Center Experience Research Articles

Systemic Bevacizumab for Recurrent Respiratory Papillomatosis: A Single Institution's Experience.

Medical therapies to limit disease recurrence are critically needed for recurrent respiratory papillomatosis (RRP). Systemic bevacizumab is emerging as an exciting adjuvant therapy toward this end, but uptake has been poor due to the lack of experience and awareness of best prescribing practices. The objective of this study was to describe a single tertiary care academic medical center's experience using systemic bevacizumab for the treatment of RRP. A retrospective review was performed to identify patients with RRP on systemic bevacizumab. Demographic and clinical characteristics, findings on imaging reports, and disease response at all anatomic subsites involved in papilloma were documented. Of the 17 RRP patients on systemic bevacizumab, 9 (52.9%) were male, and 12 (70.6%) were diagnosed with juvenile-onset RRP. The total lifetime number of surgeries was high, with more than half (n = 9; 52.9%) undergoing more than 50 surgeries. Following induction of systemic bevacizumab, a significant reduction in patients with laryngeal (n = 15; 94.1% vs. n = 7; 41.2%, p < 0.001) and tracheal (n = 11; 64.7% vs. n = 5; 29.4%, p = 0.04) RRP was noted. Surgical frequency was significantly lower following systemic bevacizumab (2.5 vs. 0.5 surgeries per year; p < 0.001). The most common complications were new-onset hypertension (n = 4; 23.5%) and proteinuria (n = 5; 29.4%). Systemic bevacizumab is effective in reducing the number of surgeries needed for RRP while exhibiting a relatively safe complication profile. Papillomas in the larynx and trachea are most responsive to systemic bevacizumab, while pulmonary RRP is most likely to exhibit a partial-to-stable response. 4 Laryngoscope, 134:3253-3259, 2024.

Central Line Repair in Pediatric Patients in the Pediatric Emergency Department by Emergency Physicians-A Single-Center Experience.

Central venous catheters are more common with advanced treatments for a variety of conditions. These catheters may need to be repaired after local damage. This has been performed by those more involved with catheter placement such as pediatric surgeons and interventional radiologists. Gastroenterologists who treat many of these patients have also been involved with catheter repair. Repair by pediatric emergency physicians has not been explored. Such repairs by the emergency physician may be time saving and avoid unnecessary admissions. This was a retrospective study of a single center's experience with catheter repair. The performers and outcomes were compared. During the study period, 52 catheter repairs were performed on 7 children. Forty of the repairs were performed by a pediatric emergency physician, 5 by pediatric surgeons, and 5 by interventional radiology. All but 1 repair was successful. Infectious complications were reported in 3 cases. None required catheter removal. The length of stay was significantly shorter for repair by emergency physician. Central line catheter repair can be performed successfully by pediatric emergency physician with minimal complications. A dedicated process of repair tutoring is required and may avoid infectious complications.

Endoscopic Ventricular Lavage in Pediatric Pyogenic Cerebral Ventriculitis Associated with Shunt: Outcomes and Technical Notes

Pyogenic cerebral ventriculitis (PCV) is a rare infectious entity characterized by a potent inflammatory reaction of the ventricular ependyma, which in most cases leads to death. We aim to present the technical note and protocol, based on our center's 10-year experience of the use of endoscopic ventricular lavage in pediatric patients with PCV and ventriculoperitoneal shunt (VPS). For this study, all pediatric patients (<16 years) with VP shunts and PCV who were treated with endoscopic ventricular lavage between January 2012 and January 2022 were included. 34 pediatric patients with ventriculitis were analyzed. The median age was 6 years, with 61.7% being male. Most consultations occurred on Day 2 of symptoms. Fever (38.2%) and altered consciousness (26.5%) were the most common initial symptoms. Early ventriculitis was observed in 67.7% of patients. Pathogen identification in the initial cerebrospinal fluid (CSF) sample was 70.6%, while samples from ventricular peritoneal shunt (VPS) yielded 23.53% and catheter culture 79.4%. Gram-positive bacteria, mainly S.epidermidis (44.1%), was the most commonly isolated agent. Endoscopic ventricular lavage was performed in 73.5% on the second or third day. Reinfection occurred in 23.5%, and 26.5% of patients died. Concordance analysis showed 85.3% agreement between LP and catheter tip samples. Functionality improved, with 55.88% achieving a Lansky score of 90. Early ventriculitis was associated with better Lansky scores. Endoscopic ventricular lavage can be a useful tool in the management of PCV in cases with VP shunts. Our study suggests a higher chance of isolating an infection-causing germ in the catheter tip culture specimen compared to the cerebrospinal fluid (CSF) culture. However, future studies with a larger number of patients, or multicentric studies are required for further analysis.

Response predictors to cardiac resynchronization therapy in chronic heart failure: a 10-year-cardiovascular center experience.

Cardiac resynchronization therapy (CRT) has been established as an effective therapy for heart failure with reduced ejection fraction. Randomized clinical trials have shown its impact on mortality and HF hospitalizations, as well as improvement of symptoms and quality of life. Finding clinical, electrocardiographic, and echocardiographic variables that may predict the response to cardiac resynchronization therapy (CRT). We performed a single-center, observational, analytic, and retrospective study that included 102 patients with heart failure (HF) diagnosis who underwent CRT according to guideline-directed therapy from January 2010 to April 2020 in a third-level center. CRT response was defined as an improvement of New York Heart Association functional class in at least 1 category associated with a recovery of ≥ 5% in the left ventricular ejection fraction (LVEF). Our study population was 102 patients of which 61 (59.8%) were men. The mean age at HF diagnosis was 54 ± 18.7 years. Ischemic heart disease was the etiology in 37 (36.3%) cases. Fifty-one (50%) patients were classified as responders. Responders had wider QRS, and lower LVEF and right ventricular fractional area change at baseline. After CRT, responders had a greater reduction of QRS duration, and improvement in LVEF, global longitudinal strain, and echocardiographic dyssynchrony parameters. Multivariate regression analysis showed that left bundle branch block (LBBB), left ventricular end-diastolic volume (LVEDV), tricuspid annular plane systolic excursion (TAPSE), and baseline difference of pre-ejection periods were predictors of a positive response to CRT in this population. LBBB, TAPSE, LVEDV, and pre-ejection time difference are independent variables that can predict adequate response to CRT.

Outcomes of Rehabilitation Strategies for Pulmonary Atresia with Ventricular Septal Defect: A Single Center's Experience.

Both systemic-to-pulmonary shunt and right ventricle-pulmonary artery (RV-PA) connection are extensively applied to initially rehabilitate the pulmonary artery in pulmonary atresia with the ventricle septal defect (PA/VSD). However, which of these options is the most ideal for promoting pulmonary artery development and improving outcomes remains controversial. A total of 109 PA/VSD patients undergoing initial rehabilitative surgery at Guangdong Provincial People's Hospital from 2010 to 2020 were enrolled in this study. A series of clinical data were collected to compare the perioperative and postoperative outcomes between systemic-to-pulmonary and RV-PA connection. The mean duration of follow-up was 61.1 months in the systemic-to-pulmonary shunt group and 70.3 months in the RV-PA connection group (p 0.05). The RV-PA connection technique resulted in a significantly higher , lower red blood cells (RBC), lower hemoglobin, and lower hematocrit (Hct) (p 0.05). The cumulative incidence curve estimated a cumulative complete repair rate of 56 7% after 5 years in the RV-PA connection group, significantly higher than 36 7% after 5 years in the systemic-to-pulmonary shunt group (p 0.05). The Kaplan-Meier curve revealed a similar estimated survival rate between the two groups (p = 0.73). The RV-PA connection was identified as an independent predictor for complete repair in the multivariable analysis (HR = 2.348, 95% CI = 1.131-4.873). The RV-PA connection is a more ideal initial rehabilitative technique than systemic-to-pulmonary shunt in treating PA/VSD as a consequence of comparable probability of survival but improved definitive complete repair rate.

A guideline on guidelines: neuro-oncology guideline standards for low- and middle-income countries.

Guidelines for low- and middle-income countries (LMICs) are needed in complex, multidisciplinary areas such as oncology, requiring mobilising considerable resources and specialists for coordinated care. Neuro-oncology guidelines have been primarily established in countries where technological advancements and robust care pathways facilitate broad resource utilisation. In contrast, LMICs require complex and region-specific interventions to provide equitable care. The present opinion paper is a culmination of our own centre's experience collaborating and developing loco-regional guidelines for brain tumour care, keeping in mind LMIC experiences and expertise available. We intend for the process and methodology to apply to a broader audience of other LMIC authors and clinicians collaborating with LMIC institutions to develop guidelines and clinical recommendations.

A Brazilian Rare-Disease Center's Experience with Glucosylsphingosine (lyso-Gb1) in Patients with Gaucher Disease: Exploring a Novel Correlation with IgG Levels in Plasma and a Biomarker Measurement in CSF.

Gaucher disease (GD, OMIM 230800) is one of the most common lysosomal disorders, being caused by the deficient activity of the enzyme acid β-glucocerebrosidase (Gcase). Three clinical forms of Gaucher's disease (GD) are classified based on neurological involvement. Type 1 (GD1) is non-neuronopathic, while types 2 (GD2) and 3 (GD3) are neuronopathic forms. Gcase catalyzes the conversion of glucosylceramide (GlcCer) into ceramide and glucose. As GlcCer accumulates in lysosomal macrophages, it undergoes deacylation to become glycosylsphingosine (lyso-Gb1), which has shown to be a useful and reliable biomarker for the diagnosis and monitoring of treated and untreated patients with GD. Multiple myeloma (MM) is one of the leading causes of cancer-related death among patients with GD and monoclonal gammopathy of undetermined significance (MGUS) is a non-neoplastic condition that can be a telltale sign of a B clonal proliferation caused by the chronic activation of B cells. This study aimed to quantify Lyso-Gb1 levels in dried blood spots (DBS) and cerebrospinal fluid (CSF) as biomarkers for Gaucher disease (GD) and discuss the association of this biomarker with other clinical parameters. This is a mixed-methods study incorporating both cross-sectional and longitudinal elements within a cohort design with a convenience-sampling strategy. Data collection took place from January 2012 to March 2023. Lyso-Gb1 extraction from DBS involved the use of a methanol-acetonitrile-water mixture, followed by incubation and centrifugation. Analysis was performed using UPLC-MS/MS with MassLynx software version 4.2 and the control group for the DBS measurements included general newborns. CSF Lyso-Gb1 was extracted using ethyl acetate, analyzed by UPLC-MS/MS with a calibration curve, and expressed in pmol/L. Lysosomal activity in CSF was assessed by measuring chitotriosidase (Cht), and other lysosomal enzyme activities were assessed as previously described in the literature. Patients with metachromatic leukodystrophy (MLD) were used as controls. Thirty-two treated patients (twenty-nine GD1 and three GD3, all on ERT except for one GD type on SRT with eliglustat) and three untreated patients (one GD1, one GD2, and one GD3) were included. When analyzing only the treated GD1 group, a significant correlation was found between lyso-Gb1 and age (rho = -0.447, p = 0.001), ChT, and IgG levels (rho = 0.73, p < 0.001; and rho = 0.36, p = 0.03, respectively). Five GD1 patients (three females, mean age 40 years) also had their CSF collected and analyzed. The average measurement of lyso-Gb1 in CSF was 94 pmol/L (range: 57.1-157.9 pmol/L) versus <6.2 pmol/L in the control group (MLD). This is the first time, to the best of our knowledge, that lyso-Gb1 has been associated with IgG levels. While this finding reflects a risk for MGUS or MM and not only chronic plasma B-cell activation, it still requires further studies. Moreover, the analysis of CSF lyso-Gb1 levels in GD1 patients was demonstrated to be significantly higher than the control group. This raises the hypothesis that CSF lyso-Gb1 may serve as a valuable indicator for neurological involvement in GD, providing insights into the potential implications for neurological manifestations in GD, including GD1. The correlation between lyso-Gb1 and ChT levels in treated GD1 patients further underscores the interconnectedness of lysosomal markers and their relevance in monitoring.

The Evaluation of Objective and Subjective Fate of Teeth in the Mandible Fracture Line and the Management-A Center's Experience.

The decision to retain or extract teeth in the line of mandibular fractures has been a subject of debate in much of the scientific literature, and there is a need for further evidence to clear this issue. Thus, the aim of this study was to investigate both the positive and negative effects of teeth in the line of mandibular fractures provide more evidence in this field, as well as take into consideration patients' quality of life after the surgery. Patients after trauma with teeth in the line of mandibular fractures were included in this study. Open reduction and rigid internal fixation, elastic intermaxillary reduction were expected to achieve a satisfactory occlusion and/or anatomical reduction in the fragments as assessed by orthopantomogram, computed tomography scans, and clinical examination. The remaining cases had maxillomandibular fixation (MMF) with an arch bar and bridle wire. All the patients included in this study will take the Visual Analog Scale score evaluation before and after surgery subjectively to further verify the impact on their life qualify, as well as the further treatment needed. A total of 78 patients with teeth in the line of mandibular fractures were included in this study. Open reduction and rigid internal fixation was used in 37 patients, whereas another 35 patients accepted elastic intermaxillary reduction. Six cases underwent MMF. The number of involved teeth was 83. Three of the 83 teeth involved in the fracture lines healed with complications. In the cases where the teeth had been removed before fracture treatment, or in cases of delayed extractions, no complications were noted. The majority of the patients felt good about the whole treatment, however, 4 in the MMF group complained about worry about their oral health due to MMF leading to mouth open limitation. The factors that should be considered for removal include the condition of the teeth and alveolar bone, the timing and the type of treatment, as well as the patients' desire, if possible. This is an individual-based decision that needs to consider more objective and subjective potential risks to avoid complications.

Left Gastric Vein Direct Anastomosis as Alternative to Portal Flow Reconstruction in Liver Transplantation.

The most relevant limiting factor for performing end-to-end anastomosis is portal vein thrombosis (PVT), which leads to challenging vascular reconstructions. This study aimed to analyze a single center's experience using the left gastric vein (LGV) for portal flow reconstruction in liver transplantation (LT). This retrospective observational study reviewed laboratory and imaging tests, a description of the surgical technique, and outpatient follow-up of patients with portal system thrombosis undergoing LT with portal flow reconstruction using the LGV. This study was conducted at a single transplant reference center in the northeast region of Brazil from January 2016 to December 2021. Between January 2016 and December 2021, 848 transplants were performed at our center. Eighty-two patients (9.7%) presented with PVT, most of whom were treated with thrombectomy. Nine patients (1.1% with PVT) had extensive thrombosis of the portal system (Yerdel III or IV), which required end-to-side anastomosis between the portal vein and the LGV without graft, and had no intraoperative complications. All patients had successful portal flow in Doppler ultrasound control evaluations. The goal was to reestablish physiological flow to the graft. A surgical strategy includes using the LGV graft. According to our reports, using LGV fulfilled the requirements for excellent vascular anastomosis and even allowed the dispensing of venous grafts. This is the largest case series in a single center of reconstruction of portal flow with direct anastomosis with the LGV without needing a vascular graft.

Influence of donor age on liver transplantation outcomes: A multivariate analysis and comparative study.

The growing disparity between the rising demand for liver transplantation (LT) and the limited availability of donor organs has prompted a greater reliance on older liver grafts. Traditionally, utilizing livers from elderly donors has been associated with outcomes inferior to those achieved with grafts from younger donors. By accounting for additional risk factors, we hypothesize that the utilization of older liver grafts has a relatively minor impact on both patient survival and graft viability. To evaluate the impact of donor age on LT outcomes using multivariate analysis and comparing young and elderly donor groups. In the period from April 2013 to December 2018, 656 adult liver transplants were performed at the University Hospital Merkur. Several multivariate Cox proportional hazards models were developed to independently assess the significance of donor age. Donor age was treated as a continuous variable. The approach involved univariate and multivariate analysis, including variable selection and assessment of interactions and transformations. Additionally, to exemplify the similarity of using young and old donor liver grafts, the group of 87 recipients of elderly donor liver grafts (≥ 75 years) was compared to a group of 124 recipients of young liver grafts (≤ 45 years) from the dataset. Survival rates of the two groups were estimated using the Kaplan-Meier method and the log-rank test was used to test the differences between groups. Using multivariate Cox analysis, we found no statistical significance in the role of donor age within the constructed models. Even when retained during the entire model development, the donor age's impact on survival remained insignificant and transformations and interactions yielded no substantial effects on survival. Consistent insignificance and low coefficient values suggest that donor age does not impact patient survival in our dataset. Notably, there was no statistical evidence that the five developed models did not adhere to the proportional hazards assumption. When comparing donor age groups, transplantation using elderly grafts showed similar early graft function, similar graft (P = 0.92), and patient survival rates (P = 0.86), and no significant difference in the incidence of postoperative complications. Our center's experience indicates that donor age does not play a significant role in patient survival, with elderly livers performing comparably to younger grafts when accounting for other risk factors.

Efficacy of surgical treatment of primary hyperparathyreoidism using intraoperative PTH monitoring – one center experience

Efficacy of surgical treatment of primary hyperparathyreoidism using intraoperative PTH monitoring – one center experience

Thoracic aortic aneurysms, a single center's 10-years experience and analysis of outcomes.

Thoracic aortic aneurysms (TAAs) are an increasingly prevalent pathology with significant associated morbidity and mortality. Thoracic endovascular aortic repair (TEVAR) is the primary line of treatment. The purpose of this study was to analyse a single center's experience in the treatment of TAAs and identify possible risk factors for worse outcomes. A retrospective review of our institutional database was done to identify all patients treated for TAAs in a 10-year period, from 1 January 2012 to 31 December 2022. Data were extracted from patients' medical records. Primary outcome was all-cause mortality and secondary outcomes were procedure related morbidity (vascular access complications, medullary ischaemia, stroke, endoleaks, migration, aneurysm sac enlargement >5mm) and need for reintervention at 1-, 6- and 12-month follow-up. A descriptive and inferential analysis of the data was performed. Statistical analyses were conducted using the IBM Statistical Package for Social Sciences (SPSS) software. We identified 34 patients treated for TAAs in this period. Mean age was 68years [47-87] and 79.4% of patients were male. Mean aneurysm diameter was 63mm [35-100], 55.9% fusiform and 44.1% saccular. The majority (91.2%) were located at the descending thoracic aorta and 3 (8.8%) of them extended to the aortic arch. The most common aetiology was degenerative in 22 patients (64.7%), followed by aortic dissection in 8 patients (23.5%). Elective surgery was performed in 19 (61.3%) patients and 12 (38.7%) had urgent repair. TEVAR was the treatment of choice in 24 (77.4%) patients, and the remaining 7 (22.6%) were treated with hybrid surgery. Mean length of hospital stay was 10days [2-80] (6 days for elective repair versus 16days for urgent repair, p = .016). Follow-up period ranged from 1month to 10years. At 1 year follow-up, all-cause mortality was 15%, morbidity was 30% (with 6 (22%) patients having a type Ia endoleak) and need for reintervention was 22%. Aneurysm diameter was a significant risk factor for procedure related morbidity (median diameter of 73.5mm versus 56.0mm in patients with no morbidity; p = .027). The presence of type Ia endoleak was significantly associated with higher reintervention rates (p = .001), but not with higher mortality rates (p = .515). Age, female sex, aetiology and urgent repair weren't associated with any significant differences in the outcomes. TEVAR proved to be effective in the treatment of TAAs, with good outcomes at short and mid-term follow-up. TAAs should be diagnosed earlier and be promptly treated when meeting criteria to prevent worse outcomes.

Stimulation-Induced Seizures in Children Undergoing Stereo-EEG Evaluation.

This study reports our center's initial experience with the use of low-frequency stimulation in provoking stimulation-induced seizures (SIS) in children with drug-resistant epilepsy undergoing stereo-EEG evaluations. This retrospective study enrolled children aged 2 to 18 years with drug-resistant focal epilepsy who underwent stereo-EEG evaluation and extraoperative direct electrical cortical stimulation to elicit seizures. The low-frequency stimulation parameters consisted of biphasic square waveforms at frequency of 1 Hz, pulse width 1 millisecond, current 1 to 3 mA, and train duration of 20 seconds. Various epilepsy-related, imaging, neurophysiology, and surgery-related variables were collected and summarized. Fourteen children (mean age 13 years; 57.1% girls) were included, 10 of whom had unilateral stereo-EEG coverage. Cortical stimulation for provoking seizures was performed after a median of 5 days after electrode implantation. The median number of electrode-contacts stimulated per patient was 42. Four patients (28.6%) experienced habitual SIS (all extratemporal). The etiology in three patients was focal cortical dysplasia. Interictal high-frequency oscillations at electrode-contacts provoking SIS were observed in three cases (75%). Two of these individuals (50%) had class 1 International League Against Epilepsy seizure outcome at last follow-up, after the resection of the brain regions generating SIS. Low-frequency (1-Hz) stimulation could provoke habitual SIS in nearly one-fourth of children with focal epilepsy undergoing stereo-EEG monitoring. This study provides a limited pediatric experience with the low-frequency cortical stimulation and SIS.

Is CRT Optimization Obsolete? A Referral Center's Experience.

Cardiac resynchronization therapy (CRT) is a well-established therapy for patients with heart failure (HF). However, 30% of HF patients do not show any improvement in clinical status after CRT implantation. In this study, we report our echocardiography-based CRT optimization methodology, in daily practice at our CRT referral center. We included 350 ambulatory patients, who were referred to our center for optimization after CRT implantation. A protocol-driven echocardiographic approach for adjusting mechanical dyssynchrony, whereby adjusting for ventriculoventricular (VV) delays with strain and atrioventricular (AV) delays with Doppler echocardiography was performed. We defined changes in left ventricular ejection fraction (LVEF) and New York Heart Association (NYHA) classes as outcome variables in the evaluation of the CRT outcomes. Optimization was obtained in 288 (82%) patients. VV and AV timings were adjusted to 61% and 51%, respectively. In 3%, biventricular pacing was turned off and in 3% left ventricular (LV) only pacing was programmed. The LVEF and NYHA class showed significant improvements in all patients who underwent CRT optimization. CRT optimization remains valuable in improving LVEF and functional status measured using the NYHA class in all patients receiving CRT devices.

A Single Center's Experience With Spinal Anesthesia for Pediatric Patients Undergoing Surgical Procedures

PurposeTo perform a single institution review of spinal instead of general anesthesia for pediatric patients undergoing surgical procedures. Spinal success rate, intraoperative complications, and postoperative outcomes including unplanned hospital admission and emergency department visits within seven days are reported. MethodsRetrospective chart review of pediatric patients who underwent spinal anesthesia for surgical procedures from 2016 until 2022. Data collected included patient demographics, procedure and anesthetic characteristics, intraoperative complications, unplanned admissions, and emergency department returns. ResultsThe study cohort included 1221 patients. Ninety-two percent of the patients tolerated their surgical procedure without requiring conversion to general anesthesia, and 78% of patients that had spinals placed successfully did not receive any sedation following lumbar puncture. The most common intraoperative event was systolic blood pressure below 60 mm Hg (14%), but no cases required administration of vasoactive agents, and no serious intraoperative adverse events were observed. Post-Anesthesia Care Unit Phase I was bypassed in 72% of cases with a median postoperative length of stay of 84 min. Forty-six patients returned to the emergency department following hospital discharge, but no returns were due to anesthetic concerns. ConclusionsSpinal anesthesia is a viable and versatile option for a diversity of pediatric surgical procedures. We noted a low incidence of intraoperative and postoperative complications. There remain numerous potential advantages of spinal anesthesia over general anesthesia in young pediatric patients particularly in the ambulatory setting. Level of EvidenceIV. Type of StudyRetrospective cohort treatment study.

Rarely Used Endoscopic Transnasal Transdiaphragmatic Technique in Patients with Suprasellar Extension: A Tertiary Center's Experience with Eleven Patient Cases

OBJECTIVEIn our age,as surgical techniques are advancing towards being less invasive,the use of endoscopy in brain surgery supports this trend.There are numerous endoscopic surgical approaches defined,especially for skull base pathologies.The current study summarizes our experience of using the rarely reported endoscopic transnasal transdiaphragmatic approach through the existing hole in the diaphragma sella to access lesions extending into the suprasellar region. METHODSOur surgical team performed 4876 endoscopic endonasal surgeries between August 1997 and December 2022 at the Department of Neurosurgery, Pituitary Research Center, Faculty of Medicine, Kocaeli University. The current study retrospectively analyzed data from 11 patients who underwent endoscopic transnasal transdiaphragmatic surgery since January 2020. Pre- and postoperative magnetic resonance imaging, pituitary function examination, and clinical observation was carried out. RESULTSThe mean age of the patients was 31.1±10.7 years and the female: male ratio was 6:5. Pathological subtypes observed included breast cancer metastasis(n=1), ACTH-secreting adenoma(n=4), GH-secreting adenoma(n=3), craniopharyngioma(n=2), and Rathke’s cleft cyst(n=1).The mean postoperative hospital stay was 4.7±1.1 days and none of the patients exhibited cerebrospinal fluid leakage during this period. CONCLUSIONSThe endoscopic transnasal transdiaphragmatic approach may be considered as an alternative for the conventional extended endoscopic transnasal approach in patients with lesions extending into the suprasellar region. The main strength of this method is that it facilitates suprasellar region access through a small dural incision and bone defect in the base of the skull. As a result, it also reduces the risk of postoperative cerebrospinal fluid leakage and associated complications.

Timing the clinical onset of epileptic spasms in infantile epileptic spasms syndrome: A tertiary health center's experience.

Lead time to treatment (clinical onset of epileptic spasms [ES] to initiation of appropriate treatment) is known to predict outcomes in infantile epileptic spasms syndrome (IESS). Timing the clinical onset of ES is crucial to establish lead time. We investigated how often ES onset could be established to the nearest week. We aimed to (1) ascertain the exact date or estimate the nearest week of ES onset and (2) compare clinical/demographic factors between patients where date of ES onset was determined or estimated to the nearest week and patients whose date of ES onset could not be estimated to the nearest week. Reasons for difficulties in estimating date of ES onset were explored. Retrospective chart review of new onset IESS patients (January 2019-May 2022) extracted the date or week of the clinical onset of ES. Predictors of difficulty in date of ES onset estimation to the nearest week were examined by regression analysis. Sources contributing to difficulties determining date of ES onset were assessed after grouping into categories (provider-, caregiver-, disease-related). Among 100 patients, date of ES onset was estimated to the nearest week in 47%. On univariable analysis, age at diagnosis (p = .021), development delay (p = .007), developmental regression/stagnation (p = .021), ES intermixed with other seizures (p = .011), and nonclustered ES at onset (p = .005) were associated with difficulties estimating date of ES onset. On multivariable analysis, failure to establish date of ES onset was related to ES intermixed with other seizures (p = .004) and nonclustered ES at onset (p = .003). Sources contributing to difficulties determining date of ES onset included disease-related factors (ES characteristics, challenges interpreting electroencephalograms) and provider/caregiver-related factors (delayed diagnosis). Difficulties with estimation of lead time (due to difficulties timing ES onset) can impact clinical care (prognostication), as even small increments in lead time duration can have adverse developmental consequences.

Acute kidney injury in Hodgkin’s lymphoma, non-Hodgkin lymphoma/leukemia, and lymphoplasmacytic lymphoma: One center experience

Background: Acute kidney injury (AKI) is a common complication in patients with leukemia and lymphoma. In this single-center non-interventional retrospective study from 2010 to 2022, we aimed to evaluate causes, clinical features, and pathology finding in patients with Hodgkin lymphoma (HL), non-Hodgkin lymphoma/leukemia (NHL/CLL), or lymphoplasmacytic lymphoma (LPL) and AKI. Methods: We identified 101 patients with HL, NHL/CLL, and LPL. We analyzed AKI incidence and various clinical, laboratory, and pathology parameters and outcomes. Results: About 46.5% of patients with HL, NHL/CLL, and LPL presented with AKI. The incidence of AKI was the highest in the CLL setting (65.0%), it was lower and almost equal (48.0% and 47.0%) in NHL and HL, respectively, and the lowest (14.2%) in LPL. Study group comprised of 47 patients, M/F 31/16, median age was 64.0 [52.0; 71.0] years, and the patients with HL were significantly younger ( p = 0.022). The median disease duration prior to the AKI presentation was 14 [5; 44] months. Pre-renal AKI was diagnosed in 48.9% of cases: tumor lysis syndrome (TLS), specific lymphoid infiltration (LI), and post-renal AKI presented in 12.7% each. Kidney biopsy in 49% of patients revealed LI in 39.1% of cases. Focal segmental glomerulosclerosis presented in 21.7% of cases, proliferative glomerulonephritis with monoclonal immunoglobulin deposits in 13.0%, AA amyloidosis, IgA nephropathy, and AA amyloidosis in 8.6% each. AKI recovered in 63.8% of patients and 59.5% of patients were alive and dialysis-free at last evaluation: 100% with HL versus 58.3%, 46.1%, and 0% with NHL, LL, and LPL, respectively ( p = 0.025); and 8.5% remained alive on dialysis. About 25.5% of patients died after discontinuation or without need for dialysis; and 6.3% died on dialysis. Conclusion: AKI in patients with leukemia and lymphoma is heterogeneous and most commonly caused by volume depletion. AKI recovery, though not complete, occurred in 63.8% of cases with 68% survival.

Effectiveness of combined plasma cell therapy and costimulation blockade based desensitization regimen in heart transplant candidates.

Desensitization is one of the strategies to reduce antibodies and facilitate heart transplantation in highly sensitized patients. We describe our center's desensitization experience with combination of plasma cell (PC) depletion therapy (with proteasome inhibitor or daratumumab) and costimulation blockade (with belatacept). We reviewed five highly sensitized patients who underwent desensitization therapy with plasma cell depletion and costimulation blockade. We evaluated the response to therapy by measuring the changes in cPRA, average MFI, and number of positive beads>5000MFI. Five patients, mean age of 56 (37-66) years with average cPRA of 98% at 5000 MFI underwent desensitization therapy. After desensitization, mean cPRA decreased from 98% to 70% (p=.09), average number of beads>5000 MFI decreased from 59 to 37 (p=.15), and average MFI of beads>5000 MFI decreased from 16713 to 13074 (p=.26). Combined PC depletion and CoB could be a reasonable strategy for sustained reduction in antibodies in highly sensitized patients being listed for heart transplantation.

Re-hospital admission, morbidity and mortality rate in patients undergoing tunnelled catheter implantation for haemodialysis

Despite the effort to increase the proportion of patients starting dialysis on native accesses, many of them are still dialyzed on tunnelled catheter. Catheter-related complications are often serious and responsible for re-hospital admission, high morbidity and mortality. Several multicenter trials have reported results in the use of tunnelled dialysis catheter (TDC). However, few single-center studies have been published to verify the outcome from real-world experience. This study presents our center's experience in managing such patients in the context of relevant literature. Demographics and operative data were retrospectively collected from medical charts. A prospective follow-up was performed to investigate complications, number of re-hospitalizations and mortality. Kaplan-Meier estimate was used to evaluate catheter primary patency and patients' overall survival. Among a total 298 haemodialysis accesses interventions, 105 patients (56 men, 53.3% and 49 women, 46.7%) with a median age of 65 years (range 32-88 years) were included in the study. All insertions were successful with an optimal blood flow achieved during the first session of dialysis in all cases. A catheter-related complication was detected in 33.3% (n=35) patients (48.6% infections; 28.6% TDC dysfunction; 14.3% local complications; 5.7% accidental catheter retractions; 2.8% catheter migrations). At a median follow-up of 10.5±8.5 months, a total of 85 patients (80.9%) was re-hospitalized, in 28 cases (26.7%) for a catheter-related cause. The median catheter patency rate was 122 days. At the last follow-up, 39 patients (37.1%) were still dialyzed on catheter, 30(28.6%) were dialyzed on an arteriovenous fistula and 7(6.7%) received a kidney transplantation. Two patients (2%) were transferred to peritoneal dialysis and two patients (2%) recover from renal insufficiency. Mortality rate was 23.8% (25 patients). Causes of death were myocardial infarction (n=13, 52%), sepsis (n=9, 36%); one patient (4%) died from pneumonia, one (4%) from uremic encephalopathy and one (4%) from massive hematemesis. TDCs may represent the only possible access in some patients, however they are burned with a high rate of complications, re-hospital admission and mortality. Results from this institutional experience are in line with previously published literature data in terms of morbidity and mortality. The present results reiterate once more that TDC must be regarded as a temporary solution while permanent access creation should be prioritized. Strict surveillance should be held in patients having TDC for the early identification of complications allowing the prompt treatment and modifying the catheter insertion site whenever needed.