Single-center Cohort Of Patients Research Articles

Preoperative ultra-hypofractionated radiotherapy for soft-tissue sarcomas: low rate of wound complications

Background: Normofractionated pre-operative radiotherapy (nRT) with 50 Gy applied in 25 fractions represents the most widely used radiotherapy (RT) regimen in combined local treatment of soft tissue sarcomas (STS). STS are characterized by a low α/β ratio of 4-5 Gy, which may translate into a higher sensitivity for hypofractionation. Increasing data from cohorts and phase II trials on ultra-hypofractionated RT (uhRT) regimens is available. We prospectively assessed our preoperative uhRT sarcoma patient cohort with a focus on short term wound complications (WC). Methods: This is a prospective registry analysis of a single center patient cohort, treated from 03.2020-10.2023 with uhRT (25 Gy in 5 fractions in 1 week). The same radiation oncologists (GS/CG) and surgeon (BF) performed the treatment (61/61 and 58/60), as well as the same reference pathologist (BB) confirmed all histopathological diagnoses. WC (according to CAN-NCIC-SR2 trial) and intermediate local control (LC) rates were assessed and compared with outcome data of a previously published cohort of 67 extremity/trunk sarcoma patients treated with nRT by the same authors (7% WC, 98% LC at 3 years). Results: After a mean/median follow-up (FU) of 19/19 months (range 0-46), LC at 1.5y was 94%. Surgery was performed at a mean/median of 20/16 days (range 4-60) after uhRT completion. WC were observed in 7/60 operated patients (12%), and in 5/51 (10%) extremity/trunk lesions. Early tolerance was excellent, limited to G0-1, even in 3 patients with prior RT to the same region. Clear resection margins were achieved in 55/60 patients (92%). Pathologic necrosis of ≥95% was reported in 5%, 75% achieved less than <50% necrosis. Conclusion: These results show low rates of WC and high LC for uhRT and are comparable to our previously published nRT data. This study supports the routine use of preoperative uhRT for STS.

Limited Generalizability of Retrospective Single-Center Cohort Study in Comparison to Multicenter Cohort Study on Prognosis of Hepatocellular Carcinoma.

We aimed to evaluate the generalizability of retrospective single-center cohort studies on prognosis of hepatocellular carcinoma (HCC) by comparing overall survival (OS) after various treatments between a nationwide multicenter cohort and a single-center cohort of HCC patients. Patients newly diagnosed with HCC between January 2008 and December 2018 were analyzed using data from the Korean Primary Liver Cancer Registry (multicenter cohort, n=16,443), and the Asan Medical Center HCC registry (single-center cohort, n=15,655). The primary outcome, OS after initial treatment, was compared between the two cohorts for both the entire population and for subcohorts with Child-Pugh A liver function (n=2797 and n=5151, respectively) treated according to the Barcelona-Clinic-Liver-Cancer (BCLC) strategy, using Log rank test and Cox proportional hazard models. Patients of BCLC stages 0 and A (59.3% vs 35.2%) and patients who received curative treatment (42.1% vs 32.1%) were more frequently observed in the single-center cohort (Ps<0.001). Multivariable analysis revealed significant differences between the two cohorts in OS according to type of treatment: the multicenter cohort was associated with higher risk of mortality among patients who received curative (adjusted hazard ratio [95% confidence interval], 1.48 [1.39-1.59]) and non-curative (1.22 [1.17-1.27]) treatments, whereas the risk was lower in patients treated with systemic therapy (0.83 [0.74-0.92]) and best supportive care (0.85 [0.79-0.91]). Subcohort analysis also demonstrated significantly different OS between the two cohorts, with a higher risk of mortality in multicenter cohort patients who received chemoembolization (1.72 [1.48-2.00]) and ablation (1.44 [1.08-1.92]). Comparisons of single-center and multicenter cohorts of HCC patients revealed significant differences in OS according to treatment modality after adjustment for prognostic variables. Therefore, the results of retrospective single-center cohort studies of HCC treatments may not be generalizable to real-world practice.

Clinical characteristics and genetic substrate of lone atrial fibrillation: a single center experience

Abstract Background Juvenile-onset lone atrial fibrillation (LAF) may be the initial manifestation of a concealed channelopathy or cardiomyopathy (CMP) or it may be due to an atrial specific genetic susceptibility. Clinical characteristics, genetic background and proper management are still largely unknown. Purpose Aim of the present study is to describe the clinical and genetic characteristics of patients with juvenile-onset LAF. Methods We enrolled patients referred to our center for ablation between 1/2018 and 11/2023 with a first diagnosis of LAF before the age of 60, a structurally normal heart (absence of known CMP, ischemic, valvular, congenital heart disease) and no overt channelopathy. A concealed Brugada syndrome was systematically excluded. Genetic testing was performed using Next Genome Sequencing and a dedicated gene panel. Results 89 patients were included (84% male, LAF onset 43 ± 8 years, BMI 25 ± 7 kg/m2): 32% had a first-degree family history of AF (including 15% below 60 years), 18% of sudden death, 8% of cardiac conduction disorders and 2% of CMP. Cardiovascular risk factors included arterial hypertension in 24%, smoking in 47% and obstructive sleep apnea syndrome in 4%; 20% were competitive athletes. Most patients (89%) presented with paroxysmal AF and 92% were symptomatic (mainly palpitations). At presentation LVEF was 61±5% and LAVI 35±14 ml/m2. Median follow-up time after the first AF episode was 6 years (IQR 4-10). Most patients (81, 91%) underwent at least one transcatheter ablation procedure after failed anti-arrhythmic drug (AAD) therapy (mostly IC). The first procedure (73% radiofrequency, 27% cryoablation) included pulmonary veins isolation (PVI) in all and additional lesions in 5%; the second procedure (n=18, 22% of the ablation group) included PV re-isolation in all (mostly due to multiple vein reconnections) and additional lesions in 17%; the third procedure (n=8, 10% of the ablation group) included PV re-isolation in 25% and additional lesions in 75%. The remaining 8 patients were treated with AAD only (mostly IC). By the last follow-up, 7% of patients in the ablation group and 25% in the AAD group had documented recurrences (even though 50% of those who received ablation were still on AAD). Among the 35 patients subjected to genetic investigation, 10 have available results: 30% tested positive for C4-C5 variants (2 for KCNQ1, 1 for TTN), 50% for C3 variants (RYR2, ABCC9, LMNA, KCNH2 and RMB20) and 20% were negative. Conclusions In the present single center cohort of patients with juvenile-onset LAF, a positive family history was found in almost one third of patients. Despite prompt diagnosis and treatment (including &amp;gt;1 ablation in 20% of patients), recurrences rate is not trivial. The yield of genetic test so far is high, supporting its use in this population.

Clinical findings and outcomes of Brugada syndrome in women

Abstract Introduction Brugada syndrome (BrS) is a genetic disorder characterized by typical ECG changes and association with spontaneous ventricular tachyarrhythmia (VTA) and sudden death in young patients. BrS has been widely established to be particularly prevalent in men, however less information exists regarding female patients and their long-term risk. Objectives The purpose of this study was to investigate the clinical characteristics and long-term prognosis of women with BrS. Methods This study includes a retrospective analysis of a single-center cohort of patients followed and observed for relevant clinical outcomes. The data collected included demographic data, severity of symptoms (e.g. syncope, VTA), cardioverter-defibrillator logs and occurrence of sudden cardiac death. Results 317 patients were diagnosed with BrS between 1998 and 2023. Among those, 112 (36%) were female. At presentation, 5 patients (4%) were symptomatic with history of survived cardiac arrest and/or documented VTA. 29 (26%) had history of unexplained syncope and 78 (70%) were asymptomatic. The average age at diagnosis was 42.1±15.1 yrs. as opposed to 40.1±14.2 yrs. in men. Females presented less frequently with a type 1 ECG pattern (5% vs 21%; p&amp;lt;0.01), had a trend to a higher rate of family history of sudden cardiac death (31% vs 25%; p=n.s.), and had less sustained VTA during programmed ventricular stimulation (4% vs 19%; p&amp;lt;0.01). Genetic testing was performed in 89 females (79% of the sample) and pathogenic variants were present in 36 (40%) women. An implantable cardioverter defibrillator was inserted in 24 (31%) females for primary prevention. During mean follow-up of 138±72 months, 11 women had cardiovascular events (syncope, VT/VF or death). Syncope was most common and occurred in 8 cases. No VTA were documented in any female patient. In the group of ICD patients, 8 shocks have occurred. None of these were deemed appropriate by medical controls. There were 3 deaths. Two were considered non-arrhythmic due to pulmonary embolism and breast carcinoma. One ICD patient died suddenly during sleep. Unfortunately, no device interrogation data was available. Conclusion Women typically present less symptomatic and demonstrate a reduced incidence of life-threatening arrhythmias during follow-up , often experiencing a frequent occurrence of inappropriate implantable defibrillator therapy. The less frequent prevalence of the BrS ECG pattern in women adds complexity to the diagnostic process. Further studies are still needed to elucidate gender differences for risk stratification of BrS.

Comprehensive approach and real-life patient registry: exploring management and patient outcomes in dealing with septal complications during left bundle branch area pacing

Abstract Background Left bundle branch area pacing (LBBAP) aims to capture the left bundle branch (LBB) through a transventricular septal approach. Pacing this left-sided conduction system requires deep septal lead positioning due to the subendocardial course of the LBB at the left-sided septum. Such deep septal lead deployment might cause iatrogenic septal complications and collateral damage to septal vasculature. Purpose This study aims to explore both the immediate and medium-term clinical results of septal complications during LBBAP in a large single-center patient cohort, while also entailing the management strategies employed to address these issues. Methods The LBBAP implant procedure was conducted using a lumen-less (LLL) or a stylet-driven pacing lead (SDL) and a dedicated delivery sheath. Septal complications encompassed septal perforations into the left ventricular cavity, septal coronary artery or venous fistulas (SCAF/SCVF) and septal hematomas, which were identified using fluoroscopy, echocardiography and electrocardiographic criteria. Enrollment included adult patients who required permanent pacing for bradycardia or heart failure indications in whom LBBAP was attempted between November 2019 and October 2023. Results The study enrolled 508 patients, with a mean age of 71±16 years and 60% male. Successful LBBAP was achieved in 471 (93%) patients. Septal complications during implant occurred in 24/508 patients (5%), including 19 cases of septal perforation (4%), 4 cases of SCAF (0.8%) and 1 case of SCVF (0.2%). All cases of septal perforation were identified during the implantation procedure and were effectively managed by lead retraction and septal repositioning, eventually resulting in successful LBBAP. With regard to SCAF and SCVF, all but one were detected via septography during implantation and were addressed by lead repositioning. One case of SCAF was detected the day after implant on post-procedural echocardiography and was conservatively managed without lead repositioning. No ventricular septal defects were observed on echocardiography, either immediately or during follow-up (mean follow-up period of 25±12 months). Septal kinetics remained unaffected on follow-up echocardiography (mean follow-up 23±9 months), and all SCAF cases healed spontaneously. Additionally, no septal hematomas were observed. Importantly, no late septal perforations occurred during the follow-up period. Furthermore, no cardiac ischemia or thoracic discomfort were reported among any of these patients. Conclusions The findings of this study underscore the safety of deep septal lead deployment in LBBAP, and highlight that LBBAP provides near physiologic pacing with a high success rate and a low incidence of septal complications. The majority of these complications are promptly detected during the implant procedure and often remain asymptomatic. Furthermore, they exhibit spontaneous healing without enduring persistent septal harm throughout the follow-up period.

Clinical Presentation, Diagnosis, and Management of Hyperinsulinemic Hypoglycemia in Adults: A Single-center Experience.

Hyperinsulinemic Hypoglycemia (HH) is a rare condition characterized by inappropriately elevated insulin levels in the presence of low glucose levels. A proper diagnostic framework is fundamental to avoid patients undergoing unnecessary diagnostic and therapeutic invasive procedures. The study aimed to assess the clinical presentation, diagnostic work-up, and treatment of a single-center cohort of patients affected by HH. We conducted a retrospective analysis of data collected from January 2000-2023. Our study included 104 patients: 81 (58% F) affected by insulinoma, 11 (91% F) by autoimmune hypoglycemia, 7 (71% M) by post-gastric surgery hypoglycemia, and 5 (80% F) by factitious hypoglycemia. HH was more frequent in females (63 F vs. 41 M, p-value 0.039). The median age at diagnosis was lower in insulinoma than in the autoimmune group (52.7 vs. 63.7 y, p < 0.001). During the hypoglycemic event, insulin and C-peptide levels were significantly higher in autoimmune hypoglycemia than in insulinoma (insulin 324.6 vs. 36.4 μU/ml, p-value 0.033; C-peptide 14.25 vs. 3.99 ng/ml, p-value 0.003). Specifically, C-peptide levels <9.6 ng/ml and insulin levels <75 μU/ml exhibited 97.3% vs. 93.4% sensitivity and 80% vs. 90% specificity for insulinoma diagnosis, respectively. Regarding insulinoma, the sensitivity of localizing imaging was 88% for Endoscopic Ultrasound (EUS), 86% for Magnetic Resonance Imaging (MRI), 82% for Computed Tomography (CT) scan, 52% for nuclear imaging, and 100% for angiography with the Doppman test. Among insulinoma patients, 79% received surgical treatment while 4% radiofrequency ablation. Symptomatic remission occurred in 100% of cases. We have confirmed insulinoma as the primary cause of HH. The autoimmune form should be suspected when insulin and C-peptide levels are markedly elevated.

Prevalence of Autoimmune Diseases Among Patients With Psoriasis: A Single Tertiary Center Experience.

Background Psoriasis is a common chronicinflammatory skin disease with an autoimmune etiology. Psoriasis has been presumed to be associated with several autoimmune diseases. We sought to determine the prevalence of autoimmune diseases in patients with psoriasis in a large referral tertiary care center. Methods This is a retrospective and cross-sectional chart review of patients with confirmed psoriasis diagnoses in the dermatology clinic of King Abdulaziz Medical City, Riyadh, Saudi Arabia. The electronic charts of patients were individually reviewed for autoimmune diseases such as hypothyroidism, hyperthyroidism, alopecia areata, vitiligo, atopic dermatitis, and inflammatory bowel diseases like Crohn's disease and celiac diseases. Results A total of 839 cases were included, 56.4% of whom were females. Most patients were between 31 and 50 years old (37.1%). The most common autoimmune disease was hypothyroidism (6.8%), seen more in females. The second most common autoimmune disease was alopecia areata (3.6%), followed by atopic dermatitis (2.9%). Rheumatoid arthritis,systemic lupus erythematosus, and inflammatory bowel diseases were uncommon in our cohort. Conclusion In this single-center retrospective cohort of patients with psoriasis, hypothyroidismand alopecia areata were the most commonly encountered autoimmune diseases. Larger, multi-center studies are needed to evaluate the prevalence of autoimmune diseases among patients with psoriasis.

Clinical profile and management of a Spanish single-center retrospective cohort of patients with post-chikungunya associated complications

BackgroundThis study aims to describe post-chikungunya complications chronically developed cases in returning travelers from some epidemic/endemic regions, and the variables that are associated with the progression of acute or subacute cases to the chronic phase. MethodsThis single-center retrospective cohort study included chikungunya fever cases treated at La Paz-Carlos III University Hospital in Madrid, Spain, April 2014 to September 2016, when the chikungunya outbreak in Latin America started through the time of its greatest impact. ResultsThe analysis included 119 cases. Of these, 67.2 % were male, with a median age of 41.0 years [IQR 16.0 to 76.0] years. Only 25.6 % of the patients attended a pre-travel advice consultation. Most patients reported arthralgias, which significantly impacted their daily quality of life (86 %). The mean duration of joint symptoms was 129.4 days, with a median of 90 days [IQR 0 to 715]. Factors found to be associated with chronic arthralgia include female sex, country of infection, age at diagnosis, previous diseases, symptoms during the acute phase, pain in previously injured tendons/joints, acute phase severity, and various laboratory markers such as hemoglobin, hematocrit, total serum bilirubin, and creatinine. Progression to chronic arthralgia significantly increased the need for changes in daily activity. Furthermore, 42.6 % of patients with chronic arthralgia reported recurrence of symptoms once they felt they had disappeared. Targeted treatment regimens led to significant improvements in these patients. ConclusionsThe results of this study underscore the need for: (1) comprehensive pre-travel advice; (2) effective management of patients in specialized units, alongside early diagnosis and treatment, to prevent trivialization of these viral infections; and (3) the development of interdisciplinary recommendations to assist physicians in treating patients and enhancing outcomes.

PaCO2 Association with Outcomes of Patients with Traumatic Brain Injury at High Altitude: A Prospective Single-Center Cohort Study.

Partial pressure of carbon dioxide (PaCO2) is generally known to influence outcome in patients with traumatic brain injury (TBI) at normal altitudes. Less is known about specific relationships of PaCO2 levels and clinical outcomes at high altitudes. This is a prospective single-center cohort of consecutive patients with TBI admitted to a trauma center located at 2600m above sea level. An unfavorable outcome was defined as a Glasgow Outcome Scale-Extended (GOSE) score < 4 at the 6-month follow-up. We had a total of 81 patients with complete data, 80% (65/81) were men, and the median (interquartile range) age was 36 (25-50) years. Median Glasgow Coma Scale (GCS) score on admission was 9 (6-14); 49% (40/81) of patients had severe TBI (GCS 3-8), 32% (26/81) had moderate TBI (GCS 12-9), and 18% (15/81) had mild TBI (GCS 13-15). The median (interquartile range) Abbreviated Injury Score of the head (AISh) was 3 (2-4). The frequency of an unfavorable outcome (GOSE < 4) was 30% (25/81), the median GOSE was 4 (2-5), and the median 6-month mortality rate was 24% (20/81). Comparison between patients with favorable and unfavorable outcomes revealed that those with unfavorable outcome were older, (median age 49 [30-72] vs. 29 [22-41] years, P < 0.01), had lower admission GCS scores (6 [4-8] vs. 13 [8-15], P < 0.01), had higher AISh scores (4 [4-4] vs. 3 [2-4], P < 0.01), had higher Acute Physiology and Chronic Health disease Classification System II scores (17 [15-23] vs. 10 [6-14], P < 0.01), had higher Charlson scores (0 [0-2] vs. 0 [0-0], P < 0.01), and had higher PaCO2 levels (mean 35 ± 8 vs. 32 ± 6mm Hg, P < 0.01). In a multivariate analysis, age (odds ratio [OR] 1.14, 95% confidence interval [CI] 1.1-1.30, P < 0.01), AISh (OR 4.7, 95% CI 1.55-21.0, P < 0.05), and PaCO2 levels (OR 1.23, 95% CI 1.10-1.53, P < 0.05) were significantly associated with the unfavorable outcomes. When applying the same analysis to the subgroup on mechanical ventilation, AISh (OR 5.4, 95% CI 1.61-28.5, P = 0.017) and PaCO2 levels (OR 1.36, 95% CI 1.13-1.78, P = 0.015) remained significantly associated with the unfavorable outcome. Higher PaCO2 levels are associated with an unfavorable outcome in ventilated patients with TBI. These results underscore the importance of PaCO2 levels in patients with TBI and whether it should be adjusted for populations living at higher altitudes.

Endoscopic Ultrasonography-Guided Fine-Needle Biopsy for Patients with Resectable Pancreatic Malignancies

Clinicians often use endoscopic ultrasonography to survey pancreatic tumors. When endoscopists conduct this examination and find the tumor to be unresectable, a fine-needle biopsy is subsequently performed for tissue confirmation. However, if the tumor is deemed resectable, the necessity of a pre-operative fine-needle biopsy remains debatable. Therefore, we performed a retrospective analysis of a single-center cohort of patients with pancreatic tumors who underwent an endoscopic ultrasound-guided fine-needle biopsy or aspiration (EUS-FNB or FNA) between 2020 and 2022. This study focused on patients diagnosed with resectable malignant pancreatic tumors. The exclusion criteria included individuals diagnosed with benign pancreatic lesions and those with unresectable tumors. A total of 68 patients were enrolled in this study. Histological examination revealed that pancreatic adenocarcinoma was the predominant type of tumor (n = 42, 61.8%), followed by neuroendocrine tumors (n = 22, 32.3%), and metastasis (n = 4, 5.9%). Notably, 17 patients had a history of other cancers, with 23.5% being diagnosed with a metastatic tumor rather than primary pancreatic cancer. Therefore, EUS-FNA/FNB is crucial in patients with a resectable pancreatic tumor and a history of cancer to differentiate between a primary and a metastatic tumor.

Transplant Candidates of 70+ Years Have Superior Survival If Receiving Pre-Emptively a Living Donor Kidney.

Background: The number of kidney transplant recipients over 70 years of age is increasing but detailed data on patient and graft survival in the modern era of immune suppression are few. Methods: A single-center cohort of patients of 70 years and older (n = 349) at time of kidney transplantation from 2010-2020 were followed until January 2023. Results: The median age was 73 years with a median follow-up of 4.3 years. Fifty percent of recipients of a living donor kidney (LDK, n = 143) received their graft pre-emptively. Cumulative death-censored graft survival was excellent in the LDK group and reached 98% at 5 years vs. 85% in the deceased donor kidney (DDK) group. Primary non-function (38%) and rejection (43%) were the major causes of graft loss in the first year after DDK transplantation. Rejection-related graft loss was 4.6% during follow-up. Median recipient survival was superior in the subgroup of pre-emptively transplanted LDK patients compared to non-pre-emptively LDK transplanted patients (11.1 versus 6.2 years). Non-pre-emptively transplanted patients had a significantly increased incidence of infection (HR 3.81, 1.46-9.96) and cardiovascular-related causes of death (HR 3.35, 1.16-9.71). Pre-emptive transplantation was also associated with a significantly improved graft survival in the DDK recipients but this result was confounded by significantly better HLA matching and younger donor age in this group. Conclusions: Pre-emptive LDK transplantation in patients of 70 years or older confers superior graft and recipient survival.

Characterization of orofacial features in sclerodermatous chronic graft-versus-host disease.

Chronic graft-versus-host disease (cGVHD) is a leading cause of morbidity and mortality following allogeneic hematopoietic cell transplantation (alloHCT). The sclerodermatous form of cGVHD can be particularly debilitating; however, orofacial sclerodermatous involvement remains poorly described. To characterize orofacial features of sclerodermatous cGVHD in a single center cohort of patients who underwent alloHCT. Retrospective data were collected from electronic medical records and analyzed descriptively. There were 39 patients who received alloHCT between 1993 and 2017 and developed orofacial sclerodermatous cGVHD. Concomitant cutaneous sclerodermatous cGVHD was common (n = 20, 51%). Orofacial sclerodermatous cGVHD features included fibrous bands of the buccal mucosa (n = 23, 59%), limited mouth opening (n = 19, 54%), perioral fibrosis (n = 8, 21%), and focal gingival recession (n = 4, 10%). Oral mucosal fibrosis was observed at the site of active or resolved chronic lichenoid inflammation in 30 patients, with all but two also presenting with a history of ulcerations. Management included jaw stretching exercises (n = 10; 6 stable/improved), surgery (n = 3; 2 improved), and intralesional corticosteroid injections (n = 2; 2 improved). Orofacial involvement with sclerodermatous cGVHD can present with multiple manifestations including fibrous banding, limited mouth opening, perioral fibrosis, and focal gingival recession. Surgical and non-surgical management strategies may improve clinical function and reduce morbidity.

Association Between Intrapartum Nitrous Oxide for Labor Analgesia and Short-Term Neonatal Outcomes.

To evaluate the association between intrapartum nitrous oxide use and adverse short-term neonatal outcomes. This was a retrospective cohort study of individuals with singleton gestations at 35 or more weeks who attempted labor and delivered at an academic hospital between June 1, 2015, and February 28, 2020. Data were extracted from the electronic medical record using billing and diagnostic codes. Patients were classified based on whether they received no intrapartum analgesia or received nitrous oxide only. Those who received other analgesia types were excluded. The primary outcome was neonatal intensive care unit (NICU) admission. Secondary outcomes included Apgar score less than 7 at 1 minute and 5 minutes, respiratory composite outcome (including meconium aspiration syndrome, neonatal bronchopulmonary disorders, neonatal transient tachypnea, and other neonatal respiratory distress that required NICU admission), hypoglycemia, and hyperbilirubinemia. Univariable and multivariable analyses were used to estimate the association between nitrous oxide exposure intrapartum and the selected outcomes. Of 6,047 included, 4,153 (68.7%) received no analgesia, and 1,894 (31.3%) received nitrous oxide only. In comparison with individuals who received no analgesia, those who received nitrous oxide were more likely to be nulliparous, be of Black racial identity, have noncommercial insurance, and be less likely to deliver by intrapartum cesarean. The reception of nitrous oxide, compared with the reception of no analgesia, was associated with a lower likelihood of NICU admission (6.4% vs 8.1%; adjusted odds ratio [aOR] 0.77, 95% CI, 0.62-0.96) and an increased likelihood of neonatal hyperbilirubinemia (aOR 1.23, 95% CI, 1.08-1.41). Inhaled nitrous oxide exposure, in comparison with the reception of no analgesia, was not associated with the other secondary outcomes, including Apgar score less than 7 at 1 minute (odds ratio [OR] 0.74, 95% CI, 0.50-1.10) or 5 minutes (OR 0.91, 95% CI, 0.32-2.60), respiratory composite outcome (OR 0.91, 95% CI, 0.70-1.17), and hypoglycemia (OR 0.82, 95% CI, 0.64-1.05). In this single-center retrospective cohort of low-risk patients, intrapartum inhaled nitrous oxide, compared with the reception of no analgesia, was associated with a decreased risk for NICU admission but with an increased risk for hyperbilirubinemia; other outcomes did not differ. These findings may be used to counsel patients when considering nitrous oxide for labor analgesia.

Association of autoimmune pancreatitis and intraductal papillary mucinous neoplasm. A retrospective analysis from a tertiary care referral center

BackgroundAutoimmune Pancreatitis (AIP) is a rare chronic inflammatory disease affecting the pancreas. Chronic pancreatic inflammation represents a risk factor for pre-neoplastic conditions such as Intraductal Papillary Mucinous Neoplasia (IPMN). Due to the rarity of AIP, the incidence, and clinical features of IPMN occurring in AIP patients remains unknown. AimsIn the present study we aimed to explore the relationship between AIP and IPMN and to characterize the clinical features and outcomes of IPMN occurring in the context of AIP. MethodsWe retrospectively (2008–2020) analyzed the clinical and radiological records of a large single center cohort of patients with AIP and investigated the prevalence of IPMN. We then compared the clinical, laboratory and radiological characteristics of patients with IPMN and AIP with a cohort of patients with isolated IPMN. ResultsFive hundred and nineteen patients were included in this retrospective study. Sixteen patients had concomitant IPMN and AIP(3%); 61 patients had isolated AIP (12%); 442 patients had isolated IPMN (85%). The prevalence of IPMN in patients with AIP was higher than that observed in the general population (21%vs8-10%). Worrisome Features and High-Risk Stigmata were more frequently observed in IPMN occurring together with AIP compared to isolated IPMN(p < 0.05). Based on radiological features IPMN in the context of AIP was more frequently of main-duct type compared to isolated IPMN(p < 0.05). ConclusionOur data suggest that AIP represents a chronic inflammatory condition that might favor IPMN development with high-risk features. Prolonged surveillance of these patients and longitudinal studies are required to further test the association with AIP and malignant and pre-malignant conditions.

Renal outcome in multiple myeloma patients with cast nephropathy: a retrospective analysis of potential predictive values on clinical and renal outcome

ABSTRACT Objective Cast nephropathy (CN) is the leading cause of acute kidney injury (AKI) in multiple myeloma (MM). Since it is sparsely documented why some patients with CN do achieve a renal response while others do not, we describe a single-center cohort of patients with multiple myeloma and biopsy-confirmed CN to evaluate potential markers of renal response. Methods The data was collected as a retrospective, single-center analysis of CN-patients treated at the Medical University Vienna between 01/01/2004 and 01/01/2022. Baseline parameters and clinical outcome was compared between renal responders and non-responders. Results Among 28 patients with CN, n = 23 were assessed for renal response (14 responders; 9 non-responders). Renal responders were younger (median age: 61 years; 77 years, p = 0.039), showed higher overall survival (153months; 58months, p = 0.044) and achieved hematologic response (≥PR) to first-line therapy (p = 0.029), and complete hematologic response (CR) at any time (p = 0.025) significantly more often. Further, we could show that rapid initiation of anti-myeloma therapy after initial presentation correlated significantly with renal response (median 9 days; 27 days, p = 0.016). Analyses of kidney biopsy specimens revealed that patients with a high IF/TA score showed end stage renal disease (dialysis ≥ 3 months) significantly more often (p = <0.001). Discussion In summary, our data suggests, that a rapid start with systemic hematologic treatment in patients with MM and CN is crucial and achieving an early hematologic response is important for renal recovery. Moreover, achieving a deep hematologic response and subsequent renal recovery improves clinical outcome as reflected by an overall survival benefit.

Abstract TP196: Ischemic Stroke With Large Vessel Occlusion and Systemic Acidosis: Associations With Functional Outcomes and Mortality

Introduction: Systemic acidosis is common in critical conditions, including severe myocardial infarction, renal and liver injuries, and traumatic brain injury. However, the potential connection between ischemic stroke involving large vessel occlusion (LVO) and acidosis has not been extensively explored. With this study we aimed to assess the prevalence of respiratory and metabolic acidosis in ischemic stroke with LVO and their potential links to functional outcomes and mortality. Methods: Retrospective single center cohort of patients with ischemic stroke and LVO status post endovascular thrombectomy. Univariate logistic regression was used to examine the relationship between acidosis on admission and (1) hospital mortality and (2) 90-day modified Rankin Scale (mRS) dichotomized as unfavorable (&gt;3) vs favorable (≤3). In addition, we investigated association with acidosis and symptomatic ICH. Metabolic acidosis was defined by the presence of one of the following: Serum bicarbonate &lt;22 mEq/L, lactic acid &gt;2 mmol/L or pH &lt;7.35. Respiratory acidosis was defined by pCO2 &gt;45 mmHg. Results: There was a total of 346 patients, average age was 68.5 (±15) years, 169 (49%) were females. 168 (49%) patients were acidotic on admission, 4 (1%) had respiratory acidosis, 7 (2%) had mixed acidosis and the remaining 157 (45%) had metabolic acidosis. There was no statistically significant association between systemic acidosis with either hospital mortality (p=0.31]) or 90-day favorable functional outcome (p=0.23]). There was no association between acidosis with symptomatic ICH (p=0.47). Conclusion: In our retrospective cohort study, we observed that approximately half of the patients with stroke and LVO had systemic acidosis. Unlike other critical neurological conditions like traumatic brain injury, we did not identify a substantial correlation between systemic acidosis and either mortality or the functional outcome at the 90-day mark. Given the high prevalence of acidosis in patients with LVO, larger multi-center studies are needed to further investigate its association with the outcomes and mortality.

Lead performance of stylet-driven leads in left bundle branch area pacing: Results from a large single-center cohort and insights from in vitro bench testing

BackgroundLeft bundle branch area pacing (LBBAP) requires deep septal lead deployment for left-sided conduction stimulation. Advancing leads toward deep septal positions might add mechanical stress on these leads. Concerns about lead performance and reliability remain an unanswered question. ObjectiveThe purpose of this study was to analyze lead performance and integrity of stylet-driven pacing leads (SDLs) for LBBAP. MethodsThis study assessed lead fracture rates of SDL in a large single-center cohort of adult LBBAP patients. Fluoroscopic analysis of lead bending angulations at the septal insertion point and in vitro bench testing of lead preconditioning were performed to simulate clinical use conditions. Lead performance was compared between LBBAP and conventional right ventricular apical pacing (RVp) sites. ResultsThe study included 325 LBBAP patients (66% male; age 71±15 years). During median follow-up of 18 months, 2 patients (0.6%) experienced conductor fracture between tip housing and ring electrode, whereas no such fractures occurred with RVp patients (n = 149; P = .22). X-ray analysis revealed that high lead bending angulations occurred in 1.3% of the patients. Accelerated bench testing of excessive preconditioned leads showcased a higher probability of early conductor fracture compared to standard preconditioned leads. ConclusionThe incidence of early conductor failure in LBBAP seems higher than with conventional RVp sites. The most vulnerable lead part seems to be the interelectrode space between the tip housing and ring electrode. Excessive angulation and preconditioning might contribute to early fatigue fracture.

The Impact of Anterior Vertebral Body Tethering on Pulmonary Function.

Retrospective, single-center study. To examine pulmonary function tests (PFTs) in patients undergoing anterior vertebral body tethering (AVBT). The effect of AVBT on pulmonary status remains unclear. The authors examined preoperative and postoperative PFTs following AVBT in a retrospective, single-center cohort of patients. Outcomes were compared using percent predicted values as continuous and categorical variables (using 10% change as significant) and divided into categorical values based on the American Thoracic Society standards. Fifty-eight patients with adolescent idiopathic scoliosis were included, with a mean age of 12.5±1.4 years and a follow-up of 4.2±1.1 years. The mean thoracic curve was 47°±9°, which improved to 21°±12°. At baseline, the mean forced expiratory volume in one second (FEV1%) and forced vital capacity (FVC%) values were 79% and 82%, respectively. Four patients had normal FEV1% (≥100%), 67% had mild restrictive disease (70%-99%) and the rest had worse FEV1%. Mean FEV1 improved from 2.2 to 2.6 L ( P <0.05) and FVC improved from 2.5 to 3.0 L ( P <0.05); however, % predicted values remained unchanged (FEV1%: 79%-80%; FVC%: 82-80%, P >0.05) with mean postoperative PFTs at 37±12 months postoperative. The use of miniopen thoracotomy was not associated with worsening PFTs, but extension of the lowest instrumented vertebra below T12 was correlated with decreasing FEV1% in the bivariate analysis ( P <0.05). Patients with worse preoperative FVC% (80±13% vs. 90±11%, P =0.03) and FEV1% (77±17% vs. 87±12%, P =0.06) also had a greater likelihood of declining postoperative FEV1%. Pulmonary function in most patients undergoing AVBT remained stable (76%) or improved (14%); however, a subset may worsen (10%). Further studies are needed to identify the risk factors for this group, but worse preoperative PFTs and extension below T12 may be risk factors for worsening pulmonary function.

Cytokine signature in convalescent SARS-CoV-2 patients with inflammatory bowel disease receiving vedolizumab

While differential antibody responses SARS-CoV-2 in patients with inflammatory bowel disease (IBD) receiving infliximab and vedolizumab are well-characterized, the immune pathways underlying these differences remain unknown. Prior to COVID-19 vaccine development, we screened 235 patients with IBD receiving biological therapy for antibodies to SARS-CoV-2 and measured serum cytokines. In seropositive patients, we prospectively collected clinical data. We found a cytokine signature in patients receiving vedolizumab who are seropositive compared with seronegative for SARS-CoV-2 antibodies that may be linked to repeated SARS-CoV-2 infections. However, there were no differences between seropositive and seronegative patients receiving infliximab. In this single-center cohort of patients with IBD with anti-SARS-CoV-2 antibodies at the onset of the COVID-19 pandemic, and therefore without influence of vaccination, there is a cytokine signature in patients receiving vedolizumab but not infliximab. These findings lay the groundwork for further studies on immune consequences of viral infection in patients with IBD, which is postulated to evolve from aberrant host-microbe responses.

Chronic Graft-versus-Host Disease-Associated Muscle Cramps: Severity and Response to Immunomodulatory Therapies

Muscle cramps in patients with chronic graft-versus-host disease (cGVHD) are common and associated with impaired quality of life and symptom burden. Muscle cramps are not currently captured in the 2014 National Institutes of Health (NIH) response criteria, and thus characterization and response to immunomodulatory therapies are lacking. The objective of this study was to characterize muscle cramp frequency, duration, and pain level in patients with steroid-refractory cGVHD undergoing extracorporeal photopheresis (ECP). A single-center cohort of patients who underwent ECP for the indication of steroid-refractory cGVHD with muscle cramps at treatment initiation were followed from April 2021 to April 2023. Of 22 patients receiving ECP for cGVHD during the study period, 9 (41%) had muscle cramps at ECP initiation (6 males [66%]; median age, 59 years; range, 25 to 66 years). Seven of these 9 patients (78%) had multiple organs involved, and 7 (78%) had severe disease by the NIH Global Severity scale. Over a median treatment duration of 28 weeks (range, 10 to 48 weeks), 8 patients (89%) had decreased frequency of muscle cramps from a median of 5 episodes per week (range, 3 per day to 2 per week) to a median of <1 episode per week (range, 1 per month to 3 per week). The pain and duration of muscle cramps were not changed meaningfully. The NIH Global Severity score remained unchanged in 6 patients (67%) and was improved in 3 patients (33%). Muscle cramping is a morbid feature of cGVHD that may be sensitive to change with standard immunomodulatory therapies. Muscle cramp frequency should be further validated as a response measure in cGVHD.