Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + At a Glance Download Section PDF Listen ++ Very rare syndrome characterized by hemolytic anemia of unknown origin associated with early-onset emphysema and cutis laxa. Poor prognosis. + Synonym Download Section PDF Listen ++ Cutis Laxa, Emphysema, and Hemolytic Anemia; Emphysema Hemolytic Anemia Syndrome. + Incidence and Genetic Inheritance Download Section PDF Listen ++ Extremely rare (three cases described in consanguineous family); autosomal recessive. + Clinical Aspects Download Section PDF Listen ++ Features include severe congenital hemolytic anemia, early-onset pulmonary emphysema, diffuse pulmonary giant cell infiltration, hemorrhagic adrenal necrosis, cutis laxa. Two of the three cases described died of septic shock before 7 years of age. + Anesthetic Considerations Download Section PDF Listen ++ Evaluate severity of anemia (clinical, full blood count), and respiratory function (clinical, chest radiographs, CT, pulmonary function test, arterial blood gas analysis). + References Download Section PDF Listen ++Anderson CE, Finklestein JZ, Nussbaum E, et al: Association of hemolytic anemia and early onset pulmonary emphysema in three siblings. J Pediatr105:247, 1984.