PURPOSE: Medullary sponge kidney (MSK) is a rare congenital disease characterized by diffuse ectasia or dilatation of precalyceal collecting tubules. MSK incidence and prevalence in the general population is uncertain and only a few patients are reported especially in the pediatric age. There has been increasing reports of patients with MSK who have other malformative disorders. Also several case reports concerning about etiological association of some genes. METHODS: Collaborative study through nation-wide survey was done to investigate the incidence and etiological association of some genes such as GDNF gene, ATP6V1B1, ATP6V0A4 gene in developing MSK in Korean children. RESULTS: Four cases of MSK who have various other malformative disorders were collected. There are no mutations of GDNF gene, ATP6V1B1, ATP6V0A4 gene in all patients. CONCLUSION: MSK is one of the very rare diseases in pediatric age. The etiological association of GDNF gene , ATP6V1B1, ATP6V0A4 gene in developing MSK in Korean children is not proved.
Child ; Dilatation ; Dilatation, Pathologic ; Glial Cell Line-Derived Neurotrophic Factor ; Humans ; Incidence ; Medullary Sponge Kidney ; Prevalence ; Rare Diseases

Purpose: : The purpose of this study was to understand and describe the experiences of the advanced nurse practitioner (APN) system used by healthcare providers including APNs, doctors who worked with APNs, and APN master’s course professors at a graduate school. Methods: : Qualitative data were collected via snowball sampling. The participants were nine APNs, six doctors, and three professors. They were divided into three focus groups, each of which consisted of all three types of healthcare providers. Data were collected via interviews with the three focus groups conducted from September to October 2019. All interviews were audiotaped and transcribed verbatim. The transcribed data then underwent qualitative content analysis. Results: : Based on the data, we extracted four themes and 14 categories. The themes were “Role and system of APNs started according to healthcare environment changes”, “Optimal healthcare provider to ensure quality of care”, “Confused role and system of APNs due to incomplete medical law”, and “Tasks for the stable operation of the APN system.” Conclusion : For quality treatment and safety of patients, a legal basis must be established for the APN system. For its stable operation, social consensus regarding legislation about APNs’ scope of practice is required. Finally, a discussion is necessary about the integration of APNs’ 13 fields.

Purpose: : The purpose of this study was to understand and describe the experiences of the advanced nurse practitioner (APN) system used by healthcare providers including APNs, doctors who worked with APNs, and APN master’s course professors at a graduate school. Methods: : Qualitative data were collected via snowball sampling. The participants were nine APNs, six doctors, and three professors. They were divided into three focus groups, each of which consisted of all three types of healthcare providers. Data were collected via interviews with the three focus groups conducted from September to October 2019. All interviews were audiotaped and transcribed verbatim. The transcribed data then underwent qualitative content analysis. Results: : Based on the data, we extracted four themes and 14 categories. The themes were “Role and system of APNs started according to healthcare environment changes”, “Optimal healthcare provider to ensure quality of care”, “Confused role and system of APNs due to incomplete medical law”, and “Tasks for the stable operation of the APN system.” Conclusion : For quality treatment and safety of patients, a legal basis must be established for the APN system. For its stable operation, social consensus regarding legislation about APNs’ scope of practice is required. Finally, a discussion is necessary about the integration of APNs’ 13 fields.

Purpose: This study aimed to investigate the nationwide intention to delegate clinical practice of medical specialists in accordance with the enactment of the scope of practice for advanced practice nurses (APNs). Methods: Data were collected from October to December 2021 using Google Surveys. In total, 147 medical specialists from 12 provinces responded to the survey. The survey questionnaire was categorized into four legislative draft duties, according to the scope of practice (a total of 41 tasks): Twenty-nine tasks on treatments, injects, etc., performed under the guidance of a physician and other activities necessary for medical treatment (treatment domain); two tasks on collaboration and coordination; six tasks on education, counseling, and quality improvement; four regarding other necessary tasks. Participants were asked whether they were willing to delegate the tasks to APN. Results: The intention to delegate tasks to APN was higher for non-invasive tasks such as blood sampling (97.3%) or simple dressing (96.6%). Invasive tasks such as endotracheal tube insertion (10.2%), sampling: bone marrow biopsy & aspiration (23.8%) showed low intention to delegate in the treatment domain. Participants who were older, male, and had more work careers with APN, showed a higher intention to delegate tasks. Conclusion To prevent confusion in the clinical setting, a clear agreement on the scope of APN practice as APN delegated by physicians should be established. Based on this study, legal practices that APN can perform legally should be established.

Background and Objectives: The directed differentiation of pluripotent stem cells into motor neurons is critical for the development of disease modelling and therapeutics to intervene degenerative motor neuron diseases. Cell surface receptor Cdo functions as a coreceptor for Sonic hedgehog (Shh) with Boc and Gas1 in the patterning of ventral spinal cord neurons including motor neurons. However, the discrete function of Cdo is not fully understood. Methods: and Results: In this study, we examined the role of Cdo in motor neuron generation by utilizing in vitro differentiation of Cdo＋/＋ and Cdo−/− embryonic stem cells (ESCs). In response to Shh, Cdo−/− ESCs exhibited impaired expression of motor neuron specification markers while dorsal interneuron specification markers were significantly increased, compared to Cdo＋/＋ ESCs. Reactivation of Shh signalling pathway with Smoothened (Smo) agonist (SAG) restored motor neuron specification in Cdo−/− ESCs. In addition, electrophysiological analysis revealed the immature electrical features of Cdo−/− ESCs-derived neurons which was restored by SAG. Conclusions Taken together, these data suggest that Cdo as a Shh coreceptor is required for the induction of motor neuron generation by fully activating Shh signalling pathway and provide additional insights into the biology of motor neuron development.

Acute airway obstruction after hemoptysis occurs due to the presence of blood clots. These conditions may result in life-threatening ventilation impairment. We report a case of obstruction of the large airway by endobronchial blood clots which were removed using bronchoscopic cryotherapy at the bedside of intensive care unit. A 66-year-old female with endometrial cancer who had undergone chemotherapy, was admitted to the intensive care unit due to neutropenic fever. During mechanical ventilation, the minute ventilation dropped to inadequately low levels and chest radiography showed complete opacification of the left hemithorax. Flexible bronchoscopy revealed large blood clots obstructing the proximal left main bronchus. After unsuccessful attempts to remove the clots with bronchial lavage and forceps extraction, blood clots were removed using bronchoscopic cryotherapy. This report shows that cryotherapy via flexible bronchoscopy at the bedside in the intensive of intensive care unit is a simple and effective alternative for the removal of endobronchial blood clots.
Aged ; Airway Obstruction ; Bronchi ; Bronchoalveolar Lavage ; Bronchoscopy ; Cryotherapy* ; Drug Therapy ; Endometrial Neoplasms ; Female ; Fever ; Hemoptysis ; Hemorrhage ; Humans ; Intensive Care Units* ; Radiography ; Respiration, Artificial ; Surgical Instruments ; Thorax ; Ventilation

We report our experience with a case of performing laparoscopy-assisted total gastrectomy along with pancreas-preserving splenectomy for treating early gastric cancer. Laparoscopy-assisted total gastrectomy was planned for a 62-year-old male patient with a double early gastric cancer located in the upper and lower third of the stomach. Five trocars were placed and we used a harmonic scalpel to dissect the greater curvature. Enlarged splenic hilar lymph node was encountered and they were proved to be metastasis by frozen section biopsy. We then performed total gastrectomy with pancreas-preserving splenectomy for the purpose of completely dissecting the lymph nodes along the splenic artery and splenic hilum. We created a 4 cm sized longitudinal mini-laparotomy below the xiphoid process to remove the specimen, and anastomosis was done via the Roux-en-Y method. The patient was discharged on the 9th postoperative days after an uneventful recovery. Our experience shows that laparoscopy-assisted total gastrectomy with pancreas-preserving splenectomy is a relatively safe procedure for treating upper third early gastric cancer with metastatic splenic hilar lymph nodes.
Biopsy ; Frozen Sections ; Gastrectomy* ; Humans ; Lymph Nodes ; Male ; Middle Aged ; Neoplasm Metastasis ; Splenectomy* ; Splenic Artery ; Stomach ; Stomach Neoplasms* ; Surgical Instruments

PURPOSE: This study aimed to propose a common scope of practice (SOP) for 13 specialties of Advanced Practice Nurses (APNs) in Korea.METHODS: The first draft of a common SOP was extracted from domestic and international laws with a literature review by 17 experts from the Korean Association of Advanced Practice Nurses (KAAPN). Then, the common SOP was finalized after comparing the activities of APNs in clinical settings.RESULTS: A total of 70 duties were identified and six categories were suggested for the common SOP. The SOP proposed by the KAAPN featured the following: 1) identification of and discrimination between health problems; 2) prescription and implementation of diagnostic tests; 3) treatment of injuries and diseases while implementing measures to prevent exacerbation; 4) prescription of medicinal products in line with 1) to 3); 5) referral and consultation; and 6) education and counseling. It was then confirmed that the proposed six categories in the common SOP reflected all the duties performed by APNs in clinical practice, including all 40 activities.CONCLUSION: The results of this study can be used as evidence for the legalization of a common SOP for APNs. Given the increasing multidisciplinary team approach adopted in Korean hospitals, it may be desirable to establish a broader SOP to reflect the diverse duties of APNs.
Counseling ; Diagnostic Tests, Routine ; Discrimination (Psychology) ; Education ; International Law ; Korea ; Prescriptions ; Professional Practice ; Referral and Consultation

BACKGROUND/AIMS: Delay in symptom-to-door time (SDT) in patients with acute ST-segment elevation myocardial infarction (STEMI) is the most important factor in the prediction of short and long-term mortality. The purpose of this study was to investigate the social and clinical factors affecting SDT in patients with STEMI. METHODS: We analyzed 784 patients (61.0 +/- 13.2 years, 603 male) diagnosed with STEMI from November 2005 to February 2012. The patients were divided into four groups according to SDT: Group I (n = 163, < or = 1 h), Group II (n = 183, 1-2 h), Group III (n = 142, 2-3 h) and Group IV (n = 296, > 3 h). RESULTS: Delay in SDT increased with age (Group I, 58.4 +/- 12.0; Group II, 59.4 +/- 13.3; Group III, 62.0 +/- 12.8; Group IV, 63.0 +/- 13.8 years, p = 0.001). In 119 patients, transportation was less frequently used as the delay in SDT (41.7% vs. 29.0% vs. 26.1% vs. 9.8%, p < 0.001). By multiple logistic regression analysis, family history [OR, 0.488; CI, 0.248-0.959; p = 0.037], previous ischemic heart disease [OR, 0.572; CI, 0.331-0.989; p = 0.045], no occupation [OR, 1.600; CI, 1.076-2.380; p = 0.020] and method of transportation [OR, 0.353; CI, 0.239-0.520; p < 0.001] were independent predictors of delay in SDT. CONCLUSIONS: Our study shows that general education about cardiovascular symptoms and a prompt emergency call could be important to reduce SDT in STEMI.
Education ; Emergencies ; Humans ; Logistic Models ; Mortality ; Myocardial Infarction* ; Myocardial Ischemia ; Occupations ; Transportation

BACKGROUND/AIMS: We compared the efficacy and safety of the second-generation everolimus-eluting stent (EES) and the third generation biolimus-eluting stent (BES) in patients with acute myocardial infarction (AMI). METHODS: We analyzed 629 consecutive patients (mean age 65.1 +/- 11.2 years, 426 males) with AMI undergoing percutaneous coronary intervention from February 2008 to April 2012. They were divided into two groups according to stent type (EES group, n = 426; BES group, n = 203). The primary end-point was 2-year major adverse cardiac events (MACEs), defined as the composite of all-cause death, myocardial infarction, target vessel revascularization, non-target vessel revascularization and target lesion revascularization. The secondary end-point was 2-year target lesion failure (TLF). RESULTS: There were no significant differences in baseline characteristics, except that the patients with EES had a significantly higher prevalence of diabetes mellitus (34.7 vs. 22.7%, p = 0.002) and were older (67.1 +/- 11.3 vs. 64 +/- 12.9 years, p = 0.039) compared with the patients with BES. After propensity score matching, 2-year clinical outcomes showed no differences in composite MACEs or TLF between the two groups. Multivariate Cox regression analysis showed that stent type was not a predictor of 2-year mortality or MACEs. However, older age (hazard ratio [HR] 1.037, 95% confidence interval [CI] 1.014-1.060, p = 0.001), diabetes mellitus (HR 2.247, 95% CI 1.426-3.539, p = 0.001) and a left ventricular ejection fraction < or = 45% (HR 3.007, 95% CI 1.978-4.573, p = 0.001) were independent predictors for 2-year MACEs in patients undergoing EES or BES. CONCLUSIONS: Patients with BES had similar clinical 2-year outcomes compared with EES patients with AMI.
Diabetes Mellitus ; Humans ; Mortality ; Myocardial Infarction* ; Percutaneous Coronary Intervention ; Prevalence ; Prognosis ; Propensity Score ; Stents* ; Stroke Volume