• Journal of Genetic Medicine
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• v.13 no.2
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• pp.105-110
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• 2016

Congenital myotonic dystrophy (CMD) which is transmitted in an autosomal-dominant manner, can also be observed in newborns born to asymptomatic parents who have a myotonic dystrophy type 1 or premutation allele, especially from the mother. A mother with myotonic dystrophy could be subfertile and the pregnancy could be complicated with the risk of a preterm birth. Newborns with CMD may demonstrate symptoms such as hypotonia and poor motor activity, as well as respiratory and feeding difficulties. Additionally, CMD has a high mortality rate at birth. Detection of the signs and symptoms during pregnancy is helpful for a prenatal diagnosis of CMD in cases where the family history is not known.

• Anatomy and Cell Biology
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• v.56 no.4
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• pp.421-427
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• 2023

Bladder exstrophy is a rare congenital condition of the pelvis, bladder, and lower abdomen that opens the bladder against the abdominal wall, produces aberrant growth, short penis, upward curvature during erection, wide penis, and undescended testes. Exstrophy affects 1/30,000 newborns. The bladder opens against the abdominal wall in bladder exstrophy, a rare genitourinary condition. This study is vital to provide appropriate therapy choices as a basis to improve patient outcomes. This study may explain bladder exstrophy and provide treatment. Epispadias, secretory placenta, cloacal exstrophy, and other embryonic abnormalities comprise the exstrophy-spades complex. The mesenchymal layer does not migrate from the ectoderm and endoderm layers in the first trimester, affecting the cloacal membrane. Embryological problems define the exstrophy-aspidistra complex, which resembles epimedium, classic bladder, cloacal exstrophy, and other diseases. Urogenital ventral body wall anomalies expose the bladder mucosa, causing bladder exstrophy. Genetic mutations in the Hedgehog cascade pathway, Wnt signal, FGF, BMP4, Alx4, Gli3, and ISL1 cause ventral body wall closure and urinary bladder failure. External factors such as high maternal age, smoking moms, and high maternal body mass index have also been associated to bladder exstrophy. Valproic acid increases bladder exstrophy risk; chemicals and pollutants during pregnancy may increase bladder exstrophy risk. Bladder exstrophy has no identified cause despite these risk factors. Exstrophy reconstruction seals the bladder, improves bowel function, reconstructs the vaginal region, and restores urination.

• Women's Health Nursing
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• v.25 no.2
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• pp.194-206
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• 2019

Purpose: Maternal Fetal Intensive Care Unit (MFICU), which provides intensive care to high-risk mothers with increasing maternal age and high-risk newborns, has become a new field of nursing work in South Korea. The present study was conducted to identify the educational needs and self-assessing clinical competence of nurses in MFICU. Methods: The education needs and competencies of MFICU nurses were measured through prepared questionnaires by researchers based on the previous studies on job analysis of nurses in MFICU. Data were collected from January 2019 to March 2019. The study involved 168 nurses working in MFICUs at 12 hospitals nationwide as study subjects. The data were analyzed using the SPSS WIN 23.0 program. Results: The education needs of nurses in MFICU had an average of 4.21 points (${\pm}0.50$) and their nursing competence was average 3.38 points (${\pm}0.60$). The items reported as high education needs but low competency by nurses in MFICU were as following: 'postpartum hemorrhage and shock,' 'cardiopulmonary resuscitation (CPR) for neonate,' 'CPR during pregnancy,' 'disseminated intravascular coagulation,' 'sepsis,' and 'mechanical ventilation during pregnancy.' Conclusion: Based on these results, it is proposed that a comprehensive education program for nurses in MFICU should be developed by considering low capabilities among MFICU nurses as a priority factor.

• Journal of Chest Surgery
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• v.49 no.5
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• pp.337-343
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• 2016

Background: Recent developments in surgical techniques and hospital care have led to improved outcomes following total anomalous pulmonary venous return (TAPVR) repair. However, the surgical repair of TAPVR remains associated with a high risk of mortality and need for reoperation. We conducted this retrospective study to evaluate mid-term outcomes following in situ TAPVR repair without total circulatory arrest (TCA), and to identify the risk factors associated with surgical outcomes. Methods: We retrospectively reviewed 29 cases of surgical intervention for TAPVR conducted between April 2000 and July 2015. All patients were newborns or infants who underwent in situ TAPVR repair without TCA. Results: Four anatomic subtypes of TAPVR were included in this study: supracardiac (20 cases, 69.0%), cardiac (4 cases, 13.8%), infracardiac (4 cases, 13.8%), and mixed (1 case, 3.4%). The median follow-up period for all patients was 42.9 months. Two (6.9%) early mortalities occurred, as well as 2 (6.9%) cases of postoperative pulmonary venous obstruction (PVO). Preoperative ventilator care (p=0.027) and preoperative PVO (p=0.002) were found to be independent risk factors for mortality. Conclusion: In situ repair of TAPVR without TCA was associated with encouraging mid-term outcomes. Preoperative ventilator care and preoperative PVO were found to be independent risk factors for mortality associated with TAPVR repair.

• Journal of Korean Academy of Nursing
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• v.51 no.5
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• pp.511-524
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• 2021

The coronavirus disease 2019 (COVID-19) has emerged as a threat to human health and public safety. People of all ages are susceptible to severe acute respiratory syndrome coronavirus 2 infection. However, the clinical manifestations of this infection differ by age. This study purposes to describe healthcare considerations for special populations, such as children, pregnant and lactating women, and older adults, who may have unique healthcare needs, in the pandemic situation. To realize the research purpose, we conducted a review of the practice guidelines of public documents and qualified studies that were published online/offline during a specific period. The review identified current knowledge on care for newborns, children in schools, pregnant women (from antenatal to postpartum care), and older adults suffering from high-risk conditions. Subsequently, we summarize vaccination guidance for special populations and, finally, discuss the issues currently affecting special populations. Therefore, this current knowledge on care for special populations helps nurses to provide accurate information on vaccinations aimed at preventing COVID-19 and protecting the masses from infection. Currently, the scarcity of information on COVID-19 variants necessitates further research on measures to reduce pandemic spread.

• Clinical and Experimental Pediatrics
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• v.62 no.3
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• pp.79-84
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• 2019

Newborn infants, including premature infants, are high-risk patients susceptible to various microorganisms. Catheter-related bloodstream infections are the most common type of nosocomial infections in this population. Regular education and training of medical staffs are most important as a preventive strategy for central line-associated bloodstream infections (CLABSIs). Bundle approaches and the use of checklists during the insertion and maintenance of central catheters are effective measures to reduce the incidence of CLABSIs. Chlorhexidine, commonly used as a skin disinfectant before catheter insertion and dressing replacement, is not approved for infants <2 months of age, but is usually used in many neonatal intensive care units due to the lack of alternatives. Chlorhexidine-impregnated dressing and bathing, recommended for adults, cannot be applied to newborns. Appropriate replacement intervals for dressing and care sets are similar to those recommended for adults. Umbilical catheters should not be used longer than 5 days for the umbilical arterial catheter and 14 days for the umbilical venous catheter. It is most important to regularly educate, train and give feedback to the medical staffs about the various preventive measures required at each stage from before insertion to removal of the catheter. Continuous efforts are needed to develop effective and safe infection control strategies for neonates and young infants.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.349-357
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• 2010

Purpose : Transient tachypnea of the newborn (TTN) is usually benign and improves within 72 hours. However, it can also progress to prolonged tachypnea over 72 hours, profound hypoxemia, respiratory failure, and even death. The aim of this study is to find predictable risk factors and describe the clinical courses and outcomes of prolonged TTN (PTTN). Methods : The medical records of 107 newborns, >$35^{+0}$ weeks of gestational age with TTN, who were admitted to the NICU at Seoul Asan Medical Center from January 2001 to September 2007 were reviewed. They were divided into 2 groups based on duration of tachypnea. PTTN was defined as tachypnea ${\geq}72$ hours of age, and simple TTN (STTN) as tachypnea <72 hours of age. We randomly selected 126 healthy-term newborns as controls. We evaluated neonatal and maternal demographic findings, and various clinical factors. Results : Fifty-five infants (51%) with total TTN were PTTN. PTTN infants had grunting, tachypnea >90/min, $FiO_2$ >0.4, and required ventilator care more frequently than STTN infants. PTTN had lower level of serum total protein and albumin than STTN. The independent predictable risk factors for PTTN were grunting, maximal respiration rate >90/min, and $FiO_2$ >0.4 within 6 hours of life. Conclusion : When a newborn has grunting, respiration rate >90/min, and oxygen requirement >0.4 of $FiO_2$ within 6 hours of life, the infant is at high risk of having persistent tachypnea ${\geq}72$ hours. We need further study to find the way to reduce PTTN.

• Neonatal Medicine
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• v.25 no.1
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• pp.16-22
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• 2018

Purpose: This study investigated predictive factors for severe neonatal thrombocytopenia, which greatly increases the need for intensive care and is associated with a high mortality rate in premature infants. Factors adopted for prompt identification of at-risk newborns include blood test results and birth history. This study analyzed the relationship between the presence of severe neonatal thrombocytopenia and the mortality rate. The causes of thrombocytopenia in premature infants were also examined. Methods: This retrospective study evaluated 625 premature infants admitted to the neonatal intensive care unit (NICU) at Chung-Ang University Medical Center. The neonates were classified into 3 groups according to the severity of thrombocytopenia: mild ($100{\times}10^9/L{\leq}platelet<150{\times}10^9/L$), moderate ($50{\times}10^9/L{\leq}platelet<100{\times}10^9/L$), or severe (platelet<$50{\times}10^9/L$). Analysis of blood samples obtained at the onset of thrombocytopenia included platelet count, white blood cell (WBC) count, hemoglobin level, hematocrit level, absolute neutrophil count, and high-sensitivity C-reactive protein level. Results: Of the 625 premature infants admitted to our NICU, 214 were detected with thrombocytopenia. The mortality rate in thrombocytopenic neonates was 18.2% (39/214), whereas a mortality rate of only 1.0% was observed in non-thrombocytopenic neonates. The major causes of thrombocytopenia were perinatal insufficiency and sepsis in premature infants. Severe thrombocytopenia was noted more frequently in premature infants with higher WBC counts and in those with a younger gestational age. Conclusion: Platelet count, WBC count, and gestational age are reliable predictors for severe neonatal thrombocytopenia. The major causes of thrombocytopenia were perinatal insufficiency and sepsis in premature infants.

• Advances in pediatric surgery
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• v.16 no.2
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• pp.101-107
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• 2010

Herniorrhaphy of Indirect inguinal hernia (IIH) is one of the most frequently performed surgical procedures in children. The overall incidence of inguinal hernias in childhood ranges from 0.8 to 4.4 %. The incidence is up to 10 times higher in boys than girls, especially much higher in premature infants. IIHs in children are basically an arrest of embryologic development rather than an acquired weakness, which explains the increased incidence in premature infants. In normal development, the processus vaginalis closes, obliterating the peritoneal opening of the internal ring between 36th and 40th week of gestation. This process is often incomplete, leaving a small patent processus in many newborns. However, closure continues postnatally, and the rate of patency is inversely proportional to age of the child. The presence of a patent processus vaginalis is a necessary but not sufficient variable in developing a congenital IIH. In other words, all congenital IIHs are preceded by a patent processus vaginalis, but not all patent processus vaginalis go on to become IIHs. The overall incidence of IIH in population is approximately 1 to 2 % and the incidence of a processus vaginalis is approximately 12 to 14%, clinically appreciable IIH should develop in approximately 8 to 12 % of patients with a patent processus vaginalis. Although the classic open inguinal hernia repair remains the gold standard for most pediatric surgeons, laparoscopic repair is being performed in many centers. Like open technique, laparoscopic technique is fundamentally a high ligation of the indirect hernia sac with or without internal ring ligation. The advantages of laparoscopic approach include the ease of examining the contralateral internal ring, the avoidance of access damage to vas and vessels during mobilization of cord, decreased operative time, and an ability to identify unsuspected direct or femoral hernias. Almost all groin hernias in children are IIHs and occur as a result of incomplete closure of processus vaginalis. The treatment is repair by high ligation of hernia sac, which can be done by an open or laparoscopic technique. The contralateral side can be explored by laparoscopy or left alone, open exploration is no longer indicated due to potential risk of infertility.

• Neonatal Medicine
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• v.16 no.2
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• pp.190-196
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• 2009

Purpose: Candida infection has increased in neonatal intensive care units (NICU). However, recent reports on systemic candida infections in preterm newborns are rare in Korea. The aim of this study was to examine the epidemiological features of systemic candida infection in very low birth weight infants (VLBW) over the past five years. Methods: We retrospectively reviewed the medical records of 19 patients with systemic candida infections in VLBW that were admitted to the neonatal intensive care units of three hospitals affiliated with the College of Medicine, The Catholic University of Korea from January 2004 to December 2008. We analyzed the birth weight, gestational age, age at diagnosis, risk factors, co-morbidity, antifungal treatment, and mortality rates among the 19 patients. Results: Systemic candida infections occurred in 19 cases (4.7%) among the VLBW infants. The mean birth weight and gestational age were 959.0$\pm$255.9 g and 26.7$\pm$2.1 weeks. The isolated Candida species were C. albicans (4), C. parapsilosis (9), C. glabrata (2), C. famata (2), and unkown subspecies (2). Most patients had various associated risk factors, including a central venous catheter, broad spectrum antibiotics, parenteral nutrition, intravenous lipid emulsion, endotracheal intubation and $H_2$ blocker therapy. There was no significant difference in the risk factors between newborns that survived and those that died with regard to the systemic candida infection, except for gestational age. Nine (47.4%) out of 19 patients with a candida infection died and four cases (21.2%) were directly related to the candida infection. Conclusion: The prevalence of systemic candida infection is increasing in VLBW infants. The majority of Candida species has shifted to C. non-albicans, especially C. parapsilosis. Because of the high mortality associated with candida infection in the NICU, prophylaxis and early treatment based on epidemiological features is necessary.