1.A Case Report of Alobar Holoprosencepha ly with Cyclopia and Arrhinia: The Long est-Surviving Infant without Life-Sustain ing Interventions
Yejun LEE ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG
Perinatology 2024;35(4):146-151
Holoprosencephaly (HPE) is a rare congenital disorder characterized by the incomplete separation of the prosencephalon. The clinical spectrum of HPE depends on the subtypes observed on imaging, which includes lobar, semilobar, and alobar variants. Cyclopia, the most severe form of alobar HPE, is typically associated with early postnatal death within a few days. We report the case of a female infant was born at 38 weeks and 6 days of gestation, weighing 2,410 g, to a 39-year-old mother via vaginal delivery. She was the second baby of dichorionic-diamniotic twins. The neonate was born with multiple defects including facial anomalies such as cyclopia and arrhinia and was diagnosed with alobar HPE with cyclopia. This condition was suspected prenatally at 17 weeks of gestation and confirmed postnatally using brain magnetic resonance imaging. The cause of death in our patient was aspiration pneumonia with aggravated central apnea, and she survived for 8 months (240 days) with comfort care alone, without aggressive life support. This is the first report of an infant with alobar HPE, cyclopia, and arrhinia surviving for 240 days with only comfort care, marking the longest documented survival for this typically lethal condition without invasive interventions.
2.A Case Report of Alobar Holoprosencepha ly with Cyclopia and Arrhinia: The Long est-Surviving Infant without Life-Sustain ing Interventions
Yejun LEE ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG
Perinatology 2024;35(4):146-151
Holoprosencephaly (HPE) is a rare congenital disorder characterized by the incomplete separation of the prosencephalon. The clinical spectrum of HPE depends on the subtypes observed on imaging, which includes lobar, semilobar, and alobar variants. Cyclopia, the most severe form of alobar HPE, is typically associated with early postnatal death within a few days. We report the case of a female infant was born at 38 weeks and 6 days of gestation, weighing 2,410 g, to a 39-year-old mother via vaginal delivery. She was the second baby of dichorionic-diamniotic twins. The neonate was born with multiple defects including facial anomalies such as cyclopia and arrhinia and was diagnosed with alobar HPE with cyclopia. This condition was suspected prenatally at 17 weeks of gestation and confirmed postnatally using brain magnetic resonance imaging. The cause of death in our patient was aspiration pneumonia with aggravated central apnea, and she survived for 8 months (240 days) with comfort care alone, without aggressive life support. This is the first report of an infant with alobar HPE, cyclopia, and arrhinia surviving for 240 days with only comfort care, marking the longest documented survival for this typically lethal condition without invasive interventions.
3.A Case Report of Alobar Holoprosencepha ly with Cyclopia and Arrhinia: The Long est-Surviving Infant without Life-Sustain ing Interventions
Yejun LEE ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG
Perinatology 2024;35(4):146-151
Holoprosencephaly (HPE) is a rare congenital disorder characterized by the incomplete separation of the prosencephalon. The clinical spectrum of HPE depends on the subtypes observed on imaging, which includes lobar, semilobar, and alobar variants. Cyclopia, the most severe form of alobar HPE, is typically associated with early postnatal death within a few days. We report the case of a female infant was born at 38 weeks and 6 days of gestation, weighing 2,410 g, to a 39-year-old mother via vaginal delivery. She was the second baby of dichorionic-diamniotic twins. The neonate was born with multiple defects including facial anomalies such as cyclopia and arrhinia and was diagnosed with alobar HPE with cyclopia. This condition was suspected prenatally at 17 weeks of gestation and confirmed postnatally using brain magnetic resonance imaging. The cause of death in our patient was aspiration pneumonia with aggravated central apnea, and she survived for 8 months (240 days) with comfort care alone, without aggressive life support. This is the first report of an infant with alobar HPE, cyclopia, and arrhinia surviving for 240 days with only comfort care, marking the longest documented survival for this typically lethal condition without invasive interventions.
4.A Case Report of Alobar Holoprosencepha ly with Cyclopia and Arrhinia: The Long est-Surviving Infant without Life-Sustain ing Interventions
Yejun LEE ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG
Perinatology 2024;35(4):146-151
Holoprosencephaly (HPE) is a rare congenital disorder characterized by the incomplete separation of the prosencephalon. The clinical spectrum of HPE depends on the subtypes observed on imaging, which includes lobar, semilobar, and alobar variants. Cyclopia, the most severe form of alobar HPE, is typically associated with early postnatal death within a few days. We report the case of a female infant was born at 38 weeks and 6 days of gestation, weighing 2,410 g, to a 39-year-old mother via vaginal delivery. She was the second baby of dichorionic-diamniotic twins. The neonate was born with multiple defects including facial anomalies such as cyclopia and arrhinia and was diagnosed with alobar HPE with cyclopia. This condition was suspected prenatally at 17 weeks of gestation and confirmed postnatally using brain magnetic resonance imaging. The cause of death in our patient was aspiration pneumonia with aggravated central apnea, and she survived for 8 months (240 days) with comfort care alone, without aggressive life support. This is the first report of an infant with alobar HPE, cyclopia, and arrhinia surviving for 240 days with only comfort care, marking the longest documented survival for this typically lethal condition without invasive interventions.
5.A Case Report of Alobar Holoprosencepha ly with Cyclopia and Arrhinia: The Long est-Surviving Infant without Life-Sustain ing Interventions
Yejun LEE ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG
Perinatology 2024;35(4):146-151
Holoprosencephaly (HPE) is a rare congenital disorder characterized by the incomplete separation of the prosencephalon. The clinical spectrum of HPE depends on the subtypes observed on imaging, which includes lobar, semilobar, and alobar variants. Cyclopia, the most severe form of alobar HPE, is typically associated with early postnatal death within a few days. We report the case of a female infant was born at 38 weeks and 6 days of gestation, weighing 2,410 g, to a 39-year-old mother via vaginal delivery. She was the second baby of dichorionic-diamniotic twins. The neonate was born with multiple defects including facial anomalies such as cyclopia and arrhinia and was diagnosed with alobar HPE with cyclopia. This condition was suspected prenatally at 17 weeks of gestation and confirmed postnatally using brain magnetic resonance imaging. The cause of death in our patient was aspiration pneumonia with aggravated central apnea, and she survived for 8 months (240 days) with comfort care alone, without aggressive life support. This is the first report of an infant with alobar HPE, cyclopia, and arrhinia surviving for 240 days with only comfort care, marking the longest documented survival for this typically lethal condition without invasive interventions.
6.Fecal Calprotectin at Postinduction Is Capable of Predicting Persistent Remission and Endoscopic Healing after 1 Year of Treatment with Infliximab in Pediatric Patients with Crohn’s Disease
Yoo Min LEE ; Eun Sil KIM ; Sujin CHOI ; Hyo-Jeong JANG ; Yu Bin KIM ; So Yoon CHOI ; Byung-Ho CHOE ; Ben KANG
Gut and Liver 2024;18(3):498-508
Background/Aims:
The recent update on Selecting Therapeutic Targets in Inflammatory Bowel Disease initiative has added a decrease in fecal calprotectin (FC) to an acceptable range as an intermediate target for Crohn’s disease (CD). We aimed to investigate whether postinduction FC could predict future persistent remission (PR) and endoscopic healing (EH) after 1 year of treatment with infliximab (IFX) in pediatric patients with CD.
Methods:
This multicenter retrospective observational study included pediatric patients with CD who were followed up for at least 1 year after starting IFX. The association of postinduction FC with PR and EH was investigated.
Results:
A total of 132 patients were included in this study. PR and EH were observed in 71.2% (94/132) and 73.9% (82/111) of the patients, respectively. In multivariate logistic regression analysis, only the postinduction FC level was associated with PR (odds ratio [OR], 0.26; 95% confidence interval [CI], 0.08 to 0.66; p=0.009). The FC levels at initiation of IFX and postinduction were significantly associated with EH (OR, 0.73; 95% CI, 0.53 to 0.99; p=0.044 and OR, 0.20; 95% CI, 0.06 to 0.49; p=0.002, respectively). According to the receiver operating characteristic curve analysis, the optimal cutoff level for postinduction FC associated with PR was 122 mg/kg, and that associated with EH was 377 mg/kg.
Conclusions
Postinduction FC was associated with PR and EH after 1 year of treatment with IFX in pediatric patients with CD. Our findings emphasize the importance of FC as an intermediate target in the treat-to-target era.
7.Unenhanced Breast MRI With Diffusion-Weighted Imaging for Breast Cancer Detection: Effects of Training on Performance and Agreement of Subspecialty Radiologists
Yeon Soo KIM ; Su Hyun LEE ; Soo-Yeon KIM ; Eun Sil KIM ; Ah Reum PARK ; Jung Min CHANG ; Vivian Youngjean PARK ; Jung Hyun YOON ; Bong Joo KANG ; Bo La YUN ; Tae Hee KIM ; Eun Sook KO ; A Jung CHU ; Jin You KIM ; Inyoung YOUN ; Eun Young CHAE ; Woo Jung CHOI ; Hee Jeong KIM ; Soo Hee KANG ; Su Min HA ; Woo Kyung MOON
Korean Journal of Radiology 2024;25(1):11-23
Objective:
To investigate whether reader training improves the performance and agreement of radiologists in interpreting unenhanced breast magnetic resonance imaging (MRI) scans using diffusion-weighted imaging (DWI).
Materials and Methods:
A study of 96 breasts (35 cancers, 24 benign, and 37 negative) in 48 asymptomatic women was performed between June 2019 and October 2020. High-resolution DWI with b-values of 0, 800, and 1200 sec/mm 2 was performed using a 3.0-T system. Sixteen breast radiologists independently reviewed the DWI, apparent diffusion coefficient maps, and T1-weighted MRI scans and recorded the Breast Imaging Reporting and Data System (BI-RADS) category for each breast. After a 2-h training session and a 5-month washout period, they re-evaluated the BI-RADS categories. A BI-RADS category of 4 (lesions with at least two suspicious criteria) or 5 (more than two suspicious criteria) was considered positive.The per-breast diagnostic performance of each reader was compared between the first and second reviews. Inter-reader agreement was evaluated using a multi-rater κ analysis and intraclass correlation coefficient (ICC).
Results:
Before training, the mean sensitivity, specificity, and accuracy of the 16 readers were 70.7% (95% confidence interval [CI]: 59.4–79.9), 90.8% (95% CI: 85.6–94.2), and 83.5% (95% CI: 78.6–87.4), respectively. After training, significant improvements in specificity (95.2%; 95% CI: 90.8–97.5; P = 0.001) and accuracy (85.9%; 95% CI: 80.9–89.8; P = 0.01) were observed, but no difference in sensitivity (69.8%; 95% CI: 58.1–79.4; P = 0.58) was observed. Regarding inter-reader agreement, the κ values were 0.57 (95% CI: 0.52–0.63) before training and 0.68 (95% CI: 0.62–0.74) after training, with a difference of 0.11 (95% CI: 0.02–0.18; P = 0.01). The ICC was 0.73 (95% CI: 0.69–0.74) before training and 0.79 (95% CI: 0.76–0.80) after training (P = 0.002).
Conclusion
Brief reader training improved the performance and agreement of interpretations by breast radiologists using unenhanced MRI with DWI.
8.Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data
Kyung-Nam KOH ; Jung Woo HAN ; Hyoung Soo CHOI ; Hyoung Jin KANG ; Ji Won LEE ; Keon Hee YOO ; Ki Woong SUNG ; Hong Hoe KOO ; Kyung Taek HONG ; Jung Yoon CHOI ; Sung Han KANG ; Hyery KIM ; Ho Joon IM ; Seung Min HAHN ; Chuhl Joo LYU ; Hee-Jo BAEK ; Hoon KOOK ; Kyung Mi PARK ; Eu Jeen YANG ; Young Tak LIM ; Seongkoo KIM ; Jae Wook LEE ; Nack-Gyun CHUNG ; Bin CHO ; Meerim PARK ; Hyeon Jin PARK ; Byung-Kiu PARK ; Jun Ah LEE ; Jun Eun PARK ; Soon Ki KIM ; Ji Yoon KIM ; Hyo Sun KIM ; Youngeun MA ; Kyung Duk PARK ; Sang Kyu PARK ; Eun Sil PARK ; Ye Jee SHIM ; Eun Sun YOO ; Kyung Ha RYU ; Jae Won YOO ; Yeon Jung LIM ; Hoi Soo YOON ; Mee Jeong LEE ; Jae Min LEE ; In-Sang JEON ; Hye Lim JUNG ; Hee Won CHUEH ; Seunghyun WON ;
Cancer Research and Treatment 2023;55(1):279-290
Purpose:
Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea.
Materials and Methods:
From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed.
Results:
Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001).
Conclusion
The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.
9.Status and Needs for the Return to School of Childhood and Adolescent Cancer Survivors
Eun Sil PARK ; Eun Young JU ; Jae Young LIM ; Hyun Sik KANG ; Jin Kyung SUH ; Meerim PARK ; Jun Ah LEE ; Bo-Yoon JEONG ; Young Ae KIM ; Hyeon Jin PARK
Clinical Pediatric Hematology-Oncology 2023;30(2):60-69
Background:
As the treatment effects for childhood and adolescent cancer continue to improve, the survivor group is gradually growing and their age is increasing. This study aimed to determine the perceptions toward childhood and adolescent cancer survivors and assess the current situation regarding their return to school, to undertake policy development to help them grow into healthy members of society.
Methods:
From December 2019, for about 4 months, a structured questionnaire was used to conduct one-on-one interviews with childhood and adolescent cancer survivors and their parents, as well as with parents of healthy children and adolescents.
Results:
The participants included 79 survivors of childhood and adolescent cancer, 186 parents of cancer survivors, and 661 general parents; their mean age was 21.8, 13.9, and 12.5 years, respectively. After completing their cancer treatment, 77.2% of the cancer survivors returned to school, with the majority returning to regular schools in the same grade as their peers. Reasons for not returning to school (20.3%) included concerns about health management (43.8%), concerns about psychological and emotional adjustment (12.5%), and poor school attendance (12.5%). Among the parents of cancer survivors, 48.9% stated that they were “satisfied” with their children’s school life; a better health status in children was associated with a higher level of satisfaction (P=0.0071). In addition, they stated that national-level support was needed in the following areas for a successful return to school: a continuous health management system (36.1%) and understanding homeroom teachers who enable flexible participation in classes and school events (29.5%).
Conclusion
For survivors of childhood and adolescent cancer to successfully return to school and society, nationwide awareness-raising activities should be expanded, in addition to services that are tailored to the survivors’ needs and characteristics such as management of physical and mental health and educating homeroom teachers and peers.
10.The Effect of Forest Healing Program on the Psychological and Physiological Changes of the Employees: A Preliminary Study
Bo-Mi JUNG ; Su-Mi HYUN ; Bo-Hyun YOON ; Jye-Heon SONG ; Kyung-Min KIM ; Hyoung-Yeon KIM ; Deuk-Sil OH ; Seok-Myun LEE
Mood and Emotion 2023;21(1):1-7
Background:
This study aimed to investigate the effect of a forest healing program on psychological and physiological changes in employees exposed to work-related stress.
Methods:
This study included 38 employees whose workplaces were located in Naju and Jeonnam. They were given a set of forest healing programs. They completed self-rating scales to assess changes in anxiety, depression, and workrelated stress. Salivary cortisol levels, electroencephalography changes, and photoplethysmography components were also assessed. The mean differences in these parameters before and after treatment were analyzed.
Results:
The mean ages of male and female employees were different (p<0.010). After controlling for age as a covariate, all variables were examined for main and sex-associated effects using two-way repeated measures analysis of variance. Although the mean changes in saliva cortisol levels were significantly lower after treatment, the sex-associated and interaction effects were found to be insignificant.
Conclusion
Although the short-term forest healing program did not provide notable results, it may have beneficial effects on certain psychological and physiological aspects. Further studies are warranted to determine the feasibility of the forest healing program as a suitable stress management strategy for employees.

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