4-Hydroxybutyric acid (4HB) is accumulated in succinic semialdehyde dehydrogenase deficiency, an inherited metabolic disease severely affecting the CNS during postnatal development. Thus, the present study was designed to evaluate the in vitro influence of 4HB on lipid synthesis and CO2 production from [U-14C] acetate in cerebral cortex of 30-day-old Wistar rats. In the presence of 4HB, there was an inhibition of lipid synthesis in cerebral cortex prisms and homogenates. However, no inhibition of lipid synthesis occurred in the homogenates free of nuclei and mitochondria. In addition, CO2 production was inhibited by 4HB in cerebral cortex prisms, and homogenates and in the mitochondrial fraction. These results might possibly be explained by an impairment of mitochondrial metabolism by 4HB which may secondarily inhibit lipid synthesis. The results reported here may help to better understand the neuropathophysiology of 4-hydroxybutyric aciduria.
Introduction: Hearing loss causes comprehension difficulties, worsens speech perception and discrimination, and decreases the deaf quality of life. Objective: To describe the results of variations in impedance measurements (IM) and the advances of hearing categories in cochlear implant (IC) patients. Method: Qualitative, descriptive, and longitudinal study. Three consanguineous patients implanted and treated in speech therapy with aurioral approach. Telemetry of five-step neural responses was recorded, followed by impedance measurements, sound location tests, simplified Glendonald hearing detection procedure (GASP) tests, Ling sound tests, and recognition of vowels and words. All subjective measures classified the state of hearing categories that ranged 1 (no sound recognition and no oral communication) to 5 (sound localization and oral communication established). The follow-up period was 12 months. Results: There was an overall increase in impedance measurements in all implanted ears. Training in sound localization, auditory memory, auditory closure, background figure, and temporal ordering skills promoted better speech performance. It was noticed that hearing abilities development was adequate because of restored social hearing and communication. Conclusion: The variation of Impedance Measures was continuous and progressive and was concomitant and proportional to the performance improvement of hearing categories, ranging from condition 1 to 5 which improved oral communication in these cases.
Funding Acknowledgements Type of funding sources: None. INTRODUCTION Left atrial cardiopathy (LAC) is an independent predictor of atrial fibrillation (FA) and embolic stroke. It is more frequent in patients with embolic stroke of undetermined source (ESUS) than in non-embolic strokes. The current definition doesn’t include supraventricular ectopy. AIM The aim of this work was to describe the importance of LAC in ESUS and to study the impact of adding the number of atrial premature complexes per hour (APC/h) to LAC criteria. METHODS Retrospective analysis of 123 ESUS patients (pts) admitted to Neurology service from 2014 to 2019. LAC was defined according to two criteria (LAC2: severe left atrial enlargement or p-wave terminal force in lead V1 [PTFV1] >5000 µV*ms) or 3 criteria (LAC3: additionally, >30 APC/h). Survival analysis for the occurrence of AF, stroke recurrence and death according to LAC2 and LAC3. Diagnostic test performance analysis for each criterion with ROC curves. RESULTS 43 (35%) of the ESUS pts had LAC2. Pts with LAC2 (35.0%) were older (p = 0.007), more frequently had hypertension (p = 0.004) and lower total cholesterol levels (p = 0.044) than patients without LAC2. The incidence of AF (median follow-up 21 months, IQR = 9-35) was higher both in LAC2 (p = 0.038) and LAC3 (p = 0.001). There were no differences in stroke recurrence or death between patients with or without LAC2 or 3. Among the 3 atrial dysfunction criteria included in LAC3 definition, the number of APC/h was associated with a higher area under the curve for the occurrence of AF (AUC = 0.822). Cox regression revealed that PTFV1 > 5000 µV·ms (HR = 5.12, IC95%=1.28-20.56, p = 0.021) and >30 APC/h (HR = 13.02, IC95%=3.57-47.56) were independent predictors of AF. In addition, the single predictor of the composite endpoint (occurrence of AF, stroke recurrence and death) was >30 APC/h (HR = 5.2, p < 0,001). CONCLUSION In ESUS pts, the subgroup with LAC2 had different clinical characteristics and a higher AF incidence. APC/h were also independently associated with AF incidence and had better diagnostic test performance than the other criteria. In sum, APC/h inclusion as a diagnostic criterion for LAC should be considered and may help in a better therapeutic approach.
BACKGROUND: INCA estimated, for 2020 – 2022, Brazil would have 8,460 new pediatric cancer cases and 5% are bone cancer. Pain is the most prevalent symptom and is present in 75% - 90%. Advanced ill patients have 40% pain undertreated. OBJECTIVE: This study was to evaluate pain in pediatric bone cancer patients and investigate if there was a difference between those who survived and those at end-of-life. PROCEDURE: Patients were registered at INCA Pediatric Department, January 2011 – October 2016, with the diagnosis confirmed of primary bone cancer and under 19 years old. Pain was evaluated and registered at three moments during their treatment: at registration, three months after and the last visit before the end of this study or patients death, using pain scores. RESULTS: 157 patients were bone cancer, 15 (9.6%) had lost the follow up; 142 were analyzed, osteosarcoma 69.7% and Ewing sarcoma 30.3%, metastatic patients 50.7%. At the registration 53.5% had pain, 69.71% were receiving pain treatment and 42.25% had pain medications changed. Comparison of the three study moments was observed a decrease of pain status, with the absence of excruciating pain, and an increased use of opioids. There was no difference (p = 0.68) in pain status between groups of who survived and who died (39.4%). CONCLUSION: Pain management resulted in reduction of pain complaint and reduction in pain intensity, together with increasing opioid use. End-of-life patients did not have more pain than others, but disease progression was associated to more pain.
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