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Malignancy in Patients With Inborn Errors of Immunity Beyond Infectious Complication: Single Center Experience for 30 Years

  • Doo Ri Kim;Kyung-Ran Kim;Hwanhee Park;Joon-sik Choi;Yoonsun Yoon;Sohee Son;Hee Young Ju;Jihyun Kim;Keon Hee Yoo;Kangmo Ahn;Hee-Jin Kim;Eun-Suk Kang;Junhun Cho;Su Eun Park;Kihyun Kim;Yae-Jean Kim
    • 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.

The causative organisms of pediatric empyema in Korea (소아 농흉 원인균에 대한 다기관 연구(1999. 9-2004. 8))

  • Yum, Hye-yung;Kim, Woo Kyung;Kim, Jin Tak;Kim, Hyun Hee;Rha, Yeong Ho;Park, Yong Min;Sohn, Myung Hyun;Ahn, Kang Mo;Lee, Soo Young;Hong, Su Jong;Lee, Hae Ran
    • Clinical and Experimental Pediatrics
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    • v.50 no.1
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    • pp.33-39
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    • 2007
  • Purpose : In spite of medical advances, empyema is a serious complication of pneumonia in children. Vaccination practices and antibiotic prescribing practices promote the change of clinical manifestations of empyema and causative organisms. So we made a nationwide clinical observation of 122 cases of empyema in children from 32 hospitals during the 5 year period from September 1999 to August 2004. Methods : Demographic data, and clinical information on the course and management of empyema patients were collected retrospectively from medical records in secondary and tertiary hospitals in Korea. Results : One hundred twenty two patients were enrolled from 35 hospitals. The most frequent age group was 1-3 years, accounting for 48 percent of all cases. The male to female sex ratio was 1.2:1. The main symptoms were cough, fever, respiratory difficulty, lethargy and chest pain in order of frequency. Hematologic findings on admission revealed decreased hemoglobin levels ($10.4{\pm}1.6g/dL$) and increased leukocyte counts ($16,234.3{\pm}10,601.8/{\mu}L$). Pleural fluid obtained from patients showed high leukocyte counts ($30,365.8{\pm}64,073.0/{\mu}L$), high protein levels ($522.3{\pm}1582.3g/dL$), and low glucose levels ($88.1{\pm}523.5mg/dL$). Findings from pleural fluid cultures were positive in 80 cases(65.6 percent). The most common causative agent was Streptococcus pneumoniae. The majority of patients were treated with antibiotics and closed drainage. Some patients needed open drainage (16.4 percent) or decortication (3.3 percent). The mean duration of hospitalization was $28.6{\pm}15.3days$. Conclusion : We analyzed childhood empyema patients during a period of 5 years in Korean children. The most frequent age group was 1-3 years and the most common causative agent was Streptococcus pneumoniaeiae. The majority of patients were treated with antibiotics and close drainage.

Community Dynamics of Phytoplankton and Bacteria as Affected by Physicochemical Environmental factors in Hoeya Dam Reservoir (회야댐 저수지에서 물리 ${\cdot}$ 화학적 환경요인에 따른 식물플랑크톤과 세균 군집의 변화)

  • Kim, Dae-Kyun;Choi, Ae-Ran;Lee, Hye-Kyeong;Kwon, O-Seob;Kim, Jong-Seol
    • Korean Journal of Ecology and Environment
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    • v.37 no.1 s.106
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    • pp.26-35
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    • 2004
  • We investigated the effect of physicochemical environmental factors on the community dynamics of phytoplanktons and bacteria at the Hoeya Dam Reservoir, a drinking water reservoir for Ulsan city. Water samples were collected and analyzed every two to four weeks at three sites along the reservoir from April to October, 2001. During the study period, the Secchi depths were between 0.4 and 3.5 m. At the surface layer of water column, temperature ranged 10.2 ~ $32.0^{\circ}C$, pH 7.3${\sim}$9.6, dissolved oxygen 5.5 ${\sim}$ 12.4 mg $L^{-1}$, $BOD_5$ 0.8 ${\sim}$ 5.0 mg $L^{-1}$, $COD_{Mn}$ 3.7 ${\sim}$ 10.0 mg $L^{-1}$, and Chl-a 8.9 ${\sim}$ 60.9 mg $m^{-3}$. At the bottom layer, temperature varied 7.2 ${\sim}$ $28.9^{\circ}C$, pH 7.1 ${\sim}$ 9.3, dissolved oxygen 0.6 ${\sim}$ 9.7 mg $L^{-1}$, $BOD_5$ 0.8 ${\sim}$ 4.5 mg $L^{-1}$, $COD_{Mn}$ 3.9 ${\sim}$ 10.0 mg $L^{-1}$, and Chl-a 4.3 ${\sim}$ 81.9 mg $m^{-3}$. The numbers of phytoplanktons were 7.4${\pm}10^2{\sim}2.6{\pm}10^5$ cells $mL^{-1}$ at surface and 2.5${\pm}10^2{\sim}2.4{\pm}10^4$ cells $mL^{-1}$ at bottom, and were positively correlated with water temperature and Chl- a concentration. Genus Stephanodiscus and genus Oscillatoria dominated on April and on May, respectively. Cyanobacterial blooms of Aphanizomenon, Microcystis, Anabaena were observed from June to early September, and thereafter Stephanodiscus and Aulacoseiral dominated again. Total microbial counts ranged 1.73${\pm}10^4{\sim}1.68{\pm}10^5$ cells $mL^{-1}$, and were positively correlated with water temperature and phytoplankton counts at surface water. Heterotrophic plate counts (HPCs) ranged 30${\sim}4.1{\pm}10^3$ CFU $mL^{-1}$, and were positively correlated with $BOD_5$ and $NO^3\;^-$-N concentration at bottom water. Unlike the total microbial counts, the numbers of fecal coliforms and fecal streptococci as well as HPCs were higher at the bottom than the surface layer and were highest at the upper a site among the three sampling sites. Since the concentrations of fecal coliforms and streptococci were still high at the bottom of site c, where intake for water treatment plant is located, it appeared that special management of water treatment processes may be needed especially after strong rainfall.

Oestrogenic Activity of Parabens In Vitro Estrogen Assays (에틸, 프로필, 이소프로필, 부틸, 이소부틸 파라벤의 In Vitro 검색시험 연구에서의 내분비독성)

  • Lee Sung-Hoon;Kim Sun-Jung;Park Jung-Ran;Jo Eun-Hye;Ahn Nam-Shik;Park Joon-Suk;Hwang Jae-Woong;Jung Ji-Youn;Lee Yong-Soon;Kang Kyung-Sun
    • Journal of Food Hygiene and Safety
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    • v.21 no.2
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    • pp.100-106
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    • 2006
  • The use of underarm and body care cosmetics with oestrogenic chemical excipients (particularly the parabens) and the hypothesized association with breast cancer incidence, particularly in women. It is noted that the type of cosmetic product is irrelevant (e.g. antiperspirant/deodorant versus body lotion, moisturizers or sprays versus creams) and attention must focus on issues of actual exposure to chemicals through continued dermal application of body care products and the endocrine/hormonal activity and toxicity of the chemicals in the formulations. To evaluate the estrogenic activities of parabens such as ethylparaben, butylparaben, propylparaben, isobutylparaben and isopropylparaben, we used recombinant yeasts containing the human estrogen receptor [Saccharomyces cerevisiae ER+LYS 8127], human breast cancer MCF-7 cell lines and human estrogen receptor ${\alpha}\;and\;{\beta}$. In E-screen assays, isopropylparaben is the most estrogenic paraben, and in ER competition assay, isobutylparaben is the most estrogenic paraben. We evaluated isopropylparaben was most active in the recombinant yeast assay, followed by propylparaben, ethylparaben, isobutylparaben and butylparaben. Results from this study demonstrate that parabens are observed in human endocrine system. Therefore, we have shown that the parabens is induced the estrogenic activities similar to $17{\beta}$-estradiol and Bisphenol-A.

A Survey on the Dietary Behavior of High School Students -About Regularity of Meal and Number of Meal Per Day- (남녀 고등학생의 식생활태도에 관한 조사 -식사의 규칙성과 1일 식사횟수에 대하여-)

  • Kim, Geum-Ran;Kim, Mi-Jung
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.40 no.2
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    • pp.183-195
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    • 2011
  • This study was conducted to investigate dietary behavior patterns of high school students. As for regularity of meal, female students were significantly more regular than those of the male students in a day. They answered 'between 4th and 6th grades in elementary school' as 'the time for formation about number of meal intake'. As for a highly influential meal, males were higher answered 'lunch (41.0%)' while females were higher answered 'breakfast (39.8%)'. About 'number of meals per day by the grade', they ate 3 times per day mostly. As for the time for formation about number of meal intake: 'before 4th to 6th in elementary school'> 'before elementary school'. In the result of regularity of meal and general characteristics, a family of 5 was higher in regularity and those with pocket money showed lower regularity in meal. As for the person who prepares a meal, mothers prepared meals regularly. Also, higher parents age and education level resulted in more regularity in meals. In number of meals per day and general characteristics, they were eating 3 times; moreover, this trend was evident as parents' age and education level and the household income was higher. Students answered generally regularity in meal in family where parents' jobs were administrative assistant (father job (56.9%)) and housewife (mother (56.9%). In the formation time of meal intake number and general characteristics, they answered order 'middle school'> 'before elementary school'. A highly influential meal, they answered as the highest 'lunch' (37.6%). This study may provide information on dietary behavior of high school students, suggesting that nutrition education or counseling can improve food habits and develop positive behavior.

Assessment of Demand and Use of Fresh-Cut Produce in School Foodservice and Restaurant Industries (학교급식 및 외식업체에서의 신선편이 농산물 사용실태 및 요구도 평가)

  • Sun, Shih-Hui;Kim, Ju-Hee;Kim, Su-Jin;Park, Hye-Young;Kim, Gi-Chang;Kim, Haeng-Ran;Yoon, Ki-Sun
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.39 no.6
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    • pp.909-919
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    • 2010
  • The purpose of this study was to investigate the demand and use of fresh-cut produce in school foodservice and restaurant industries. The subjects of this survey study were 200 school nutritionists and 70 cooks or managers in the restaurant industry nationwide. The data were collected by means of self-administered or e-mail questionnaires. Data analysis was completed using the SPSS window (ver. 12.0) program including frequency, $\chi^2$-test and t-test. Survey questions assessed the general characteristic of respondents, and the supply, use, and demand of fresh-cut produce in school foodservice and restaurant industries. Over 74% of the subjects have used fresh-cut produce. Most of the school foodservice (84.0%) kept fresh-cut produce for one day, while restaurant industry (28.3%) kept them up to three days. The nutritionists of school foodservice and managers of restaurant industry considered origin and date of production as the most important factor, respectively, when fresh-cut produce were being used. Fresh-cut root vegetable, such as potato and carrot was used mostly. The main reason not to use the fresh cut produce was due to the distrust of the fresh-cut produce safety in school foodservice and cost in restaurant industry. The main problem in fresh-cut produce use was the need of rewashing (29.9%) in school foodservice and irregular size (39.0%) in restaurant industry. These results indicate that the quality standard and size specification must be prepared with production guideline of safe fresh-cut produce.

Perception of University Students on Nutrition Information According to Food & Nutrition Labeling Systems in Family Restaurant (패밀리 레스토랑의 영양표시제도 시행에 따른 대학생들의 영양정보에 관한 인식 연구)

  • Yang, Jung-Hwa;Heo, Young-Ran
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.42 no.12
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    • pp.2068-2075
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    • 2013
  • The purpose of this study is to investigate the perception of university students on nutritional information according to food and nutrition labeling systems. A total of 310 customers, who visited family restaurant, were surveyed by a self-recorded questionnaire from March 2006 to April 2011. A total of 286 respondents were surveyed; of the respondents, 108 were males and 178 were females. Two surveys were conducted on the perception of the respondent's health: once in 2006 and once in 2011. According to these surveys, 63.6% and 54% of respondents perceived themselves as unhealthy, respectively. When ordering a meal, respondents were more concerned with price rather than taste, nutrition, new menu items, and food presentation. Compared with 2006, in 2011 more respondents felt that family restaurants provided enough nutritional information and practical use of that information to their customers. When surveyed, respondents felt that the total calories played a significantly higher role in ordering food than foods with higher nutritional values. There was a significant increase in satisfaction with the current nutrition labeling system; in 2006, $2.87{\pm}0.99$, and 2011, $3.35{\pm}0.84$. There was also a significant increase in individuals who felt that there was a need for an ingredient labeling system; $3.68{\pm}0.9$ in 2006 and $4.32{\pm}0.61$ in 2011. There was also a higher demand for nutritional information; $2.85{\pm}0.66$ in 2006, $3.06{\pm}0.65$ in 2011. From these results, it was concluded that the nutrition labeling system adopted by family restaurants did not affect the degree of customers' interest in nutritional information. Contrast to the results, the amount and frequency of nutritional information provided to customers have increased continuously since 2006. Therefore, the nutrition labeling system and recommended dietary allowance should be expanded in order to promote a healthy diet.

The Comparison of Results Among Hepatitis B Test Reagents Using National Standard Substance (국가 표준물질을 이용한 B형 간염 검사 시약 간의 결과 비교)

  • Lee, Young-Ji;Sim, Seong-Jae;Back, Song-Ran;Seo, Mee-Hye;Yoo, Seon-Hee;Cho, Shee-Man
    • The Korean Journal of Nuclear Medicine Technology
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    • v.14 no.2
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    • pp.203-207
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    • 2010
  • Purpose: Hepatitis B is infection caused by Hepatitis B virus (HBV). Currently, there are several methods, Kits and equipments for conducting Hepatitis B test. Due to ununiformed methods, it would cause some differences. To manage these differences, it needs process evaluating function of test system and reagent using particular standard substance. The aim of this study is to investigate tendency of RIA method's reagent used in Asan Medical Center through comparing several other test reagents using national standard substance. Materials and Methods: The standard substance in National Institute of Food and Drug Safety Evaluation's biology medicine consists of 5 things, 4 antigens and 1 antibody. We tested reagents using A, B company's Kits according to each test method. All tests are measured repeatedly to obtain accurate results. Results: Test result of "HBs Ag Mixed titer Performance panel" is obtained match rate compared S/CO unit standard with RIA method and EIA 3 reagents, CIA 2 reagents is that company A's reagent is 94.4% (17/18), 83.3% (15/18), B is 88.9% (16/18), 77.8% (14/18). Test result of "HBs Ag Low titer Performance panel" is obtain that EIA 2 reagents is shown 7 posive results, CIA 3 reagents is 11, and RIA method's company A's reagent is 3, B is 2 of 13 in low panel. "HBV surface antigen 86.76 IU/vial" tested dilution. A is obtain positive results to 600 times(0.14 IU/mL), B is 300 times (0.29 IU/mL). Case of "HBV human immunoglobulin 95.45 IU/vial", A is shown positive result to 10,000 times (9.5 mIU/mL) and B is 4,000 times (24 mIU/mL). Test result of "HBs Ag Working Standards 0.02~11.52 IU/mL" is shown that Company A's kit concentration level was 0.38IU/mL, company B was 2.23 IU/mL and higher level of concentration was positive results. Conclusion: When comparing various test reagents and RIA method according to National Standard substances for Hepatitis B test, we recognized that there were no significant trends between reagents. For hepatitis B virus antigen-antibody titers even in parts of the test up to 600 times the antigen, antibodies to 10,000 times the maximum positive results could be obtained. Therefore, we confirmed that results from Asan Medical Center are performed smoothly by reagents and system for hepatitis B virus test.

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Current feeding practices and maternal nutritional knowledge on complementary feeding in Korea (이유기 보충식 현황과 어머니 인식 조사)

  • Yom, Hye Won;Seo, Jeong Wan;Park, Hyesook;Choi, Kwang Hae;Chang, Ju Young;Ryoo, Eell;Yang, Hye Ran;Kim, Jae Young;Seo, Ji Hyun;Kim, Yong Joo;Moon, Kyung Rye;Kang, Ki Soo;Park, Kie Young;Lee, Seong Soo;Shim, Jeong Ok
    • Clinical and Experimental Pediatrics
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    • v.52 no.10
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    • pp.1090-1102
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    • 2009
  • Purpose:To evaluate current feeding practices and maternal nutritional knowledge on complementary feeding. Methods:Mothers of babies aged 9-15 months who visited pediatric clinics of 14 general hospitals between September and December 2008 were asked to fill questionnaires. Data from 1,078 questionnaires were analyzed. Results:Complementary food was introduced at 4-7 months in 89% of babies. Home-made rice gruel was the first complementary food in 93% cases. Spoons were used for initial feeding in 97% cases. At 6-7 months, <50% of babies were fed meat (beef, 43%). Less than 12-month-old babies were fed salty foods such as salted laver (35%) or bean-paste soup (51%) and cow's milk (11%). The following were the maternal sources of information on complementary feeding: books/magazines (58%), friends (30%), internet web sites (29%), relatives (14%), and hospitals (4%). Compared to the 1993 survey, the incidence of complementary food introduction before 4 months (0.4% vs. 21%) and initial use of commercial food (7% vs. 39%) had decreased. Moreover, spoons were increasingly used for initial feeding (97% vs. 57%). The average maternal nutritional knowledge score was 7.5/10. Less percentage of mothers agreed with the following suggestions: bottle formula weaning before 15-18 months (68%), no commercial baby drinks as complementary food (67%), considering formula (or cow's milk) better than soy milk (65%), and feeding minced meat from 6-7 months (57%). Conclusion:Complementary feeding practices have considerably improved since the last decade. Pediatricians should advise timely introduction of appropriate complementary foods and monitor diverse information sources on complementary feeding.

Intake of Snacks, and Perceptions and Use of Food and Nutrition Labels by Middle School Students in Chuncheon Area (춘천지역 중학생들의 간식 섭취 실태와 식품·영양표시에 대한 인식 및 이용실태)

  • Kim, Yoon-Sun;Kim, Bok-Ran
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.41 no.9
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    • pp.1265-1273
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    • 2012
  • The purpose of this study was to investigate the BMI, intake of snacks, and perceptions and use of food and nutrition labels by middle school students (144 boys and 189 girls) in Chuncheon area. The average height and weight of boys were $171.0{\pm}6.4$ cm and $61.0{\pm}11.4$ kg, respectively, whereas those of girls were $160.0{\pm}4.8$ cm and $50.8{\pm}6.6$ kg, respectively. Average body mass index (BMI) of boys and girls were $20.8{\pm}3.3$ and $19.8{\pm}2.4$, respectively (p<0.01). Dietary intake attitude score of girls ($34.39{\pm}5.66$) was higher than that of boys ($33.92{\pm}5.40$) (p<0.05). Subjects bought and ate snacks 1 to 3 times per week (40.2%) by themselves, and most consumed snacks were cookies (23.1%), instant noodles (16.2%), ice cream (13.2%), and candy and chocolates (13.2%). The most important factor in purchasing of snacks was 'taste' ($4.49{\pm}0.67$). When subjects bought processed foods, the rates of reading food labels was 86.6%. The most important factor of the food labels was 'expiration date' (42.9%). The degree of reading food labels on processed foods by girls ($22.70{\pm}5.72$) was higher than that of boys ($20.96{\pm}5.35$) (p<0.01). Of the 13.2% of subjects that did not read food labels, the reason why was that they were not interested (50.0%). Of the 78.4% of subjects that read nutrition labels, the most important component of the nutrition labels was 'calories' (75.9%). The main reason for reading nutrition labels was 'to control weight' (45.6%). In general, use of food labels correlated positively with dietary intake attitude score (p<0.05) and use of nutrition labels (p<0.01). Using multiple regression analysis, we found that 'usefulness of dietary life' was the most significant variable that affects the importance of food and nutrition labels. Therefore, development of an educational program on food and nutrition labels for adolescents will be effective in improving dietary life.