Chylomicron retention disease, also known as Anderson's disease, is a rare hereditary hypocholesterolemic disorder, recessive inherited, characterized by nonspecific symptoms as abdominal distension, steatorrhea, and vomiting associated with failure to thrive. We describe a patient with failure to thrive, chronic diarrhea and steatorrhea who the diagnosis of chylomicron retention disease was established after several months of disease progression. The genetic study confirmed a homozygosity mutation in SAR1B gene, identifying a mutation never previous described [c.83_84delTG(p.Leu28Argfs*7)]. With this case report the authors aim to highlight for this very rare cause of failure to thrive and for the importance of an attempting diagnosis, in order to start adequate management with low fat diet supplemented with fat-soluble vitamins, reverting the state of malnutrition and avoiding possible irreversible and desvantating complications.
Diagnosis ; Diarrhea ; Diet ; Disease Progression ; Failure to Thrive ; Humans ; Malnutrition ; Rare Diseases* ; Steatorrhea ; Vitamins ; Vomiting

Background/Aims: Metastases of malignant melanoma (MM) are rare and associated with poor prognosis. The objective of this study was to analyze the clinical and endoscopic characteristics of gastric metastases of MM by systematically reviewing cases and case series involving patients diagnosed using upper gastrointestinal endoscopy. Methods: The PubMed and LILACS databases were searched. Reports containing individual patient data were included. Outcomes such as clinical data, endoscopic findings, treatments, and survival were analyzed. Results: A total of 88 studies with individual data from 113 patients with gastric metastases of MM were included. The primary sites of MM were the skin (62%), eyes (10%), and mucous membranes (6%). Most patients (56%) had multiple metastases in the stomach, located predominantly in the gastric body (approximately 80%). The overall survival rate at 2 years was 4%. There was a significant reduction in the survival of patients with multiple gastric metastases compared to that of patients with single metastasis (hazard ratio, 0.459; 95% confidence interval, 0.235−0.895; p=0.022). Conclusions Gastric metastases of MM have a poor prognosis, especially in patients with multiple implants in the stomach. Additional studies are needed to verify whether ocular and mucosal melanomas are associated with a higher risk of gastric metastases than that of cutaneous melanomas.

OBJECTIVES: In this study, we evaluated the influence of different radiant exposures provided by single-peak and polywave light-curing units (LCUs) on the degree of conversion (DC) and the mechanical properties of resin cements. MATERIALS AND METHODS: Six experimental groups were established for each cement (RelyX ARC, 3M ESPE; LuxaCore Dual, Ivoclar Vivadent; Variolink, DMG), according to the different radiant exposures (5, 10, and 20 J/cm²) and two LCUs (single-peak and polywave). The specimens were made (7 mm in length × 2 mm in width × 1 mm in height) using silicone molds. After 24 hours of preparation, DC measurement was performed using Fourier transform infrared spectrometry. The same specimens were used for the evaluation of mechanical properties (flexural strength, FS; elastic modulus, E) by a three-point bending test. Data were assessed for normality, after which two-way analysis of variance (ANOVA) and post hoc Tukey's test were performed. RESULTS: No properties of the Variolink cement were influenced by any of the considered experimental conditions. In the case of the RelyX ARC cement, DC was higher when polywave LCU was used; FS and E were not influenced by the conditions evaluated. The LuxaCore cement showed greater sensitivity to the different protocols. CONCLUSIONS: On the basis of these results, both the spectrum of light emitted and the radiant exposure used could affect the properties of resin cements. However, the influence was material-dependent.
Elastic Modulus ; Fourier Analysis ; Fungi ; Resin Cements* ; Silicon ; Silicones ; Spectrum Analysis

Jaboticaba is a Brazilian berry, which is rich in fibers and bioactive compounds and shows high antioxidant and antiproliferative activities. Prostate cancer (PCa) is the second most common type of cancer among men and its progression is influenced by androgens and inflammation. Previous studies reported the ability of the jaboticaba to modulate pathways involved in prostate diseases. The main objective of this study was to provide significant data about molecular targets of the jaboticaba peel extract (JPE) and its mechanisms of action in PCa cell lines with different androgenic status (LNCaP and PC-3). The results showed that JPE was able to decrease cell viability in both cell lines. LNCaP showed more sensitivity to JPE exposure, indicating the efficacy of the JPE treatment in terms of androgen responsiveness. JPE showed a distinct hormone dependent effect on the NF-κB signaling, with reduced NF-κB levels for LNCaP and increased NF-κB levels in PC-3 cells. Mechanisms related to cell death by apoptosis were stimulated after the JPE treatment, modulating B-cell lymphoma 2 and BAX for LNCaP and PC-3. Particularly for PC-3, the JPE treatment resulted in cytokine-cytokine receptor interaction activation mostly by up regulating pro-inflammatory, pro-angiogenic, immunostimulatory and immunosuppressive genes. Also, a set of genes related to angiogenesis and metastasis were down-regulated by JPE. In conclusion, JPE exerted an antitumor effect on PCa for both cell lines which can be enhanced if androgenic reliance is considered.

This work aimed to evaluate the effects on renal tissue integrity after hydroxyethyl starch (HES) 130/0.4 and Ringer's lactate (RL) administration in pigs under general anesthesia after acute bleeding. A total of 30 mL/kg of blood were passively removed from the femoral artery in two groups of Large White pigs, under total intravenous anesthesia with propofol and remifentanil. After bleeding, Group 1 (n = 11) received RL solution (25 mL/kg) and Group 2 (n = 11) received HES 130/0.4 solution (20 mL/kg). Additionally, Group 3 (n = 6) was not submitted to bleeding or volume replacement. Pigs were euthanized and kidneys were processed for histopathological and immunohistochemical analyses. Minimal to moderate glomerular, tubular, and interstitial changes, as well as papillary necrosis, were observed in all experimental groups. Pre-apoptosis and apoptosis indicators were higher in pigs that received HES 130/0.4, indicating a higher renal insult. Both HES 130/0.4 and RL administration may cause renal injury, although renal injury may be more significant in pigs receiving HES 13/0.4. Results also suggest that total intravenous anesthesia with propofol and remifentanil may cause renal injury, and this effect can be dose related.
Anesthesia, General ; Anesthesia, Intravenous ; Apoptosis ; Femoral Artery ; Hemorrhage ; Kidney ; Lactic Acid ; Necrosis ; Propofol ; Starch ; Swine

Background: and Purpose Recent studies suggested an increased incidence of cerebral venous thrombosis (CVT) during the coronavirus disease 2019 (COVID-19) pandemic. We evaluated the volume of CVT hospitalization and in-hospital mortality during the 1st year of the COVID-19 pandemic compared to the preceding year. Methods: We conducted a cross-sectional retrospective study of 171 stroke centers from 49 countries. We recorded COVID-19 admission volumes, CVT hospitalization, and CVT in-hospital mortality from January 1, 2019, to May 31, 2021. CVT diagnoses were identified by International Classification of Disease-10 (ICD-10) codes or stroke databases. We additionally sought to compare the same metrics in the first 5 months of 2021 compared to the corresponding months in 2019 and 2020 (ClinicalTrials.gov Identifier: NCT04934020). Results: There were 2,313 CVT admissions across the 1-year pre-pandemic (2019) and pandemic year (2020); no differences in CVT volume or CVT mortality were observed. During the first 5 months of 2021, there was an increase in CVT volumes compared to 2019 (27.5%; 95% confidence interval [CI], 24.2 to 32.0; P<0.0001) and 2020 (41.4%; 95% CI, 37.0 to 46.0; P<0.0001). A COVID-19 diagnosis was present in 7.6% (132/1,738) of CVT hospitalizations. CVT was present in 0.04% (103/292,080) of COVID-19 hospitalizations. During the first pandemic year, CVT mortality was higher in patients who were COVID positive compared to COVID negative patients (8/53 [15.0%] vs. 41/910 [4.5%], P=0.004). There was an increase in CVT mortality during the first 5 months of pandemic years 2020 and 2021 compared to the first 5 months of the pre-pandemic year 2019 (2019 vs. 2020: 2.26% vs. 4.74%, P=0.05; 2019 vs. 2021: 2.26% vs. 4.99%, P=0.03). In the first 5 months of 2021, there were 26 cases of vaccine-induced immune thrombotic thrombocytopenia (VITT), resulting in six deaths. Conclusions During the 1st year of the COVID-19 pandemic, CVT hospitalization volume and CVT in-hospital mortality did not change compared to the prior year. COVID-19 diagnosis was associated with higher CVT in-hospital mortality. During the first 5 months of 2021, there was an increase in CVT hospitalization volume and increase in CVT-related mortality, partially attributable to VITT.