Objective: Fungiform papillae enlargement is a common oral manifestation of neurofibromatosis type 1 (NF1). This study aimed to objectively evaluate the size, number, and symmetry of fungiform papillae in NF1 individuals and investigate the relationship between these alterations and taste perception, salivary flow, dietary habits, and BMI.
Materials And Methods: A cross-sectional case-control study was conducted on 80 participants (40 with NF1 and 40 controls), matched by age and sex.
Int J Colorectal Dis
August 2024
Introduction: The characterization of tumor microenvironment (TME) related factors and their impact on tumor progression have attracted much interest. We investigated cancer cells and cancer-associated fibroblasts (CAFs) to evaluate biomarkers that are associated with neoplastic progression, observing them in different interface zones of colorectal cancer.
Methods: On 357 CRC tissue microarrays, using immunohistochemistry, we examined the associations of podoplanin and α-SMA expressed in cancer cells and CAFs and evaluated them in different areas: tumor core, invasive front, tumor budding, tumor-stroma ratio (TSR) scoring, and desmoplastic stroma.
Objective: To evaluate the prevalence and clinical aspects of oral candidiasis in patients hospitalised in the intensive care unit.
Methods: This is a longitudinal and prospective study that included 48 participants hospitalised in the intensive care unit. Sociodemographic data, presence of systemic disorders, use of medications, laboratory tests, cause of hospital admission, type of breathing, and length of hospital stay were obtained from medical records.
J Magn Reson Imaging
May 2023
Head Face Med
September 2022
Background: The diagnosis of oral lichenoid lesions (OLL) remains a challenge for clinicians and pathologists. Although, in many cases, OLL cannot be clinically and histopathologically distinguishable from oral lichen planus (OLP), one important difference between these lesions is that OLL has an identifiable etiological factor, e.g.
View Article and Find Full Text PDFOrphanet J Rare Dis
September 2022
Background: Neurofibromatosis 1 (NF1) is a common autosomal dominant syndrome with complete penetrance and highly variable expressivity. The cutaneous neurofibroma (Cnf) and plexiform neurofibroma (Pnf), café-au-lait spots, and freckle-like lesions are common in NF1, but many other manifestations can occur. We aimed to evaluate head circumference, height, weight, body mass index (BMI), head circumference-to-height ratio (HCHR) and waist-hip ratio (WHR) in adult NF1 Brazilian individuals versus a paired control group and investigate their correlation with the presence of clinically visible Pnfs, and number of "skin neurofibromas" (Snf), which include both cutaneous and subcutaneous neurofibromas.
View Article and Find Full Text PDFBrain Behav
June 2022
Background: Neurofibromatosis type 1 (NF1) is a chronic and progressive autosomal dominant genetic and sporadic disease characterized by cutaneous and neurological abnormalities. Plexiform neurofibroma (PN), a significant cause of clinical complications in NF-1, is a benign tumor of the peripheral nerve sheath that involves multiple nerve fascicles. Although there is an important number of patients who are affected by NF1 in Brazil, there is little data on the behavior of the disease in the national literature as well as in other low- and middle-income countries.
View Article and Find Full Text PDFOral Oncol
January 2022
Dentinogenic ghost cell tumor (DGCT) represents a rare benign odontogenic neoplasm that can appear in a central or peripheral form and may rarely undergo malignant transformation to ghost cell odontogenic carcinoma (GCOC). We aim to report a case of a central DGCT with focal cytological malignant aspects. A 24-year-old woman exhibited a painful enlargement and dental mobility in the left posterior maxilla for about one year, which appeared as an expansive well-defined hypodense maxillary image with hyperdense foci invading ipsilateral maxillary sinus.
View Article and Find Full Text PDFObjectives: The aim of this study was to evaluate the mandibular condyles of neurofibromatosis 1 (NF1) individuals without facial plexiform neurofibroma using cone beam computed tomography images.
Materials And Methods: Eighty cone beam computed tomography scans (160 mandibular condyles) were analyzed: 40 from NF1 individuals (study group) and 40 from individuals without NF1 (control group). The anteroposterior and mediolateral dimensions, height, and volume of the mandibular condyles were measured.
Purpose: By incorporating major developments in genetics, ophthalmology, dermatology, and neuroimaging, to revise the diagnostic criteria for neurofibromatosis type 1 (NF1) and to establish diagnostic criteria for Legius syndrome (LGSS).
Methods: We used a multistep process, beginning with a Delphi method involving global experts and subsequently involving non-NF experts, patients, and foundations/patient advocacy groups.
Results: We reached consensus on the minimal clinical and genetic criteria for diagnosing and differentiating NF1 and LGSS, which have phenotypic overlap in young patients with pigmentary findings.
Background: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with a prevalence of 1:3000 births and a wide variety of clinical manifestations. Cutaneous neurofibromas (cNF) are among the most common visible manifestations of NF1 and present a major clinical burden for patients. NF1 patients with cNF often report decreased quality of life, emotional well-being and physical comfort.
View Article and Find Full Text PDFThe tumor stroma plays a relevant role in the initiation and evolution of solid tumors. Tumor-stroma ratio (TSR) is a histological feature that expresses the proportion of the stromal component that surrounds cancer cells. In different studies, the TSR represents a potential prognostic factor: a rich stroma in tumor tissue can promote invasion and aggressiveness.
View Article and Find Full Text PDFIntroduction: Neurofibromin, a protein encoded by the NF1 gene, is mutated in neurofibromatosis 1, one of the most common genetic diseases. Oral manifestations are common and a high prevalence of hyposalivation was recently described in individuals with neurofibromatosis 1. Although neurofibromin is ubiquitously expressed, its expression levels vary depending on the tissue type and developmental stage of the organism.
View Article and Find Full Text PDF: To investigate the efficacy and safety of deoxycholic acid (DOC) for SMF reduction.: We conducted a systematic review and meta-analysis of randomized controlled trials. We searched PubMed/MEDLINE, EMBASE, and Cochrane databases until June 2020.
View Article and Find Full Text PDFThe Ki-67 labeling index is traditionally used to investigate tumor aggressiveness. However, no diagnostic or prognostic value has been associated to the heterogeneous pattern of nuclear positivity. The aims of this study were to develop a classification for the patterns of Ki-67-positive nuclei; to search scientific evidence for the Ki-67 expression and location throughout the cell cycle; and to develop a protocol to apply the classification of patterns of Ki-67-positive nuclei in squamous epithelium with different proliferative activities.
View Article and Find Full Text PDFLichen planus (LP) is a mucocutaneous immune-mediated disease of unknown etiology. It is more prevalent in women and usually occurs between the third and sixth decades of life. Oral lesions may or may not be associated with skin and genital lesions.
View Article and Find Full Text PDFRes Rep Urol
December 2020
Purpose: Urinary antiseptics including methenamine and methylene blue are used in the symptomatic treatment of urinary tract infections (UTIs).
Patients And Methods: This was a prospective, double-blind, randomized, double-dummy safety and efficacy study of 2 urinary antiseptic combinations in the symptomatic treatment of recurrent cystitis: methenamine 120mg + methylene blue 20mg (Group A) versus acriflavine 15mg + methenamine 250mg + methylene blue 20mg + L. 15mg (Group B).
Am J Clin Pathol
April 2021
Objectives: To evaluate the expression of progesterone receptor (PR), estrogen receptor (ER), and G protein-coupled estrogen receptor 1 (GPER-1) in cutaneous neurofibromas (cNFs) and their correlation with demographic, clinical, and laboratory data of individuals with neurofibromatosis 1 (NF1). The association of PROGINS polymorphism and PR expression in cNFs, as well as the serum steroidal hormones and the number of cNFs, was investigated.
Methods: The sample comprised 80 large and 80 small cNFs from 80 individuals with NF1.
Purpose: We report the results of low back pain treatment using a combination of nucleotides, uridine (UTP), cytidine (CMP) and vitamin B, vs a combination of vitamins B, B, and B.
Patients And Methods: Randomized, double-blind, controlled trial, of a 60-day oral treatment: Group A (n=317) receiving nucleotides+B and Group B (n=317) receiving B vitamins. The primary endpoint was the percentage of subjects in each group presenting adverse events (AEs).
In vitro-expanded bone marrow stromal cells (BMSCs) have long been proposed for the treatment of complex bone-related injuries because of their inherent potential to differentiate into multiple skeletal cell types, modulate inflammatory responses, and support angiogenesis. Although a wide variety of methods have been used to expand BMSCs on a large scale by using good manufacturing practice (GMP), little attention has been paid to whether the expansion procedures indeed allow the maintenance of critical cell characteristics and potency, which are crucial for therapeutic effectiveness. Here, we described standard procedures adopted in our facility for the manufacture of clinical-grade BMSC products with a preserved capacity to generate bone in vivo in compliance with the Brazilian regulatory guidelines for cells intended for use in humans.
View Article and Find Full Text PDFThe β2-microglobulin amyloidosis is a newly recognized type of systemic amyloidosis, which occurs in patients with chronic renal failure undergoing long-term hemodialysis. The dialysis-related amyloidosis (DRA) of the tongue is an uncommon and late complication, which occurs in patients undergoing chronic hemodialysis for more than 20 years. The aim of this paper is to report two cases of lingual amyloidosis associated with long-term hemodialysis.
View Article and Find Full Text PDFJ Cutan Pathol
October 2018
Background: Lipomatous neurofibroma (Lnf) is a histopathological variant with adipocytes noted among cells of cutaneous neurofibromas. We aimed to investigate the prevalence and clinicopathological features of Lnfs of neurofibromatosis 1 (NF1)-associated cutaneous neurofibromas and to review the literature systematically. We also evaluated the expression of leptin (a hormone involved in lipid metabolism) in neurofibromas to better understand the pathogenesis of Lnfs.
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