Lysosomal acid lipase deficiency (LAL-D) is an underdiagnosed autosomal recessive disease with onset between the first years of life and adulthood. Early diagnosis is crucial for effective therapy and long-term survival. The objective of this article is to recognize warning signs among the clinical and laboratory characteristics of LAL-D in pediatric patients through a scope review. Electronic searches in the Embase, PubMed, Livivo, LILACS, Web of Science, Scopus, Google Scholar, Open Gray, and ProQuest Dissertations and Theses databases. The dataset included observational studies with clinical and laboratory characteristics of infants, children and adolescents diagnosed with lysosomal acid lipase deficiency by enzyme activity testing or analysis of mutations in the lysosomal acid lipase gene (LIPA). The reference selection process was performed in two stages. The references were selected by two authors, and the data were extracted in June 2020. The initial search returned 1593 studies, and the final selection included 108 studies from 30 countries encompassing 206 patients, including individuals with Wolman disease and cholesteryl ester storage disease (CESD). The most prevalent manifestations in both spectra of the disease were hepatomegaly, splenomegaly, anemia, dyslipidemia, and elevated transaminases. Vomiting, diarrhea, jaundice, and splenomegaly may be correlated, and may serve as a starting point for investigating LAL-D. Familial lymphohistiocytosis should be part of the differential diagnosis with LAL-D, and all patients undergoing upper gastrointestinal endoscopy should be submitted to intestinal biopsy.

This overview aimed at assessing botulinum toxin type A (BoNT-A)’s effectiveness in managing bruxism compared to a placebo, the absence of treatment, or other interventions in adults. Only systematic reviews (SRs), with or without a meta-analysis, were included. A comprehensive literature search was conducted on 16 June 2024, encompassing seven databases and grey literature sources. Experts and reference lists of the included SRs were also consulted. Study selection was conducted in two phases by two independent authors. Methodological quality was evaluated using AMSTAR-2. Overlap was assessed using the corrected covered area. The 14 included SRs addressed several outcomes. In most studies, BoNT-A showed effectiveness in reducing pain (n = 10), the frequency of bruxism events (n = 7), and the maximum bite force (n = 5). None of the SRs effectively evaluated BoNT-A’s impact on functional movements. All of the included SRs scored “critically low” and “low” confidence rates in AMSTAR-2. Only one SR attempted to assess the certainty of the evidence, although unsuccessfully. The overlap across included SRs was rated as “high”, with a corrected covered area of 10.11%. The current systematic reviews on BoNT-A for bruxism lack methodological quality, limiting the reliability of their findings. Despite evidence indicating a potential reduction in pain, bruxism events, and the maximum bite force, methodological limitations prevent definitive conclusions from being drawn. High-quality research with standardized methodologies is essential to establish BoNT-A’s efficacy and support evidence-based clinical practice.

Thiazolidine scaffolds have been investigated for decades, due to their wide range of biological activity. In this way, the main objective of this systematic review was to elucidate the anti-inflammatory activity of thiazolidine derivatives against nitric oxide (NO) production in lipopolysaccharide (LPS)-induced RAW 264.7 macrophages. From 9718 identified registers, 13 articles were included, where 11 studies evaluated thiazolidinediones. The summary of relevance demonstrated that seven studies (53.8%) were relevant without restrictions, and 6 (46.2%) were relevant with restrictions. The certainty in cumulative evidence was considered moderate and the six studies included in the meta-analysis demonstrated the positive activity of thiazolidinediones against NO production when compared to the negative LPS control.