Speech, Language and Non-verbal Communication in CLN2 and CLN3 Batten Disease.

CLN2 and CLN3 diseases, the most common types of Batten disease (also known as neuronal ceroid lipofuscinosis), are childhood dementias associated with progressive loss of speech, language and feeding skills. Here we delineate speech, language, non-verbal communication and feeding phenotypes in 33 individuals (19 females) with a median age of 9.5 years (range 3-28 years); 16 had CLN2 and 17 CLN3 disease; 8/15 (53%) participants with CLN2 and 8/17 (47%) participants with CLN3 disease had speech and language impairments prior to genetic diagnosis.

A National Assessment of Pediatric Surgical Critical Care Fellowship Structure: Defining the Training Experience.

Background: Pediatric Surgical Critical Care (PSCC) is a unique specialty incorporating fundamental principles of surgical, neonatal, and pediatric critical care. This study aims to characterize the current landscape of PSCC training to identify opportunities for educational standardization and improvement.

Methods: An anonymous electronic survey-based assessment was distributed to the program directors (PDs) of all current ACGME-accredited PSCC fellowships (n = 14).

Finding the Right Partner: Triplet Therapy for First-Line Advanced Biliary Tract Cancers.

Journal Journal of Clinical Oncology

Tumor burden with AFP improves survival prediction for TACE-treated patients with HCC: An international observational study.

Background & Aims: Current prognostic models for patients with hepatocellular carcinoma (HCC) undergoing transarterial chemoembolization (TACE) are not extensively validated and widely accepted. We aimed to develop and validate a continuous model incorporating tumor burden and biology for individual survival prediction and risk stratification.

Methods: Overall, 4,377 treatment-naive candidates for whom TACE was recommended, from 39 centers in five countries, were enrolled and divided into training, internal validation, and two external validation datasets.

Investigating changes in connected speech in nonfluent/agrammatic primary progressive aphasia following script training.

Script training is a speech-language intervention designed to promote fluent connected speech via repeated rehearsal of functional content. This type of treatment has proven beneficial for individuals with aphasia and apraxia of speech caused by stroke and, more recently, for individuals with primary progressive aphasia (PPA). In the largest study to-date evaluating the efficacy of script training in individuals with nonfluent/agrammatic primary progressive aphasia (nfvPPA; Henry et al.

Editorial: Understanding the Nature of Attention Problems in Neurofibromatosis Type 1: Clinical and Research Implications.

Attention-deficit/hyperactivity disorder (ADHD) is one of the most common psychiatric disorders of childhood, and though highly heritable, there is a clear pattern of polygenic inheritance with many genes contributing to its expression. Yet, as described in this issue of the Journal, ADHD is also overrepresented in neurofibromatosis type 1 (NF1), an autosomal dominant disorder resulting from mutations in a single gene and affecting 1 in 3,000 people. Although NF1 is named after the characteristic neurofibromas, or benign nerve sheath tumors, and morbidity and mortality are mostly related to the potential development of malignant nerve sheath and other tumors of the nervous system, patients with NF1 also have general cognitive and behavioral difficulties.

Outcomes of Stereotactic Body Radiotherapy Compared with Surgical Resection in Patients with Hepatocellular Carcinoma and Macrovascular Invasion: A Propensity Score-Matched Analysis.

Introduction: Patients with advanced hepatocellular carcinoma (HCC) and macrovascular invasion (MVI) are recommended to receive systemic therapy according to guidelines. Stereotactic body radiotherapy (SBRT) and surgery are increasingly used in this patient population. This study compares outcomes from these local treatments.

Liver Transplantation for Hepatocellular Carcinoma: An Expanding Cornerstone of Care in the Era of Immunotherapy.

Liver transplantation (LT) has been accepted as a cornerstone of care in hepatocellular carcinoma (HCC) for almost three decades. In recent years, its role has been evolving to include patients with disease burden beyond the widely used Milan criteria. The integration of dynamic biomarkers such as alpha-fetoprotein together with downstaging approaches and tumor evolution after enlistment has allowed the selection of patients most likely to benefit, resulting in 5-year survival rates greater that 70%.

Dataset for indoor air temperature measurements using ultrasonic travel-time tomography.

This dataset presents the indoor air temperature collections using the novel technique of Acoustic travel time TOMography (ATOM) in the ultrasonic frequency range. The presented measuring system employs early reflections as sound propagation paths in addition to the travel time of the direct path, effectively combining room acoustics with tomography techniques. The data was collected across various measurement scenarios within the climate chamber laboratory at the Building Physics Department at the Bauhaus-University Weimar.

Interbreeding area movement of an adult humpback whale between the east Pacific Ocean and southwest Indian Ocean.

Humpback whales undertake one of the longest known migrations of any mammal. While their migration route generally extends between latitudes, the breeding stocks are longitudinally separated and display high site fidelity to their feeding grounds. While there is an indication of certain breeding stocks overlapping with each other, the current information on the migration routes of humpback whales within the Southern Hemisphere limits our understanding of the extent of this exchange.

EPA guidance on lifestyle interventions for adults with severe mental illness: A meta-review of the evidence.

There is growing interest in lifestyle interventions as stand-alone and add-on therapies in mental health care due to their potential benefits for both physical and mental health outcomes. We evaluated lifestyle interventions focusing on physical activity, diet, and sleep in adults with severe mental illness (SMI) and the evidence for their effectiveness. To this end, we conducted a meta-review and searched major electronic databases for articles published prior to 09/2022 and updated our search in 03/2024.

Outcome and management of patients with hepatocellular carcinoma who achieved a complete response to immunotherapy-based systemic therapy.

Background And Aims: The outcome of patients with HCC who achieved complete response (CR) to immune-checkpoint inhibitor (ICI)-based systemic therapies is unclear.

Approach And Results: Retrospective study of patients with HCC who had CR according to modified Response Evaluation Criteria in Solid Tumors (CR-mRECIST) to ICI-based systemic therapies from 28 centers in Asia, Europe, and the United States. Of 3933 patients with HCC treated with ICI-based noncurative systemic therapies, 174 (4.

Towards a risk assessment framework for micro- and nanoplastic particles for human health.

Background: Human exposure to micro- and nanoplastic particles (MNPs) is inevitable but human health risk assessment remains challenging for several reasons. MNPs are complex mixtures of particles derived from different polymer types, which may contain plenty of additives and/or contaminants. MNPs cover broad size distributions and often have irregular shapes and morphologies.

Comparative analysis of treatment modalities for solitary, small (≤3 cm) hepatocellular carcinoma: A systematic review and network meta-analysis of oncologic outcomes.

Background: Solitary hepatocellular carcinoma measuring ≤3 cm represents approximately 30% of hepatocellular carcinoma cases, yet treatment guidelines lack robust evidence. This study compares oncologic outcomes after ablation, liver resection, and liver transplantation for solitary, small hepatocellular carcinoma.

Methods: We systematically searched databases up to 7 February 2022, for studies including adults with solitary hepatocellular carcinoma ≤3 cm treated by any ablation, liver resection, or liver transplantation.

Solitary Fibrous Tumor of the Larynx: A Case Report and Brief Literature Review.

Solitary fibrous tumors (SFTs) are a rare type of soft tissue tumor first identified as arising from pleura and usually arising in the thoracic cavity. The incidence of SFTs arising in the head and neck region is much lower, accounting for about 7% of SFT cases. Involvement of the larynx specifically is exceedingly rare and can have inconspicuous presentations, making them challenging to diagnose.

Preclinical characterization of the Omicron XBB.1.5-adapted BNT162b2 COVID-19 vaccine.

As SARS-CoV-2 evolves, increasing in potential for greater transmissibility and immune escape, updated vaccines are needed to boost adaptive immunity to protect against COVID-19 caused by circulating strains. Here, we report features of the monovalent Omicron XBB.1.

Systemic approaches in biliary tract cancers: a review in the era of multidirectional precision medicine.

Introduction: Despite a rising incidence, biliary tract cancers (BTCs) are still considered a rare tumor entity. The disease's subtle clinical presentation and lack of effective early detection strategies often lead to a diagnosis at an advanced or unresectable stage, where curative options are limited.

Areas Covered: This review provides an overview of current systemic therapies and emerging novel approaches for BTC.

Grief-Specific Cognitive Behavioral Therapy vs Present-Centered Therapy: A Randomized Clinical Trial.

Importance: Prolonged grief disorder (PGD) is included as a new diagnosis in international classification systems. Treatments following a cognitive behavioral model are most effective, but comparisons with active control treatments are scarce.

Objective: To examine whether integrative cognitive behavioral therapy for prolonged grief (PG-CBT) is superior to present-centered therapy (PCT).

Comparative Outcome Analysis of Lenvatinib Versus Sorafenib for Recurrence of Hepatocellular Carcinoma After Liver Transplantation.

Background: Hepatocellular carcinoma (HCC) recurs after liver transplantation (LT) in ~17% of patients. We aimed to retrospectively compare the outcomes of patients treated with different tyrosine kinase inhibitors (TKIs) for recurrent HCC post-LT.

Methods: Patients with recurrent HCC post-LT between 2006 and 2019 were included.