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   atrial septal defect device closure in patients with metabolic or genetic diseases  
   
نویسنده sayadpour zanjani keyhan ,heidari morteza ,nazari nima ,alimadadi hosein ,mohsenipour reihaneh
منبع innovative journal of pediatrics - 2023 - دوره : 33 - شماره : 6 - صفحه:1 -9
چکیده    Background: the coexistence of a metabolic or genetic disease can complicate the course of an atrial septal defect device closure. methods: the database of our hospital was searched for patients who had undergone atrial septal defect (asd) device closure and had concurrent metabolic and genetic diseases. out of 188 such patients, 11 were identified. results: this cohort study included 11 patients with type 1 diabetes mellitus, insulin resistance, mitochondrial diseases, rickets, seckel syndrome, alagille syndrome, cystic fibrosis, down syndrome, and crigler-najjar syndrome type ii. the patients were followed for a median of 4 years. two patients experienced thromboembolic events. one procedure failed as the device was embolized. large devices with a waist circumference greater than 1.5 times the body weight were used in 3 patients. one patient died 19 days after the procedure due to multi-organ failure, which was not related to device closure. conclusions: in patients with metabolic or genetic diseases, this procedure may be complicated by factors such as small patient size, hypercoagulation, organ failure (cardiac, renal, or hepatic), vascular abnormalities, and issues with anesthesia or transesophageal echocardiography. it is recommended that careful attention be given to the specific challenges associated with each disease. the utilization of large devices can be considered safe, particularly in patients beyond 4 years of age.
کلیدواژه alagille syndrome ,case report ,crigler najjar syndrome ,mitochondrial disease ,seckel syndrome
آدرس tehran university of medical science, department of pediatric, iran. pediatrics center of excellence, children’s medical center, iran, tehran university of medical science, department of pediatric, iran. pediatrics center of excellence, children’s medical center, iran, pediatrics center of excellence, children’s medical center, iran. tehran university of medical science, department of anesthesiology, iran, pediatrics center of excellence, children’s medical center, iran. tehran university of medical science, pediatric gastroenterology and hepatology research center, department of pediatric, iran, pediatrics center of excellence, children’s medical center, iran. tehran university of medical science, growth and development research center, department of pediatric, iran
پست الکترونیکی rmohsenipour@yahoo.com
 
     
   
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