RT Book, Section
A1 Laliberte, Bryan
A2 Freeman, Brian S.
A2 Berger, Jeffrey S.
SR Print(0)
ID 1135741323
T1 Porphyria
T2 Anesthesiology Core Review: Part Two Advanced Exam
YR 2016
FD 2016
PB McGraw-Hill Education
PP New York, NY
SN 9781259641770
LK accessanesthesiology.mhmedical.com/content.aspx?aid=1135741323
RD 2023/03/21
AB The  porphyrias  are  a  group  of  inherited  disorders  of  hemoglobin  biosynthesis,  commonly  classified  according  to  the  broad  definitions  of  neuropsychiatric,  dermatological  (cutaneous),  or  mixed.  Other  classifications  of  the  porphyrias  are  based  on  the  deficient  enzyme,  the  location  of  abnormal  porphyrin  production,  or  according  to  the  risk  of  an  acute  attack  (Table  101-1).  There  are  eight  susceptible  enzymes  in  the  biosynthetic  pathway,  each  characterized  by  accumulation  of  porphyrins  in  body  tissues  as  a  result  of  overproduction  or  accumulation  of  porphyrin  precursors  (Figure  101-1).  Abnormal  porphyrin  production  is  seen  in  either  the  hepatic  or  the  erythropoietic  system.