Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common monogenic disorder of the cerebral small blood vessels. It is caused by mutations in the NOTCH3 gene on chromosome 19, and more than 280 distinct pathogenic mutations have been reported to date. CADASIL was once considered a very rare disease with an estimated prevalence of 1.3–4.1 per 100,000 adults. However, recent large-scale genomic studies have revealed a high prevalence of pathogenic NOTCH3 variants among the general population, with the highest risk being among Asians. The disease severity and age at onset vary significantly even among individuals who carry the same NOTCH3 mutations. It is still unclear whether a significant genotype–phenotype correlation is present in CADASIL. The accumulation of granular osmiophilic material in the vasculature is a characteristic feature of CADASIL. However, the exact pathogenesis of CADASIL remains largely unclear despite various laboratory and clinical observations being made. Major hypotheses proposed so far have included aberrant NOTCH3 signaling, toxic aggregation, and abnormal matrisomes. Several characteristic features have been observed in the brain magnetic resonance images of patients with CADASIL, including subcortical lacunar lesions and white matter hyperintensities in the anterior temporal lobe or external capsule, which were useful in differentiating CADASIL from sporadic stroke in patients. The number of lacunes and the degree of brain atrophy were useful in predicting the clinical outcomes of patients with CADASIL. Several promising blood biomarkers have also recently been discovered for CADASIL, which require further research for validation.

Although renal angiomyolipoma (AML) is a benign tumor, treatment may be necessary occasionally because it can cause potentially life-threatening retroperitoneal hemorrhage. Transarterial embolization (TAE) is a safe and effective treatment option to prevent the hemorrhagic rupture of AMLs and relieve the symptoms caused by enlarged lesions or active bleeding. However, there is no clear consensus regarding the indications for prophylactic TAE in patients with sporadic renal AMLs. In urgent TAE for bleeding AMLs, there is a likelihood of incomplete embolization when the focus is on stabilizing the clinical symptoms. This pictorial essay discusses the patient selection and technical considerations to achieve optimal therapeutic effects as well as the follow-up findings after TAE.

BACKGROUND: Individual particulate respirator use may offer protection against exposure to particulate matter < 2.5 µm in diameter (PM(2.5)). Among elderly Korean women, we explored individual particulate respirator use and cardiopulmonary function.METHODS: Recruited in Seoul, Korea, 21 elderly, non-smoking women wore particulate respirators for six consecutive days (exlcuding time spent eating, sleeping, and bathing). We measured resting blood pressure before, during, and after respirator use and recorded systolic and diastolic blood pressure, mean arterial blood pressure, pulse pressure, and lung function. We also measured 12-hour ambulatory blood pressure at the end of the 6-day long experiment and control periods. Additionally, we examined physiological stress (heart rate variability and urinary 8-hydroxy-2′-deoxyguanosine) while wearing the particulate respirators. Person- and exposure-level covariates were also considered in the model.RESULTS: After the 6-day period of respirator use, resting blood pressure was reduced by 5.3 mmHg for systolic blood pressure (P = 0.013), 2.9 mmHg for mean arterial blood pressure (P = 0.079), and 3.6 mmHg for pulse pressure (P = 0.024). However, particulate respirator use was associated with changes in physiological stress markers. A parasympathetic activity marker (high frequency) significantly decreased by 24.0% (P = 0.029), whereas a sympathetic activity marker (ratio of low-to-high frequency) increased by 50.3% (P = 0.045). An oxidative stress marker, 8-hydroxy-2′-deoxyguanosine, increased by 3.4 ng/mg creatinine (P = 0.021) during the experimental period compared with that during the control period. Lung function indices indicated that wearing particulate respirators was protective; however, statistical significance was not confirmed.CONCLUSION: Individual particulate respirator use may prevent PM(2.5)-induced blood-pressure elevation among elderly Korean women. However, the effects of particulate respirator use, including physiological stress marker elevation, should also be considered.TRIAL REGISTRATION: Clinical Research Information Service Identifier: KCT0003526

Background: Nurses play a central role in the process of blood transfusion because they have the closest interactions with patients. Providing nurses with the appropriate knowledge and competency through education can help ensure transfusion safety. This study investigated the effectiveness of the first nationwide transfusion-related education for nurses by analyzing questionnaires for a self-assessment of competency on transfusion-related knowledge before and after the education and general evaluation for the educational program. Methods: The education program was composed of four lectures and was conducted in seven regions in South Korea. One hundred and ninety-two nurses participated, and the questionnaires for 170 nurses were analyzed. Results: The participants consisted of 90 nurses (53.0%) from tertiary hospitals, 23 (13.5%) from general hospitals, and 57 (33.5%) from other hospitals. The majority of the participants (103/170, 60.6%) were from hospitals with ≥500 beds, and 69.4% had a work period of ≥10 years. The scores for pre-/post-education self-assessment of competency were as follows: blood components, 3.03/3.73; pretransfusion testing, 2.86/3.64; management of transfusion, 3.18/3.84; and transfusion reactions, 3.11/3.78. In all categories, there was a significant increase in the score after the education program. The majority of participants (99.4%) provided a positive response regarding the necessity of a transfusion-related education program. Conclusion South Korea’s first attempt at transfusion-related education for nurses showed a strong positive effect by improving participants’ transfusion-related competency. Considering the important role of nurses in blood transfusion, the educational program should continue and be expanded in the future.

Background: Nurses play a central role in the process of blood transfusion because they have the closest interactions with patients. Providing nurses with the appropriate knowledge and competency through education can help ensure transfusion safety. This study investigated the effectiveness of the first nationwide transfusion-related education for nurses by analyzing questionnaires for a self-assessment of competency on transfusion-related knowledge before and after the education and general evaluation for the educational program. Methods: The education program was composed of four lectures and was conducted in seven regions in South Korea. One hundred and ninety-two nurses participated, and the questionnaires for 170 nurses were analyzed. Results: The participants consisted of 90 nurses (53.0%) from tertiary hospitals, 23 (13.5%) from general hospitals, and 57 (33.5%) from other hospitals. The majority of the participants (103/170, 60.6%) were from hospitals with ≥500 beds, and 69.4% had a work period of ≥10 years. The scores for pre-/post-education self-assessment of competency were as follows: blood components, 3.03/3.73; pretransfusion testing, 2.86/3.64; management of transfusion, 3.18/3.84; and transfusion reactions, 3.11/3.78. In all categories, there was a significant increase in the score after the education program. The majority of participants (99.4%) provided a positive response regarding the necessity of a transfusion-related education program. Conclusion South Korea’s first attempt at transfusion-related education for nurses showed a strong positive effect by improving participants’ transfusion-related competency. Considering the important role of nurses in blood transfusion, the educational program should continue and be expanded in the future.

BACKGROUND: Individual particulate respirator use may offer protection against exposure to particulate matter < 2.5 µm in diameter (PM(2.5)). Among elderly Korean women, we explored individual particulate respirator use and cardiopulmonary function. METHODS: Recruited in Seoul, Korea, 21 elderly, non-smoking women wore particulate respirators for six consecutive days (exlcuding time spent eating, sleeping, and bathing). We measured resting blood pressure before, during, and after respirator use and recorded systolic and diastolic blood pressure, mean arterial blood pressure, pulse pressure, and lung function. We also measured 12-hour ambulatory blood pressure at the end of the 6-day long experiment and control periods. Additionally, we examined physiological stress (heart rate variability and urinary 8-hydroxy-2′-deoxyguanosine) while wearing the particulate respirators. Person- and exposure-level covariates were also considered in the model. RESULTS: After the 6-day period of respirator use, resting blood pressure was reduced by 5.3 mmHg for systolic blood pressure (P = 0.013), 2.9 mmHg for mean arterial blood pressure (P = 0.079), and 3.6 mmHg for pulse pressure (P = 0.024). However, particulate respirator use was associated with changes in physiological stress markers. A parasympathetic activity marker (high frequency) significantly decreased by 24.0% (P = 0.029), whereas a sympathetic activity marker (ratio of low-to-high frequency) increased by 50.3% (P = 0.045). An oxidative stress marker, 8-hydroxy-2′-deoxyguanosine, increased by 3.4 ng/mg creatinine (P = 0.021) during the experimental period compared with that during the control period. Lung function indices indicated that wearing particulate respirators was protective; however, statistical significance was not confirmed. CONCLUSION Individual particulate respirator use may prevent PM(2.5)-induced blood-pressure elevation among elderly Korean women. However, the effects of particulate respirator use, including physiological stress marker elevation, should also be considered.TRIAL REGISTRATION: Clinical Research Information Service Identifier: KCT0003526

PURPOSE: The purpose of this study was to develop Korean versions of the National Comprehensive Cancer Network/Functional Assessment of Cancer Therapy (NCCN-FACT) Ovarian Symptom Index-18 (NFOSI-18) and FACT/Gynecologic Oncology Group (FACT-GOG) Neurotoxicity 4-item (NTX-4), evaluating their reliability and reproducibility. MATERIALS AND METHODS: In converting NFOSI-18 and NTX-4, the following steps were performed: forward translation, backward translation, expert review, pretest of preliminary format, and finalization of Korean versions (K-NFOSI-18 and K-NTX-4). Patients were enrolled from six institutions where each had completed chemotherapy for ovarian, tubal, or peritoneal cancer at least 1 month earlier. In addition to demographics obtained by questionnaire, all subjects were assessed via K-NFOSI-18, K-NTX-4, and a Korean version of the EuroQoL-5 Dimension. Internal structural validity and reliability were evaluated using item internal consistency, item discriminant validity, and Cronbach's α. To evaluate test-retest reliability, K-NFOSI-18 and K-NTX-4 were readministered after 7-21 days, and intraclass correlation coefficients (ICCs) were calculated. RESULTS: Of the 250 women enrolled during the 3-month recruitment period, 13 withdrew or did not respond, leaving 237 (94.8%) for the analyses. Mean patient age was 54.3±10.8 years. Re-testing was performed in 190 patients (80.2%). The total K-NFOSI-18 and K-NTX-4 scores were 49 (range, 20 to 72) and 9 (range, 0 to 16), respectively, with high reliability (Cronbach's α=0.84 and 0.89, respectively) and reproducibility (ICC=0.77 and 0.84, respectively) achieved in retesting. CONCLUSION: Both NFOSI-18 and NTX-4 were successfully developed in Korean with minimal modification. Each Korean version showed high internal consistency and reproducibility.
Demography ; Drug Therapy ; Fallopian Tubes* ; Female ; Humans ; Ovarian Neoplasms ; Reproducibility of Results

Urinary tract infection is common in the pediatric population. The most common causative agents are bacteria, among which Escherichia coli is the most frequent uropathogen. Although fungal urinary tract infection is rare in the healthy pediatric population, it is relatively common among hospitalized patients. Fungus may be isolated from the urine of immunocompromised patients or that of patients with indwelling catheters. The most common cause of funguria is Candida albicans. Although more than 50% of Candida isolates belong to non-albicans Candida , the prevalence of non-albicans candiduria is increasing. Herein, we report a case of community-acquired candiduria in a 4-month-old immunocompetent male infant who had bilateral vesicoureteral reflux and was administered antibiotic prophylaxis. He was diagnosed with urinary tract infection caused by Candida lusitaniae and was managed with fluconazole.
Antibiotic Prophylaxis ; Bacteria ; Candida ; Candida albicans ; Catheters, Indwelling ; Escherichia coli ; Fluconazole ; Fungi ; Humans ; Immunocompromised Host ; Infant ; Male ; Prevalence ; Urinary Tract Infections ; Urinary Tract ; Vesico-Ureteral Reflux

PURPOSE: We have summarized the experience of our institution related to what treatment has been performed in patients with pancreatic fistula and their outcome.METHODS: Seventy-eight pancreatico-enteric anastomosis failure (PEAF) patients of 403 pancreaticoduodenectomy (PD) were included for this retrospective study. PEAF was defined by the presence of rich amylase (over 10,000 IU/L) in drainage fluid at postoperative day 5 to 7 and radiographic demonstration of the anastomotic breakdown and associated local fluid collection. The management was analyzed by observation group (O group), intervention (I group) and surgery group (S group).RESULTS: Preoperative clinical status of the PEAF group and non-PEAF group was similar. Bile duct cancer was the highest risk subgroup of the PEAF (P=0.001) and the pancreatic adenocarcinoma showed the least risk for the PEAF (P<0.001). Among the 78 PEAF patients, 50 were managed as a conservative treatment, 15 patients were received radiologic intervention and 13 patients performed rescue surgery. Among these three subgroups, there was no statistical significance in the patient's demographics, clinical status, surgical factors and disease nature. However, mortality was significantly higher in the S group (P<0.001). The mortality cases were developed one and six patients in O and S group, respectively. Surgical procedures in S group were completion total pancreatectomy with or without splenectomy (n=12) and pancreatectomy preserving spleen in four (28.6%). Pancreaticogastrostomy repair and Roux-en-Y pancreaticojejunostomy reconstruction were performed each case, respectively.CONCLUSION: Proper drainage catheter indwelling during the PD or postoperative radiological intervention can effectively manage the PEAF without surgical interventional treatment.
Adenocarcinoma ; Amylases ; Bile Duct Neoplasms ; Catheters ; Demography ; Drainage ; Humans ; Mortality ; Pancreatectomy ; Pancreatic Fistula ; Pancreaticoduodenectomy ; Pancreaticojejunostomy ; Retrospective Studies ; Spleen ; Splenectomy

BACKGROUND: The pain-relief properties of tricyclic antidepressants can be attributed to several actions. Recent observations suggest that adenosine is involved in the antinociceptive effect of amitriptyline. The A3 adenosine receptor (A3AR) is the only adenosine subtype overexpressed in inflammatory and cancer cells. This study was performed to investigate the role of A3AR in the anti-nociceptive effect of amitriptyline. METHODS: Spinal nerve-ligated neuropathic pain was induced by ligating the L5 and L6 spinal nerves of male Sprague-Dawley rats. The neuropathic rats were randomly assigned to one of the following three groups (8 per group): a neuropathic pain with normal saline group, a neuropathic pain with amitriptyline group, and a neuropathic pain with amitriptyline and 3-ethyl-5-benzyl- 2-methyl-4-phenylethynyl-6-phenyl-1,4-(±)-dihydropyridine-3,5-dicarboxylate (MRS) group. Amitriptyline or saline was administered intraperitoneally and 3-ethyl-5-benzyl-2-methyl-4-phenylethynyl-6-phenyl-1,4-(±)-dihydropyridine-3,5-dicarboxylate (MRS-1191), an A3AR antagonist, was injected subcutaneously immediately before amitriptyline administration. The level of extracellular signal-regulated kinase P44/42 (ERK1/2), cyclic AMP response element-binding protein (CREB), and proinflammatory cytokines were assessed using immunoblotting or reverse-transciption polymerase chain reaction. RESULTS: Amitriptyline increased the mechanical withdrawal threshold of the neuropathic rats. The level of phospho-ERK1/2 and phospho-CREB proteins, and proinflammatory cytokines produced by spinal nerve ligation were significantly reduced by amitriptyline administration. However, the use of MRS-1191 before amitriptyline administration not only reduced the threshold of mechanical allodynia, but also increased the signaling protein and proinflammatory cytokine levels, which were reduced by amitriptyline. CONCLUSIONS: The results of this study suggest that the anti-nociceptive effect of amitriptyline involves the suppression of ERK1/2 and CREB signaling proteins, and A3AR activation also affects the alleviation of the inflammatory response.
Adenosine ; Amitriptyline ; Animals ; Antidepressive Agents, Tricyclic ; Cyclic AMP Response Element-Binding Protein ; Cytokines ; Humans ; Hyperalgesia ; Immunoblotting ; Ligation ; Male ; Neuralgia ; Phosphotransferases ; Polymerase Chain Reaction ; Rats ; Rats, Sprague-Dawley ; Receptors, Purinergic P1 ; Spinal Nerves