A Case of Nasopharyngeal Tuberculosis Which Was Difficult to Differentiate From Sarcoidosis / 대한이비인후과학회지

Nasopharyngeal mass is a disease that can cause unilateral or bilateral nasal obstruction. Common diseases that can cause nasal obstruction include antrochoanal polyp, adenoid remnants, juvenile nasopharyngeal angiofibroma, and nasopharyngeal carcinoma. Nasopharyngeal tuberculosis and nasopharyngeal sarcoidosis are two rare diseases that involve the nasopharynx and manifest as nasopharyngeal mass, and they share common histopathologic findings of granulomatous inflammation. We report a case where a patient complaining of nasal obstruction was found with nasopharyngeal mass with smooth surface, which was difficult to differentiate nasopharyngeal tuberculosis from nasopharyngeal sarcoidosis due to similar clinical manifestations and histopathologic findings.

Comparison of upper and lower body forced air blanket to prevent perioperative hypothermia in patients who underwent spinal surgery in prone position: a randomized controlled trial / 대한마취과학회지

Background@#We compared upper- and lower-body forced-air blankets in terms of their ability to prevent perioperative hypothermia, defined as a reduction in body temperature to < 36.0°C, during the perioperative period in patients undergoing spine surgery in the prone position. @*Methods@#In total, 120 patients scheduled for elective spine surgery under general anesthesia were divided into an upper-warming group (n = 60) and a lower-warming group (n = 60). After inducing anesthesia and preparing the patient for surgery, including prone positioning, the upper and lower bodies of the patients in the upper- and lower-warming groups, respectively, were warmed using a forced-air warmer with specified upper and lower blankets. Body temperature was measured using a tympanic membrane thermometer during the pre- and post-operative periods and using a nasopharyngeal temperature probe during the intraoperative period. Patients were evaluated in terms of shivering, thermal comfort, and satisfaction in the post-anesthesia care unit (PACU). @*Results@#The incidence of intraoperative and postoperative hypothermia was lower in the upper-warming group than in the lower-warming group ([55.2% vs. 75.9%, P = 0.019] and [21.4% vs. 49.1%, P = 0.002]). Perioperative body temperature was higher in the upper-warming group (P < 0.001). However, intraoperative blood loss, postoperative thermal comfort scale and shivering scores, patient satisfaction, and PACU duration were similar in the two groups. @*Conclusions@#The upper-body blanket was more effective than the lower-body blanket for preventing perioperative hypothermia in patients who underwent spine surgery in the prone position.

Incidence of Extravasation in Acute Care Hospitals and Nurses’ Knowledge and Attitude toward Extravasation Prevention and Management / 임상간호연구

Purpose@#Extravasation of diagnostic and therapeutic materials might occur when the intravascular solution leaks into the surrounding tissues. Injury associated with extravasation depends on various factors. It may range from mild skin reaction to severe necrosis. However, the incidence rate for extravasation is largely unknown because of the limited reporting in Korea. Therefore, this study was conducted to identify the incidence of extravasation and nurses’ attitude and knowledge of extravasation for providing high-quality nursing care. @*Methods@#Three acute care hospitals were surveyed to estimate the occurrence of extravasation. Knowledge and attitude toward extravasation were investigated from 793 nurses working in six hospitals. @*Results@#The incidence rate of extravasation was 0.5%. Extravasation commonly occurred in elderly patients aged 66 or older (59.9%) and internal medicine (48.2%), and it happened 13.73±20.68 days after hospitalization on average.It mostly occurred in the forearm site (52.9%) and was mainly caused by parenteral nutrition (33.6%). The mean scores of nurses’ knowledge and attitude were 14.63±2.86 and 28.91±36.00, respectively. There was a significant negative correlation between the subjects' knowledge and attitude (r=-.11, p=.002). @*Conclusion@#It is necessary to have a reporting system that can accurately monitor the occurrence of extravasation for patient safety management. In addition, it is necessary to develop a protocol that can be applied to clinical practice and a nurse education program.

Effects of a Competency-Based Education Program for Inpatient Psychiatric Nurses: A Pre-Post Intervention Study / 정신간호학회지

Purpose@#This study aimed to develop a competency-based educational program for strengthening the competency of psychiatric nurses and evaluate its effects. @*Methods@#The study used a quasi-experimental design. Participants included 64 psychiatric nurses in South Korea: 33 nurses (32 females, 1 male) in the experimental group and 31 nurses (26 females, 5 males) in the control group. A competency-based education program for psychiatric nurses was developed through a literature review and discussion with 12 experts. The program addressed eight topics and provided 24 hours of education over 4 days, for 6 hours each day. The collected data via questionnaires were analyzed statistically. @*Results@#The education program enhanced overall clinical nursing competence and strengthened professional self-growth competence. The management and teaching competencies of both the experimental and the control groups also increased significantly. @*Conclusion@#The findings of this study presented an educational program to strengthen the clinical competency of psychiatric nurses and it was proven that the education had a capacity-building effect.

SLC9A6 -related developmental and epileptic encephalopathy with spike-and-wave activation in sleep: A case report

The gene encoding solute carrier family 9 member 6 (SLC9A6) on Xq26.3 is associated with Christianson syndrome (CS) mimicking Angelman syndrome. In CS, developmental and epileptic encephalopathy (DEE) appears in about 20%, and DEE with spike-and-wave activation in sleep (SWAS) is reported only in several cases. A 10-year-old boy with DEE showed multidrug resistant focal seizures from 6 months of age. He had progressive microcephaly, regression, global developmental delay without speech, hyperkinesia, and truncal ataxia; he had a long thin face, esotropia, and happy demeanor. Brain magnetic resonance imaging demonstrated cerebellar atrophy. Electroencephalogram at 7.5 years of age showed nearly continuous diffuse paroxysms in slow wave sleep. The seizures were responsive to corticosteroids for a while. Trio whole exome sequencing exhibited a likely pathogenic variant of SLC9A6 in the proband and his asymptomatic mother: c.1194dup (p.Leu399AlafsTer12).This is a rare case report of CS with DEE-SWAS in a Korean patient.

Effective ketogenic diet in CACNA1A-related ‘epilepsy of infancy with migrating focal seizures’

Genetic causes of developmental and epileptic encephalopathy (DEE) have been rapidly uncovered from mid-2010s. The mutations of gene enconding calcium channel, voltage-dependent, P/Q type, alpha 1A subunit (CACNA1A) are recently detected in DEE, which gene is already known well in familial hemiplegic migrine type 1 or episodic ataxia type 2. Ketogenic diet therapy (KDT) is effective in some DEE, which data is short in CACNA1A encephalopathy. A 3-month-old male with global developmental delay and multidrug-resistant focal seizures was diagnosed as epilepsy of infancy with migrating focal seizures (EIMFS). Brain magnetic resonance imaging and metabolic screening were all normal. Whole exome sequencing revealed two variants of CACNA1A : c.899A>C, and c.2808del that is from his mother. His seizures disappeared within 3 days whenever on KDT, which recurred without it. To our knowledge, this rare case of EIMFS with novel mutations of CACNA1A, is the first report in CACNA1A encephalopathy becoming seizure-free on KDT.

A Rare Case of Pulmonary Arteriovenous Malformation Caused by Hereditary Hemorrhagic Telangiectasia in a Hemodialysis Patient / 대한내과학회지

Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant disorder resulting in vascular malformation, such as pulmonary arteriovenous malformation (PAVM). Here, we report a rare case of pulmonary arteriovenous malformation caused by HHT in a hemodialysis (HD) patient. A 34-year-old man receiving maintenance HD via radiocephalic arteriovenous fistula developed progressive dyspnea without definite pulmonary edema. His mother had been diagnosed with HHT. He had experienced multiple episodes of epistaxis and had been intermittently treated with blood transfusions because of severe anemia. Blood gas analysis showed hypoxia. Chest computed tomography revealed multiple dilated vessels of variable sizes, continuous with the pulmonary artery throughout both lung fields, consistent with PAVM. After treating pulmonary artery embolization at the largest PAVM, he recovered from his dyspnea symptoms and hypoxia.

A Case of Laryngeal Lymphoepithelial Carcinoma

Laryngeal lymphoepithelial carcinoma (LEC) is a rare tumor with only 34 cases in the published literature. Epidemiologically, laryngeal LEC is extremely rare in Asian. Originally, LEC is a common type of carcinoma in nasopharynx. Laryngeal LEC resembles nasopharyngeal LEC, except that most cases of laryngeal LEC are not associated with Epstein-Barr virus. We present a case of laryngeal LEC which developed at the left false cord extending to true vocal cord, para-glottic space and pre-epiglottic space. Total laryngectomy with bilateral neck dissection was performed. LEC was reported as biopsy confirmation result. The patient underwent postoperative radiotherapy and showed no evidence of recurrence during follow-up period of 42 months. In consideration that LEC in larynx have not been reported in South Korea yet, we introduce the clinical features and treatment outcomes of laryngeal LEC with literature review.

West syndrome with hyperkinesia and cortical visual impairment: A case report of GRIN1 encephalopathy

West syndrome (WS) presenting with infantile spasms, developmental delay, and hypsarrhythmia has genetic etiology in some patients. Movement disorders or visual impairment that share genetic underpinnings with infantile spasms can provide diagnostic clues for specific genetic mutations. Mutations of the GRIN1 gene encoding the glutamate receptor inotropic Nmethyl-D-aspartate subunit can result in WS with hyperkinetic movements, cortical visual impairment, autistic features, and bilateral polymicrogyria. An 11-month-old boy with WS showed hyperkinetic movements and visual impairment. Brain magnetic resonance imaging and metabolic investigations revealed no abnormalities. Whole-exome sequencing revealed a novel likely pathogenic variant (c.1561_1563del; p.Asn521del) of GRIN1 (NM_007327.3). The proband was treated with vigabatrin and became seizure-free within one week. Notably, the cortical blindness improved within 3 months and the hyperkinetic movements resolved one year after the proband became seizure-free. To the best of our knowledge, this is the first report of GRIN1 encephalopathy in Koreans.

Cohen–Gibson syndrome in a family: The first familial case report

Cohen–Gibson syndrome (CGS) was first reported by Cohen et al., who identified the mutation of the gene encoding the embryonic ectoderm development (EED) in a patient with phenotypes similar to Weaver syndrome. CGS manifests as an overgrowth and intellectual disability, in addition to the characteristic facial features and organ anomalies. CGS has been reported in only 11 unrelated patients since 2015. A girl aged 6 years and 3 months presented with seizures. She had macrosomia, a dysmorphic face, and intellectual disability. Her mother and younger sister and brother also had macrosomia, intellectual disability, and similar facial features; additionally, her mother experienced seizures and had an arachnoid cyst, while her siblings had valvar pulmonary stenosis. Whole-exome sequencing for the proband revealed a mutation of EED (c.581A>G, p.Asn194Ser), which was also verified in the mother and both siblings using Sanger sequencing. This is the first report of familial CGS.

West syndrome with hyperkinesia and cortical visual impairment: A case report of GRIN1 encephalopathy

West syndrome (WS) presenting with infantile spasms, developmental delay, and hypsarrhythmia has genetic etiology in some patients. Movement disorders or visual impairment that share genetic underpinnings with infantile spasms can provide diagnostic clues for specific genetic mutations. Mutations of the GRIN1 gene encoding the glutamate receptor inotropic Nmethyl-D-aspartate subunit can result in WS with hyperkinetic movements, cortical visual impairment, autistic features, and bilateral polymicrogyria. An 11-month-old boy with WS showed hyperkinetic movements and visual impairment. Brain magnetic resonance imaging and metabolic investigations revealed no abnormalities. Whole-exome sequencing revealed a novel likely pathogenic variant (c.1561_1563del; p.Asn521del) of GRIN1 (NM_007327.3). The proband was treated with vigabatrin and became seizure-free within one week. Notably, the cortical blindness improved within 3 months and the hyperkinetic movements resolved one year after the proband became seizure-free. To the best of our knowledge, this is the first report of GRIN1 encephalopathy in Koreans.

Cohen–Gibson syndrome in a family: The first familial case report

Cohen–Gibson syndrome (CGS) was first reported by Cohen et al., who identified the mutation of the gene encoding the embryonic ectoderm development (EED) in a patient with phenotypes similar to Weaver syndrome. CGS manifests as an overgrowth and intellectual disability, in addition to the characteristic facial features and organ anomalies. CGS has been reported in only 11 unrelated patients since 2015. A girl aged 6 years and 3 months presented with seizures. She had macrosomia, a dysmorphic face, and intellectual disability. Her mother and younger sister and brother also had macrosomia, intellectual disability, and similar facial features; additionally, her mother experienced seizures and had an arachnoid cyst, while her siblings had valvar pulmonary stenosis. Whole-exome sequencing for the proband revealed a mutation of EED (c.581A>G, p.Asn194Ser), which was also verified in the mother and both siblings using Sanger sequencing. This is the first report of familial CGS.

A Rare Case of Pulmonary Arteriovenous Malformation Caused by Hereditary Hemorrhagic Telangiectasia in a Hemodialysis Patient / 대한내과학회지

Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant disorder resulting in vascular malformation, such as pulmonary arteriovenous malformation (PAVM). Here, we report a rare case of pulmonary arteriovenous malformation caused by HHT in a hemodialysis (HD) patient. A 34-year-old man receiving maintenance HD via radiocephalic arteriovenous fistula developed progressive dyspnea without definite pulmonary edema. His mother had been diagnosed with HHT. He had experienced multiple episodes of epistaxis and had been intermittently treated with blood transfusions because of severe anemia. Blood gas analysis showed hypoxia. Chest computed tomography revealed multiple dilated vessels of variable sizes, continuous with the pulmonary artery throughout both lung fields, consistent with PAVM. After treating pulmonary artery embolization at the largest PAVM, he recovered from his dyspnea symptoms and hypoxia.

A Case of Laryngeal Lymphoepithelial Carcinoma

Laryngeal lymphoepithelial carcinoma (LEC) is a rare tumor with only 34 cases in the published literature. Epidemiologically, laryngeal LEC is extremely rare in Asian. Originally, LEC is a common type of carcinoma in nasopharynx. Laryngeal LEC resembles nasopharyngeal LEC, except that most cases of laryngeal LEC are not associated with Epstein-Barr virus. We present a case of laryngeal LEC which developed at the left false cord extending to true vocal cord, para-glottic space and pre-epiglottic space. Total laryngectomy with bilateral neck dissection was performed. LEC was reported as biopsy confirmation result. The patient underwent postoperative radiotherapy and showed no evidence of recurrence during follow-up period of 42 months. In consideration that LEC in larynx have not been reported in South Korea yet, we introduce the clinical features and treatment outcomes of laryngeal LEC with literature review.

A Rare Case of Fibromuscular Dysplasia with Postpartum Renal Artery Rupture in a Non-Hypertensive Woman / 대한내과학회지

Fibromuscular dysplasia (FMD) of the renal artery is a non-atherosclerotic, non-inflammatory vascular disease that causes stenosis, aneurysm, dissection, and occlusion of the vessel. We report a rare case of a postpartum FMD patient who presented with spontaneous acute perirenal hematoma due to renal artery aneurysm rupture after cesarean section. The 40-year-old patient presented with sudden onset of abdominal pain 2 days after an elective cesarean section performed at full gestational term. A computed tomography scan of the abdomen revealed perirenal hematomas with signs of FMD in both renal arteries. The bleeding was successfully controlled by transcatheter arterial embolization. Short-term continuous renal replacement therapy was performed until her renal function recovered. FMD of the renal artery is rare in pregnant patients. Additionally, aneurysm rupture can be life threatening and requires immediate medical attention and prompt management.

Association between Blood Pressure and Renal Progression in Korean Adults with Normal Renal Function

Background@#Although hypertension (HTN) is a well-established major risk factor for renal progression in patients with chronic kidney disease (CKD), few studies investigating its role in renal deterioration in the general population with normal renal function (NRF) have been published. Here, we analyzed the correlation between blood pressure (BP) and impaired renal function (IRF) in Korean adults with NRF. @*Methods@#Data for the study were collected from the national health screening database of the Korean National Health Insurance Service. Patients whose baseline estimated glomerular filtration rate (eGFR) was less than 60 mL/min/1.73 m 2 or whose baseline urinalysis showed evidence of proteinuria were excluded. IRF was defined as an eGFR below 60 mL/min/1.73 m 2 . We performed follow up for eGFR for 6 years from 2009 to 2015 and investigated IRF incidence according to baseline BP status. We categorized our study population into two groups of IRF and NRF according to eGFR level in 2015. @*Results@#During 6 years of follow-up examinations, IRF developed in 161,044 (2.86%) of 5,638,320 subjects. The IRF group was largely older, and the incidence was higher in females and patients with low income, HTN, diabetes mellitus, dyslipidemia, and obesity compared with the NRF group. Subjects whose systolic BP was more than 120 mmHg or whose diastolic BP was more than 70 mmHg had an increased risk of developing IRF compared with subjects with lower BP (odds ratio [OR], 1.037; 95% confidence interval [CI], 1.014–1.061 vs. OR, 1.021; 95% CI, 1.004–1.038). @*Conclusion@#BP played a major role in renal progression in the general population with NRF.Strict BP control may help prevent CKD in the general population.

Potential Utility of Therapeutic DrugMonitoring of Adalimumab in Predicting Short-Term Mucosal Healing and Histologic Remission in Pediatric Crohn's Disease Patients

Background@#Limited data exist regarding mucosal healing (MH) and therapeutic drug monitoring (TDM) in pediatric Crohn's disease (CD) patients treated with adalimumab (ADL). We aimed to investigate the associations between ADL trough levels (TLs) and MH, and between ADL TLs and histologic remission (HR) at 16 weeks from ADL treatment in pediatric CD patients. @*Methods@#This was a prospective study on moderate-to-severe luminal pediatric CD patients receiving ADL. Ileocolonoscopies and biopsies, as well as clinical activity assessments, laboratory examinations, including tests for ADL TLs and antibody to ADL, were performed 16 weeks after ADL initiation. MH was defined as a Simple Endoscopic Score for CD of 0. HR was defined as the complete absence of microscopic inflammation. @*Results@#Seventeen subjects (13 males, 4 females) were included. At 16 weeks from ADL initiation, 14 (82.4%), 8 (47.1%), and 4 (23.5%) patients achieved clinical remission, MH, and HR, respectively. ADL TLs were significantly higher in patients who achieved MH compared to those who did not (13.0 ± 6.5 vs. 6.2 ± 2.6 μg/mL, respectively; P = 0.023) and also significantly higher in patients who achieved HR compared to those who did not (17.9 ± 5.3 vs. 6.8 ± 2.5 μg/mL, respectively; P = 0.02). The optimal TL for predicting MH was 8.76 μg/mL. @*Conclusion@#Serum ADL TLs at 16 weeks were significantly higher in pediatric patients with CD who achieved MH and HR, respectively. TDM may guide in optimizing treatment efficacy and better target MH in the era of treat-to-target.

Effects of 10-min of pre-warming on inadvertent perioperative hypothermia in intraoperative warming patients: a randomized controlled trial

Background@#This study aimed to evaluate the efficacy of 10-min pre-warming in preventing inadvertent perioperative hypothermia, which is defined as a reduction in body temperature to less than 36.0℃ during the perioperative period in intraoperative warming patients. @*Methods@#In this prospective randomized study, 60 patients scheduled for elective surgery under general anesthesia lasting less than 120 min were divided into two groups: the 10-min pre-warming group (n = 30) and the control group (n = 30). Patients in the 10-min pre-warming group were pre-warmed for 10 min in the pre-anesthetic area using a forced-air warmer set at 47ºC. Intraoperatively, we warmed all patients with a forced-air warmer. Body temperature was measured using a tympanic membrane thermometer pre- or postoperatively and a nasopharyngeal temperature probe intraoperatively. Patients were evaluated on the shivering and thermal comfort scale in the pre-anesthetic area and post-anesthesia care unit. @*Results@#The incidences of intraoperative hypothermia and postoperative hypothermia were similar in both groups (10.7% vs. 28.6%, P = 0.177; 10.7% vs. 10.7%, P = 1.000 respectively). Body temperature was higher in the 10-min pre-warming group (P = 0.003). Thermal comfort during the pre-warming period was higher in the 10-min pre-warming group (P < 0.001). However, postoperative thermal comfort and shivering grades of both groups were similar. @*Conclusions@#Ten minutes of pre-warming has no additional effect on the prevention of inadvertent perioperative hypothermia in intraoperative warming patients.

Nurses' Job Analysis in a Regional Emergency Center Using the DACUM / 임상간호연구

Purpose@#This study aimed to establish the role of and to analyze the job of nurses in a regional emergency center using the Developing a Curriculum(DACUM) process, a job analysis technique. @*Methods@#The DACUM committee was organized, and three workshops were held from June 1st to July 4th, 2016. One hundred two nurses in a regional emergency center participated in a survey from October 3rd to 10th, 2016. The frequency, importance and difficulty of each tasks and task elements in the DACUM were investigated on a Likert scale. @*Results@#The roles of nurses in a regional emergency center were identified and the DACUM chart was developed with 6 duties, 29 tasks and 153 task elements. According to the survey, some tasks such as ‘CPR,’ ‘acute respiratory distress care,’ and ‘drug administration’ showed high frequency and importance or difficulty in all nursing units in common, and different results reflected the characteristics of each nursing unit were also found. @*Conclusion@#The results showed the feature of the regional emergency center. Based on these results, it is necessary to develop educational programs for practice.

Renal Artery Stenosis Presenting at Sudden Flash Pulmonary Edema in a Patient with Solitary Kidney / 대한내과학회지

Flash pulmonary edema is characterized by a sudden episode of dyspnea resulting from acute pulmonary venous congestion, which resolves rapidly. We report a case of renal artery stenosis presenting as flash pulmonary edema in a patient with solitary kidney treated by angioplasty with stent implantation. A 75-year-old man with solitary kidney visited the emergency room with acute shortness of breath. His blood pressure had risen to 206/90 mmHg and a chest radiograph revealed pulmonary edema. Echocardiography and coronary arteriography showed no clear abnormalities, but abdominal computed tomography revealed severe focal stenosis in the left proximal renal artery. A captopril renal scan found that the time to peak and half-time of radioactivity were delayed in the left kidney. Percutaneous transluminal angioplasty was performed, followed by stent implantation. After this procedure, the stenotic segment was completely dilated and blood pressure returned to the normal range.