Acute Porphyria Attack after anesthesia : A case report - ThaiJo

(1)

Acute Porphyria Attack after anesthesia

:

A case report

Surisa Siriwong M.D.*

ABSTRACT

Porphyria is

the

group

of

diseases

with

abnormal heme metabotism. This conditions is unusual

in

Thai

population.

Patient

may

present

with muttipte

organ invotvement _such as cutaneous manifestation, neurotogic

or mental

symptoms

or both

base

on

each type

of

porphyrias. ln some cases show visceral symptoms

that

may

be

misdiagnosis

with

acute abdominal condition. Many drugs may be trigger porphyria attack

and

should

be

concerned in

treatment

ptanning.

A

36 years

old

Thai man,

was

^referred

to

Buriram hospital

with acute

abdominal pain

.

He

also

had ctinicat dehydration and severe tachycardia intraoperative period with no abnormaI surgicaI condition in peritoneum. Postoperative period he had dark urine . After investigation shows positive urine spectophotometry and urine porphobitnogen is detected.

His diagnosis

is acute

porphyria attack.

ln this

case

offered

symptomatic

and

supportive

treatment with

analgesic

and

high gtucose

diets.

His

symptom

was resolved

and he

had discharged

from

hospital

in 2

week. Retrospective review shows induction

with

thiopental.

may be precipitated this attack.

Key words :

Porphyria, Acute porphyria attack, AbdominaI pain with change in neurologic or men-

tal

symptom, dark urine.

*Department of anesthesiotogy, Surin Buriram, Buriram province

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trophotometry lviarau':n ifrodarflu

Acute porphyria

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high ^gtucose

diet rfluriru?'ru tianl:m:?o urine

porpho-

bitinogen 104.5 uglml.

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Chemistry

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!

Porphyrias

a"rlrl:nutj{

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u z

n{ulraqi1(')

lfiuri erythropoietic

porphyria

uav

hepatic porphyria fi lfi or nr:vr1{nAfr n ua

nrix

riu I:n"Lu

n{l

erythropoietic porphyria

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ahi

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Porphyria (AlP), Hereditary Coproporphyria (HCP), Var-

iegate

Porphyria(VP),

ttau

P[umbopophyria (PLP)

tun{ud Acute lntermittent

Porphyria

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oI

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<ia

Iqeififiornr:u'r{an niu anxiety,

depression, confusion r,ttofi or nr:v'r{:uLJUtJ :u arvrir:rfir u

dr v , ., f j

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periphera|.neoropathy 1oufr

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involve motor

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hyperten- sion(o'8)

ttaufin?']!finrJnfrtrol

hypothal.amus n:srlu"L#rfrrrnmv SIADH

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.i d u o & ! r- t

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^ou

du@sy

ua{""'

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6 ut, 3 J Jo@ea <

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Luun0{n?1}J

a,a.d,idui

iln!nn no !n1:tuauua?o{uaal?u

{.11?{[L:n

rfioirlriunl?u

severe tachycardia

flo{{thu v,-,d,

nuu:nara'uirt[]uornntrv dehydration

uau

ern fl'r?u peritonitis uorri'ilra

ra6'rorntuilt n1?v dehydration

lrfl(rhaudr

rirn.:fifl

aar:s

ilfrnoej _Ugfr,r-hufinr:t# btood

component fiofit

nl?s

hemoglobinuria

dranr:n:?{

Coomb

qv J - A ,

test'lfrruaau dail:su?arYuo1nl:6uuor{rJ'ra

ld'uri o r n r :rJ r rr fr'o r :u _i u:

r

^rfiô^rilôâi^rⁿô^tiô^r^fro

r

tr.ivrunrurfi nr,Jno fi

nmv

tachycardia d.:flnfiI

nmv

porphyria 6.:16'lirn1:Ft:?o spectrosco-

.j o { , , - jjd

py lru rlJoul urine

[ua0{u{ytyttJ[a{a']1{

flu

r d j a C, aZ

?1il n1::?tJ naila _t n nu n 0u L[au tu a uua [uuau''l n1aod1{d'atau

uayrianl:s}:?0

urine porpho-

bitinogen lfi'ruauln(") rrmlu(r-ha:rud

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hinuor

nr:fr nrjnm^mrl:uuuil:vff

.tyt rractilfi

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nou

nouilulyt={il?u uu"l

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ur 26 0!!r ³ nu8in - Eu?ln! 2554

ovfiornrnJ:nfrol {rhatfrfi.rur prednisolone('2)

porphyria

6o

Hematin

filrfluurilrm:grudt{oqi inurnrrv

nephrotic syndrome

d.rrfluurlun{u lufloq-Tu

uusiororio"Lfirfionrrvuyr:ndou6u1

ld

Possibty porphyrinogenic uavtu:vy'jxnr:

ah6'o fnhu16fu _!

nr:rhaauffruar

Thiopentat r{hr

ur'Lun{:r

Documented porphyrinogenic("''o) id,--i

fi.:orortJufl oo'ufi trigger

l#I:nfi quu:rf,u dru o1n1:to{T:u n"pf,roti. ryndro." fi{rhu

lor u- v t d , e

:'r uutJ Ln:u

n1:?u0fl

u

n0uil0r nr:rJ':nyrol tJ:vlrru z illqti finr:finr*rnu'jr

nephrotic synd

rome orou'eitutn'urtiolnlu

porphyria

^ tu d,, - .j e.

Ul.ltuo unlJnt!utuetyltUu

Erythropoietic porphyria('5)

trnn'jr

Hepatic porphyria rufiou

vtt X

,.r1J?U:1UU

n1:Ynu1 fr)ru Acute (Hepatic) porphyria niut u u u r ^yrr ^.:t o r fl a ^.:rYut u n : ni fi r n u fi r.J : s

f

^B

ddi'd

u5ofr nmv rfr

u{}Jrriou yrnilornr:rrao.:rr#rtvi

inurulurJ:v6'uti:vnol niu nt:n?unilntta

udsr-'d'ot!

4ilil''rrutvr [qutafl1u0u'r{u{tri0Lnfl

acute porphyrl. crisis

orofinl?u

severe tachycardia

n 1: rfl

r:

^u

i{

nr

u

^fi^qrJ^nGr odail o a 6 15 nTn

:tad n1:u::ryt1r'trlvtJrnuavo'1fl1:

n6u1#or16uu torfrr-h _Ut

u

n1:n?unilo1n1:yt1{:c!ur.J:yary niu

f,n

rafo peripheral neuropathy mvo1ofro{d't

o r J J ytt d <

:u?.:9r0[u0{ tuo{a1n r{u?u01ail01n1:ya{tua0

oa'td'urufir

_rJ

r fl lqu#{d

oiorrfionurfifrlonra

tau{uouo0n1:n:unuflrrs acute

Avty porphyria

tunq'l Safe uas possibly not

porphyrinogenic("'o)

n1:;nuluuuo"r tnrvtory0.:(3'15'16) fi

nt:

"L# _carbo hyd

rate

loa d i n

g

ryr' 0lfr'uij ufl r 6'u rio

u ndurfirau ro{ Louhi

ALA synythetase

6l r{iurouts}ifluriulunr:n:cu?un1:aflr

heme precursors Tnut# glucose

tuo'sr:r 20

nYtqio

d'rll.: raio

400

nilqio{u

fintd'zuatun:fifirflu

niu acute renaI failure, throbophleblitis

yio dose retated

coagutopathy

toi

"LuorlSnrfi

n1:dl heme

arginate

lr"Ldlunr:inrgr

ldrrri panhematin dose 3-4 mg/kg/day

liriri{r-hufi

aSudos

01nl:quJ

uacao0n:1nl:n:0.1 telu{ [u L:{fl u1u1a fi n r :fi nur"[u#n {yrn a

orvru'ir

nr:"Lv1a

r

^ci^meti-

dine arrr:nfl

or n1unr:nau r{tu.u3rld urlhifi l.ra e1 n1:yr ^qao.:luruuti(

")

lu

ui r o r n r : fl o r rYu iir r{J u fr'o

rtri'rirh

u

a 4 s ! a, u u - '

:uvr:1u0{

L:nu

ruu

n1::u?{ uaurl

olnunrrn

-i r qu s .5v, d

tau{n0n1:ltu1tu9el1{61

[0uu1ytltu0u

uaytJ q sl, _-<t_r.r-_|- - ^_(12_14)

nfuailU9ltuu

porphyrinogenic _vrttUOUl' niu

urrTu{nndt

barbiturates, u1t't Lidocaine,

Ulaau tdu

Thiopentat, ketamine ulann?1il nlu niu spironolactones 14io methytdopa ur

6'rutfio

u!

nju

Cotrimoxazote

uio

Rifampicin,

oqAulyu

ulnlJnltuo rljuau

uonQ1flu

010rl0{afl!a{u tfrureionr:n:ym'unr:rfiqnrrv

i,e porphyria

odrr

A qu r d A c d, v

0u lottn n?lil!n:un

n1:9tilttoanosoa ruuau

y9

uonnlnfltuan; nrrsfr'rn::rioron:vfr'ulfrrfi

_I o

acute porphyria attack

Li' 6'lrfu

nr:raf,nrdul

!"od.

n1:n.:n::nnluLu

1

ilu:nualrfln

attack ryr:ry

n

rr vilr

erfr'o

{r

ruu e}s{ n

::do

r o

lirl#rfi

rr nr r

l

u q us 4u \y4

a!au

tunl:?u0a u [:n uayil 0a :1n1:9] 1 u fl { [9tn{

42 ^o/o(18)

A v', e v u

tlJor,llJ?u _ur

porphyna

n0{[1J1:uu:n1:

aU d 4 a a4 v

il1{? A

ryru

^n1:^ra⁰^flyt^Q'r:tu1't⁰

n1::v{un?1il

v49atu-.J

tann0{fla1:il't0u't{:utJo:c'1.1

g tuo{Q1n fl1?u

4 I u y oova

tn:ua01nn1:rilrloolQn:u9luyt't [14tnfl Acute

porphyric crisis trT"''o) lun:fitdiEnr::vl'u

nmuff; n

uuurf:hi

tv{rd o nt{u rlun

q'l

^pro pofo ^t Midazota m ua'n rd

al

urrjraau nq'l Thiopenta ^L

Ketamine Etomidate Dizepam doq'lun{l

m i [d case ^u^rvtriir tu r v uo'r uo{ ri o n

1:inur

ac ute

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504 ^n?61^nn{6 ?1tal5n1lltrr!

l:rrrnr rraaia:rnr a3uri riirJ

Hyperkalemia lu{r.Jraffio1n']: motor weakness

tr4uw

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^fi¹ⁿ^mOnn1::v{U n?l}J:An LtUU tA?\1UA?U

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fl -Jo ta

u

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ul

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aa!

u1l4r.ioundlurilo tvA

u''ruriil?andil

opioids

y 4. t e g i- qq

n a 0 o xu u 1 ttn qyl DU 1 14 u ou n

alll

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aur:nrfionlffLund!

^5HT3antagonists

r,tdntdarnr:"Ld metoctopramide u'tttrillel Diclofenac eirrailnr:'L{

succinyl.choIine

tu{rJru porphyrias

ar}rr:olfflfrodrlrJaoqfiu

,ux rd 4 Au i -

LLgrU{ t}Jiln1:finU1vrt9rL8Utn U? nU n',l:

tnqnl?u

orr fr'or:vfirr:ui{or nr:vr1.i:vuuil

:v 41il ua v riu fr n n r : n : r r i r r n r u"tfrt'pr ^tou ri o u

lir

fi'n n n r

:

i4

tuau u'r11fr a]].r1:fl

Laontd Li'rrri

bupivacaine

radnrdurnr:ld Lidocaine riounr::sSlnmu

y<vd..J

idnnarrlnr:

oremedication Lfloann1?v stress

gr j

Lrau

n1:oeto1141:{{o10n:voiubi :uv'i1{n1:

uY4auYaY4

:u{un?lil:anttauva{:u{un?1il:an fl0{iln1:

_gU

rfl

r:virfi

rvil1ualJ

6'onr:tuittn?1iln1?s

auto- nomic dysfunction uav sequatae ttiu myocar-

dial

ischemia utav heart faiture uflud'u naoo

outJ:vffiunruf,f;nnruav

cognitive function

U

odrr:vrJpr:vir

-4da

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ri ouuta orotl^16'iu nr

ui:lfi

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r

nt:tolT:nvt'lt'

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u

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r

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neurovisceraI symptoms

6l

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lfr

drvtud

il1nil o 1n 1:u ^?oyt0

{:u

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o n

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fi

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?uaau uu uufrl? uq { n a 1? ua'r

Lunl::

nu1 Fl 1il ttu?vt1{il:u6'urJ:vnofl# glucose loading uav

o jia a o v u I

tuvtvltJUl Hematin o10Yr01:fu1Lun1::nu10?u

d,dq,

ultuun:ru1

LU u1nflu:v14?1{n1:ar1na

flu1u'l!

lro{141u1fi014n:u{u _t porphyria

radnGulnl:ld

urd'r

ndrr

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,flu vrnfrr-hErfiil:vlTodrGu

_UUT'

nr:lfrnrruirfi ur

ri'ufl o {u

-;

ua v u 1fr o ¹0 n : v 6'u1:n _t! urri frr-h ufi nr r u

t6'odruaurvar

fisrfin::rrJ:snro

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BnTil:

nsl'',il6una_{U Ui} o'r u rg: r[?\ il dI: ^{^fl^{u 1}^u¹^{a a u}rt { rvr :

r

^riti'r^utfi'n'r

r]inrgruuvrir rn urniu

nr:ifrodal:n

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tr

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a?ua?::ri

fi'a u uruvr tiuirr

ostt

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un::il I:rilurura

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