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Risk Also known as syndromic bile duct paucity Affects cardiac, musculoskeletal, ocular, facial, and neurodevelopmental systems Most common inherited disorder that causes chronic liver disease in children 1:100,000 births with equal gender incidence Perioperative Risks Cardiac congenital anomaly and hemodynamic…

Risk Risk of adrenal insufficiency: 1/1000 to 1/10,000 (if steroids used in prior y). With steroids >20 mg/d (cortisol equivalent), >7 to 14 d within 1 y (large variability in pt response to dose, route(s), duration and timing of prior…

Risk Prevalence 1:100,000 persons M:F ratio: 1:1.8 Perioperative Risks CV instability, labile BP, hypotension, shock Hypovolemia, hyperkalemia, cardiac dysrhythmia Limited response to vasopressors Worry About N/V and diarrhea leading to dehydration, electrolyte imbalances, and acid/base disorder. Acute adrenal insufficiency leading…

Risk Recent data estimates the incidence at 190,000 cases per year in USA. True incidence is unknown due to difficulty in defining the disease and making the diagnosis. Represents 10.4% of all ICU admissions and 23.4% of pts requiring mechanical…

Risk Prevalence: <200,000 cases of porphyria in USA, AIP: 5 to 10 in 100,000 worldwide. Highest in Northern Europeans. More commonly manifests in females, typically third to fourth decades of life. Perioperative Risks Drugs and/or chemicals that induce heme containing…

Risk People within USA: Prevalence is 40 cases/million; incidence is 3 to 8 new cases Occurs with equal frequency in men and women and most frequently diagnosed in third to fifth decades of life (5 to 20 y lag between…

Risk USA incidence of HIV: 50,000 per year USA prevalence of HIV: 1.2 million Sub-Saharan Africa prevalence: 25.8 million in 2014 USA prevalence of AIDS (HIV stages 3 and 4): 26,688 All but 8 were age 13 or older Perioperative…

Risk Incidence in USA: Unknown Present in a variety of disease states, from mild to severe systemic illness Perioperative Risks Hemodynamic instability (related to arteriolar vasodilation, acidosis, and decreased cardiac output) Hyperkalemia Insulin resistance and hyperglycemia Acute respiratory failure Worry…

Risk Incidence in USA: Unknown Present in a variety of disease states, from mild to severe systemic illness Perioperative Risks Hemodynamic instability (due to arteriolar vasodilation and decreased cardiac output) Hyperkalemia Insulin resistance and hyperglycemia Stimulation of inflammation and suppression…

Risk 1 per 15,000 to 40,000 births worldwide Females ≥ males No race predilection Most common type of dwarfism Perioperative Risks Cervical spine instability Spinal cord compression Cardiopulmonary disease Worry About Difficult airway and ventilation Central and/or obstructive sleep apnea…