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BACKGROUND: Targeted treatment modalities for non-small cell lung carcinoma (NSCLC) patients are expanding rapidly and demand a constant adaptation of molecular testing strategies. In this regard, broad reflex testing via next-generation sequencing (NGS) might have several advantages. However, real-world data regarding practical feasibility and clinical relevance are scarce, especially for RNA-based NGS. METHODS: We performed a retrospective study comparing NGS use in two consecutive years (2019 and 2020). In 2019, reflex testing mainly consisted of DNA-based NGS for mutations and immunohistochemistry (IHC) for ALK, ROS1, and NTRK fusion products. At the beginning of 2020, our approach has changed, with DNA- and RNA-based NGS panels now being simultaneously performed. This change in protocol allowed us to retrospectively evaluate if broad molecular reflex testing brings additional value to lung cancer patients. RESULTS: Within the whole cohort (n=432), both DNA- and RNA-based NGS yielded almost always evaluable results. Only in 6 cases, the RNA content was too little for an appropriate analysis. After integrating RNA-based NGS in the reflex testing approach, the number of detected fusions increased significantly (2.6% vs. 8.2%; P=0.0021), but also more patients received targeted therapies. Furthermore, exceedingly rare alterations were more likely to be detected, including the so far undescribed EGFR-NUP160 fusion. CONCLUSIONS: Our study demonstrates that a comprehensive approach to reflex NGS testing is practically feasible and clinically relevant. Including RNA-based panels in the reflex testing approach results in more detected fusions and more patients receiving targeted therapies. Additionally, this broad molecular profiling strategy identifies patients with emerging biomarkers, underscoring its usefulness in the rapidly evolving landscape of targeted therapies.

Management of secondary central nervous system (SCNS) involvement in relapsed or refractory aggressive B-cell lymphomas remains an area of unmet medical need. We report a single-center retrospective analysis of 7 adult patients with SCNS lymphoma (SCNSL) who underwent chimeric antigen receptor (CAR) T-cell therapy for their refractory disease, and we describe the safety of whole brain radiation therapy (WBRT) as a bridging therapy. Six patients (85.7%) achieved a complete response at day 28, and 1 patient had progressive disease. The median progression-free survival was 83 days (range, 28-219 days), and median overall survival was 129 days (range, 32-219 days). Three patients died as a result of disease progression. Of the 5 patients who received WBRT as bridging therapy, 3 had no immune effector cell–associated neurotoxicity syndrome (ICANS), but 2 patients had grade 1 or grade 3 ICANS. No grade 4 ICANS was reported in this subset of patients. We conclude that SCNSL should not preclude patients from receiving CAR T-cell therapy as a treatment option because of concerns regarding ICANS, and bridging with WBRT is not associated with increased ICANS., Key Points SCNSL should not preclude patients from receiving CAR T-cell therapy because of concerns regarding ICANS.WBRT is not associated with increased ICANS when used as a bridge to CAR T-cell therapy with a short median interval in SCNSL.

The prevalence of intracardiac thrombus (ICT) is gradually increasing, though it is rare among children. Data related to the occurrence of ICT among children are limited, and treatment recommendations have been made utilizing adult guidelines. The primary objective of this study is to determine associated factors, management, and outcomes of intracardiac thrombosis in children. Between January 2013 and January 2020, patients diagnosed with ICT at the Pediatric Hematology-Oncology and Pediatric Cardiology departments in our hospital were included in the study. Demographic characteristics, clinical and laboratory findings, treatment protocols, and outcomes were analyzed retrospectively. The median age at diagnosis was 10.5 months (2 days to 14.5 years), and the median follow-up period was 6.5 months (1 month to 3.1 years). The most common primary diagnoses of the patients, in order of frequency, were heart disease (n: 8), metabolic disease (n: 3), prematurity and RDS (n: 3), burns (n: 2), pneumonia (n: 2), and asphyxia (n: 2). CVC was present in 19/23 of the patients. The reasons for CVC insertion were the need for plasmapheresis in one patient with a diagnosis of HUS and the need for well tolerated vascular access because of long-term hospitalization in others. LMWH was administered to all patients as first-line therapy. Complete response was achieved in 19 (79%) of 24 patients and 4 patients (16.6%) were unresponsive to medical treatment. It was found out that the thrombus location, type, sepsis, and hemoculture positivity, as well as the presence of CVC, had no impact on treatment response (chi-square P = 0.16, 0.12, 0.3, 0.49, 0.56). Moreover, no correlation was determined between thrombus size and treatment response (Mann Whitney U test P = 0.47). The mortality rate was determined to be 12.5% (3/24). Spontaneous occurrence of ICT is rare in childhood, without any underlying primary disease or associated factor. The presence of CVC, sepsis, and heart disease are factors associated with ICT. The success rate is increased with medical treatment. There was no significant difference in treatment response between the newborn and 1 month to 18-year-old patient group. It has been demonstrated that thrombus size, type, localization; sepsis, and hemoculture positivity had no impact on the treatment response.

Background To evaluate long-term efficacy of left atrial appendage occlusion with multiple devices. Methods All consecutive patients who underwent left atrial appendage occlusion (LAAO) with a follow-up of at least 4 years, were included in this single center, retrospective registry. No specific exclusion criteria were applied. LAA occlusion was performed with the Watchman, Watchman FLX, Amplatzer Cardiac plug or Amplatzer Amulet occluder devices. Results A total of 224 consecutive patients underwent LAAO occlusion. Mean age was 72.5 ± 9.0 years. A history of stroke was present in 29%, TIA in 8.5% and a previous episode of bleeding in 64.7% of patients. In 63% there was a contraindication to oral anticoagulants. The average CHADS-VASc was 4.0 ± 1.6 and the average HAS-BLED was 3.4 ± 1.3. There was a reduction of strokes of 72.9%, thromboembolic events of 59.7% and major bleeding events of 70.9% compared to historic data. During follow-up, 48.3% of the ischemic and major bleeding events occurred within the first year. The annual mortality rate of 7.5 deaths/ 100 patients years. There were no significant differences in terms of outcome between the devices used and there were no late events associated with any device. The main antithrombotic regimen in the long term was with single antiplatelet therapy and the second one was no therapy. Conclusion LAAO is a safe and effective procedure, that reduces ischemic and bleeding events in the long-term, regardless of the type of device used, in AF patients at high risk of ischemic stroke and major bleeding, without the need of anticoagulation.

Background: Lymphangiomas represent 5% of all benign pediatric tumors. Abdominal lymphangiomas (ALs) are extremely rare. Therapy includes surgery, sclerotherapy, or pharmacological treatment. Lapar...

Background and study aims Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessively inherited disease that causes intrahepatic-hepatocellular cholestasis. PFIC constitutes approximately 10–15% of cholestatic liver diseases in children. The aim of this study is to draw attention to this group of diseases, which pose a higher risk, in societies where consanguineous marriage is more common, and to share our experiences since the studies in the literature, regarding this group of diseases are case series with small number of patients. Patients and methods This cross-sectional study was conducted on 34 patients who were admitted with jaundice and diagnosed by genetic analysis, between January 2015 and July 2020. Results We found 17.6% of patients with PFIC type 1, 55.9% patients had PFIC type 2, 14.7% patients had PFIC type 3, 8.8% patients had PFIC type 4 and 2.9% patients had PFIC type 5. Partial internal biliary diversion was performed in 5 (14.7%) patients, who had severe itching during follow-up, did not respond to medical treatment, and did not have significant fibrosis in liver biopsy yet. The degree of itching before PIBD was rated as +4 (cutaneous erosion, bleeding and scarring), in 5 patients and the rates were 0 (absent) in two patients, and +1 (mild itching) in 3 patients, 6 months after PIBD, these differences were statistically significant(p = 0.027). The mean weight z score was-1.43 (-3.72-+0.73), before PIBD, while it was 0.39(-1.86 -+2.45), six months after PIBD; the diference was statistically significant(p = 0.043). Liver transplantation was performed in 12 (35.3%) patients with significant fibrosis in liver biopsy and developing signs of portal hypertension. Conclusion The PFIC disease group is a heterogeneous disease group that is difficult to diagnose and treat. It should be considered in patients with cholestasis and/or pruritus and those with a history of consanguineous marriage between parents and death of a sibling with similar clinical symptoms.

Background: Minimally invasive surgery (MIS) is now the gold standard for nephrectomy in pediatric patients. Retroperitoneoscopic (using either one or two instruments) and transperitoneal (TP) approaches are described. We compared the perioperative outcomes of different techniques [single-instrument retroperitoneoscopic (SIRP), two-instrument retroperitoneoscopic (TIRP), TP, and open approach]. Patients and Methods: Retrospective review of patients who underwent nephrectomy surgery in the period from January 2009 to January 2020 at a single center was carried out. We excluded patients who underwent other procedures under the same anesthetic, underwent heminephrectomy, and those with incomplete records. The primary outcome measures were operative time, intraoperative complications, postoperative complications, and length of hospital stay. One-way analysis of variance (ANOVA) test was used to analyze continuous variables. Chi square test was used to compare categorical variables. Results: A total of 213 nephrectomies were analyzed; SIRP (n = 35), TIRP (n = 50), TP (n = 74), and open (n = 54). Median age (months) was 71 for SIRP, 113 for TIRP, 67 for TP, and 21 for open. No statistical difference was identified for mean operative time (P = .067) or mean hospital stay (P = .69). Intraoperative complications were significantly more in the open group (P = .03). Postoperative complications were rare and only noted in the open group. There was no conversion to open surgery in the SIRP and TIRP groups. Conversion rate was 5.4% (4/74) in the TP group. Conclusion: MIS nephrectomy is safe, and no difference among techniques (SIRP, TIRP and TP) has been demonstrated. They are comparable to open surgery in terms of operative time and hospital stay, but are associated with significantly less complications.

Objective Chordoma is a rare neoplasm of the neuraxis derived from remnants of the primitive notochord. The importance of wide margins and use of adjuvant therapy in spinal chordomas are still contentious points in the management of spinal chordomas. We conducted a systematic review of the surgical management of spinal chordomas alongside our 10-year institutional experience. Methods A systematic search of the literature was performed in November 2020 using Embase and MEDLINE for articles regarding the surgical management of chordomas arising from the mobile spine and sacrum. We also searched for all adult patients who were surgically managed for spinal chordomas at our institute between 2010 and 2020. In both the systematic review and our institutional case series, data on adequacy of resection, use of adjuvant therapy, complications, recurrence (local or metastatic), and survival outcomes were collected. Results We identified and analyzed 42 articles, yielding 1531 patients, from which the overall gross total or wide resection rate was 54.9%. Among the 8 cases in our institutional experience (4 sacral, 3 cervical, and 1 lumbar), we achieved gross total resection in 50% of initial operations. The recurrence rate was 25% in our gross total resection group and 50% where initial resection was subtotal. Of patients, 75% had no evidence of recurrence at most recent follow-up. Conclusions Albeit difficult at times because of the proximity to neurovascular tissue, achieving a wide resection followed by adjuvant therapy for spinal chordomas is of great importance. Multidisciplinary discussion is valuable to ensure the best outcome for the patient.

Association between opioid abuse and intracranial aneurysms rupture has been suggested in recent studies. However, these observations are limited to single center studies and could be benefited from validation in larger cohorts. Hence, we aimed to study the association between age at aneurysmal subarachnoid hemorrhage (aSAH) and opioid use disorders (OUD) using a large, national database.This study was conducted using the 2016 and 2017 National Inpatient Sample (NIS) with ICD-10 codes. Cohorts were categorized as "Non-users", "OUD", and "Multi-drug users". Linear regression models were used to examine the association between OUD and multi-drug users with age at aneurysm rupture, and multiple logistic regression models were used for the association between in-hospital mortality and drug abuse.A total of 17,391 patients with aSAH were captured in the 2016 and 2017 NIS database. Out of these patients, 235 (1.4%) were included in the OUD group and 59 (0.3%) in the multi-drug users' group. Adjusted linear regression showed an unstandardized coefficient (UC) = -12.3 [95%CI = -14.4/-10.1, p 0.001] for OUD patients and an UC = -16.8 [95%CI = -21.1/-12.5, p 0.001] for multi-drug users, compared to non-users. The risk of in-hospital mortality was significantly increased in drug user, OR = 1.47 [95%CI: 1.1-2.01, p = 0.017] for OUD patients, and OR = 2.35 [95%CI: 1.35-4.11, p = 0.003] for multi-drug users.This is the first national study to examine the association between age at intracranial aneurysms rupture and opioid abuse. aSAH patients with history of OUD were 12 years younger compared to non-users, when OUD was combined with other drugs, the age at aneurysms rupture was 17 years younger. Further elucidation regarding the mechanisms by which opioids triggers aneurysms rupture and predispose to worsen outcomes following aSAH is required, as well as appropriate prevention, and management strategies for aSAH patients with OUD.

Key points Question Is hepatitis C virus (HCV) micro-elimination achievable at the hospital level with the structured strategies? Findings The multidirectional program included the HCV reflex test for hospital personnel, outpatient surveillance, a call-back system, and surveillance of cancer patients prior to chemotherapy. Through the plans of the study, 97.8% of the HCV-viremic patients successfully received linkage-to-treat. The results of each strategy sufficiently met the 2030 elimination goal by the World Health Organization (WHO). Meaning HCV micro-elimination is achievable at the hospital level based on patient safety, staff occupational safety and infection control., Background and aims Scarce data are available on in-hospital hepatitis C virus (HCV) micro-elimination strategies. This pilot study was prospectively conducted to assess the outcomes of HCV in-hospital micro-elimination program (HCV-HELP) in a single center in Taiwan. Methods The study included the HCV reflex test for plans A (hospital personnel), B (outpatient surveillance), C (a call-back system for anti-HCV+ patients), and D (surveillance of cancer patients prior to chemotherapy). The primary outcome measurement was that > 80% of eligible patients were enrolled in linkage-to-treat; the secondary outcome measurement was the surveillance efficacy. Results We recruited 930, 6072, 2376 and 233 participants into plans A, B, C, and D, respectively, from Oct 2020 to May 2021. The anti-HCV-seropositivity prevalences were 0.22% for plan A, 4.3% for B, and 3.9% for D. Two staff members were identified as HCV-viremic in plan A; these staff members successfully achieved a sustained virological response (SVR). We identified 39, 95 and 2 HCV-viremic patients in plans B, C, and D, respectively. Of these 138 HCV-viremic patients, 135 (97.8%) received direct-acting antiviral therapy, and 134 achieved SVR. Two 4-month phases were stratified to compare efficacies in the liver clinic. In the late phase, the adjusted number of HCV-viremic patients was 4.36/10,000 outpatient visits (90/200,689), which was 3.18-fold higher than that of the early phase (1.37/10,000 outpatient visits [30/212,658], odds ratio 3.18; 95% confidence interval 2.10–4.81, p

Objectives We used single-center data to evaluate the long-term outcome of percutaneous transluminal angioplasty (PTA) for pediatric renovascular hypertension (RVH) and to analyze the factors that influence effectiveness. Methods We retrospectively evaluated 33 pediatric RVH patients (18 boys; mean age: 9.1 ± 4 years, range: 2–16) who underwent PTA from January 2007 to December 2019. 15 patients had Takayasu arteritis (TA) and 18 were non-TA. The median follow-up from the initial PTA was 69 months (range: 12–157; IQR: 25.5–89). Results The technical success rate of 52 PTA procedures was 90.4% in 33 children. Renal artery stents were implanted in two patients, external guidewires were used in two patients, and a drug-coated balloon was used in only one patient. The overall effective rate of PTA was 63.6%, including cured 39.4% and improved 24.2%, at the end of follow-up. Overall clinical outcomes were not statistically different between the TA and non-TA groups ( p = 0.316), nor were cure rates ( p = 0.072). 15 patients received reintervention due to restenosis after the first successful PTA; the interval was 2–56 months (median: 12 months). Four patients received reintervention due to a failed PTA. A total of four patients received open surgery. Binary logistics regression analysis showed that stenosis length and residual stenosis rate were strongly correlated with effective PTA ( p = 0.045, p = 0.044). Conclusions As a primary treatment for pediatric RVH, PTA can achieve satisfactory results, which are influenced by lesion length and residual stenosis rate.

Background An association between specific endoscopic findings and high-grade dysplasia/carcinoma in superficial nonampullary duodenal epithelial tumors ≤ 5 mm in size has not been reported. We sought to identify the endoscopic findings associated with high-grade dysplasia/carcinoma in patients with superficial nonampullary duodenal epithelial tumors ≤ 5 mm. Methods We retrospectively assessed the data of 84 patients (88 lesions; low-grade dysplasia: n = 35, high-grade dysplasia/carcinoma: n = 53) with superficial nonampullary duodenal epithelial tumors who underwent initial treatment at a single center (from July 2009 to April 2021). All the patients had lesions sized ≤ 5 mm. We assumed that the endoscopic findings were independently associated with high-grade dysplasia/carcinoma and determined the accuracy, sensitivity, and specificity of a combination of independent factors for diagnosing high-grade dysplasia/carcinoma and low-grade dysplasia. Results Multivariate logistic regression of significant factors in the univariate analysis revealed that lesions with depressed morphology (odds ratio: 23.9, 95% confidence interval: 2.8-204.2; p = 0.0037) and a reddish color (odds ratio: 175.7, 95% confidence interval: 11.4-2697.1; p = 0.0002) were independently associated with high-grade dysplasia/carcinoma. McNemar's test revealed that combining the macroscopic type and color provided significantly higher sensitivity for diagnosing high-grade dysplasia/carcinoma than color alone (98.1%, 95% confidence interval: 90.1-99.7 vs. 71.7%, 95% confidence interval: 58.4-82.0; p = 0.0002). Conclusions Reddish and depressed-type lesions before treatment were associated with high-grade dysplasia/carcinoma. Combining the macroscopic type and color can help detect high-grade dysplasia/carcinoma. These findings could help clinicians determine the best therapeutic strategy for patients with smaller (≤ 5 mm) superficial nonampullary duodenal epithelial tumors in clinical settings.

Aim Method Results Conclusion The study aimed to describe clinical characteristics and outcomes of pregnant women with COVID‐19 undergoing cesarean section, and evaluated the association of blood values at admission with severe COVID‐19 disease in this group of patients.We retrospectively analyzed the clinical data of 110 patients infected with COVID‐19 who underwent cesarean section at Adana City Education and Research Hospital in Turkey. The COVID‐19 severity of the patients was classified as either severe or nonsevere disease according to World Health Organization of COVID‐19 clinical management guidance. We compared blood values, clinical characteristics, and outcomes between severe and nonsevere patients. Receiver operating characteristics (ROC) curves analyses and area under the ROC curve (AUC) value was calculated to evaluate the predictive value of blood parameters on the COVID‐19 severity.Of the 110 women, 12 were severe cases. Severe patients had higher ferritin, neutrophil‐to‐lymphocyte ratio (NLR), lactate dehydrogenase (LDH), alanine transaminase (ALT), aspartate transaminase (AST), and procalcitonin levels on admission (p

BackgroundAlthough most evidence supports the fact that kidney transplant (KT) recipients are at significant risk of morbidity and mortality, risk factors of accruing COVID-19 in this population have remained poorly defined.MethodsAll KT recipients who had been transplanted in Sina Hospital and were actively followed between March 1996 and January 2021 were enrolled in a retrospective manner. The demographic characteristics, immunosuppressive treatment before KT, and death were gathered by calling patients with a designed questionnaire.Results108 (about 21%) of 523 KT recipients were diagnosed with COVID-19. The mean age of COVID-19 patients was 46.9 ± 13.6, of whom 43% were women. In the multivariate model, body mass index (BMI) ≥ 30 independently increased the risk of COVID-19 incidence with OR 2.00 (95% CI 1.23, 3.26) (P = 0.00), and besides, having diabetes had a marginal association with COVID-19 incidence (OR 1.62 [95% CI 0.98, 2.66];P = 0.057). The mortality rate of COVID-19 was 15%. In the multivariate model, only pre-transplantation diabetes significantly increased the risk of death by COVID-19 with OR of 3.90 (95% CI 1.00–15.16) (P = 0.04).ConclusionGiven the higher incidence rate in KT recipients with obesity and diabetes and higher mortality rate in KT recipients with diabetes as the cause of ESRD, more attention should be paid to KT recipients with these risk factors.

Purpose The relationship between posterior-circulation lesion volume (PCLV) and clinical outcomes is poorly investigated. We aimed to analyze, in patients with acute basilar artery occlusion (ABAO), if pre-endovascular treatment (EVT) PCLV was a predictor of outcomes. Methods We analyzed consecutive MRI selected, endovascularly treated ABAO patients. Baseline PCLV was measured in milliliters on apparent diffusion-coefficient map reconstruction. Univariable and multivariable logistic models were used to test if PCLV was a predictor of 90-day outcomes. After the received operating characteristic (ROC) analysis, the optimal cut-off was determined to evaluate the prognostic value of PCLV. Results A total of 110 ABAO patients were included. The median PCLV was 4.4 ml (interquartile range, 1.3-21.2 ml). Successful reperfusion was achieved in 81.8% of cases after EVT. At 90 days, 31.8% of patients had a modified Rankin scale ≤ 2, and the mortality rate was 40.9%. PCLV was an independent predictor of functional independence and mortality (odds ratio [OR]:0.57, 95% confidence interval [CI], 0.34-0.93 and 1.84, 95% CI, 1.23-2.76, respectively). The ROC analysis showed that a baseline PCLV ≤ 8.7 ml was the optimal cut-off to predict the 90-day functional independence (area under the curve [AUC] = 0.68, 95% CI, 0.57-0.79, sensitivity 88.6%, and specificity 49.3%). In addition, a PCLV ≥ 9.1 ml was the optimal cut-off for the prediction of 90-day mortality (AUC = 0.71, 95% CI, 0.61-0.82, sensitivity 80%, and specificity 60%). Conclusions Pre-treatment PCLV was an independent predictor of 90-day outcomes in ABAO. A PCLV ≤ 8.7 and ≥ 9.1 ml may identify patients with a higher possibility to achieve independence and a higher risk of death at 90 days, respectively.

PURPOSE: To assess whether live birth rates (LBR) and maternal/neonatal complications differed following single fresh and frozen-warmed blastocyst transfer. METHODS: The present retrospective observational study analyzed 4,613 single embryo transfers (SET) (646 fresh and 3,967 frozen) from January 1, 2014, to December 31, 2018. Fresh embryo transfer at blastocyst stage was considered according to the age of the patient and her prognosis. In case of the risk of ovarian hyperstimulation syndrome, premature progesterone rise, non-optimal endometrial growth, or supernumerary embryos, cryopreservation with subsequent frozen embryo transfer (FET) was indicated. RESULTS: No differences in LBR were recorded. Fresh embryo transfers yielded an increase both in neonatal complications OR 2.15 (95% CI 1.20–3.86, p 0.010), with a higher prevalence of singletons weighting below the 5th percentile (p 0.013) and of intrauterine growth retardation (p 0.015), as well as maternal complications, with a higher placenta previa occurrence OR 3.58 (95% CI 1.54–8.28, p 0.003), compared to FET. CONCLUSION: LBR appears not to be affected by the transfer procedure preferred. Fresh embryo transfer is associated with higher risk of neonatal complications (specifically a higher prevalence of singletons weighting below the 5th percentile and of intrauterine growth retardation) and placenta previa. Reflecting on the increased practice of ART procedures, it is imperative to understand whether a transfer procedure yields less complications than the other and if it is time to switch to a “freeze-all” procedure as standard practice. Trial registration: Clinical Trial Registration Number: NCT04310761. Date of registration: March 17, 2020, retrospectively registered. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10815-021-02362-3.

Insights into the molecular pathogenesis of respiratory viral infections were investigated using serum and peripheral blood from patients with clinical syndromes. Signatures of expression of cytokines, genes and apoptotic proteins that discriminate symptomatic individuals from healthy individuals were determined among 21 patients. In symptomatic patients, significant upregulation of IL-1β, IL-2, IL-4, IL-6, IL-8, IL-12, IL-15, TNF-a and IFN-g (P

Backgroud/Objectives: Transoral laser laryngeal microsurgery (LTLM) has been widely used in the treatment of early-stage glottic laryngeal squamous cell carcinoma (LSCC) for the past few decades. Although T stage, tumor grade, anterior commissure involvement, type of cordectomy, positive surgical margin, and postoperative additional therapies were accused as the prognostic factors for recurrence, there is still controversy about these data in the literature. The purpose of this study was to evaluate the oncological results of our patients with early glottic LSCC treated with LTLM as a single-modality therapy in a single-center study. Methods: Patients with early-stage (Tis-1–2/N0) glottic LSCC who underwent LTLM as a primary treatment from 2011 to 2019 were retrospectively reviewed. The clinicopathological factors and oncologic outcomes were analyzed. Results: One hundred and sixty-one patients were enrolled in this study. The 5-year overall (OS), disease-specific (DSS), disease-free (DFS), and laryngectomy-free survival rates were 84.5%, 97.9%, 79.2%, and 93.5%, respectively. The most common stage, histopathological type, and type of endoscopic cordectomy were T1 stage, well-differentiated cancer, and type 2 cordectomy, respectively. A positive surgical margin was defined in 20 (12.4%) patients. There was a significant relationship between histopathological grade and positive surgical margins (p = 0.038). OS and DSS rates of “wait and see” modality were lower, while DFS of radiotherapy was lower than that of other treatment modalities in patients with positive surgical margins, but the differences were not statistically significant. Nineteen (11.8%) patients had a recurrence. DSS was statistically significantly lower in patients with recurrence (p < 0.001). Conclusion: The results of our study showed that anterior commissure involvement, surgical margin positivity, and higher T stage statistically did not reduce survival rates in early-stage LSCC patients treated with LTLM. As the histopathological grade of the tumor worsens, the risk of surgical margin positivity increases. RT may have a negative effect on recurrence and organ preservation in the additional treatment of patient with positive surgical margins.

Seizures represent a core symptom of autoimmune encephalitides with specific therapeutic issues. To date, patients with new-onset seizures or established epilepsy are not systematically tested for autoimmune antibodies. We aimed to identify clinical and paraclinical criterion that could help to select patients requiring additional autoimmune antibodies serum and cerebrospinal fluid (CSF) detection. In this retrospective single center study from the French Salpetriere Hospital, data from 286 adult patients with epilepsy who received an autoantibody assay for the first time were analyzed. All patients were evaluated at our institution between January 2007 and December 2018 for assessment of new-onset epilepsy (n = 90) or established epilepsy (n = 196). We only analyzed patients that were screened for autoimmune antibodies. Demographic, clinical and neuroimaging measures were compared between patients with and without autoimmune encephalitis using Fisher’s exact test for categorical variables and Welch’s t test for continuous variables. Our primary goal was to identify significant factors that differentiated patients with and without autoimmune encephalitis. We identified 27 patients with autoimmune epilepsy (9.4% of the patients who had been tested for autoantibodies). The significant factors differentiating patients with and without autoimmune encephalitis were: (i) the existence of a new-onset focal epilepsy + (e.g., newly diagnosed epilepsy

STUDY DESIGN Retrospective single center propensity-matched observational cohort study that included patients who underwent 1- to 3-level lumbar fusion surgery for degenerative conditions. OBJECTIVE To compare 90-day complication rates between robotic-assisted and non-robotic-assisted lumbar spinal fusions in propensity-matched cohorts. SUMMARY OF BACKGROUND DATA A recent administrative database (PearlDiver) study reported increased 30-day complications with the utilization of robotic-assisted enabling technology. METHODS Of 146 robotic-assisted cases that met inclusion criteria, 114 were successfully propensity matched to 114 patients from 214 cases who had 1 to 3 level lumbar fusion without robotic assistance based on age, sex, body mass index, smoking status, American Society of Anesthesiologist grade, number of surgical levels, primary versus revision, and surgical approach (posterior-only or anterior-posterior). We excluded tumor, trauma, infection, or deformity cases. Outcomes included surgical and medical (major/minor) complications at intraoperative, immediately postoperative, 30- and 90-day postoperative intervals, including reoperations, and readmissions within 90 days. RESULTS All cause intraoperative complication rates were similar between non-robotic-assisted (5.3%) and robotic-assisted groups (10.5%, P = 0.366). Immediate postoperative medical complication rate was also similar between non-robotic-assisted (6.1%) and robotic-assisted groups (1.8%, P = 0.089). Thirty-day complication rates, 90-day complication rates, reoperation rates, and readmission rates showed no difference between non-robotic-assisted and robotic-assisted groups. There was no difference between return to OR for infection between the cohorts (non-robotic-assisted: 6 [5%] vs. robotic-assisted: 1 [0.8%], P = 0.119). There was however improved length of stay (LOS) in the robotic-assisted group compared with non-robotic-assisted group (2.5 vs. 3.17 days, P = 0.018). CONCLUSION In propensity-matched cohorts, patients undergoing 1- to 3-level robotic-assisted posterior lumbar fusion for degenerative conditions did not have increased 90-day complication rate, and had a shorter length of stay compared with non-robotic-assisted patients. There findings differ from a prior administrative database study as the robotic-assisted group in the current study had 0% return to OR for malpositioned screws and 0.8% return to OR for infection.Level of Evidence: 2.

Objective: Being vaccinated against encapsulated bacteria is the most efficient way to reduce painful crises and mortality in patients with sickle cell disease (SCD). Although guidelines strongly recommend vaccination, vaccination rates remain under the desired levels. In this study, we aim to determine vaccination rates and understand the reasons for non-vaccination in patients with SCD. Material and methods: We included 76 patients with SCD in this study. We administered a questionnaire consisting of 21 questions and examined the electronic vaccination records of these patients. Results: The vaccination rates were 36.5% for the pneumococcal vaccine, 22.4% for the Hemophilus influenza type b vaccine, and 19.7% for the meningococcal vaccine. Residence in rural areas and annual control visits were found to increase the pneumococcal vaccination rates (OR: 11.90, 95% CI: 2.549–56.107, p = 0.002 and OR: 9.08, 95% CI: 1.120–73.624, p = 0.039, respectively) and meningococcal vaccination rates (OR: 2.75, 95% CI: 1.464–5.186, p = 0.002 and OR:1.36, 95% CI: 1.159–1.610, p < 0.001, respectively). Thirty-four (44.7%) of the cases stated that their doctors did not give any information about these vaccinations. Conclusion: Vaccination rates are low in patients with SCD. Residence in rural areas, annual control visits, educational level, and doctor recommendations affect these vaccination rates.

INTRODUCTION: Traumatic hip dislocation in childhood is a rare injury. The aim of this study was to present the experience of a single center for traumatic hip dislocation in the pediatric and adolescent age group. MATERIALS AND METHODS: The orthopedic trauma database of a single center was retrospectively searched for patients who had sustained a traumatic hip dislocation between 2004 and 2019. Data related to age, mechanism of injury, additional fractures, presence of any open fracture or neurovascular injury, and dislocation side were retrieved from the patient files. Functional outcome was evaluated using the Harris Hip Scoring system. RESULTS: The mean age of 13 patients at the time of injury was 7.7 years and the mean follow-up time was 124.5 months (10.4 years). All traumatic hip dislocations were posterior dislocations. The mean Harris Hip score was 95.7 points (range 55–100 points) with 12 patients scored as excellent and 1 as poor results. Ten complications including limb length inequality, avascular necrosis and hip arthritis were determined in seven patients. CONCLUSION: Traumatic hip dislocation is a rare injury in the pediatric age group. Early diagnosis and concentric reduction of the femoral head is essential for better functional results and to avoid complications. Limb length inequality is a frequently seen complication, which has not been reported in previous studies.

Amaç: Tekrarlayıcı seronegatif pitting ödemli simetrik sinovit sendromu (R3SPE), akut poliartrit başlangıcı olan, negatif romatoid faktör ve pitting ödem ile karakterize nadir bir hastalıktır. Eklem erozyonu yapmaması ve düşük doz steroide iyi yanıt vermesi diğer özellikleridir. Bu çalışma, RSPE sendromu özelliklerini ve eşlik eden durumları değerlendirmeyi ve literature katkıda bulunmayı amaçlamaktadır. Gereç ve Yöntem: Bu tanımlayıcı vaka bazlı retrospektif çalışma Romatoloji kliniğinde Ocak 2019- Ekim 2020 tarihleri arasında yapıldı. 18 yaş üstü on beş RS3PE hastası çalışmaya dahil edildi. Bulgular: RS3PE tanısı konulan 15 hastanın yedisi kadın ve sekizi erkekti. Tekrarlayan polikondrit, skleroderma, seropozitif romatoid artrit, gut, önemi bilinmeyen monoklonal gamopati ve primer miyelofibroz komorbid bozuklukları mevcuttu. Hematolojik malignitesi olanlar ve tekrarlayan polikondrit tanısı konanlar dışındaki hastaların çoğu steroid tedavisine hızla yanıt verdi ve nüks gözlenmedi. Sonuç: RS3PE sendromu, heterojen bir hastalıktır. Neoplazi, ilaçlar ve çeşitli romatizmal hastalıklar ile ilişkili olabilmekte ayrıca paraneoplastik romatizmal hastalik olabileceği düşünülmektedir. Bu sendrom nadir görülmesi nedeniyle klinisyenlerin gözünden kaçabilir ve bu yüzden klinisyenler arasında hastalık açısından farkındalığını arttırmak esastır., Objective: Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare disorder characterized by pitting edema, the onset of acute polyarthritis, and negative rheumatoid factor. The absence of joint erosion and good long-term response to low-dose steroids are its other characteristics. The present study aimed to evaluate the characteristics and accompanying conditions of RS3PE for contributing to the literature on the issue. Material and method: This is cross-sectional descriptive study was carried out in a rheumatology clinic between January 2019- October 2020. Fifteen patients over 18 diagnosed with RS3PE were included in the study. Results: Of 15 patients diagnosed with RS3PE, seven were female, and eight male. The following comorbid disorders were present: relapsing polychondritis, scleroderma, seropositive rheumatoid arthritis, gout, monoclonal gammopathy of unknown significance, and primary myelofibrosis. Most patients other than the ones who have hematological malignities and those diagnosed with relapsing polychondritis responded rapidly to steroids, and no recurrence occurred. Conclusion: RS3PE may be associated with neoplasia, drugs, and various rheumatismal conditions, suggesting that it may be heterogeneous and considered a paraneoplastic rheumatic disease. The rare occurrence of this syndrome leads clinicians to miss it commonly. It is essential to increase awareness of this entity among clinicians.

Aim: Vitamin D deficiency is highly prevalent among children worldwide. This study aims to assess the frequency of vitamin D deficiency in children and how it varies according to gender, age, and season in Istanbul. Materials and methods: The study included 3096 children aged 0-18 admitted to the pediatric outpatient clinic in Istanbul. The serum 25 hydroxyvitamin D concentration was categorized into four groups: deficiency (100 ng/mL). Descriptive methods, Chi-square, Independent Samples T-Test, ANOVA, and correlation test were used in the statistical analysis of the data. Results: Of children, 52% were girls and 48% boys. The mean serum 25 hydroxyvitamin D concentration was 21.8 ± 15.8 ng/mL, and the frequency of vitamin D deficiency was 53.1%. There was a different distribution of vitamin D status between age groups. Vitamin D deficiency was more common in older children. There was also a significant difference between girls and boys in the frequency of vitamin D deficiency (57.6% versus 48.3%). The mean serum 25 hydroxyvitamin D concentration was significantly lower in winter and spring. A moderate negative correlation was found between age and serum 25 hydroxyvitamin D concentration (correlation coefficient:-0.36). Conclusion: This study showed that female sex, older children, and the winter/spring seasons were significantly associated with a higher frequency of vitamin D deficiency and a lower mean serum 25 hydroxyvitamin D concentration.

BACKGROUND AND AIMS Liver transplantation (LT) in alcohol-associated hepatitis (AH) remains controversial, in part because spontaneous recovery (SR) can occur. There is a paucity of data on SR in patients with severe AH who undergo LT evaluation. The purpose of this study was to determine factors associated with SR and survival in patients with severe AH who undergo LT evaluation. APPROACH AND RESULTS This is a retrospective study of ALD patients with Model for End-Stage Liver Disease (MELD) >25 and

The aim of this study was to explore the associations between the aspartate aminotransferase-to-alanine aminotransferase ratio (AST/ALT) and coronary artery lesions (CALs) among patients with Kawasaki disease (KD). Medical records of KD patients presenting to a single center between January 2019 and December 2020 were retrospectively collected and analyzed. Univariate, multivariable-adjusted analyses, subgroup analyses, restricted cubic spline test, and fitted curves were used to evaluate the associations between AST/ALT and CALs. A total of 831 patients were enrolled, of which 201 (24.2%) had CALs on admission and 21 (2.5%) developed CALs de novo after intravenous immunoglobulin (IVIG). Multivariable-adjusted analyses models revealed that a lower AST/ALT was associated with an increased risk of CALs on admission when AST/ALT was a continuous variable (P = 0.007) and when it was a categorical variable (P for trend = 0.004). Each unit increase in AST/ALT was associated with a 22% lower risk of CALs on admission (odds ratio = 0.78, 95% confidence interval 0.65–0.94). A negative linear relationship was noted between AST/ALT and the risk of CALs on admission in both observed and fitted models. However, such associations were not observed in AST/ALT and CALs de novo after IVIG. None of the variables significantly modified the association between AST/ALT and CALs on admission and CALs de novo after IVIG (P > 0.05). Our findings suggested that AST/ALT was a risk factor of CALs, but was not associated with progressive CALs.

INTRODUCTION. СЗ-glomerulopathy (СЗ-GP) is a spectrum of diseases caused by dysregulation of the alternative complement pathway. The present study was carried out taking into account the limited number of foreign and the absence of original studies in the Russian population.THE AIM. Analysis of clinical and morphological manifestations of СЗ-GP at the time of primary diagnosis.PATIENTS AND METHODS. Retrospective analysis of the etiology, clinical data and morphology of СЗ-GP identified in the period 2006-2021.RESULTS. The study included 60 cases. The average age of patients is 4З±17 years. Nephrotic syndrome was detected in 47 % of patients; in 58 % of cases the estimated glomerular filtration rate was 2. The dominant morphological pattern was membranoproliferative glomerulonephritis (75% cases). In 2 cases, СЗ-GP debuted with clinical and morphological manifestations of the disease of minimal changes, in which the identification of characteristic electron-dense deposits became obvious when performing a second biopsy. 4 cases had at the onset classical signs of complement-mediated thrombotic microangiopathy (atypical hemolytic uremic syndrome) in combination with C3-GP or its subsequent development.Ultrastructural examination was performed in 40 cases. 8 patients (20%) were diagnosed dense deposit disease, 32 patients - C3-glomerulonephritis. Primary C3-GP was detected in 87 % of patients, secondary - in 13 % (monoclonal gammopathies - 10 %, autoimmune diseases - 3 %).Increased level of antibodies to factor H was detected in 2 of 12 patients who underwent this study. Nucleotide variants of unknown clinical significance in complement genes were detected in 2 out of 6 patients during molecular genetic testing.CONCLUSION. C3-GP is a severe variant of glomerular damage with a heterogeneous etiological structure, which requires the use of ultrastructural and molecular diagnostics, as well as clinical analysis and identification of pathogenetic mechanisms to determine approaches to therapy.

Introduction: Targeted therapy and immune checkpoint inhibitors (ICI) have drastically improved outcomes of metastatic non-small cell lung cancer (NSCLC) patients in outpatient settings. Because trials on critically ill patients are improbable, little is known about their efficacy among patients admitted to intensive care units (ICU). Methods: We retrospectively analyzed the clinical outcomes of critically-ill NSCLC patients receiving either ICI or targeted therapy during ICU admission at the MD Anderson Cancer Center from April 2016 to August 2020. We collected data on ICU admission diagnoses, sequential organ failure assessment (SOFA), previous cancer therapies, tumor gene mutations or translocations, and PD-L1 expression. Overall survival (OS) was calculated from the date of drug initiation using the Kaplan-Meier method. Results: Of 9898 ICU admissions, 9 patients with metastatic NSCLC who received either targeted therapy (5) or PD-1 ICI (4) during ICU admission were included. The most common reasons for ICU admissions were tumor visceral crisis (3/9) and sepsis (3/9). The median (range) admission SOFA was 4 (2-11). Six patients were naïve to systemic therapy. Five patients required mechanical ventilation. The median OS was 77 days (95%CI, 36-NA), and 5 patients were discharged alive (all received targeted therapy). The median OS of patients who received ICI was 25.5 days (95%CI, 8-NA) and for those who received targeted therapy was 218 days (95%CI, 77-NA). At 6 and 12 months follow-up, 3 and 2 patients who received targeted therapy were still alive, respectively. Conclusions: Our exploratory findings indicate a possible benefit of targeted therapy but suggest a lack of clinical utility of PD-1 ICI for critically ill metastatic NSCLC patients. Because of the small sample size, further studies are needed to expand on this topic.

R ± DHAX has been traditionally administered to inpatient due to the timing of chemotherapy administration and the perceived need for close monitoring of adverse effects. To administer R ± DHAX outpatient, a multidisciplinary task force created clinical and educational guidelines which were implemented through two phases: pilot and expansion. The pilot program determined the feasibility of transitioning R ± DHAX outpatient at a single infusion site. The expansion phase led to a service-wide implementation across all outpatient sites. A total of 40 patients were included, of which 23 patients completed all cycles, outpatient, 12 transitioned inpatient to outpatient administration, and 5 transitioned outpatient to inpatient administration. The success rate of outpatient R ± DHAX administration was 90% (36 patients successfully completed outpatient administration/40 total patients). No cytarabine-related cerebellar or ophthalmic toxicity was reported. Outpatient R ± DHAX saved 192 hospital days. R ± DHAX could be successfully administered outpatient with minimal safety concerns and reduced hospital bed utilization.

PURPOSE The double purse-string telescoped pancreaticogastrostomy (PG) technique has been suggested as an alternative approach to reduce the risk of postoperative pancreatic fistula (POPF). Its efficacity in high-risk situations has not yet been explored. This study compared the incidence of clinically relevant POPF (CR-POPF) between patients with high-risk anastomosis undergoing PG and those undergoing pancreaticojejunostomy (PJ). METHODS From 2013 to 2019, 198 consecutive patients with high-risk anastomosis, an updated alternative fistula risk score > 20%, and who underwent pancreatoduodenectomy with the PJ (165) or PG (33) technique were included. Optimal mitigation strategy (external stenting/octreotide omission) was applied for all patients. The primary endpoint was the incidence of CR-POPF. RESULTS The mean ua-FRS was 33%. CR-POPF (grade B/C) was found in 42 patients (21%) and postoperative hemorrhage in 30 (15%); the mortality rate was 4%. CR-POPF rates were comparable between the PJ (19%) and PG (33%) groups (P = 0.062). The PG group had a higher rate of POPF grade C (24% vs. 10%; P = 0.036), longer operative time (P = 0.019), and a higher transfusion rate (P

Giriş: Yeni koronavirüs hastalığı 2019 (COVID-19) dünya çapında bir pandemiye neden oldu. COVID-19'un çok çeşitli klinik belirtileri vardır. Eşlik eden hastalıkları olan 65 yaş üstü hastalar ciddi hastalıklara karşı daha savunmasızdır. Bu çalışma, hastalığın klinik, laboratuvar ve görüntüleme özelliklerini karakterize etmeyi amaçlamaktadır. Yöntemler: Bu çalışma Eskişehir Şehir Hastanesi'nde yapılmıştır. 15 Nisan 2020 ile 15 Haziran 2020 tarihleri arasında doğrulanmış COVID-19 olan 435 hastanın tıbbi kayıtları geriye dönük olarak analiz edildi. Hastalar yaşlarına göre, Introduction: The novel coronavirus disease 2019 (COVID-19) has caused a worldwide pandemic. COVID-19 has a wide range of clinical manifestations. Patients over the age of 65 who have comorbidities are more vulnerable to severe disease. This study aims to characterize the clinical, laboratory, and imaging features of the disease. Methods: This study was conducted in Eskisehir City Hospital, Turkey. Medical records of 435 patients with confirmed COVID-19 between 15 April 2020, and 15 June 2020, were analyzed retrospectively. The patients were divided into three groups according to age

Pediatric autoimmune encephalitis (AE) remains a diagnostic and therapeutic challenge in resource-poor settings. Minimizing delay in diagnosis and appropriate escalation of treatment will help reduce both the short- and long-term neurodisabilities. A retrospective observational study was performed on children consecutively diagnosed with possible AE and then prospectively followed up in a single tertiary care children's hospital in Sri Lanka. Serum and cerebrospinal fluid were tested for neuroglial surface-binding autoantibodies using cell-based assays in majority of these children. Twenty-five children (mean age 7.6 years, standard deviation = 4) were recruited. In these children, presenting symptom was psychiatric in 11 children (44%), seizures in 10 (40%), language regression in 2 (8%), and combination of psychosis and convulsions in 2 (8%). Psychiatric presentations were more common in older (>6 years) compared with young children (p = 0.001), while neurological presentations were more common in children aged ≤6 years (p = 0.001). N-methyl-D-aspartate receptor (NMDAR) antibodies were detected in 9 (45%) and unspecified voltage-gated potassium channel antibodies in 1 (5%) of the 20 tested. All received intravenous steroids and immunoglobulins; 19 (76%) plasma exchange; 7 (29%) rituximab. Complete/substantial improvement at 3 months occurred in 64%. Pediatric Cerebral Performance Category score at last review was 1 (normal function for age) in 43%. Higher proportion of younger children required less intense therapy and had better recovery (56%). Death (8%), incomplete recovery (71%), and relapses (8%) were more in older children. Clinical presentation and disease outcomes were different in children aged

Purpose: As routine health examinations become more common, many patients first diagnosed with glaucoma have advanced glaucoma. We analyzed the routes to diagnosis and the characteristics of patients initially diagnosed with advanced glaucoma.Methods: We retrospectively retrieved the medical records of patients first diagnosed with advanced glaucoma in our tertiary care center. The inclusion criteria were a mean deviation (MD) less than -12 dB on the visual field test, accompanied by structural damage. All patients were classified in terms of unilateral/bilateral disease, the intraocular pressure before medication, and lens status. We divided patients into those with monocular or binocular advanced glaucoma, high- or normal-pressure glaucoma, and those who were pseudophakic or phakic.Results: We included 73 patients of mean age 69.3 years. The visual field test MD was -19.6 dB. In those with binocular advanced glaucoma, incidental ophthalmic examination was the most common means of diagnosis (52.2%). Central-island visual field defects were the most common defects (54.2%). In those with monocular advanced glaucoma, glaucoma-associated symptoms most commonly triggered diagnosis (46.9%). Both superior and inferiorvisual field defects were the most common defects (42.8%). Glaucoma-associated symptoms were present in 68.2 and 22.8% of patients with high- and normal-pressure glaucoma, respectively. Central-island visual field defects were present in 43.6 and 29.4% of those with high- and normal-pressure glaucoma, respectively.Conclusions: We analyzed the routes to diagnosis and the clinical characteristics of patients with advanced glaucoma. In those with binocular disease, glaucoma was most commonly diagnosed on incidental ophthalmic examination. Central-island visual field defects were the most common defects in patients with binocular and high-pressure glaucoma, and the pseudophakic group. A multi-center longitudinal study on risk factors for delayed glaucoma diagnosis is needed.

Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction. The study aimed to evaluate the diagnostic utility of ultrasound in confirming the diagnosis of BCS and to provide an overview of the clinical picture.In this retrospective single-center study, patients with an initial diagnosis of BCS were included. The files were analyzed concerning the ultrasound images and compared to computed tomography (CT) and magnetic resonance imaging (MRI). Main clinical signs of BCS were collected.Data of 25 patients were analyzed. Doppler sonography showed the highest sensitivity (78.9%) with the highest specificity 97.4 (%) in confirming the correct diagnosis of BCS. Main imaging signs were obstruction in the hepatic veins (68.0%, 17/25 thrombotic), collaterals (91.7%, 11/12 intrahepatic), inhomogeneous liver parenchyma (7/21), and a hypertrophied lobus caudatus (18/21) (p0.01). All imaging signs could be detected with sonography. Hypertrophied lobus caudatus was seen exclusively in BCS. Furthermore, portal hypertension (9/25), liver cirrhosis (9/25), and ascites (19/25) can be diagnosed as non-specific signs of BCS (p0.01).The main clinical findings were elevated γ-GT levels in the laboratory (92.0%, 23/25, p0.01) and esophageal varices in endoscopy (12/25 p0.01). An association with myeloproliferative neoplasia (MPN) was frequently seen (10/25) (p0.01).The present study demonstrates that sonography is an appropriate tool for the diagnosis of BCS and should be used as the first imaging procedure.Die Diagnostik mittels Ultraschalls eignet sich besonders gut für Organe im Oberbauch wie z.B. die Leber. Das Budd-Chiari-Syndrom (BCS) ist eine seltene Erkrankung, bei der der venöse Abfluss der Lebervenen behindert ist.Ziel dieser retrospektiven Single-Center-Studie ist es, den diagnostischen Nutzen und die Leistungsfähigkeit des Ultraschalls bei der Diagnosesicherung des BCS zu evaluieren.Insgesamt 25 BCS-Fälle mit Erstdiagnose im Universitätskrankenhaus Tübingen (UKT) aus dem Zeitraum 2005–2018 wurden in diese Studie eingeschlossen. Die Akten wurden auf sonografische (bildgebende) und klinische Zeichen des BCS und im Vergleich von Ultraschall (B-Bild-farbkodierte Dopplersonografie) und Computertomografie (CT) und Magnetresonanztomografie (MRT) analysiert.Als Zeichen in der Bildgebung (Sonografie/CT/MRT) wurden Obstruktionen in den Lebervenen (68,0%, 17/25 thrombotisch), Kollateralen (zu 91,7%, 11/12 intrahepatisch), eine inhomogene Leber (7/21) und ein hypertrophierter Lobus caudatus (18/21) beobachtet (p0,01). Portale Hypertension (9/25), Leberzirrhose (9/25) und Aszites (19/25 wurden als unspezifische Zeichen eines BCS gefunden (p0,01). Im Labor fanden sich erhöhte γ-GT-Werte (92,0%, 23/25, p0,01) und in der Endoskopie wurden Ösophagusvarizen nachgewiesen (12/25 p0,01). Ein Zusammenhang mit der Myeloproliferativen Neoplasie (MPN) ist typisch für BCS (10/25) (p0,01). Die Sonografie zeigte die höchste Sensitivität 78,9% bei der höchsten Spezifität 97,4%.Die Sonografie ist ein geeignetes Instrument für die Diagnose des BCS. Sie sollte als erstes bildgebendes Verfahren eingesetzt werden. Alle in der Bildgebung auftretenden Zeichen können mit der Sonografie erkannt werden. Der kontrastmittelverstärkte Ultraschall (CEUS) könnte die Sensitivitäten und Spezifitäten erhöhen und möglicherweise eine weitere Bildgebung überflüssig machen.

Introduction: Evidence of whether to use hemiarthroplasty (HA) or total hip arthroplasty for displaced femoral neck fractures (FNF) is still widely debated, especially when taking ambulatory status, age, and patient cognitive status into account. The current study aims to report the rates of dislocations, revisions and other complications for primary cemented HA in patients with displaced FNF. Materials and methods: Single-center retrospective follow-up study of an unselected historic cohort. 743 consecutive hips (551 W and 192 M) at mean (SD) age of 83.6 (8.4) years received primary cemented HA for displaced FNF by posterolateral surgical approach between January 2012 and December 2019. Patient files and radiographs were evaluated for dislocations, revisions, and other complications until death or end of the follow-up period, and the educational level of the surgeon was noted. Results: During a mean (SD) follow-up period of 2.7 (2.2) years, there were 6.1% (n = 45) dislocations, in which 82% (first dislocation) appeared within the first 30 postoperative days, and 51% (n = 23) of the dislocations requiring subsequent surgery. At the time of the last available follow-up, 57% (n = 421) of the patients were dead. A non-dislocation related revision was needed in 3.4% (n = 25) of the patients [in which infection accounted for 40% (n = 10) and traumatic periprosthetic fracture for 32% (n = 8)]. Thirty-day mortality was 9.2% and 1-year mortality 25.8%. There were no differences in patient's age, gender, or educational level of the surgeon between the dislocation and the no dislocation groups. Patients aged < 70 years presented with a higher dislocation rate (p < 0.001) than the patients aged > 70 years. Conclusion: Primary HA presents a safe and robust approach with acceptable complication rates in a genuine unselected cohort of displaced FNF, particularly for patients aged > 70.

OBJECTIVES There is no consensus in the current literature on the relevance of serum bicarbonate levels, cutoff benchmarks and the management of dehydration; therefore, this study aims to explore whether an association can be established between initial serum bicarbonate levels and the subsequent management of children between the ages of 0 to 36 months presenting to the emergency department (ED) with dehydration. METHODS The study is a single center, retrospective review of 335 charts of children between 0 and 36 months of age presenting to the ED of an urban academic tertiary hospital between June 2014 and June 2016 with a medical history suggestive of dehydration and documented serum bicarbonate levels during their visits. RESULTS A total of 310 charts were analyzed. No significant difference was found between mean serum bicarbonate levels of admitted and discharged patients (18.82 mmol/L vs 18.75 mmol/L; P = 0.89). Children with serum bicarbonate levels below 15 mmol/L were significantly more likely to receive a fluid bolus (P = 0.00) in the ED but neither the length of stay in the ED (P = 0.07) nor in the hospital (P = 0.41) was affected. Bounce backs within 7 days of discharge were not associated with serum bicarbonate levels at first presentation, but rather with a shorter duration of diarrhea (P = 0.013). CONCLUSIONS Initial serum bicarbonate level of dehydrated children does not appear to be associated with the severity of dehydration, vomiting, diarrhea and the patients' management in the ED or the hospital. Initial serum bicarbonate is associated with the decision to administer fluid boluses and potential bounce back.

BACKGROUND Chemotherapy-induced oral mucositis can result in severe pain. Intravenous (IV) opioids are recommended, but management protocols vary. We systematically reviewed studies reporting IV opioid use for pain related to chemotherapy-induced severe oral mucositis in children and conducted a large single-center case series. METHODS Ovid MEDLINE, PubMed, and Cochrane databases were searched for studies reporting IV opioid duration and/or dose requirements for severe mucositis. Secondly, our pain service database was interrogated to describe episodes of opioid administration by patient- or nurse-controlled analgesia (PCA/NCA) for children with mucositis and cancer treatment-related pain. RESULTS Seventeen studies (six randomized trials, two prospective observational, three retrospective cohort, six retrospective case series) included IV opioid in 618 patients (age 0.3-22.3 years), but reported parameters varied. Mucositis severity and chemotherapy indication influenced IV opioid requirements, with duration ranging from 3 to 68 days and variable dose trajectories (hourly morphine or equivalent 0-97 mcg/kg/h). Our 7-year series included PCA/NCA for 364 episodes of severe mucositis (302 patients; age 0.12-17.2 years). Duration ranged from 1 to 107 days and dose requirements in the first 3 days from 1 to 110 mcg/kg/h morphine. Longer PCA/NCA duration was associated with: higher initial morphine requirements (ρ = 0.46 [95% CI 0.35, 0.57]); subsequent increased pain and need for ketamine co-analgesia (118/364 episodes with opioid/ketamine 13.9 [9.8-22.2] days vs opioid alone 6.0 [3.9-10.8] days; median [IQR]); but not with age or sex. CONCLUSIONS Management of severe mucositis pain can require prolonged IV opioid therapy. Individual and treatment-related variability in analgesic requirements highlight the need for regular review, titration, and management by specialist services.

The thyroid gland normally presents as a hyperdense organ on noncontrast computed tomography (CT) in adults. The correlation between thyroid gland CT density and its functional status has been studied; however, little is known regarding its density in children with normal thyroid functions. We aimed to assess the correlation between thyroid gland CT density and age in children with normal thyroid functions.From April 2017 to March 2021, we enrolled 74 patients who had normal thyroid functions within 1 month before or after undergoing neck or cervical spine CT for trauma, neck masses, congenital diseases, and airway stenosis. Their CT images were retrospectively analyzed by 2 independent radiologists. Based on age, patients were divided into 4 groups: infant, preschool-aged, school-aged, and adolescence groups.Patients with thyroid gland hypodensity in the infant group (70%, 14 of 20) were significantly more numerous than those in preschool-aged (25%, 4 of 16), school-aged (20%, 5 of 25), and adolescence (15.4%, 2 of 13) groups (P = 0.007, 0.001, and 0.002, respectively, Fisher exact test). The mean CT density of the thyroid gland was also lower in the infant group compared with the densities in other age groups. There was a weak positive correlation between thyroid CT density and age (r = 0.264, P = 0.023, linear regression analysis).Thyroid CT density is related to age in children. The thyroid gland normally has a low density on noncontrast CT in most infants with normal thyroid function.

Relapsed/refractory Primary Central Nervous System Lymphoma (R/R PCNSL) has a poor prognosis with no established preferred treatment. We report the efficacy and toxicity of a combination chemotherapy regimen: methotrexate, carmustine, etoposide, and prednisone with or without rituximab (RMBVP). This retrospective study included thirty patients who received a median of two 28-day cycles (0.5-5). The median age was 66 years (23-81); median KPS was 70 (30-90); 14 (46.7%) were women. Patients received a median of 2 prior lines of therapy and all received prior methotrexate. Of 29 evaluable patients, the overall response rate was 73.3% (n = 22). Median progression-free survival (PFS) was 15.6 months. Patients who recurred or progressed

OBJECTIVE Initial posaconazole dosing regimens in children often do not achieve target concentrations, and data continue to support the need for higher initial dosing regimens. The objective of this study is to contribute to the current data regarding suboptimal posaconazole dosing in pediatric patients by retrospectively observing dosing strategies and subsequent drug concentrations. METHODS This study was conducted at a single institution in 27 patients aged 1 to 21 years. Patients who were initiated on any formulation of posaconazole for prophylaxis or treatment while admitted to the hospital were included. The primary outcome was to determine the percentage of pediatric patients who achieved the targeted trough concentration using their initial posaconazole dosing regimen. Secondary outcomes included percentage of patients who experienced a breakthrough invasive fungal infection (IFI), percentage of patients with elevated liver function tests (LFTs), and discontinuation for any reason. RESULTS There were 15 patients (55.5%) who reached desired trough serum concentration after the initial dosing regimen. The number of dose modifications to achieve the desired trough ranged from 1 to 3. Most patients received delayed-release tablets (n = 17), and the average doses for reaching prophylactic and treatment trough concentrations were 6.1 mg/kg/day and 11 mg/kg/day, respectively. There were 2 patients (7.4%) who experienced breakthrough IFI. Overall, 5 patients developed elevated LFTs and 7 patients discontinued treatment early. CONCLUSIONS The results describe a single population of pediatric patients, of whom 55% were able to achieve target trough concentrations of posaconazole with the initial dosing strategy used.

OBJECTIVE To evaluate the impact of obesity on etanercept (ETN) drug exposure in children with juvenile idiopathic arthritis (JIA). METHODS We conducted a pilot, cross-sectional, observational study in a real-world cohort of children with JIA receiving ETN as standard of care from a single center. We analyzed the relationship between body size and ETN plasma concentrations, adjusting for dosage. Body size was analyzed as a continuous measure using weight and body mass index (BMI) percentiles and categorically using BMI percentile classifications according to the CDC guidelines. RESULTS We enrolled a total of 29 children. Each child provided one plasma sample for ETN concentration measurement, and all participants were receiving subcutaneous ETN dosed weekly. We observed that the ETN concentration normalized for dose decreased significantly as a function of weight (p = 0.004) and BMI percentile (p = 0.04). Similarly, we observed a progressive decline in mean and median dose-normalized concentrations across higher body size categories. Because of reaching maximum ETN dosage (50 mg), 7 of 8 children (87.5%) with obesity received a weight-based dosage < 0.8 mg/kg/dose. CONCLUSIONS We found that higher body weight and BMI percentile are significantly and negatively associated with ETN drug serum concentration, accounting for differences in dosing. Our data suggest that children who are obese may be routinely under-dosed using current dosing strategies. Inadequate dosing may increase the risk for therapeutic failure and long-term morbidity in a developing child. As a result, characterizing adequate drug exposure in children of all sizes is an important step toward precision dosing.

In patients with bcr-abl-negative myeloproliferative neoplasms (MPN), concerns are often raised about the use of anticoagulants because of an increased bleeding risk. However, there are few MPN studies focusing on bleeding. To investigate bleeding complications in MPN, we report our retrospective, single-center study of 829 patients with a median follow-up of 5.5years (range: 0.1-35.6). A first bleeding event occurred in 143 of 829 patients (17.2%), corresponding to an incidence rate of 2.29% per patient/year. During the follow-up period, one out of 829 patients (0.1%) died due to bleeding. Regarding anticoagulation, most bleeding occurred in patients on antiplatelet therapies (60.1%), followed by patients on anticoagulation therapies (20.3%) and patients not on anticoagulation (19.6%). In multivariate analysis, administration of antiplatelet (HR 2.31 [1.43, 3.71]) and anticoagulation therapies (HR 4.06 [2.32, 7.09]), but not age, gender or mutation status, was associated with an increased bleeding risk. Comparing the "probability of bleeding-free survival" between the MPN subtypes, no significant difference was observed (p=0.91, log-rank test). Our retrospective study shows that antiplatelet and anticoagulation therapies significantly increase the risk of bleeding in MPN patients without affecting mortality. However, there is no reason to refrain from guideline-conform primary or secondary anticoagulation in MPN patients. © 2021 The Authors. European Journal of Haematology published by John Wiley & Sons Ltd.

Endoscopic mucosal resection (EMR) and radiofrequency ablation (RFA) for Barrett’s esophagus (BE)-related high-grade dysplasia (HGD) and early esophageal adenocarcinoma (EAC) are considered effective treatments for eradication of BE. Little is known about the impact of achieving complete eradication of intestinal metaplasia (CE-IM) following the complete eradication of neoplasia (CE-N), specifically if CE-IM reduces the risk of recurrent dysplasia. Retrospective cohort study of consecutive patients with BE and HGD or intramucosal cancer (IMC)-treated endoscopically at a tertiary referral center between 2001 and 2019. Association between CE-IM and recurrent dysplasia after CE-N was evaluated. A total of 433 patients treated with EMR and/or RFA were included. Of these, 381 (88%) achieved CE-N, of which 345 (80%) had adequate follow-up for inclusion in the analysis. A total of 266 (77%) patients achieved CE-IM; with a median follow-up since initial treatment for HGD/IMC of 45.9 months (IQR 25.9, 93.1); 20 patients (5.8%) had recurrent dysplasia after achieving CE-N. Kaplan Meier survival curves revealed that time free of recurrence in those who achieved CE-IM was significantly higher (p = 0.002). In the multivariable analysis, CE-IM was associated with a significant lower hazard of recurrence (HR 0.2, 95% CI 0.1, 0.6), whereas the number of endoscopic treatments to achieve CE-N was associated with a significant higher hazard of recurrence (HR 1.1, 95% CI 1.0, 1.2). Achieving CE-IM following CE-N reduces the risk of recurrent dysplasia and should be considered a treatment target among patients with BE undergoing endoscopic therapies for HGD or EAC.