• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제24권3호
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• pp.288-294
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• 2021

Purpose: Despite aggressive medical and nutritional management, patients with methylmalonic acidemia (MMA) often suffer from multi-organ damage. Early deceased donor liver transplantation (DDLT) has emerged as an intervention to prevent disease progression. We investigated the efficacy of living donor LT (LDLT) with a potential carrier of MMA and a small volume of graft in patients with MMA as an alternative to DDLT. Methods: Of five patients (three male, two female; median age 5.7 years; range, 1.3-13.7 years), four underwent carrier LDLT, while one underwent non-carrier auxiliary LDLT. All patients received pre- and post-LT continuous renal replacement therapy and were provided with minimal restriction diet according to serum MMA level after LT. MMA levels in the serum and urine, the incidence of metabolic crisis, and clinical findings before and after LT were compared. Results: The survival rate was 100% during 2.2 years of follow up period after LT. In all five cases, MMA titer in the serum after transplantation decreased with less restrictive diet. Metabolic crisis was not observed during the follow-up period. In addition, no patient showed progression of severe renal impairment requiring hemodialysis. Progression of delayed cognitive development was not observed. Social functioning with improved neuropsychiatric development was observed. Conclusion: This study showed that LDLT achieved improved quality of life with less restrictive diet, therefore it could be a feasible alternative option to DDLT for the treatment of patients with MMA, even with an auxiliary LT.

• Asian Pacific Journal of Cancer Prevention
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• 제15권24호
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• pp.10729-10733
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• 2015

Background: Adjuvant androgen deprivation therapy (ADT) is a treatment option for prostate cancer (PC) patients after radical prostatectomy (RP). Although it can achieve a good progression-free survival rate, some patients still develop clinical metastasis. We here investigated risk factors of clinical metastasis in post-prostatectomy patients who received immediate adjuvant ADT. Materials and Methods: We identified 197 patients with non-metastatic PC who underwent RP at our institution between 2000 and 2012, followed by adjuvant ADT. The associations of various clinicopathologic factors with clinical metastasis (primary endpoint) and cancer-specific survival (secondary endpoint) were assessed. Multivariate analysis was conducted using a Cox proportional hazards model. Median follow-up was 87 months after RP. Results: Nine (4.6%) patients developed clinical metastasis and six (3.0%) died from PC. Eight of nine metastatic patients had a pathologic Gleason score (GS) 9 and developed bone metastasis, while the remaining one had pathologic GS 7 and developed metastasis only to para-aortic lymph nodes. On multivariate analyses, pathologic GS ${\geq}9$ and regional lymph node metastasis (pN1) were independent predictors of clinical metastasis and pathologic GS ${\geq}9$ was an independent predictor of cancer-specific death. Conclusions: Pathologic GS ${\geq}9$ and pN1 were independent predictors of clinical metastasis in post-prostatectomy patients who received immediate adjuvant ADT. Furthermore, pathologic GS ${\geq}9$ was an indispensable condition for bone metastasis, which may imply that patients with GS ${\leq}8$ on adjuvant ADT are unlikely to develop bone metastasis.

• IMMUNE NETWORK
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• 제3권4호
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• pp.287-294
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• 2003

Background: In kidney transplantation, donor specific transfusion may induce tolerance as a result of some immune regulatory cells against the graft. In organ transplantation, the immune state arises from a relationship between the immunocompromised graft and the immunocompetent host. However, a reverse immunological situation exists between the graft and the host in hematopoietic stem cell transplantation (HSCT). In addition, early IL-2 injections after an allogeneic murine HSCT have been shown to prevent lethal graft versus host disease (GVHD) due to CD4+ cells. We investigated the induction of the regulatory CD4+CD25+ cells after a transfusion of irradiated recipient cells with IL-2 into a donor. Methods: The splenocytes (SP) were obtained from 6 week-old BALB/c mice ($H-2^d$) and irradiated as a single cell suspension. The donor mice (C3H/He, $H-2^k$) received $5{\times}10^6$ irradiated SP, and 5,000 IU IL-2 injected intraperitoneally on the day prior to HSCT. The CD4+CD25+ cell populations in SP treated C3H/He were analyzed. In order to determine the in vivo effect of CD4+CD25+ cells, the lethally irradiated BALB/c were transplanted with $1{\times}10^7$ donor BM and $5{\times}10^6$ CD4+CD25+ cells. The other recipient mice received either $1{\times}10^7$ donor BM with $5{\times}10^6$ CD4+ CD25- cells or the untreated SP. The survival and GVHD was assessed daily by a clinical scoring system. Results: In the MLR assay, BALB/c SP was used as a stimulator with C3H/He SP, as a responder, with or without treatment. The inhibition of proliferation was $30.0{\pm}13%$ compared to the control. In addition, the MLR with either the CD4+CD25+ or CD4+CD25- cells, which were isolated by MidiMacs, from the C3H/He SP treated with the recipient SP and IL-2 was evaluated. The donor SP treated with the recipient cells and IL-2 contained more CD4+CD25+ cells ($5.4{\pm}1.5%$) than the untreated mice SP ($1.4{\pm}0.3%$)(P<0.01). There was a profound inhibition in the CD4+CD25+ cells ($61.1{\pm}6.1%$), but a marked proliferation in the CD4+CD25- cells ($129.8{\pm}65.2%$). Mice in the CD4+CD25+ group showed low GVHD scores and a slow progression from the post-HSCT day 4 to day 9, but those in the control and CD4+CD25- groups had a high score and rapid progression (P<0.001). The probability of survival was 83.3% in the CD4+CD25+ group until post-HSC day 35 and all mice in the control and CD4+CD25- groups died on post-HSCT day 8 or 9 (P=0.0105). Conclusion: Donor graft engineering with irradiated recipient SP and IL-2 (recipient specific transfusion) can induce abundant regulatory CD4+CD25+ cells to prevent GVHD.

• Tuberculosis and Respiratory Diseases
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• 제70권2호
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• pp.139-142
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• 2011

Background: Lung cancer is responsible for substantial proportions of cutaneous metastasis from internal malignancies. The aim of this study was to evaluate the clinical manifestations and outcomes of cutaneous metastasis in Korean lung cancer patients. Methods: On a retrospective basis, we analyzed medical records of all patients diagnosed with lung cancer from 2000 to 2006. Results: Cutaneous metastases were found in 10 of 4,385 patients. The number of cases was highest for squamous cell carcinoma. However, there was no metastasis from 754 cases of small cell carcinomas. Cutaneous metastasis was detected during staging work-up in 4 patients and it was the presenting sign of recurrence post-operative in 2 patients. Average time from the diagnosis to discovery of cutaneous metastasis was 16.3 months and median survival was 8.5 months (range, 1.8~19.1 months). Conclusion: Physicians should be acquainted with clinical manifestations and outcomes of cutaneous metastasis from lung cancer to detect new, recurrent cancer, or disease progression, and to administer appropriate and prompt management.

• Journal of Korean Neurosurgical Society
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• 제30권4호
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• pp.472-478
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• 2001

Objectives : The authors present a retrospective analysis of 100 consecutive adult patients harboring craniopharyngiomas who underwent microsurgical resection between 1981 and 1999 to assess the long-term outcome of surgical treatment and to determine the most optimal management strategy. Methods : The extent of surgical removal was divided into four categories ; GTR(gross total removal), RSTR(radical subtotal removal), STR(subtotal removal),and PR(partial removal). The median follow-up period was 50 months(4-198). CT scan and/or MR imaging and hormonal status were evaluated to the last follow-up. Results : Visual disturbance was the most common presentation, which was improved in 42 cases and aggravated in 19 cases following the operation. Hypopituitarism was detected in 56 patients preoperatively, 82 during the immediate postoperative period, and 76 at the last follow-up. Improvement of pituitary function was not observed in any of these patients. Twenty of 100 patients showed recurrence at the mean of 27 months(3 to 196). The median progression-free survival(PFS) time of all patients was 145 months and 5-year PFS rate was 74%. Five-year PFS rate of GTR or RSTR group(71%) was significantly higher than that of STR or PR group(30%)(p=0.01). Postoperative radiation therapy significantly prolonged the PFS from 94 months in non-radiation group to 182 months(p=0.002). However, there was no statistical difference in number of patients who required hormonal replacement therapy between radiation and non-radiation group. Conclusion : Visual disturbance can be improved by early diagnosis and surgical decompression. GTR or RSTR in selected patients is considered a proper surgical strategy. Post-operative radiation therapy for residual tumors must be considered, although the ideal timing of radiation therapy is to be determined.

• 한국임상수의학회지
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• 제26권2호
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• pp.138-143
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• 2009

허혈/재관류 손상은 급성신부전의 주요 원인이며 이식된 신장의 기능지연을 유발하여 거부반응의 발생을 증가시킨다. 본 연구에서는 쥐의 허혈/재관류 손상모델에서 허혈시간에 따른 면역세포의 변화를 평가하였다. 8주령 수컷 SD rat의 좌신을 각각 30, 45, 60분간 허혈/재관류 동안에 우신을 적출 하였고, 대조군은 우신만 적출하였다. 신장기능은 0, 1, 2, 3, 5, 7일에 각각 평가하였으며, 허혈/재관류 후 1일과 7일에 신장조직을 채취하여 면역형광염색(DCs, NK cells, macrophages, B cells, CD4+ and CD8+ T cells)과 H&E 염색을 실시하였다. 허혈 시간이 증가할수록 신장기능이 감소되었으나, 신장 세뇨관괴사와 염증세포의 침윤이 증가하였다. 허혈/재관류 후 신장조직에서 DCs, NK cells, macrophages, CD4+ T cells, CD8+ T cells의 침윤 증가와 재관류 후 7일째 B cells의 침윤이 관찰되었다. 면역세포는 허혈시간 뿐 아니라 재관류 시간이 증가에 따라 뚜렷하게 관찰되었다. 이 결과는 허혈시간이 증가됨에 따라 면역반응이 증가될 수 있으며, 허혈재관류 손상이 비항원성 면역반응을 유도할 수도 있다는 것을 의미한다.