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.Quality Improvement Project to Reduce Admission Hypothermia of Preterm Infants Born at Less than 32 Weeks or 1,500 g
Hanna KIM ; Jieun HWANG ; Chan KIM ; Seung Hyun KIM ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG
Neonatal Medicine 2024;31(2):38-46
Purpose:
Hypothermia upon admission to the neonatal intensive care unit (NICU) contributes significantly to various neonatal complications, particularly in preterm infants. This study aimed to assess the impact of quality improvement (QI) interventions, including using plastic bags and head caps, and adjusting delivery room temperatures, on improving the admission body temperature and reducing hypothermia in infants born at less than 32 weeks or weighing 1,500 g.
Methods:
This study retrospectively analyzed the medical records of infants born at less than 32 weeks or weighing 1,500 g who admitted to the NICU at Samsung Medical Center from January 2022 to February 2024. The QI program that was initiated in April 2023 focused on managing admission temperatures using plastic bags and head caps, and maintaining delivery room temperatures at ≥25 °C. The admission temperature and short-term outcomes pre- and post-QI were compared.
Results:
In a study of 270 patients, implementing QI initiatives significantly raised the admission temperature from 36.2±0.5 to 36.4±0.4 °C (p<0.01), particularly impacting infants weighing ≥1,000 g, in whom mild hypothermia occurrences dropped from 76.3% to 43.9% (p<0.01). This improvement in temperature management significantly decreased both mild and severe hypothermia rates post-QI. Additionally, implementing all three initiatives was more effective than when two or fewer initiatives were implemented.
Conclusion
Simple and cost-effective QI interventions can increase admission temperatures and decrease hypothermia in neonates. Further research is essential to explore the long-term outcomes and develop effective hypothermia management strategies in neonatal care.
5.Clinical Characteristics of Hypospadias and Its Association with Very Low Birth Weight Infants with Small for Gestational Age
Seon Nyo KIM ; Seung Hyun KIM ; Chan KIM ; Hanna KIM ; Jieun HWANG ; Misun YANG ; So Yoon AHN ; Se In SUNG ; Yun Sil CHANG ; Hyeseon KIM
Neonatal Medicine 2024;31(2):17-23
Purpose:
To investigate the risk factors associated with hypospadias in very low birth weight (VLBW) infants (VLBWIs).
Methods:
We retrospectively analyzed 729 infants born at ≥24 weeks of gestational age and weighing <1,500 g from January 2012 to December 2022. We assessed the prevalence of hypospadias by birth weight percentiles and also compared the demographics and placental histopathology of the infants with hypospadias (n=52) and those without hypospadias (n=677).
Results:
Of the 729 patients analysed, hypospadias was recorded in 26 (20.3%), 14 (26.9%), and 12 (2.5%) infants in the <3rd, ≥3rd–<10th, and ≥10th–<90th percentiles, respectively. Of all of the patients with hypospadias, 50% had birth weights <3rd percentile (p<0.001). The hypospadias group demonstrated a longer mean gestational age (30.1 weeks vs. 27.9 weeks, p<0.001), lower incidence of maternal pregnancy-induced hypertension (48.1% vs. 17.3%, p<0.001), lower incidence of premature rupture of membrane (11.5% vs. 27.1%, p=0.013), lower acute chorioamniotic maternal response (9.1% vs. 35.2%, p<0.001), and higher maternal underperfusion (95.5% vs. 71.9%, p<0.001).
Conclusion
The frequency of hypospadias was found to be the highest among VBLWIs <3rd percentile, who were severely small for their gestational age (SGA). Additionally, the incidence increased with a decreasing birth weight. Physical examination is necessary at birth for VLBWIs classified as SGA. Moreover, the data on the incidence of hypospadias among VLBWIs in neonatal intensive care units can assist in tracking counseling from the prenatal to the postnatal period for patients born <3rd percentile.
6.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.
7.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.
8.Lesson from COVID-19 outbreak; importance of standard precautions to febrile neutropenia prevention in patients with breast cancer who received adjuvant chemotherapy:a retrospective observational study
Ji Eun PARK ; Jieun YANG ; Sanghoon HAN ; Jeong Rae YOO ; Misun KIM ; Donghyoun LEE ; Jaemin JO
Annals of Surgical Treatment and Research 2024;107(4):195-202
Purpose:
Intensive cytotoxic chemotherapy increases the risk of infection in patients with cancer by inducing bone marrow suppression and mucosal injury. Febrile neutropenia (FN) is the most important clinical adverse event in patients with cancer receiving cytotoxic chemotherapy. To prevent FN, standard precautions including hand and respiratory hygiene are generally recommended, but the exact effect of non-pharmacologic intervention has not been clearly proven in the clinical setting. We aimed to compare the incidence of FN between the pre-coronavirus disease 19 (COVID-19) era vs. the postCOVID-19 era.
Methods:
We retrospectively enrolled patients with breast cancer who received an adriamycin and cyclophosphamide (AC) regimen containing adjuvant chemotherapy at Jeju National University Hospital. We compared the incidence of FN between the pre- and post-COVID-19 period and analyzed characteristics of the event and other clinical risk factors.
Results:
In total, 149 patients were enrolled, including 94 who received AC chemotherapy in the pre-COVID-19 era and 55 who received it in the post-COVID-19 era. Sixteen patients (10.7%) experienced FN. Fourteen (14.9%) and 2 events (3.6%) occurred in pre-COVID-19 and post-COVID-19 eras, respectively. The post-COVID-19 era was the only risk factor for FN (P = 0.032).
Conclusion
We found an association between FN occurrence and the COVID-19 outbreak, providing indirect evidence of the importance of non-pharmacological measures to reduce FN risk in patients with breast cancer. Further research is required to confirm the standard precautions for FN prevention in patients with cancer.
9.Unilateral testicular tuberculosis in a kidney transplant recipient: a case report
Jaeseok YANG ; Yaerim KIM ; Woo Yeong PARK ; Kyubok JIN ; Seungyeup HAN ; Byung Hoon KIM ; Misun CHOE ; Jin Hyuk PAEK
Clinical Transplantation and Research 2024;38(3):235-240
Tuberculosis (TB) of the genitourinary system is a rare form of extrapulmonary TB. Testicular TB is particularly uncommon among kidney transplantation (KT) recipients. Diagnosing testicular TB is challenging due to the nonspecific nature of clinical presentations and ambiguous imaging results. In this report, we describe a case involving a 36-year-old male KT recipient who presented with left scrotal pain. He had undergone a living donor KT 8 years prior and was receiving tacrolimus, mycophenolate mofetil, and prednisolone. Laboratory tests revealed anemia, leukocytosis, and elevated inflammatory markers. Computed tomography showed left scrotal wall thickening and enlargement, suggestive of a left testicular abscess. We discontinued mycophenolate mofetil and administered intravenous antibiotics. Additionally, we performed an incision and drainage of the abscess. However, there was no improvement in his clinical course. Consequently, we performed a radical left orchiectomy. The biopsy revealed extensive chronic granulomatous inflammation with caseous necrosis, consistent with tuberculous orchiepididymitis. A quadruple anti-TB regimen was administered, leading to an improvement in the patient's condition. To the best of our knowledge, this is the first reported case of testicular TB without other organ involvement in KT recipients. Including testicular TB in the differential diagnosis of testicular infections and masses is necessary to avoid unnecessary surgical procedures.
10.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.

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