Yoo, Keon Hee;You, Dong Kil;Lee, Soo Hyun;Sung, Ki Woong;Cho, Eun Joo;Koo, Hong Hoe
Clinical and Experimental Pediatrics
/
v.48
no.2
/
pp.178-185
/
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.
Park, Chang Ro;Park, Kyung Pil;Kim, Heng Mi;Sohn, Yoon Kyung
Clinical and Experimental Pediatrics
/
v.46
no.10
/
pp.989-995
/
2003
Purpose : Dexamethasone is frequently administered to prevent or treat chronic lung disease in human neonates who are also prone to hypoxic-ischemic(HI) insults. Recently, meta-analysis of the follow-up studies reveals a significantly increased odd ratio for the occurrence of cerebral palsy or an abnormal neurologic outcome, and there is conflicting evidence regarding the impact of dexamethasone exposure on HI brain injury. This study was conducted to explore the effect of post-HI dexamethasone administration on neuronal injury in neonatal rats. Methods : HI was produced in seven-day-old rats by right carotid artery ligation followed by two hours of 8% oxygen exposure. At the end of HI, the animals were injected intraperitoneally either with dexamethasone(0.5 mg/kg) or saline. Neuronal injury was assessed seven days after the HI by the area of infarction, TUNEL reactivity, Bcl-2 and Bax expression in brain. Results : Post-insult dexamethasone administration resulted in reduction of weight gain and a higher mortality rate during seven days after HI. Dexamethasone treatment revealed no effect on the size of brain infarction induced by HI. Bax protein expression increased in dexamethasone treated brain but Bcl-2 protein expression and TUNEL reactivity revealed no significant differences between dexamethasone treated and non treated brain. Increased Bax protein expression suggest upregulation of the apoptosis by dexamethasone. Conclusion : The result suggests the adverse role of Post-HI administration of dexamethasone in neonatal HI.
Kim, Sang-Jeong;Han, Dong-Kyun;Baek, Hee-Jo;Kim, Dong-Yeon;Nam, Taek-Keun;Hwang, Tai-Ju;Kook, Hoon
Clinical and Experimental Pediatrics
/
v.53
no.4
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pp.538-547
/
2010
Purpose : This study aims to compare the outcome of total body irradiation (TBI)- or non-TBI-containing conditioning regimens for leukemia in children. Methods : We retrospectively evaluated 77 children conditioned with TBI (n=40) or non-TBI (n=37) regimens, transplanted at Chonnam National University Hospital between January 1996 and December 2007. The type of transplantation, disease status at the time of transplant, conditioning regimen, engraftment kinetics, development of graft-versus-host disease (GVHD), complications, cause of deaths, overall survival (OS), and event-free survival (EFS) were compared between the 2 groups. Results : Among 34 patients with acute lymphoblastic leukemia (ALL), 28 (82.4%) were in the TBI group, while 72.7% (24/33) of patients with myeloid leukemia were in the non-TBI group. Although the 5-year EFS of the 2 groups was similar for all patients (62% vs 63%), the TBI group showed a better 5-year EFS than the non-TBI group when only ALL patients were analyzed (65% vs 17%; $P$=0.005). In acute myelogenous leukemia patients, the non-TBI group had better survival tendency (73% vs 38%; $P$=0.089). The incidence of GVHD, engraftment, survival, cause of death, and late complications was not different between the 2 groups. Conclusion : The TBI and non-TBI groups showed comparable results, but the TBI group showed a significantly higher 5-year EFS than the non-TBI group in ALL patients. Further prospective, randomized controlled studies involving larger number of patients are needed to assess the late-onset complications and to compare the socioeconomic quality of life.
Kang Byoung-Chul;Ha Il-Soo;Kim In-One;Cheong Hae-Il;Choi Yong;Ko Kwang-Wook
Childhood Kidney Diseases
/
v.1
no.2
/
pp.101-108
/
1997
Purpose : The clinical characteristics of renovascular hypertension (RVHT) in children were analyzed. Methods : Medical records of 16 children diagnosed as RVHT on the basis of angiography during Jan. '86 to Jun. 94 in our hospital were reviewed retrospectively. Results : The mean age at the onset was 8.5 yrs and the sex ratio(M:F) was 7:9. The causes of RVHT were Takayasu arteritis in 6, Moyamoya disease in 5, and fibromuscular dysplasia in 3 patients. Abdominal bruit was noted in 6 patients (38%). Peripheral renin activity was raised in all tested patients. Bilateral renal arterial involvemnent was found in 9 patients (56%). Captopril renal scans showed good correlation with angiographic findings. Five patients were treated with antihypertensives only, and blood pressure was controlled completely in 2 and incompletely in 3. Percutaneous transluminal angioplasty was performed in 10 patients with 50% of success rate. However, hypertension was recurred due to restenosis or accompaning aortic stenosis in 3 patients. Surgical treatment was performed in 4 patients, and the blood pressure was controlled partially in 1 and poorly in the remaining 3. Conclusions : Takayasu arteritis, Moyamoya disease and fibromuscular dysplasia are the major causes of childhood RVHT in our country. The diagnosis of RVHT in children should be based on a set of tests individually selected for case by case. For the low curability of the current treatment modalities available, RVHT in children should not be regarded as 'curable' so far. We expect, however, that the outcome will be improved by more extensive application of the newly developed surgical technique.
Park Jong Un;Lee Weon Yong;Kim Kun Il;Hong Ki Woo;Chee Hyun Keun;Shin Yoon Cheol;Lee Jae Woong;Kim Eung Jung
Journal of Chest Surgery
/
v.39
no.1
s.258
/
pp.28-34
/
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.
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.
Purpose : The aim of this study was to evaluate whether the incidence of relapse or nephritis might be influenced by the duration of corticosteroid therapy in children with Henoch-Schonlein purpura(HSP). Methods : We retrospectively analyzed 186 children with a diagnosis of HSP in two major hospitals in Ilsan, Korea from the years 2000 to 2003. To evaluate whether renal involvement or relapse might be influenced by the duration of corticosteroid therapy in children with HSP, one pediatric nephrologist from hospital A, maintained corticosteroid therapy for at least 2 weeks(Group A, n=94). The other from hospital B used only during the symptomatic period(Group B, n=92). Results : There were no significant differences in age, sex, body weight, white blood cell count, hemoglobin, hematocrit, platelet count, serum protein and albumin levels between the two groups. The incidence of abdominal pain or arthralgia also did not differ between two groups. However, the duration of steroid therapy was significantly longer in Group A than in Group B and the cumulative dose of prednisolone was also higher in Group A than in Group B. The development of nephritis was more frequent in Group A. Conclusion : The longer duration of steroid use was not associated with the decreased rate of nephritis. Therefore, corticosteroids should be used carefully in a selected group of HSP children, and be tapered rapidly after control of the acute symptoms.
Hong Semie;Chie Eui Kyu;Park Suk Won;Kim Il Han;Ha Sung Hwan;Park Charn Il
Radiation Oncology Journal
/
v.21
no.2
/
pp.107-111
/
2003
Purpose: To establish the role of stereoactic radiosurgery using a linear accelerator for the treatment of patients with cavernous angloma. Materials and Methods: Between February 1995 and May 1997, 11 patients with cavernous angioma were treated with stereotactic radiosurgery using a linear accelerator. Diagnoses were based on the magnetic resonance imaging in 8 patients, and the histological in 3. The vascular lesions were located on the brainstem (5 cases), cerebellum (2 cases) thalamus (1 case) and cerebrum (3 cases). The clinical presentation at onset included previous intracerebral hemorrhages (9 cases) and seizures (2 cases). All patients were treated with a a linac-based radiosurgery. The median dose of radiation delivered was 16 Gy ranging from 14 to 24 Gy, which was typically proscribed to the 80$\%$ isodose surface (range 50 $\~$ 80$\%$), corresponding to the periphery of the lesion with a single isocenter. Ten patients were followed-up. Results: The median follow-up was 49 months ranging from 8 to 73 months, during which time two patients developed an intracerebral hemorrhage, 1 at 8 months, with the other at 64 months post radiosurgery. One patient developed neurological deficit after radiosurgery, and two developed an edema on the T2 weighted images of the MRI surrounding the radiosurgical target. Conclusion: The use of stereotactic radiosurgery in the treatment of a cavernous angioma may be effective in the prevention of rebleedlng, and can be safely delivered. However, a longer follow-up period will be required.
Kim, Jae-Hyun;Oh, Sam-Sae;Yie, Kil-Soo;Shin, Sung-Ho;Baek, Man-Jong;Na, Chan-Young
Journal of Chest Surgery
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v.40
no.3
s.272
/
pp.200-208
/
2007
Background: The effect of patient-prosthesis mismatch (PPM) on the clinical outcome following aortic valve replacement (AVR) remains controversial. This study compared the surgical outcomes of AVR between patients with a patient-prosthesis mismatch and those having undergone an aortic annular enlargement. Material and Method: Six hundred and twenty seven adult patients, who underwent AVR with stented bioprosthetic or mechanical valves, between January 1996 and February 2006, were evaluated. PPM was defined as an indexed effective orifice area (iEOA) ${\leq}0.85cm^2/m^2$, and Severe if the iEOA${\leq}0.65cm^2/m^2$ PPM was present in 103 (16.4%, PPM group) patients, and severe in 11 (1.8%, SPPM group). During the period of the study, 21 patients underwent an AVR with annular enlargement (AE group). Result: The mean iEOA of the AE group was larger than that of the PPM group ($0.95\;vs.\;0.76cm^2/m^2,\;p=0.00$). The AE group had longer CPB, ACC and operation times than the PPM group, and showed a tendency toward higher operative mortality (14.3% vs. 2.9%, p=0.06). The SPPM group had higher AV pressure gradients (peak/mean) than the AE group (72/45 mmHg vs. 38/25 mmHg, p=0.02/0.06) and suffered more AV related events (AV reoperation or severe aortic stenosis)(45.5% vs. 9.5%, p=0.03). LV masses were not regressed in the patients who experienced an AV related event. Conclusion: During AVR in patients with a small aortic annulus, annular enlargement should be carefully applied taking into account the high risk of operative mortality due to annular enlargement and co-morbidities of patients. Aortic annular enlargement; however, should be considered as an alternative method in patients expected to have a severe PPM after an AVR.
Background: Surgical resection is accepted widely as the standard therapy for complete resectable pulmonary metastases. The number of cases of pulmonary metastasectomy and its survival rate is increasing due to the development of the therapeutic modalities. We attempted to analyze the survival rate and prognosis factors of pulmonary metastasectomy during the last 10 years. Material and Method: We retrospectively analyzed the data of 89 patients who underwent 96 procedures of pulmonary metastasectomy between January 1996 and December 2005. The factors that may influence the long term prognosis such as completeness of resection, the type of primary cancer, the disease-free interval, the number and size of metastasis and the laterality were investigated. Result: There was no operative mortality. The mean disease free interval (DFI) was $29.6{\pm}27.9$ months and there were 3 cases of synchronous metastasis (3.4%). The overall 3, 5 and 10 year survival rate was 52.5%, 32.1% and 20.7%, respectively. The median survival time was 38 months. The 5-year survival rate according to the IRLM appraisal was 63.5%, 33.3%, 22.1% and 0% for stage I, II, III and IV, respectively Univariate analysis showed a better prognosis for patients with a disease free interval of 36 months or more, unilateral metastasis and 4 or less metastases. Conclusion: The survival rate for completely resectable pulmonary metastasectomy was favorable. The disease free interval, laterality and the number of metastasis were the prognosis factors.
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