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Risk FS can be inherited, acquired, or caused by exogenous factors. Incidence is sporadic. Exact incidence in USA is not clear. Most diseases associated with FS are inherited in an autosomal recessive pattern. Cystinosis is the most common cause in…

Risk Rare; hyperPP approximately 1:200,000 and hypoPP approximately 1:100,000 HyperPP with childhood onset; hypoPP with teenage onset Perioperative Risks In hyperPP, succinylcholine may provoke severe myotonia, provide no relaxation, and cause hyperkalemia (resulting postop muscle weakness over days and rhythm…

Risk Autosomal recessive transmission Complete penetrance, marked variability in expression Predominantly affects Ashkenazi Jewish population (incidence 1:10,000–20,000; carrier frequency 1:27-32) Perioperative Risks Intraop: Primarily cardiovascular with hemodynamic variability Postop: Primarily cyclic vomiting and pulmonary complications Worry About Paroxysmal dysautonomic crisis…

Risk Most common hereditary thrombophilia Autosomal dominant inheritance pattern Heterozygous form in 5% of white population in USA (up to 15% in Europe), 2% of Hispanic Americans, 1% in both African and Native Americans Homozygosity in white population 1:5000 May…

Risk Genetic disease with reported annual incidence of approximately 1:100,000, but rarity may lead to underestimation of true prevalence Panethnic Perioperative Risks Autonomic instability, particularly with neuraxial anesthesia Cardiovascular instability from conduction abnormalities and structural heart disease Periop stroke in…

Risk Prevalent in children 1–7 y; sometimes called supraglottitis, it does occur in adults (decreasing incidence in children >3 y related to vaccines against Haemophilus influenzae type B, but still found, particularly if pt is not immunized). Adult incidence remains…

Risk 1:17,000, 50% dystrophic form Racial distribution equal Perioperative Risks Difficult IV access, airway, intraop positioning, reflux, steroid dependence, intraop hemorrhage, sepsis, iatrogenic corneal abrasion, blister formation, and airway obstruction Worry About Problems similar to those found in pts with…

Risk Incidence: 3–10:100,000 population. Rheumatic heart disease is a key risk factor in lower-middle income countries. Valvular and cyanotic heart disease, DM, cancer, and IV drug use are risk factors in higher income countries. Perioperative Risks Septic embolization to other…

Risk 4% of all congenital heart disease and 0.3–0.4:1000 live births 40–50% of AV canal defects are associated with trisomy 21 Perioperative Risks Paradoxical air embolism Shunt reversal (from left to right to right to left) because of vasodilating volatile…

Risk After successful prehospital cardiac resuscitation: 59–65% of pts remain comatose. 0–5% of successful resuscitations result in chronic vegetative state. Perioperative Risks Worsening of neurologic status; blindness most common residuum. Postpone surgery in all but emergency situations. Do what is…