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Risk Overall incidence not reported Very rare congenital disorder Preoperative Risks Very high risk of recurrent pulm aspiration; hypoplasia of both pulm and vascular components of one lung (pulm hypoplasia) High mortality rate in infancy Worry About NM dysfunction of…
Risk X-linked recessive disorder due to deficiency on the enzyme HGPRT, resulting in the buildup of uric acid Incidence ∼5.2 per million male births (where symptoms appear) Perioperative Risks Hyperuricemia and hyperuricosuria (gout) Airway problems secondary to scarification from self-mutilation…