• Journal of Yeungnam Medical Science
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• v.13 no.1
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• pp.32-45
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• 1996

The AIDS is a serious health problem worldwide today and also in Korea. Increasing knowledge and modification of behavior by health education is an important goal of human immunodeficiency virus(HIV) prevention strategies. And school health education is an important part of health education. This study was conducted to evaluate the knowledge and attitude about AIDS in teachers and professors in Taegu City and to provide the basic data for school health education. A self-administered questionnaire was given to 1,124 teachers and professors from April to June 1992. Indeed, the majority of the teachers and professors have a lot of knowledges about AIDS, but many of them also have misconceptions. The level of knowledge about AIDS was positively associated with attitude toward infected students. The sources of knowledge about AIDS were mainly TV, newspapers and magazines, but a few of the subjects obtained their knowledge from health education programs. This study suggests that the reinforcement of health education for the teachers, professors and students he needed to provide the accurate knowledge on AIDS and adequate knowledge sources and materials for school health education on AIDS should be developed.

• 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.45 no.2
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• pp.183-191
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• 2002

Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied the cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells from two siblings and one cousin with XLA, as well as additional family members. Methods : Btk protein expression was analyzed by flow cytometry. Isolation of the coding sequence of the Btk gene was performed by amplification using the reverse transcription-polymerase chain reaction(RT-PCR) technique. Sequence alterations were screened by the single-stranded conformation polymorphism(SSCP) method and characterized by standard sequencing protocols. Results : Cytoplasmic expression of Btk protein in monocytes was not detected in three patients with XLA. In addition, Btk protein analysis clearly showed cellular mosaicism in monocytes from four obligate carriers, findings further supported by SSCP. A single base pair mutation(T to C) in Btk-exon three, which encodes the PH domain, was identified in four XLA patients. A diagnostic sequencing analysis was established to detect heterozygotic pattern in 4 carrier females. Furthermore, we found significant clinical heterogeneity in individuals with the same gene mutation. Conclusion : The implicating genetic alteration provided valuable clues to the pathogenesis of XLA in Korea and the flow cytometric analysis was suggested as a useful tool for rapid detection of XLA patients and carriers. The present study has identified a genetic mutation in the Btk coding region and demonstrated heterogeneity in clinical manifestations among patients with the same mutation. A flow cytometric analysis was found to be informative in establishing a deficiency of Btk protein in both patients and carriers and is recommended as a frontline procedure in the molecular diagnosis and work-up of XLA.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.302-310
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• 2002

Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells(PBMC) from three XLA families in Korea. Methods : Heparinized venous blood samples were collected from four XLA patients and additional family members in three unrelated XLA families. Mononuclear cells were separated from their blood and the intracellular Btk protein was characterized by a flow cytometry. The mutation analysis was performed using direct sequencing. Results : Cytoplasmic expression of Btk protein in monocytes was not detected in the patients with XLA. We observed a novel deletion and two point mutations within introns(intron 1 and intron 18) resulting in alternative splicings. In XLA family 2, a 980 bp deletion(from intron 9+191 T to intron 10-215 C) including exon 10 was found in patient P2. He was the only sporadic case in this study, because his mother and brother showed a normal Btk expression by flow cytometry. Conclusion : These identified genetic alterations support the molecular heterogeneity of Btk gene in XLA disease. Additionally, by means of flow cytometric analysis, we diagnosed three hypogammaglobulinemia patients as XLA. Advancements in diagnostic methods has facilitated a prompt and definite diagnosis of this disease.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.265-270
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• 2003

Purpose : To characterize the infants under 3 months of age with urinary tract infections(UTIs), and especially patients with bacteremia or meningitis Methods : Hospital records of all the infants under 3 months of age discharged from our hospital for 69 consecutive months with the diagnosis of initial episode of UTI were reviewed. UTI was defined when patients had fever with pyuria, and had urine culture results of ${\geq}10^5$ colony forming units/mL from a bag specimen. Patients with previously known urologic abnormality or immunodeficiency were excluded. Nosocomial infections were also excluded from the study. Results : The male:female ratio was 35 : 6. Of the urine cultures, 40(97.6%) yielded single pathogen, one yielded two pathogens. Escherichia coli was the predominant isolate from the urine. Five patients(12%) also had bacteremia. Pathogens isolated from the blood cultures were E. coli(4) and Enterococcus faecalis(1). No patient had culture-positive meningitis or cerebrospinal fluid pleocytosis. Clinical or laboratory findings between patients with and without bacteremia were not different significantly. The rate of vesicoureteral reflux(VUR) was 44%. The sensitivity of ultrasound for detection of VUR was 38%; specificity was 50%. Conclusion : Clinical and laboratory data were not helpful for identifying patients with bacteremia at the time of presentation. Consequently, blood cultures need to be obtained from all febrile infants under 3 months of age with UTIs. A large-scale study including the indication of lumbar puncture for infants with a febrile UTI and study of evaluation and treatment of infants under 3 months of age with UTIs are required.

• Pediatric Infection and Vaccine
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• v.3 no.2
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• pp.175-184
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• 1996

Purpose : This study was intended to measure seropositivities and the levels of mumps- and rubella-specific IgG of MMR vaccinees over 17 months of age in Korea. Materials and Methods : From June 1994 to April 1995 we obtained sera from visitors of well baby clinic and patients in Korea University Hospital, who were MMR vaccinees over 17 months of age and had no evidence of immunodeficiency. These 275 study population include 145 males and 130 females. Mumps- and rubella-specific IgG antibody levels were measured by ELISA. Cut-off values for seropositivity were 20 GU(Gamma Unit) in mumps and 0.17 in rubella. Results : 1) As age increased, seropositivities of mumps-specific IgG increased significantly, being 69.0% in 1.5~2 year, 75.0% in 3~4 year, 76.0% in 5~6 year, 90.0% in 7 year, 100% in 8 year, 96.9% in 9 year, 97.4% in 10 year, 97.4% in 11 year, and 96.6% in 12 year of age(p<0.001). 2) As age increased, the levels of mumps-specific IgG antibody(mean${\pm}$standard deviation, GU) increased significantly, being $64.9{\pm}66.5$ in 1.5-2 year, $117.7{\pm}126.4$ in 3~4 year, $152.3{\pm}147.1$ in 5~6 year, $194.3{\pm}168.2$ in 7 year, $258.1{\pm}190.6$ in 8 year, $193.1{\pm}130.1$ in 9 year, $225.7{\pm}119.6$ in 10 year, $220.7{\pm}114.3$ in 11 year, and $222.3{\pm}127.1$ in 12 year of age(p<0.001). There was positive correlation between age and mumps-specific antibody level (r=0.3282, p<0.001). 3) As age increased, seropositivities of rubella-specific IgG decreased significantly, being 72.4% in 1.5~2 year, 75% in 3~4 year, 72% in 5~6 year, 60% in 7 year, 44.4% in 8 year, 40.6% in 9 year, 28.2% in 10 year, 23.1% in 11 year, and 17.2% in 12 year of age(p<0.001). 4) As age increased, rubella-specific IgG decreased significantly, being $0.462{\pm}0.356$ in 1.5~2year, $0.438{\pm}0.306$ in 3~4 year, $0.287{\pm}0.179$ in 5~6 year, $0.204{\pm}0.139$ in 7 year, $0.189{\pm}0.153$ in 8 year, $0.124{\pm}0.121$ in 9 year, $0.093{\pm}0.114$ in 10 year, $0.104{\pm}0.135$ in 11 year, and $0.080{\pm}0.001$ in 12 year of age(p<0.001). There was negative correlation between age and rubella-specific IgG titer (r=-0.551, p<0.001). Conclusions : Eventhough seropositivities and the level of mumps-specific IgG increased as age increased, they are not enough to prevent mumps infection in 1.5 to 6 years of age. Seropositivities and the level of rubella-specific IgG decreased as age increased. Appropriate change in vaccine schedule may be needed to decrease the risks of mumps and rubella infection.

• Tuberculosis and Respiratory Diseases
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• v.45 no.4
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• pp.805-812
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• 1998

Background: Among the variety of opportunistic infections, pneumonia comprises the major morbidity in immunocompromised patients. Pneumocystis carnii pneumonia (PCP) and cytomegalovirus (CMV) pneumonia are common infectious illness of immunocompromised hosts. Although there are many reports regarding to the co-infection of PCP and CMV diagnosed by bronchoalveolar lavage (BAL) fluid examination, the effects of CMV co-infection on the outcome of PCP is still controversial. The purpose of this investigation is to evaluate the effects of CMV detected by BAL fluid examination on the clinical course of PCP in the immunocompromised patients other than human immunodeficiency virus infection. Method: Ten patients with PCP were enrolled and retrospective analysis of their medical records were done. HIV infected persons were excluded. The PCP was diagnosed by BAL fluid examination with Calcofluor-White staining. CMV was detected in BAL fluid by Shell-vial culture system. Chest radiographic findings were reviewed. We used Fisher's exact test and Mann-Whitney U test for statistical analysis of data. Results: The underlying disorders of patients were idiopathic pulmonary fibrosis (n=1), renal transplantation (n=4), necrotizing vasculitis (n=l), systemic lupus erythematosus (n=1), brain tumor (n=1), chronic myelogenous leukemia (n=1), unidentified (n=1). There were no difference in clinical course, APACHE III score, arterial blood gas analysis, white blood cell count, lymphocyte count, serum albumin concentration, chest radiographic findings and mortality between patients with PCP alone (n=4) and those with CMV co-infection (n=6). Univariate analysis regarding to the factors that associated with mortality of PCP were revealed that the application of mechanical ventilation (p=0.028), the level of APACHE III score (p=0.018) and serum albumin concentration (p=0.048) were related to the mortality of patients with PCP. Conclusion: The clinical course of PCP patients co-infected by CMV were not different from PCP only patients. Instead, accompanied respiratory failure, high APACHE III score and poor nutritional status were associated with poor outcome of PCP.

• Tuberculosis and Respiratory Diseases
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• v.42 no.4
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• pp.465-473
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• 1995

Background: The prevalence of pulmonary tuberculosis has decreased progressively after the control of the tuberculosis began as national control. But as diabetes, malignancy, immunodeficiency disease recently tend to be increased, the tuberculosis become to the important national health problem. So, this study was designed to observe the state and the change of the prevalence and the clinical status of pulmonary tuberculosis for recent 10 years at one women's university. Method: We retrospectively investigated the epidemiology and the clinical status of 612 patients who were registered at the Ewha Womans University Health Center by analyzing records from 1983 to 1992. Results: 1) The prevalence rate had been steadily decreased from 0.63% in 1983 to 0.11 % in 1992. The prevalence of freshman and the incidence rate according to the entrance year while in the university significantly decreased since 1989. 2) In classifying by registered source, 45.6% of students were detected by annual periodical health examination, 34.5% by entrance physical examination, 12.0% by hospital, 5.4% by health center clinic, 2.5% by reentrance physical examination, sequentially. 3) The students with past history of tuberculosis were 70(11.4%) and 61(10%) suffered from pulmonary tuberculosis. The patients with family history of tuberculosis were 142(23.3%). 4) There were 530(86.6%) with minimal disease, 79(12.9%) with moderate and only 3(0.5%) with far advanced, when classified by the severity of disease. 5) The initial symptoms were mild breathing difficulty in 30.1%, sweating in 14.9%, fatigue in 14.3%, febrile sense in 11.7%, hemoptysis in 8.2%, sequentially. 6) The duration of treatment was $10.6{\pm}3.6$ months in mild group, $14.9{\pm}5.2$ months in the moderate group(P<0.05). 7) The side reactions of the drug were GI trouble in 7.2%, hepatitis in 1.8%, skin rash in 0.8% and streptomycin side in used patients in 9.1%. Conclusion: The prevalence of pulmonary tuberculosis among the students in one women's university was significantly lower than that of university students and 20-24 year-old age group announced in tuberculosis survey on a national scale, and significantly decreased since 1989. The treatment effect was desirable in student's group managed by university health center.

• Tuberculosis and Respiratory Diseases
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• v.85 no.1
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• pp.56-66
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• 2022

Background: Because the etiologies of bronchiectasis and related diseases vary significantly among different regions and ethnicities, this study aimed to develop a diagnostic bundle for bronchiectasis in South Korea. Methods: A modified Delphi method was used to develop expert consensus statements on a diagnostic bundle for bronchiectasis in South Korea. Initial statements proposed by a core panel, based on international bronchiectasis guidelines, were discussed in an online meeting and two email surveys by a panel of experts (≥70% agreement). Results: The study involved 21 expert participants, and 30 statements regarding a diagnostic bundle for bronchiectasis were classified as recommended, conditional, or not recommended. The consensus statements of the expert panel were as follows: A standardized diagnostic bundle is useful in clinical practice; diagnostic tests for specific diseases, including immunodeficiency and allergic bronchopulmonary aspergillosis, are necessary when clinically suspected; initial diagnostic tests, including sputum microbiology and spirometry, are essential in all patients with bronchiectasis, and patients suspected with rare causes such as primary ciliary dyskinesia should be referred to specialized centers. Conclusion: Based on this Delphi survey, expert consensus statements were generated including specific diagnostic, laboratory, microbiological, and pulmonary function tests required to manage patients with bronchiectasis in South Korea.

• Pediatric Infection and Vaccine
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• v.30 no.3
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• pp.129-138
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• 2023

Purpose: Cancer incidence is known to be higher in patients with inborn errors of immunity (IEI) compared to the general population in addition to traditionally well-known infection susceptibility. We aimed to investigate cancer occurrence in patients with IEI in a single center. Methods: Medical records of IEI patients treated at Samsung Medical Center, Seoul, Korea were retrospectively reviewed from November 1994 to September 2023. Patients with IEI and cancer were identified. Results: Among 194 patients with IEI, seven patients (3.6%) were diagnosed with cancer. Five cases were lymphomas, 4 of which were Epstein-Barr virus (EBV)-associated lymphomas. The remaining cases included gastric cancer and multiple myeloma. The median age at cancer diagnosis was 18 years (range, 1-75 years). Among patients with cancer, underlying IEIs included X-linked lymphoproliferative disease-1 (XLP-1, n=3), activated phosphoinositide 3-kinase delta syndrome (APDS, n=2), and cytotoxic T-lymphocyte antigen 4 (CTLA-4) haploinsufficiency (n=2). Seventy-five percent (3/4) of XLP-1 patients, 40.0% (2/5) of APDS patients, and 50.0% (2/4) of CTLA-4 haplo-insufficiency patients developed cancer. Patients with XLP-1 developed cancer at earlier age (median age 5 years) compared to those with APDS and CTLA-4 (P<0.001). One patient with APDS died during hematopoietic cell transplantation. Conclusions: Cancer occurred in 3.6% of IEI patients at a single center in Korea. In addition to infectious complications and inflammation, physicians caring for IEI patients should be aware of the potential risk of cancer, especially in association with EBV infection.