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Purpose@#Prognostic Index for Natural Killer Lymphoma (PINK) is the most widely accepted prognostic model for patients withextranodal natural killer/T-cell lymphoma (ENKTL) treated with non-anthracycline–based therapy. We aimed to evaluate the prognostic implications of serum β-2 microglobulin (β2M) in the context of PINK and proposed a new prognostic model. @*Materials and Methods@#A total of 138 patients who were newly diagnosed with ENKTL and treated with non-anthracycline-based chemotherapy were identified. The cut-off value of high serum β2M was calculated by maximal-chi square methods (4.1 mg/L). A new prognostic model incorporating serum β2M into PINK was proposed and validated in an independent validation cohort (n=88). @*Results@#The patients’ median age was 53.5 years (range, 19 to 80 years). Patients with high serum β2M levels had significantly worse overall survival (OS) and progression-free survival (PFS). In multivariate analysis, high serum β2M was an independent adverse prognostic factor for OS. A new PINK-B (Prognostic Index for Natural Killer Lymphoma-serum β-2 microglobulin) model stratifiedpatients into three groups with distinct OS and PFS in the training cohort (3-year OS, 84.1% [95% confidence interval, 75.1 to 94.2], 46.8% [36.1 to 60.8] and 17.6% [6.3 to 49.2] for the low-, intermediate, and high-risk groups, respectively; 3-year PFS, 70.6% [59.4 to 83.8], 35.9% [25.9 to 49.8], and 7.35% [1.1 to 46.7] for the low-, intermediate-, and high-risk groups, respectively). The PINK-B model was further validated in an independent cohort. @*Conclusion@#Serum β2M is an independent prognostic factor for ENKTL patients. The new serum β2M-based prognostic model may be useful for identifying ultra-high-risk patients, and it can easily be adopted into daily clinical practice.
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Background@#Guselkumab is a monoclonal antibody that selectively blocks the p19 subunit of interleukin-23. It has shown good efficacy and safety profile in several clinical trials of plaque psoriasis. However, studies on the efficacy of guselkumab in patients treated with other biologics are lacking. @*Objective@#We aimed to investigate the efficacy and safety profile of guselkumab in patients with moderate-to-severe plaque psoriasis. We also compared the efficacy of guselkumab between biologic-naïve (Bio-Naïve) and biologicexperienced (Bio-Ex) patients. @*Methods@#This multicenter, retrospective study included 72 patients treated with guselkumab. The patients’ clinical characteristics and psoriasis area and severity index (PASI) scores were recorded at each visit. The PASI90 and PASI100 responses and mean PASI scores were compared between the Bio-Naïve and Bio-Ex groups. @*Results@#Fifty-five Bio-Naïve patients and 17 Bio-Ex patients were included in the study. At week 20, there were no significant differences in the PASI90 (64.2% vs. 53.8%) and PASI100 (28.3% vs. 15.4%) responses between the groups. However, at weeks 36 and 44, the PASI90 response (week 36: 89.2% vs. 36.4% and week 44: 97.8% vs. 63.6%) and the PASI100 response (week 36: 64.9% vs. 18.2% and week 44: 68.9% vs. 27.3%) were significantly higher in the Bio-Naïve group (p＜0.05). There were no differences in PASI90 and PASI100 responses between the groups in terms of other clinical characteristics and comorbidities at week 20. @*Conclusion@#The efficacy of guselkumab remained consistent among patients in whom other biologics had failed. However, the efficacy was slightly lower in the Bio-Ex group than in the Bio-Naïve group.
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The areola is a rare location for squamous cell carcinoma (SCC) because sunlight exposure, the main risk factor for SCC, is unusual on it. Acantholytic SCC (ASCC) is a rare histologic variant of SCC, characterized by pseudoglandular appearance with acantholytic tumor cells. A 59-year-old male presented a painful erythematous papule on his right areola. He had a history of psoralen ultraviolet A phototherapy for psoriasis in his 20s. Biopsy revealed an epithelial tumor and pseudoglandular structures with acantholytic tumor cells. In immunohistochemistry, cytokeratin 5/6, epithelial membrane antigen, and p63 were positive, while cytokeratin 7, carcinoembryonic antigen, S-100, and estrogen and progesterone receptors were negative. Periodic acid-Schiff stain was negative. Ki-67 labeling index was 79.7%. The final diagnosis was ASCC of the areola. After wide local excision, recurrence have not been reported. Here, we report a case of ASCC on the areola, focusing on its rare histologic variant and uncommon location.
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Background@#The nuclear factor erythroid 2-related factor 2/Kelch-like ECH-associated protein 1 (Nrf2/Keap1) signaling pathway is involved in the regulation of cellular responses to oxidative stress. Nrf2 acts as a cell protector from inflammation, cellular damage, and tumorigenesis, whereas Keap1 is a negative regulator of Nrf2. Dysregulation of the Nrf2/ Keap1 pathway results in tumorigenesis and the active metabolism of tumor cells, leading to high resistance to radiotherapy. This study aimed to evaluate the predictive role of Nrf2 and Keap1 in the radiosensitivity and prognosis of locally advanced rectal cancer (LARC). @*Methods@#In total, 90 patients with LARC underwent surgery after preoperative chemoradiotherapy (CRT). Endoscopic biopsies from the tumors were obtained before radiation, and the Nrf2 and Keap1 expressions were assessed by immunohistochemistry. The response to therapy was evaluated after surgery following CRT according to the pathologic tumor regression grade. The disease-free survival (DFS) and overall survival rates were also documented. The association between the Nrf2 and Keap1 immunoreactivity and the clinicopathological parameters was analyzed. @*Results@#The overexpression of the nuclear Nrf2 before CRT showed a significant correlation with better DFS. The cytoplasmic Nrf2 expression was associated with more residual tumors after radiotherapy and a more unfavorable DFS, indicating lower radiosensitivity. @*Conclusion@#CRT is an important issue in LARC and is a major aspect of treatment. Thus, the Nrf2/Keap1 expression may be a potential predictor of preoperative therapeutic resistance.The Nrf2-Keap1 modulators that interact with each other may also be effectively applicable to CRT effect in LARC.
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Infants are highly vulnerable, requiring the protection of caregivers for their survival. Unfortunately, infant abandonment continues to be committed regularly, posing a serious challenge to the dignity of human life. To identify the motivation and background information of this crime, we analyzed 20 cases of infant abandonment to determine their critical characteristics. We aimed to gather data to assist in crime prevention and ultimately to address the need for new policies to prevent this crime. The results of our analysis are as follows: The mothers who abandoned their infants were in their teens and twenties in 15 cases (75%). The relationship between the biological parents was that of longterm partners in 12 cases (60%). The place of childbirth was the perpetrator’s home in 16 cases (80%). The place of abandonment wasoutdoors in 9 cases (5 cases alive, 4 cases dead) and indoors in 10 cases (5 cases alive, 5 cases dead). Basic life support for the infant was provided in 4 cases. The most common excuse for abandonment was to conceal childbirth in 12 cases, followed by economic hardship in 8 cases. Executions were suspended in 19 cases, and the reasons for the sentences often seemed inadequate, even in light of the provisions of the criminal code. These results indicate that our society has the need to develop the appropriate acts or policies to stop the crime of infant abandonment.
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Syringocystadenoma papilliferum (SCAP) and apocrine hidrocystoma (AH) are benign apocrine neoplasms that usually occur separately. SCAP arises predominantly in head and neck, while AH typically develop in periorbital area. We report a case of a 68-year-old male with an asymptomatic erythematous papulonodule that occurred on his back 3 years ago. Histologic examination showed cystic invagination extending from the epidermis into the dermis with some papillary projections. The invaginated portion was lined by epithelial bilayer composed of cuboidal and columnar cells, and decapitation secretion was observed in the inner epithelial layer. In the deep dermis, multiple cystic spaces with variable sizes were observed, and these cysts also presented double layers of the epithelium and decapitation secretion.According to such histologic features, the coexistence of SCAP and AH within a single lesion was demonstrated. The patient was recommended to completely remove the remaining lesion after punch biopsy, but he refused further surgical management. Herein, we report an unusual case of complex apocrine tumor with a rare composition in an atypical site.
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no abstract available.
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Background@#Polo-like kinase 4 (PLK4) is a serine/threonine protein kinase located in the centriole of the chromosome during the cell cycle. PLK4 overexpression has been described in a variety of many common human epithelial tumors. Conversely, PLK4 acts as a haploinsufficient tumor suppressor in some situations, highlighting the importance of strict regulation of PLK4 expression, activity, and function. Meanwhile, the importance of chemoradiation resistance in rectal cancer is being emphasized more than ever. We aimed to analyze PLK4 expression and the tumor regression grade (TRG) in patients with rectal cancer, treated with chemoradiotherapy (CRT). @*Methods@#A retrospective study was conducted on 102 patients with rectal cancer who received preoperative CRT. Immunohistochemistry for PLK4 in paraffin-embedded tissue was performed from the biopsy and surgical specimens. @*Results@#We found significant association between high expression of PLK4 and poor response to neoadjuvant CRT (according to both Mandard and The Korean Society of Pathologists TRG systems) in the pre-CRT specimens. Other clinicopathologic parameters did not reveal any correlation with PLK4 expression. @*Conclusions@#This study revealed an association between high expression of PLK4 in the pre-CRT specimens and TRG. Our results indicated that PLK4 could potentially be a new predictor for CRT effect in patients with rectal cancer.
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Background@#Herpes zoster (HZ) is a neurocutaneous disease caused by reactivation of the varicella zoster virus after primary infection. In Korea, it is recommended that all adults aged ＞60 be vaccinated against HZ. However, breakthrough infection with HZ can occur infrequently in vaccinated individuals. @*Objective@#This study aimed to investigate the effects of the HZ vaccine on the clinical manifestations and courses of HZ in immunocompetent elderly individuals aged 60 years and older. @*Methods@#Among 848 immunocompetent HZ patients, 52 were vaccinated and 796 were unvaccinated. From this cohort, we chose 156 unvaccinated patients through a 1:3 propensity score matching analysis using three parameters;age, sex, and involved dermatome. We subsequently compared the clinical manifestations and courses between the vaccinated (n=52) and unvaccinated (n=156) groups. @*Results@#The vaccinated group had a lower peak numeric rating scale pain score and a lower rate of consultation with the pain control clinic than the opposite. Additionally, mild skin lesions without vesicles were more frequent in the vaccinated group than in the opposite. The vaccinated group had a shorter duration of total follow-up, shorter antiviral treatment period, lower admission rate, and shorter hospital stay than the opposite. Although postherpetic neuralgia occurred more frequently in the unvaccinated group than in the unvaccinated group, the incidences of other HZ-related complications showed no significant difference between the two groups. @*Conclusion@#Despite the possibility of breakthrough infection, the HZ vaccine should be recommended in the elderly as it can weaken the clinical features and course of the disease.
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Background@#Fever and skin eruption are common manifestations in patients; however, assessing them can be challenging because of the wide range of differential diagnosis. @*Objective@#We aimed to obtain tips that could assist in differential diagnoses in patients with fever and skin eruption through the analysis of clinical features. @*Methods@#Medical records of hospitalized patients who had fever and skin eruption were examined from January 2010 to December 2019. @*Results@#The patients of the adverse drug reaction group were older (p=0.024), and they had underlying diseases (p＜0.001), whole body skin eruption (p=0.018), elevated alanine aminotransferase (p=0.027). The autoimmune disease group was associated with longer total fever and skin eruption duration (p=0.049, 0.037), leukocytosis (p=0.015), and neutrophilia (p=0.006). Infectious erythema group was associated with targetoid lesions (p=0.003). @*Conclusion@#Clinicians should examine patients with fever and skin eruption, considering that there may be various causes. This can be assisted by delicate skin exam and laboratory tests.
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Background/Aims@#The incidence of multiple myeloma (MM) in Korea is rapidly increasing. The diagnostic criteria of MM have been updated and novel therapeutic agents are available. This study explored the features of MM patients registered at Asan Medical Center (AMC) and the outcomes over the past 15 years. @*Methods@#Data were obtained from the AMC MM registry, which has been collecting the data of MM patients prospectively. The 774 MM patients included in our analysis were diagnosed from 2003, when thalidomide became available as a novel therapeutic agent, until April 2019. The 2-year survival rate of these patients was assessed. Patients were divided into two groups based on whether they were older or younger than 65 years, which is the cutoff age for the indication of autologous stem cell transplantation. Patients were also grouped according to the year of diagnosis: up to 2006, when bortezomib became available, and up to 2010, when the cost of lenalidomide was reimbursed. @*Results@#Patients < 65 years of age had better prognostic features, including a better performance, less advanced disease stage, and fewer abnormalities in their fluorescent in-situ hybridization (FISH) analysis results. A comparison of our Korean patients with patients registered in the Myeloma Related Disorder Registry data of Australia and New Zealand, showed ethnic discrepancies. The median overall survival of all patients was 3.7 years, with a 5-year survival rate of 41.8% and a 10-year survival rate of 23.4%. Survival progressively improved in patients diagnosed later. Age, performance status, renal function, C-reactive protein level, lactate dehydrogenase level, and cytogenetic findings were identified as significant prognostic factors. @*Conclusions@#This real-world survey revealed the clinical features and survival rates of patients at a tertiary Korean Hospital who were diagnosed with MM at the beginning of 21st century.
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Crimes committed by the mentally ill may give rise to critical conflicts on whether the criminals should receive punishment or treatment. Therefore, our criminal code has a legal provision for mental handicap and criminal responsibility, which can reduce or exempt the criminal penalty. This process requires a psychiatric evaluation of the defendants as well as normative decisions by judges. The psychiatric evaluation and diagnosis precede the court judgement, and are used by the court to judge the mental handicap of the defendant at the time of the crime. However, judgments on the criminal responsibility of a defendant with mental disorder are very complex and challenging because of time gaps and difficulties faced in the evaluation of human mental ability. We analyzed court rulings in 19 cases where the defendants claimed to be mentally handicapped. The analysis results showed that the mental handicap was recognized in nine cases, rejected in four cases, and not mentioned by the court in six cases. Further, psychiatric evaluation was not performed in three cases. Apart from the result of the evaluation, the judges seem to consider other factors for sentencing, like recidivism risk, premeditation of crimes, and their brutality. These results suggest that the rationale and procedure of court rulings involving mentally handicapped persons may be debatable. We argue that psychiatric evaluations should be made indispensable in judicial procedures for cases involving mentally handicapped persons, and judges should clearly indicate their decision on mental handicap or criminal responsibility in the sentencing.
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Endovascular repair of popliteal artery aneurysms (PAA) using a stent graft is suitable for patients with favorable anatomy. In the domestic situation where Gore Medical withdrew, we report two cases of unusual complications of pseudoaneurysm after endovascular repair of PAA. A 44-year-old male with a history of bypass surgery for a PAA presented with recurrent vein graft pseudoaneurysm. Endovascular treatment using a domestic stent graft was performed. However, pseudoaneurysm developed due to the graft fabric tear 1 month later, requiring surgical removal. In another case, an 84-year-old female presented with acute limb ischemia related to PAA. Endovascular aneurysm repair with the same domestic stent graft was performed. However, stent graft failure occurred 2 years later and the patient underwent open surgical repair. There was a graft fabric disintegration. When proper endovascular device is not available, open surgical treatment is the best option for treating PAA.
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In the case of hemodynamically stable broad complex tachycardia, it is essential to evaluate the causes and to diagnose correctly in the selection of appropriate management and drugs. We report two neonates diagnosed with idiopathic ventricular tachycardia, which is extraordinarily rare. One presented with idiopathic fascicular ventricular tachycardia (right bundle branch block pattern with a superior axis), and the other presented with right ventricular outflow tract ventricular tachycardia (left bundle branch block pattern with an inferior axis). These two forms are representative of benign ventricular tachycardia. No features of cardiovascular shock were observed. While the recommended initial drug treatments are different, the conditions were well controlled by propranolol without the development of any adverse events. There was no recurrence of arrhythmia for several months in the outpatient clinic.
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We present the case of a healthy 28-day-old female full-term neonate who was admitted to the neonatal intensive care unit for severe metabolic acidosis, hypoglycemia, and an initial sinus rhythm. The first diagnostic hypothesis was hypovolemic shock, and fluid resuscitation was started immediately. During fluid therapy, cardiovascular collapse occurred with supraventricular tachycardia. The latter was successfully treated with adenosine and beta-blockers. After 8 days, electrocardiography showed ventricular pre-excitation, and Wolff-Parkinson-White syndrome was diagnosed. A novel variant of the MYL2 gene that is related to hypertrophic cardiomyopathy and conduction defect was found after discharge. Cardiogenic shock should be considered, despite being a rare cause of shock in neonates.
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Purpose@#Meconium obstruction of prematurity (MOP) predisposes premature infants to intestinal perforation and prolonged hospitalization if not diagnosed and treated promptly. A standard contrast enema is less effective to treat infants with distal ileal obstructions because the contrast may not reach the obstructed areas. In an effort to avoid risky surgery, we administered oral contrast media to seven clinically diagnosed patients with MOP whose obstructions were not relieved via conventional sonography-guided contrast enema. We retrospectively evaluated whether oral nonionic water-soluble contrast media relieves MOP. @*Methods@#Seven of 67 premature infants with MOP were administered oral contrast media from June 2015 to January 2019. Patients were followed-up radiographically for bowel distention and evacuation of contrast media after oral administration. We recorded radiographic improvements, meconium evacuation, time to first feeding after oral contrast media administration, maternal history, and neonatal clinical factors. @*Results@#We evaluated five male and two female infants. The median gestational ages and body weights at birth were 27+5 weeks and 890 g, respectively. Radiography in five infants revealed multiple distended intestinal loops without air-fluid interfaces. Two infants had gasless abdomens, in which only stomach gas was visible. Oral contrast media (median, 2.5 mL) were administered at a median age of 7 days; five infants (5/7, 71.4%) responded to this treatment. The remaining two infants, who had ileal stenosis and hypoganglionosis, were surgically managed. Five infants (5/7, 71.4%) had maternal risk factors, and two (28.6%) were small for gestational age. @*Conclusion@#Nonionic oral water-soluble contrast medium can serve as a valuable adjunct treatment in premature infants with meconium obstruction.
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Munchausen syndrome by proxy (MSBP) is a rare form of mental disorder and is known as a particular type of child abuse. MSBP has been described since 1977 as a severe form of abuse with illness falsification or the intentional harming by guardians, mostly mothers. The perpetrator of MSBP may inflict damage to the child directly or indirectly through medical procedures. The perpetrator’s alleged motive is to satisfy her psychological needs, and she has a history of mental illness, mostly, factitious disorder, personality disorder, and somatic disorder. The pathology is not well known; as such, it is difficult for medical personnel to detect it early. In addition, it is hard to be handled effectively by the police and child welfare agencies because of the scarcity of evidence. Therefore, the authors attempt to examine the essential information from early detection and child abuse prevention by analyzing its clinical characteristics and the perpetrator’s characteristics, including alerting signs of MSBP. For this purpose, we focus on the role of nursing staff to detect this unusual cause of child abuse.
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Among the various crimes, the ones committed by the mentally ill provoke more serious public concern, which is probably caused by such characteristics such as unpredictability, brutality, unknown motives, or random targeting of victims. The purpose of this study was to analyze the characteristics of crimes committed by the mentally ill, to investigate the type of mental disorders and assaulting weapons, and to examine the correlation between the type of mental disorders and their criminal behavior patterns. Through the combination of the forensic and psychological approach, the authors examined the psychological and behavioral characteristics of mentally ill criminals to identify the criminal and behavioral characteristics of crimes committed by them. The cases for this analysis were collected through the search for precedents at National Law Information Center where 19 cases were selected. In conclusion, the diagnoses of mental disorders were schizophrenia, pedophilia, intellectual disability, personality disorder, and depressive disorder, in descending order. Among them, schizophrenia accounted for 6 cases (31.6%), where especially the paranoid type (3 cases) committed the premeditated crimes. Mental handicap was recognized in 9 cases and was rejected in 4 cases. However, in 6 cases, it was not mentioned by court. Further, the judgment of being mental handicapped was made without reference of mental appraisal in 3 out of 19 cases, and so its credibility might be argued. The number of defendants who had past criminal histories was 7 (33.3%) out of 21 defendants, which may illustrate the rate of recidivism of mental disorder criminals.
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BACKGROUND@#Mounting evidence implicates an association between ambient air pollution and impaired reproductive potential of human. Our study aimed to assess the association between air pollution and ovarian reserve in young, infertile women.@*METHODS@#Our study included 2276 Korean women who attended a single fertility center in 2016-2018. Women's exposure to air pollution was assessed using concentrations of particulate matter (PM@*RESULTS@#The mean age was 36.6 ± 4.2 years and AMH level was 3.3 ± 3.1 ng/mL in the study population. Average AMH ratio was 0.8 ± 0.7 and low AMH was observed in 10.3% of women (n=235). The average concentration of six air pollutants was not different between the normal ovarian reserve and low AMH groups for all averaging periods. In multivariable models, an interquartile range (IQR)-increase in 1 month-average PM@*CONCLUSIONS@#In a cohort of infertile Korean women, there was a suggestive evidence of the negative association between ambient PM concentration and ovarian reserve, highlighting the potential adverse impact of air pollution on women's fertility.
Subject(s)
Adult , Female , Humans , Air Pollutants/adverse effects , Air Pollution/adverse effects , Environmental Exposure/adverse effects , Infertility, Female/etiology , Ovarian Reserve/physiology , Republic of KoreaABSTRACT
Hand-foot-mouth disease (HFMD) is a viral infection that occurs commonly in children. It is characterized by vesicles with surrounding erythema on the extremities and mouth. Most common pathogens are Coxsackievirus A16 and Enterovirus 71. HFMD caused by Coxsackievirus A6 is uncommon and accompanied by more extensive and atypical eruptions. A 42-year-old man presented with erythematous papules and vesicles on the right hand which occurred 7 days prior to presentation. The rash spread extensively with high fever, chills, headache, and myalgia. He also had whitish ulcer-like lesions on the oral mucosa with swallowing difficulty. One day after hospitalization, he developed arthralgia on his shoulders, pelvis, knees, and fingers. The biopsy specimen showed an intraepidermal blister with reticular degeneration, epidermal necrosis, and neutrophilic infiltration. There were no inclusion cells or giant cells. The serum antibody titer of Coxsackievirus A6 showed a significant increase, at 64 times. He was diagnosed with HFMD caused by Coxsackievirus A6.