3 research outputs found
Risk factors for mortality in fulminant acute necrotizing encephalopathy following influenza A in an adolescent boy
Key clinical Message Acknowledging the risk factors of mortality and morbidity of each disease is effective for its final outcome. Recognizing these cases can have the value of preventing the occurrence of unfortunate events, such as not recommending the use diclofenac in an influenza epidemic
Guillain-Barre Syndrome Deterioration After an Abdominal Surgery; A Case Report
Guillain-Barre syndrome is an acute ascending paralysis accompanied with autonomic symptoms like abdominal pain. Here we present a 13-year-old boy with lower extremities pain and sensory disturbances with presumptive diagnosis of Guillain-Barre syndrome who experienced severe progression of disease after receiving general anesthesia due to an appendectomy. Whether the progression was due to the natural history of GBS, immunosuppression induced by surgical stress, or usage of anesthetic medications remained unclear
The Efficacy and Safety of Rapamycin in Children with Tuberous Sclerosis: A Cross-sectional Study: Efficacy of Rapamycin in treatment of Tuberous Sclerosis
Objectives
Mutations in TSC1 or TSC2 genes have been proposed as the main causative factors responsible for developingTuberous Sclerosis Complex (TSC). Given the effect of these two genes on the mTOR pathway,rapamycin hasemerged as a novel therapeutic agent.The present study evaluated the effectiveness and safety of rapamycin on the multiple manifestations of TSC.
Materials & Methods
Twenty-three eligible children were enrolled in the present crosssectional study. They were prescribed rapamycin1mg tablet twice daily for the first two weeks of treatment and then once daily for at least one year.Periodicevaluations through follow-up visits were performed Besides, growth and developmental statuses wereevaluated. All data, including the number and size of brain tuberomas, size of renal angiomyolipomas, and skinlesions, were gathered and recorded, and then analyzed.
Results
During the study period, the mean number of epileptic episodes significantly reduced (p<0.0001), and nine cases were seizure-free at the final visit. The mean number of brain tuberomas decreased from 19.3±11.0 at the initial visit to 11.1±5.6 and 8.2±3.2 in the subsequent visits (p<0.001). The mean size of brain tuberomas similarly decreased from 17.9±18.5 cm at enrollment to 13.7±5.1 cm and 6.9±5.1 cm in the second and third visits, respectively (p=0.029). The mean size of renal angiomyolipomas significantly decreased (p<0.001). A significant trend toward a decrease in the number of skin lesions was observed (p<0.0001). No relationship was observed between the effects of rapamycin and the patient’s age or sex (p>0.05). Changes in patients’ growth and developmental features were not statistically significant through subsequent visits (p=0.507).
Conclusion
This study revealed the effectiveness and safety of rapamycin on TSC among our patient