• Journal of Yeungnam Medical Science
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• v.22 no.1
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• pp.72-80
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• 2005

Background: Nasopharyngeal cancer is a rare disease with a relatively poor prognosis because it tends to be diagnosed at an advanced stage. The aim of this study was to establish the clinical characteristics of nasopharyngeal cancer. Materials and Methods: The medical records of 54 patients with nasopharyngeal cancer from January 1993 to December 2002 were reviewed retrospectively. Forty one cases were male (75.9%) and thirteen were female (24.1%). The average age was of 46.9 (range 16 to 78 years) years. The majority of patients (79.6%) were diagnosed in the advanced stage. The most common complaints were a neck mass (55.5%) and the WHO type III (53.7%) was the most frequent histological type. Results: The cumulative survival rate for a 5-year period was 46.5% and the T stage, N stage, pathologic type, and clinical stage were not significantly related to the survival rate. Sixteen of 54 (31.5%) cases presented with a distant metastasis of the bone, lung, brain, spine, and liver, and six cases (11.1%) presented with a locoregional recurrence. Conclusion: TNM staging is not appropriate for predicting survival rate of nasopharyngeal carcinoma patients. Therefore, a newer staging system, which includes new factors, is needed to predict the prognosis.

• The korean journal of orthodontics
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• v.34 no.3 s.104
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• pp.261-267
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• 2004

Nonsyndromic cleft lip and/or palate (NSCLP) is one of the most common congenital deformities and its prevalence in Far East Asia, such as within Korean and Japanese populations, is relatively high. However, in the eastern part of Europe, clefts are relatively rare situations. These ethnic differences infer a genetic background of the disease. The objective of this study was to compare the frequency of single nucleotide polymorphism (SNP) in $TGF-{\beta}3$ between Korean and Romanian cleft families. Korean cleft families samples were collected from twenty-six families (n=78) and Romanian cleft families samples were collected from eighteen families (n=41). For sequencing, the blood or saliva of the subjects was sampled. A single nucleotide polymorphism was observed in the intron 5 of $TGF-{\beta}3$ (Al8141G). The frequency of each allele was significantly different between the Korean and Romanian samples. The Ah allele was present in 18 out of 78 Korean samples (23.1%) and in 27 out of 41 Romanian samples (65.9%). The AG was present in 27 (34.6%) out of 78 Koreans and in 13 (31.7%) out of 41 Romanians. The GG was found in 33 (42.3%) Koreans and in 1 (2.4%) Romanian. The difference between the groups was significant (p<0.001). In conclusion, the frequency of observed SNP was significantly different between the two countries. SNP in $TGF-{\beta}3$ in the Korean population seemed to have a higher possibility of occurrence for nonsyndromic cleft palate than the Romanian population.

• Tuberculosis and Respiratory Diseases
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• v.59 no.2
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• pp.170-178
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• 2005

Background : Idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare but significantly imperative in inducing chronic pulmonary hypertension. Clinically, it is difficult to distinguish between IPAH and CTEPH. However, the treatment of pulmonary hypertension is different depending on the disease. The present study was performed to analyze the similarities and differences in clinical features between IPAH and CTEPH. Methods : During a nine-year period, thirty-three patients with IPAH and twenty-two patients with CTEPH were enrolled. Symptoms, physical findings, chest radiograph, electrocardiograph, pulmonary function test, echocardiograph, perfusion lung scan, right heart catheterization results were analyzed between both the groups. Results : The median age of IPAH group was 33 (6~70) years that was lower than that (52(27~80) years) of CTEPH group. Amongst the IPAH patients, there was female predominance (76 %) and there was no sex difference between the patients with CTEPH. Both the groups exhibited similarity in the results of chest radiograph, electrocardiograph, pulmonary function test, and echocardiograph. In the perfusion lung scan, all IPAH patients exhibited findings with normal (28%) or low probability (72%) of pulmonary embolism and all CTEPH patients exhibited findings with high probability of pulmonary embolism. Conclusion : Although IPAH and CTEPH bear similarities in terms of symptoms, physical signs and general investigation results, there were differences in age distribution, sex predominance and results of perfusion lung scan.

• Tuberculosis and Respiratory Diseases
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• v.44 no.4
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• pp.766-775
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• 1997

Background : Primary malignant tumors of the trachea are extremely rare entities and account for a mere 0.1 per cent of all malignancies of the respiratory tract. Because of vague localizing signs, symptoms and a usually negative routine chest film, the patients with tracheal tumors are often treated for asthma or chronic obstructive pulmonary disease for considerable period of time before correct diagnosis. Method : We have made a review of the 17 cases of primary tracheal tumors in recent 15 years. We reviewed the clinical features including history of smoking and respiratory symptoms, the official readings of initial routine chest film, the cytologic examination of sputum, the time of delay in diagnosis, and the response according to the therapeutic modalities. Results : Eight out of 9 patients with squamous cell carcinoma(SCC) were above 50 years old, five out of 6 patients with adenoid cystic carcinoma(ACC) were below 50 years old. The most common location of primary tracheal tumors was the upper one-third of trachea in 8 cases(47%). The most frequent symptoms were dyspnea in 13/17 cases(76%) and then stridor or wheezing, cough. and sputum in order. The routine chest roentgenographic examinations were not helpful to diagnose tracheal carcinoma and the cytologic examinations of sputums were helpful to diagnose tracheal carcinoma in only one case with adenocarcinoma. The mean times of delay in diagnosis of patients with sec and ACC were 5 months and 24.9 months respectively. We had bronchial asthma in 8 cases(47%) and tracheal tumors in 4 cases(23%) as initial clinical impression. Conclusion : We would like to perform more comprehensive diagnostic tools(high KVP technique, the fibroptic bronchoscopic examination, chest CT scan etc.) in patients who had the suggestive points for the tracheal tumorse(1. unexplained hemoptysis or hoarsness, 2. inspiratory wheezing or stridor, 3. wax and waning of dyspnea according to changes of position, 4. progressive asthmatics unresponsive to antiasthmatic therapy) and radical resection of tumor or external radiation therapy with curative aim as possible.

• Pediatric Infection and Vaccine
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• v.12 no.2
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• pp.149-156
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• 2005

Purpose : Acute septic arthritis and acute osteomyelitis are not rare diseases in pediatric population. But when the diagnosis is delayed or inappropriate treatments are given, permanent disabilities of joint or bone can be followed. We analysed clinical manifestations, laboratory findings, X-ray findings, causative microorganisms and antibiotic susceptibility results of the two diseases in children. Methods : During January 1992 and May 2002, we conducted a retrospective study of 103 children who were diagnosed as acute septic arthritis and acute osteomyelitis. We selected out 34 children who had positive culture results in the blood or involved sites. Results : 19 cases were diagnosed as acute septic arthritis and 15 cases were acute osteomyelitis. These diseases were most common in preschool children and next in neonates. Hip joints and tibia were the most common sites in each disease. X-ray findings showed abnormalities in 6 cases(36%) of acute septic arthritis and 7 cases(50%) of acute osteomyelitis on admission. The most common microorganism isolated from the involved sites was Staphylococcus aureus; 12 out of 14 cases in acute septic arthritis and 6 out of 13 cases in acute osteomyelitis. Conclusion : It is difficult to make a clear initial diagnosis of the two diseases. We could not find any differences between these two diseases on clinical manifestations such as fever, swelling, tenderness and limitation of movements in joint and bone. The most common microorganism was Staphylococcus aureus.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.564-569
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• 2005

Background: Thymic carcinoma is a rare malignant disease with sparse data for treatment and prognosis. We intended to investigate the prognostic factors of thymic carcinoma. Material and Method: Data of 42 patients, who were diagnosed and treated for thymic carcinoma from January of 1986 to August of 2003 were reviewed retrospectively. Influences of characteristics of patients, Masaoka stage, histologic grade, completeness of resection and adjuvant treatment on survival were evaluated. Result: There were 30 male and 12 female patients and their mean age was $52.0\pm15.7$ years old. There were 28 patients with low-grade histology and 13 patients with high-grade histology. Clinical stage according to Masaoka stage were I in 2, II in 2, III in 15 $(35.7\%)$, IVa in 10 $(23.8\%),\;and\;IVb\;in\;13\;(31\%)$ patients. Surgical resection was done in 22 patients, Complete resection was possible in 13 patients and incomplete resection was done in 9 patients. Among 20 patients without resection, 8 patients received chemotherapy, 7 patients received radiotherapy and 5 patients received combined therapy. Median survival time was $31.7\pm6.1$ months and 5 year survival rate was $28.5\%$. High grade histology (hazard ratio=3.009, $95\%\;confidence\;interval=1.178\sim7.685,$ p=0.021) and incompleteness of resection (hazard ratio=3.605, $95\%$ confidence interval= $1.1541\sim1.580$, p=0.023) were the prognostic factors of thymic carcinoma. Conclusion: In thymic carcinoma, low grade histology is a good prognostic factor and complete resection can prolong the survival of patients.

• Journal of Chest Surgery
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• v.38 no.10 s.255
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• pp.675-679
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• 2005

Background: Clinical reports on replacement of all three (AV + MV + TV) valves are rare. Material and Method: From January 1992 to December 2003, 38 patients received triple valve replacement (aortic, mitral, tricuspid) at Yonsei Cardiovascular Center. Mean age of patients was $49.5\pm10.7 (28\~69)$ years, and 24 patients $(63.1\%)$ were female. Rheumatic valve disease was the most common cause of operation (n=37). Preoperative New York Heart Association functional class were II in 4, III in 24 and IV in 10. Fifteen patients (group 1) received triple valve replacement at their first operation. Twenty three patients (group 2) received one or more operations before tricuspid valve replacement (TVR). Seven patients received tricuspid valve annuloplasty at first operation and received TVR later. Result: Six patients died at hospital after operation $(15.8\%)$ and all these patients were in group 2. All patients in group I survived and were discharged. Three patients $(9.4\%)$ died during follow up periods. Most of the survivors had improved functional class (I in 22, II in 8, III in 1, IV in 1). During follow up period, there were 4 valve related complications. The 10-year survival rate was $68.8\%$ and survival rate for free from valve related event at 10 years was $85.5\%$. Conclusion: After triple valve replacement, most patients showed improvement of symptoms. And during follow up period, valve related complications and survival were acceptable. Therefore, if indicated, triple valve replacement is recommended before the patients' conditions get worse.

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.991-997
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• 2005

Purpose : Cow's milk protein-induced enterocolitis(CMPIE) is a symptom complex of vomiting and/or diarrhea caused by delayed hypersensitivity and may result in serious complications. This study was undertaken to identify high risk factors to facilitate the early recognition of CMPIE. Methods : We reviewed the data of 101 patients, aged 15 to 45 days, admitted due to vomiting and/or diarrhea between 2003 and 2004. After excluding 13 patients absolutely breast-fed and 2 patients transferred from other hospitals with the impression of CMPIE, the 86 study subjects were divided into three groups based on the underlying etiologies; CMPIE, infectious and non-infectious group. Results : CMPIE was diagnosed in 11 patients(12.8%). On admission, failure to gain weight(P=0.003), hypoalbuminemia(P=0.003), peripheral leukocytosis(P=0.015), and metabolic acidosis(P=0.014) were more significant in the CMPIE group than in the others. Multiple logistic regression analysis showed that the independent predictors of high risks for CMPIE were failure to gain weight <10 g/day(OR, 10.25[95% CI, 1.62-65.06]) and serum hypoalbuminemia <3.5 g/dL(OR, 9.18[95% CI, 1.69-49.74]). Cow's milk challenges were performed in the 11 CMPIE patients; vomiting(81.8%), abnormal stool test(80.0%), peripheral leukocyte count and absolute neutrophil count(ANC) increase(100.0%) (P<0.05), and enteropathy(100.0%). Conclusion : CMPIE is not a rare clinical disease in early infancy. The high risk factors of CMPIE were identified as follow : failure to gain weight below 10 g/day, hypoalbuminemia on admission and a rapid decrease during admission. Cow's milk challenge test with endoscopic duodenal biopsy was helpful to confirm CMPIE.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.299-306
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• 2008

Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.

• Journal of Korean Neurosurgical Society
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• v.30 no.3
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• pp.349-357
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• 2001

Purpose : Meningeal hemangiopericytoma(M-HPC), characterized by a high local recurrency and metastatic potential, is a rare neoplasm arising from perivascular pericytes. A retrospective study was performed to identify the recurrence and extraneural metastasis in M-HPC. Materials and Methods : We reviewed the records of 31 M-HPC patients treated from 1982 through 1999 at our institution. The time to recurrence and the various parameters affecting recurrence were determined. Extreneural metastasis was also analyzed. Results : The rate of local recurrency was 38.7%(12/31). The overall average recurrence-free period(RFP) before the first recurrence was 104 months, with overall recurrence-free rates(RFRs) at 5 and 10 years after first surgery of 59.2% and 33.6%, respectively. Of the 12 patients who experienced local recurrence, 4 had recurrences 5 years later after the first surgery. Complete excision at the first operation significantly extended the average time before first recurrence from 43 to 111 months. The 5-year RFRs for the groups of complete excision and incomplete excision were 72.7% and 20.8%, respectively(p=0.0060). Although there was no statistical significance, complete excision followed by adjuvant radiotherapy of more than 50Gy extended the RFP. The 5-year RFRs for the groups of complete excision and complete excision with adjuvant radiotherapy were 70.3% and 100%, respectively(p=0.3359). Four patients(12.9%) presented one or more extraneural metastases that were developed at an average of 107 months after the first operation with the 5- and 10-year metastasis rates of 4.4% and 24.9%, respectively. Conclusions : M-HPC has a propensity to recur either locally or at distant sites after surgical resection. Complete excision is the most important factor to reduce recurrence. However, even with complete excision, adjuvant radiotherapy of more than 50Gy significantly reduces the risk of recurrence. Local and distant recurrences may occur after a prolonged disease-free interval, emphasizing the need for long-term follow-up.