To evaluate the clinical and pathogenetic roles of TSH receptor antibodies in autoimmune thyroid diseases, TBII were measured by TSH-radioreceptor assay methods in 352 patients with Graves' disease, 108 patients with other thyroid diseases and 69 normal persons. The normal range of TBII activity was less than 15%. The frequencies of detectable TBII in 169 patients with untreated Graves' disease, 31 patients with hyperthyroidism under treatment and 70 patients with euthyrodism under treatment were 92.4%, 87.1% and 54.3% respectively. However 12 (21.8%) out of 55 patients who have been in remission more than one year after discontinuation of antithyroid drugs treatment had detectable TBII activities in their sera. In 196 patients with untreated Graves' disease, the frequency of TBII increased by increasing size of goiter and the frequency of proptosis was significantly high in patients whose TBII activities were more than 60%. TBII activities were roughly correlated with total $T_3,\;T_4$ and free $T_4$ index but low $\gamma^2$ value(less than 0.1). In 67 patients with Graves' disease who were positive TBII before antithyroid drugs treatment, TBII activities began to decrease from the third months and it was converted to negative in 35.8% of patients at 12 months after treatment. There were no significant differences of the declining and disappearing rates of TBII activities between high dose and conventional dose groups. TBII activities were significantly increased initially (2-4 months) and then began to decrease from 5-9 months after $^{131}I$ treatment. There were two groups, one whose TBII activities decreased gradually and the other did not change untill 12 months after subtotal thyroidectomy. Although preoperative clinical and laboratory findings of both groups were not different, TBII activities of non-decreasing group were significantly higher than those of decreasing group$(74.6{\pm}18.6%\;vs\;39.2{\pm}15.2%;\;P<0.01)$. Thirty three(55.9%) out of 59 patients with Graves' disease relapsed within 1 year after discontinuation of antithyroid drugs. The positive rate of TBII at the end of antithyroid drug treatment in relapse group(n=33) was significantly higher than those in remission group (n=26) (63.6% vs 23.1%; P < 0.05). The mean value of TBII activities at the end of antithyroid drug treatment in relapse group was significantly elevated $(29.7{\pm}21.4%\;vs\;14.7{\pm}11.1%,\;P<0.05)$. Positive predictive value of TBII for relapse was 77.8%, which was not different from those of TRH nonresponsiveness(78.6%). The frequencies of detectable TBII in 68 patients with Hashimoto's thyroiditis, 10 patients with painless thyroiditis and 5 patients subacute thyroiditis were 14.7%, 20% and 0%, respectively. However in 25 patients with primary nongoitrous myxedema, 11 patients(44%) showed TBII activities in their sera. 9 out of 11 patients who had TBII activities in their sera showed high TBII activities(more than 70% binding inhibition) and their IgG concentrations showing 50% binding inhibition of $^{125}I-bTSH$ to the TSH receptor were ranges of 0.1-2.6 mg/dl. One patient who had high titer of TBII in her serum delivered a hypothyroid baby due to transplacental transfer of maternal TBII. These findings suggested that 1) TSH receptor antibodies are closely related to a pathogenetic factor of Graves' hyperthyroidism and of some patients with primary non-goitrous myxedema, 2) measurement of TSH receptor antibodies is helpful in evaluating the clinical outcome of patients with Graves' disease during antithyroid drug treatment and in predicting the neonatal transient hypothyroidism of baby delivered from primary myxedema patients. 3) there are 2 or more different types of TSH receptor antibodies in autoimmune thyroid diseases including one which stimulates thyroid by binding to the TSH receptor and another which blocks adenylate cyclase stimulation by TSH.
Journal of The Korean Society of Inherited Metabolic disease
/
v.17
no.3
/
pp.92-95
/
2017
Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.
Lee, Jun Hyun;Nam, Yoo Hee;Hur, Hoon;Jeon, Hae Myung;Kim, Wook
Journal of Gastric Cancer
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v.8
no.3
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pp.141-147
/
2008
Purpose: The aim of this study was to compare the short-term operative outcomes of laparoscopy-assisted total gastrectomy (LATG) with those of open total gastrectomy (OTG) for patients suffering with advanced upper gastric cancer. Materials and Methods: Of the 47 patients who underwent LATG with $D1+{\beta}$ or D2 lymphadenectomy from July 2004 to March 2008, 29 patients with pathologically proven advanced gastric cancer were compared with 35 patients who underwent conventional OTG during the same time period. The comparison was based on the clinicopathological characteristics, the surgical outcome, the follow-up survival and tumor recurrence. Results: The patients' age, gender and body mass index were similar between the two groups. However, there were statistically differences in tumor size ($9.2{\pm}3.9$ vs $6.1{\pm}3.6cm$, P=0.002) and the proximal resected margin ($2.1{\pm}2.0$ vs $3.6{\pm}2.1cm$ P=0.004). There was no significant difference in most of the peri- and post-operative courses such as the time to first flatus, the time to starting a solid diet and the length of the hospital stay, except for a longer operating time (289.0 vs. 361.3 minutes, P<0.001) in the LATG group. The complication rate was higher in the LATG group (13.8%) than that in the OTG group (5.7%). The mean overall survival and disease free survival times were 32 and 31 months, and 24 and 28 months, respectively, with an average 18.8 months follow-up duration. The main recurrent sites were peritoneum and lymph node in both groups. Conclusion: The early results of the current study suggest that LATG for AGC is technically feasible and it does not show any inferiorities of the postoperative outcomes as compared to those of conventional open total gastrectomy.
Jeon, Dae-Geun;Cho, Wan Hyeong;Park, Hwanseong;Nam, Heeseung
Journal of the Korean Orthopaedic Association
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v.54
no.1
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pp.37-44
/
2019
Purpose: Tumor infiltration around the knee joint or skip metastasis, repeated infection sequelae after tumor prosthesis implantation, regional recurrence, and mechanical failure of the megaprosthesis might require combined distal femur and proximal tibia replacement (CFTR). Among the aforementioned situations, there are few reports on the indication, complications, and implant survival of CFTR in temporarily arthrodesed patients who had a massive bony defect on either side of the knee joint to control infection. Materials and Methods: Thirty-four CFTR patients were reviewed retrospectively and 13 temporary arthrodesed cases switched to CFTR were extracted. All 13 cases had undergone a massive bony resection on either side of the knee joint and temporary arthrodesis state to control the repeated infection. This paper describes the diagnosis, tumor location, number of operations until CFTR, duration from the index operation to CFTR, survival of CFTR, complications, and Musculoskeletal Tumor Society (MSTS) score. Results: According to Kaplan-Meier plot, the 5- and 10-year survival of CFTR was 69.0%±12.8%, 46.0%±20.7%, respectively. Six (46.2%) of the 13 cases had major complications. Three cases underwent removal of the prosthesis and were converted to arthrodesis due to infection. Two cases underwent partial change of the implant due to loosening and periprosthetic fracture. The remaining case with a deep infection was resolved after extensive debridement. At the final follow-up, the average MSTS score of 10 cases with CFTR was 24.6 (21-27). In contrast, the MSTS score of 3 arthrodesis cases with failed CFTR was 12.3 (12-13). The average range of motion of the 10 CFTR cases was 67° (0°-100°). The mean extension lag of 10 cases was 48° (20°-80°). Conclusion: Although the complication rates is substantial, conversion of an arthrodesed knee to a mobile joint using CFTR in a patient who had a massive bony defect on either side of the knee joint to control infection should be considered. The patient's functional outcome was different from the arthrodesed one. For successful conversion to a mobile joint, thorough the eradication of scar tissue and creating sufficient space for the tumor prosthesis to flex the knee joint up to 60° to 70° without soft tissue tension.
Kim, Haeng-Beom;Lee, Ro-Min;Lee, Min-Ho;Choi, Yang-Sik;Kim, Jong-In;Lee, Yun-Ho;Lee, Jae-Dong
Journal of Acupuncture Research
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v.25
no.2
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pp.151-164
/
2008
Objectives : This study was designed to compare the effects on Korean Western Ontario and McMaster Universities Osteoarthritis Index(KWOMAC), 36-ltems Short-Form Health Survey(SF-36), Visual Analog Scale(VAS) and Nine Point Scale between 'Intramuscular Bee Venom Herbal Acupuncture' and 'Intracutaneous Bee Venom Herbal Acupuncture' in knee osteoarthritis patients for 4weeks. Methods : All the voluntary subject were diagnosed as knee osteoarthritis. Patients were randomly assigned either to two groups : Intramuscular bee venom herbal acupuncture treatment group(IM-BV group) or Intracutaneous bee venom herbal acupuncture treatment group(IC-BV group). The IM-BV group received bee venom herbal acupuncture intramuscularly on muscles closest to the pathologic site on ultrasonography. The IC-BV group received bee venom herbal acupuncture intracutaneously on $LE_{110}$, $LE_{201}$, $ST_{35}$, $ST_{36}$ and $LR_8$. Bee venom herbal acupuncture was applied twice a week for 4 weeks by an oriental medical doctor at Kyung-hee Oriental Medical Hospital. Both groups were checked of pathologic site by ultrasonography before treatment and follow up after four-week treatment. The patients were assessed by KWOMAC and SF-36 before and after four-week treatment. The changes in patients' pain was measured by VAS before treatment and after one-, two-, three- and four-week treatment. The patients' overall treatment outcome assesment was measured by Nine Point Scale after four-week treatment. Results : The results were as follows ; 1. The IM-BV group showed significant improvement on total, pain, stiffness and physical function in KWOMAC, and the IC-BV group showed significant improvement on total and physical function in KWOMAC after 4weeks compared to the pre-treatment. In KWOMAC, the IM-BV group showed more improvement on average compared to the IC-BV group, but there were no significant difference between two groups. 2. The IM-BV group showed significant improvement on VAS after one-week treatment. The IC-BV group showed significant improvement on VAS after two-week treatment. But there were no significant difference between two groups. 3. The IM-BV group showed significant improvement on physical functioning(PF) and bodily pain(BP) in SF-36, and the IC-BV group showed significant improvement on vitality(VT) and bodily pain(BP) in SF-36 after 4weeks compared to the pre-treatment. But there were no significant difference between two groups. 4. 88.2% of IM-BV group and 93.3% of IC-BV group graded their improvement as 'Fair' or better on Nine Point Scale. But there were no significant difference between two groups. Conclusions : This study suggests that in the treatment of knee osteoarthtitis patients, effects of 'Intramuscular Bee Venom Herbal Acupuncture' were not different from 'Intracutaneous Bee Venom Herbal Acupuncture' statistically. But both treatment showed effects on pain and physical function in knee osteoarthtitis patients.
Kim, Se Heui;Kim, Kyeong Sik;Lee, Jong Kyun;Choi, Jae Young;Sul, Jun Hee;Lee, Sung Kyu;Park, Young Whan;Cho, Bum Koo
Clinical and Experimental Pediatrics
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v.46
no.3
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pp.259-264
/
2003
Purpose : We have performed an analysis on patients who received Rastelli operation in our institute and reviewed their progress postoperatively. Various factors with suspected relationship to the outcome have been considered to help in future treatment and follow-up. Methods : We analyzed retrospectively 43 patients who either received Rastelli operation in Yonsei University Cardiovascular Center from March 1995 to April 1997 or who received post-procedural cardiac catheterization and follow-up echocardiography in the out-patient department after the procedure. Results : No statistically valid relationships were found between the age of the patient, their body weight, preoperative pulmonary arterial index and pressure, presence of pulmonary branchial stenosis and postoperative results. Cases with atrioventricular concordance showed lower age and body weight, and discordant cases exhibited lower ejection fraction 3 days postoperatively. Upon follow up, lower NYHA score was seen in patients with severe residual stenosis. In the group that received cardiac catheterization after the procedure, residual stenosis and right ventricular pressure measurement in echocardiography showed good correlation with the catheterization data. Conclusion : In cases where conduit insertions of the right ventricular outflow tract are required to achieve total correction in complex cardiac deformity, early operation does not seem to provide a clear risk to the patient. In patients with atrioventricular discordance, careful postoperative observation of the ventricular function seems to be needed. Also, echocardiography appears to be a sound method in follow-up of patients after the correctional procedure.
Purpose : Cerebral edema in meningitis is a potentially complication. Hypertonic saline of various concentrations are frequently used to treat cerebral edemas in meningitis. We analyzed the safety and efficacy of osmotic therapy in cerebral edema by comparison of various hypertonic saline concentrations and mannitol. Methods : The medical records of 42 patients, who were followed up in the Department of Pediatrics, Busan Medical Center, from Jan. 2002 to Oct. 2005, were analyzed retrospectively. We measured intracranial pressure, mean flow velocity, and various laboratory parameters. Results : In cerebral edema developed in meningitis, intracranial pressure and symptoms were improved in treatment of hypertonic saline and mannitol. Serial bolus infusion of 3 percent hypertonic saline resulted in the best outcome. There was not a statistically considerable difference on the mean values of the intracranial pressure gap. On transcranial doppler, mean flow velocity was increased and pulsatilty index was decreased. Laboratory findings (osmolarity, Na, Cl, pH, lactic acid, Ca) were diffenent during the treatment period as opposed to K, Hb, bicarbonate, base excess. There was not a specific form of hypertonic saline used in meningitis treatment with cerebral edema. Conclusion : The therapy for cerebral edema in meningitis remains largely empirical. Serial bolus infusion of 3 percent hypertonic saline is better than other hypertonic salines. Various concentrations and different infusion methods of hypertonic saline statistically does not influence the result of treatment. More research aimed at improving cerebral edema treatment is needed to identify new, effective forms of treatment.
Lee Sang Jin;Jung Jin Hee;Sohn Dong Suep;Cho Dai Yun
Journal of Chest Surgery
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v.38
no.1
s.246
/
pp.56-62
/
2005
Because of high morbidity and mortality, traumatic diaphragm injury remains a diagnostic challenge. In this study, we evaluate that some factors and scores can be used as predictors. Material and Method: From May 1995 to June 2003, 23 patients with traumatic diaphragm injury were enrolled. We examined the clinical features of patients. RTS, TRISS, ISS and APACHE II scores for each patient are calculated for analyzing the relationship of mortality and ICU duration. Result: The study identified 15 men $(65.2{\%})$ and 8women $(34.8{\%})$. There are right sided diaphragmatic injury in 11 patients $(47.8{\%})$, left sided in 11 $(47.8{\%})$, and both sided in 1 ($0.4{\%}$). Plain X-ray, CT, upper GI contrast study and esophagogastroscopy were used as diagnostic tools. Age, hemodynamic status, early diagnosis are not associated with outcome. As prognostic factor, RTS and ISS are associated with mortality and there was negative relationship between RTS and ICU duration (r=0.737, p=0.026). Conclusion: An early diagnosis of traumatic diaphragm injury can frequently be missed in the acute trauma setting. So high index of suspicion and a careful examination are important in multiple trauma patients. An RTS can probably be used effectively as a predictor for the severity and prognosis in patients with traumatic diaphragm injury.
This study analyses the business performance according to the business diversification of Korean mass media enterprises from year 2003 to 2006. The conclusions drawn which could be divided into five main parts are the followings: First, newspaper companies pursue unrelated diversification in various industrial areas, in order to gain maximum profit while broadcasting companies exert themselves to provide better service by diversifying the major contents. Second, overall the interviewed companies display a constant decline in profit gained from their major business area thus establishing strategies to broaden their focus on diversification of any sort. Third, the researcher completed group analysis in regard of diversification measure resulting in division of three groups. The group which had the most immense diversification range gained the highest ROE, the lowest ROE volatility, and lesser probability of risk taking. The analysis adresses the companies broadening their business areas by researching and focusing on diversification are relatively stable in terms of the profit they gain. Fourth, the middle level group in terms of sales scale, debts, enterprise history, major share rate and high ROE group carry out diversification progressively. The sales scale affects positively to diversification, while the major share rate affects negatively to diversification. Fifth, in accordance to the research, diversification overall contributes to obtainance of successful outcome. Since there was not an immense amount of studies to be referred in the media area, the researcher interviewed and did panel discussion with numerous strategists and managers who are in charge of diversification of media companies. However, collection of only 4 years of data limits the research to be considered to be a generalized study, and does not reflect time gap between business diversification and business performance. Development is required in future studies to be established regarding the media companies' specificity different to other industries, classified the media companies into media types, and consider the time gap in the diversification activities and business performance.
Purpose : Craniopharyngiomas are often accompanied by severe endocrine disorders. Although there is universal growth hormone deficiency(GHD), the resulting growth pattern is very heterogeneous. We report the growth and endocrine outcome of 44 children with craniopharyngioma, with emphasis on initial symptoms, growth before and during growth hormone(GH) treatment and spontaneous growth in spite of GHD. Methods : We performed a retrospective study of 44 children treated at our centre between 1984 and 2002. Results : About 30% of patients had symptoms suggesting endocrine disorder at diagnosis. After surgery, multiple endocrinopathies were almost universal. Before GH therapy, height velocity was $8.00{\pm}2.71cm/yr$ in the normal growth group(n=11) and $1.79{\pm}1.10cm/yr$ in the subnormal growth group(n=7) during the first year and during the second year, $6.76{\pm}2.49cm/yr$ and $2.29{\pm}1.33cm/yr$, respectively. There was no difference of body mass index(BMI) change between before and after surgery in the two groups. Height standard deviation score(SDS) was $-1.46{\pm}0.74$ in the normal growth group and $-0.43{\pm}0.97$ in the subnormal growth group. Before GH treatment height SDS was $-1.31{\pm}1.25$ and BMI was $20.46{\pm}3.60$. During GH treatment, height SDS increased to $-0.60{\pm}1.37$ in the first, and to $-0.41{\pm}1.54$ in the second year(P<0.05), but BMI did not change significantly. Conclusion : The endocrine morbidity could develop in most children with craniopharyngioma before and after the operation and should be managed properly. Although all treated patients benefit from GH therapy, further studies are necessary to find out the possible mechanism of growth regulation in normally growing children, despite GH deficient.
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