Satisfaction Survey of the Regional Networks for Blood Transfusion Management Project / 대한수혈학회지

Background@#The Korean Blood Safety Commission established the Regional Networks for Blood Transfusion Management (RNBTM) Project which has operated in 14 regions across the country since 2017 to help blood transfusion management in small and medium-sized medical institutions. Since implementation of the RNBTM, participant’s satisfaction has not been evaluated, therefore in order to evaluate participant’s satisfaction and assist in future planning a survey was conducted. @*Methods@#Fourteen facilitators participated in an anonymous on-line survey (5 questions). Laboratory Medicine (LM) doctors from small and medium-sized medical institutions from 14 regions also participated in a separate anonymous on-line survey (15 questions). @*Results@#14/14 (100%) facilitators responded to the survey. In addition to the RNBTM they were also in favor of establishing a Network of University Hospitals. 135 LM doctors responded to the survey. Of the RNBTM participants, 94.1% (111/118) replied that RNBTM was helpful in providing and exchanging blood transfusion information. Respondents indicated that they wished to remain part of RNBTM into the future because they felt that RNBTM not only helped improve quality of blood transfusions but also other aspects in the laboratory. In 70.4% (95/135) of participating medical institutions, LM doctors have been the Chairperson of the Hospital Transfusion Committee. @*Conclusion@#Extremely positive survey results were received from LM doctors. These survey results from LM doctors and facilitators will be helpful in developing and planning future projects related to RNBTM.

Successful Sirolimus Treatment for Korean Patients with Activated Phosphoinositide 3-kinase δ Syndrome 1: the First Case Series in Korea

Activated phosphoinositide 3-kinase δ syndrome (APDS)1 is caused by gain-of-function mutations in PIK3CD, which encodes the catalytic p110δ subunit of phosphoinositide 3 kinase. We describe three patients with APDS1, the first thereof in Korea. Therein, we investigated clinical manifestations of APDS1 and collected data on the efficacy and safety profile of sirolimus, a mammalian target of rapamycin inhibitor and pathway-specific targeted medicine. The same heterozygous PIK3CD mutation was detected in all three patients (E1021K). After genetic diagnosis, all patients received sirolimus and experienced an excellent response, including amelioration of lymphoproliferation and improvement of nodular mucosal lymphoid hyperplasia in the gastrointestinal tract. The median trough level of sirolimus was 5.5 ng/mL (range, 2.8–7.5) at a dose of 2.6–3.6 mg/m2. Two patients who needed highdose, short-interval, immunoglobulin-replacement treatment (IGRT) had a reduced requirement for IGRT after initiating sirolimus, and the dosing interval was extended from 2 and 3 weeks to 4 weeks. The IgG trough level after sirolimus treatment (median, 594 mg/dL; range, 332–799 mg/dL) was significantly higher than that before sirolimus treatment (median, 290 mg/dL; range, 163–346 mg/dL) (p<0.001). One episode of elevated serum creatinine with a surge of sirolimus (Patient 2) and episodes of neutropenia and oral stomatitis (Patient 1) were observed. We diagnosed the first three patients with APDS1 in Korea. Low-dose sirolimus may alleviate clinical manifestations thereof, including hypogammaglobulinemia.

Analysis of the Effects of the Implementation of Transfusion-Related Education for Nurses at Medical Institutions / 대한수혈학회지

Background@#Nurses play a central role in the process of blood transfusion because they have the closest interactions with patients. Providing nurses with the appropriate knowledge and competency through education can help ensure transfusion safety. This study investigated the effectiveness of the first nationwide transfusion-related education for nurses by analyzing questionnaires for a self-assessment of competency on transfusion-related knowledge before and after the education and general evaluation for the educational program. @*Methods@#The education program was composed of four lectures and was conducted in seven regions in South Korea. One hundred and ninety-two nurses participated, and the questionnaires for 170 nurses were analyzed. @*Results@#The participants consisted of 90 nurses (53.0%) from tertiary hospitals, 23 (13.5%) from general hospitals, and 57 (33.5%) from other hospitals. The majority of the participants (103/170, 60.6%) were from hospitals with ≥500 beds, and 69.4% had a work period of ≥10 years. The scores for pre-/post-education self-assessment of competency were as follows: blood components, 3.03/3.73; pretransfusion testing, 2.86/3.64; management of transfusion, 3.18/3.84; and transfusion reactions, 3.11/3.78. In all categories, there was a significant increase in the score after the education program. The majority of participants (99.4%) provided a positive response regarding the necessity of a transfusion-related education program. @*Conclusion@#South Korea’s first attempt at transfusion-related education for nurses showed a strong positive effect by improving participants’ transfusion-related competency. Considering the important role of nurses in blood transfusion, the educational program should continue and be expanded in the future.

Analysis of the Effects of the Implementation of Transfusion-Related Education for Nurses at Medical Institutions / 대한수혈학회지

Background@#Nurses play a central role in the process of blood transfusion because they have the closest interactions with patients. Providing nurses with the appropriate knowledge and competency through education can help ensure transfusion safety. This study investigated the effectiveness of the first nationwide transfusion-related education for nurses by analyzing questionnaires for a self-assessment of competency on transfusion-related knowledge before and after the education and general evaluation for the educational program. @*Methods@#The education program was composed of four lectures and was conducted in seven regions in South Korea. One hundred and ninety-two nurses participated, and the questionnaires for 170 nurses were analyzed. @*Results@#The participants consisted of 90 nurses (53.0%) from tertiary hospitals, 23 (13.5%) from general hospitals, and 57 (33.5%) from other hospitals. The majority of the participants (103/170, 60.6%) were from hospitals with ≥500 beds, and 69.4% had a work period of ≥10 years. The scores for pre-/post-education self-assessment of competency were as follows: blood components, 3.03/3.73; pretransfusion testing, 2.86/3.64; management of transfusion, 3.18/3.84; and transfusion reactions, 3.11/3.78. In all categories, there was a significant increase in the score after the education program. The majority of participants (99.4%) provided a positive response regarding the necessity of a transfusion-related education program. @*Conclusion@#South Korea’s first attempt at transfusion-related education for nurses showed a strong positive effect by improving participants’ transfusion-related competency. Considering the important role of nurses in blood transfusion, the educational program should continue and be expanded in the future.

Changes in Cytomegalovirus Seroprevalence in Korea for 21 Years: a Single Center Study

PURPOSE: Cytomegalovirus (CMV) infection is mostly asymptomatic but can be detrimental to certain hosts. We investigated changes of CMV seroprevalence in Koreans before and after the year 2000. METHODS: We reviewed laboratory values of patients who were tested for CMV immunoglobulin G (IgG) at Samsung Medical Center, Seoul, Korea, from January 1995 to December 2015. Changes in seroprevalence were analyzed by gender, age, region, and tested year period (period 1, 1995–2005 vs. period 2, 2006–2015). RESULTS: Overall CMV seropositivity was 94.1% (10,900/11,584). There was no significant difference for CMV seropositivity among the two periods (94.2% vs. 94.1%) (P=0.862). CMV seropositivity in the 11 to 20-year age group in period 2 (78.8%) was significantly lower than that of period 1 (89.9%) (P=0.001). The seropositivity of individuals aged 31–40 years (97.4%) was significantly higher than that of younger age groups (P < 0.001) and lower than that of older age groups (P < 0.001). Of 2,441 females of reproductive age (from 15 to 49), CMV seropositivity was 97% (2,467/2,441). The seropositivity in women aged 20–24-years was higher than that of men in the same age group (97.6% vs. 85.6%, P=0.003). No significant difference was observed among different regions. CONCLUSIONS: Overall CMV seropositivity of Koreans was estimated to be 94% and the average seropositivity of reproductive women was 97%. Monitoring of the changes in seroprevalence including the reproductive age group is needed in the future.

Bronchiectasis and Recurrent Respiratory Infections with a De Novo STAT1 Gain-of-Function Variant: First Case in Korea

Bronchiectasis is a chronic disease characterized by airway infection and inflammation, leading to permanent dilation of the bronchi. Evaluation of underlying etiology is important in managing young bronchiectasis patients with recurrent infections caused by unusual pathogens. The signal transducer and activator of transcription 1 (STAT1) protein plays a key role in STAT signaling and immune system regulation. Heterozygotes for gain-of-function (GOF) alleles of the STAT1 gene usually display autosomal dominant chronic mucocutaneous candidiasis (CMC) and a wide range of clinical features, such as bronchiectasis. Here, we report on a patient with CMC and bronchiectasis with various types of infections who carried a pathogenic variant of the STAT1 gene. The 24-year-old female presented with recurrent respiratory bacterial and nontuberculous mycobacterial infections complicated by severe bronchiectasis and CMC. Whole-exome sequencing revealed a c.800C>T (p.Ala267Val) heterozygous mutation in the STAT1 gene. Further analysis by Sanger sequencing of STAT1 from the patient and her parents revealed the patient had a de novo occurrence of the variant. This is the first report of a Korean patient with a GOF pathogenic variant in STAT1. Physicians should be aware of the existence of this variant as a genetic factor associated with CMC and bronchiectasis complicated by recurrent infection.

Assessment of Medical Trainnees' Knowledge and Their Educational Needs on Transfusion Medicine / 대한수혈학회지

BACKGROUND: Transfusion is one of the most important treatments in patient care. It has been known that there is a lack of transfusion medicine knowledge of doctors in other countries, however it has not been investigated yet in Korea. In this study, we assessed the educational need and the knowledge on transfusion medicine for trainees to provide basic raw data for future education in transfusion medicine. METHODS: Transfusion is one of the most important treatments in patient care. It has been known that there is a lack of transfusion medicine knowledge of doctors in other countries, however it has not been investigated yet in Korea. In this study, we assessed the educational need and the knowledge on transfusion medicine for trainees to provide basic raw data for future education in transfusion medicine. RESULTS: Among trainees, 89.9% said they received more than one hour of transfusion medicine education at medical schools, but 57.3% did not receive transfusion medicine education after finishing medical school. Moreover, 76.4% and 86.5% of respondents stated that additional transfusion medicine education was needed during and after medical school respectively. Among respondents, 43.5%, 53.9%, and 28.1% reported their knowledge on blood products, pretransfusion testing, and transfusion reactions as low or very low. In the assessment of knowledge of transfusion medicine, the mean percentage of correct answers was 65.2%. The rates of correct answers (average) ranged from 25.8% to 80.9% (58.2%), 49.4% to 94.4% (75.7%) 70.8% to 89.9% (80.2%) in case of blood products, pretransfusion testing, and transfusion reactions respectively. CONCLUSION: Transfusion medicine education among trainees after finishing medical school was found to be insufficient, and additional education for transfusion medicine is demanded by trainees.

Serious Adverse Transfusion Reactions Reported in the National Recipient-Triggered Trace Back System in Korea (2006-2014)

BACKGROUND: Adverse transfusion reactions (ATRs) are clinically relevant to patients with significant morbidity and mortality. This study aimed to review the cases of ATR reported in the recipient-triggered trace back system for a recent nine-year period in Korea. METHODS: Nine-year data obtained from 2006 to 2014 by the trace back system at the Division of Human Blood Safety Surveillance of the Korean Centers for Disease Control (KCDC) were reviewed. The suspected cases were assessed according to six categories: (i) related to, (ii) probably related to, (iii) probably not related to, (iv) not related to transfusion, (v) unable to investigate, and (vi) under investigation. RESULTS: Since 2006, 199 suspected serious ATRs were reported in hospitals and medical institutions in Korea, and these ATRs were reassessed by the division of Human Blood Safety Surveillance of the KCDC. Among the reported 193 cases as transfusion related infections, hepatitis C virus (HCV) infection (135, 67.8%) was reported most frequently, followed by hepatitis B virus (HBV) infection (27, 13.6%), HIV infection (13, 6.5%), syphilis (9, 4.5%), malarial infection (4, 2.0%), other bacterial infections (3, 1.5%), HTLV infection (1, 0.5%), and scrub typhus infection (1, 0.5%), respectively. Of the 199 cases, 13 (6.5%) cases were confirmed as transfusion-related (3 HCV infections, 3 malarial infections, 1 HBV infection, 2 Staphylococcus aureus sepsis, 3 transfusion-related acute lung injuries, and 1 hemolytic transfusion reaction). CONCLUSIONS: This is the first nationwide data regarding serious ATRs in Korea and could contribute to the implementation of an effective hemovigilance system.

A Survey of Physicians' Opinion on Informed Consent for Transfusion after the Introduction of the National Blood Transfusion Guideline / 대한수혈학회지

BACKGROUND: The decision on whether or not to transfuse should be based on agreement from the patient's own understanding and free will. In Korea, the transfusion guideline first developed in 2009 specified that informed consent should be obtained before transfusion. The standard consent form has also been distributed since 2010. The purpose of this study was to evaluate the degree of improvement in obtaining informed consent for blood transfusion. METHODS: Prior to the development of the transfusion guideline in 2008, an e-mail survey was conducted on 105 clinicians. In 2015, questionnaires were sent to 1,179 clinicians, 32 academic societies, and 6 institutions. RESULTS: Forty-three and 374 answers were received in 2008 and 2015, respectively (response rate, 41.0% and 30.7%). Compared with 2008, the use of explanatory materials increased from 20.9% to 70.5%, and consent acquisitions using a written form increased from 27.9% to 81.7%. The number of respondents who answered that informed consent for transfusions is absolutely necessary also increased from 51.2% to 71.3%. Among the reasons why obtaining informed consent for transfusion can be difficult, the response that standardized consent form is unavailable decreased from 51.2% to 26.9%. CONCLUSION: The transfusion guideline and the standard consent form have contributed greatly to the improvement of obtaining informed consent before blood transfusion at medical institutions in Korea.

A Survey on the Awareness and Usage of the National Transfusion Guideline in Korea / 대한수혈학회지

BACKGROUND: Transfusion guidelines play an important role for the appropriate use and quality assurance of blood and transfusion services. The Korean national transfusion guideline was developed in 2009 and went under full amendment in 2016. The purpose of this study was to investigate the awareness and practicality of the transfusion guideline in Korea. METHODS: Questionnaires about the Korean national transfusion guideline were sent by traditional mail or e-mail to a total of 1,179 clinicians, 32 academic societies, and 6 institutions. RESULTS: Three hundred and seventy-four answers were received; a response rate of 30.7%. The proportion of respondents with good awareness of the guideline was 23.3%, which is a significant increase compared with 10.9% in 2008. Respondents with good awareness were more dependent on the transfusion guideline when making transfusion decisions. CONCLUSION: There was a considerable increase in the awareness of the national transfusion guideline in Korea.

Growth Dynamics of Staphylococcus aureus, Escherichia coli, and Pseudomonas aeruginosa as a Function of Time to Detection in BacT/Alert 3D Blood Culture Bottles with Various Preincubation Conditions

BACKGROUND: Delayed entry of blood culture bottles is inevitable when microbiological laboratories do not operate for 24 hr. There are few studies reported for prestorage of these bottles. The growth dynamics of Staphylococcus aureus, Escherichia coli, and Pseudomonas aeruginosa were investigated with respect to various preincubation conditions. METHODS: Fifteen or 150 colony-forming units (CFU) of bacteria were inoculated into standard aerobic or anaerobic blood culture bottles. Bottles were preincubated at 25degrees C or 37degrees C for 0, 2, 4, 8, 12, 24, or 48 hr. The time to detection (TTD) then was monitored using the BacT/Alert 3D system (bioMerieux Inc., USA). RESULTS: Significant difference in TTD was observed following preincubation for 8 hr at 25degrees C vs. 4 hr at 37degrees C for S. aureus, 4 hr at 25degrees C vs. 4 hr at 37degrees C for E. coli, 12 hr at 25degrees C vs. 4 hr at 37degrees C for P. aeruginosa, compared to no preincubation (P<0.005). TTD values did not vary significantly with bacterial CFU or with aerobic or anaerobic bottle type. The BacT/Alert 3D system returned false negatives following preincubation of P. aeruginosa for 48 hr at 25degrees C or 24 hr at 37degrees C. CONCLUSIONS: TTD was mainly affected by preincubation temperature and duration rather than by input CFU quantity or bottle type for the 3 experimental bacteria.

Effect of Sodium Citrate on Growth of Bacteria in Blood Culture / 대한임상미생물학회지

BACKGROUND: This study compared the growth of Staphylococcus aureus, Escherichia coli, Pseudomonas aeruginosa, Streptococcus pneumoniae, and Haemophilus influenzae in blood culture bottles containing anticoagulants, sodium polyanethol sulfonate (SPS) and sodium citrate. METHODS: One hundred and fifty colony forming units of five different bacterial species were inoculated into standard aerobic (SA) and standard anaerobic (SN) bottles and were combined with 5 mL of human blood in solution with SPS or sodium citrate. Time to detection (TTD) was then monitored using the BacT/Alert 3D system (bioMerieux Inc.). RESULTS: Compared to the bacteria-only controls, cultures containing S. aureus, E. coli, P. aeruginosa, and S. pneumoniae plus SPS blood or citrated blood trended toward reduced TTD in both SA and SN bottles; however, there was no significant difference in TTD between SPS and sodium citrate anticoagulant. Although H. influenzae showed a remarkable difference in TTD between SPS (SA 14.8 h, SN 15.0 h) and sodium citrate (SA 23.5 h, SN 18.3 h), this difference was not statistically significant (P=0.10). CONCLUSION: Addition of blood enhanced growth of bacteria. All experimental bacteria except H. influenzae showed similar TTD in SPS blood and citrated blood. These results support the usefulness of sodium citrate anticoagulant for artificial inoculation in blood culture bottles.

Proton-Pump Inhibitor-Induced Hypocalcemia and Hypomagnesemia

A 7-day-old female neonate who visited emergency depar tment due to generalized tonic seizure. Laboratory test results showed hypocalcemia (5.7 mg/dL), hypomagnesemia (0.55 mmol/L), low parathyroid hormone (7.5 pg/mL), and normal 25(OH) vitamin D3. Symptom and metabolic abnormalities were normalized with intravenous calcium gluconate and magnesium sulfate. Discharged with supplement of oral calcium, vitamin D, phenobarbital, and lansoprazol, she was re-admitted with hypocalcemia (4.8 mg/dL) with normal level of parathyroid hormone (12.3 pg/mL). Hypocalcemia was resolved with discontinuation of proton pump inhibitor. We report a case of recurrent hypocalcemia and hypomagnesemia due to proton-pump inhibitor.

Triple High Dose Chemotherapy Followed by Autologous Stem Cell Transplantation for Pediatric Neuroblastoma / 임상소아혈액종양

BACKGROUND: High-risk group neuroblastoma (HRNB) is one of the most incurable diseases in pediatric oncology field. This report explores the effectiveness, safety and feasibility of triple high dose chemotherapy followed by autologous stem cell transplantation (HDCT/ASCT) in contrast with single HDCT/ASCT for HRNB.METHODS: From Jan. 2001 to Dec. 2009, 25 patients newly diagnosed as HRNB have been analyzed. This study is a retrospective analysis with the medical records of these 25 HRNB patients.RESULTS: Eleven (44%) and the other fourteen (56%) patients were treated with single HDCT/ASCT and triple HDCT/ASCT, respectively. The 5-year event-free survival (EFS) of the whole group in HRNB is 37.2+/-10.0%. The 5-year EFS of single HDCT/ASCT and triple HDCT/ASCT were 27.3+/-13.4% and 46.8+/-13.8%, respectively (P=0.38). Between two groups, there were no statistically significant differences including clinical features, outcome, transplantation-related toxicities, and short-term/long-term complications.CONCLUSION: Triple HDCT/ASCT group showed similar transplantation-related toxicities and long-term complications when compared to single HDCT/ASCT group. We need more exploration to conclude the triple HDCT/ASCT as an optimal treatment for HRNB patients.

Triple High Dose Chemotherapy Followed by Autologous Stem Cell Transplantation for Pediatric Neuroblastoma / 임상소아혈액종양

BACKGROUND: High-risk group neuroblastoma (HRNB) is one of the most incurable diseases in pediatric oncology field. This report explores the effectiveness, safety and feasibility of triple high dose chemotherapy followed by autologous stem cell transplantation (HDCT/ASCT) in contrast with single HDCT/ASCT for HRNB. METHODS: From Jan. 2001 to Dec. 2009, 25 patients newly diagnosed as HRNB have been analyzed. This study is a retrospective analysis with the medical records of these 25 HRNB patients. RESULTS: Eleven (44%) and the other fourteen (56%) patients were treated with single HDCT/ASCT and triple HDCT/ASCT, respectively. The 5-year event-free survival (EFS) of the whole group in HRNB is 37.2+/-10.0%. The 5-year EFS of single HDCT/ASCT and triple HDCT/ASCT were 27.3+/-13.4% and 46.8+/-13.8%, respectively (P=0.38). Between two groups, there were no statistically significant differences including clinical features, outcome, transplantation-related toxicities, and short-term/long-term complications. CONCLUSION: Triple HDCT/ASCT group showed similar transplantation-related toxicities and long-term complications when compared to single HDCT/ASCT group. We need more exploration to conclude the triple HDCT/ASCT as an optimal treatment for HRNB patients.