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1.
Article in English | WPRIM | ID: wpr-1044463

ABSTRACT

Hereditary breast and ovarian cancer syndrome (HBOC) is an inherited disorder associated with a higher than normal risk of breast and ovarian cancer. Most HBOC patients possess certain pathogenic variants (PVs) in BRCA1/2 genes. However, studies have indicated that HBOC patients may also have PVs in other cancer-related genes. Therefore, we analyzed variants in BRCA1/2 and other hereditary cancer-related genes in suspected HBOC patients using the multi-gene next-generation sequencing (NGS) panel method. We enrolled a total of 148 patients with cancers related to HBOC including breast, ovarian, primary peritoneal, prostate, and fallopian tube cancer. The 48 multi-gene NGS assay was applied to all samples, and multiplex ligation-dependent probe amplification (MLPA) and direct sequencing were used to confirm variants in BRCA1/2 and Lynch syndrome-related genes. We identified 17 PVs or likely PVs in 148 participants (11.5%), with PVs in BRCA1/2 detected in 7 patients (4.7%). We found PVs other than BRCA1/2 in 10 patients through the NGS panel and MLPA (7.1%). Apart from BRCA1/2, the genes in which PVs were detected included RAD51D, MLH1, MSH2, and MSH6. The NGS method shows significant potential in diagnosing and treating suspected HBOC patients, particularly those who test negative for BRCA1/2 genes.

2.
Article in English | WPRIM | ID: wpr-977179

ABSTRACT

Background@#Since the onset of the coronavirus disease 2019 pandemic, virtual simulation has emerged as an alternative to traditional teaching methods as it can be employed within the recently established contact-minimizing guidelines. This prospective education study aimed to develop a virtual reality simulator for a lumbar transforaminal epidural block (LTFEB) and demonstrate its efficacy. @*Methods@#We developed a virtual reality simulator using patient image data processing, virtual X-ray generation, spatial registration, and virtual reality technology. For a realistic virtual environment, a procedure room, surgical table, C-arm, and monitor were created. Using the virtual C-arm, the X-ray images of the patient’s anatomy, the needle, and indicator were obtained in real-time. After the simulation, the trainees could receive feedback by adjusting the visibility of structures such as skin and bones. The training of LTFEB using the simulator was evaluated using 20 inexperienced trainees. The trainees’ procedural time, rating score, number of C-arm taken, and overall satisfaction were recorded as primary outcomes. @*Results@#The group using the simulator showed a higher global rating score (P = 0.014), reduced procedural time (P = 0.025), reduced number of C-arm uses (P = 0.001), and higher overall satisfaction score (P = 0.007). @*Conclusions@#We created an accessible and effective virtual reality simulator that can be used to teach inexperienced trainees LTFEB without radiation exposure. The results of this study indicate that the proposed simulator will prove to be a useful aid for teaching LTFEB.

3.
Article in Korean | WPRIM | ID: wpr-1001679

ABSTRACT

Patellofemoral joint problems refer to a spectrum of conditions affecting the patellofemoral joint, which is the joint between the patella and femur. These conditions can cause pain and instability in the knee and affect an individual’s ability to perform daily activities. Patellofemoral joint problems commonly cause knee pain, particularly among young athletes and physically active individuals. This review article discusses current patellofemoral joint problems, including their epidemiology, pathophysiology, diagnosis, and management.Current Concepts: Patellofemoral joint problems are presented as clinical symptoms of pain and instability. Dividing the diagnostic criteria into anterior knee pain, patella instability, and patellofemoral arthritis is useful. Anterior knee pain is diagnosed after excluding possible causes. Patellar instability is classified into recurrent dislocation, habitual dislocation (extension and flexion types), and permanent dislocation. Moreover, patellar instability can progress to the final stage of patellofemoral arthritis. Thus, patellar instability should be treated according to the Dejour criterion, and patellofemoral arthritis treatment requires artificial joint replacement surgery.Discussion and Conclusion: The pathological mechanism of patellofemoral joint problems still needs to be properly established, and multifactorial causes make it difficult to treat patellofemoral joint problems. Accurate diagnosis is considered an essential factor for successful treatment.

4.
Article in English | WPRIM | ID: wpr-892179

ABSTRACT

Background@#Emergency departments (EDs) generally receive many casualties in disaster or mass casualty incidents (MCI). Some studies have conceptually suggested the surge capacity that ED should have; however, only few studies have investigated measurable numbers in one community. This study investigated the surge capacity of the specific number of accommodatable patients and overall preparedness at EDs in a metropolitan city. @*Methods@#This cross-sectional study officially surveyed surge capacity and disaster preparedness for all regional and local emergency medical centers (EMC) in Seoul with the Seoul Metropolitan Government's public health division. This study developed survey items on space, staff, stuff, and systems, which are essential elements of surge capacity. The number of patients acceptable for each ED was investigated by triage level in ordinary and crisis situations. Multivariate linear regression analysis was performed on hospital resource variables related to surge capacity. @*Results@#In the second half of 2018, a survey was conducted targeting 31 EMC directors in Seoul. It was found that all regional and local EMCs in Seoul can accommodate 848 emergency patients and 537 non-emergency patients in crisis conditions. In ordinary situations, one EMC could accommodate an average of 1.3 patients with Korean Triage and Acuity Scale (KTAS) level 1, 3.1 patients with KTAS level 2, and 5.7 patients with KTAS level 3. In situations of crisis, this number increased to 3.4, 7.8, and 16.2, respectively. There are significant differences in surge capacity between ordinary and crisis conditions. The difference in surge capacity between regional and local EMC was not significant. In both ordinary and crisis conditions, only the total number of hospital beds were significantly associated with surge capacity. @*Conclusion@#If the hospital's emergency transport system is ideally accomplished, patients arising from average MCI can be accommodated in Seoul. However, in a huge disaster, it may be challenging to handle the current surge capacity. More detailed follow-up studies are needed to prepare a surge capacity protocol in the community.

5.
Article in Korean | WPRIM | ID: wpr-893556

ABSTRACT

The study aimed to review the current postpartum care service system and suggest measures of improvement for the public reinforcement of the private-postpartum care center (PCC). We conducted a review of literature, including an internet search, and we analyzed the in-depth interviews using questionnaires answered by workers from the 6 private-PCCs operated in Goyang city. Using these data, we derived possible measures which could improve the publicity of private-PCCs. It was found that the 11 nationwide public-PCCs had serious operating deficits, and its utilization by the vulnerable was insufficient. In areas where private-PCCs are concentrated, we can propose the following policies to strengthen publicity, rather than establishing another public-PCC. First, it is important to provide support for private-PCCs to apply cost reductions for vulnerable groups, similar to the maternal and newborn medical care of public-PCCs. Second, publicity would be strengthened by reorganizing the pregnancy and childbirth education program, while also offering additional services for mothers with stillbirths and abortions. For the integrated management of postpartum care services, this should be shifted towards reinforcing the publicity of private-PCCs, rather than operating a public-PCC in relatively concentrated private-PCC areas. Through these policies, it is expected that financial and administrative waste will be reduced, and an integrated management system for postpartum care services will be established.

6.
Article in English | WPRIM | ID: wpr-899883

ABSTRACT

Background@#Emergency departments (EDs) generally receive many casualties in disaster or mass casualty incidents (MCI). Some studies have conceptually suggested the surge capacity that ED should have; however, only few studies have investigated measurable numbers in one community. This study investigated the surge capacity of the specific number of accommodatable patients and overall preparedness at EDs in a metropolitan city. @*Methods@#This cross-sectional study officially surveyed surge capacity and disaster preparedness for all regional and local emergency medical centers (EMC) in Seoul with the Seoul Metropolitan Government's public health division. This study developed survey items on space, staff, stuff, and systems, which are essential elements of surge capacity. The number of patients acceptable for each ED was investigated by triage level in ordinary and crisis situations. Multivariate linear regression analysis was performed on hospital resource variables related to surge capacity. @*Results@#In the second half of 2018, a survey was conducted targeting 31 EMC directors in Seoul. It was found that all regional and local EMCs in Seoul can accommodate 848 emergency patients and 537 non-emergency patients in crisis conditions. In ordinary situations, one EMC could accommodate an average of 1.3 patients with Korean Triage and Acuity Scale (KTAS) level 1, 3.1 patients with KTAS level 2, and 5.7 patients with KTAS level 3. In situations of crisis, this number increased to 3.4, 7.8, and 16.2, respectively. There are significant differences in surge capacity between ordinary and crisis conditions. The difference in surge capacity between regional and local EMC was not significant. In both ordinary and crisis conditions, only the total number of hospital beds were significantly associated with surge capacity. @*Conclusion@#If the hospital's emergency transport system is ideally accomplished, patients arising from average MCI can be accommodated in Seoul. However, in a huge disaster, it may be challenging to handle the current surge capacity. More detailed follow-up studies are needed to prepare a surge capacity protocol in the community.

7.
Article in Korean | WPRIM | ID: wpr-901260

ABSTRACT

The study aimed to review the current postpartum care service system and suggest measures of improvement for the public reinforcement of the private-postpartum care center (PCC). We conducted a review of literature, including an internet search, and we analyzed the in-depth interviews using questionnaires answered by workers from the 6 private-PCCs operated in Goyang city. Using these data, we derived possible measures which could improve the publicity of private-PCCs. It was found that the 11 nationwide public-PCCs had serious operating deficits, and its utilization by the vulnerable was insufficient. In areas where private-PCCs are concentrated, we can propose the following policies to strengthen publicity, rather than establishing another public-PCC. First, it is important to provide support for private-PCCs to apply cost reductions for vulnerable groups, similar to the maternal and newborn medical care of public-PCCs. Second, publicity would be strengthened by reorganizing the pregnancy and childbirth education program, while also offering additional services for mothers with stillbirths and abortions. For the integrated management of postpartum care services, this should be shifted towards reinforcing the publicity of private-PCCs, rather than operating a public-PCC in relatively concentrated private-PCC areas. Through these policies, it is expected that financial and administrative waste will be reduced, and an integrated management system for postpartum care services will be established.

8.
Article in English | WPRIM | ID: wpr-913483

ABSTRACT

Purpose@#There are few reports on the therapeutic effects of gonadotropin-releasing hormone agonists in boys with central precocious puberty, and studies reported in Korea are very rare. We aimed to assess the significance of clinical factors and the effects of gonadotropin-releasing hormone agonist treatment on final adult height in boys diagnosed with central precocious puberty. @*Methods@#We retrospectively evaluated the medical records of 18 boys treated for idiopathic central precocious puberty between 2007 and 2018 at Chosun University Hospital. Gestational age, birth weight, and parental height were assessed at the initial visit. Chronological age, bone age, bone age/chronological age ratio, height and height standard deviation scores, predicted adult height, body mass index, and hormone levels were assessed during the treatment period. @*Results@#At the time of diagnosis, the chronological age was 9.9±0.6 years, the bone age was 11.6±1.0 years, and the bone age/chronological age ratio was 1.20±0.1. The bone age/chronological age ratio decreased significantly to 1.12±0.1 at the end of treatment (P<0.05). The luteinizing hormone/follicular stimulating hormone ratios were 3.4±1.2, 0.6±0.4, and 0.6±1.0 at the start of treatment, after 1 year of treatment, and at the end of treatment, respectively. After gonadotropin-releasing hormone agonist treatment, the final adult height reached 172.0±4.8 cm compared to the target height range of 171.0±4.0 cm. @*Conclusion@#In boys with central precocious puberty, gonadotropin-releasing hormone agonist treatment improved growth potential.

9.
Psychiatry Investigation ; : 1164-1170, 2021.
Article in English | WPRIM | ID: wpr-918715

ABSTRACT

Objective@#This study aims to understand borderline personality disorder (BPD) features by employing the Personality Psychopathology Five (PSY-5) scales from the Minnesota Multiphasic Personality Inventory-2 (MMPI-2). @*Methods@#A total of 156 psychiatric patients completed PSY-5 scales of MMPI-2 and Personality Assessment Inventory-Borderline Subscale (PAI-BOR). Pearson’s partial correlation analysis was conducted to control the impact of age and gender and to determine the relationship between PSY-5 scales and BOR. A hierarchical multiple regression analysis was implemented to examine whether PSY-5 scales predicted the BOR-total, and a path analysis was performed to determine whether PSY-5 scales predicted each PAI-BOR subscale. @*Results@#The BOR-total score had a significant correlation with all PSY-5 scores, even after controlling for age and gender. However, only aggressiveness (AGGR), disconstraint (DISC), negative emotionalityeuroticism (NEGE), and introversion/low positive emotionality (INTR), excluding psychoticism (PSYC), significantly predicted BOR-total. The path analysis indicates that PSYC did not predict any BOR subscale, while NEGE predicted all BOR subscales. @*Conclusion@#The study findings indicate that NEGE best reflects BPD features, while PSYC is far from the core domain that describes BPD. In addition, the influence of age should be considered when understanding BPD, since age predicted the BOR-total and two BOR subscales.

10.
Article in English | WPRIM | ID: wpr-920187

ABSTRACT

Background and Objectives@#Auditory training involves active listening to auditory stimuli, and it has garnered attention in recent years because it enhances speech-in-noise recognition and the satisfaction of hearing aids. The purpose of this study was to determine the efficacy of a closed-set auditory training protocol for adult hearing aid users. We also evaluated the retention effect of training at a 1-month follow-up test after the completion of training. @*Subjects and Methods@#Twenty-two hearing-impaired listeners who have used bilateral hearing aids for more than two months participated in this study. Out of 22 participants, 12 participants (training group, TG) received an 8-week auditory training while 10 participants did not receive any training (non-training group, NTG). For training, three types of closed-set training materials (environmental sounds, words, and sentences) were used. The training was conducted eight times over eight weeks (one session per week, about one hour per session). The difficulty level was adjusted by controlling the number of closed-set choices and the signal-to-noise ratios. To determine the efficacy of training, open-set speech recognition abilities and subjective hearing aid satisfaction were evaluated. @*Results@#All the open-set speech recognition performances of the TG were enhanced after the closed-set auditory training, while the performance of the NTG was not significantly changed. The auditory training had a positive impact on the TG individuals’ subjective satisfaction of hearing aids. The improvement from training was maintained over one month after the completion of the auditory training. @*Conclusion@#The closed-set auditory training protocol might lead to enhanced speech understanding as well as more satisfaction with hearing aids for adult hearing aid users.

11.
Article in Korean | WPRIM | ID: wpr-920193

ABSTRACT

Background and Objectives@#A Korean Matrix Sentence Test is one of the adaptive tests used to evaluate speech intelligibility in noise. The present study aimed to investigate the influence of measurement procedure (presentation level and mode) on the Korean matrix sentence-in-noise intelligibility of normal-hearing listeners.Subjects and Method Twenty-seven normal-hearing adults participated in the study. The speech reception threshold (SRT) was determined using a software-based adaptive procedure, which converges on the signal-to-noise ratio (SNR), corresponding to 50% intelligibility. The speech or speech-shaped noise was given at three presentation levels based on the previous categorical loudness judgments (between soft and medium: 55 dB SPL, medium: 65 dB SPL, and loud level: 80 dB SPL). The effect of the presentation mode was explored by fixing either the sentence level or noise level, while the noise or sentence level was adaptively adjusted according to the subject’s response. @*Results@#The presentation level significantly affected the SRTs, whereas the SRTs did not depend on the presentation mode. The sentence-in-noise intelligibility was similar between 55 and 65 dB SPL, yet the sentence-in-noise intelligibility significantly decreased at 80 dB SPL regardless of the presentation mode. Correlation analyses showed moderate to high positive correlations among the results of two presentation modes. @*Conclusion@#A presentation level of 55 or 65 dB SPL yielded comparable adaptive measurement results for normal-hearing subjects, yet the sentence-in-nose intelligibility became worse at 80 dB SPL. Clinicians should be careful with the influence of the presentation level when identifying speech-in-noise intelligibility.

12.
Article in Korean | WPRIM | ID: wpr-920238

ABSTRACT

Background and Objectives@#Although sound-field speech recognition test is an essential evaluation method to verify the benefit of hearing aids, the aided sound-field speech recognition test has not been used as a standard audiometric protocol. This study investigated whether a traditional measure of unaided speech recognition measured at an individual’s most comfortable level (MCL) can predict aided speech recognition performance and the benefit of hearing aids.Subjects and Method One hundred twenty-one ears (72 adult hearing aid users) with moderate, moderately severe, and severe degrees of hearing loss were tested. The word recognition score (WRS) and sentence recognition score (SRS) were measured at MCLs using earphones (earphone WRS and earphone SRS). From the sound-field speech audiometry, both unaided and aided WRS and SRS were evaluated at an average conversational speech level (at 65 dB SPL) using a loudspeaker (located at 0° azimuth, 1 m). This study determined the differences between earphone scores (at MCL) and aided recognition scores (at 65 dB SPL), which is known as earphone to aided difference (EAD). Also, the hearing aid benefit was simply derived by differences between the unaided and aided scores, both measured at 65 dB SPL. @*Results@#The differences between the unaided and aided scores were significantly lower with a higher degree of hearing loss, yet the earphone scores measured at MCLs did not differ across varied hearing loss. More than half of the tested ears had the EAD of greater than ±10%. The ears with a higher degree of hearing loss tended to have +EAD (earphone score>aided score), whereas the ears with less hearing loss showed -EAD (earphone score≤aided score). The earphone score measured at MCLs was not a predominant contributor to predict either aided performance or hearing aid benefit. @*Conclusion@#Clinicians should not assume that the unaided speech recognition score measured with earphones at MCL can accurately predict aided speech recognition or the benefit of hearing aids. Instead, it is crucial to directly assess aided sound-field speech recognition performance at a conversational speech level.

13.
Article in English | WPRIM | ID: wpr-875137

ABSTRACT

With the increase in the number of cases of silicone implant insertion either for cosmetic surgery or breast reconstruction after mastectomy, it is not unusual to encounter patients with silicone implants in clinical settings. Recently, the first case of breast implant-associated anaplastic large cell lymphoma was reported in Korea. In addition to previously known complications, such as implant rupture or contracture, the number of implant-associated imaging examinations has also increased. Considering this background, radiologists should have sufficient knowledge about the type of examination required in patients who have undergone implant insertion and imaging findings to correctly identify implant-associated complications. In this article, various complications of silicone implants are discussed, including various imaging findings, which radiologists should know.

14.
Article in English | WPRIM | ID: wpr-896710

ABSTRACT

The first case of coronavirus disease 2019 (COVID-19) in Korea was reported in January 2020.As the secondary transmissions accelerated within the country, the government revised the outbreak alert for COVID-19 from attention to caution. Mid-February, when a massive outbreak was reported from a church in Daegu, our institution initiated testing for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). More than 300 laboratory tests were performed within the first 2 months, before the number of cases began to decline. Here, we describe our experience of 4 months at the department of Laboratory Medicine, Keimyung University Dongsan Hospital, located in Daegu, where a massive COVID-19 outbreak occurred.

15.
Article in 0 | WPRIM | ID: wpr-835565

ABSTRACT

Background and Objectives@#The Gaps-In-Noise (GIN) test is a clinically effective measure of the integrity of the central auditory nervous system. The GIN procedure can be applied to a pediatric population above 7 years of age. The present study conducted the GIN test to compare the abilities of auditory temporal resolution among typically developing children, children with speech sound disorder (SSD), and children with cognitive difficulty (CD). @*Subjects and Methods@#Children aged 8 to 11 years-(total n=30) participated in this study. There were 10 children in each of the following three groups: typically developing children, children with SSD, and children with CD. The Urimal Test of Articulation and Phonology was conducted as a clinical assessment of the children’s articulation and phonology. The Korean version of the Wechsler Intelligence Scale for Children-III (K-WISC-III) was administered as a screening test for general cognitive function. According to the procedure of Musiek, the pre-recorded stimuli of the GIN test were presented at 50 dB SL. The results were scored by the approximated threshold and the overall percent correct score (%). @*Results@#All the typically developing children had normal auditory temporal resolution based on the clinical cutoff criteria of the GIN test. The children with SSD or CD had significantly reduced gap detection performance compared to age-matched typically developing children. The children’s intelligence score measured by the K-WISC-III test explained 37% of the variance in the percent-correct score. @*Conclusions@#Children with SSD or CD exhibited poorer ability to resolve rapid temporal acoustic cues over time compared to the age-matched typically developing children. The ability to detect a brief temporal gap embedded in a stimulus may be related to the general cognitive ability or phonological processing.

16.
Article in 0 | WPRIM | ID: wpr-835571

ABSTRACT

Background and Objectives@#Although many studies have evaluated the effect of the digital noise reduction (DNR) algorithm of hearing aids (HAs) on speech recognition, there are few studies on the effect of DNR on music perception. Therefore, we aimed to evaluate the effect of DNR on music, in addition to speech perception, using objective and subjective measurements. @*Subjects and Methods@#Sixteen HA users participated in this study (58.00±10.44 years; 3 males and 13 females). The objective assessment of speech and music perception was based on the Korean version of the Clinical Assessment of Music Perception test and word and sentence recognition scores. Meanwhile, for the subjective assessment, the quality rating of speech and music as well as self-reported HA benefits were evaluated. @*Results@#There was no improvement conferred with DNR of HAs on the objective assessment tests of speech and music perception. The pitch discrimination at 262 Hz in the DNR-off condition was better than that in the unaided condition (p=0.024); however, the unaided condition and the DNR-on conditions did not differ. In the Korean music background questionnaire, responses regarding ease of communication were better in the DNR-on condition than in the DNR-off condition (p=0.029). @*Conclusions@#Speech and music perception or sound quality did not improve with the activation of DNR. However, DNR positively influenced the listener’s subjective listening comfort. The DNR-off condition in HAs may be beneficial for pitch discrimination at some frequencies.

17.
Article in English | WPRIM | ID: wpr-889006

ABSTRACT

The first case of coronavirus disease 2019 (COVID-19) in Korea was reported in January 2020.As the secondary transmissions accelerated within the country, the government revised the outbreak alert for COVID-19 from attention to caution. Mid-February, when a massive outbreak was reported from a church in Daegu, our institution initiated testing for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). More than 300 laboratory tests were performed within the first 2 months, before the number of cases began to decline. Here, we describe our experience of 4 months at the department of Laboratory Medicine, Keimyung University Dongsan Hospital, located in Daegu, where a massive COVID-19 outbreak occurred.

18.
Laboratory Medicine Online ; : 144-151, 2020.
Article in English | WPRIM | ID: wpr-1045696

ABSTRACT

Background@#Hematologic malignancies have a relatively lower prevalence than major solid cancers, although the incidence of hematologic malignancies has significantly increased in recent years. However, understanding the current status of hematologic malignancy is significantly challenging because basic data regarding this malignancy are insufficient in the Korean population. @*Methods@#From 2005 to 2015, the status of seven codes of hematologic malignancy, containing 24 subcodes defined using a classification defined by the Korean Classification of Disease-6, was analyzed. The number of new patients, crude incidence rate, prevalence rate, and age-standardized incidence rate were also investigated. Results were analyzed based on National Health Insurance Service (NHIS) data. @*Results@#The number of new patients showed an overall increase over time and a rate of increase up to 56.7% for 10 years. The number of male patients was higher than that of female patients, with the majority of patients aged greater than 60 years. The incidence and prevalence rates have increased steadily. @*Conclusions@#Consistent with the previous studies, this study might be useful to understand the current status of hematologic malignancy and might contribute to the improvement of national public healthcare.

19.
Article | WPRIM | ID: wpr-830463

ABSTRACT

Purpose@#The clinical significance of birth weight relative to gestational age in girls with central precocious puberty is unclear. This study sought to compare clinical parameters such as final adult height (FAH) and menarche onset after treatment with gonadotropin-releasing hormone agonist (GnRHa) on birth weight in girls with central precocious puberty treated. @*Methods@#This retrospective study reviewed data of 69 girls with precocious puberty who had reached their FAH in a long-term trial of GnRHa treatment between January 2007 and December 2017. The subjects were divided into small for gestational age (SGA) (n=19) and appropriate for gestational age (AGA) (n=50) groups. @*Results@#When starting GnRHa treatment, bone age was 10.9±0.9 and 10.3±0.8 years in the SGA and AGA groups, respectively (P<0.05). The predicted adult height (PAH) (established according to the Bayley-Pinneau average table) and advanced PAH (established according to the Bayley-Pinneau advanced table) were 151.5±4.8 cm and 155.8±4.9 cm in the SGA group, respectively, and 153.4±5.3 cm and 159.0±6.0 cm in the AGA group. After treatment, no significant difference in bone age was found between the groups. The time to menarche after treatment was 12.5±7.6 and 21.1±12.3 months in the SGA and AGA groups, respectively (P<0.05). FAH in the SGA and AGA groups was 161.0±4.7 cm and 161.6±5.0 cm, respectively, without a significant difference. @*Conclusion@#SGA girls with precocious puberty have increased bone age and earlier menarche relative to AGA girls. However, no difference in FAH after treatment was found between these groups.

20.
Article in 0 | WPRIM | ID: wpr-831549

ABSTRACT

Background@#Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. @*Methods@#We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. @*Results@#A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. @*Conclusion@#In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

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