Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$$aureus$) and $Pseudomonas$$aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.
Purpose : To evaluate interim results in terms of failure, cosmetic results and survival after breast conserving operation and radiation therapy in early breast cancer. Material and Methods : From January 1992 through December 1997, seventy two patients with early stage 0, I and II breast cancer were treated with conservative surgery plus radiotherapy at Keimyung University Dongsan Medical Center. Age distribution was 25 to 77 years old with median age of 43. According to TNM stage, five patients had stage 0, thirty three were stage I, twenty five were IIa, and nine were IIb. Most patients underwent excision of all gross tumor and ipsilateral axillary dissection. Breast was irradiated through medial and lateral tangential fields of 6 MV photons to 50.4 Gy in 28 fractions over 5.5 weeks. We delivered a boost irradiation dose of 10 to 16 Gy in 1 to 2 weeks to excision site. Adjuvant chemotherapy was administered in forty one patients with CMF (cyclophosphamide, methotrexate, 5-fluorouracil) regimens of 6 cycles concurrently or before radiation. Cosmetic results were assessed by questionnaire to patients grading of excellent, good, fair, poor. Follow-up periods were 22 to 91 months with median 40 months. Results : Five year disease free survival rate (5YDFS) was $95.8\%$. According to stage, 5YDFS was $100\%,\;96.9\%,\;96\%\;and\;88.9\%$ in stage 0, I, IIa and IIb, respectively. Two patients had distant metastasis and one had local and distant failure. One patient with distant failure had bone and liver metastasis at 14 months after treatment and the other had lung and both supraclavicular metastasis at 21 months after treatment. Patient with local and distant failure had local recurrence on other quadrant in same breast and then salvaged with total mastectomy and chemotherapy but she died due to brain metastasis at 55 months. Complications were radiation pneumonitis in five patients (four patients of asymptomatic, one patients of symptomatic) and hand or arm edema(4 patients). Fifty nine patients answered our cosmetic result questionnaire and cosmetic results were good to excellent in fifty one patients $(86\%)$. Conclusion : We considered that conservative surgery and radiation for the treatment of early stage invasive breast cancer was safe and had excellent survival and cosmetic results. We need to assess about prognostic factors with longer follow up and with large number of patients.
Background: The radial arteries are being used more often for coronary artery bypass grafting, We tried to the endoscopic radial artery harvest to reduce the cosmetic problems and neurologic complications of the conventional open harvesting and report the techniques and early results. Material and Method: The 86 patients underwent coronary artery bypass grafting between May 2003 and April 2005 had their nondominant radial artery endoscopically removed through a 2 cm incision at the wrist. The radial pedicle was dissected and was divided at antecubial area through a 5 mm counterincision. Result: The 23 patients complained of neuralgias on territory of superficial raidal none but no one complained of neuralgias on territory of lateral antebrachial cutaneous none. There was no functional impairment of the hand. There was no wound complication except a localized hematoma. All patients were contacted by telephone after postoperative 7.9$\pm$3.6 months. The 4 patients still complained of neuralgia. All the patients were satisfied with the aesthetics of the wounds. The multidetectional tomography was done on the 66 patients for the estimation of early patency of radial artery. There were 2 cases of stenosis and a case of occlusion. Conclusion: Endoscopic radial artery harvest had no functional impairment of the hand, lesser rate of neurologic complications and outstanding aesthetics. The results of early patency of the radial artery was similar to conventional methods. Therefore, we think that endoscopic radial artery harvest is the optimal procedure.
All kinds of tissue valves must be pretreated for the inactivation of immunologic properties and the strengthening of tissue before implantation. However, the tissue valves are gradually denatured with the calcification process and they eventually lose their functions. Recent reports have shown the existence of specific calcium binding non collagenous proteins in the calcified area of implanted biomaterials. This experiment was intended to confirm the effect of pretreatment with RGDS(Arg-Gly-Asp-Ser) tetrapeptide on the calcification of subcutaneously implanted bovine pericardium in rats. RGDS tetrapeptide has the same amino acid sequence of attachment site of specific calcium binding non collagenous proteins. Material and Method: All bovine pericardial pieces were fixed with 0.6% glutaraldehyde. The pretreatments were done using 5 different methods, groupI, with normal saline for 60 minutes, groupII, with 0.5% GRSD(Gly-Arg-Scr-Asp) tetrapeptide solution for 60 minutes, group III : with 0.5% RGDS(Arg-Gly-Asp-Ser) tctrapeptide for 30 minutes, group IV ; with 0.5% RGDS for 60 minutes, and group V : with 0.5% RGDS for 120 minutes. The pretreated bovine pericardial pieces were implanted subcutaneously at the abdominal sites of rats. 30 days after the implantation, the implanted bovine pericardial tissue were examined radiologically, biochemically, and histologically to measure the severity of calcification. Result: On the radiological examination, group I ; 68.42$\pm$3.06, group II , 64.25$\pm$5.58 showed significant difference with group III: 48.00$\pm$3.57, group IV; 43.67$\pm$2.31, and group V ; 2.58$\pm$2.47(p<0.05). There was no difference between group I and II(p=0.105). On the biochemical examination, the amount of calcium in group I was , 33.09$\pm$6.59 mg, in group II ; 28.12$\pm$5.50mg, in group III ; 25.42$\pm$7.67mg, in group Ⅵ ; 20.51$\pm$5.11mg, and in group V : 15.43$\pm$4.25mg.
Kim, In-Ju;Kim, Seong-Jang;Kim, Yong-Ki;Kim, Yun-Seong;Lee, Min-Ki;Park, Soon-Kew
The Korean Journal of Nuclear Medicine
/
v.32
no.3
/
pp.266-275
/
1998
Purpose: We measured pulmonary epithelial permeability by $^{99m}Tc-DTPA$ radioaerosol clearance in patients with diabetes and correlated with the presence of microangiopathy to understand the pathophysiology of pulmonary microangiopathy and evaluate $^{99m}Tc-DTPA$ radioaerosol clearance as a diagnostic test to assess pulmonary microangiopathy. Materials and Methods: We performed $^{99m}Tc-DTPA$ radioaerosol scan in 10 normal subjects, 10 asymptomatic smokers, 20 diabetic patients without history of smoking (10 with microangiopathy, 10 without microangiopathy). $^{99m}Tc-DTPA$ clearance half-time ($T_{1/2}$) was calculated, then compared with the result of chest radiography and pulmonary function test. Results: Chest radiography and pulmonary function test were normal in all subjects. There were no significant difference of clinical or laboratory characteristics between these groups except age. The diabetic patients with microangiopathy were significantly older (p<0.05). The $T_{1/2}$ of normal subjects and asymptomatic smokers were significantly different ($65.2{\pm}23.7min$ vs $39.6{\pm}9.8min$, p<0.05). For diabetic patients with microangiopathy, the $T_{1/2}$ was $90.5{\pm}46.5min$ and significantly delayed when compared with those of normals and asymptomatic smokers (p<0.05). However, the $T_{1/2}$ of diabetic patients without microangiopathy, $70.0{\pm}12.7min$, was not significantly different from those of normals or asymptomatic smokers (p>0.05). No significant correlation was found between the $T_{1/2}$ and spirometric parameters including DLco, FVC, $FEV_1,\;FEV_1/FVC$ (%) and $FEF_{25-75%}$ in all subjects, and between the $T_{1/2}$ and duration of diabetes ;in diabetic patients. Conclusion: Eventhough the influence of age can't be excluded, delayed $^{99m}Tc-DTPA$ clearance half-time ($T_{1/2}$) in diabetic patients with microangiopathy indicates decreased pulmonary capillary permeability as one of the pathophysiologic results of pulmonary microangiopaththy. Further studies are needed in larger number of age matched control and diabetic patients to evaluate the diagnostic efficacy.
Park, Ryeong-Hwang;Kim, Min-Jung;Lee, Sang-Kyu;Park, Kwang-Woo;Jeon, Byeong-Cheol;Cho, Jeong-Hee;Yoo, Beong-Gyu;Lee, Jong-Seok
Journal of radiological science and technology
/
v.34
no.4
/
pp.341-349
/
2011
This study was to measure the patient dose difference between 3D treatment planning CT and 4D respiratory gating CT. Study was performed with each 10 patients who have lung and liver cancer for measured patient exposure dose by using SOMATON SENSATION OPEN(SIMENS, GERMANY). CTDIvol and DLP value was used to analyze patient dose, and actual dose was measured in the location of liver and kidney for abdominal examination and lung, heart and spinal cord for chest examination. Rando phantom were used for the experiment. OSLD was used for in-vitro and in-vivo dosimetry. Increasing overall actual dose in 4D respiratory gated CT-simulation using OSLD increase the dose by 5.5 times for liver cancer patients and 6 times for lung cancer patients. In CT simulation of 10 lung cancer patients, CTDIvol value was increased by 5.7 times and DLP 2.4 times. For liver cancer patients, CTDIvol was risen by 3.8 times and DLP 1.6 times. The accuracy of treatment volume could be increased in 4D CT planning for position change due to the breaths of patient in the radiation therapy. However, patients dose was increased in 4D CT than 3D CT. In conclusion, constant efforts is required to reduce patients dose by reducing scan time and scan range.
Background : Complicated exudative pleural fluid collections have traditionally been treated by either closed tube thoracostomy drainage or by open surgical drainage. Complete drainage is important in order to control pleural sepsis, restore pulmonary function, and entrapment. Recently intracavitary fibrinolytic therapy has been advocated as a method to facillitate drainage of complicated exudative pleural effusion and to allow enzymatic debridemant of the restrictive fibrinous sheets covering the pleural surface. The purpose of this study is to prospectively evaluate the effects of image-guided catheter drainage with high dose urokinase(UK) instillation in the treatment of complicated pleural effusions. Patients : Twenty complicated pleural effusion patients that poorly respond to image-guided drainage were allocated to receive UK. There were 8 pneumonia and 12 tuberculosis. Methods : Drugs were diluted in 250 mL normal saline and were infused intrapleurally through the chest tube or pig-tail catheter in a daily dose of 250,000 IU of UK. Response was assessed by clinical outcome, fluid drainage, chest radiography, pleural ultrasound and/or computed tomography. Results : The mean UK instillation time was $1.63{\pm}0.10$. The mean volume drained UK instillation was $381.3{\pm}314.4\;mL$, and post-UK was $321.6{\pm}489.5\;mL$. The follow up duration after UK therapy was mean $212.9{\pm}194.5$ days. We had successful results in 19 cases (95.0%). There were 12 pleural thickenings (60.0%), 2 markedly decreased effusions (10.0%) and 5 cases of no thickening or effusion. There was recurrence after treatment in only one patient(5%) with complicated pleural effusiondue to tuberculosis. Conclusions : Image-guided drainage with high dose UK instillation (250,000 U/day) in complicated pleural effusion is a safe and more effective method than closed thoracostomy drainage. And this management, in turn, can obviate surgery in most cases.
Background : Ever since Flexible Fiberoptic Bronchoscopy was introduced into clinical practice, it has played an important role in both diagnosis and therapy of respiratory diseases. Repeated bronchoscopic examinations is are not so uncommon. This study was designed prospectively to assess the clinical availability of the Repeated Flexible Bronchoscopy (RFB). Methods : Pre-established indications were as follows : 1) To confirm diagnosis or the cell type in proven malignancy 2) to diagnose or locate hemoptysis 3) to follow-up or confirm recurrence 4) to use in therapy. We performed RFB and analyzed the data in 156 patients during 28-month period. Results : The frequency of RFB was 23.0%. The indication for diagnosis or cell type of malignancy was 25 cases, of which 2 cases were confirmed by a third bronchoscopic examination and 3 cases by surgical procedures. Localization of the bleeding site was confirmed in 53.8%. RFB for small cell lung cancer yielded more information on residual or recurred lesion not apparent even with the CT scan in 30%. Previous cases of bronchostenosis due to endo-bronchial tuberculosis was shown to have worsened in 66.7%. Therapeutic manipulations were done in 126 cases, and bronchial suction was most common. Complications showed decreasing tendency with repeated examinations. Conclusion : The RFB for diagnosis or cell type of malignancy was useful in that comfirmation of diagnosis was possible in 85.7% of malignancy. More aggressive procedures should be employed including TBLB or TBNA. The RFB showed possible usefulness in the follow-up of patients with small cell lung cancer. For the patients with hemoptysis or endobronchial tuberculosis, the RFB did not the significance did not show significance because its results did not influence the diagnosis, therapy or clinical course.
Background : This report reviews our experience with persistent air leaks in the peumothorax that were not considered candidates for surgical treatment in order to evaluate the efficacy and risks of the OK-432 plus autoblood or OK-432 pleurodesis. Material & Methods : From March 2004 to July 2005, 8 consecutive patients who had an air leak in the pneumothorax over 5 days and had been treated with OK-432 plus autoblood or OK-432 pleurodesis. The patients were not considered candidates for surgical treatments because the chest CT findings revealed severe chronic lung disease with multiple bullae and/or bullous changes. A prolonged air leak with/without dead space was treated with either OK-432 plus autoblood or OK-432 pleurodesis. The efficacy and side effects of OK-432 pleurodesis were assessed by determining the duration of the air leak, the number of pleurodesis, the patients' symptoms, measurements of the white blood cell count and the c-reactive protein level. Results : All of eight patients were male and the mean age was $72.4{\pm}8.5$. The mean number of pleurodesis was $1.9{\pm}1.1$ and the mean duration of the air leak was $4.6{\pm}4.6days$ after pleurodesis. Side effects after pleurodesis were encountered in 7 patients, which included a chilling sensation in 7 cases, chest pain in 5 cases, headache in 3 cases, local heat sensation in 2 cases, and fever in 1 case. Leukocytosis was observed in 6 patients, and the mean of WBC count and CRP were $14500{\pm}2100$ and $21.9{\pm}11.4mg/dL$, respectively. Conclusion : Either OK-432 plus autoblood or OK-432 pleurodesis has acceptable side effects, and can be considered a treatment option for persistent air leaks in the pneumothorax that are not candidates for surgical treatment.
Nam Taek-Keun;Nah Byung-Sik;Chung Woong-Ki;Ahn Sung-Ja;Song Ju-Young
Radiation Oncology Journal
/
v.22
no.1
/
pp.25-32
/
2004
Purpose : Evaluate the efficacies and toxicities of concurrent chemoradiotherapy (CCRT), with or without intraluminal brachytherapy (ILB), using a retrospective analysis in esophageal carcinomas with respect to survival. Materials and Methods : From April 1995 to July 2001, a total of 65 patients, diagnosed with an esophageal carcinoma, were treated by CCRT, with 21 also treated by ILB after CCRT. External radiotherapy was peformed using 6 or 10 MV X-rays, with a dose range of $46.8~\69.6$ Gy (median; 59.4). The ILB was peformed using high-dose-rate brachytherapy with Ir-192. The fractionation of ILB was 3 Gy by 4, or 5 Gy by 2 fractions. Cisplatin $(75\;mg/m^2)$ was given on each first day of weeks 1, 5, 9 and 13, and 5-FU $(1,000\;mg/m^2)$ as a continuous infusion for the first 4 days of each course. Results : The median survival time of all patients was 15 months, and the 1, 2 and 3-year survival rates were 55.4, 29.2 and $20.7\%$, respectively. The 2-year survival rates of the patients with and without ILB were 33.3 and $27.3\%$, respectively (p=0.80). The 2-year survival rates of the patients with a complete, partial and no response were 44.1, 13.8 and $0\%$, respectively (p=0.02). The response to treatment was the only significant factor affecting the overall survival from a multivariate analysis. Conclusion : This study has shown that the survival outcomes of CCRT were much better than previous results with radiotherapy alone. However, the addition of ILB after CCRT showed no advantage over that of CCRT alone.
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