Protocol For Patients With Suspected Acute Porphyria. / Protocolo de actuación en pacientes con sospecha de porfiria aguda.
Rev Clin Esp
; 220(9): 592-596, 2020 Dec.
Article
en En, Es
| MEDLINE
| ID: mdl-32143835
Porphyrias are a group of congenital errors in porphyrin metabolism and in the heme biosynthetic pathway. Accumulation of porphyrin precursors (delta-aminolaevulinic acid and porphobilinogen) is responsible for the neurovisceral crises of acute porphyria, which, when expressed clinically, start with intense abdominal pain. During crises, the urinary elimination of porphobilinogen and delta-aminolaevulinic acid is always very high. Excessive porphobilinogen concentration in urine is easily identified using the simple Hoesch test. A negative test rules out a current porphyric crisis. The clinical protocol for patients with acute abdominal pain of unknown origin in whom a positive Hoesch test leads to the suspicion of acute porphyria is based on the following aspects: initial clinical assessment in the emergency department, suppression of potential triggers, specific treatment for the crisis with hemin and/or glucose overload and symptomatic treatment.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Tipo de estudio:
Guideline
Idioma:
En
/
Es
Revista:
Rev Clin Esp
Asunto de la revista:
MEDICINA
Año:
2020
Tipo del documento:
Article
Pais de publicación:
España