• Child Health Nursing Research
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• v.28 no.3
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• pp.187-196
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• 2022

Purpose: This study aimed to clarify the factors related to mothers' decisions to provide disease explanations to their children, investigate the associations between those factors and social adaptive skills, and examine support relative to children's growth and development. Methods: Data were collected from anonymous, self-administered questionnaires answered by 71 mothers of outpatient school-aged children with congenital heart disease. The questionnaire items included characteristics of mothers and children, decision factors for providing disease explanations, and children's social adaptive skills (Asahide-Shiki social adaptive skills test). Factor analysis was performed on the decision factors, and multiple regression analysis was performed to examine the association between the identified factors and each social adaptive skill. Results: The decision factors for providing disease explanations were the mother's explanation ability, the receptive capability of the child, and the child's interest and literacy regarding the explanation. Multiple regression analysis showed that language and social life skills were significantly associated with the child's receptive capability, and language and daily life skills were significantly associated with the child's interest and literacy regarding the explanation. Conclusion: Improving children's language, social life, and daily life skills may enhance their receptive capability and literacy regarding explanations of their disease.

• Journal of Chest Surgery
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• v.20 no.1
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• pp.161-170
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• 1987

Fifty-nine cases of congenital chest wall defects experienced in the department of thoracic surgery of Seoul National University Hospital were analyzed and the relevant literatures were reviewed. They are 52 cases of funnel chest, 3 cases of pigeon breast, one case of superior sternal fissure, one case of costochondral incurvation, one case of Cantrell`s pentalogy, and one case of Poland`s syndrome. Funnel chest affected males more frequently than females by 44 to 8. All of the funnel deformities were corrected by Ravitch operation or its modification except one which was the first case of this series and was corrected by a sterno-turnover. Two cases required a mechanical ventilation for 3 days and 5 days respectively. Four minor complications which were two cases of skin wound infection and 2 cases of fluid accumulation were noted. Skin would infection was repaired by a secondary closure and fluid accumulation was treated by aspiration only. The result are all excellent without recurrence or reoperation. In 3 cases of pigeon breast, they were treated by subperichondrial resection of all of the involved costal cartilages and shortening their course with reefing sutures in the perichondrium with excellent result. The superior sternal fissure which was combined by a ventricular septal defect was treated by a simple wire closure with a good result. The costochondral incurvation was corrected by subperichondrial resection of deformed cartilages and a rib graft removed from the contralateral normal side. The Poland syndrome and the Cantrell`s pentalogy was already presented previously.

• Journal of Chest Surgery
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• v.24 no.10
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• pp.960-966
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• 1991

From February 1984 through July 1991, 104 infants less than 1 year of age with congenital heart defects underwent open heart repair with conventional cardiopulmonary bypass which occupied 10.7% of all patients with congenital heart defects operated on during same period. There were 66 boys and 38 girls 7 days to 12 months [mean age, 8.2 months]. Four patients were neonates, 8 were 1 to 3 months, 23 were 4 to 6 months, and 69 were 7 to 12 months of age. Mean body weight at repair was 6.9kg and mean BSA, 0.36m2 Indications for operation were intractable congestive heart failure and severe pulmonary hypertension in patients with VSD and severe cyanosis and anoxic spells in patients with TOF. Conditions corrected were VSD[79], TOF[8], AVSD[4], PS[2], PA+IVS[2], TAPVC [2], MR[2], DOLV[l], Truncus arteriosus[1], D-TGA[1], and PA-VSD[1]. Twenty-three of 79 patients with VSD had associated cardiovascular anomalies which included PDA in 16 patients, PS in 9 patients, ASD in 5 patients, LSVC in 2 patients, MR in 1 patient, dextrocardia in 1 patient, and single coronary artery in 1 patient. The hospital mortality rate was 24.0% which was much higher than that of 6% in patients over 1 year of age. The greatest mortality occurred in babies of low weight under 6 months of age, There was no late death. Surviving infants showed marked symptomatic improvement and change in growth patterns. These surgical results were to be overcome with proper pre- and post-operative management and improvement of surgical technique

• Journal of Veterinary Clinics
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• v.26 no.5
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• pp.467-471
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• 2009

This report describes Neural Tube Defects (NTDs) with Abdominal Wall Defects (AWDs) on the sibling of Yorkshire terriers. The NTDs and AWDs are rare serious congenital defects. The NTDs are neurulation abnormality that results from to failed transformation of the neurual tube by the incomplete closure of the embryonic neural plate. These dysraphic states range form mild to severe according to developmental malformation that include fusion defects of skull (crania bifida; CB) and fusion defects of vertebrae (spina bifida; SB). The AWDs are genetic defects that results from to failed formation of abdominal wall and cavity. These dysraphic states are omphalocele and gastroschisis. The 12-month dam was delivered by caesarian section and 4 littermate had obvious malformations. One male dead stillbirth fetus (L1) was revealed the extruded abdominal viscera, omphalocele. One female fetus (L2) was died within 1 hour after birth with defects of abdominal muscle upper umbilicus, gastroschisis. 3rd fetus (L3) was died within 36 hours after parturition and revealed a copious dermal and vertebral defects on the midline thorax, upper SB asperta. 4th fetus (L4) is still growing well now at 6 months but at the 2 week age, appears hairy nevus on the frontal cranium and dorsal thoracic portion. The radiograph of L1 and L2 are shown decrease bony density of calvarium and L3 was shown defect of spinose processes of the T9-T13. On our knowledge, this is first report of the SB and CB in Yorkshire terrier. And also sibling of NTDs with AWDs that has not previously been reported in the dog.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.17-26
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• 1997

Objeitives. The surgical mortality of congenital heart defects has been reduced (or the very young age group. Especially, young age at repair is an important risk factor for mortality after repair of tetralogy of Falloff. Some risk factors were analyzed Methods. Three hundred and sixty six patients underwent surgical Intervention. Ages ranged from 5 days to 64 years, and 80 patients were adults(over 15 years of age). The defects consisted of 313(84.2%) acyanotic and 53(15.8%) cyanotic anomalies. The surgical mortalities were evaluated by univariate and multivariate analysis. Results. The overall surgical mortality was 10.4%. Mos deaths occurred in the infant group younger than 6 months(20/38 deaths) and in cyanotic group(21/38 deaths). Surgical infant mortality younger than 12 months was 24.8%(25/101). Risk factors of mortality in open heart surgery were age(p< 0.0001), body weight(p< 0.0001), pump time(p< 0.0001), aortic cross clamp time(p< 0.0001), use of total circulatory arrest(p<0.0001) and cyanotic disease(p<0.0001) by univariate analysis. But by multivariate analysis, the risk factor of mortality in open heart surgery was disease entity(p=0.002) only. A disease group with the highest risk was a cyanotic group(odds ratio was 15.3 relative to ventricular septal defect) excluding tetralogy of Falloff(odds ratio=0.27). Conclusions. Even though the most important risk factor was disease entity, we should conentrate our efforts on the technically improvable factors affecting surgical mortality indicated by univarlate analysis.

• Journal of the Korea Convergence Society
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• v.10 no.3
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• pp.347-354
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• 2019

Purpose: The purpose of this study was to determine the relationships among health related quality of life and uncertainty in adult patients with congenital heart diseases. Methods: The subjects were 136 adult congenital heart disease patients at the congenital heart center out-patient clinic of the A General Hospital in S city. Data were analyzed using descriptive statistics, t-test, ANOVA, Pearson's correlation coefficient and multiple regression. Results: There were significant negative correlations of quality of life with general motor function area and uncertainty. Among predictors, gross motor(${\beta}=-.38$) and daily activity (${\beta}=-.21$) had statistically significant influences on uncertainty(F=6.15, p=.018). Conclusion: Nursing interventions to promote gross motor and daily activity might be essential for adult patients with congenital heart disease in order to reduce uncertainty.

• Journal of Yeungnam Medical Science
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• v.40 no.2
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• pp.187-192
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• 2023

Background: This study aimed to present the short- and midterm outcomes after complete atrioventricular canal defect (CAVC) repair using a single-patch technique. Methods: This study included 30 children who underwent surgical correction of the CAVC using a single-patch technique. Results: The median age of the patients was 5.7 months (interquartile range [IQR], 5.0-7.5 months), and 23 patients (76.7%) had type A CAVC. Fourteen patients (46.7%) were female and 17 (56.7%) had been diagnosed with Down syndrome. The in-hospital mortality rate was 0%. No deaths were observed during a median follow-up of 4 years (IQR, 3.5-5.0 years). Patients without Down syndrome were associated with late moderate mitral regurgitation (MR) (p=0.02). Late MR less than moderate degree was observed in 96.6%, 78.5%, and 50% of patients after 2, 4, and 5 years of follow-up, respectively, while late tricuspid valve regurgitation less than moderate degree was observed in 96.7%, 85.9%, and 59.0% of patients after 2, 4, and 6 years of follow-up, respectively. After a median follow-up of 4 years, only one patient had required surgical repair of a left ventricular outflow tract obstruction, which occurred 26 months after the first operation. Multivariable logistic regression analysis adjusted for the type of CAVC, sex, Down syndrome, age, and weight revealed that the absence of Down syndrome was a risk factor for late moderate MR (MR-2) (odds ratio, 0.05; 95% confidence interval, 0.006-0.50; p=0.01). Conclusion: A single-patch technique for CAVC surgical repair is a safe method with acceptable short- and midterm results.

• Journal of Veterinary Clinics
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• v.19 no.1
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• pp.110-113
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• 2002

An atrial septal defect (ASD) is a congenital hole in the atrial septum that allows flow between the two atria. Small ASDs are usually well-tolerated defects and do not result in significant clinical abnormalities. In large ASDs or in the presence of other cardiac defects, clinically significancy is increased. Atrial septal defects in 2 Dogs with cardiac and respiratory signs were diagnosed at seoul animal medical center. In ascultation, systolic murmur and the splitting of second heart sound were heard at pulmonary or tricuspid valve region. In radiograph, right-sided cardiomegaly, pulmonary artery dilation, increased pulmonary vasculature makings, and pleural effusion or pulmonary edema signs were observed. In echocardiography, the region, location and size of septal defect was identified. Also, the direction and degree of shunt was measured. These dogs were treated with medicine for cardiac failure. One dog is well-tolerated, the other dog died.

• Clinical and Experimental Reproductive Medicine
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• v.49 no.3
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• pp.215-218
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• 2022

Herein, we report an exceptionally rare case of a 25-year-old woman with cloacal exstrophy/omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) syndrome achieving a viable pregnancy despite many gastrointestinal and genitourinary malformations and multiple respective corrective operations. The patient was born with two vaginas, two uteruses, four ovaries, an imperforate anus, a large omphalocele including bowel and bladder exstrophy, and diaphysis of the pubic rami. This patient is the only documented OEIS patient not to have tethered spinal cord as an anomaly, perhaps contributing to her successful pregnancy. After experiencing preeclampsia with severe features at 35 weeks, the baby was born via cesarean section.

• Clinical and Experimental Pediatrics
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• v.63 no.3
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• pp.79-87
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• 2020

Inherited platelet disorders (IPDs), which manifest as primary hemostasis defects, often underlie abnormal bleeding and a family history of thrombocytopenia, bone marrow failure, hematologic malignancies, undefined mucocutaneous bleeding disorder, or congenital bony defects. Wide heterogeneity in IPD types with regard to the presence or absence of thrombocytopenia, platelet dysfunction, bone marrow failure, and dysmegakaryopoiesis is observed in patients. The individual processes involved in platelet production and hemostasis are genetically controlled; to date, mutations of more than 50 genes involved in various platelet biogenesis steps have been implicated in IPDs. Representative IPDs resulting from defects in specific pathways, such as thrombopoietin/MPL signaling; transcriptional regulation; granule formation, trafficking, and secretion; proplatelet formation; cytoskeleton regulation; and transmembrane glycoprotein signaling are reviewed, and the underlying gene mutations are discussed based on the National Center for Biotechnology Information database and Online Mendelian Inheritance in Man accession number. Further, the status and prevalence of genetically confirmed IPDs in Korea are explored based on searches of the PubMed and KoreaMed databases. IPDs are congenital bleeding disorders that can be dangerous due to unexpected bleeding and require genetic counseling for family members and descendants. Therefore, the pediatrician should be suspicious and aware of IPDs and perform the appropriate tests if the patient has unexpected bleeding. However, all IPDs are extremely rare; thus, the domestic incidences of IPDs are unclear and their diagnosis is difficult. Diagnostic confirmation or differential diagnoses of IPDs are challenging, time-consuming, and expensive, and patients are frequently misdiagnosed. Comprehensive molecular characterization and classification of these disorders should enable accurate and precise diagnosis and facilitate improved patient management.