found that the differences in the signal intensities demonstrated by the concentrations of the contrast mediums were affected by the TR. Accordingly, the efficacy of the Diethylenetriaminetriacetic acid derivative was shown to be better than the Gd-DTPA and also possible to get the optimum image quality by the use of an appropriate TR with appropriate concentrations of contrast medium.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.178-185
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• 2005

Purpose : The purpose of this study was to evaluate the outcome of children with juvenile myelomonocytic leukemia(JMML) treated with allogeneic hematopoietic stem cell transplantation(allo-HSCT). Methods : Eleven JMML patients aged 8-39 months underwent allo-HSCT. The sources of grafts were unrelated donors(n=7), HLA-matched siblings(n=3) and an HLA 1-antigen mismatched familial donor. All patients had received chemotherapy ${\pm}13$-cis-retinoic acid(CRA) before transplant, and CRA was used, posttransplant, in six patients. Results : Only three patients were in complete remission(CR) at the time of transplantation. Initial chimeric status revealed complete donor chimerism(CC) in five patients, mixed chimerism(MC) in five and autologous recovery(AR) in one. One patient with MC having persistent splenomegaly eventually turned to CC and CR after rapid tapering of cyclosporine, combined with daily use of CRA. An AR case relapsed shortly after transplant but was rescued with second, unrelated cord blood transplantation. Ultimately, six patients are alive, event-free, with a median follow-up of 15.5 months posttransplant. All three deaths occurred in patients who failed to achieve CC, leading to disease progression. Conclusion : We suggest that graft-versus-leukemia effect play an important role and CRA a possible role in posttransplant leukemic involution in JMML. In patients whose leukemic burden is still high with MC after transplant, early tapering of immunosuppressants and introduction of CRA might provide a chance of a cure for some patients.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.548-553
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• 2010

Purpose : IgA nephropathy (IgAN) is the most commonly occurring form of chronic glomerulonephritis in pediatric cases. Human leukocyte antigen (HLA) genes have been implicated in various inflammatory and autoimmune diseases. The present study was conducted to investigate the association between 2 single nucleotide polymorphisms (SNPs) of the HLA-G gene and childhood IgAN. Methods : The authors analyzed and compared $HLA-G$ gene SNPs (rs1736936 and rs2735022) in 174 patients with childhood IgAN and in 438 healthy controls. In addition, IgAN patients were dichotomized and compared with respect to proteinuria (< and >$4mg/m^2/hour$), the presence or absence of podocyte foot process effacement, and the presence of pathologically early and advanced disease markers such as interstitial fibrosis, tubular atrophy, or global sclerosis. Results : No significant SNP frequency differences were observed for the $HLA-G$ gene between IgAN patients and the control group. Moreover, no significantly associated SNP was observed with the presence of proteinuria, podocyte foot process effacement, or pathologically advanced markers. However, the haplotype, composed of rs1736936 and rs2735022, showed a significant association with the susceptibility to develop childhood IgAN (haplotype T/C: dominant model, $P$=0.049; haplotype C/T: recessive model, $P$=0.030). Conclusion : Our results indicate that rs1736936 and rs2735022 as the $HLA-G$ gene promoter haplotype might be associated with the susceptibility to develop childhood IgAN in the Korean population.

• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.178-181
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• 2007

Purpose : The renal manifestations of tuberous sclerosis complex (TSC) are remarkably diverse, including polycystic kidney disease, simple renal cysts, renal cell carcinomas, and angiomyolipomas. All of these occur in children as well as adults in TSC. Angiomyolipomas, which can cause spontaneous life-threatening hemorrhages, are by far the most prevalent and the greatest source of morbidity. Here, we will address our experience, adding to the literature on pediatric patients with TSC requiring evaluation and treatment for renal manifestations. Methods : A retrospective analysis was made on 19 patients in whom TSC was diagnosed between May 2001 and Oct. 2005 at Severance Hospital. All patients had clinical diagnoses of TSC as defined by the 1998 tuberous sclerosis complex consensus conference. Results : The patients consisted of 13 boys and 6 girls with a mean age of 7.3 years (range 1 to 22). The renal disease associated with TSC included angiomyolipoma in nine patients (47.4 percent), renal simple cyst in one (5.3 percent), hydronephrosis in one (5.3 percent) patient. Eight patients (42.1 percent) presented with normal kidney contours at abdominal ultrasonography. One patient underwent renal replacement therapy due to chronic renal insufficiency after nephrectomy. Hemorrhage from angiomyolipoma was not detected. Conclusion : In our review of 19 cases of TSC, renal manifestations are reported in 57.9 percent of patients. Asymptomatic angiomyolipoma associated with TSC grow gradually, although severe hemorrhages are rare. So patients with TSC should be followed up with serial computerized tomography or abdominal ultrasonography. And also, renal function should be monitored conservatively.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.2
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• pp.199-206
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• 2009

Purpose: To analyze the clinical spectrum of posttransplantation lymphoproliferative disorder (PTLD) after liver transplantation in children. Methods: From January 1988 to June 2009, we retrospectively reviewed the medical records of 8 PTLD cases among 148 pediatric patients underwent liver transplantation. The age at transplantation, time of presentation after transplantation, clinical manifestations, histologic diagnosis, results of EBV (Epstein-Barr virus) assessments, managements and outcomes of PTLD were investigated. Results: The prevalence of PTLD in liver transplant pediatric recipients was 5.4% (8 of 148). The mean age of patients was 25.4${\pm}$21.3 months (range 10 to 67 months). Seven of 8 patients (87.5%) underwent liver transplantation before 1 year of age. The common clinical presentations were persistent fever (8 of 8, 100%) and bloody diarrhea (6 of 8, 75%). PTLD was diagnosed with gastrointestinal endoscopic biopsies in five patients and surgical biopsies in three. Histologic findings showed early lesion in three patients, polymorphic in two, and monomorphic in three. Burkitt lymphoma and lymphoblastic lymphoma were found in two of 3 monomorphic patients. Seven of 8 patients were found with EBV-positive. Eight patients were treated with dose reduction of immunosuppressants and infusion of ganciclovir. Rituximab was added to four patients. PTLD were successfully managed in all patients except one who died of sepsis during chemotherapy. Conclusion: Major risk factor of PTLD was to undergo liver transplantation before 1 year of age. Continuous monitoring for EBV viral load and gastrointestinal endoscopic biopsy may be useful to early detection of PTLD.

• Pediatric Infection and Vaccine
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• v.11 no.1
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• pp.73-80
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• 2004

Purpose : The serial clinical findings, biochemical results, and serological hepatitis B virus(HBV) markers in Korean children with chronic HBV infection were analyzed to determine the relationships among these factors. Methods : Ninety children have been chosen from those who have visited to the Department of Pediatrics at St. Vincent's Hospital in The Catholic University of Korea from July 1st, 1995 to June 30th, 2000. The sample patients were followed up for over six months. HBV markers and liver function tests were all performed. Results : All children were asymptomatic at presentation. Eighty-three percent of the children had a history of chronic HBV infection in their families. Eighty-one percent were HBeAg positive, 16% were anti-HBe positive, while 3% were all HBeAg and anti-HBe negative. The prevalence of HBeAg among three age groups : 0~5; 6~10; and 11~15 year-old was 90%, 96% and 61% respectively. The prevalence of HBeAg in less than 10 year-old group was significantly higher than 11~15 year-old group(P=0.001). Serum ALT levels were within 40 IU/L in 64% children, 41~80 IU/L in 17%, 81~200 IU/L in 10%, and beyond 201 IU/L in 9%. The percentage of abnormality of ALT levels in HBeAg positive patients was significantly higher than that of HBeAg negative(P=0.036). Eleven of the 73 HBeAg positive children lost their HBeAg and seroconverted to anti-HBe. In these cases, all had transient elevations in ALT levels before HBeAg seroconversions. The annual rates of spontaneous seroconversion of HBeAg and HBsAg were 9.7% and 0.6%, respectively. Conclusion : Recognition of the dynamics of these changes in viral markers and biochemical findings is needed in the selection and evaluation of therapeutic regimens, establishment of treatment, and calling for controlled trials with adequate follow-up. The hepatitis B carrier state may be asymptomatic in children however, continued surveillance of carriers is important to determine the individual adverse prognostic factors of chronic HBV infections.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.220-228
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• 2007

Purpose : We studied the value of clinical signs, laboratory findings and $^{99m}technetium$ dime-rcaptosuccinic acid(DMSA) scan in predicting the presence of vesicoureteral reflux(VUR) in children with first febrile urinary tract infection(UTI). Methods : A retrospective analysis of 84 hospitalized children with first febrile UTI was performed. They underwent DMSA scan and voiding cystourethrography(VCUG) during the acute phase, and were divided into three groups according to the results of the VCUG: absence of VUR, mild(grade I-II) and severe VUR(grade III-V). We studied the relation of VUR to age, gender, fever duration, causative organism, white blood cell count, serum C-reactive protein and result of DMSA scan. Results : Among 84 patients, 6 had mild and 17 had severe VUR. Thirty-eight had abnormal DMSA scan. results Patients with VUR were older than those without VUR(P<0.01). There was a lower probability of infection with Escherichia coli in patients with severe VUR than in those with mild and absent VUR(P<0.01). An abnormal DMSA scan correlated with the presence and severity of VUR(P<0.05). Severe VUR was present in 10.9% of patients with normal DMSA scan. The sensitivity, specificity, positive and negative predictive values of the DMSA scan in predicting the presence of VUR were 69.6%, 63.9%, 42.1%, and 84.8%, respectively. Conclusion : An abnormal DMSA scan correlated to the presence and severity of VUR, but the sensitivity, specificity and positive predictive value of the DMSA scan were low. There-fore, patient with an abnormal DMSA scan requires a VCUG. In order to prevent missing the 10.9% of patients with severe VUR but normal DMSA scans, a VCUG should be performed in patient with normal DMSA scan.

• Journal of Chest Surgery
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• v.39 no.1 s.258
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• pp.28-34
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• 2006

Background: There has been an increase in the number of elderly patients considered for coronary artery bypass grafting (CABG). Recently, there were many satisfactory reports of coronary artery bypass grafting (CABG) in old age due to the development in operative technique and postoperative management. We evaluated operative and follow-up results of patients 70 years of age and older compared to 60 years old. Material and Method: We retrospectively studied the cases of 74 consecutive patients 70 years or older (group A) who underwent a elective CABG from January 2000 to December 2003 and compared that of relatively young age group (group B, 60-69 years old). We compared preoperative characteristics, operation technique, postoperative results that effect outcome, also we investigated late mortality and cardiac events at follow-up periods. Result: Preoperative demographic and clinical characteristics of two groups were not different, except preoperative renal dysfunction(serum creatinine: $\geq$1.4 mg/dl) (group A 17, 23$\%$ vs group B 14, 9$\%$) (p=0.024). There was no difference of the mean number of distal anastomosis and the left ventricular ejection fraction in group A decreased significantly from 53.7$\pm$13$\%$ preoperatively to 49.9$\pm$ 12$\%$ postoperatively (p=0.02), but not changed in group B. There was no difference at operative mortality rate and postoperative major morbidity rate, but wound problem of saphenous vein harvest site was significantly higher in group A than group B (6.8$\%$ vs 0.7$\%$, p=0.02). The mean follow up duration was 24.3$\pm$13 months and the cumulative survival were 95.4$\%$ at 2 year and 79.9$\%$ at 4 year in group A and 95.4$\%$ at 2 year and 90.1$\%$ at 4 year in group B (p=ns). Conclusion: We conclude that age is not a factor of determination when we decide about operation because coronary artery bypass grafting in elderly more than 70 years old can be performed with a low mortality rate and acceptable morbidity rate.

• Journal of Chest Surgery
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• v.30 no.4
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• pp.363-372
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• 1997

One hundred forty-four patients underwent operation for coarctation of the aorta at Seoul National University Children's Hospital between June 1986 and Decembsr 1995. Age ranged 0.1 to 191 months. Of these 78.5%(113) were infants. We classified the patients in terms of the anatomic location of coarctatiln and the associatCd anomalies(I[401= primary coarctation, 11(741=isthmic hypoplasia, lIIf30)=tubular hypoplasia involving transverse arch, Ar63 =with ventricular septal defect, B(28)=with other major cardiac defects). Subcalvian flap coarctoplasty(60), resection & anastomosis(44), extended aortoplasty(26), and onlay patch(14) were used as surgical methods. Overall operative mortality was 16.0(23/144)%. The hospital mortality was signific'antly higher in patheints with type 111, subtype B, younger age(under 3 months), extended aortoplasty(p(0.01). However, one-stage total repair in patients with subtype A or B were not found to be a predictor of hospital death. Restenosis had occured in 18 patients among 121 survivals(14. 9%). The mean follow-up period was 29.1 $\pm$28.8(0~129.2) months. Preoperative, immediate postoperative(within 3 months after operation) and postoperative(later than 6 months after operation) echocardiographic data on the dimensions of ascending aorta(AA), transverse arch(TA), an4 aortic isthmus(Al) were available in 77 patients(I=20, ll=42, 111= 15). Preoperative and postoperative aortic isthmus(All) and tra sverse arch indices(TAI), defined as TAIAA & AIIAA respectively, were compared. Immediate postoperative All in type 1, II and TAI in type 111 were significantly smaller in stenotic than non-stenotic group suggesting incomplete relieves of stenotic segment Younger age, subclavian coarctoplasty in patient under 3 months of age were round to be the risk factors for restenosis in this series. In conclusion, We found that aortic arch index and transverse arch index can be a useful tool to figure out the anatomic and clinical characteristics of the patients with aortic coarctation, and that anatomy, associated anomalies, age, and surgical methods may influence the surgical outcome of the coarctation repair.