The evaluation, management, and future of indolent systemic mastocytosis.

Indolent systemic mastocytosis (ISM) is a chronic hematologic malignancy that has traditionally been managed primarily by allergists. However, with improved molecular diagnostics such as droplet digital PCR (ddPCR) for KIT D816V on bone marrow aspirate specimens and the availability of targeted therapies against KIT, hematologists are increasingly involved in the care of these patients. This review summarizes current understanding of the evaluation, diagnosis, treatment, and future therapeutic strategies in ISM, with a focus on clinically relevant insights for hematologists.

Contemporary CMML Risk Stratification and Management.

Purpose Of Review: Chronic myelomonocytic leukemia (CMML), a rare myelodysplastic/myeloproliferative neoplasm (MDS/MPN) overlap syndrome, poses diagnostic and therapeutic challenges due to its heterogeneity and rarity. This review highlights updates to CMML diagnostic criteria, examines risk stratification models and their clinical implications, and outlines both established and emerging therapies for this rare and likely underrecognized hematologic malignancy.

Recent Findings: Updated diagnostic criteria from 2022 reduce the monocyte threshold to 0.

Uninsured status is associated with worse outcomes in a cohort of pediatric patients with traumatic brain injury.

Background: Pediatric traumatic brain injury (pTBI) is a leading cause of morbidity and mortality in children. In this study, we sought to investigate the impact of insurance status on pTBI outcomes using a single-center registry.

Methods: A retrospective study was conducted of pTBI patients at a Level I Pediatric Trauma Center from January 2012 to July 2023.

Outcomes, complications, and dosing of intrathecal baclofen in the treatment of multiple sclerosis: a systematic review.

Objective: The purpose of this systematic review was to evaluate empirical outcomes of studies in the literature that investigated effectiveness of intrathecal baclofen (ITB) in the treatment of multiple sclerosis (MS)-related spasticity (MSRS) based on various metrics. Since the first description of this route of baclofen delivery for MS patients by Penn and Kroin in 1984, numerous studies have contributed to the medical community's knowledge of this treatment modality. The authors sought to add to the literature a systematic review of studies over the last 2 decades that elucidates the clinical impact of ITB in treating MSRS with the following endpoints: impact on patient-centered outcomes, such as spasticity reduction (primary), complications (secondary), and dosing (secondary).

Primary Sellar Neuroblastoma Masquerading as a Pituitary Macroadenoma.

Olfactory neuroblastomas, or esthesioneuroblastomas, are rare and aggressive malignant tumors that typically arise from the olfactory neuroepithelium in the upper nasal cavity. In rare instances, they can be ectopic originating from areas outside the upper nasal cavity such as the sellar region. These tumors, also known as primary sellar neuroblastomas, may be mistaken for pituitary macroadenomas.

Predictive Model Evaluating Risk of Hemorrhage in Intracranial Aneurysms: Analysis from Prospectively Collected HEAT Trial Database.

Objective: We analyzed the data of patients enrolled in the Hydrogel Endovascular Aneurysm Treatment (HEAT) trial to develop and validate a model to predict the risk of aneurysmal hemorrhage.

Methods: Analysis included data from 600 patients enrolled for the HEAT trial and included single saccular aneurysms of 3-14 mm size. Baseline characteristics were compared between patients with ruptured and unruptured aneurysms.

Hospital Length of Stay Independently Predicts Mortality in Patients Emergently Admitted for Esophageal Hemorrhage: Sex, Frailty, and Age as Additional Mortality Factors.

Introduction: Upper gastrointestinal bleeding results in greater than $7.6 billion of in-hospital economic burden in the United States yearly. With a worldwide incidence between 40-100/100,000 individuals and a mortality rate of approximately 2-10%, upper gastrointestinal bleeding represents a major source of mortality and morbidity.

Cerebrospinal Venous Fistula Presenting with Cognitive Decline: Systematic Literature Review and Report of Two Cases.

Objective: A cerebrospinal fluid (CSF) venous fistula (CVF) is an aberrant connection between the subarachnoid space and a vein resulting in CSF loss. The presentation and management of CVF with cognitive decline is incompletely understood.

Methods: A systematic review was completed following the PRISMA guidelines.

Risk factors for posttraumatic hydrocephalus after decompressive hemicraniectomy in pediatric patients with traumatic brain injury.

Objective: Traumatic brain injuries (TBIs) play a significant role in pediatric mortality and morbidity. Decompressive hemicraniectomy (DHC) is a treatment option for severe pediatric TBI (pTBI) not amenable to medical management of intracranial pressure. Posttraumatic hydrocephalus (PTH) is a known sequela of DHC that may lead to further injury and decreased capacity for recovery if not identified and treated.

A comparison of treating physician versus independent core lab assessments of post-aneurysm treatment imaging outcomes: an analysis of prospectively collected data from a randomized trial.

Objective: Aneurysm occlusion has been used as surrogate marker of aneurysm treatment efficacy. Aneurysm occlusion scales are used to evaluate the outcome of endovascular aneurysm treatment and to monitor recurrence. These scales, however, require subjective interpretation of imaging data, which can reduce the utility and reliability of these scales and the validity of clinical studies regarding aneurysm occlusion rates.

Clinical outcomes of first- and second-generation hydrogel coils compared with bare platinum coils: a systematic literature review.

Endovascular coiling has revolutionized intracranial aneurysm treatment; however, recurrence continues to represent a major limitation. The hydrogel coil was developed to increase packing density and improve neck healing and therefore decrease recurrence rates. In this paper, we review treatment outcomes of first- (1HCs) and second-generation (2HCs) hydrogel coils and compare them to those of bare platinum coils (BPC).

Real-Time MRI-Guided Stereotactic Aspiration of Spontaneous Intracerebral Hematoma: A Preclinical Feasibility Study.

Background: Minimally invasive surgical techniques have reinvigorated the role of surgical options for spontaneous intracranial hematomas; however, they are limited by the lack of real-time feedback on the extent of hematoma evacuation.

Objective: To describe the development of a MRI-guided catheter-based aspiration system, the ClearPoint Pursuit Neuroaspiration Device (ClearPoint Neuro) and validation in phantom models.

Methods: In this preclinical experimental trial, 8 phantom brains with skull models were created to simulate an intracranial hematoma with 2 clot sizes, 30 cc (small clot) and 60 cc (large clot).

NRF2 Loss Accentuates Parkinsonian Pathology and Behavioral Dysfunction in Human α-Synuclein Overexpressing Mice.

Nuclear factor (erythroid-derived 2)-like 2 (NRF2) is a central regulator of cellular stress responses and its transcriptional activation promotes multiple cellular defense and survival mechanisms. The loss of NRF2 has been shown to increase oxidative and proteotoxic stress, two key pathological features of neurodegenerative disorders such as Parkinson's disease (PD). Moreover, compromised redox homeostasis and protein quality control can cause the accumulation of pathogenic proteins, including alpha-synuclein (α-Syn) which plays a key role in PD.

Quality of Life of Patients with Unruptured Intracranial Aneurysms Before and After Endovascular Coiling: A HEAT Trial Secondary Study and Systematic Review of the Literature.

Background: The study of quality of life (QOL) in patients with asymptomatic diseases receiving interventional treatment provides an essential metric for the assessment of procedural benefits in the surgical patient population. In this study, we analyzed QOL data collected from patients with unruptured intracranial aneurysms (UIAs) before and after endovascular coiling in the HEAT Trial, alongside a systematic review on QOL in unruptured brain aneurysms.

Methods: HEAT was a randomized controlled trial comparing recurrence rates in aneurysms treated with either bare platinum coils or hydrogel coils.