No abstract available.
Tonsillectomy*

General anesthesia is the main strategy for almost all thoracic surgeries. However, a growing body of literature has reported successful cases of non-intubated thoracic surgery with regional anesthesia. This alternative strategy not only prevents complications related to general anesthesia, such as lung injury, incomplete re-expansion and intubation related problems, but also accords with trends of shorter hospital stay and lower overall costs. We experienced a successful case of non-intubated thoracoscopic decortication for a 68-year-old man who was diagnosed as empyema while the patient kept spontaneously breathing with moderate sedation under thoracic epidural anesthesia. The patient showed a fast recovery without concerns of general anesthesia related complications and effective postoperative analgesia through thoracic epidural patient-controlled analgesia device. This is the first report of non-intubated thoracoscopic surgery under thoracic epidural anesthesia in Korea, and we expect that various well designed prospective studies will warrant the improvement of outcomes in non-intubated thoracoscopic surgery.
Aged ; Analgesia ; Analgesia, Patient-Controlled ; Anesthesia, Conduction ; Anesthesia, Epidural* ; Anesthesia, General ; Conscious Sedation ; Empyema* ; Humans ; Intubation ; Korea ; Length of Stay ; Lung Injury ; Prospective Studies ; Respiration ; Thoracic Surgery ; Thoracoscopy*

Background: Patients with stable psoriasis showing clearear-clear response can consider extending the dosing interval of biologics. However, few studies have reported the treatment outcomes following irregular dosing intervals of biologics in patients with psoriasis. Objective: We compared treatment outcomes after regular and irregular dosing intervals of biologics in patients with psoriasis. Methods: This single-center, retrospective observational study included patients who received biologics for treatment of plaque psoriasis between January 1, 2014 and December 31, 2019. We compared patient demographics, clinical characteristics, biologics administered, and treatment outcomes based on the regularity of the dosing interval. Results: Among 95 patients investigated, 63 (66.3%) received biologics at regular dosing intervals. We observed no significant intergroup differences in the final Psoriasis Area Severity Index (PASI) scores (1.2 vs. 1.8, p=0.16) and in the percentage improvement in PASI scores from baseline levels (−89.8% vs. −90.8%, p=0.68). The rate at which biologics were switched was higher in the irregular-dosing group than in the regular-dosing group; however, the difference was statistically nonsignificant (28.1% vs. 12.7%, p=0.06). We observed a significant intergroup difference in patients who were administered guselkumab at baseline (12 [21.8%] vs. 0 [0.0%], p=0.01). Conclusion This study showed that compared with regular dosing intervals, irregular dosing intervals of biologics were associated with high rates of switching of these agents, although we observed no statistically significant differences with regard to PASI scores. Therefore, it is important to adhere to the standard dosing schedule prescribed for biologics, and guselkumab may improve patient compliance.

Background: Patients with stable psoriasis showing clearear-clear response can consider extending the dosing interval of biologics. However, few studies have reported the treatment outcomes following irregular dosing intervals of biologics in patients with psoriasis. Objective: We compared treatment outcomes after regular and irregular dosing intervals of biologics in patients with psoriasis. Methods: This single-center, retrospective observational study included patients who received biologics for treatment of plaque psoriasis between January 1, 2014 and December 31, 2019. We compared patient demographics, clinical characteristics, biologics administered, and treatment outcomes based on the regularity of the dosing interval. Results: Among 95 patients investigated, 63 (66.3%) received biologics at regular dosing intervals. We observed no significant intergroup differences in the final Psoriasis Area Severity Index (PASI) scores (1.2 vs. 1.8, p=0.16) and in the percentage improvement in PASI scores from baseline levels (−89.8% vs. −90.8%, p=0.68). The rate at which biologics were switched was higher in the irregular-dosing group than in the regular-dosing group; however, the difference was statistically nonsignificant (28.1% vs. 12.7%, p=0.06). We observed a significant intergroup difference in patients who were administered guselkumab at baseline (12 [21.8%] vs. 0 [0.0%], p=0.01). Conclusion This study showed that compared with regular dosing intervals, irregular dosing intervals of biologics were associated with high rates of switching of these agents, although we observed no statistically significant differences with regard to PASI scores. Therefore, it is important to adhere to the standard dosing schedule prescribed for biologics, and guselkumab may improve patient compliance.

WAGR (Wilms tumor, aniridia, genitourinary anomalies, and mental retardation) syndrome is a rare contiguous gene deletion syndrome caused by deleting genes including WT1 and PAX6 genes in 11p13 region, which is characterized by Wilms tumor, aniridia, genitourinary abnormalities, and intellectual disability. We report the clinical and cytogenetic characteristics of one Korean patient with WAGR syndrome. The patient shows bilateral sporadic aniridia and genital anomalies at 2 months of age. A heterozygous 14.5 Mb interstitial deletion of 11p14.3p12 region was detected by array comparative genomic hybridization. At 2 years and 10 months of age, Wilms tumor is found through regularly abdominal ultrasonography and treated by chemotherapy, radiation therapy and surgery.
Aniridia ; Comparative Genomic Hybridization ; Cytogenetics ; Drug Therapy ; Gene Deletion ; Humans ; Intellectual Disability ; Ultrasonography ; Urogenital Abnormalities ; WAGR Syndrome* ; Wilms Tumor*

PURPOSE: Neurofibromatosis type 2 (NF2) is characterized by multiple tumors, including vestibular schwannoma (VS) and others affecting cranial and peripheral nerves. NF2 is caused by mutation of the NF2 gene. The mutation spectrum of NF2 has not been characterized in Korean patients. In the current study, the clinical and genetic characteristics of Korean NF2 patients were analyzed. MATERIALS AND METHODS: Twenty-five unrelated Korean families were enrolled according to the Manchester criteria. Genetic analysis was performed by direct sequencing and multiplex ligation-dependent probe amplification methods using genomic DNA from peripheral lymphocytes or tumor tissues. RESULTS: All patients had bilateral/unilateral VS and/or other cranial and peripheral nerve tumors. Two patients were familial cases and the other 24 patients were sporadic. Germline NF2 mutations were detected in peripheral lymphocytes from both familial cases, but only in 26.1% of the 23 sporadic families. Somatic mutations were also found in tumor tissues from two of the sporadic families. These somatic mutations were not found in peripheral lymphocytes. A total of 10 different mutations including 2 novel mutations were found in 40.0% of studied families. Five mutations (50.0%) were located in exon 6 of NF2, the FERM domain coding region. CONCLUSION: Family history was an important factor in identifying germline NF2 mutations. Further study is required to investigate whether exon 6 is a mutation hotspot in Korean NF2 patients and its correlation to phenotypic severity.
Clinical Coding ; DNA ; Exons ; Genes, Neurofibromatosis 2 ; Humans ; Korea ; Lymphocytes ; Multiplex Polymerase Chain Reaction ; Neurofibromatoses* ; Neurofibromatosis 2* ; Neuroma, Acoustic ; Peripheral Nerves ; Peripheral Nervous System Neoplasms

Hamartomatous polyps in the stomach have been described as gastric lesions of familial polyposis coli or not associated with polyposis coli. However, submucosal tumor-like lesion of the gastric hamartoma is very rare. We have experienced an unusual hamartoma in the stomach in a 69-year-old man. He was hospitalized with epigastric discomfort. Endoscopy revealed a submucosal mass at the greater curvature of the high body of the stomach. The resected mass measured 3 X 2 X 1.5 cm and was characterized by cystic dilation of glandular structures. The glandular structures consisted of various types of lining cells, including surface foveolar cell types, pyloric cell types and parietal-like cells, and irregularly arranged smooth muscle bundles and collagen fibers were noted. We report this unusual gastric hamartoma presenting as a submucosal tumor with a review of literatures.
Adenomatous Polyposis Coli ; Aged ; Collagen ; Endoscopy ; Hamartoma* ; Humans ; Muscle, Smooth ; Polyps ; Stomach

Background: This study evaluated the relationship between guideline adherence for heart failure (HF) with reduced ejection fraction (HFrEF) at discharge and relevant clinical outcomes in patients with acute HF with preserved ejection fraction (HFpEF) with or without atrial fibrillation (AF). Methods: We analyzed Korean Acute Heart Failure Registry data for 707 patients with HFpEF with documented AF and 687 without AF. Guideline adherence was defined as good or poor according to the prescription of angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, β-blockers, and mineralocorticoid receptor antagonists. Anticoagulation adherence was also incorporated for the AF group. Results: Among patients with normal sinus rhythm, those with poor guideline adherence had a reduced prevalence of comorbidities and favorable clinical characteristics when compared with those with good guideline adherence. Using inverse probability of treatment weighting (IPTW) to address the bias of nonrandom treatment assignment, good adherence was associated with a poor 60-day composite endpoint in the multivariable Cox model (weighted hazard ratio [wHR], 1.74; 95% confidence interval [CI], 1.01–3.00; P = 0.045). For patients with AF, baseline clinical characteristics were similar according to the degree of adherence. The IPTW-adjusted analysis indicated that good adherence was significantly associated with the 60-day composite endpoint (wHR, 0.47; 95% CI, 0.27–0.79; P = 0.005). In the analysis excluding warfarin, good adherence was associated with 60-day rehospitalization (wHR, 0.60; 95% CI, 0.37–0.98; P = 0.040), 1-year re-hospitalization (wHR, 0.67; 95% CI, 0.48–0.93; P = 0.018), and the composite endpoint (wHR, 0.77; 95% CI, 0.59–0.99; P = 0.041). Conclusion Our findings indicate that good adherence to guidelines for HFrEF is associated with a better 60-day composite endpoint in patients with HFpEF with AF.

Background: This study evaluated the relationship between guideline adherence for heart failure (HF) with reduced ejection fraction (HFrEF) at discharge and relevant clinical outcomes in patients with acute HF with preserved ejection fraction (HFpEF) with or without atrial fibrillation (AF). Methods: We analyzed Korean Acute Heart Failure Registry data for 707 patients with HFpEF with documented AF and 687 without AF. Guideline adherence was defined as good or poor according to the prescription of angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, β-blockers, and mineralocorticoid receptor antagonists. Anticoagulation adherence was also incorporated for the AF group. Results: Among patients with normal sinus rhythm, those with poor guideline adherence had a reduced prevalence of comorbidities and favorable clinical characteristics when compared with those with good guideline adherence. Using inverse probability of treatment weighting (IPTW) to address the bias of nonrandom treatment assignment, good adherence was associated with a poor 60-day composite endpoint in the multivariable Cox model (weighted hazard ratio [wHR], 1.74; 95% confidence interval [CI], 1.01–3.00; P = 0.045). For patients with AF, baseline clinical characteristics were similar according to the degree of adherence. The IPTW-adjusted analysis indicated that good adherence was significantly associated with the 60-day composite endpoint (wHR, 0.47; 95% CI, 0.27–0.79; P = 0.005). In the analysis excluding warfarin, good adherence was associated with 60-day rehospitalization (wHR, 0.60; 95% CI, 0.37–0.98; P = 0.040), 1-year re-hospitalization (wHR, 0.67; 95% CI, 0.48–0.93; P = 0.018), and the composite endpoint (wHR, 0.77; 95% CI, 0.59–0.99; P = 0.041). Conclusion Our findings indicate that good adherence to guidelines for HFrEF is associated with a better 60-day composite endpoint in patients with HFpEF with AF.