Rothmund-Thomson syndrome (RTS), is a rare autosomal recessive disorder, characterized by : skin photosensitivity, poikiloderma, sparse hair, sparse eyebrows/lashes, short stature, skeletal abnormalities, cataracts, and an increased risk of malignancy. Skeletal abnormalities include : dysplasia, absent or malformed bones, such as absent radii, osteopenia, and delayed bone formation. RTS is thought to result from chromosomal instability, and children with RTS are at risk of cancer. Reported cancers in children with RTS include : basal cell carcinoma, squamous cell carcinoma of the skin and osteosarcoma of bone. We report an 11 year-old boy, who presented to our institution with poikilodermatous skin change with telangiectasia and hyperpigmentation, absence of radius and thumb, and the development of osteosarcoma of the left tibia. The patient is now receiving supportive care and is receiving maintenance chemotherapy after surgery for osteosarcoma.
Kim, Hyunseong;Lee, Gihyun;Sohn, Sung-Hwa;Lee, Chanju;Kwak, Jung Won;Bae, Hyunsu
The Korean Journal of Physiology and Pharmacology
/
v.20
no.3
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pp.261-268
/
2016
$Foxp3^+$$CD25^+CD4^+$ regulatory T (Treg) cells are crucial for the maintenance of immunological self-tolerance and are abundant in tumors. Most of these cells are chemo-attracted to tumor tissues and suppress anti-tumor responses inside the tumor. Currently, several cancer immunotherapies targeting Treg cells are being clinically tested. Cisplatin is one of the most potent chemotherapy drugs widely used for cancer treatment. While cisplatin is a powerful drug for the treatment of multiple cancers, there are obstacles that limit its use, such as renal dysfunction and the development of cisplatin-resistant cancer cells after its use. To minimize these barriers, combinatorial therapies of cisplatin with other drugs have been developed and have proven to be more effective to treat cancer. In the present study, we evaluated the effect of the combination therapy using methyl gallate with cisplatin in EL4 murine lymphoma bearing C57BL/6 mice. The combinatorial therapy of methyl gallate and cisplatin showed stronger anti-cancer effects than methyl gallate or cisplatin as single treatments. In Treg cell-depleted mice, however, the effect of methyl gallate vanished. It was found that methyl gallate treatment inhibited Treg cell migration into the tumor regardless of cisplatin treatment. Additionally, in both the normal and cisplatin-treated tumor-bearing mice, there was no renal toxicity attributed to methyl gallate treatment. These findings suggest that methyl gallate treatment could be useful as an adjuvant method accompanied with cisplatin therapy.
Multiple Myeloma is malignant tumor that malignant proliferous plasma cell to originate from bone marrow invades bone multiply. Objectives : Therapy for Multiple Myeloma includes chemotherapy, radiation therapy and self-stem cell transplantation, but it has no effect for a majority of Multiple Myeloma patients. So we diagnosed it as Wei symptom, oliguria, or dysuria(遺尿) in Oriental medicine, and treated it using the Oriental medical system. Methods : The patient was treated using acupuncture, electroacupuncture, herbal acupuncture treatment, moxibustion, physical treatment and western medicine. We observed 12 kinds of symptoms in the patient when admitted to the hospital. Results : 1. Paraplegia, urination desire, voluntary urination, and other symptoms improved except for a period of complication. 2. Defecation desire, sensory disturbance of the body and lower extremities, self-made changes, maintenance of body posture, and other symptoms improved during admitting days. 3. Voluntary defecation, pains of the neck and lower extremities, and other symptoms had irregular changes during admitting days. Conclusions : This study demonstrates the necessity of having more clinical study about Mutiple Myeloma.
Tai, Cheng-Jeng;Pan, Chin-Kwun;Chen, Ching-Shyang;Hung, Chin-Sheng;Wu, Chih-Hsiung;Chiou, Hung-Yi
Asian Pacific Journal of Cancer Prevention
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v.14
no.3
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pp.1981-1984
/
2013
Objective: The optimal duration of adjuvant trastuzumab treatment in patients with HER2-positive breast cancer is not known. The aim of this study was to evaluate the efficacy of 6 months of adjuvant trastuzumab treatment in patients with stage II or III HER2-positive breast cancer. Methods: The records of patients with HER2-positive stage II or III breast cancer who were admitted to the Breast Center of Taipei Medical University Hospital and Yuan's General Hospital between 2000 and 2008 were reviewed. All patients received adjuvant trastuzumab at an initial dose of 4 mg/kg followed by a maintenance dose of 2 mg/kg/week for 22 weeks in combination with chemotherapy. Results: A total of 51 patients were included with a mean age of 46.9 years. Approximately 55% of the patients had stage III disease. The mean follow-up time from initiation of treatment was 45.2 months (range, 0.9 to 85 months). During follow-up, 46 patients (90.2%) did not experience tumor recurrence. The mean estimated disease free survival was 80.2 months. The estimated 1-, 2-, 5-, and 7-year survival rates were 97.9%, 93.1%, 93.1%, and 93.1%, respectively. The most common adverse effects were gastrointestinal symptoms (21.6%), chills (17.6%), dizziness (9.8%), and bone pain (7.8%). No cardiac or hematologic adverse events occurred. Conclusion: Adjuvant therapy with trastuzumab for 6 months resulted in a clinical benefit in patients with HER2-positive breast cancer.
The Journal of Korea Assosiation for Disability and Oral Health
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v.11
no.1
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pp.5-8
/
2015
Neuroblastoma is a common malignant tumor of the sympathetic nervous system in childhood, arises from embryonic neural crest cells. The period of tooth development is matched with peak times of diagnosis and treatment of neuroblastoma. The intensive multimodality treatment including radiotherapy and chemotherapy is used in patients with neuroblastoma has been shown to have late adverse effects and disturbances in dental development like tooth agenesis, microdontia, enamel hypoplasia and short roots. A 8-year old girl had been on medication and radiotherapy for neuroblastoma since she was 15 months old at Department Pediatrics, Chonnam National University Hospital. Oligodontia, microdontia, and short root formation was notable in clinical and radiological examination. Mobility of lower permanent incisor was detected and measured at about degree 2. Resin wire splint using mini-screw implantation on buccal alveolar bone was conducted for maintenance of mandibular incisors and alveolar bone. Excessive mobility has been eliminated and maintained well so far. Further treatment is planned for re-evaluation of mobility, preventing dental caries and regular oral hygiene management. Although we need further evaluation, this treatment could be one of alternative therapy for those who have similar dental anomalies.
Background: Having been known as a virulent disease in 1970s, cancer is now onsidered a chronic disease and 64% of cancer patients live for five years after diagnosis. Home care has gradually gained more importance and it is a great burden on the shoulders of caregivers. Caregivers have to undertake the responsibility of the cancer patient's home management, and organize care and arrange health care services according to the ever-changing condition of patients. Caregivers should be prepared for home care so they can provide accurate and complete care to patients. This descriptive study aims to investigate challenges that caregivers encounter in the home care of patients and the reasons for these challenges. Materials and Methods: The research group consisted of caregivers of outpatients in a daily treatment center in a university hospital. The research sampling consisted of 137 voluntary caregivers of patients who attended the Daily Treatment Center for control, chemotherapy or other supportive cares services between January-June, 2011. Data were collected with face-to-face interviews in the Daily Treatment Center. Ethics Committee approval was taken university hospital; caregivers and their patients were informed about the research and their approval was taken as well. Results: It was found that 54.01% of caregivers help patient's nutrition, 50.36% help medicine use, 26.28% help oral hygiene, 26.28% help to meet urinary needs and 51.82% help to change clothes, 69.34% of caregivers help to change bed sheets, 38.69% help the patient to communicate with their environment and 71.53% help to bring the patient to hospital or outside. Conclusions: This study, it was found that caregivers experience challenges due to following factors: patient nutrition, medicine use, oral and body hygiene, colostomy maintenance and stomach tube feeding, concern of dropping the patient, feeling incompetency in body temperature and fever control, fatigue, and lack of personal time.
Lee Kang Kyoo;Park Kyung Ran;Lee Jong Young;Shin Hyun Soo;Lee Chong In;Chang Woo Ick;Shim Young Hak
Radiation Oncology Journal
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v.16
no.1
/
pp.7-16
/
1998
Purpose : To evaluate survival rate and prognostic factors affecting survival of patients with esophageal cancer treated with concurrent chemoradiation. Materials and Methods : Eligibility included biopsy proven invasive carcinoma of the cervical or thoracic esophagus, confined to esophagus and mediastinum with or without regional lymph node and supraclavicular lymph node, and ECOG Performance status $H_0-H_2$. Patients received radiation therapy with 5940cGy over 7 weeks and chemotherapy, consisted of 5-FU(1000 $mg/m^2/day$ in continuous infusion for 5 days, days 1 to 5 and days 29 to 33) and mitomycin C($8mg/m^2$ intravenous bolus at day 1). After concurrent chemoradiation, maintenance chemotherapy was followed with 5-FU(1000 $mg/m^2/day$ in continuous infusion for 5 days at 9th, 13th, and 17th weeks) and cisplatin($80mg/m^2$ intravenous bolus at the first day of each cycle). Results : From November 1989 to November 1995, 44 patients were entered in this study. After treatment, complete response rate and partial response rate were $59\%$ and $41\%$. Overall 1, 2, and 5-year survivals were $59\%$, $38\%$, and $9.6\%$(median 17 months), Prognostic factors affecting survival were response to treatment and T-stage. Among 26 complete responders, there were 6 local recurrences, 3 distant recurrences, 1 local and distant recurrence, and 2 unknown site recurrences Acute and chronic complication rates with grade 3 or more were $20\%$ and $13.0\%$ and there was no treatment-related mortality. Conclusion : Concurrent chemoradiation, compared with historical control groups that treated with radiation alone, improved median survival and did not significantly increase treatment-related complications. Complete responders had longer survival duration than partial responders. Predominant failure pattern was local failure. So, efforts to improve local control should be proposed.
Purpose: The balance between synthesis and degradation of proteins plays a critical role in the maintenance of skeletal muscle mass. Mitochondrial dysfunction has been closely associated with skeletal muscle atrophy caused by aging, cancer, and chemotherapy. Polygalacin D is a saponin derivative isolated from Platycodon grandiflorum (Jacq.) A. DC. This study aimed to investigate the effects of polygalacin D on myoblast differentiation and muscle atrophy in association with mitochondrial function in in vitro and in zebrafish models in vivo. Methods: C2C12 myoblasts were cultured in differentiation media containing different concentrations of polygalacin D, followed by the immunostaining of the myotubes with myosin heavy chain (MHC). The mRNA expression of markers related to myogenesis, muscle atrophy, and mitochondrial function was determined by real-time quantitative reverse transcription polymerase chain reaction. Wild type AB* zebrafish (Danio rerio) embryos were treated with 5-fluorouracil, leucovorin, and irinotecan (FOLFIRI) with or without polygalacin D, and immunostained to detect slow and fast types of muscle fibers. The Tg(Xla.Eef1a1:mitoEGFP) zebrafish expressing mitochondria-targeted green fluorescent protein was used to monitor mitochondrial morphology. Results: The exposure of C2C12 myotubes to 0.1 ng/mL of polygalacin D increased the formation of MHC-positive multinucleated myotubes (≥ 8 nuclei) compared with the control. Polygalacin D significantly increased the expression of MHC isoforms (Myh1, Myh2, Myh4, and Myh7) involved in myoblast differentiation while it decreased the expression of atrophic markers including muscle RING-finger protein-1 (MuRF1), mothers against decapentaplegic homolog (Smad)2, and Smad3. In addition, polygalacin D promoted peroxisome proliferator-activated receptor-gamma coactivator (Pgc1α) expression and reduced the level of mitochondrial fission regulators such as dynamin-1-like protein (Drp1) and mitochondrial fission 1 (Fis1). In a zebrafish model of FOLFIRI-induced muscle atrophy, polygalacin D improved not only mitochondrial dysfunction but also slow and fast muscle fiber atrophy. Conclusion: These results demonstrated that polygalacin D promotes myogenesis and alleviates chemotherapy-induced muscle atrophy by improving mitochondrial function. Thus, polygalacin D could be useful as nutrition support to prevent and ameliorate muscle wasting and weakness.
Journal of the Korean Association of Oral and Maxillofacial Surgeons
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v.27
no.4
/
pp.314-320
/
2001
Matrix metalloproteinases have long been viewed as ideal candidates for proteinases that enables tumor cells to permeated basement membrane defenses and invade surrounding tissue. There is growing evidence that the MMPs have an expanded role, as they are important for the creation and maintenance of a microenvironment that facilitates growth and angiogenesis of tumors at primary and metastatic sites. MT-MMPs are not secreted but instead remaining attached to cell surfaces. Although not all of the MT-MMPs are fully characterized, MT-MMPs have important role in localizing and activating secreted MMPs. The MMP genes are transcriptionally responsive to a wide variety of oncogene, growth factors, cytokine, and hormones. Currently, a number of MMP inhibitors are being developed and some have reached clinical trials as anti-metastatic or anti-cancer therapies. MT1-MMP is involved in the activation of proMMP-2. MT1-MMP is significant not only as a tumor marker but as a new target for chemotherapy against cancer. The purpose of this study was to evaluate the effects of protein kinase C inhibitor(genistein) on the proliferation of HT1080 and expression of MT1-MMP mRNA. Human fibrosarcoma cell line HT1080 was cultured and divided 2 groups. The experimental group was treated with $100{\mu}M$ genistein and incubated 12h, 24h for $[3^H]-thymidine$ uptake assay and northern hybridization individually. And the control group was treated with same amount of PBS for the above procedures. $[3^H]-thymidine$ incorporation was measured with ${\beta}$ ray detector. And RT-PCR and northern blotting for MT1-MMP mRNA was performed. The results were as follows 1. $[3^H]-thymidine$ uptake was reduced in experimental group with statistical significance. 2. MT1-MMP mRNA expression was significantly reduced in experimental group. These results showed that protein kinase C inhibitor (genistein) inhibited proliferation of HT1080 and almost completely blocked transcription of MT1-MMP mRNA. So, it is possible to use the protein kinase inhibitor (genistein) as anti-metastatic and anti-proliferative agent.
Objective : The maintenance of the correction of kyphotic deformity is one of the difficult problem in tuberculous spondylitis after anterior debriment and fusion with tricortical bone graft. The goal of this study is to find out the efficacy of titanium mesh cage impacted with autogenous bone chip in tuberculous spondylitis treated with anterior intervertebral fusion. Materials and Method : Twelve patients were treated with anterior intervertebral fusion using titanium mesh cage for tuberculous spondylitis from January 1996 to June 1999. We analized the changes in the correction of kyphotic deformity, changes of ESR and CRP, fusion state and recurrence after anterior intervertebral fusion with titanium mesh cage. Results : Clinical symptoms were improved in all twelve patients without any neurologic complications. The mean kyphotic angle corrected was 7.3 degrees immediately after operation, but the loss of correction of kyphotic angle was 2.2 degrees after 3 months and 2.6 degrees after 6 months. We found that the loss of correction of kyphotic deformity occurred mainly within the first 3 months after surgery. Only one patient, suffered from acute hepatic failure after first operation and had an insufficient anti-tuberculous medication therapy, showed recurrence of tuberculous spondylitis after 6 months. The patient underwent a second operation with posterior fixation procedure with good outcome. The changes of ESR and CRP were not specifically important factor to reveal recurrence of tuberculosis of the spine in our series. Conclusion : The surgical procedure of tuberculous spondylitis using titanium mesh cage with bone chip seems to be an effective procedure to minimize loss of the correction of kyphotic deformity without any aggravating inflammatory change and recurrence with titanium mesh cage, when sufficient debridement and anti-tuberculous chemotherapy are achieved.
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