1.Bilateral Extensive Serous Retinal Detachment in a Patient with Multiple Myeloma
Jeong Hwa SHIN ; Ik-Chan SONG ; Deog Yeon JO ; Ki Yup NAM
Journal of the Korean Ophthalmological Society 2024;65(4):290-296
Purpose:
To describe a case of bilateral serous retinal detachment in a patient with multiple myeloma.Case summary: A 75-year-old woman presented with sudden-onset of reduced visual acuity in both eyes which had begun 2 days prior. Initial ophthalmological assessment revealed that visual acuities were 0.03 in the right eye and 0.05 in the left eye. Optical coherence tomography indicated pronounced macular elevation and extensive subretinal fluid accumulation in the posterior pole of both eyes. Fluorescein angiography did not detect any significant vascular leakage. Blood tests showed an albumin level of 2.1 g/dL, and plasma protein electrophoresis revealed an M protein level of 0.19 g/dL. Multiple myeloma-related hypoalbuminemia and paraprotein were suspected. The patient's retinal statuses were closely monitored and compared with blood test outcomes. Over 2 months, there was significant improvement, characterized by decreasing macular elevation and reduction of subretinal fluid. This progress was sustained thereafter. Plasma protein electrophoresis showed a gradual decline in paraproteins which coincided with the amelioration of serous retinal detachment.
Conclusions
We encountered a case of acute and extensive serous retinal detachment in both eyes of a patient with multiple myeloma. When fluorescein angiography does not definitively show vascular leakage, light chain deposition disease-related serous retinal detachment should be considered. In such instances, it is important to address and monitor the underlying multiple myeloma.
2.Generalized Tetanus Treated with Intrathecal Baclofen Infusion
Yeon-Jung MUN ; Wonjeong SON ; Gyeong-Jo BYEON ; Eun Hwa LEE ; Jin-Hong SHIN
Journal of the Korean Neurological Association 2023;41(3):220-224
Tetanus is a bacterial infection by Clostridium tetani. Its neurotoxin causes spastic paralysis and autonomic dysfunction. Intrathecal infusion of baclofen has been suggested as a pertinent treatment for generalized spasm. Our case describes a patient who had a severe generalized form of tetanus, and was effectively treated with intrathecal baclofen infusion. He showed cerebral and brainstem dysfunction during baclofen infusion, which were reversed without sequelae when baclofen was discontinued.
3.Allergen sensitization trajectories in children with respiratory and allergic diseases
So Won JO ; Soyoung JEON ; Hye Sun LEE ; Ha Min KIM ; Yoon Young NO ; Mi Reu PARK ; Jae Hwa JUNG ; Soo Yeon KIM ; Jong Duck KIM ; Min Jung KIM ; Yong Ju LEE ; Kyung Won KIM ; Myung Hyun SOHN ; Yoon Hee KIM
Allergy, Asthma & Respiratory Disease 2023;11(1):34-42
Purpose:
There is a lack of a report about the trajectories of allergen sensitization, although it is important to understand the change of allergen sensitization to manage allergic disease. This study aimed to analyze the change and trajectories of allergen sensitization in children with respiratory and allergic diseases.
Methods:
From 2006 to 2020, children with respiratory and allergic diseases or screened for allergic sensitization were evaluated. We visualized the alterations and the trajectories of allergen sensitization using stacked area graphs, box plots, and Sankey diagrams.
Results:
A total of 2,804 subjects were included, and allergic rhino-conjunctivitis was diagnosed in 1,931 children (68.9%). The mean age for the first test was 4.1 years, and that for the second test was 6.5 years. Children sensitized to class 1 food allergen before age 5 showed sensitizations more for other allergens and at a younger age after age 5 than children who were not. The atopic tendency continued once it had been obtained before the early school age in the persistence or the new development of sensitization.
Conclusion
Allergen sensitization has changed over time and has shown different patterns according to age. Its trajectory has taken a wide variety of courses in children with respiratory and allergic diseases until the early school age. These changes reflect the allergic diseases and socio-environmental characteristics of children and adolescents.
4.Definitions of Chronic Obstructive Pulmonary Disease and Chronic Obstructive Pulmonary Disease Exacerbation: A Modified Delphi Survey
Yong Bum PARK ; Jin Hwa LEE ; Seung Won RA ; Hye Yun PARK ; Ji Ye JUNG ; Young Ae KANG ; Chin Kook RHEE ; Deog Kyeom KIM ; Kwang Ha YOO ; Yong Il HWANG ; Seong Yong LIM ; Jae Seung LEE ; Kyung-Wook JO ; Yeon-Mok OH
Tuberculosis and Respiratory Diseases 2023;86(3):196-202
Background:
The Global Initiative for Chronic Obstructive Lung Disease (GOLD) update 2023 proposed new definitions of chronic obstructive pulmonary disease (COPD) and COPD exacerbation. However, an agreement on the definitions has not been made, either internationally or domestically. This study aimed to reach an agreement between experts on the new definitions of COPD and COPD exacerbation in South Korea.
Methods:
A modified Delphi method was used to make an agreement on the definitions of COPD and COPD exacerbation proposed by the GOLD update 2023. We performed two rounds of the survey including 15 Korean experts on COPD, asthma, and tuberculosis.
Results:
More than two-thirds of the experts agreed on 12 of the 13 statements related to the definitions of COPD and COPD exacerbation in the two rounds of the survey. The experts agreed on the definitions of COPD and COPD exacerbation that should be revised in line with the definitions proposed by the GOLD update 2023. However, the experts showed an uncertain opinion on the statement that the definition of COPD includes patients with persistent airflow obstruction due to bronchiectasis.
Conclusion
Based on this Delphi survey, experts’ agreement was made on the definitions of COPD and COPD exacerbation proposed by the GOLD update 2023.
5.Developing a Diagnostic Bundle for Bronchiectasis in South Korea: A Modified Delphi Consensus Study
Hayoung CHOI ; Hyun LEE ; Seung Won RA ; Jong Geol JANG ; Ji-Ho LEE ; Byung Woo JHUN ; Hye Yun PARK ; Ji Ye JUNG ; Seung Jun LEE ; Kyung-Wook JO ; Chin Kook RHEE ; Changwhan KIM ; Sei Won LEE ; Kyung Hoon MIN ; Yong-Soo KWON ; Deog Kyeom KIM ; Jin Hwa LEE ; Yong Bum PARK ; Eun Hee CHUNG ; Yae-Jean KIM ; Kwang Ha YOO ; Yeon-Mok OH
Tuberculosis and Respiratory Diseases 2022;85(1):56-66
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.
6.Long-term follow-up results of cytarabine-containing chemotherapy for acute promyelocytic leukemia
Young Hoon PARK ; Dae-Young KIM ; Yeung-Chul MUN ; Eun Kyung CHO ; Jae Hoon LEE ; Deog-Yeon JO ; Inho KIM ; Sung-Soo YOON ; Seon Yang PARK ; Byoungkook KIM ; Soo-Mee BANG ; Hawk KIM ; Young Joo MIN ; Jae Hoo PARK ; Jong Jin SEO ; Hyung Nam MOON ; Moon Hee LEE ; Chul Soo KIM ; Won Sik LEE ; So Young CHONG ; Doyeun OH ; Dae Young ZANG ; Kyung Hee LEE ; Myung Soo HYUN ; Heung Sik KIM ; Sung-Hyun KIM ; Hyukchan KWON ; Hyo Jin KIM ; Kyung Tae PARK ; Sung Hwa BAE ; Hun Mo RYOO ; Jung Hye CHOI ; Myung-Ju AHN ; Hwi-Joong YOON ; Sung-Hyun NAM ; Bong-Seog KIM ; Chu-Myong SEONG
The Korean Journal of Internal Medicine 2022;37(4):841-850
Background/Aims:
We evaluated the feasibility and long-term efficacy of the combination of cytarabine, idarubicin, and all-trans retinoic acid (ATRA) for treating patients with newly diagnosed acute promyelocytic leukemia (APL).
Methods:
We included 87 patients with newly diagnosed acute myeloid leukemia and a t(15;17) or promyelocytic leukemia/retinoic acid receptor alpha (PML-RARα) mutation. Patients received 12 mg/m2/day idarubicin intravenously for 3 days and 100 mg/m2/day cytarabine for 7 days, plus 45 mg/m2/day ATRA. Clinical outcomes included complete remission (CR), relapse-free survival (RFS), overall survival (OS), and the secondary malignancy incidence during a 20-year follow-up.
Results:
The CR, 10-year RFS, and 10-year OS rates were 89.7%, 94.1%, and 73.8%, respectively, for all patients. The 10-year OS rate was 100% for patients that achieved CR. Subjects were classified according to the white blood cell (WBC) count in peripheral blood at diagnosis (low-risk, WBC < 10,000/mm3; high-risk, WBC ≥ 10,000/mm3). The low-risk group had significantly higher RFS and OS rates than the high-risk group, but the outcomes were not superior to the current standard treatment (arsenic trioxide plus ATRA). Toxicities were similar to those observed with anthracycline plus ATRA, and higher than those observed with arsenic trioxide plus ATRA. The secondary malignancy incidence after APL treatment was 2.7%, among the 75 patients that achieved CR, and 5.0% among the 40 patients that survived more than 5 years after the APL diagnosis.
Conclusions
Adding cytarabine to anthracycline plus ATRA was not inferior to anthracycline plus ATRA alone, but it was not comparable to arsenic trioxide plus ATRA. The probability of secondary malignancy was low.
7.Early diagnosis of Gaucher disease in Korean patients with unexplained splenomegaly: a multicenter observational study
Young Rok DO ; Yunsuk CHOI ; Mi Hwa HEO ; Jin Seok KIM ; Jae-ho YOON ; Je-Hwan LEE ; Joon Seong PARK ; Sang Kyun SOHN ; Sung Hyun KIM ; Sungnam LIM ; Joo Seop CHUNG ; Deog-Yeon JO ; Hyeon Seok EOM ; Hawk KIM ; So Yeon JEON ; Jong-Ho WON ; Hee Jeong LEE ; Jung Won SHIN ; Jun-Ho JANG ; Sung-Soo YOON
Blood Research 2022;57(3):207-215
Background:
Gaucher disease (GD) is an autosomal recessive disorder characterized by excessive accumulation of glucosylceramide in multiple organs. This study was performed to determine the detection rate of GD in a selected patient population with unexplained splenomegaly in Korea.
Methods:
This was a multicenter, observational study conducted at 18 sites in Korea between December 2016 and February 2020. Adult patients with unexplained splenomegaly were enrolled and tested for β-glucosidase enzyme activity on dried blood spots (DBS) and in peripheral blood leukocytes. Mutation analysis was performed if the test was positive or indeterminate for the enzyme assay. The primary endpoint was the percentage of patients with GD in patients with unexplained splenomegaly.
Results:
A total of 352 patients were enrolled in this study (male patients, 199; mean age, 48.42 yr). Amongst them, 14.77% of patients had concomitant hepatomegaly. The most common sign related to GD was splenomegaly (100%), followed by thrombocytopenia (44.32%) and, anemia (40.91%). The β-glucosidase activity assay on DBS and peripheral leukocytes showed abnormal results in sixteen and six patients, respectively. Eight patients were tested for the mutation, seven of whom were negative and one patient showed a positive mutation analysis result. One female patient who presented with splenomegaly and thrombocytopenia was diagnosed with type 1 GD. The detection rate of GD was 0.2841% (exact 95% CI, 0.0072‒).
Conclusion
The detection rate of GD in probable high-risk patients in Korea was lower than expected.However, the role of hemato-oncologists is still important in the diagnosis of GD.
8.Transfusion and Plasmapheresis in Heart and Lung Transplantation in Korea
Hyun Ji LEE ; Kyung-Hwa SHIN ; Su-Yeon JO ; Hyung-Hoi KIM
Korean Journal of Blood Transfusion 2021;32(2):129-131
To increase the success rate of heart and lung transplantation, appropriate transfusion and desensitization treatment should be performed. In each hospital, transfusion should be performed according to the patient and hospital situation and monitored to ensure that an appropriate amount of transfusion is achieved is necessary. If HLA desensitization treatment is performed using plasmapheresis and immunosuppressants, the incidence of rejection and complications after transplantation can be reduced. For desensitization treatment that considers individual patient characteristics, close cooperation between clinical medical staff, blood bank personnel, and medical staff will be required.
9.Transfusion and Plasmapheresis in Heart and Lung Transplantation in Korea
Hyun Ji LEE ; Kyung-Hwa SHIN ; Su-Yeon JO ; Hyung-Hoi KIM
Korean Journal of Blood Transfusion 2021;32(2):129-131
To increase the success rate of heart and lung transplantation, appropriate transfusion and desensitization treatment should be performed. In each hospital, transfusion should be performed according to the patient and hospital situation and monitored to ensure that an appropriate amount of transfusion is achieved is necessary. If HLA desensitization treatment is performed using plasmapheresis and immunosuppressants, the incidence of rejection and complications after transplantation can be reduced. For desensitization treatment that considers individual patient characteristics, close cooperation between clinical medical staff, blood bank personnel, and medical staff will be required.
10.Report of the Korean Society of Infectious Diseases Roundtable Discussion on Responses to the Measles Outbreaks in Korea in 2019
Hyeri SEOK ; Dae Won PARK ; Kwang Nam KIM ; Min Ja KIM ; Sung-Han KIM ; Jeong Yeon KIM ; Su Eun PARK ; Se Yoon PARK ; Byung Wook EUN ; Mi Suk LEE ; Hyun-Ha CHANG ; Hyungul JUNG ; Hye Won JEONG ; Soo-Nam JO ; BumSik CHIN ; Young Hwa CHOI ; Eun Hwa CHOI ; Hee Jung CHOI ; Youngmee JEE ; Yang Soo KIM
Infection and Chemotherapy 2021;53(3):405-420
During the 2019 domestic measles outbreak in Korea, measles occurred in healthcare workers with two doses of the measles, mumps and rubella vaccine, and the strict application of the Occupational Safety and Health Act required medical institutions to identify healthcare workers' immunity to measles and vaccinate the susceptible pockets. In response to the frontline medical institutions' request to review the measles recommendations and guidelines, the Korean Society of Infectious Diseases held a roundtable discussion on the causes of measles outbreak, timing of vaccinations, antibody tests, and booster vaccinations for healthcare workers, and financial support from the government and municipality as well as response strategies against the outbreak in healthcare settings. In Korea, the seroprevalence of measles is decreasing in the vaccine-induced immunity group during the maintenance of measles elimination over several years. The susceptible group against measles is in their 20s and 30s, and this may be because of waning immunity rather than nonresponse considering Korea's vaccine policy. The risk of measles nosocomial infection from community increases as these susceptible pockets actively engage in medical institutions.Thus, data on the immunity of low seroprevalence group in Korea are needed, further discussion is needed on the booster vaccination based on the data. Especially, antibody testing and vaccination in healthcare workers may be necessary to prevent the spread of measles in medical insutitutions, and further discussion is needed regarding specific testing methods, and the timing and frequency of test and vaccination.

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