1.Neonatal Diabetes Mellitus Due to KCNJ11 (KIR6.2) Mutation Successfully Treated with Sulfonylurea
Sehun JANG ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG ; Won Soon PARK
Neonatal Medicine 2021;28(2):94-98
Neonatal diabetes mellitus (NDM) is a rare disease that occurs at less than 6 months of age and is presumably caused by a mutation in the gene that affects pancreatic beta-cell function. Approximately 80% of NDM cases reveal a known genetic mutation, and mutations in potassium inwardly rectifying channel subfamily J member 11 (KCNJ11) and ABCC8 affecting the pancreatic beta-cell adenosine triphosphate-sensitive potassium channel may be treated with oral sulfonylurea. Early recognition of mutations in KCNJ11 and ABCC8 is important because early administration of sulfonylurea can not only control blood glucose levels but also improve neurodevelopmental outcomes. In the present study, we report a case of NDM that initially presented as diabetic ketoacidosis at the age of 1 month, accompanied by seizures during hospitalization. After confirmation of the KCNJ11 gene mutation (c.989A>C), we started administering oral sulfonylurea (glimepiride) at the age of 2 months. After gradually increasing the dosage of glimepiride, insulin was discontinued at the age of 3 months. To date, the infant’s blood glucose levels have been well controlled without significant hypoglycemic events. No further episodes of seizures have occurred, and his developmental status is favorable.
2.Neonatal Diabetes Mellitus Due to KCNJ11 (KIR6.2) Mutation Successfully Treated with Sulfonylurea
Sehun JANG ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG ; Won Soon PARK
Neonatal Medicine 2021;28(2):94-98
Neonatal diabetes mellitus (NDM) is a rare disease that occurs at less than 6 months of age and is presumably caused by a mutation in the gene that affects pancreatic beta-cell function. Approximately 80% of NDM cases reveal a known genetic mutation, and mutations in potassium inwardly rectifying channel subfamily J member 11 (KCNJ11) and ABCC8 affecting the pancreatic beta-cell adenosine triphosphate-sensitive potassium channel may be treated with oral sulfonylurea. Early recognition of mutations in KCNJ11 and ABCC8 is important because early administration of sulfonylurea can not only control blood glucose levels but also improve neurodevelopmental outcomes. In the present study, we report a case of NDM that initially presented as diabetic ketoacidosis at the age of 1 month, accompanied by seizures during hospitalization. After confirmation of the KCNJ11 gene mutation (c.989A>C), we started administering oral sulfonylurea (glimepiride) at the age of 2 months. After gradually increasing the dosage of glimepiride, insulin was discontinued at the age of 3 months. To date, the infant’s blood glucose levels have been well controlled without significant hypoglycemic events. No further episodes of seizures have occurred, and his developmental status is favorable.
3.Current Status and Associated Factors of Post-Hemorrhagic Hydrocephalus in Infants of 22 to 28 Weeks Gestation With Severe Intraventricular Hemorrhage in Korea: A Nationwide Cohort Study
Misun YANG ; Sumin KIM ; Se In SUNG ; Yun Sil CHANG ; Won Soon PARK ; So Yoon AHN
Journal of Korean Medical Science 2024;39(15):e139-
Background:
Post-hemorrhagic hydrocephalus (PHH), a common complication of severe intraventricular hemorrhage (IVH) in very low birth weight (BW) infants, is associated with significant morbidity and poor neurological outcomes. The objective of this study was to assess the current status of PHH and analyze the risk factors associated with the necessity of treatment for PHH in infants born between 22 and 28 weeks of gestation, specifically those with severe IVH (grade 3 or 4).
Methods:
The analysis was conducted on 1,097 infants who were born between 22–28 gestational weeks and diagnosed with severe IVH, using data from the Korean Neonatal Network. We observed that the prevalence of PHH requiring treatment was 46.3% in infants with severe IVH.
Results:
Higher rates of mortality, transfer during admission, cerebral palsy, and ventriculoperitoneal shunt after discharge were higher in infants with PHH than in those without PHH. PHH in severe IVH was associated with a higher rate of pulmonary hemorrhage, seizures, and IVH grade 4 in the entire cohort. In addition, it was associated with a lower rate of small for gestational age and chorioamnionitis. In the subgroup analysis, high BW, outborn status, pulmonary hemorrhage, seizure, sepsis, and IVH grade 4 were associated with a higher incidence of PHH between 22 and 25 gestational weeks (GW). In infants born between 26 and 28 GW, a higher incidence of PHH was associated with seizures and IVH grade 4.
Conclusion
It is necessary to maintain meticulous monitoring and neurological intervention for infants with PHH not only during admission but also after discharge. In addition, identifying the clinical factors that increase the likelihood of developing PHH from severe IVH is crucial.
4.A Network Analysis of 15O-H2O PET Reveals Deep Brain Stimulation Effects on Brain Network of Parkinson's Disease.
Hae Jeong PARK ; Bumhee PARK ; Hae Yu KIM ; Maeng Keun OH ; Joong Il KIM ; Misun YOON ; Jong Doo LEE ; Jin Woo CHANG
Yonsei Medical Journal 2015;56(3):726-736
PURPOSE: As Parkinson's disease (PD) can be considered a network abnormality, the effects of deep brain stimulation (DBS) need to be investigated in the aspect of networks. This study aimed to examine how DBS of the bilateral subthalamic nucleus (STN) affects the motor networks of patients with idiopathic PD during motor performance and to show the feasibility of the network analysis using cross-sectional positron emission tomography (PET) images in DBS studies. MATERIALS AND METHODS: We obtained [15O]H2O PET images from ten patients with PD during a sequential finger-to-thumb opposition task and during the resting state, with DBS-On and DBS-Off at STN. To identify the alteration of motor networks in PD and their changes due to STN-DBS, we applied independent component analysis (ICA) to all the cross-sectional PET images. We analysed the strength of each component according to DBS effects, task effects and interaction effects. RESULTS: ICA blindly decomposed components of functionally associated distributed clusters, which were comparable to the results of univariate statistical parametric mapping. ICA further revealed that STN-DBS modifies usage-strengths of components corresponding to the basal ganglia-thalamo-cortical circuits in PD patients by increasing the hypoactive basal ganglia and by suppressing the hyperactive cortical motor areas, ventrolateral thalamus and cerebellum. CONCLUSION: Our results suggest that STN-DBS may affect not only the abnormal local activity, but also alter brain networks in patients with PD. This study also demonstrated the usefulness of ICA for cross-sectional PET data to reveal network modifications due to DBS, which was not observable using the subtraction method.
Aged
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Brain/*radionuclide imaging
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Cross-Sectional Studies
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Deep Brain Stimulation/*methods
;
Female
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Functional Laterality/*physiology
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Humans
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Male
;
Middle Aged
;
Parkinson Disease/radionuclide imaging/*therapy
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Positron-Emission Tomography
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Severity of Illness Index
;
Subthalamic Nucleus/*physiopathology
5.The Impact of Surgical Intervention on Neurodevelopmental Outcomes in Very Low Birth Weight Infants: a Nationwide Cohort Study in Korea
Se In SUNG ; Na Hyun LEE ; Hyun Ho KIM ; Hye Seon KIM ; Yea Seul HAN ; Misun YANG ; So Yoon AHN ; Yun Sil CHANG ; Won Soon PARK
Journal of Korean Medical Science 2019;34(43):e271-
BACKGROUND: To investigate the incidence of surgical intervention in very low birth weight (VLBW) infants and the impact of surgery on neurodevelopmental outcomes at corrected ages (CAs) of 18–24 months, using data from the Korean Neonatal Network (KNN). METHODS: Data from 7,885 VLBW infants who were born and registered with the KNN between 2013 to 2016 were analyzed in this study. The incidences of various surgical interventions and related morbidities were analyzed. Long-term neurodevelopmental outcomes at CAs of 18–24 months were compared between infants (born during 2013 to 2015, n = 3,777) with and without surgery. RESULTS: A total of 1,509 out of 7,885 (19.1%) infants received surgical interventions during neonatal intensive care unit (NICU) hospitalization. Surgical ligation of patent ductus arteriosus (n = 840) was most frequently performed, followed by laser therapy for retinopathy of prematurity and laparotomy due to intestinal perforation. Infants who underwent surgery had higher mortality rates and greater neurodevelopmental impairment than infants who did not undergo surgery (P value < 0.01, both). On multivariate analysis, single or multiple surgeries increased the risk of neurodevelopmental impairment compared to no surgery with adjusted odds ratios (ORs) of 1.6 with 95% confidence interval (CI) of 1.1–2.6 and 2.3 with 95% CI of 1.1–4.9. CONCLUSION: Approximately one fifth of VLBW infants underwent one or more surgical interventions during NICU hospitalization. The impact of surgical intervention on long-term neurodevelopmental outcomes was sustained over a follow-up of CA 18–24 months. Infants with multiple surgeries had an increased risk of neurodevelopmental impairment compared to infants with single surgeries or no surgeries after adjustment for possible confounders.
Cohort Studies
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Ductus Arteriosus, Patent
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Follow-Up Studies
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Hospitalization
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Humans
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Incidence
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Infant
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Infant, Newborn
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Infant, Very Low Birth Weight
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Intensive Care, Neonatal
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Intestinal Perforation
;
Korea
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Laparotomy
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Laser Therapy
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Ligation
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Mortality
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Multivariate Analysis
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Odds Ratio
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Retinopathy of Prematurity
6.Continuous Renal Replacement Therapy in Preterm Infants
Eu Seon NOH ; Hyun Ho KIM ; Hye Seon KIM ; Yea Seul HAN ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG ; Won Soon PARK
Yonsei Medical Journal 2019;60(10):984-991
PURPOSE: Despite the increasing use of continuous renal replacement therapy (CRRT) in the neonatal intensive care unit (NICU), few studies have investigated its use in preterm infants. This study evaluated the prognosis of preterm infants after CRRT and identified risk factors of mortality after CRRT. MATERIALS AND METHODS: A retrospective review was performed in 33 preterm infants who underwent CRRT at the NICU of Samsung Medical Center between 2008 and 2017. Data of the demographic characteristics, predisposing morbidity, cardiopulmonary function, and CRRT were collected and compared between surviving and non-surviving preterm infants treated with CRRT. Univariable and multivariable analyses were performed to identify factors affecting mortality. RESULTS: Compared with the survivors, the non-survivors showed younger gestational age (29.3 vs. 33.6 weeks), lower birth weight (1359 vs. 2174 g), and lower Apgar scores at 1 minute (4.4 vs. 6.6) and 5 minutes (6.5 vs. 8.6). At the initiation of CRRT, the non-survivors showed a higher incidence of inotropic use (93% vs. 40%, p=0.017) and fluid overload (16.8% vs. 4.0%, p=0.031). Multivariable analysis revealed that fluid overload >10% at CRRT initiation was the primary determinant of mortality after CRRT in premature infants, with an adjusted odds ratio of 14.6 and a 95% confidence interval of 1.10–211.29. CONCLUSION: Our data suggest that the degree of immaturity, cardiopulmonary instability, and fluid overload affect the prognosis of preterm infants after CRRT. Preventing fluid overload and earlier initiation of CRRT may improve treatment outcomes.
Birth Weight
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Gestational Age
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Humans
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Incidence
;
Infant, Newborn
;
Infant, Premature
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Intensive Care, Neonatal
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Mortality
;
Odds Ratio
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Prognosis
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Renal Replacement Therapy
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Retrospective Studies
;
Risk Factors
;
Survivors
7.Comparison of Respiratory Outcomes between Less Invasive Surfactant Administration and the IntubationSurfactant-Extubation Technique in Premature Infants with Respiratory Distress Syndrome
Hye Seon KIM ; Hyun Ho KIM ; Misun YANG ; Yea Seul HAN ; Se In SUNG ; So Yoon AHN ; Yun Sil CHANG ; Won Soon PARK
Neonatal Medicine 2020;27(3):99-104
Purpose:
To compare respiratory outcomes between less invasive surfactant admi nistration (LISA) and the intubation-surfactant-extubation (INSURE) technique in premature infants with respiratory distress syndrome (RDS).
Methods:
We performed a retrospective medical chart review for 75 premature in fants who were born at a gestational age (GA) of ≤34 weeks (between January 2017 and December 2019) and developed RDS after birth. Data on the demographic and outcome variables, including respiratory outcomes, were collected and compared between the infants who received LISA and those who received INSURE as a rescue therapy for RDS.
Results:
No signifcant differences in GA, birth weight, and other demographic characteristics were found between the LISA and INSURE groups (GA: 28.7 weeks vs.28.8 weeks, P=0.449; birth weight: 1,236 g vs. 1,124 g, P=0.714). At the delivery room, although the infants showed no significant difference in positive pressure ventilation rate after birth, the LISA group showed a higher rate of continuous positive airway pressure application than the INSURE group. The infants in the LISA group presented a higher risk of requiring multiple doses of surfactant for RDS than the infants in the INSURE group (57% vs. 17.5%, P=0.001). However, the duration of invasive and/ or noninvasive respiratory support and incidence of bronchopulmonary dysplasia showed no signifciant difference between the two groups.
Conclusion
In the present study, no significant differences in the incidence of inhospital respiratory outcomes such as bronchopulmonary dysplasia were found between the LISA and INSURE groups. These results suggest that LISA can be an alternative therapeutic option for treating RDS to avoid intubation and mechanica ventilation in premature infants.
8.Mortality and Morbidities according to Time of Birth in Extremely Low Birth Weight Infants
Misun YANG ; So Yoon AHN ; Heui Seung JO ; Se In SUNG ; Yun Sil CHANG ; Won Soon PARK ; Korean Neonatal Network
Journal of Korean Medical Science 2021;36(13):e86-
Background:
Although the overall quality of high-risk neonatal care has improved recently, there is still concern about a difference in the quality of care when comparing off-hour births and regular-hour births. Moreover, there are no data in Korea regarding the impact of time of birth on mortality and morbidities in preterm infants.
Methods:
A total of 3,220 infants weighing < 1,000 g and born at 23–34 weeks in 2013–2017 were analyzed based on the Korean Neonatal Network data. Mortality and major morbidities were analyzed using logistic regression according to time of birth during off-hours (nighttime, weekend, and holiday) and regular hours. The institutes were sub-grouped into hospital group I and hospital group II based on the neonatal intensive care unit (NICU) care level defined by the mortality rates of < 50% and ≥ 50%, respectively, in infants born at 23–24 weeks' gestation.
Results:
The number of births during regular hours and off-hours was similar. In the total population and hospital group I, off-hour births were not associated with increased neonatal mortality and morbidities. However, in hospital group II, increased early mortality was found in the off-hour births when compared to regular-hour births.
Conclusion
Efforts to improve the overall quality of NICU are required to lower the early mortality rate in off-hour births. Also, other sensitive indexes for the evaluation of quality of NICU care should be further studied.
9.Mortality and Morbidities according to Time of Birth in Extremely Low Birth Weight Infants
Misun YANG ; So Yoon AHN ; Heui Seung JO ; Se In SUNG ; Yun Sil CHANG ; Won Soon PARK ; Korean Neonatal Network
Journal of Korean Medical Science 2021;36(13):e86-
Background:
Although the overall quality of high-risk neonatal care has improved recently, there is still concern about a difference in the quality of care when comparing off-hour births and regular-hour births. Moreover, there are no data in Korea regarding the impact of time of birth on mortality and morbidities in preterm infants.
Methods:
A total of 3,220 infants weighing < 1,000 g and born at 23–34 weeks in 2013–2017 were analyzed based on the Korean Neonatal Network data. Mortality and major morbidities were analyzed using logistic regression according to time of birth during off-hours (nighttime, weekend, and holiday) and regular hours. The institutes were sub-grouped into hospital group I and hospital group II based on the neonatal intensive care unit (NICU) care level defined by the mortality rates of < 50% and ≥ 50%, respectively, in infants born at 23–24 weeks' gestation.
Results:
The number of births during regular hours and off-hours was similar. In the total population and hospital group I, off-hour births were not associated with increased neonatal mortality and morbidities. However, in hospital group II, increased early mortality was found in the off-hour births when compared to regular-hour births.
Conclusion
Efforts to improve the overall quality of NICU are required to lower the early mortality rate in off-hour births. Also, other sensitive indexes for the evaluation of quality of NICU care should be further studied.
10.The COVID-19 Pandemic Response System at University Level: The Case of Safe Campus Model at Ewha Womans University
Kyunghee Jung CHOI ; Nackmoon SUNG ; Sun Hwa LEE ; Misun CHANG ; Hee Jung CHOI ; Chung-Jong KIM ; Nam-Kyong CHOI ; Hanna KIM ; Yi-Jun KIM ; Whanhee LEE ; Hyesook PARK ; Eunhee HA
The Ewha Medical Journal 2022;45(4):e18-
In response to the changes in the Coronavirus disease 2019 (COVID-19) epidemic situation, Ewha Womans University established Ewha Safe Campus (ESC), an on-campus infection outbreak management system, to allow students and faculty members to safely resume face-to face classes in 2022. The COVID-19 testing station, Ewha Safe Station, is the core element of ESC. Symptomatic students and faculty members perform a combo swab self-PCR test or receive a nasopharyngeal swab PCR test from experts to prevent the spread of COVID-19 through early detection and management. ESC is significant in that it detects infection risks and proactively implements preemptive measures in a university. The COVID-19 health response system model at the university level was applied for the first time in South Korea, reaching a milestone in the history of university health in South Korea. In particular, it is highly valuable that the test was free of charge, as it enabled all of the examinees to have easy access to the test through joint cooperation with the Seegene Medical Foundation. This is a successful example of cooperation between schools and private institutions for public health improvement. In the future, the direct and indirect effects of the establishment and implementation of ESC need to be evaluated and confirmed, and areas requiring improvements need to be identified in preparation for another infectious disease outbreak in the future.