Objective-To report the epidemiology and ocular phenotype of retinal capillary hemangioblastomas associated with von Hippel-Lindau (VHL) disease in a large cohort of patients and to correlate patient and ocular characteristics to visual morbidity in this population. Design-Prospective consecutive case series.Participants-In 220 unrelated pedigrees, 335 patients affected with VHL disease and retinal capillary hemangioblastomas (RCHs) in at least 1 eye.Methods-Demographics of the patient population were recorded and the ocular phenotype of each patient was obtained with a comprehensive ocular examination.Main Outcome Measures-The patient population was characterized and the ocular phenotype described in relationship to tumor location, number, and extent of retinal involvement. Correlations between patient demographics, ocular phenotype, and visual function were analyzed.Results-RCHs were detected unilaterally in 42.1% and bilaterally in 57.9% of patients. No correlation was detected between the age, gender, and laterality of involvement. Eighty-seven (86.6%) percent of involved eyes had tumors that could be individually visualized; of these, tumors were commonly found in the peripheral retina (84.7%) only, and less commonly in the juxtapapillary area (15.3%). The tumor count in the periphery averaged 2.5±1.8 per eye, with 25.2% of eyes having greater than 1 quadrant of retinal involvement. Thirteen (13.4%) percent of involved eyes were enucleated or pre-phthsical; approximately 1 in 5 patients had one or more eyes so affected. Severe visual impairment (visual acuity worse or equal to 20/160) in affected eyes were more likely to be associated with increasing age, the presence of juxtapapillary lesions, and an increasing number and extent of peripheral lesions.Conclusions-This large cohort of VHL patients with RCHs has enabled a systematic and quantitative characterization of the demographics, ocular features, and visual function in VHL disease. Clinical correlations between the visual morbidity and ocular features of the disease were also performed, producing measures that can help clinicians better estimate visual prognoses based on the ocular phenotype of the disease. RCHs in VHL disease are benign tumors that have a characteristic orange-red color, a globular shape, and typically dilated feeding and draining vessels. Histologically, they are comprised of capillary-like vascular channels surrounded by large vacuolated stromal cells, and also smaller "tumorlet" cells that express stem cell markers 3 . RCHs can be highly variable in their number, size, and location. Although often slow-growing in nature and benign in their effect, they can sometimes result in vision loss and structural disruption to the integrity of the globe through exudative and tractional effects on the retina 4 .The diagnosis of VHL disease is often performed clinically, using criteria involving a family history of the disease and the detection and characteristic tumors in various organ systems 5 . Genetic testing for VHL disease is now...
Caractérisation de la myasthénie grave à l'aide de la classification clinique et de la stimulation répétitive nerveuse Introduction. Les tests de stimulation nerveuse sont essentiels pour le diagnostic précoce et le suivi des maladies neuromusculaires. L'objectif de l'étude était de caractériser et de valider cliniquement les tests de stimulation nerveuse répétitive (RNS) chez des patients atteints de la maladie neuromusculaire auto-immune chronique, la myasthénie grave (MG). Matériel et méthodes. Une évaluation clinique et des tests RNS ont été réalisés chez 30 patients myasthéniques. Les évaluations cliniques ont été classées selon la classification clinique de la Myasthenia Gravis Foundation of America (MGFA). Résultats. 30 patients myasthéniques ont été sélectionnés, avec un âge moyen de 48.57±2.4 ans et un rapport femme/homme de 2/1. La classification MGFA
Epidural steroid injections (ESIs) have been mentioned by international medical literature as an option for treatment and pain relief for patients with lumbar disc herniation (LDH). Several studies worldwide have recognized the efficacy of treating LDH with ESIs to be between 72-86%. Our study aimed to elucidate the ESIs technique's effectiveness, advantages, and disadvantages. A 30-day pre-post study in 100 LDH patients underwent the ESIs technique. Patients meeting the criteria received physical examinations and magnetic resonance imaging (MRI) scans before undergoing the method. The Visual Analog Scale (VAS) was applied to assess the patient's hospitalization condition after ESIs 1 day, 7 days, and 30 days. After 1 day of ESIs, 85% of patients responded well (10% excellent and 75% moderate), and this prevalence increased up to 95% after 7 days and 30 of follow-up. The ESIs safety assessment after 1 day of treatment recorded that 2% of patients had complications and 24% had medication side effects. After 7 days, 4% of patients had epigastric pain and no more complications. Treatment outcomes of ESIs were effective and high safety in patients with LDH.
Purpose: To evaluate the characteristics of basal gastric juice in Helicobacter pylori-positive patients before and after Helicobacter pylori eradication therapy. Methods: This was a cross-sectional descriptive study on 150 gastritis patients admitted at the Hospital of Can Tho University of Medicine and Pharmacy. The patients were divided into 2 groups: study group (Helicobacter pylori gastritis patients) and control group (non-Helicobacter pylori gastritis patients). The pH, HCO3- concentration, and activities and concentrations of pepsin, lipase, and amylase were determined before and after treatment in study group. Results: Patients with abnormal gastric juice comprised 76 % of the study population. Mean gastric potential of hydrogen (pH) was 2.31 (range: 1.64 - 7.68), while median concentration of HCO3- was 4.06 mmol/L (range: 0 - 73.04 mmol/L). The concentrations of pepsin, lipase, and amylase were 8.93, 0.93 and 1.38 ppm, respectively. Activities of pepsin, lipase, and amylase were 2.23, 0.28 and 0.04 U/mL, respectively. After the successful eradication of Helicobacter pylori, pH and HCO3- levels decreased, and there were significant differences in activities of pepsin and lipase before and after treatment (p < 0.05). Moreover, the levels of these parameters differed between patients in whom successful eradication was achieved and those in whom eradication failed (p < 0.05). The concentrations and activities of pepsin and lipase were statistically different between pre-treatment and post-treatment stages in both successful and failed Helicobacter pylori eradication categories (p < 0.05). Conclusion: Basal gastric juice differs significantly between Helicobacter pylori-positive and Helicobacter pylori-negative patients. Intragastric ammonia produced by H. pylori may have a role in the increased pH of gastric juice.
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