• Journal of Genetic Medicine
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• 제13권2호
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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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• 제56권4호
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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.

• 여성건강간호학회지
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• 제25권2호
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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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• 제49권5호
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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.

• 대한간호학회지
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• 제51권5호
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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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• 제62권3호
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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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• 제53권3호
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• pp.349-357
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• 2010

목 적 : TTN은 대부분의 경우 출생 24-72시간 이내에 좋아지는 것으로 알려져 있으나 때로는 72시간 이상 빈호흡이 지속되며 심각한 저산소증과 호흡부전, 기흉 등의 합병증이나 사망에까지 이르기도 한다. 지금까지 이러한 지속성 TTN에 대한 연구가 드물어 이들에 대한 발생 위험인자와 임상경과를 알아보고자 본 연구를 시행하였다. 방 법 : 2001년 1월 1일부터 2007년 9월 30일까지 서울아산 병원에서 출생한 12,021명의 신생아 중 신생아 중환자실에 입원하여 TTN으로 진단받은 재태연령 $35^{+0}$주 이상의 신생아 107명을 대상으로 환아와 그 어머니의 의무기록을 후향적으로 검토, 분석하였다. 빈호흡에 대한 다른 기저 질환 없이 빈호흡이 생후 72시간 이상 지속되는 경우를 PTTN으로 정의하고 빈호흡이 출생 72시간 이전에 호전되는 경우를 STTN으로 구분하여 TTN의 위험인자와 임상경과를 비교하였다. 대조군은 같은 기간 동안 신생아실에 입원한 정상 신생아 126명을 무작위로 선택하여 비교하였다. 결 과 : 전체 TTN환아 107명 중 55명(51%)이 PTTN에 해당하였다. PTTN군에서 출생 6시간 이내 발생하는 끙끙거림(grunting)의 빈도, 분당 90회 이상의 빈호흡, 산소 치료농도 0.4 이상, 기흉, 보조적 호흡기 치료 등이 빈번하였고 출생 첫날 측정한 총 단백과 알부민, 체중 감소 비율이 STTN군에 비해 낮았다. PTTN의 발생과 가장 관련 있는 예측 위험 인자로는 출생 6시간 이내 발생한 끙끙거림, 최대 호흡수가 90회 이상, 산소 치료농도가 0.4 이상인 경우 등이었다. 결 론 : TTN 환아에서 출생 6시간 이내의 끙끙거림, 최대 호흡수 분당 90회 이상, $FiO_2$ 0.4 이상인 경우 등의 위험 인자가 있는 경우 더 나쁜 임상 경과를 보일 것으로 예측할 수 있을 것이다. PTTN의 발생을 낮추고 질환의 빈도와 증상을 줄일 수 있는 방법에 대한 더 많은 연구가 필요하다.

• Neonatal Medicine
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• 제25권1호
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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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• 제16권2호
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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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• 제16권2호
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• pp.190-196
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• 2009

목 적 : 신생아 집중치료실에서 극소 저체중 출생아의 칸디다 감염이 증가하고 있다. 그러나 우리나라에서 미숙아의 전신성 칸디다 감염에 대한 최근 자료는 부족하다. 본 연구는 최근 5년 간 극소 저체중 출생아에서 발생한 전신성 칸디다 감염의 역학적 양상에 대해 알아보고자 한다. 방 법 : 2004년 1월 1일부터 2008년 12월 31일까지 가톨릭의과대학교 부속 3개 병원 신생아 집중치료실에 입원한 극소 저체중 출생아 중 전신성 칸디다 감염이 발생한 환자 19명의 의무기록지를 후향적으로 검토하여 출생 체중, 재태기간, 진단시 나이, 위험 인자, 동반 질환, 항진균제 치료, 사망 등을 분석하였다. 결 과 : 19명(4.7%)의 극소 저체중 출생아에서 전신성 칸디다 감염이 진단되었다. 출생 체중은 평균 959.0$\pm$255.9 g이었고 재태기간은 26.7$\pm$2.1주였다. 동정된 균종은 C. albicans (4례), C. parapsilosis (9례), C. glabrata (2례), C. famata (2례)였고 아종을 분류하지 못한 경우가 2건 있었다. 위험 인자인 중심 정맥 카테터 사용, 항생제 정맥 영양과 지방 유제 투어, 기도 삽관, $H_2$ blocker 사용은 대부분의 환자에서 연관이 있었다. 칸디다 감염 환자 중 사망한 경우와 생존한 경우 간에 재태기간을 제외한 차이는 없었다. 19명의 감염자 중 9명(47.4%)이 사망하였고 칸디다 관련 사망은 4건(21.1%)이 있었다. 결 론 : 극소 저체중 출생아에서 전신성 칸디다 감염의 발생 빈도가 꾸준히 증가하고 있으며 이전에 비해 C. non-albicans 특히 C. parapsilosis로 인한 감염이 빈번했다. 신생아 중환자실에서 칸디다 감염은 사망률이 매우 높기 때문에 역학적 자료에 근거한 예방 요법 및 조기 치료가 필요할 것으로 생각된다.