• Asian Pacific Journal of Cancer Prevention
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• v.16 no.11
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• pp.4787-4790
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• 2015

Objective: To review ovarian cancer cases in children and adolescents in Siriraj Hospital and assess the prognosis, recurrence of disease, and reproductive outcomes after treatment. Materials and Methods: A retrospective descriptive study was conducted in ovarian cancer patients 21 years and younger who had been treated at Siriraj Hospital between January 1990 and December 2009. Medical records were reviewed and relevant data were recorded. Results: A total of 48 cases met the criteria; their mean age was 16.4 years. Abdominal distension was the major symptom. 91.6% were germ cell tumors and the remaining cases were sex cord-stromal and epithelial tumors. More than half (25/48 cases) presented with stage I disease. The most common used chemotherapy regimen for germ cell tumors was BEP (bloemycin, etoposide, cisplatin). Most of patients had favorable outcomes; 46/48 cases had complete remission and retained their good health at the time of the review. We had only one recurrent case and one dead case. Ten of contacted patients had married and 3 of them had successful full-term pregnancies. Conclusions: Ovarian malignancy in children and adolescents is a rare disease. The authors reported 48 cases in 20 year-period of work. Most of them have favorable outcomes. Return of ovarian function and fertility are the topics of interest.

• Asian Pacific Journal of Cancer Prevention
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• v.13 no.10
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• pp.5237-5240
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• 2012

Objective: This observational study was to identify risk factors for vulvar cancer recurrence. Materials and Methods: In the study 107 patients with primary vulvar cancer were analyzed. Surgical treatment consisted of radical excision of the primary tumor in combination with unilateral or bilateral superficial and deep inguinofemoral lymphadenectomy through separate incisions. Patients with deeper tumor invasion >1 mm or wider than 2 cm and/or groin lymphnode metastases were referred for adjuvant radiotherapy. Those with large privary vulvar tumors received neoadjuvant radiotherapy of 30Gy followed by surgical treatment and adjuvant radiotherapy. Results: Most of patients had only primary radiotherapy to the vulva and inguinal lymph nodes and only 34.5% of patients were eligible for surgical treatment. In 5 year follow-up period 25.2% (27) patients were alive without the disease, 15.0% (16) were alive with the disease and 59.8% (64) were dead. 60.7% (65) patients experienced local recurrence and 2.8% (3) patients had distant metastases. Median survival for patients without recurrent disease was $38.9{\pm}3.2$ months and $36.0{\pm}2.6$ months with no statistically significant difference. Patients with early stage vulvar cancer had longer mean survival rates-for stage I $53.1{\pm}3.4$ months, $38.4{\pm}4.4$ months for stage II and $33.4{\pm}2.6$ and $15.6{\pm}5.2$ months for patients with stage III and stage IV vulvar cancer, respectively. The only signifficant prognostic factor predicting vulvar cancer recurrence was involvement of the midline. Conclusions: Patients having midline involvement of vulvar cancer has lower recurrence risk, probably because of receiving more aggressive treatment. There is a tendency for lower vulvar cancer recurrence risk for patients over 70 years of age and patients who are receiving radiotherapy as an only treatment without surgery, but tendency for higher risk of recurrence in patients with multifocal vulvar cancer.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.92-96
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• 1997

The prognosis of Behfet's disease characterized by recurrent orogenltal ulcers and ocular and skin lesions depends upon the complications in the central nervous system, the gastrointestinal tract And the vascular system. Cardiac involvement, especially aortic regurgitation, is quite uncommon and hemodynamic instability is usually treated with ope heart surgery. But serious postoperative complications had been reported in many cases, which are prosthetic valve detachment, paravalvular leakage, conduction disturbance, and false aneurysm. Many efforts to prevent the complications have been made such as application of cryopreseved homograft. We have described an experience of root replacement with homograft in d 39 year-old male patient for prosthetic valve detachment because of Behfet's aorlitis with a review of the literatures regarding treatment, complication, and prognosis.

• Journal of Chest Surgery
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• v.26 no.1
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• pp.32-35
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• 1993

During the period from September 1989 through December 1992, 118 cases of coronary arterial bypass graft were performed at Department of Cardiothoracic Surgery, Asan Medical Center. Twenty-one of these had history of recent or remote percutaneous transluminal coronary angioplasty. They consisted of 13 males[age,58.7 + 5.4 years] and 8 females[age, 63.6 + 2.8years] with the mean age of 60.6. History of old myocardial infarction was noted in 24%[5/21] of the patients and congestive heart failure in 2 cases. The angina by type of presentation is unstable in all of the patients. The patterns of involvement of coronary arterial disease were left main disease[1], single vessel disease[5], double vessel involvement[10], and triple vessel involvement[5]. We performed 4 cases of single bypasses, 7 cases of double, 8 cases of triple, and 2 cases of quadruple bypasses. Total of 51 grafts[LIMA:12, RSVG:39] were inserted in 21 cases with average of 2.4 grafts per patient. The methods of myocardial protection were cold blood cardioplegia[8 cases], intermittent aortic occlusion[11], and continuous coronary perfusion with local coronary sharing[2]. There were no operative or late death. The only cardiac complication was 1 case of low cardiac output required IABP. The other complications were 1 case of sternal wound infection and 1 case of postoperative bleeding required reoperation. And there was no case of perioperative myocardial infarction. Postoperatively, 3 cases of recurrent angina were detected at 5, 7, and 18months after surgery. One of them was managed successfully with repeat PTCA[who was recurred 18 months postoperatively], and the other two with medication. I conclude that we can approach the patients more aggressively with PTCA, because of our acceptable operative risks.

• Advances in pediatric surgery
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• v.14 no.1
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• pp.27-36
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• 2008

Fundoplication is a common surgical procedure for gastroesophageal reflux disease (GERD). Recently the procedure has been performed with increased frequency laparoscopically. The aim of this study is to review our 11 years experience with fundoplication in infants and children. From October 1994 to December 2005, 59 fundoplications in 55 patients were performed at Sungkyunkwan University Samsung Medical Center. Medical records and laboratory results of these children were retrospectively reviewed for sex, age, symptoms and signs, coexisting disease, diagnostic methods, treatment modalities and length of operative time. Open fundoplication was performed in 41 cases and laparoscopic fundoplication in 18 cases. Simultaneous gastrostomy was done in 27 cases. Recurrent GERD symptom occurred in four patients (7.2 %) within 2 years after first fundoplication and all 4 patients had re-do fundoplication. There were no intra- and immediate post-operative complications. Gastrointesitnal symptoms were the most common indication for fundoplication in neurologically normal patients. The most frequent diagnostic studies were upper gastrointestinal series (76.3 %) and 24 hour esophageal pH monitoring (78.2 %). Fundoplication had been increased since 2004 and mostly done laparoscopically. In conclusion, our 11 years' practice of open and laparoscopic fundoplication indicates that both approaches are safe and effective in the treatment of GERD for infants and children.

• Journal of Chest Surgery
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• v.25 no.5
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• pp.511-517
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• 1992

From January 1988 to December 1990, 18 adult patients with aortic disease underwent surgical repair using hypothermia and total circulatory arrest. The age at operation ranged from 17 years to 64 years[mean 45.2$\pm$10.7 years]. We disease entities included aortic dissection in 12, aortoannuloectasia in 3 and thoracic aortic aneurysm in 3 cases. Partial cardiopulmonary bypass via femoral vessels along with surface cooling was used upon the induction of deep hypothermia[18~20oC]. Modified Bentall operation was performed in 7 cases, ascending aorta replacement in 6, graft interposition in descending thoracic aorta in 3 and others in 2 cases. The circulatory arrest was maintained for periods of 2 minutes to 86 mimutes[mean 34.7$\pm$5.0 minutes]. Overall hospital mortality was 27.8%[5/18]: brain damage was responsible for the death of 2 patients. 4 patients out of 13 survivors experienced postoperative neurologic dysfunction, which was proved to be self-limited except one case showing left hemiparesis. 12 patients were followed up postoperatively with the mean follow-up period 22.7$\pm$10.1 months. There was no death. No new neurologic problems were observed during follow-up period. All but one patient showing recurrent dissection and aortic regurgitation are in exellent clinical condition. These clinical data suggests that the principle of deep hypothermia and total circulatory arrest can be applied rather safely in adult patients, especially in the treatment of patients with aortic disease, it can be a valuable adjunct with better clinical results.

• Korean Journal of Transplantation
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• v.31 no.4
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• pp.157-169
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• 2017

Regulatory T cells (Treg) naturally rein in immune attacks, and they can inhibit rejection of transplanted organs and even reverse the progression of autoimmune diseases in mice. The initial safety trials of Treg against graft-versus-host disease (GVHD) provided evidence that the adoptive transfer of Treg is safe and capable of limiting disease progression. Supported by such evidence, numerous clinical trials have been actively investigating the efficacy of Treg targeting autoimmune diseases, type I diabetes, and organ transplant rejection, including kidney and liver. The limited quantity of Treg cells harvested from peripheral blood and subsequent in vitro culture have posed a great challenge to large-scale clinical application of Treg; nevertheless, the concept of CAR (chimeric antigen receptor)-Treg has emerged as a potential resolution to the problem. Recently, two CAR-T therapies, tisagenlecleucel and axicabtagene ciloleucel, were approved by the US FDA for the treatment of refractory or recurrent acute lymhoblastic leukemia. This approval could serve as a guideline for the production protocols for other genetically engineered T cells for clinical use as well. The phase I and II clinical trials of these agents has demonstrated that genetically engineered and antigen-targeting T cells are safe and efficacious in humans. In conclusion, both the promising results of Treg cell therapy from the clinical studies and the recent FDA approval of CAR-T therapies are paving the way for CAR-Treg therapy in clinical use.

• Archives of Craniofacial Surgery
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• v.24 no.5
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• pp.211-217
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• 2023

Background: Oral cavity cancers, the second most common type in India, are responsible for 10% of the overall cancer burden. With a recurrence rate of 30% to 40% and a 5-year survival rate of 50%, these malignancies account for substantial morbidity and mortality. Despite advances in treatment modalities, survival rates following treatment completion have not improved significantly. The present study aimed to establish specific epidemiological and pathological factors responsible for recurrence after treatment completion in buccal mucosa cancers. Methods: A retrospective analysis of the data of 116 patients treated for biopsy-proven cancers of the buccal mucosa was undertaken 1 year after treatment completion. Factors such as age, sex, education, lymphovascular invasion, extranodal extension (ENE), perineural invasion, depth of invasion, and pathological margin status were compared between patients who presented with recurrence and those who did not. Statistical significance was set at p< 0.05. Results: Of the 116 patients, 40 (34.5%) developed a recurrent disease within 1 year. The mean age of the study population was 43.3 years, and males constituted 91.4% of the included patients. Ipsilateral buccal mucosa was the commonest site of disease recurrence. Neck node metastasis, ENE, and margins of resection < 5 mm were significantly related to the recurrence of disease. However, surprisingly, lymphovascular invasion, perineural invasion, and depth of invasion > 10 mm did not show statistically significant associations. Conclusion: Neck node metastasis, ENE, and margins of resection < 5 mm were the histopathological factors associated with recurrence in cancers of the buccal mucosa.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.2
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• pp.108-113
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• 2017

Moyamoya disease is a disorder in which certain arteries in the brain are constricted. Blood flow can be blocked by the constriction and blood clots. The patients frequently experience transient ischemic attacks (TIA), cerebral hemorrhage, or may not experience any symptoms at all. It is reported that they have a higher risk of recurrent stroke and a distinct underlying pathophysiology. A 3-year-8-month old boy with moyamoya disease experienced cerebral infarctions five times, and he underwent a cerebrovascular anastomosis surgery four years ago. He showed swallow disturbance, general delayed development, hemiplegia, and strabismus. Also he had hypocalcified teeth with or without multiple caries lesions in all dentitions. Dental treatment under general anesthesia using sevoflurane was performed due to his lack of cooperation. Moyamoya disease is associated with various medical conditions requiring a thoughtful deliberation and a careful examination before and during dental treatment. Pain and anxiety control during dental treatment is important because hyperventilation induced by crying has been seen to trigger TIA. Both isoflurane and sevoflurane are commonly used in patients with MMD, but dynamic autoregulation is better preserved during sevoflurane than isoflurane anesthesia. So sevoflurance general anesthesia may be recommendable to manage dental patients having multiple caries with moyamoya disease.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.2
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• pp.69-76
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• 2017

Citrin deficiency is an autosomal recessive disorder caused by mutations in the SLC25A13 gene on chromosome 7q21.3, and a type of urea cycle disorder that causes hyperammonemia. Although neonatal intrahepatic cholestasis and adult-onset type II citrullinemia, a type of citrin deficiency, have been described well in many articles for several decades, failure to thrive and dyslipidemia caused by citrin deficiency (FTTDCD), the other type of citrin deficiency, has been only identified recently. There was previously no case report about FTTDCD in Korea. Patients with FTTDCD could present with loss of appetite, fatigue, failure to thrive, hypoglycemia, hypercitrullinemia, dyslipidemia, and an increased lactate/pyruvate ratio. Routine evaluation may not reveal the cause of hypoglycemia caused by citrin deficiency. We recently had a case that presented with recurrent hypoglycemia in a 30-month-old boy. Chemistry profiling, urine organic acid analysis, plasma acylcarnitine analysis, and hormone studies indicated values within the normal range or non-specific findings. Mutation analysis to identify the cause of hypoglycemia identified the subject as a compound heterozygote carrying each of the c.852_855del ($p.Met285Profs^*2$), and c.1177+1G>A mutant alleles. We report here on this unusual case of citrin deficiency presenting with FTTDCD for the first time in Korea.