WebWe present our experience and discuss the different anesthetic and surgical problems in the management of a giant umbilical hernia in a patient with Hurler’s syndrome. Case report. An 11 year old girl with Hurler’s syndrome presented with a giant umbilical hernia 31*22 cm in diameter with skin necrosis opposite (Figure 1). WebChildren with Hurler syndrome frequently present difficulties with airway management that increases with age. About half of direct laryngoscopy and tracheal intubations are …
Anesthetic management in children with Hurler’s syndrome …
WebHurler syndrome is an inherited condition caused by a faulty gene. Children with Hurler syndrome lack an enzyme that the body needs to digest sugar. As a result, undigested sugar molecules build up in the body, causing progressive damage to the brain, heart, and other organs. Symptoms most often begin to appear between ages 3 and 8. WebHurler’s Syndrome is a rare genetic disorder characterized by a deficiency in the enzyme α-L-iduronidase, leading to an accumulation of glycosaminoglycans in the soft tissues. masonic auto emblems metal
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WebThe problem in Hurler’s syndrome is the deficiency of L-iduronidase and the deposition of acid mucopolysaccharide in every system of body. The clinical status of this disease has some properties, which influence its anesthesia. In this case we reviewed the difficulty of airway management in Hurler’s syndrome. Keywords: Hurler Syndrome ... Web27 mrt. 2024 · Anesthesia Implications of Syndromes and Unusual Disorders This Appendix contains brief descriptions of many common syndromes and their associated anesthetic considerations for safe pediatric anesthesia practice. Also described are some quite rare syndromes that have important anesthesia considerations. Web12 jul. 2024 · Introduction. Hurler syndrome was first described by German pediatrician, Gertrud Hurler in 1919. It is one of the 11 disorders of the mucopolysaccharidoses (MPS). Hurler syndrome is considered as mucopolysaccharidosis type I (MPH I) and formerly known as gargoylism. In 1962, a milder form of MPS I was identified and named as … masonic band camp