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Very rare syndrome characterized by hemolytic anemia of unknown origin associated with early-onset emphysema and cutis laxa. Poor prognosis.

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Cutis Laxa, Emphysema, and Hemolytic Anemia; Emphysema Hemolytic Anemia Syndrome.

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Extremely rare (three cases described in consanguineous family); autosomal recessive.

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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.

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Evaluate severity of anemia (clinical, full blood count), and respiratory function (clinical, chest radiographs, CT, pulmonary function test, arterial blood gas analysis).

Anderson CE, Finklestein JZ, Nussbaum E, et al: Association of hemolytic anemia and early onset pulmonary emphysema in three siblings. J Pediatr105:247, 1984.

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