Vedolizumab (VDZ) has been approved for the treatment of inflammatory bowel diseases (IBDs) in patients aged ≥18 years. We report a case of a pediatric patient with Crohn disease (CD) who was successfully treated with VDZ. A 16-year-old female developed severe active pulmonary tuberculosis (TB) during treatment with infliximab (IFX). IFX was stopped, and TB treatment was started. After a 6-month regimen of standard TB medication, her pulmonary TB was cured; however, gastrointestinal symptoms developed. Due to the concern of the patient and parents regarding TB reactivation on restarting treatment with IFX, VDZ was started off-label. After the second dose of VDZ, the patient was in clinical remission and her remission was continuously sustained. Ileocolonoscopy at 1-year after VDZ initiation revealed endoscopic healing. Therapeutic drug monitoring conducted during VDZ treatment showed negative antibodies to VDZ. No serious adverse events occurred during the VDZ treatment. This is the first case report in Korea demonstrating the safe and effective use of VDZ treatment in a pediatric CD patient. In cases that require recommencement of treatment with biologics after recovery of active pulmonary TB caused by anti-tumor necrosis factor agents, VDZ may be a good option even in pediatric IBD.
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Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome—HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m2/day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.
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Background We aimed to analyze the effectiveness of albumin to globulin ratio (AGR) in predicting postoperative febrile urinary tract infection (fUTI) after ureteroscopic lithotripsy (URS) and retrograde intrarenal surgery (RIRS).
Methods From January 2013 to May 2018, 332 patients underwent URS and RIRS. The rate of postoperative fUTI and risk factors for postoperative fUTI were analyzed using logistic regression. Patients were divided into postoperative fUTI and non-postoperative fUTI (non-fUTI) groups. AGR with other demographic and perioperative data were compared between the two groups to predict the development of fUTI after URS.
Results Of the 332 patients, postoperative fUTI occurred in 41 (12.3%). Preoperative pyuria, microscopic hematuria, diabetes mellitus, hypoalbuminemia, and hyperglobulinemia were more prevalent in the fUTI group. Patients in the fUTI group had larger stone size, lower preoperative AGR, longer operation time, and longer preoperative antibiotic coverage period. In a multivariable logistic analysis, preoperative pyuria, AGR, and stone size were independently correlated with postoperative fUTI (p<0.001, p=0.008, and p=0.041, respectively). Receiver operating curve analysis showed that the cutoff value of AGR that could predict a high risk of fUTI after URS was 1.437 (sensitivity, 77.3%; specificity, 76.9%), while the cutoff value of stone size was 8.5 mm (sensitivity, 55.3%; specificity, 44.7%).
Conclusion This study demonstrated that preoperative pyuria, AGR, and stone size can serve as prognostic factors for predicting fUTI after URS.
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Background Trigeminal neuralgia (TN) is a severe, paroxysmal pain in the distribution of the fifth cranial nerve. Microvascular decompression (MVD) is the most widely used surgical treatment for TN. We undertook this study to analyze the effects of and complications of MVD and to refine the surgical procedure for treating TN.
Methods A total of 88 patients underwent for TN underwent surgery at our hospital. Among them, 77 patients underwent MVD alone, and 11 underwent partial sensory rhizotomy (PSR) with or without MVD. The medical records of these patients were retrospectively analyzed for patient characteristics, clinical results, offending vessels, and complications if any.
Results The mean follow-up duration was 43.2 months (range, 3–216 months). The most common site of pain was V2+V3 territory (n=27), followed by V2 (n=25) and V3 (n=23). The most common offending vessels were the superior cerebellar artery and anterior inferior cerebellar artery in that order. The overall rate of postoperative complications was 46.1%; however, most complications were transient. There were two cases of permanent partial hearing disturbance. In the MVD alone group, the cure rate was 67.5%, and the improvement rate was 26.0%. Among 11 patients who underwent PSR with or without MVD, the cure rate was 50.0%, and the improvement rate was 30.0%.
Conclusion The clinical results of MVD were satisfactory. Although the outcomes of PSR were not as favorable as those of pure MVD in this study, PSR can be considered in cases where there is no significant vascular compressive lesion or uncertainty of the causative vessel at the surgery.
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We report the case of a 16-month-old patient with chronic immune thrombocytopenia (ITP) patient who experienced delayed treatment-free response (TFR) after romiplostim treatment. He received intravenous immunoglobulin every month to maintain a platelet count above 20,000/μL for 2 years. Thereafter, he received rituximab and cyclosporine as second-line therapy, with no response, followed by romiplostim. After 4 weeks of treatment, the platelet count was maintained above 50,000/μL. Following 7 months of treatment, he discontinued romiplostim, and the platelet count decreased. His platelet counts remained above 50,000/μL, without any bleeding symptoms, 2 years after romiplostim discontinuation. This is the first report of TFR after romiplostim treatment in pediatric chronic ITP.
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Background Postoperative acute kidney injury (AKI), which increases the risk of postoperative morbidity and mortality, poses a major concern to surgeons. We conducted this study to analyze the risk factors associated with the occurrence of AKI after orthopedic surgery.
Methods This was a retrospective study that included 351 patients who underwent total hip or knee replacement surgery at Inje University Haeundae Paik Hospital between January 2012 and December 2016.
Results AKI occurred in 13 (3.7%) of the 351 patients. The patients’ preoperative estimated glomerular filtration rate (eGFR) was 66.66±34.02 mL/min/1.73 m2 in the AKI group and 78.07±21.23 mL/min/1.73 m2 in the non-AKI group. The hemoglobin levels were 11.21±1.65 g/dL in the AKI group and 12.39±1.52 g/dL in the non-AKI group. Hemoglobin level was related to increased risk of AKI (odds ratio [OR], 0.13; 95% confidence interval [CI], 0.02–0.68; p=0.016). Administration of crystalloid or colloid fluid alone and the perioperative amount of fluid did not show any significant relationship with AKI. Further analysis of the changes in eGFR was performed using a cutoff value of 7.54. The changes in eGFR were significantly related to decreased risk of AKI (OR, 0.74; 95% CI, 0.61–0.89; p=0.002).
Conclusion Renal function should be monitored closely after orthopedic surgery if patients have chronic kidney disease and low hemoglobin level. Predicting the likelihood of AKI occurrence, early treatment of high-risk patients, and monitoring perioperative laboratory test results, including eGFR, will help improve patient prognosis.
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Background Dialysis patients are prone to having disabilities. We aimed to evaluate the association between disability and various clinical outcomes in Korean dialysis patients.
Methods This study consisted of 1,615 dialysis patients from 27 centers. We evaluated disability by using four questions on the activities of daily living (ADLs) concerning whether help was needed for feeding, dressing/undressing, getting in/out of bed, or taking a bath/shower. We divided the patients into three groups: no disability (Non-D, none of the four ADL domains required help; n=1,312), mild disability (Mild-D, one ADL domain required some/full help; n=163), or moderate to severe disability (MS-D, two or more ADL domains required some/full help; n=140). We evaluated falls, frailty, health-related quality of life (HRQoL), mortality, and hospitalization.
Results The numbers of participants with a fall during the last 1 year were 199 (15.2%), 42 (25.8%), and 44 (31.4%) in the Non-D, Mild-D, and MS-D groups, respectively (p<0.001). The numbers of participants with frailty in the Non-D, Mild-D, and MS-D groups were 381 (29.0%), 84 (51.5%), and 93 (66.4%), respectively (p<0.001). In both univariate and multivariate analyses, the physical component scale and mental component scale scores decreased as the grade of disability increased (p<0.001 for both scores). Hospitalization-free survival rate at 500 days was 64.2%, 56.7%, and 51.1% in the Non-D, Mild-D, and MS-D, respectively (p=0.001 for trend). Patient survival rate at 500 days was 95.3%, 89.5%, and 92.3% in the Non-D, Mild-D, and MS-D, respectively (p=0.005 for trend).
Conclusion Disability was associated with falls, frailty, HRQoL scales, and survival trends in Korean dialysis patients.
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Background This study is an analysis of evaluator factors affecting physician-patient interaction (PPI) scores in clinical performance examination (CPX). The purpose of this study was to investigate possible ways to increase the reliability of the CPX evaluation.
Methods The six-item Yeungnam University Scale (YUS), four-item analytic global rating scale (AGRS), and one-item holistic rating scale (HRS) were used to evaluate student performance in PPI. A total of 72 fourth-year students from Yeungnam University College of Medicine in Korea participated in the evaluation with 32 faculty and 16 standardized patient (SP) raters. The study then examined the differences in scores between types of scale, raters (SP vs. faculty), faculty specialty, evaluation experience, and level of fatigue as time passes.
Results There were significant differences between faculty and SP scores in all three scales and a significant correlation among raters’ scores. Scores given by raters on items related to their specialty were lower than those given by raters on items out of their specialty. On the YUS and AGRS, there were significant differences based on the faculty’s evaluation experience; scores by raters who had three to ten previous evaluation experiences were lower than others’ scores. There were also significant differences among SP raters on all scales. The correlation between the YUS and AGRS/HRS declined significantly according to the length of evaluation time.
Conclusion In CPX, PPI score reliability was found to be significantly affected by the evaluator factors as well as the type of scale.
MicroRNAs (miRNAs) are a class of noncoding RNAs that negatively regulate target messenger RNAs. In multicellular eukaryotes, numerous miRNAs perform basic cellular functions, including cell proliferation, differentiation, and death. Abnormal expression of miRNAs weakens or modifies various apoptosis pathways, leading to the development of human cancer. Cell death occurs in an active manner that maintains tissue homeostasis and eliminates potentially harmful cells through regulated cell death processes, including apoptosis, autophagic cell death, and necroptosis. In this review, we discuss the involvement of miRNAs in regulating cell death pathways in cancers and the potential therapeutic functions of miRNAs in cancer treatment.
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Homozygous mutations in NUDT15 R139C are known as the major factor associated with thiopurine-induced early leukopenia, particularly in Asian patients. Therefore, NUDT15 genotyping is currently recommended before thiopurine treatment to identify patients who are NUDT15 poor metabolizers and consider the use of an alternative immunomodulatory therapy. We report a case of a 12-year-old Korean girl with Crohn’s disease (CD), in whom thiopurine-induced leukopenia was prevented by initiation of azathioprine (AZA) therapy at a low dose (0.5 mg/kg/day) and early detection of significant hair loss and white blood cell (WBC) count decrease at 17 days from the start of AZA treatment. The WBC count dropped from 8,970/μL to 3,370/μL in 2 weeks, and AZA treatment was stopped because of concerns of potential leukopenia in the near future. Her WBC count recovered to 5,120/μL after 3 weeks. Gene analysis later revealed that she had a homozygous mutation in NUDT15 R139C, resulting in a poor metabolizing activity of NUDT15. In situations when NUDT15 genotyping is unavailable, initiation of AZA therapy at 0.5 mg/kg/day with close observation of hair loss and WBC counts within 2 weeks may be an alternative way to prevent thiopurine-induced early leukopenia in Asian children with CD.
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Fibrosis is characterized by excessive accumulation of extracellular matrix components. The fibrotic process ultimately leads to organ dysfunction and failure in chronic inflammatory and metabolic diseases such as pulmonary fibrosis, advanced kidney disease, and liver cirrhosis. Idiopathic pulmonary fibrosis (IPF) is a common form of progressive and chronic interstitial lung disease of unknown etiology. Pathophysiologically, the parenchyma of the lung alveoli, interstitium, and capillary endothelium becomes scarred and stiff, which makes breathing difficult because the lungs have to work harder to transfer oxygen and carbon dioxide between the alveolar space and bloodstream. The transforming growth factor beta (TGF-) signaling pathway plays an important role in the pathogenesis of pulmonary fibrosis and scarring of the lung tissue. Recent clinical trials focused on the development of pharmacological agents that either directly or indirectly target kinases for the treatment of IPF. Therefore, to develop therapeutic targets for pulmonary fibrosis, it is essential to understand the key factors involved in the pathogenesis of pulmonary fibrosis and the underlying signaling pathway. The objective of this review is to discuss the role of kinase signaling cascades in the regulation of either TGF--dependent or other signaling pathways, including Rho-associated coiled-coil kinase, c-jun N-terminal kinase, extracellular signal-regulated kinase 5, and p90 ribosomal S6 kinase pathways, and potential therapeutic targets in IPF.
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Synchronous gastric cancer and adenomatous colorectal polyp in patients with Klebsiella pneumoniae-induced pyogenic liver abscess (KP-PLA) and bacteremia is a rare presentation. A 58-year-old man with a 6-month history of diabetes mellitus (DM) presented with febrile sensation and dull abdominal pain in the right upper quadrant of the abdomen. Subsequent to laboratory test results and abdominal computed tomography findings, KP-PLA with bacteremia was diagnosed. After intravenous antibiotic administration, his symptoms improved, and upper endoscopy and colonoscopy were performed to evaluate the cause of KP-PLA. Biopsy specimens of the prepyloric anterior wall revealed a moderately differentiated adenocarcinoma. Endoscopic mucosal resection of the colon revealed high-grade dysplasia. Early gastric cancer (EGC) and adenomatous colorectal polyps with high-grade dysplasia concomitant with KP-PLA and bacteremia were diagnosed in our patient who had DM. Intravenous antibiotic treatment for KP-PLA, subtotal gastrectomy for EGC, and colonoscopic mucosal resection for the colon polyp were performed. After 25 days of hospitalization, subtotal gastrectomy with adjacent lymph node dissection was performed. Follow-up ultrasound imaging showed resolution of the abscess 5 weeks post-antibiotic treatment, as well as no tumor metastasis. Upper gastrointestinal endoscopy and colonoscopy should be performed to evaluate gastric cancer in patients with PLA or bacteremia, accompanied with DM or an immunocompromised condition.