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Acute porphyria in an intensive care unit

Calvo De Mora Almaz√°n M, Acu√Īa M, Garrido-Astray C, Arcos Pulido B, G√≥mez-Abecia S, Chicot Llano M, Gonz√°lez Parra E, Gracia Iguacel C, Alonso Alonso PP, Egido J, Enr√≠quez de Salamanca R

Unidad de Cuidados Intensivos, Hospital de La Princesa, Universidad Autónoma de Madrid, Spain. Departmento de Nefrología, Fundación Jiménez Díaz, Madrid, Spain. Unidad de Porfirias, Hospital Doce de Octubre, Universidad Complutense, Madrid, Spain. Departamento de Ciencias Morfológicas y Biomedicina de la Universidad Europea de Madrid, Spain.

Porphyrias are rare hereditary metabolic disorders caused by the inactivity of certain
enzymes that participate in heme synthesis. We report 3 cases in which porphyria debuted
with acute episodes in young patients. As is often the case, diagnosis was delayed, and
intensive care was required for severe encephalopathy. Symptoms improved rapidly after
hemin therapy was started, but peripheral polyneuropathy persisted for several months in
1 patient. We report the first case of a porphyria-related seizure triggered by use of the
morning-after pill (levonorgestrel).

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