• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.410-416
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• 2006

Purpose : Ciliary abnormalities of the respiratory system usually accompany recurrent or persistent respiratory diseases such as paranasal sinusitis, bronchiectasis, rhinitis, and/or otitis media, since they cause certain derangements in ciliary cleaning activities. This disease is usually inherited by autosomal recessive trait, but may also be found to be acquired or transient in rare cases after heavy exposure to pollutants, cigarette smoking or severe infection. We performed this study in children with frequently recurrent or persistent respiratory diseases to clarify if the ciliary abnormalities are preceding factors. Methods : We enrolled 17 children with suspected respiratory ciliary abnormalities. The indications for evaluation of ciliary ultrastructure were recurrent or persistent respiratory infections. Children with immunologic abnormalities were excluded. From August 2000 to July 2003, we performed a biopsy on nasal mucosa and examined the structure of ciliary status by using an electron microscope. Results : Of the subjects, there were seven males and 10 females, aged 2 to 10 years. Out of the 17 subjects, 12 cases of chronic paranasal sinusitis, nine chronic coughs, nine frequent upper respiratory infections, seven cases of recurrent otitis media, four cases of recurrent pneumonia, and four cases of bronchial asthma were found. Out of the 17 cases on which histologic examinations were conducted, four cases showed pathologic findings, including one case of inner dynein arm defect, one of microtubular transposition, one of supernumerous tubules, and one singlet, respectively. Conclusion : It is essential for differential diagnosis and effective treatment to identify the abnormalities of ultrastructure of nasal cilia in children with symptoms of frequently recurrent or persistent respiratory diseases, if immunodeficiency or respiratory allergy could be excluded.

• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1132-1138
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• 2005

Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.

• Journal of Chest Surgery
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• v.41 no.6
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• pp.695-702
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• 2008

Background: The choice between a bioprosthetic and a mechanical valve is an important decision in cardiac valve surgery, and the durability of the tissue valve is a major decision factor. We retrospectively evaluated the midterm results of bioprosthetic valve replacement in the mitral position. Material and Method: The subjects were all patients who had undergone mitral bioprosthesis replacement between July 1989 and August 200.7. Among the 216 patients, there were 236 surgical cases. The mean age was $63{\pm}15$ years, and the male to female ratio was 1 : 3. We retrospectively analyzed hospital and outpatient records such that the total follow-up duration amounted to 760.2 patient-years, and the mean follow-up duration was $41.9{\pm}40.7$ months (range $0{\sim}212$ months). Result: Early death occurred in 18 patients (8.3%), and 13 of these underwent concomitant cardiac procedures. The survival rate after 5 years was $79.9{\pm}3.5%$, and the survival rate after 8 years was $65.5{\pm}5.5%$, while freedom from structural valve deterioration (SVD) was $96.2{\pm}2.2%$ at 5 years and $85.9{\pm}5.3%$ at 8 years. Freedom from reoperation was $90.6{\pm}1.7%$ at 5 years and $90.4{\pm}4.2%$ at 8 years, while freedom from reoperation for SVD was $98.1{\pm}1.2%$ at 5 years and $92.3{\pm}4.1%$ at 8 years. On multivariate analysis of preoperative risk factors, small valve size (between 25mm and 27mm) was a significant risk factor for reoperation, and low LV ejection fraction (<40%) was a significant risk factor for SVD and mortality. Conclusion: Survival and freedom from reoperation for SVD in mitral bioprosthesis replacement had acceptable midterm results, but freedom from SVD Was relatively low. In particular, since SVD increased sharply at the eighth postoperative year, frequent follow-up and echocardiograms around that time will be helpful for the early detection of SVD. It will be necessary to conduct further studies involving long-term follow-up and more patients.

• Journal of Gastric Cancer
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• v.7 no.4
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• pp.213-218
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• 2007

Purpose: Laparoscopy-assisted distal gastrectomy (LADG) is gaining wider acceptance for the treatment of early gastric cancer. However, firm evidence supporting the long-term outcome after LADG for gastric cancer is unknown. This study compared long-term quality of life after LADG versus an open distal gastrectomy (ODG) for early gastric cancer. Methods: This study included 29 patients who underwent LADG and 57 patients who underwent ODG for the treatment of stage I gastric cancer. Quality of life was evaluated based on the Korean version of EORTC QLQ-C30 (version 3.0) and EORTC QLQ-STO22 one year after surgery. All patients underwent a Billroth II gastrectomy for stage I gastric cancer between January 2003 and December 2004. Results: A total of 86 (58%) out of 154 patients responded to the questionnaire. Demographic features showed no difference between the two groups of patients for age, sex, depth of invasion, lymph node metastasis except for tumor size and the number of retrieved lymph nodes. The mean score for global health status was not statistically different (LADG, $60.3{\pm}20.4$ vs ODG, $57{\pm}20.6$; P=0.413). The total score of 21 items related to stomach cancer (EORTC QLQ-STO22) also was not statistically different (LADG, $68.9{\pm}64.9$ vs ODG, $94.5{\pm}97.3$; P=0.340). Conclusion: Based on the results of the Korean version of EORTC QLQ-C 30 (version 3.0) and EORTC. QLQ-STO22, LADG does not seem to have any long-term benefit over ODG on 'quality of life'.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.875-881
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• 2006

Purpose : The aims of this study were to analyze the clinical characteristics of children with Kikuchi's disease(KD) at a medical center and to investigate the etiologic role of human herpesvirus 8(HHV 8) or Epstein-Barr virus(EBV) in children with KD. Methods : Twenty six children who were diagnosed as KD between Jan. 1998 and Dec. 2005 were included. Medical records were reviewed on the clinical characteristics of children with KD. Follow up data were collected by chart review and telephone contact. Polymerase chain reaction(PCR) was performed in order to detect HHV 8 DNA, and in situ hybridization(ISH) was perfomed in order to detect EBV RNA from 20 lymph node tissues. Results : There were 15 girls and 11 boys with a mean age of 13 years. Posterior cervical lymph nodes were involved in 72 percent(18/25) of the patients. Extracervical lymphadenopathy was associated in one patient. Fever was an associated symptom in 31 percent(8/26) of the patients. Leukopenia was observed in six (46 percent) patients. The cervical lymphadenopathy usually resolved spontaneously within 6 months. Only one patient had a recurrence of lymphadenopathy with fever during follow-up. No children with KD in our series developed systemic lupus erythematosus. HHV 8 DNA was not amplified by nested PCR in any of the cases, and all cases were negative for EBV RNA by ISH. Conclusion : KD should be differentiated as a cause of cervical lymphadenopathy in children. HHV 8 and EBV may not play major causative roles in KD in children.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.292-297
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• 2006

Purpose : In medulloblastoma, craniospinal radiation therapy combined with chemotherapy improves the prognosis of tumors but results in significant endocrine morbidities. We studied the endocrine morbidity, especially growth pattern changes. Methods : The medical records of 37 patients with medulloblastoma were reviewed retrospectively for evaluation of endocrine function and growth. We performed the growth hormone stimulation test in 16 patients whose growth velocity was lower than 4 cm/yr. Results : The height loss was progressive in most patients. The height standard deviation score (SDS) decreased from $-0.1{\pm}1.3$ initially to $-0.6{\pm}1.0$ after 1 year(P<0.01). Growth hormone deficiency(GHD) developed in 14 patients. During the 2 years of growth hormone(GH) treatment, the improvements of height gain or progressions of height loss were not observed. Twelve patients(32.4 percent) revealed primary hypothyroidism. One of six patients diagnosed with compensated hypothyroidism progressed to primary hypothyroidism. Primary and hypergonadotropic hypogonadism were observed in two and one patients respectively. There was no proven case of central adrenal insufficiency. Conclusion : Growth impairment developed frequently, irrespective of the presence of GHD in childhood survivors of medulloblastoma. GH treatment may prevent further loss of height. The impairment of the hypothalamic-pituitary-gonadal and hypothalamic-pituitary-thyroidal axis is less common, while central adrenal insufficiency was not observed.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.973-979
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• 2002

Purpose : Recently, a number of rapid antigen detection tests have been available to diagnose group A streptococcal pharyngotonsillitis. The purpose of this study was to determine the sensitivity, specificity and consistency of the two rapid antigen detection tests. Methods : Among the patients who visited our clinic from November 2001 to February 2002, 61 patients who had clinical findings of pharyngeal erythema or edema, pharyngeal exudates and soft palatine petechiae were enrolled in our study. A total of 61 patients were tested with rapid antigen detection tests and throat culture. BD $LINK2^{TM}$ Strep A(Becton, Dickinson & Company, U.S.A.) and $QuickVue^{(R)}$ $In-Line^{TM}$(Quidel Corporation, U.S.A.) were selected for rapid antigen detection tests. Results : Of the 61 patients tested, 22 patients were confirmed as group A streptococcal pharyngotonsillitis by throat culture. The BD $LINK2^{TM}$ Strep A had a sensitivity of 81.8% and a specificity 89.7%. The positive and negative predictive values were 81.8% and 89.7%, respectively. The $QuickVue^{(R)}$ $In-Line^{TM}$ had a sensitivity of 77.3% and a specificity of 100%. The positive and negative predictive values were 100% and 88.6%, respectively. The kappa values of BD $LINK2^{TM}$ Strep A and $QuickVue^{(R)}$ $In-Line^{TM}$ were 0.72 and 0.81, respectively. Conclusion : In addition to high sensitivity, specificity and consistency, both kits are easy to use and simple to interpret, and therefore have the potential to be used with backup throat culture for diagnosis of acute pharyngotonsillitis.

• Pediatric Infection and Vaccine
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• v.17 no.2
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• pp.91-100
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• 2010

Purpose : Recurrent otitis media (ROM) is usually defined as ${\geq}$3 distinct and well-documented episodes within 6 months or ${\geq}$4 episodes within 12 months. ROM is sufficiently important to warrant consideration of chemoprophylaxis, tympanostomy tube placement. There also is evidence that children with ROM are at risk for both hearing loss and speech delay. However, studies of ROM have been notably insufficient. In addition, even though environmental, racial, and sociocultural differences can affect risk factors, few studies have been conducted with regard to recurrent otitis media in Korea. Methods : This study was conducted from July 2009 to January 2010 with infants and children who were younger than 60 months old, who visited the out-patient clinics at Han-Il General Hospital and Kyunghee University Hospital. Data were collected by interview using a pre-formed sheet. Among a total of 892 infants and children, 457 were excluded, and the remaining 435 were allocated to 104 with ROM and 331 as a control group. Results : Attendance at daycare centers (P<0.001, OR=2.85), allergic rhinitis (P=0.026, OR=2.32), past history of bronchiolitis (P=0.003, OR=2.33), and low socioeconomic status (P=0.005, OR=2.00) were found to have a close significant correlation with ROM. Risk factors such as sex, having a sibling, breast-feeding, use of pacifiers, atopy, pneumococcal vaccination, influenza vaccination, smoking of parents, and indoor smoking are not relevant. Conclusion : Attendance at daycare centers, allergic rhinitis, past history of bronchiolitis, and low socioeconomic status have been identified as risk factors for ROM.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1982.05a
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• pp.11.2-12
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• 1982

The major advancement in phonosurgery due to recent development of laryngomicrosurgery enabled more accurate diagnosis and treatment of patient with voice disorders. Among large proportion of voice disordered patients, prominent linear furrow running parallel along the free edge of vocal cord extending from the vocal process to anterior commissure can be seen as well as incomplete closure during phonation. These cases were illustrated and coined as sulcus vocalis by Salvi in 1901, since then other similar paper was reported in Europe and Japan, but has not been reported in Korea. The exact etiology and therapeutic methods of sulcus vocalis has not been elaborated. At Department of Otolaryngology of Yonsei University College of Medicine a series of voice analysis were performed among those 35 patients with sulcus vocalis visited to Vocal Dynamics Laboratory from May, 1981 to March, 1982. Following is the result of clinical statistical investgation and therapeutic modality. 1) The incidance of sulcus vocalis among 290 patients with voice disorder visited to Vocal Dynamics Laboratory was approximately 12%(35 cases). 2) Onset of this voice disorder was most frequent among patient under 10 year-old groups; 19 cases (54%) followed by second decade, third decade groups in decreasing frequency respectably. 3) The etiology of sulcus vocalis was mostly unknown. The sequelae after measle (4 cases) and severe upper respiratory infection (3 cases) and congenital deformity (2 cases) were the possible causes of sulcus vocalis. 4) These patients were involved bilaterally in 25 cases (71%), left side only in 8 cases (23%) and right side only in 2 cases (6%). 5) Almost all patients complained hoarseness and 7 patients were suffering from chronic laryngitis. 6) In aerodynamic analysis, Maximal Phonation Time was decreased in 20 cases (57%), Phonation Quotient was increased in 22 cases (63%) and Mean Air Flow Rate was increased in 23 cases (66%). 7) Among them, 33 cases were analyzed with stroboscopy. The findings were as follows; incomplete glottic closure during phonation in 31 cases (93%), regular vocal cord movement in whole cases, asymmetric cord movement in 4 cases (12%), decreased amplitude in 5 cases (21%) and small mucosal wave in 24 cases (73%). 8) Intracordal Teflon injection in 5 cases and Sulcusectomy in 1 cases were performed as therapeutic management, however, the therapeutic results were not effective except one case with Teflon injection.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.288-291
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• 2007

Background: Myxoma makes up close to 50% of adult primary cardiac tumors, and this mainly occurs in the left atrium, and rarely in the right atrium or ventricle. The patients clinically present with symptoms of hemodynamic obstruction, embolization or constitutional changes. Diagnosis is currently established most appropriately with 2-D echocardiography. Surgical resection of myxoma is a safe and effective treatment, Material and Method: We reviewed our clinical experience in the diagnosis and management of 57 cases of cardiac myxoma that were seen over a 20-year period from July 1984 to July 2004. Result: The mean age of the patients was $53.5{\pm}14.0$ years (range: 12 to 76 years). There were 38 (67%) females and 19 (33%) males. The preoperative symptoms included dyspnea on exertion in 27 patients, palpitation in 4, chest pain in 9 and syncopal episode in 4. The diagnosis was made by echocardiography alone in 51, and by combination of echocardiography, CT and angiography in 6. The tumor attachment sites were the interatrial septum in 50, the mital valve annulus in 3 and the left atrial wall in cases, The tumor was excised successfully via biatriotomy in 33 (58%), left atriotomy in 15 (26%), the septal approach via right atriotomy in 3, Inverted T incision in 3 and the extended septal approach in 3. The follow-up time ranged from 1 to 229 months (mean follow-up: $84.0{\pm}71.3$ months). There were no early and late deaths and no recurrence during the follow-up period except for follow-up loss in 5 patients. Conclusion: It's concluded that excision of cardiac myxoma is curative and the long-term survival is excellent. Immediate surgical treatment was indicated because of the high risk of embolization or of sudden cardiac death. Radical tumor excision may prevent recurrences.