• Journal of Genetic Medicine
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• v.18 no.1
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• pp.64-69
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• 2021

Primary cilium has a signal transduction function that is essential for brain development, and also determines cell polarity and acts as a mediator for important signaling systems, especially the Sonic Hedgehog (SHH) pathway. TBC1D32 is a ciliary protein, implicated in SHH signaling. Biallelic mutations in the TBC1D32 gene causes a kind of ciliopathy, heterogeneous developmental or degenerative disorders that affect multiple organs, including the brain. Here we report a boy who carried compound heterozygous variants in TBC1D32. The patient showed hypotonia, respiratory difficulty, and multiple anomalies at his birth. He was diagnosed with congenital hypopituitarism and treated with T4, hydrocortisone, and growth hormone. Despite the hormonal replacement, the patient needed long-term respiratory support with tracheostomy and nutritional support with a feeding tube. His developmental milestones were severely retarded. Hydrocephalus and strabismus developed and both required surgery, during the outpatient follow-up. Whole-exome sequencing indicated compound heterozygous variants, c.2200C>T (p.Arg734^*) and c.156-1G>T, in TBC1D32 gene. This is the first Korean case of TBC1D32-related ciliopathy and we reported detailed and sequential clinical features. This case demonstrated the utility of whole-exome sequencing and provided valuable clinical data on ultra-rare disease.

• Journal of Veterinary Clinics
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• v.39 no.6
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• pp.400-404
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• 2022

A 3-year-old Thoroughbred gelding presented with left laryngeal hemiplegia with a history of laryngoplasty (tie-back surgery) failure. Postoperative endoscopy revealed no abduction or no inflammatory changes in the left arytenoid cartilage. The owner opted for the horse to undergo partial arytenoidectomy due to failed laryngoplasty. A tracheostomy tube was intubated through a mid-cervical tracheotomy to secure the airway under general anesthesia, and; laryngotomy was performed to access the arytenoid cartilage in dorsal recumbency. A partial arytenoidectomy was performed with endoscopic assistance through the left nostril, and the left arytenoid cartilage was removed, excluding the muscular process. Antibiotic and anti-inflammatory agents were administered postoperatively, and the incision site was cleaned using normal saline and antibiotic ointment twice daily. On the 12th postoperative day, endoscopy revealed redundant corniculate process mucosa at the surgical site, which was removed using rongeur forceps directly through the previous laryngotomy incision. The horse showed no significant complications during the hospitalization. Two months after surgery, the surgical site reportedly recovered with no evidence of granulation tissue. The horse returned to training and racing 3 and 7 months postoperatively, respectively. This is the first case report of a partial arytenoidectomy in a horse in South Korea. In this case, the horse returned to training after partial arytenoidectomy without significant complications, indicating that partial arytenoidectomy could be beneficial for failed laryngoplasty.

• Tuberculosis and Respiratory Diseases
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• v.46 no.4
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• pp.500-511
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• 1999

Background: Noninvasive positive pressure ventilation (NPPV) using facial or nasal mask have been widely used for several years in stable patients with chronic neuromuscular disease or central alveolar hypoventilation, and recently have been tried in patients with acute respiratory failure. In a few studies, NPPV was also used to rescue the patients with post-extubation respiratory failure. However, yet it has not been adopted as a weaning method in patients on long-term mechanical ventilation. So we performed this prospective clinical study to evaluate the usefulness of NPPV as a weaning method after removing endotracheal tube intentionally in patients on long-term mechanical ventilation. Method: Twelve patients who had been on invasive mechanical ventilation over 10 days were enrolled and 14 trials of NPPV were done. All had failed at least one weaning trial and showed ventilator dependence(pressure support requirement between 8-15cm $H_2O$, and PEEP requirement between 5-10cm $H_2O$), so tracheostomy was being considered. After removing the endotracheal tube, NPPV was applied using facial mask. Respiratory rate, arterial blood gas, pressure support level, and PEEP level were monitored just before intended extubation, at 30 minutes, 1 to 6, 6 to 12, 12 to 24 hours, 2nd day, and 3rd day following initiation of NPPV, and just before weaning from NPPV. The successful weaning was defined as spontaneous breathing off the ventilator for 48 hours or longer without respiratory distress. Results: The weaning through NPPV after intended extubation was successful in 7(50%) of 14 trials, and tracheostomy could be avoided in them. There were no differences in age, sex, APACHE III score, duration of invasive mechanical ventilation, baseline respiratory rate, $PaCO_2$ $PaO_2/FiO_2$, and ventilatory requirement(PS and PEEP) between the success and failure groups. In the success group, respiratory rate, pH, $PaCO_2$, and $PaO_2/FiO_2$ were not different between invasive MV and NPPV period. But in the failure group, pH decreased after 30 minutes of NPPV initiation compared with that of invasive MV($7.40\pm0.08$ vs. $7.34\pm0.06$, p<0.05). The causes of failure were worsening of ABG(n=3), retained tracheal secretion(n=2), mask intolerance(n=1), and flail chest(n=1). Conclusion: NPPV may be worth trying as a bridge method in weaning patients on long-term invasive mechanical ventilation.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1981.05a
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• pp.6-7
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• 1981

With the improvement of living standard and educational level of the people, there is an increasing awareness about the dangers of toxic substances and lethal drugs. In addition to the above, the governmental control of these substances has led to a progressive decrease in the accidents with corrosive substances. However there are still sporadic incidences of suicidal attempts with the substances due to the unbalance between the cultural development in society and individual emotion. The problem is explained by the fact that there is a variety of corrosive agents easily available to the people due to the considerable industrial development and industrialization. Salzen(1920), Bokey(1924) were pioneers on the subject of the corrosive esophagitis and esophageal stenosis by dilatation method. Since then there had been a continuing improvement on the subject with researches on various acid(Pitkin, 1935, Carmody, 1936) and alkali (Tree, 1942, Tucker, 1951) corrosive agents, and the use of steroid (Spain, 1950) and antibiotics. Recently, early esophagoscopic examination is emphasized on the purpose of determining the way of the treatment in corrosive esophagitis patients. In order to find the effective treatment of such patients in future, the authors selected 96 corrosive esophagitis patients who were admitted and treated at the ENT department of Severance hospital from 1971 to March, 1981 to attempt a clinical study. 1. Sex incidence……male: female=1 : 1.7, Age incidence……21-30 years age group; 38 cases (39.6%). 2. Suicidal attempt……80 cases(83.3%), Accidental ingestion……16 cases (16.7%). Among those who ingested the substance accidentally, children below ten years were most numerous with nine patients. 3. Incidence acetic acid……41 cases(41.8%), lye…20 cases (20.4%), HCI……17 cases (17.3%). There was a trend of rapid rise in the incidence of acidic corrosive agents especially acetic acid. 4. Lavage……57 cases (81.1%). 5. Nasogastric tube insertion……80 cases (83.3%), No insertion……16 cases(16.7%), late admittance……10 cases, failure…4 cases, other……2 cases. 6. Tracheostomy……17 cases(17.7%), respiratory problems(75.0%), mental problems (25.0%). 7. Early endoscopy……11 cases(11.5%), within 48 hours……6 cases (54.4%). Endoscopic results; moderate mucosal ulceration…8 cases (72.7%), mild mucosal erythema……2 cases (18.2%), severe mucosal ulceration……1 cases (9.1%) and among those who took early endoscopic examination; 6 patients were confirmed mild lesion and so they were discharged after endoscopy. Average period of admittance in the cases of nasogastric tube insertion was 4 weeks. 8. Nasogastric tube indwelling period……average 11.6 days, recently our treatment trend in the corrosive esophagitis patients with nasogastric tube indwelling is determined according to the finding of early endoscopy. 9. The No. of patients who didn't given and delayed administration of steroid……7 cases(48.9%): causes; kind of drug(acid, unknown)……12 cases, late admittance……11 cases, mild case…9 cases, contraindication……7 cases, other …8 cases. 10. Management of stricture; bougienage……7 cases, feeding gastrostomy……6 cases, other surgical management……4 cases. 11. Complication……27 cases(28.1%); cardio-pulmonary……10 cases, visceral rupture……8 cases, massive bleeding……6 cases, renal failure……4 cases, other…2 cases, expire and moribund discharge…8 cases. 12. No. of follow-up case……23 cases; esophageal stricture……13 cases and site of stricture; hypopharynx……1 case, mid third of esophagus…5 cases, upper third of esophagus…3 cases, lower third of esophagus……3 cases pylorus……1 case, diffuse esophageal stenosis……1 case.

• Journal of Korean Academy of Fundamentals of Nursing
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• v.6 no.2
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• pp.228-239
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• 1999

In 1990 Home Care Education Programs started when legislation established certification for Home Care Nurses. The Ministry of Health and Welfare proposed a home care education curriculum which has 352 class hours and 248 hours of 'family nursing and practice'. Though Home Care Education Programs have been offered in 11 home care educational institutes, there has been no formal revision for the home care education programs. Also a first and second home care demonstration projects have been carried out, but there has been no research on outcomes for home care education as applied in home care practice. The purposes of this study were to identify the important content areas for home care nursing as perceived by home care nurses, and to identify their clinical competence in each of these areas, and from these to identify the education needs. The sample was 107 home care nurses who were working in home care demonstration hospitals and community-based institutions which have been offering home care services. Responses were received from 88 nurses, comprising a 82.2% return rate, and 86 were included in the final analysis. The instrument used was a modification of the instrument developed by Caie-Lawrence et(1995) and Moon's(1991) instrument on home care knowledge. The instrument's Cronbach's coefficient was 0.982. Among the respondents, 64% were working at home care demonstration hospitals and 36% were working at community-based institutions. Their home care experiences were from one month to six years, with a mean of 20.6 months. The importance rating for home care education content was 3.42 0.325, which means importance was rated relatively high. Technical aspects of home care were identified the most important. Five items 'education skill', 'counseling skill', 'interview skill', 'wound care skill', 'bed sore care skill' received 100% importance ratings. The competency rating was 2.87 0.367 and 'technical aspects of home care' was the highest, and 'application to home care skill' was the lowest. Home care nurses' education needs were identified and compared to the importance ratings and competency ratings. Eleven items were identified as the highest in the importance areas and eleven items were in the lowest competency areas. High importance ratings matched with low competency ratings determined training needs, but there was no matching items in this study. In the lowest competency areas four items were excluded, because of not being applicable in current home care practice. Therefore total eighteen items were identified as home care education needs. These items are 'bed sore care skill', 'malpractice', 'wound care skill', 'general infection control', 'change and management of tracheostomy tubes', 'CVA patient care', 'Hospice care', 'pain management', 'urinary catheterization and management', 'L-tube insertion and managements', 'Respirator use and management skill', 'infant care', 'prevention to burnout', 'child assessment', 'CAPD', 'infant assessment', 'computer literacy', and 'psychiatry patient care'.

• The Journal of the Korea Contents Association
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• v.14 no.5
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• pp.211-223
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• 2014

The aim of this study was to identify the levels of family members' burden and to describe factors influencing on famiCX*//8ly caregiver's burden when they are planned to transfer to general ward from the intensive care units (ICU). A descriptive survey was used with a convenience sampling of 101 family members of ICU patients at S university hospital in C city, Korea from August to November, 2010. The data were analyzed with SPSS 18.0 program. The level of object burden in family members had statistically significant differences with level of consciousness, number of patients' monitoring devices, and tracheostomy tube. The level of subject burden had statistically significant differences according to patients' gender, patients' education level, and relationship with patients. Transfer anxiety was showed statistically positive correlation with objective and subjective burden. We found transfer anxiety was statistically significant predictor of family caregiver's objective burden with explanatory power 12.7%. Family members' subjective burden were statistically influenced by patients' gender and transfer anxiety. These factors explained 23.8% out of total variance of family members' objective burden. The structured individualized method of transfer is recommended with further research of ICU families to further examine the dimension of transfer anxiety and how it affects family members' burden and patient outcomes.

• Journal of Chest Surgery
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• v.8 no.1
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• pp.37-44
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• 1975

Authors have reviewed the records of seven patients of multiple rib fractures with severe flail chest who were admitted to Hanyang University Hospital during the 3 years period from 1972 through 1975. Of the seven patients studied, automobile accidents led to the injuries in 4 cases, two patients were injured in fall from a tree and on the ox-heading. All who had a blunt trauma without any open wound on the chest. The numbers of the fractured ribs accounted for 6 to 9 of the ribs including double fractures from 3 to 5 ribs. The left side fractures occurred in the 6 patients and in the right only one patient. Thus the flail segment was more often located in the left antero-lateral position than in the right lateral position [the ratio was 6:1].. All cases had associated injuries. The injuries and multiple fractures were the most common associated injuries occurring in four and five of the patients respectively. The patients were classified as having associated head injuries when they were admitted in comatose or semicomatose state. When a major degree of instability of the thoracic cage exists, adequate respiratory change is not possible. For this reason the tracheostomy was performed in five patients in an acutely injured patient with flail chest only after an endotracheal tube has been inserted or after an endotracheal suction. All patients had secondary complications in the pleural cavity, such as hemothorax or hemopneumothorax with or without intrapulmonary hemorrhage and subcutaneous emphysema. Therefore, closed thoracostomy was performed in five patients in the emergency room. The thoracotomy was required in four patients: immediate operation without closed thoracostomy was performed in two patients and the thoracotomy was indicated in two patients after closed thoracostomy, because of increasing intrathoracic hemorrhage. As to the fixation of the flail segments, authors employed two techniques; one was towel clip traction of the flail segments and the other was intramedullary insertion of Kirschner`s wire in to the double fractured rib fragments for the fixation of the flail segments [Kirschner`s wire fixation]. Because` of an different results in the course of treatment between two techniques, data from patients with towel clip traction was compared with those from patients with thoracotomy and Kirschner`s wire fixation of the flail segments. Of the three patients with towel clip traction, two patients required bronchoscopic toilet due to lung atelectasis which developed because of inadequate motion of thoracic cage and poor expectoration. This was in contrast to the four patients with thoracotomy and Kirschner`s wire fixation, who didn`t these complication because of adequate motion of the thoracic cage and subsequent good expectoration.

• Archives of Reconstructive Microsurgery
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• v.8 no.2
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• pp.154-162
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• 1999

The laryngopharyngectomy for tumor ablation is the most common indication for pharyngoesophageal reconstruction in our country. Most of these cases are advanced laryngeal cancer that has spread beyond the larynx, pharynx and cervical esophagus. Such patients are obviously unable to breathe, swallow, or speak in the normal manner. The ideal reconstruction would restore normal anatomy, permitting patients to breathe and swallow without aspiration, and would not require a permanent tracheostomy. Reconstruction of the pharyngoesophageal defect traditionally been carried out with tubed local random flap, deltopectoral or musculocutaneous flap. Another approach is the pedicled enteric flap. But microsurgical reconstruction of the pharyngoesophagus, using either the free jejunal or the tubed radial forearm flap, have now become the preferred technique. Among them, we used jejunal free flap in 39 cases, tubed radial forearm free flap in 5 cases, patched radial forearm free flap in 2 cases and pectoralis major myocutaneous island flap in 2 cases from December 1990 to Febrary 1999. In this paper we illustrated that both forearm and jejunal free flap is a usful alternative in reconstruction of hypopharynx and cervical esophagus.

• Journal of Korean Academic Society of Home Health Care Nursing
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• v.7 no.1
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• pp.26-38
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• 2000

The purpose of this study was to estimate home care nursing cost for the patient with Cerebrovascular Disease based on a bundle of home care nursing services This study was conducted through four steps. The first step was to investigate home care nursing activities that were offered to the patient with Cerebrovascular Disease(CD) by home care nurse. The second step was to investigate the time spent on home care nursing service and to calculate labor and manufacturing cost. The third step was to calculate home care nursing cost per minute. And at the fourth step, home care nursing cost for a patient with Cerebrovascular Disease based on a bundle of home care nursing service was calculated. The results of the study were as follows: 1) The number of direct home care nursing activities for the patient with CD was 108, and the time of each activity was spent from 1 to 10 minutes. 2) Average time per visit was 51 minute, and the firs visit time were spent 1.6 times higher than 2nd visit time. 3) Nursing cost per minute(cost per visit ${\\}\;22,565\;\div\;$ average time per visit 51 minutes) was ${\\}\;442$. The cost per visit was calculated on Basic visiting cost(nurse's labor cost ${\\}\;15,760$ + management cost ${\\}\;6,805$) divided by average time per visit(51 minutes). 4) Home care nursing cost to the patient with CD based on bundle of home care nursing service was consisted of basic home care nursing cost, the cost of a bundle of service practiced on visit. and transportation fee. Basic home nursing cost(the time spent on basic home nursing service 20 minutes ${\times}$ nursing cost per minute ${\\}\;442$) was ${\\}\;8,840$. The cost of the bundle of home care nursing services to the patient with CD was calculated as self care ${\\}\;2.898$, Tracheostomy care ${\\}\;10,166$, immobility care ${\\}\;6,188$, sore care ${\\}\;6,188$. Foley care ${\\}\;6,630$, and Levin tube or Gastrostomy care ${\\}\;7.514$. Transportation fee which was composed of the labor cost for transportation(${\\}\;5,122$) and the car management cost(${\\}\;3.876$) was ${\\}\;8,998$. Home care nursing cost to the patient with CD based on bundle of home care nursing services consisted of basic home care nursing cost, the cost of a bundle of service practiced on visit, and transportation fee. It will contribute to improve quality of home care service, because of giving appreciate incentives to home care nurses. And it will be more efficient than current cost of hospital based home care. But it need to management than calculation of the current fee-for-services of home care.

• Journal of Korean Academy of Nursing
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• v.13 no.3
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• pp.1-33
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• 1983

The role and function of nursing care nowadays, tend to acquire sophisicated technology because specialization has expended due to increase of the medical population and the improvement of national health standards. To implement nursing care independently as a professional nurse, the apprehension of specific knowledge and skill should be acquired during basic nursing education. So it is important for nursing education not only to include theory and actual techniques, but also to strengthen the practical training in the actual clinical setting. This study was carried out with the following objectives; 1. To survey the detailed content and frequency of actual nursing students display during their clinical training. 2. To investigate the detailed content and frequency of actual nursing behavior which students display in each clinical a area. 3. To identify the motive for selection of nursing as their major and to determine the degree of self confidence, extent of knowledge and recognition of nursing responsibility. 4. To observe the relationship between actual nursing behavior and each of the following; 1) Motive for selecting nursing as a major 2) Self confidence 3) Knowledge of nursing care 4) Recognition of nursing responsibility The conclusions of this study were as follows; 1. Among the detailed nursing behavior which junior nursing college students carry out in clinical training; taking respiration's showed the highest frequency, and taking body temperatures, blood pressures, and pulses and making beds were next in frequency in this order. 2. In detailed nursing behaviors according to clinical area; taking vital signs showed the highest frequency in the emergency room, pediatric ward, orthopedic ward, general surgical ward and internal medicine ward. However, in the operating room, assisting with endotracheal tube insertion and sterile techniques were showen to have the highest frequencies. In nursery, umbilical cord care and the measurement of body weight were the highest in frequency In neurosurgical ward, the measurement of vital signs, changing position and tracheostomy care were the highest in frequency. In obstetric and gynecological ward and in the delivery room, checking duration, intensity and frequency of contractions was the highest in frequency. 3. In regard to the motive for majoring in nursing, the aptitude and interest of the student had the highest percentage(32.86%), and self-confidence in nursing activities (M=3.36), knowledge in nursing activities.(M=3. 09), and the recognition of the nursing activity (M= 3.76) wire in the middle range. 4. When the detailed nursing behaviors were compared with motive, self confidence, knowledge and recognition, it was found that when the nursing behavior was difficult and regarding much endeavor although the motive was high, the frequency of the nursing behavior was rather low. But in the cases in which there was much self confidence and a high level of skill was required, nursing behavior was carried more frequently. When there was muck self confidence and skill was not required, the frequency of nursing behavior was rather low. In the cases of a high level of knowledge, the frequency of nursing behavior was low and when recognition for nursing behavior was given the frequency of nursing behavior was low.