• Clinical and Experimental Pediatrics
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• 제58권5호
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• pp.178-182
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• 2015

Purpose: The purpose of this study was to determine the frequency of $CD4^+CD25^+FoxP3^+$ regulatory T cells (Treg) in the peripheral blood of patients with childhood chronic immune thrombocytopenic purpura (ITP) exhibiting thrombocytopenia and spontaneous remission. The findings of this study indicate the possibility of predicting spontaneous recovery and pathogenesis of childhood chronic ITP. Methods: Eleven children with chronic ITP (seven thrombocytopenic and four spontaneous remission cases; mean age, 8.8 years; range, 1.7-14.9 years) were enrolled in this study. Five healthy children and eight healthy adults were included as controls. The frequency of Treg was evaluated by flow cytometry in the peripheral blood. Results: In this study, four patients (36%) achieved spontaneous remission within 2.8 years (mean year; range, 1.0-4.4 years). The frequency of Treg was significantly lower in patients with persisting thrombocytopenia ($0.13%{\pm}0.09%$, P<0.05), than that in the patients with spontaneous remission ($0.30%{\pm}0.02%$), healthy adults controls ($0.55%{\pm}0.44%$), and healthy children controls ($0.46%{\pm}0.26%$). A significantly positive correlation was found between the frequency of Treg and the platelet count in children. Conclusion: These data suggest that a lower frequency of Treg contributes to the breakdown of self-tolerance, and may form the basis for future development of specific immunomodulatory therapies. Furthermore, Treg frequency has prognostic implication toward the natural course and long-term outcomes of childhood chronic ITP.

• Childhood Kidney Diseases
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• 제9권1호
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• pp.83-90
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• 2005

Focal segmental glomerulosclerosis(FSGS) has been detected in approximately 10% of cases of Idiopathic nephrotic syndrome in children, and exhibits a poor response to initial steroid therapy, as well as a higher rate of progression to chronic renal failure and relapse after kidney transplantation. We describe a case of an eleven year-old boy with steroid-resistant FSGS who exhibited a response to a second trial of cyclosporin h(CsA) therapy. At the age of 26 months, this patient was diagnosed with steroid-resistant FSGS. For 9 years, he had undergone a gauntlet of therapies to induce remission; oral steroids, cyclophosphamide, methylprednisolone(mehyIPd) pulse therapy, CsA, and ibuprofen therapy. Although these therapies failed to induce remission, the patient's renal function remained In the normal range during the nine years of treatment. At the age of ten years, the patient's proteinuria decreased, and complete remission was attained with a second administration of CsA, coupled with a low dose of oral steroids. This patient continues to receive CsA without relapse. Therefore, our major concern involves the possibility of relapse after the discontinuation of CsA therapy Our findings in this case suggest that, in cases of refractory FSGS, if renal insufficiency does not emerge, aggressive therapy for the amelioration of proteinuria should be continuously pursued.

• Clinical and Experimental Pediatrics
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• 제62권3호
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• pp.85-89
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• 2019

Purpose: We compared thyroid hormone profiles in children with nephrotic syndrome (NS) during the nephrotic phase and after remission. Methods: This study included 31 pediatric NS patients. The thyroid hormone profiles included serum levels of triiodothyronine (T3), thyroxine (T4), thyroid-stimulating hormone (TSH), and free T4. Results: Of the 31 patients, 16 (51.6%) showed abnormal thyroid hormone profiles: 6 had overt hypothyroidism, 8 had subclinical hypothyroidism, and 2 had low T3 syndrome. The mean serum T3, T4, and free T4 levels in the nephrotic phase and after remission were $82.37{\pm}23.64$ and $117.88{\pm}29.49ng/dL$, $5.47{\pm}1.14$ and $7.91{\pm}1.56{\mu}g/dL$, and $1.02{\pm}0.26$ and $1.38{\pm}0.23ng/dL$, respectively; the levels were significantly lower in the NS nephrotic phase (P=0.0007, P<0.0001, and P=0.0002). The mean serum TSH levels during the nephrotic phase and after remission were $8.05{\pm}3.53$ and $4.08{\pm}2.05{\mu}IU/mL$, respectively; they were significantly higher in the nephrotic phase (P=0.0005). The urinary protein/creatinine ratio during the nephrotic phase was significantly correlated with serum T3, T4, and free T4 levels (r=-0.5995, P=0.0032; r=-0.5797, P=0.0047; r=-0.5513, P=0.0078) as well as with TSH levels (r=0.5022, P=0.0172). A significant correlation was found between serum albumin and serum T3 levels during the nephrotic phase (r=0.5385, P=0.0018) but not between serum albumin and T4, TSH, or free T4 levels. These significant correlations all disappeared after remission. Conclusion: Abnormal thyroid hormone profile findings were observed in 51.6% of pediatric patients with NS. Thyroid hormone levels normalized after remission, regardless of levothyroxine therapy.

• Journal of Gynecologic Oncology
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• 제29권6호
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• pp.89.1-89.8
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• 2018

Objective: Highly effective chemotherapy for patients with low-risk gestational trophoblastic neoplasia (GTN) is associated with almost a 100% cure rate. However, 20%-30% of patients treated with chemotherapy need to change their regimens due to severe adverse events (SAEs) or drug resistance. We examined the treatment outcomes of second-line chemotherapy for patients with low-risk GTN. Methods: Between 1980 and 2015, 281 patients with low-risk GTN were treated. Of these 281 patients, 178 patients were primarily treated with 5-day intramuscular methotrexate (MTX; n=114) or 5-day drip infusion etoposide (ETP; n=64). We examined the remission rates, the drug change rates, and the outcomes of second-line chemotherapy. Results: The primary remission rates and drug resistant rates of 5-day ETP were significantly higher (p<0.001) and significantly lower (p=0.002) than those of 5-day MTX, respectively. Forty-seven patients (26.4%) required a change in their chemotherapy regimen due to the SAEs (n=16) and drug resistance (n=31), respectively. Of these 47 patients failed the first-line regimen, 39 patients (39/47, 82.9%) were re-treated with single-agent chemotherapy, and 35 patients (35/39, 89.7%) achieved remission. Four patients failed second-line, single-agent chemotherapy and eight patients (17.0%) who failed first-line regimens were treated with combined or multi-agent chemotherapy and achieved remission. Conclusions: Patients with low-risk GTN were usually treated with single-agent chemotherapy, while 20%-30% patients had to change their chemotherapy regimen due to SAEs or drug resistance. The second-line regimens of single-agent chemotherapy were effective; however, there were several patients who needed multiple agents and combined chemotherapy to achieve remission.

• Clinical and Experimental Pediatrics
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• 제50권12호
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• pp.1217-1224
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• 2007

목 적 : 소아 ALL 관해유도 시 스테로이드는 중추적인 역할을 하는 약제로 덱사메타손은 프레드니솔론에 비해 항백혈병 효과가 우수하지만 감염, 혈당 불내성, 골 괴사 등의 합병증의 가능성도 높다. 본원에서는 백혈병 세포에 대한 높은 항암효과를 고려하여 관해유도 시 덱사메타손을 사용했지만 이 약제에 의한 합병증 감소를 위해 2005년부터 시행한 치료지침(CMCPL-2005)에는 덱사메타손의 투여기간을 총 4주에서 3주로 일주일 단축해서 투여하였다. 이에 저자들은 수정한 치료지침의 관해유도율 및 주요 합병증 발생률을 기존지침(CMCPL-2001)의 성적과 비교 분석하기로 하였다. 방 법 : 가톨릭대학교 성모병원 소아과에서 2001년 1월부터 2006년 12월까지 ALL로 진단 받은 환아들의 기록을 후향적으로 조사하였다. 2001년 1월부터 2004년 12월까지 기간 동안 ALL을 진단받은 환아들은 CMCPL-2001지침 하에 관해유도를 받았고 2005년 이후에 진단받은 환아들은 CMCPL-2005지침 하에 관해유도를 받았다. 대상 환아에 대한 기초자료로 나이, 성별, 진단 시 백혈구 수, 면역표현형, 세포유전학적 특징, 그리고 위험군 분류에 대해 조사하였다. 각 CMCPL-2001 및 CMCPL-2005군의 전처치 기간 동안의 스테로이드 투여에 대한 반응을 확인하고 관해유도 결과를 분석하였다. 관해유도를 위해 입원한 기간 동안 발생한 감염, 간독성, 신경계, 위장관계, 내분비계 합병증 등은 NCI 독성 기준분류를 토대로 조사하였다. 결 과 : 총 141명이 CMCPL-2001지침으로, 88명이 CMCPL- 2005지침으로 관해유도를 받았다. 고위험군에 해당하는 10세 이상 환아, 예후 불량인자에 포함되는 염색체전위가 CMCPL-2005군에 유의하게 많았다. CMCPL-2001군에서는 총 132례(94%), CMCPL-2005군에서는 81례(92%)가 전처치 스테로이드에 양호한 반응을 보여 통계적인 차이는 없었고 관해유도 치료 후 시행한 골수검사에서도 CMCPL-2001군에서는 136례(96%), CMCPL- 2005군에서는 82례(93%)가 완전관해를 보여 동등한 치료성적을 보였다. CMCPL-2005군이 신경계, 위장관계, 심혈관계 등의 스테로이드와 연관된 합병증 없이 관해유도를 받을 확률이 CMCPL- 2001군보다 높았지만 감염성 합병증과 같은 중증합병증에 대해서는 두 군간에 차이가 없었다. 결 론 : 소아 ALL의 관해유도 치료지침에서 덱사메타손의 투여를 일주일 단축해도 기존의 4주간 스테로이드 투여방법과 동등한 관해 유도율을 확인하였으나 감염성 합병증 등 스테로이드 투여와 연관된 합병증의 감소는 없었다.

• Clinical and Experimental Pediatrics
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• 제46권10호
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• pp.1013-1018
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• 2003

목 적 : 천식 환자에서 특징적으로 존재하는 BHR의 기전으로 호산구성 기도 염증 반응이 중요시 생각되고 있다. 그러나 장기간 천식 관해 상태인 청소년에서 BHR이 지속되는 기전에 대해서는 아직 명확하지 않은 실정이다. 본 연구는 장기간 천식 관해 된 청소년에서의 BHR이 호산구성 기도 혈액내 총 호산구 증가와 혈청 ECP 농도의 증가와 연관되어 있는지 알아보고자 시행하였다. 방 법 : 서울대학교 어린이병원 소아과 외래에서 아토피성 천식으로 진단되었던 청소년 중 임상적으로 장기간 관해(최근 2년간 천식 증상이 없고 치료가 필요 없었던 경우)되었으나 BHR이 잔존하는 35명과 이들과 서로 대응하는 BHR을 갖고 있으면서 현증을 보이는 아토피 천식 청소년 35명을 대상으로 하였다. 이 두 군간의 총 호산구 수와 혈청 ECP 농도를 비교하고 각 군에서 총 호산구 수 및 혈청 ECP 농도와 $PC_{20}$ 사이에 상관 관계가 있는 지 분석하였다. 결 과 : 장기간 관해 된 군의 혈액 내 호산구 수와 혈청 ECP 농도의 평균값이 현증을 보이는 군보다 통계적으로 유의하게 낮았다($273{\pm}108$ vs. $365{\pm}178/{\mu}L$; $16.3{\pm}9.4$ vs. $26.5{\pm}15.1{\mu}g/L$, both, P<0.05). 현증을 보이는 군에서는 메타콜린 $PC_{20}$에 따라 혈액 내 호산구 수 및 혈청 ECP 농도가 각각 통계적으로 유의한 음의 상관관계를 보였으나(r=-0.385, P=0.022; r=-0.439, P=0.008) 장기간 관해 된 군에서는 유의한 상관관계를 보이지 않았다(r=-0.292, P=0.089; r=-0.243, P=0.159). 결 론 : 이와 같은 소견은 장기간 관해시의 BHR은 호산구성 염증과 연관성이 없으며 현증 천식에서의 BHR과는 다른 기전으로 설명될 수 있을 것으로 추정된다. 장기간 천식 관해 된 청소년에서 BHR이 잔존하는 기전에 대하여 정확히 밝히기 위해서 향후 BAL, 조직 검사, 유도 객담 등을 이용한 연구를 시행할 필요가 있다.

• Journal of Chest Surgery
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• 제25권10호
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• pp.1035-1040
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• 1992

Myasthenia gravis is an autoimmune disease in which the thymus gland is thought to play a central role in the pathogenesis. Thymectomy is now standard therapy, and improvements in surgical techniques, anesthesia, and respiratory care and the use of plasmapheresis have markedly reduced the operative morbidity. We experienced 7 myasthenia gravis with maximal thymectomy from November 1989. As compared with the results of previous classic transsternal thymectomy group the 25 patients who were operated from June 1979 to December 1991[Group B], excellent surgical result was obtained in maximal thymectomy group[Group A]. During follow-up period that ranged from a month to 10.7 years[mean 2 years], the remission rate for the entire group was 15.6 percent and an additional 71.9 percent had improvement[87.5 percent benefited]. In Group A, the remission rate was 42.9 percent with 100 percent of the patients benefiting from operations. But in Group B, 8 percent had remission and a total of 80 percent benefited. In both Groups, there was no operative mortality but there was one case`s mortality in Group B during follow-up period.

• Clinical and Experimental Pediatrics
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• 제54권8호
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• pp.317-321
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• 2011

Children who suffer from steroid-resistant nephrotic syndrome (SRNS) require aggressive treatment to achieve remission. When intravenous high-dose methylprednisolone fails, calcineurin inhibitors, such as cyclosporine and tacrolimus, are used as the first line of treatment. A significant number of patients with SRNS progress to end-stage renal disease if remission is not achieved. For these children, renal replacement therapy can also be problematic; peritoneal dialysis may be accompanied by significant protein loss through the peritoneal membrane, and kidney allograft transplantation may be complicated by recurrence of SRNS. Plasmapheresis and rituximab were initially used for treatment of recurrent SRNS after transplantation; these are now under consideration as rescue therapies for refractory SRNS. Although the prognosis of SRNS is complicated and unfavorable, intensive treatment in the early stages of the disease may achieve remission in more than half of the patients. Therefore, timely referral of pediatric SRNS patients to pediatric nephrology specialists for histological and genetic diagnosis and treatment is highly recommended.

• Asian Pacific Journal of Cancer Prevention
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• 제15권4호
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• pp.1681-1684
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• 2014

Objective: To investigate interferon (IFN) alpha 2 b for treating patients with JAK2V617F positive polycythemia vera (PV) and essential thrombocytosis (ET). Methods: Interferon alpha 2 b was used to treat patients with JAK2V617F positive PV and ET. In control group, hydroxyurea was used. Endpoint of study was to compare rates of hematological and molecular remission. Results: Patients in the interferon alpha 2 b group achieved higher rates of hematologic and molecular remission than patients in the hydroxyurea group, with a lower incidence of thrombosis. Conclusion: Compared with hydroxyurea, interferon alpha 2 b could reduce JAK2V617F load for patients with PV and ET, and achieve higher molecular remission, improve treatment efficacy and reduce complications.

• Journal of Korean Biological Nursing Science
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• 제10권2호
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• pp.170-175
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• 2008

Purpose: The purpose of this study was to predict treatment outcome of chemotherapy compared with and bone marrow transplantation in acute leukemia patients. Methods: We respectively reviewed the characteristics of subjects, cytokine, complete remission time and survival time of 111 patients with acute leukemia, admitted in St. Mary's hospital, between July 2007 and August 2008. Results: The complete remission rate with chemotherapy group was 70.8% and bone marrow transplantation group was 54.3% but without statistically significance. The prognostic factors related with survival is classification of acute leukemia and complete remission time. Conclusion: This study suggests a need for nursing research and nursing intervention for acute leukemia patients.