• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.6 no.2
• /
• pp.129-139
• /
• 2003

Purpose: The aim of the study was to evaluate the long-term outcome of glycogen storage disease (GSD) type 1 with particular reference to hepatic adenoma and hepatocellular carcinoma, and to analyze risk factors affecting the development of hepatic adenoma in GSD type 1. Methods: Forty-three GSD type 1 patients (31 males and 12 females, mean age $13.9{\pm}6.4$ years) were analyzed retrospectively. Hepatic adenoma was detected on abdominal USG and diagnosed on histologic examination. Clinical profiles were compared between patients with hepatic adenoma (n=16) and age-matched controls without hepatic adenoma (n=16). Results: 1) Of 43 GSD type 1 patients, 16 (37.2%) had hepatic adeoma. Hepatic adenoma was detected at the age of mean $14.2{\pm}4.1$ years (range: 7.9~25.7 years). Fourteen (87.5%) adenomas were multiple at detection. 2) Comparison of the clinical profiles between adenoma group and non-adenoma group revealed that age at first introduction of uncooked cornstarch treatment was significantly late in adenoma group compared with non-adenoma group ($9.1{\pm}5.2$ years vs. $3.0{\pm}1.8$ years, p=0.003). Portocaval shunt surgery was performed in 11 (68.8%) patients in adenoma group and 3 (18.8%) in non-adenoma group (p=0.004). Hepatic adenoma developed mean $5.8{\pm}4.2$ years after shunt operation. 3) One patient was diagnosed as hepatocellular carcinoma at the age of 25.7 years. Conclusion: Hepatic adenoma is an important late complication of GSD type 1 with the risk of malignant transformation. Early introduction of cornstarch therapy with strict metabolic control is needed to prevent the development of hepatic adenoma in GSD type 1.

• Radiation Oncology Journal
• /
• v.20 no.2
• /
• pp.123-129
• /
• 2002

Purpose : The purpose of this study 띤as to determine the potential role of three-dimensional conformal radiotherapy (3D-CRT) in the treatment of primary unresectable hepatocellular carcinoma. The preliminary results on the efficacy and the toxicity of 3D-CRT are reported. Materials and Methods : Seventeen patients were enrolled in this study, which was conducted prospectively from January 1995 to June 1997. The exclusion criteria included the presence of extrahepatic metastasis, liver cirrhosis of Child-Pugh classification C, tumors occupying more than two thirds of the entire liver, and a performance status of more than 3 on the ECOG scale. Two patients were treated with radiotherapy only while the remaining 15 were treated with combined transcatheter arterial chemoembolization. Radiotherapy was given to the field including the tumor plus a 1.5 cm margin using a 3D-CRT technique. The radiation dose ranged from $36\~60\;Gy$ (median; 59.4 Gy). Tumor response was based on a radiological examination such as the CT scan, MR imaging, and hepatic artery angiography at $4\~8$ weeks following the completion of treatment. The acute and subacute toxicities were monitored. Results : An objective response was observed in 11 out of 17 patients, giving a response rate of $64.7\%$. The actuarial survival rate at 2 years was $21.2\%$ from the start of radiotherapy (median survival; 19 months). Six patients developed a distant metastasis consisting of a lung metastasis in 5 patients and bone metastasis in one. The complications related to 30-CRT were gastro-duodenitis $(\geq\;grade\;2)$ in 2 patients. There were no treatment related deaths and radiation induced hepatitis. Conclusion : The preliminary results show that 3D-CRT is a reliable and effective treatment modality for primary unresectable hepatocellular carcinoma compared to other conventional modalities. Further studies to evaluate the definitive role of the 3D-CRT technique in the treatment of primary unresectable hepatocellular carcinoma are needed.

• Journal of Hospice and Palliative Care
• /
• v.13 no.3
• /
• pp.153-160
• /
• 2010

Purpose: The purpose of the present study was to investigate needs and satisfaction on the medical services of cancer patients in Jeju Special Self-Governing Province. Methods: Total 174 cancer patients, who visited at the clinic of Jeju National University Hospital, submitted informed consent and participated in this study from July 13 to July 30, 2009. Self questionnaire was used and data were analyzed with Kolmogorov-Smirnov test, Mann-Whitney U test, ANOVA, and Kruskal-Wallis test. Results: Participants expressed the needs of most economical support (3.38 out of 4), followed by counseling of treatment plan (3.22), information of disease (3.07), and disease management except cancer (2.97). Participants were satisfied most with religious counseling (3.41), followed by nursing service support (3.39), employment counseling (3.26), and counseling for family or interpersonal relationships (3.26). The satisfaction of economical support was the lowest (1.98). Satisfaction of men was higher than women, and needs in patients who were living with children was the highest. Patients who were living alone or with children showed the lowest satisfaction about the medical services. There were no significant differences in the general characteristics, however, participants who were older than 60 years of age or had higher income showed lower needs and higher satisfaction. There were no significant differences in the medical characteristics, however, thyroid cancer patients and patients who were treated with radiation therapy or transarterial embolization showed low satisfaction. Conclusion: Cancer patients seemed to need more economical support, information of treatment or disease, and symptom management. Furthermore, there were various needs about the services, depending on family formation or economical support of patients. Therefore, it is certain that patients who were suffering from other cancers, except the 5 major cancers, needed more services. In conclusion, continuous and systemic policy to consider patient's characteristics and needs are needed in community as well as health care system.

• Radiation Oncology Journal
• /
• v.28 no.3
• /
• pp.155-165
• /
• 2010

Purpose: In order to evaluate the positional uncertainty of internal organs during radiation therapy for treatment of liver cancer, we measured differences in inter- and intra-fractional variation of the tumor position and tidal amplitude using 4-dimentional computed radiograph (DCT) images and gated orthogonal setup kilovolt (KV) images taken on every treatment using the on board imaging (OBI) and real time position management (RPM) system. Materials and Methods: Twenty consecutive patients who underwent 3-dimensional (3D) conformal radiation therapy for treatment of liver cancer participated in this study. All patients received a 4DCT simulation with an RT16 scanner and an RPM system. Lipiodol, which was updated near the target volume after transarterial chemoembolization or diaphragm was chosen as a surrogate for the evaluation of the position difference of internal organs. Two reference orthogonal (anterior and lateral) digital reconstructed radiograph (DRR) images were generated using CT image sets of 0% and 50% into the respiratory phases. The maximum tidal amplitude of the surrogate was measured from 3D conformal treatment planning. After setting the patient up with laser markings on the skin, orthogonal gated setup images at 50% into the respiratory phase were acquired at each treatment session with OBI and registered on reference DRR images by setting each beam center. Online inter-fractional variation was determined with the surrogate. After adjusting the patient setup error, orthogonal setup images at 0% and 50% into the respiratory phases were obtained and tidal amplitude of the surrogate was measured. Measured tidal amplitude was compared with data from 4DCT. For evaluation of intra-fractional variation, an orthogonal gated setup image at 50% into the respiratory phase was promptly acquired after treatment and compared with the same image taken just before treatment. In addition, a statistical analysis for the quantitative evaluation was performed. Results: Medians of inter-fractional variation for twenty patients were 0.00 cm (range, -0.50 to 0.90 cm), 0.00 cm (range, -2.40 to 1.60 cm), and 0.00 cm (range, -1.10 to 0.50 cm) in the X (transaxial), Y (superior-inferior), and Z (anterior-posterior) directions, respectively. Significant inter-fractional variations over 0.5 cm were observed in four patients. Min addition, the median tidal amplitude differences between 4DCTs and the gated orthogonal setup images were -0.05 cm (range, -0.83 to 0.60 cm), -0.15 cm (range, -2.58 to 1.18 cm), and -0.02 cm (range, -1.37 to 0.59 cm) in the X, Y, and Z directions, respectively. Large differences of over 1 cm were detected in 3 patients in the Y direction, while differences of more than 0.5 but less than 1 cm were observed in 5 patients in Y and Z directions. Median intra-fractional variation was 0.00 cm (range, -0.30 to 0.40 cm), -0.03 cm (range, -1.14 to 0.50 cm), 0.05 cm (range, -0.30 to 0.50 cm) in the X, Y, and Z directions, respectively. Significant intra-fractional variation of over 1 cm was observed in 2 patients in Y direction. Conclusion: Gated setup images provided a clear image quality for the detection of organ motion without a motion artifact. Significant intra- and inter-fractional variation and tidal amplitude differences between 4DCT and gated setup images were detected in some patients during the radiation treatment period, and therefore, should be considered when setting up the target margin. Monitoring of positional uncertainty and its adaptive feedback system can enhance the accuracy of treatments.