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Long-term follow-up of Fanconi anemia: clinical manifestation and treatment outcome

  • Yoon, Byung Gyu;Kim, Hee Na;Han, Ui Joung;Jang, Hae In;Han, Dong Kyun;Baek, Hee Jo;Hwang, Tai Ju;Kook, Hoon
    • Clinical and Experimental Pediatrics
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    • v.57 no.3
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    • pp.125-134
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    • 2014
  • Purpose: The aim of this study was to characterize Korean patients with Fanconi anemia (FA), which is a rare but very challenging genetic disease. Methods: The medical records of 12 FA patients diagnosed at Chonnam National University Hospital from 1991 to 2012 were retrospectively reviewed. Results: The median age at diagnosis was 6.2 years. All patients showed evidence of marrow failure and one or more physical stigmata. Chromosome breakage tests were positive in 9 out of 11 available patients. The median follow-up duration was 69.5 months. The Kaplan-Meier (KM) survival of all patients was 83.3% at 10 years and 34.7% at 20 years, respectively. Seven patients underwent 9 stem cell transplantations (SCTs). Among them, 5 were alive by the end of the study. Ten-year KM survival after SCT was 71.4% with a median follow-up of 3.4 years. All 5 patients treated with supportive treatment alone died of infection or progression at the median age of 13.5 years, except for one with short followup duration. Acute leukemia developed in 2 patients at 15.4 and 18.1 years of age. Among 6 patients who are still alive, 3 had short stature and 1 developed insulin-dependent diabetes mellitus. Conclusion: We provide information on the long-term outcomes of FA patients in Korea. A nation-wide FA registry that includes information of the genotypes of Korean patients is required to further characterize ethnic differences and provide the best standard of care for FA patients.

Long-term Clinical Outcomes and Prognostic Factors After Endovascular Treatment in Patients With Chronic Limb Threatening Ischemia

  • Jung-Joon Cha;Jong-Youn Kim;Hyoeun Kim;Young-Guk Ko;Donghoon Choi;Jae-Hwan Lee;Chang-Hwan Yoon;In-Ho Chae;Cheol Woong Yu;Seung Whan Lee;Sang-Rok Lee;Seung Hyuk Choi;Yoon Seok Koh;Pil-Ki Min;K-VIS (Korean Vascular Intervention Society) investigators
    • Korean Circulation Journal
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    • v.52 no.6
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    • pp.429-440
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    • 2022
  • Background and Objectives: Endovascular therapy (EVT) first strategy has been widely adopted for the treatment of chronic limb threatening ischemia (CLTI) patients in real-world practice. This study aimed to investigate long-term outcomes of CLTI patients who underwent EVT and identify prognostic factors. Methods: From the retrospective cohorts of a Korean multicenter endovascular therapy registry, 1,036 patients with CLTI (792 men, 68.8 ± 9.5 years) were included. The primary endpoint was amputation-free survival (AFS) defined as the absence of major amputation or death. Secondary endpoints were major adverse limb events (MALE; a composite of major amputation, minor amputation, and reintervention). Results: Five-year AFS and freedom from MALE were 69.8% and 61%, respectively. After multivariate analysis, age (hazard ratio [HR], 1.476; p<0.001), end-stage renal disease (ESRD; HR, 2.340; p<0.001), Rutherford category (RC) 6 (HR, 1.456; p=0.036), and suboptimal EVT (HR, 1.798; p=0.005) were identified as predictors of major amputation or death, whereas smoking (HR, 0.594; p=0.007) was protective. Low body mass index (HR, 1.505; p=0.046), ESRD (HR, 1.648; p=0.001), femoropopliteal lesion (HR, 1.877; p=0.004), RC-6 (HR, 1.471; p=0.008), and suboptimal EVT (HR, 1.847; p=0.001) were predictors of MALE. The highest hazard rates were observed during the first 6 months for both major amputation or death and MALE. After that, the hazard rate decreased and rose again after 3-4 years. Conclusions: In CLTI patients, long-term outcomes of EVT were acceptable. ESRD, RC-6, and suboptimal EVT were common predictors for poor clinical outcomes.

Incidence and Survival Rates among Pediatric Osteogenic Sarcoma Cases in Khon Kaen, Thailand, 1985-2010

  • Wiromrat, Pattara;Jetsrisuparb, Arunee;Komvilaisak, Patcharee;Sirichativapee, Winai;Kamsa-Ard, Supot;Wiangnon, Surapon
    • Asian Pacific Journal of Cancer Prevention
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    • v.13 no.9
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    • pp.4281-4284
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    • 2012
  • Background: Osteosarcoma is the most common bone cancer in children, responsible for a high rate of amputation and death. This is the first long-term, population-based, epidemiologic and survival study in Thailand. Objective: To study the incidence and survival rates of pediatric osteosarcoma in Khon Kaen. Method: Childhood osteosarcoma cases (0-19 years) diagnosed between 1985-2010 were reviewed. The data were retrieved from the population-based data set of the Khon Kaen Cancer Registry and medical records from Srinagarind Hospital, Faculty of Medicine, Khon Kaen University. All cases were censored until the end of April 2012. The age-standardized incidence rate (ASR) was calculated using the standard method. Survival experience was analyzed using the standard survival function (STATA 9.0) and presented with a Kaplan-Meier curve. Results: 58 cases were enrolled. The overall ASR was 14.1 per million. Males and females were equally affected. The peak incidence was for 15-19 year-olds in both sexes (ASR=10.4 per million in males and 8.5 in females). The 5-year overall survival rate was 27.6% (95% CI: 15.8-40.8%). The median survival time was 1.6 years (95% CI: 1.2-2.1). In a subgroup analysis, the patients who received only chemotherapy survived longer (5-year survival 45.7%, median survival time 4.1 years, p=0.12). Conclusion: The incidence rate for childhood osteosarcoma was slightly less than those reported for Western countries. The survival rate was also lower than reports from developed countries. Further evaluation of the treatment protocol and risk factor stratification is needed.

Thyroid Function after Postoperative Radiation Therapy in Patients with Breast Cancer

  • Wolny-Rokicka, Edyta;Tukiendorf, Andrzej;Wydmanski, Jerzy;Roszkowska, Danuta;Staniul, Boguslaw;Zembron-Lacny, Agnieszka
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.10
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    • pp.4577-4581
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    • 2016
  • Objective: The aim of this study was to assess thyroid function in breast cancer patients exposed to therapeutic external beam radiation. The focus was on possible progressive changes and any relationships between the incidence of primary hypothyroidism, the time required to become hypothyroid, and factors such as chemotherapy, hormonotherapy and immunotherapy. Materials and Methods: Seventy females undergoing 3D conformal and IMRT radiation therapy for breast cancers were enrolled in a non-randomized prospective study. The patients was divided into two groups: those after mastectomy or breast conserving surgery (BCS) were irradiated to a scar of the chest wall/breast and the ipsilateral supraclavicular and the axillary areas (supraclavicular radiotherapy group - SC-RT group - 32 patients) and the control group receiving adjuvant chest wall/breast RT only (BCT group - 38 patients).The total doses were 50.0 to 70 Gy in 5 to 7 weeks. The median follow-up term was 24 months (range, 1-40 months). Thyroid function was evaluated by measuring thyroid stimulating hormone (TSH), free thyroxine (fT4), and free triiodothyronine (fT3) levels. The minimum, maximum and mean thyroid gland doses for 20 Gy (V20) were calculated for all patients. Results: Statistically significant results were obtained for the SC-RT group. Two yearsa fter the end of RT the chance of an event was increased in 6% of the population (p=0.009) in the SC-RT group. In the BCT group no significance was noted. No statistically significant differences were found for V20, chemio-, immunotherapy and hormonotherapy or Ki67 values (p=0.12). No significant results were obtained for development of hypothyroidism and clinical factors (age, thyroid volume, treatment modalities). Conclusion: Radiotherapy is associated with a higher incidence of thyroid toxicity in breast cancer patients. Routine thyroid function monitoring should be recommended in such cases.

Trend Analysis of Gastrointestinal Cancer Incidences in Guilan Province: Comparing Rates over 15 Years

  • Atrkar-Roushan, Zahra;Kazemnejad, Anoshirvan;Mansour-Ghanaei, Fariborz;Zayeri, Farid
    • Asian Pacific Journal of Cancer Prevention
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    • v.14 no.12
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    • pp.7587-7593
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    • 2013
  • Background: Cancers of gastric and esophagus are the most frequent gastrointestinal (GI) tract cancers in Iran. This study aimed to analyze time trends of GI tract cancers in Guilan province by gender and age to provide solid scientific evidence for cancer prevention and control. Materials and Methods: The data were obtained from the Guilan Cancer Registry System and Guilan Provincial Health Center, over the 15 year period between 1997 and 2011. Crude incidence and age standardized (AS)incidence rates were calculated and annual percent change was estimated by Joinpoint software for long term trend analysis. Results: During the study period, 8,332 cases of GI malignances with a male to female ratio of 1:1.73 were registered in Guilan province. The AS rates for esophageal, gastric, colon and rectal cancers were 5.97, 14.5, 7.59 and 3.58 per 105 respectively. While the trend was declining and relatively constant for esophageal and gastric cancer, respectively, the incidence trend for colon and rectal cancers was of increase over the period of the study. Conclusions: The results indicated that the incidence of GI cancers was relatively low in Guilan province compared to neighboring provinces. An effective cancer control program including prevention measures, early detection and effective treatment needs to be implemented to reduce cancer morbidity and mortality.

Stereotactic radiotherapy of the prostate: fractionation and utilization in the United States

  • Weiner, Joseph P.;Schwartz, David;Shao, Meng;Osborn, Virginia;Choi, Kwang;Schreiber, David
    • Radiation Oncology Journal
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    • v.35 no.2
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    • pp.137-143
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    • 2017
  • Purpose: To analyze the utilization and fractionation of extreme hypofractionation via stereotactic body radiotherapy (SBRT) in the treatment of prostate cancer. Materials and Methods: Data was analyzed on men diagnosed with localized prostate cancer between 2004-2012 and treated with definitive-intent radiation therapy, as captured in the National Cancer Database. This database is a hospital-based registry that collects an estimated 70% of all diagnosed malignancies in the United States. Results: There were 299,186 patients identified, of which 4,962 (1.7%) were identified as receiving SBRT as primary treatment. Of those men, 2,082 had low risk disease (42.0%), 2,201 had intermediate risk disease (44.4%), and 679 had high risk disease (13.7%). The relative utilization of SBRT increased from 0.1% in 2004 to 4.0% in 2012. Initially SBRT was more commonly used in academic programs, though as time progressed there was a shift to favor an increased absolute number of men treated in the community setting. Delivery of five separate treatments was the most commonly utilized fractionation pattern, with 4,635 patients (91.3%) receiving this number of treatments. The most common dosing pattern was $725cGy{\times}5fractions$ (49.6%) followed by $700cGy{\times}5fractions$ (21.3%). Conclusions: Extreme hypofractionation via SBRT is slowly increasing acceptance. Currently $700-725cGy{\times}5fractions$ appears to be the most commonly employed scheme. As further long-term data regarding the safety and efficacy emerges, the relative utilization of this modality is expected to continue to increase.

What Made Her Give Up Her Breasts: a Qualitative Study on Decisional Considerations for Contralateral Prophylactic Mastectomy among Breast Cancer Survivors Undergoing BRCA1/2 Genetic Testing

  • Kwong, Ava;Chu, Annie T.W.
    • Asian Pacific Journal of Cancer Prevention
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    • v.13 no.5
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    • pp.2241-2247
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    • 2012
  • Objective: This qualitative study retrospectively examined the experience and psychological impact of contralateral prophylactic mastectomy (CPM) among Southern Chinese females with unilateral breast cancer history who underwent BRCA1/2 genetic testing. Limited knowledge is available on this topic especially among Asians; therefore, the aim of this study was to acquire insight from Chinese females' subjective perspectives. Methods: A total of 12 semi-structured in-depth interviews, with 11 female BRCA1/BRCA 2 mutated gene carriers and 1 non-carrier with a history of one-sided breast cancer and genetic testing performed by the Hong Kong Hereditary Breast Cancer Family Registry, who subsequently underwent CPM, were assessed using thematic analysis and a Stage Conceptual Model. Breast cancer history, procedures conducted, cosmetic satisfaction, pain, body image and sexuality issues, and cancer risk perception were discussed. Retrieval of medical records using a prospective database was also performed. Results: All participants opted for prophylaxis due to their reservations concerning the efficacy of surveillance and worries of recurrent breast cancer risk. Most participants were satisfied with the overall results and their decision. One-fourth expressed different extents of regrets. Psychological relief and decreased breast cancer risk were stated as major benefits. Spouses' reactions and support were crucial for post-surgery sexual satisfaction and long-term adjustment. Conclusions: Our findings indicate that thorough education on cancer risk and realistic expectations of surgery outcomes are crucial for positive adjustment after CPM. Appropriate genetic counseling and pre-and post-surgery psychological counseling were necessary. This study adds valuable contextual insights into the experiences of living with breast cancer fear and the importance of involving spouses when counseling these patients.

Human Acellular Dermis versus Submuscular Tissue Expander Breast Reconstruction: A Multivariate Analysis of Short-Term Complications

  • Davila, Armando A.;Seth, Akhil K.;Wang, Edward;Hanwright, Philip;Bilimoria, Karl;Fine, Neil;Kim, John Y.S.
    • Archives of Plastic Surgery
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    • v.40 no.1
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    • pp.19-27
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    • 2013
  • Background Acellular dermal matrix (ADM) allografts and their putative benefits have been increasingly described in prosthesis based breast reconstruction. There have been a myriad of analyses outlining ADM complication profiles, but few large-scale, multi-institutional studies exploring these outcomes. In this study, complication rates of acellular dermis-assisted tissue expander breast reconstruction were compared with traditional submuscular methods by evaluation of the American College of Surgeon's National Surgical Quality Improvement Program (NSQIP) registry. Methods Patients who underwent immediate tissue expander breast reconstruction from 2006-2010 were identified using surgical procedure codes. Two hundred forty tracked variables from over 250 participating sites were extracted for patients undergoing acellular dermis-assisted versus submuscular tissue expander reconstruction. Thirty-day postoperative outcomes and captured risk factors for complications were compared between the two groups. Results A total of 9,159 patients underwent tissue expander breast reconstruction; 1,717 using acellular dermis and 7,442 with submuscular expander placement. Total complications and reconstruction related complications were similar in both cohorts (5.5% vs. 5.3%, P=0.68 and 4.7% vs. 4.3%, P=0.39, respectively). Multivariate logistic regression revealed body mass index and smoking as independent risk factors for reconstructive complications in both cohorts (P<0.01). Conclusions The NSQIP database provides large-scale, multi-institutional, independent outcomes for acellular dermis and submuscular breast reconstruction. Both thirty-day complication profiles and risk factors for post operative morbidity are similar between these two reconstructive approaches.

Non-Randomized Confirmatory Trial of Laparoscopy-Assisted Total Gastrectomy and Proximal Gastrectomy with Nodal Dissection for Clinical Stage I Gastric Cancer: Japan Clinical Oncology Group Study JCOG1401

  • Kataoka, Kozo;Katai, Hitoshi;Mizusawa, Junki;Katayama, Hiroshi;Nakamura, Kenichi;Morita, Shinji;Yoshikawa, Takaki;Ito, Seiji;Kinoshita, Takahiro;Fukagawa, Takeo;Sasako, Mitsuru
    • Journal of Gastric Cancer
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    • v.16 no.2
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    • pp.93-97
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    • 2016
  • Several prospective studies on laparoscopy-assisted distal gastrectomy for early gastric cancer have been initiated, but no prospective study evaluating laparoscopy-assisted total gastrectomy or laparoscopy-assisted proximal gastrectomy has been completed to date. A non-randomized confirmatory trial was commenced in April 2015 to evaluate the safety of laparoscopy-assisted total gastrectomy and laparoscopy-assisted proximal gastrectomy for clinical stage I gastric cancer. A total of 245 patients will be accrued from 42 Japanese institutions over 3 years. The primary endpoint is the proportion of patients with anastomotic leakage. The secondary endpoints are overall survival, relapse-free survival, proportion of patients with completed laparoscopy-assisted total gastrectomy or laparoscopy-assisted proximal gastrectomy, proportion of patients with conversion to open surgery, adverse events, and short-term clinical outcomes. The UMIN Clinical Trials Registry number is UMIN000017155.

Efficacy of Commiphora myrrha and Honey in Primary Dysmenorrhea: A Randomized Controlled Study

  • Aneesa K, Haleema;Roqaiya, Mariyam;Quadri, Mohd Aqil
    • CELLMED
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    • v.11 no.4
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    • pp.19.1-19.8
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    • 2021
  • Background: Dysmenorrhea is the most common menstrual complaint in young women with a prevalence as high as 90% and is responsible for substantial repeated short-term absenteeism from school and work in young women. The objective of this study was to compare the efficacy of Commiphora myrrha and honey with mefenamic acid in primary dysmenorrhea. Materials and Methods: This prospective standard controlled trial was conducted at Luqman Unani Medical College Hospital and Research Center Vijayapura, India where 40 diagnosed patients of primary dysmenorrhea were randomly assigned to receive test drug (powdered Commiphora myrrha gum resin10g with 30g honey in two divided doses) or active control drug (mefenamic acid 250mg TID) for first 3days of menstruationfor two consecutive cycles. The primary outcome measure was reduction in severity of pain assessed by numerical pain rating scale (NPRS), and secondary outcome measures were improvement in quality of life (QOL) assessed by SF-36 and reduction in perceived stress score (PSS). Results: During first cycle treatment no significant difference was found in NPRS score (p=0.085) between the groups however significant difference in NPRS score (p<0.001) was seen during 2nd treatment cycle. Significant reduction (p=0.022) in the perceived stress score was noted and overall quality of life was markedly improved after treatment in both the groups. Conclusion: These data suggest that Commiphora myrrha gum resin with honey is an effective herb in reducing symptoms of primary dysmenorrhea. These results need to be confirmed by a properly designed trial with a larger sample size. Trial registration: Clinical Trial Registry India CTRI/2017/09/009596.