A Guide fosdsasdr People With Porphyria

7/25/2019 A Guide fosdsasdr People With Porphyria

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A GUIDE FOR PEOPLEWITH PORPHYRIA


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I#de$

WHAT IS O!H"!IA #$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ 1A%&T' I(T'!)ITT'(T O!H"!IA *AI+$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$2-A!I'.AT' O!H"!IA *-+ and H'!'/ITA!" %O!OO!H"!IA *H%+$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$&)OO!H"!IA *+$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ O!H"!IA %&TA('A TA!/A *%T+$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ '!"TH!OOI'TI% !OTOO!H"!IA *'+$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$%O(.'(ITA O!H"!IA *%+$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$

TH' A%&T' ATTA%3$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ 4What is an acute attack#$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ 4What 5ay bring on an acute attack#$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$4What can I do to a6oid de6eloping an acute attack#$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ 4TH' S3I( I( O!H"!IA$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ 7

Skin care$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ 7WHAT 'S' %A( ' /O(' 8O! O!H"!IA#$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ 9WHAT AO&T )" %HI/!'(#$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ 9TH' O!H"!IAS$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ :

A )'/I%A .&I/'$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ :8eatures of the Acute Attack$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ :recipitating 8actors$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ ;The /rug ists$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ ;

'-A&ATIO( O8 /!&.S 8O! O!H"!I% ATI'(TS$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$$ perience abdo5inal pain, cra5ps, constipation,nausea or 6o5iting$ They 5ay also shoC 5arked an>iety or disturbed beha6iour$ Such attacks can bebad enough to re?uire ad5ission to hospital, and the 5ost se6ere cases 5ay go on to Ceakness andparalysis$

eople ha6e e6en died of such an attack$ 8ortunately, a fatal outco5e has beco5e rare as 5odern

hospitals noC ha6e the facilities to treat such co5plications$ This e5phasises the need for peoplee>periencing an acute attack to be ad5itted to an e>perienced hospital$ It is 5ore co55on noCadaysfor people Cith AI or - to de6elop 5ilder for5s of the acute attack Cith not 5uch 5ore than afeeling of being unCell, so5e pain in the sto5ach and, perhaps, nausea$

If you are e>periencing such proble5s, it is i5portant that you i55ediately stop any 5edication you5ay be taking and consult your doctor$ "et e6eryone has so5e of these sy5pto5s at one ti5e oranother and you cannot bla5e e6erything on your porphyriaG


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THE -.IN IN PORPHYRIA

eople Cith -, H%, % and %T 5ay ha6e a sensiti6e skin$ They co5plain that it is easily da5aged

and that e6en the slightest knock can cause the skin to break$

Often these da5aged areas take a long ti5e to heal$ Sunlight is necessary to cause the skin tobeco5e fragile and people Cith porphyria find that the only parts of their bodies that are affected arethose that are e>posed to light, particularly their hands, faces, necks, legs and feet$

They de6elop blisters and open sores$ In ti5e their skins beco5e thin, dark, scarred and often ratherhairy, particularly on the face$

' and % are so5eChat different to the other porphyria$ 8irstly, the skin 5ay already be affected asa 6ery young child or e6en in infancy, Chereas the other porphyria usually only beco5e ob6ious laterin life$ Secondly, such people often find that they react to sunlight rapidly, de6eloping a sensation ofburning or stinging shortly after going into the sun$ This is unlike -, H% and %T, Chere the da5agetakes 5uch longer to de6elop$ Affected people learn to a6oid too 5uch light because of thisdisco5fort$

Skin care

If you ha6e skin proble5s because of porphyria you should look after your skin as folloCs=

1$ A6oid sunlight as 5uch as possible$ This 5eans re5aining indoors during the sunniest part of theday, and only going out in the early 5orning or late afternoon$ &nfortunately, sunbathing is O&T ifyou ha6e skin proble5s$ This is definitely the Corst thing a porphyric Cith skin proble5s can do$

If you ha6e a ob that re?uires you to be in the sun a lot, you 5ay ha6e to consider changing youroccupation$ rotect sun=e>posed areas by Cearing long slee6es, glo6es and a hat Chene6er yougo out$ These should be 5ade of cotton, Chich screens out the sun better than nylon$ (o crea5 or

di ti i ff ti i t ti ki


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WHAT EL%E 'A) *E DO)E FOR PORPHYRIA&

&nfortunately, there noC is no cure for porphyria$ Still, there is a lot that you and your doctor can do to5ake it less se6ere$ With a little care, your sy5pto5s probably Cill be 5ild and you can li6e a nor5allife, as 5any porphyrics do$

8irst, you 5ust get an accurate diagnosis so that you can be absolutely certain you ha6e porphyriaand if so, Chat type$ Speak to your doctor in this regard$ atients Cith %T can be helped by a6oidingalcohol or any other knoCn cause of the condition$

If the skin re5ains bad, relief can be obtained by 6enesection$ This 5eans ha6ing 700 5l$ of blood

re5o6ed at regular inter6als = usually fortnightly = for about eight Ceeks$ "our doctor Cill arrange this ifnecessary$ eople Cith ' also 5ust ensure that they ha6e regular 5edical check=ups as theircondition can e6entually affect the li6er$ !egular e>a5inations and blood tests Cill detect this at anearly stage$

WHAT A*OUT +Y 'HILDRE)&If you ha6e %T, your children Cill al5ost certainly be free of porphyria$ If you ha6e AI, H%, - or

', each child has a 7070 chance of being affected$ If they are affected, this is not a terrible thingGThey Cill probably not be any 5ore seriously affected than you = they 5ay be 5ilder$ In fact, 5ore than70 of porphyrics do not shoC signs of the condition at all, though they 5ay be positi6e on testing =these are knoCn as latent cases$

With sensible precautions, your children can li6e to a nor5al age, 5arry and ha6e childrenthe5sel6es$ "oung children tend not to shoC signs of their porphyria till after puberty = that is, till afterthey reach se>ual 5aturity$ aboratory tests usually do not e6en pick it up till then$

A t tt k h d l d l i i hildh d b f th t t


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THE PORPHYRIA%:

A MEDICAL GUIDE

The orphyrias are a heterogeneous group of either inherited or ac?uired disorders of hae5biosynthesis$ In these diseases, specific abnor5alities of enDy5es in the biosynthetic pathCay causegeneralised clinical abnor5alities$ They are classified, as shoCn beloC, into acute and non=acuteporphyria$

A%&T'O!H"!IA

A%&T' I(T'!)ITT'(TO!H"!IA*SCedish orphyria+-A!I'.AT' O!H"!IA*South African .enetic orphyria+H'!'/ITA!"%O!OO!H"!IA*%oproporphyria+&)OO!H"!IA

*AA /ehydratase deficiency+

(O(=A%&T'O!H"!IA

O!H"!IA %&TA('A TA!/A*%utaneous Hepatic orphyriaSy5pto5atic orphyria+'!"TH!OOI'TI%!OTOO!H"!IA*'rythrohepatic orphyria+%O(.'(ITA O!H"!IA*.untherFs disease' th i ti h i +


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.rand=5al con6ulsions are not unco55on at the height of an attack and 5ay persist betCeen attacks$In -ariegate orphyria, and in Hereditary %oproporphyria, there 5ay also be skin in6ol6e5ent, Cithde6elop5ent of solar photosensiti6ity$

recipitating 8actors

It should be e5phasised that 5ost subects Cho ha6e inherited one of these diseases Cill enoynor5al health and go through life Cithout any knoCledge of his or her disorder or e6er e>periencing anacute attack$ Such, is the latent phase of the disease$

All porphyrics, hoCe6er, are at risk of de6eloping an attack if e>posed to 6arious precipitating factors$/rugs are the 5ost co55on precipitating agents$ Other factors that 5ay trigger attacks, includealcohol ingestion, reduced caloric intake, due to fasting or dieting, and infection$ We ha6e also notedthat s5oking can cause 5ore fre?uent attacks$

Hor5ones are also i5portant$ Attacks are 5ore co55on in fe5ales and, rarely, occur before pubertyor after the 5enopause$ regnancy and oral contracepti6es 5ay also precipitate attacks$ So5eCo5en e>perience regular attacks, co55encing in the Ceek prior to the onset of 5enstruation$

Although 5ost of the drugs incri5inated as porphyrinogenic are lipophilic and inducers of the hepatic5i>ed function o>idase syste5, it is i5possible to reliably predict fro5 che5ical structure Chether adrug Cill be safe for use in the porphyric patient$

The /rug ists$

ists in Table 1 *A+ and *+ gi6e infor5ation, alphabetically, on all drugs about Chich so5e infor5ationis knoCn$


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E-ALUATIO) OF DRUG% FOR PORPHYRI' PATIE)T%

This Table is organised under the Therapeutic Headings gi6en in The ritish (ational 8or5ulary$ Thedrugs are categorised, as folloCs

A$ Those in the Funsafe groupF Chich ha6e beenreported by three or 5ore Corkers in the fieldto be associated Cith clinical e>acerbations ofporphyria or, in the Fsafe groupF, thoseconsidered by three or 5ore authorities to behar5less to porphyric patients on the basis of

their clinical e>perience$

$ Those in the Funsafe groupF Chich ha6e beenreported by tCo or less Corkers in the field tobe associated Cith clinical e>acerbations ofporphyria or, in the Fsafe groupF, thoseconsidered by tCo or feCer authorities to behar5less to porphyric patients on the basis oftheir clinical e>perience$

In 5any instances, the clinical e>perience Cith drugs in the abo6e tCo groups Cill ha6e beencorroborated by e>peri5ental data$ There are tCo further headings under Chich the drugs are listed

%$ Those Chich ha6e been e6aluated only inani5als, Cith e>peri5entally=producedporphyria$

/ Th hi h h b l t d l i ll


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D' List

/0 GA-TROINTE-TINAL -Y-TEM

U)%AFE THOUGHT TO *E %AFE 'O)TE)TIOU%

Alu5iniu5 OHHyoscine utylbro5ide)ebe6erine H%lSulphasalaDine

%A/%

Atropine%isapride/anthron/icyclo5ine H%l/ipheno>ylate H%l/o5peridone8a5otidinei?uoriceopera5ide)agnesiu5 Sulphate)etopi5aDineirenDepineropanthelineSennaSorbitolTri5ebutine )aleate

AA%//AA/

%i5etidine)etoclopra5ideO5epraDole!anitidine

&&S&

10 CARDIOVA-CULAR -Y-TEM


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Anti=hypertensi6e Agents


Alpha )ethyl /OA%aptopril%lonidine'nalaprilHydralaDine

isinoprilheno>ybenDa5ine

A//

/

Atenolol/iaDo>ide.uanethidine.uanfacine H%labetalol

)eca5yla5ine)etipropanolol)etoprolol Tartrateropanolol!eserpineTi5olol )aleateTolaDoline

A/A/

%AA/A

%alciu5 %hannel lockers


epridil H%l(ifedipinerenyla5ine-erapa5il

//

/iltiaDe5 &


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20 RE-PIRATORY -Y-TEM:


Allylo>y =5ethylbenDa5ideAste5iDolee5egride.uaiphenesin(iketha5ideentylenetetraDolTheophylline

%/%A

eclo5ethasone dipropionate3etotifen)e?uitaDineseudoephedrine H%lSalbuta5ol

/

Antihista5ines


%le5astine/i5enhydrinate/iphenhydra5ine8lunariDine H%lTerfenadine

/

%hlorphenira5ineTri5epraDine tartrateTripelenna5ine

A/

ro5ethaDine &


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30 CENTRAL NERVOU- -Y-TEM:

Hypnotics, Sedati6es and An>iolytics


AlpraDola5A5ylobarbitoneApronalide%arbro5al%arisoprodol%hlordiaDepo>ide%hlor5eDanone%lotiaDepa5/iaDepa5/ichloralphenaDone'thchlor6ynol'thina5ate8lunitraDepa58luraDepa5.lutethi5ideHe>apropy5ateopraDola5o>apine)eproba5ate)ethyprylone(itraDepa5raDepa5Juinalbarbitone

S l i id

/AA/AA%AA///AA/

%

enDhe>ol H%l%hloral hydrate%hlorpro5aDine/roperidolofepra5ine)ethylphenidateericyaDineiraceta5rochlorperaDinero5aDineTe5aDepa5TriaDola5TrifluoperaDine

/AA//A%

ro5aDepa5%hlor5ethiaDole%lobaDa5%lonaDepa5%loraDepate'staDola5HaloperidoloraDepa5)ethotri5epraDine)idaDola5O>aDepa5ro5ethaDine

SS&&&/S&SS/S&&


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sychoanaleptics


A5ineptine H%l/othiepin H%l8lupenthi>olIproniaDid)aprotiline H%l)ianserin H%largyline

henelDineTranylcypro5ineTraDodone H%l-eralipride-ilo>aDine H%lKuclopenthi>ol

/%%%%%

%%%/%%

8luo>etine H%l8lu6o>a5ine

5aleateithiu5 saltsofepra5ine)ethylphenidate)inaprine H%l

ipothiaDinepal5itate

//

/

A5itriptyline%arpipra5ine%lo5ipra5ine H%lI5ipra5ine)etapra5ine H%l(ortriptylineTri5ipra5ine

&&/&&/&&&

Anticon6ulsants


arbiturates%arba5aDepine'thosu>i5ide/iaDepa5'thotoin

AAAA

ro5ides)agnesiu5=sulphatearaldehyde

A

%hlor5ethiaDole%lonaDepa5(a -alproate-alpro5ide

S&&/&


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Analgesics (A!%OTI%S


/e>tropropo>ypheneO>ycodoneentaDocinehenacetinTilidate

%A%

uprenorphine/ia5orphine/roperidol)eptaDinol)ethadone

)orphineethidine

AA

AA

/e>tro5ora5ide S

)igraine


%lonidine H%l/ihydroergota5ine=)esylate'rgota5ine TartrateIso5etheptene )ucateysuride )aleateOrphenadrine

AA%%

iDotifenrochlorperaDine


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40 IN5ECTION:


%olistin%hlora5phenicol%o=tri5o>aDole%ycloserine/apsone'conaDole (itrate'rythro5ycin

8luclo>acillin8lu5e?uine.riseoful6in3etoconaDole)iconaDole(alidi>ic acid(ata5ycin(o6obiocinipe5idic acid

i6a5picillinyraDina5ide!ifa5picinSulphona5idesTinidaDoleTri5ethopri5-ibra5ycin

AAA

A/A///

AA/

Acyclo6irA5inoglycosidesA5o>ycillinA5photericin /A5picillin%iproflo>acin%la6ulanic Acid

8lucytosine.enta5ycinHe>a5ine@osa5ycin)eflo?uine H%l)inocycline H%l(etil5ycin(orflo>acinOflo>acin

O>olinic acideflo>acinenicillinri5a?uineJuinineSodiu5 8usidateStrepto5ycinTala5picillinTicarcillin

- i

//A/

/%%//////

//AA

%ephalosporins%hloro?uineIsoniaDid)ebendaDole)etronidaDole(itrofurantoinyri5etha5ine

Tetracyclines

&S&SS&S

S


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Anti=/iabetic Agents


Sulphonylureas.lipiDide

A

iguanidesInsulin)etfor5inhenfor5in

%A%%

70 OB-TETRIC-8 GYNAECOLOGY AND RENAL DI-EA-E:


'rgo5etrine )aleateHyoscine utyl ro5ideOral %ontracepti6es

AAA

/inoprostO>ytocinropantheline

%

)ifepristoneOestrogens

&&

90 MALIGNANT DI-EA-E: and IMMUNO-UPPRE--ION:


A5inoglutethi5ideusulphan%hlora5bucil%yclophospha5ide%yclosporin

) t l

%%%%

%

Actino5ycin /ADathioprine%isplatin/o>orubicin H%l'toposide

Kid di

%%/%

%yproterone=Acetate'thinyl Oestradiol)elphalan-inblastine-incristine

SSS&/&/


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/;0 MU-CULOa5ethasone Hydrocortisonerednisolone

SS

* + S ifi A i !h i A


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*e+ )uscle !ela>ants and Anti=Spas5odics


aclofen%arisoprodol%hlor5eDanone%hlorDo>aDone/iaDepa5/ipyrone/rota6erine

Hyoscine utylbro5ide)ephenesinOrphenadrineO>ana5ide

/AAAA

A/%

/o5peridone(eostig5inearapenDolate rropantheline rSu>a5ethoniu5Tubocurarine

%A/A

)etoclopra5ideancuroniu5

&S

//0 THE EYE:


%hlora5phenicol%ocaineHyoscine utylbro5ide)ercuric o>ideO>yphenbutaDoneSulphaceta5ide

AA%

AcetaDola5ideA5ethocaine H%lAtropine/e>a5ethasone.enta5icin.uanethidineO>ybuprocaineTi5olol )aleate

%AA%%/

rednisolonero>y5etacaineTetracyclines

SSS


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/40 ANAE-THE-IA:


Alcuroniu5Alpha>alone =Alphadolonearbiturates%articaine H%l%hlorofor5

%ocaine'nflurane'tidocaine'to5idate8luro>eneIsofluraneignocaine)epi6acaine)ethohe>itone

rilocaineThiopentone (a

%A/

%%%%%/%

A

A5ethocaine H%lupi6acaineutacaine SO4%yclopropane/iethyl ether

/roperidol8entanyl8lu5aDenil)eptaDinol)idaDola5)ina>olone(itrous o>ideropofolrocaine

Su>a5ethoniu5TetracaineTubocurarine

%%/%A

%%//%A

%%%

HaloperidolHalothane3eta5ineancuronu5 rropanidid

ro>y5etacaine

&&SS&

S


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A'UTE PORPHYRIA DIAG)O%I%:

Acute porphyria should be considered in any patient presenting Cith une>plained abdo5inal pain,5ental dysfunction or peripheral neuropathy$ A further clue to the diagnosis is discolouration of theurine$

/uring an attack, the urine is a dark reddish broCn and this beco5es 5ore pronounced if it is leftstanding$ A si5ple bedside test can confir5 the diagnosis$ Juantitati6e studies of the differentporphyrins and precursors in the urine and faeces should be perfor5ed later by a specialist laboratoryto identify the particular type of acute porphyria$

Successful treat5ent of an acute attack of orphyria depends largely on, early diagnosis, re5o6al ofprecipitating factors, and pro6ision of intensi6e supporti6e therapy$ On first diagnosing an attack, acareful search should be 5ade for any precipitating factors, and if possible, these should be re5o6ed$

The patientFs current drug therapy should be scrutinised and a search 5ade for any underlyinginfection$ When appropriate, a pregnancy test should be perfor5ed$

-c'!!nin ? 5a$i+i!satent cases in affected fa5ilies 5ay be diagnosed, either by 5easure5ent of porphyrins and theirprecursors in urine, faeces and blood, or by 5easure5ent of the acti6ities of the enDy5es of the Hae5biosynthetic pathCay$

Where such screening is re?uired, 5ost Analytical aboratories, such as our oCn, prefer to recei6esa5ples of urine, stool and hepariniDed blood$ rophyla>is is e>tre5ely i5portant$ In particular, /rugslisted in the F&nsafe .roupingsF in Tables 1 and 2, should be a6oided$


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If the sy5pto5s are not controlled, an infusion of Hae5 Arginate should be considered$*See beloC+$This treat5ent is still on trial and 5ay be obtained fro5 the appropriate drug co5pany on a na5edpatient basis$

-%$,t$atic Th!'a,%

ain

This is a feature of 5ost attacks$ When 5ild, it 5ay be ade?uately controlled Cith aspirin, paraceta5olor dihydrocodeine$ 8or 5ore se6ere pain, pethidine *5eperidine+, 5orphine or dia5orphine 5ay be

re?uired$

uprenorphine, Chich 5ay be ad5inistered either sublingually or intra5uscularly, is also useful$ )oreconstant pain relief 5ay be achie6ed by the continuous intra6enous infusion of analgesics$

There is a danger of addiction in patients e>periencing fre?uent attacks, Cho re?uire large a5ounts ofnarcotic analgesics, and e6ery atte5pt should be 5ade to CithdraC all narcotic drugs betCeenattacks$

In a feC unfortunate patients, the pain is refractory to e6en 6ery large doses of narcotic analgesics,and signs of respiratory and cardio6ascular syste5 depression appear before pain relief is obtained$

)any of our patients report that the only ti5e the pain goes aCay is Chen they are asleep$ Thisobser6ation 5ay be used to ad6antage by encouraging sleep for se6eral hours by co5biningchlorpro5aDine or pro5aDine Cith the analgesics and lea6ing the patient undisturbed in a darkenedroo5$

So5e patients continue to co5plain of chronic abdo5inal pain, unacco5panied by any other


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sitting upright$ Whene6er there is e6idence of cardio6ascular instability, continual '%. 5onitoringshould be perfor5ed and full resuscitati6e facilities kept at hand$

%on6ulsions

%on6ulsions are not infre?uent at the peak of an attack$ Their onset 5ay be a sign of hyponatrae5ia,due to inappropriate antidiuretic hor5one secretion, and plas5a os5olality, and electrolyte 6aluesshould be checked$ If hyponatrae5ia is the underlying cause, it should be corrected by restricting fluidintake to not 5ore than :00 5l$

The onset of con6ulsions 5ay also be a sign of hypertensi6e encephalopathy, and the blood pressure

should be checked$ %on6ulsions, occurring during the attack, usually disappear as the attack resol6esand, therefore, therapy should be ai5ed at treating the underlying disease process$

So5e patients continue to e>perience con6ulsions Chile in re5ission$ This presents a therapeuticdile55a$ henobarbitone, pri5idone, phenytoin and carba5aDepine all increase cellular hae5utilisation by inducing the synthesis of hepatic 5ono>ygenases, and are contraindicated$

The benDodiaDepines and sodiu5 6alproate are not inducers of the 5ono>ygenases and, althoughthey are porphyrinogenic in e>peri5ental 5odels of porphyria, there is li5ited e6idence that they are

porphyrinogenic in 5an$ Status epilepticus, in our oCn e>perience, has been treated successfully Cithintra6enous diaDepa5$

SeiDure prophyla>is can be undertaken as a calculated risk Cith clonaDepa5 or sodiu5 6alproate ifthis is essential, although sporadic clinical reports of porphyr= inogenicity do e>ist$ Sodiu5 bro5ideand 5agnesiu5 sulphate are safe, but generally out5oded anticon6ulsants$

(europathy


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-PECI5IC THERAPY O5 THE ACUTE ATTAC.: -PECI5IC THERAPY

Hae5atin Therapy * Hae5 Arginate +

So far Ce ha6e concentrated on treating the acute attack by correcting any factors that 5ay ha6eprecipitated it, and pro6iding ade?uate supporti6e therapy Chile the attack spontaneously resol6es$ Itis also possible to treat the underlying disease process 5ore directly by ad5inistering the endproduct of the deranged pathCay as intra6enous hae5atin$

In the li6er, it is thought to supple5ent the depleted intracellular Ffree hae5 poolF, thus repressing the

acti6ity of the initial and rate=controlling enDy5e of hae5 biosynthesis, AA synthase, and reducing theo6erproduction of porphyrins and precursors for5ed prior to the enDy5e block$

In an acute attack of porphyria, the intra6enous ad5inistration of hae5atin consistently reduces boththe plas5a concentration and the urinary e>cretion of porphyrin precursors$ Its effect 5ay besupple5ented Cith inhibitors of the hae5 degradati6e enDy5e, Hae5 o>ygenase, such as Tinprotoporphyrin$ The clinical response to the therapy is 5ore difficult to assess$

In a disease characterised by spontaneous relapses and re5issions, it is difficult to be sure Chether

i5pro6e5ent is the result of therapy or ust the natural course of the disease$ Our current i5pressionis that hae5atin does curtail the clinical attack, and Ce fre?uently e5ploy it$

(o 5aor side=effects ha6e been reported Cith hae5atin Chen used in the standard doses$ hlebitis,around the inection site, occurs in a feC patients$ This can be pre6ented by inecting the solution intoa large peripheral 6ein or 6ia a central 6enous line, or by ad5inistering the hae5atin, Cith hu5analbu5in solution, to Chich it Cill bind$ /uring hae5atin therapy, there is a 5ild disturbance ofcoagulation, Cith prolongation of the prothro5bin and partial thro5boplastin ti5es, and a slightreduction in the platelet count$


29/49

)enstruationSo5e Co5en e>perience regular attacks in the Ceek prior to the onset of 5enstruation$ These areassu5ed to be initiated by the hor5onal fluctuations, and 6arious atte5pts ha6e been 5ade to pre6entthe5$

So5eti5es, 5erely increasing the carbohydrate intake at the appropriate ti5e ofthe 5onth is found to be helpful$ Interfering Cith the hor5onal fluctuations has produced 6aryingresults$

So5e patients ha6e been reported to benefit fro5 the suppression of o6ulation, using 6arious oralcontracepti6e hor5one preparations$ HoCe6er, in our oCn e>perience, also that of se6eral other%entres, the contracepti6e pill has usually precipitated attacks$

Attacks ha6e been successfully pre6ented by ad5inistering hae5atin, prophylactically, ust prior to theti5e in the 5onth Chen the attack usually starts and through the long ter5 use of synthetic H!Hanalogues$

regnancy and Acute orphyriaregnancy 5ay precipitate acute porphyria, Cith attacks being 5ost co55on in early pregnancy andduring the puerperiu5$ The first attack often occurs during pregnancy$ In patients Cith the genetic trait,Cho ha6e nor5al porphyrin e>cretion, Cho ha6e ne6er e>perienced a clinical attack, pregnancy is,usually, une6entful$

If 6o5iting is a proble5 in early pregnancy, the patient should be ad5itted to the hospital at an earlystage and de>trose ad5inistered, intra6enously, to pre6ent the reduced dietary intake fro5 inducing anattack$ /e>trose should also be ad5inistered, intra6enously, during labour$ atients Cho ha6e had


30/49

are safe inhalation agents Cith respect to the porphyrias, but they suffer the disad6antages of beingpotentially e>plosi6e and inducing post=operati6e 6o5iting$

(itrous o>ide, used Cith intra6enous narcotics, and 5uscle rela>ants, 5ay be a 5ore acceptablealternati6e$ Su>a5ethoniu5 and /=tubocurarine can be used as 5uscle rela>ants and dia5orphine,5orphine, pethidine or fentanyl are suitable narcotics for controlling post=operati6e pain$

In so5e situations, epidural anaesthesia 5ay be preferable to general anaesthesia, in Chich casebupi6acaine is the local anaesthetic of choice$ To pre6ent an attack being induced by fasting, anintra6enous infusion of de>trose should be co55enced prior to surgery, and continued until the patientcan eat properly$

hotosensiti6ity

hotosensiti6ity 5ay be found in -ariegate porphyria and Hereditary %oproporphyria$ The occurrenceof skin lesions depends on the degree of porphyrin o6erproduction and the a5ount of e>posure tosunlight$

Skin lesions should be treated by re5o6ing any inducing factors, such as drugs, alcohol, orinade?uate diet, that 5ay increase the porphyrin o6erproduction$ There is no specific treat5ent for the

skin photosensiti6ity occurring in -ariegate porphyria and Hereditary coproporphyria, although eta=carotene treat5ent has been suggested of benefit$

arrier crea5s 5ay be used$ A6oidance of e>cess sunlight is ad6ised$ The der5atological featuresoften subside after the acute attack, as the a5ount of circulating porphyrin is reduced$

rophyla>is and Treat5ent of )alaria$

The steady encroach5ent of chloro?uine=resistant 5alaria and increased tra6el to 5alarious regions


31/49

A third strategy, the one that is probably safest to suggest noC, is to a6oid che5oprophyla>isaltogether and adopt the folloCing steps=

1$ /irect prophylactic 5easures against the 6ector instead of the parasite, thus reducing the riskof an infected bite$ This has been shoCn to reduce the risk of contracting 5alaria considerablyand entails

i+ 6isiting 5alarial areas in the dry rather than the Cet seasonEii+ staying in toCns rather than the bushEiii+ co6ering up Cith long slee6es and trousers at sundoCnEi6+ liberal use of insect repellents, both on the person and in the en6iron5entE6+ the use of 5os?uito coilsE *6i+ proper use of 5os?uito netting$

2$ The 6isitor should be ad6ised to carry a course of ?uinine sulphate tablets at all ti5es and beinstructed on the possible sy5pto5s of 5alaria, particularly pyre>ia, backache, nausea andheadache$ A course of ?uinine should be co55enced at the first sign of an illness and 5edicalsupport sought as ?uickly as possible$

Such an approach is fa6oured by so5e authorities as a general strategy against chloro?uineresistant 5alaria and hence, if applied to porphyrics, as outlined, does not represent a 5aordeparture fro5 current thinking$

What of treat5ent of established 5alaria in porphyric patients# The course here is clear Theyshould recei6e ?uinine sulphate aloneE this Cill be efficacious against chloro?uine resistant5alaria and is of pro6en safety in porphyria$

8inally, Chere 5alaria, other than 8alciparu5, is suspected, pri5a?uine 5ay safely bee5ployed to eradicate the e>o=erythrocytic cycle of these parasites$


32/49

TA*LE !:

DRUG TREAT+E)T% I) A'UTE PORPHYRIA%O(/ITIO( /!&. %AT'.O!" /!&.

S'%I8I%T!'AT)'(TS

HA') A!.I(AT'.&%OS'

A/O)I(A AI( Analgesics ASI!I(&!'(O!HI('/IA)O!HI('/IH"/!O%O/'I(')O!HI('A!A%'TA)O'THI/I('

-O)ITI(. Anti='5etics %HO!!O)AKI('

%"%IKI('!O%HO!'!AKI('!O)AKI('

H"'!T'(SIO( A(/TA%H"%A!/IA

Anti=Hypertensi6es AT'(OO.&A('THI/I('A'TAO)'%A)"A)I('!O!A(OO


33/49

%O(/ITIO( /!&. %AT'.O!" /!&.

!IO%AI('

!O%AI('!OO8OS&A)'THO(I&)T&O%&!A!I('

A!TH!ITI% A(/!H'&)ATI%%O(/ITIO(S

Anti=Infla55atories AO&!I(OASI!I(%O%HI%I('8&!I!O8'(

I(/O)'THA%I((A!O'('(I%IA)I('

%A(%'! %he5otherapeuticAgents

A%TI(O)"%I( /%ISATI(%"!OT'!O('=A%'TAT'/OO!&I%I()'HAA(

-I(%!ISTI(' SO4

%A!/IO-AS%&A! Anti=Arrhyth5ic/rugsand /iuretics

%O(/ITIO(SA)IO!I/'AT!OI('&)'TA(I/'%"%O'(THIAKI/'/I.OI(/ISO"!A)I/'


34/49

TE%TI)G FOR PORPHYRIA

SA)'S1$ &!I('

200 5l$ AIJ&OT 8!O) 24=HO&! &!I(' %O'%TIO(*Cith no added preser6ati6e+O! SOT SA)' O8 AT 'AST 100 5l$ &!I('

2$ OO/10 5l$ WHO' OO/ I( ITHI&) H'A!I( %O(TAI('!

$ 8A'%'S7g 8A'%A SA)' *in sealed container+

TO ' S'(T *Cith a short case history+ TO TH'=

O!H"!IAS S'!-I%'(!%'TO o> 7


35/49

APPE)DI I 0 ALPHA*ETI'ALLY DRUG LI%TI)G

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1(&T!ITIO( A(/ OO/ *'dta+ Safe A

TH' '"' AcetaDola5ide Safe

%A!/IO-AS%&A! S"ST') /iuretics AcetaDola5ide Safe /Acetylcholine Safe

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( Actino5ycin / Safe %

I(8'%TIO( Acyclo6ir Safe /

Adenosine )onophosphate Safe

%A!/IO-AS%&A! S"ST') Adrenaline Safe

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents Alclofenac Safe %

A(A'STH'SIA Alcuroniu5 &nsafe %

)&S%&O=S3''TA and @OI(T /IS'AS' Anti=.out Agents Allopurinol Safe

!'SI!ATO!" S"ST') Allylo>y =)ethylbenDa5ide &nsafe %

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents Alpha )ethyl /opa &nsafe A

(&T!ITIO( A(/ OO/ Alpha Tocopherylacetate Safe A

A(A'STH'SIA Alpha>alone = Alphadolone &nsafe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics AlpraDola5 &nsafe /

.AST!OI(T'STI(A S"ST') Alu5iniu5 OH &nsafe %

Alu5iniu5 reparations &nsafe

A(A'STH'SIA A5ethocaine H% Safe %

TH' '"' A5ethocaine H% Safe %%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% A5idopyrine &nsafe %

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents A5idopyrine &nsafe A

%A!/IO-AS%&A! S"ST') /iuretics A5iloride Safe

%'(T!A ('!-O&S S"ST') sychoanaleptics A5ineptine H% &nsafe /

%A!/IO-AS%&A! S"ST') A5inocaproic Acid Safe

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( A5inoglutethi5ide &nsafe

I(8'%TIO( A5inoglycosides Safe

A5inophylline &nsafe

%A!/IO-AS%&A! S"ST') A5iodarone H% &nsafe /

%'(T!A ('!-O&S S"ST') sychoanaleptics A5itriptyline %ontentious &

I(8'%TIO( A5o>ycillin Safe

A5pheta5ines &nsafe (ote 2

I(8'%TIO( A5photericin / Safe /

I(8'%TIO( A5picillin Safe A%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics A5ylobarbitone &nsafe A

'(/O%!I(' S"ST') Hor5one reparations Androgens %ontentious &

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% Antipyrine &nsafe %

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics Apronalide &nsafe

(&T!ITIO( A(/ OO/ Ascorbic Acid Safe A

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% Aspirin Safe A

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents Aspitin Safe A

A .&I/' 8O! 'O' WITH O!H"!IA age 1


36/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1!'SI!ATO!" S"ST') Aste5iDole &nsafe /

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents Atenolol Safe A

%A!/IO-AS%&A! S"ST') Atropine Safe A

.AST!OI(T'STI(A S"ST') Atropine Safe A

TH' '"' Atropine Safe A

)&S%&O=S3''TA and @OI(T /IS'AS' Specific Anti=!heu5atic Agents Auranofin &nsafe

Aurothio5alate &nsafe Associated Cith acute

attacks of porphyria$)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents ADapropaDone &nsafe %

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( ADathioprine Safe %

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics aclofen &nsafe /

A(A'STH'SIA arbiturates &nsafe A

%'(T!A ('!-O&S S"ST') Anticon6ulsants arbiturates &nsafe A

!'SI!ATO!" S"ST') eclo5ethasone /ipropionate Safe

!'SI!ATO!" S"ST') e5egride &nsafe

%A!/IO-AS%&A! S"ST') /iuretics endrofluaDide %ontentious S

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents eno>aprofen &nsafe %

)&S%&O=S3''TA and @OI(T /IS'AS' Anti=.out Agents enDbro5arone &nsafe /

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics enDhe>ol H% Safe /

'(/O%!I(' S"ST') Hor5one reparations enDylthiouracil %ontentious /S

%A!/IO-AS%&A! S"ST') %alciu5 %hannel lockers epridil H% &nsafe /

eta=%arotene Safe

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics etahistine H% &nsafe /'(/O%!I(' S"ST') Anti=/iabetic Agents iguanides Safe %

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics ro5aDepa5 %ontentious S

%'(T!A ('!-O&S S"ST') Anticon6ulsants ro5ides Safe A

'(/O%!I(' S"ST') Hor5one reparations ro5ocriptine &nsafe /

%A!/IO-AS%&A! S"ST') uflo5edil H% Safe /

%A!/IO-AS%&A! S"ST') /iuretics u5etanide Safe

A(A'STH'SIA upi6acaine Safe %

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S uprenorphine Safe

'(/O%!I(' S"ST') Hor5one reparations userelin Safe A

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( usulphan &nsafe %

A(A'STH'SIA utacaine SO4 Safe /

utylscopola5ine &nsafe Associated Cith acuteattacks of porphyria$

TH' S3I( %antha>anthin Safe A

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents %aptopril &nsafe /%'(T!A ('!-O&S S"ST') Anticon6ulsants %arba5aDepine &nsafe A

'(/O%!I(' S"ST') Hor5one reparations %arbi5aDole Safe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %arbro5al &nsafe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %arisoprodol &nsafe A

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics %arisoprodol &nsafe A

%'(T!A ('!-O&S S"ST') sychoanaleptics %arpipra5ine %ontentious &

%arpipra5ine H% Safe (ote 1



37/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1A(A'STH'SIA %articaine H% &nsafe /

%efuro>i5e &nsafe (ote 2

%ephale>in &nsafe (ote 2

I(8'%TIO( %ephalosporins %ontentious &

%ephradine &nsafe (ote 2

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %hloral Hydrate Safe A

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( %hlora5bucil &nsafe %

I(8'%TIO( %hlora5phenicol &nsafe ATH' '"' %hlora5phenicol &nsafe A

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %hlordiaDepo>ide &nsafe

%'(T!A ('!-O&S S"ST') Anticon6ulsants %hlor5ethiaDole %ontentious S

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %hlor5ethiaDole %ontentious S

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %hlor5eDanone &nsafe

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics %hlor5eDanone &nsafe

A(A'STH'SIA %hlorofor5 &nsafe

I(8'%TIO( %hloro?uine %ontentious S)&S%&O=S3''TA and @OI(T /IS'AS' Specific Anti=!heu5atic Agents %hloro?uine %ontentious S

%A!/IO-AS%&A! S"ST') /iuretics %hlorothiaDide %ontentious S

!'SI!ATO!" S"ST') Antihista5ines %hlorphenira5ine Safe A

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %hlorpro5aDine Safe A

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics %hlorpro5aDine Safe A

%hlorpropa5ide &nsafe Associated Cith acute

attacks of porphyria$)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics %hlorDo>aDone &nsafe

.AST!OI(T'STI(A S"ST') %i5etidine %ontentious &

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics %innariDine &nsafe %

I(8'%TIO( %iproflo>acin Safe

.AST!OI(T'STI(A S"ST') %isapride Safe

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( %isplatin Safe /

I(8'%TIO( %la6ulanic Acid Safe /

!'SI!ATO!" S"ST') Antihista5ines %le5astine &nsafe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %lobaDa5 %ontentious &

%A!/IO-AS%&A! S"ST') %lofibrate Safe /

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents %lo5etacin &nsafe /

%lo5iphene Safe

'(/O%!I(' S"ST') Hor5one reparations %lo5iphene %itrate Safe

%'(T!A ('!-O&S S"ST') sychoanaleptics %lo5ipra5ine H% %ontentious /&

%'(T!A ('!-O&S S"ST') Anticon6ulsants %lonaDepa5 %ontentious &%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %lonaDepa5 %ontentious &

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents %lonidine &nsafe /

%'(T!A ('!-O&S S"ST') )igraine %lonidine H% &nsafe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %loraDepate %ontentious &

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics %lotiaDepa5 &nsafe /

%lo>acillin Safe

A(A'STH'SIA %ocaine &nsafe

A .&I/' 8O! 'O' WITH O!H"!IA age


38/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1TH' '"' %ocaine &nsafe

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% %o=%oda5ol Safe

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% %odeine Safe

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents %odeine O4 Safe A

)&S%&O=S3''TA and @OI(T /IS'AS' Anti=.out Agents %olchicine Safe /

%'(T!A ('!-O&S S"ST') %olistin &nsafe

'(/O%!I(' S"ST') Hor5one reparations %orticosteroids %ontentious S

'(/O%!I(' S"ST') Hor5one reparations %orticotrophin *A%TH+ Safe I(8'%TIO( %o=Tri5o>aDole &nsafe

%ou5arins Safe

TH' S3I( %rystal -iolet &nsafe

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics %ycliDine Safe

%A!/IO-AS%&A! S"ST') /iuretics %yclopenthiaDide %ontentious &

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( %yclophospha5ide &nsafe %

A(A'STH'SIA %yclopropane Safe %

I(8'%TIO( %ycloserine &nsafe )AI.(A(T /IS'AS' and I))&(OS&!'SSIO( %yclosporin &nsafe %

'(/O%!I(' S"ST') Hor5one reparations %yproterone Acetate %ontentious S

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( %yproterone=Acetate %ontentious S

'(/O%!I(' S"ST') Hor5one reparations /anaDol &nsafe

.AST!OI(T'STI(A S"ST') /anthron Safe A

I(8'%TIO( /apsone &nsafe A

/esferrio>a5ine SafeTH' '"' /e>a5ethasone Safe

)&S%&O=S3''TA and @OI(T /IS'AS' %orticosteroids /e>a5ethasone Safe

'(/O%!I(' S"ST') Hor5one reparations /e>a5ethasone Safe

%'(T!A ('!-O&S S"ST') Appetite Suppressants /e>fenflura5ine &nsafe /

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S /e>tro5ora5ide %ontentious S

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S /e>tropropo>yphene &nsafe

/e>trose Safe

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S /ia5orphine Safe

%'(T!A ('!-O&S S"ST') Anticon6ulsants /iaDepa5 &nsafe A

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics /iaDepa5 &nsafe A

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics /iaDepa5 &nsafe A

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents /iaDo>ide Safe /

%'(T!A ('!-O&S S"ST') Hypnotics , Sedat i6es and An> io lyt ics /ichloralphenaDone &nsafe A

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents /ichloralphenaDone &nsafe A

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents /iclofenac (a &nsafe A.AST!OI(T'STI(A S"ST') /icyclo5ine H% Safe /ienoestrol &nsafe

A(A'STH'SIA /iethyl 'ther Safe A

%'(T!A ('!-O&S S"ST') Appetite Suppressants /iethylpropion &nsafe %

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% /iflunisal Safe

%A!/IO-AS%&A! S"ST') /igo>in Safe A

/ihydralaDine &nsafe



39/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents /ihydrocodeine Safe A

/ihydroergota5ine &nsafe Associated Cith acuteattacks of porphyria$

%'(T!A ('!-O&S S"ST') )igraine /ihydroergota5ine=)esylate &nsafe A

%A!/IO-AS%&A! S"ST') %alciu5 %hannel lockers /iltiaDe5 %ontentious &

!'SI!ATO!" S"ST') Antihista5ines /i5enhydrinate &nsafe

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics /i5enhydrinate &nsafe

/i5ercaprol Safe/i5ethicone Safe

OST'T!I%S, ."(A'%OO." A(/ !'(A /IS'AS' /inoprost Safe %

!'SI!ATO!" S"ST') Antihista5ines /iphenhydra5ine &nsafe

.AST!OI(T'STI(A S"ST') /ipheno>ylate H% Safe

%A!/IO-AS%&A! S"ST') /ipyrida5ole Safe /

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics /ipyrone &nsafe A

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents /ipyrone &nsafe A

%A!/IO-AS%&A! S"ST') /isopyra5ide %ontentious S

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics /i>yraDine &nsafe %

.AST!OI(T'STI(A S"ST') /o5peridone Safe %

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics /o5peridone Safe %

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics /o5peridone Safe %

%'(T!A ('!-O&S S"ST') sychoanaleptics /othiepin H% &nsafe %

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( /o>orubicin H% Safe %

/o>ycycline &nsafeA(A'STH'SIA /roperidol Safe %

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S /roperidol Safe A

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics /roperidol Safe

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics /rota6erine &nsafe A

'(/O%!I(' S"ST') Hor5one reparations /ydrogesterone &nsafe %

I(8'%TIO( 'conaDole (itrate &nsafe

TH' S3I( 'conaDole (itrate &nsafe

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents 'nalapril &nsafe

A(A'STH'SIA 'nflurane &nsafe %

OST'T!I%S, ."(A'%OO." A(/ !'(A /IS'AS' 'rgo5etrine )aleate &nsafe A

'rgot %o5pounds &nsafe Associated Cith acuteattacks of porphyria$

%'(T!A ('!-O&S S"ST') )igraine 'rgota5ine Tartrate &nsafe A

I(8'%TIO( 'rythro5ycin &nsafe A

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics 'staDola5 %ontentious /S'stra5ustine &nsafe Associated Cith acuteattacks of porphyria$

%A!/IO-AS%&A! S"ST') /iuretics 'thacrynic Acid Safe

'tha5butol Safe

%A!/IO-AS%&A! S"ST') 'tha5sylate &nsafe %

(&T!ITIO( A(/ OO/ 'thanol &nsafe A

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics 'thchlor6ynol &nsafe



40/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics 'thina5ate &nsafe %

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( 'thinyl Oestradiol %ontentious S

'(/O%!I(' S"ST') Hor5one reparations 'thinyl Oestradiol %ontentious S

'thiona5ide &nsafe

'thoheptaDine %itrate Safe

%'(T!A ('!-O&S S"ST') Anticon6ulsants 'thosu>i5ide &nsafe A

%'(T!A ('!-O&S S"ST') Anticon6ulsants 'thotoin &nsafe

A(A'STH'SIA 'tidocaine &nsafe %A(A'STH'SIA 'to5idate &nsafe %

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( 'toposide Safe

8$S$H$ Safe

.AST!OI(T'STI(A S"ST') 8a5otidine Safe

8enbrufen Safe

%'(T!A ('!-O&S S"ST') Appetite Suppressants 8enflura5ine &nsafe %

%A!/IO-AS%&A! S"ST') 8enofibrate %ontentious S/

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% 8enoprofen Safe %)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents 8enoprofen Safe %

A(A'STH'SIA 8entanyl Safe %

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% 8loctafenine &nsafe /

I(8'%TIO( 8luclo>acillin &nsafe A

I(8'%TIO( 8lucytosine Safe /

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents 8lufena5ic Acid &nsafe

A(A'STH'SIA 8lu5aDenil Safe /I(8'%TIO( 8lu5e?uine &nsafe /

!'SI!ATO!" S"ST') Antihista5ines 8lunariDine H% &nsafe /

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics 8lunitraDepa5 &nsafe

%'(T!A ('!-O&S S"ST') sychoanaleptics 8luo>etine H% Safe

%'(T!A ('!-O&S S"ST') sychoanaleptics 8lupenthi>ol &nsafe %

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics 8luraDepa5 &nsafe A

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents 8lurbiprofen Safe

A(A'STH'SIA 8luro>ene &nsafe

%'(T!A ('!-O&S S"ST') sychoanaleptics 8lu6o>a5ine )aleate Safe

(&T!ITIO( A(/ OO/ 8olic Acid Safe

'(/O%!I(' S"ST') Hor5one reparations 8ollicle Sti5ulating Hor5one Safe

(&T!ITIO( A(/ OO/ 8ructose Safe A

%A!/IO-AS%&A! S"ST') /iuretics 8ruse5ide &nsafe A

8usidic Acid Safe

TH' '"' .enta5icin Safe AI(8'%TIO( .enta5ycin Safe %%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% .lafenine Safe %

.libencla5ide &nsafe Associated Cith acuteattacks of porphyria$

'(/O%!I(' S"ST') Anti=/iabetic Agents .lipiDide &nsafe

'(/O%!I(' S"ST') Hor5one reparations .lucagon Safe /

(&T!ITIO( A(/ OO/ .lucose Safe A



41/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics .lutethi5ide &nsafe A

%A!/IO-AS%&A! S"ST') .lyceryl Trinitrate Safe

)&S%&O=S3''TA and @OI(T /IS'AS' Specific Anti=!heu5atic Agents .old=*Sodiu5 Aurothio5alate+ &nsafe %

'(/O%!I(' S"ST') Hor5one reparations .oserelin Safe

.ra5icidin &nsafe

I(8'%TIO( .riseoful6in &nsafe A

TH' S3I( .riseoful6in &nsafe A

!'SI!ATO!" S"ST') .uaiphenesin &nsafe %TH' '"' .uanethidine Safe %

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents .uanethidine Safe A

%A!/IO-AS%&A! S"ST') Ant i=hypertensi6e Agents .uanfacine H% Safe /

(&T!ITIO( A(/ OO/ Hae5 Arginate Safe A

A(A'STH'SIA Haloperidol %ontentious &

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics Haloperidol %ontentious &

A(A'STH'SIA Halothane %ontentious &

%A!/IO-AS%&A! S"ST') Anticoagulants Heparin Safe %A!/IO-AS%&A! S"ST') Hepta5inol H% Safe /

I(8'%TIO( He>a5ine Safe %

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics He>apropy5ate &nsafe /

%'(T!A ('!-O&S S"ST') Anticon6ulsants Hydantoins &nsafe A

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents HydralaDine &nsafe

%A!/IO-AS%&A! S"ST') /iuretics HydrochlorothiaDide &nsafe

)&S%&O=S3''TA and @OI(T /IS'AS' %orticosteroids Hydrocortisone %ontentious STH' S3I( Hydrocortisone utyrate %ontentious S

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics Hydro>yDine &nsafe

Hyoscine &nsafe

OST'T!I%S, ."(A'%OO." A(/ !'(A /IS'AS' Hyoscine utylbro5ide &nsafe A

.AST!OI(T'STI(A S"ST') Hyoscine utylbro5ide &nsafe A

TH' '"' Hyoscine utylbro5ide &nsafe A

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics Hyoscine utylbro5ide &nsafe A

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% Ibuprofen Safe

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents Ibuprofen Safe

%'(T!A ('!-O&S S"ST') sychoanaleptics I5ipra5ine %ontentious &

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% Indo5ethacin %ontentious S

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents Indo5ethacin %ontentious S

'(/O%!I(' S"ST') Anti=/iabetic Agents Insulin Safe A

%'(T!A ('!-O&S S"ST') sychoanaleptics IproniaDid &nsafe

Iron Safe(&T!ITIO( A(/ OO/ Iron reparations Safe AA(A'STH'SIA Isoflurane &nsafe %

%'(T!A ('!-O&S S"ST') )igraine Iso5etheptene5ucate &nsafe

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics Iso5etheptene=)ucate &nsafe %

I(8'%TIO( IsoniaDid %ontentious &

I(8'%TIO( @osa5ycin Safe /

3ebuDone &nsafe

A .&I/' 8O! 'O' WITH O!H"!IA age :


42/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1A(A'STH'SIA 3eta5ine %ontentious S

I(8'%TIO( 3etoconaDole &nsafe /

TH' S3I( 3etoconaDole &nsafe /

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents 3etoprofen Safe %

!'SI!ATO!" S"ST') 3etotifen Safe /

$H$!$H$ Safe

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents abetalol Safe

e6onorgestrol &nsafe Associated Cith acuteattacks of porphyria$

A(A'STH'SIA ignocaine &nsafe %

.AST!OI(T'STI(A S"ST') i?uorice Safe

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents isinopril &nsafe

%'(T!A ('!-O&S S"ST') sychoanaleptics ithiu5 Salts Safe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics ofepra5ine Safe

%'(T!A ('!-O&S S"ST') sychoanaleptics ofepra5ine Safe

.AST!OI(T'STI(A S"ST') opera5ide Safe /

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics opraDola5 &nsafe /

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics oraDepa5 %ontentious S

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics o>apine &nsafe /

ynestrenol &nsafe Associated Cith acuteattacks of porphyria$

%'(T!A ('!-O&S S"ST') )igraine ysuride )aleate &nsafe %

.AST!OI(T'STI(A S"ST') )agnesiu5 Sulphate Safe %'(T!A ('!-O&S S"ST') Anticon6ulsants )agnesiu5=Sulphate Safe

%'(T!A ('!-O&S S"ST') sychoanaleptics )aprotiline H% &nsafe %

I(8'%TIO( )ebendaDole %ontentious S

.AST!OI(T'STI(A S"ST') )ebe6erine H% &nsafe /

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents )eca5yla5ine Safe %

)ecillina5 &nsafe Associated Cith acuteattacks of porphyria$

)eclofeno>ate H% Safe

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics )ecloDine Safe A

)edro>yprogesterone &nsafe Associated Cith acuteattacks of porphyria$

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% )efena5ic Acid %ontentious &

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents )efena5ic Acid %ontentious &

I(8'%TIO( )eflo?uine H% Safe /

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( )egestrol &nsafe %

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( )elphalan %ontentious S

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics )ephenesin &nsafe /

)ephenytoin &nsafe Associated Cith acuteattacks of porphyria$

A(A'STH'SIA )epi6acaine &nsafe /

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics )eproba5ate &nsafe A

A(A'STH'SIA )eptaDinol Safe

A .&I/' 8O! 'O' WITH O!H"!IA age ;


43/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S )eptaDinol Safe

!'SI!ATO!" S"ST') )e?uitaDine Safe

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( )ercaptopurine &nsafe

TH' '"' )ercuric O>ide &nsafe

)ercury %o5pounds &nsafe

%A!/IO-AS%&A! S"ST') /iuretics )ersalyl %ontentious S

)estranol &nsafe

%'(T!A ('!-O&S S"ST') sychoanaleptics )etapra5ine H% %ontentious /&'(/O%!I(' S"ST') Anti=/iabetic Agents )etfor5in Safe %

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S )ethadone Safe A

%'(T!A ('!-O&S S"ST') Appetite Suppressants )etha5pheta5ine &nsafe

A(A'STH'SIA )ethohe>itone &nsafe %

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( )ethotre>ate &nsafe %

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics )ethotri5epraDine %ontentious S

)etho>yflurane &nsafe

%'(T!A ('!-O&S S"ST') Anticon6ulsants )ethsu>i5ide &nsafe A)ethyl /opa &nsafe Associated Cith acute

attacks of porphyria$

)ethyl Sulphonal &nsafe Associated Cith acuteattacks of porphyria$

%'(T!A ('!-O&S S"ST') Appetite Suppressants )ethylcellulose Safe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics )ethylphenidate Safe

%'(T!A ('!-O&S S"ST') sychoanaleptics )ethylphenidate Safe /'(/O%!I(' S"ST') Hor5one reparations )ethyluracil Safe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics )ethyprylone &nsafe A

)ethysergide &nsafe

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents )etipropanolol Safe

.AST!OI(T'STI(A S"ST') )etoclopra5ide %ontentious &

)&S%&O=S3''TA and @OI(T /IS'AS' )usc le !ela>ants and Anti=Spas5odics )etoclopra5ide %ontent ious &

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics )etoclopra5ide %ontentious &.AST!OI(T'STI(A S"ST') )etopi5aDine Safe /

)etoprolol Safe

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents )etoprolol Tartrate Safe

I(8'%TIO( )etronidaDole %ontentious S

'(/O%!I(' S"ST') Hor5one reparations )etyrapone &nsafe /

%'(T!A ('!-O&S S"ST') sychoanaleptics )ianserin H% &nsafe %

I(8'%TIO( )iconaDole &nsafe /

TH' S3I( )iconaDole &nsafe /

A(A'STH'SIA )idaDola5 Safe /

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics )idaDola5 %ontentious /S

OST'T!I%S, ."(A'%OO." A(/ !'(A /IS'AS' )ifepristone %ontentious &

%'(T!A ('!-O&S S"ST') sychoanaleptics )inaprine H% Safe /

A(A'STH'SIA )ina>olone Safe %

I(8'%TIO( )inocycline H% Safe /

)ino>idil &nsafe

A .&I/' 8O! 'O' WITH O!H"!IA age alate Safe /

I(8'%TIO( (alidi>ic Acid &nsafe

'(/O%!I(' S"ST') Hor5one reparations (androlone %ontentious &

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% (apro>en Safe )&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents (apro>en (a Safe %

I(8'%TIO( (ata5ycin &nsafe

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% (efopa5 H% Safe /

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics (eostig5ine Safe A

I(8'%TIO( (etil5ycin Safe /

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% (icergoline %ontentious /&

%A!/IO-AS%&A! S"ST') %alciu5 %hannel lockers (ifedipine &nsafe

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents (ifu5ic Acid Safe /

!'SI!ATO!" S"ST') (iketha5ide &nsafe A

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics (itraDepa5 &nsafe

I(8'%TIO( (itrofurantoin %ontentious &

A(A'STH'SIA (itrous O>ide Safe A

(ordaDepa5 &nsafe

(orethisterone &nsafe Associated Cith acute

attacks of porphyria$(orethynodrel &nsafe

I(8'%TIO( (orflo>acin Safe /

%'(T!A ('!-O&S S"ST') sychoanaleptics (ortriptyline %ontentious &

I(8'%TIO( (o6obiocin &nsafe

OST'T!I%S, ."(A'%OO." A(/ !'(A /IS'AS' Oestrogens %ontentious &

I(8'%TIO( Oflo>acin Safe /

.AST!OI(T'STI(A S"ST') O5epraDole %ontentious SOST'T!I%S, ."(A'%OO." A(/ !'(A /IS'AS' Oral %ontracepti6es &nsafe A

'(/O%!I(' S"ST') Hor5one reparations Oral %ontracepti6es &nsafe A

%'(T!A ('!-O&S S"ST') )igraine Orphenadrine &nsafe %

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics Orphenadrine &nsafe

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics O>ana5ide &nsafe %

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics O>aDepa5 %ontentious &

%'(T!A ('!-O&S S"ST') Anticon6ulsants O>aDolidinediones &nsafe

I(8'%TIO( O>olinic Acid Safe /

TH' '"' O>ybuprocaine Safe %

O>ybutynin H% &nsafe

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S O>ycodone &nsafe %

TH' 'A!, (OS' A(/ O!OHA!"( O>y5etaDoline &nsafe

%A!/IO-AS%&A! S"ST') O>ypentifylline &nsafe /

TH' '"' O>yphenbutaDone &nsafe

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents O>yphenbutaDone &nsafe A



45/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1O>ytetracycline &nsafe

OST'T!I%S, ."(A'%OO." A(/ !'(A /IS'AS' O>ytocin Safe

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics ancuroniu5 %ontentious S

ancuroniu5 ro5ide Safe (ote 1

A(A'STH'SIA ancuronu5 r %ontentious S

%'(T!A ('!-O&S S"ST') Analgesics (O(=(A!%OTI% araceta5ol Safe A

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents araceta5ol Safe

%'(T!A ('!-O&S S"ST') Anticon6ulsants araldehyde Safe %'(T!A ('!-O&S S"ST') Anticon6ulsants ara5ethadione &nsafe A

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics arapenDolate r Safe /

%'(T!A ('!-O&S S"ST') sychoanaleptics argyline &nsafe %

I(8'%TIO( eflo>acin Safe /

)&S%&O=S3''TA and @OI(T /IS'AS' Specific Anti=!heu5atic Agents enicilla5ine Safe

I(8'%TIO( enicillin Safe A

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S entaDocine &nsafe A

entoliniu5 Safe!'SI!ATO!" S"ST') entylenetetraDol &nsafe

erhe>iline &nsafe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics ericyaDine Safe /

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S ethidine Safe A

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S henacetin &nsafe %

%'(T!A ('!-O&S S"ST') sychoanaleptics henelDine &nsafe %

'(/O%!I(' S"ST') Anti=/iabetic Agents henfor5in Safe %%'(T!A ('!-O&S S"ST') Anticon6ulsants henobarbitone &nsafe A

henoperidine Safe

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents heno>ybenDa5ine &nsafe /

%'(T!A ('!-O&S S"ST') Anticon6ulsants hensu>i5ide &nsafe A

hentola5ine )esylate Safe

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents henylbutaDone %ontentious &

henylhydraDine &nsafe

%'(T!A ('!-O&S S"ST') Anticon6ulsants henytoin &nsafe A

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents ipebuDone &nsafe /

I(8'%TIO( ipe5idic Acid &nsafe /

%'(T!A ('!-O&S S"ST') sychoanaleptics ipothiaDine al5itate Safe /

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics iraceta5 Safe /

irbuterol Safe

.AST!OI(T'STI(A S"ST') irenDepine Safe

iritra5ide &nsafe)&S%&O=S3''TA and @OI(T /IS'AS' Anti=.out Agents iro>ica5 &nsafe %)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents iro>ica5 &nsafe /

I(8'%TIO( i6a5picillin &nsafe

i65ecillina5 &nsafe Associated Cith acuteattacks of porphyria$

%'(T!A ('!-O&S S"ST') )igraine iDotifen Safe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics raDepa5 &nsafe /



46/49

%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1%A!/IO-AS%&A! S"ST') raDosin %ontentious S

TH' '"' rednisolone %ontentious S

)&S%&O=S3''TA and @OI(T /IS'AS' %orticosteroids rednisolone %ontentious S

'(/O%!I(' S"ST') Hor5one reparations rednisolone %ontentious S

%A!/IO-AS%&A! S"ST') %alciu5 %hannel lockers renyla5ine &nsafe /

A(A'STH'SIA rilocaine &nsafe

I(8'%TIO( ri5a?uine Safe

%'(T!A ('!-O&S S"ST') Anticon6ulsants ri5idone &nsafe A)&S%&O=S3''TA and @OI(T /IS'AS' Anti=.out Agents robenecid %ontentious

%A!/IO-AS%&A! S"ST') robucol Safe /

%A!/IO-AS%&A! S"ST') rocaina5ide H% Safe /

A(A'STH'SIA rocaine Safe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics rochlorperaDine Safe

%'(T!A ('!-O&S S"ST') )igraine rochlorperaDine Safe

%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics rochlorperaDine Safe

%'(T!A ('!-O&S S"ST') Anticon6ulsants rogabide &nsafe /rogesterone &nsafe Associated Cith acute

attacks of porphyria$

'(/O%!I(' S"ST') Hor5one reparations rogestogens %ontentious &

roguanil H% Safe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics ro5aDine Safe A

!'SI!ATO!" S"ST') Antihista5ines ro5ethaDine %ontentious &

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics ro5ethaDine %ontentious &%'(T!A ('!-O&S S"ST') Tran?uillisersAnti='5etics ro5ethaDine %ontentious &

A(A'STH'SIA ropanidid %ontentious &

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents ropanolol Safe A

.AST!OI(T'STI(A S"ST') ropantheline Safe

OST'T!I%S, ."(A'%OO." A(/ !'(A /IS'AS' ropantheline Safe

)&S%&O=S3''TA and @OI(T /IS'AS' )usc le !ela>ants and Anti=Spas5odics ropanthel ine r Safe

A(A'STH'SIA ropofol Safe

ropranolol Safe

'(/O%!I(' S"ST') Hor5one reparations ropylthiouracil Safe

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents ropyphenaDone &nsafe A

A(A'STH'SIA ro>y5etacaine %ontentious S

TH' '"' ro>y5etacaine %ontentious S

!'SI!ATO!" S"ST') seudoephedrine H% Safe

I(8'%TIO( yraDina5ide &nsafe A

(&T!ITIO( A(/ OO/ yrido>ine H% Safe A

I(8'%TIO( yri5etha5ine %ontentious S

yrrocaine &nsafe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics Juinalbarbitone &nsafe

%A!/IO-AS%&A! S"ST') Juinidine Safe %

I(8'%TIO( Juinine Safe

.AST!OI(T'STI(A S"ST') !anitidine %ontentious &

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents !eserpine Safe A



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%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1TH' S3I( !esorcinol Safe

I(8'%TIO( !ifa5picin &nsafe

!'SI!ATO!" S"ST') Salbuta5ol Safe

.AST!OI(T'STI(A S"ST') Senna Safe A

%A!/IO-AS%&A! S"ST') Si56astatin &nsafe %

Sodiu5 Aurothio5alate &nsafe

Sodiu5 ro5ide Safe

(&T!ITIO( A(/ OO/ Sodiu5 %alciu5 'detate Safe AI(8'%TIO( Sodiu5 8usidate Safe

Sodiu5 O>ybate &nsafe

%'(T!A ('!-O&S S"ST') Anticon6ulsants Sodiu5 -alproate &nsafe & (ote 2

.AST!OI(T'STI(A S"ST') Sorbitol Safe A

%A!/IO-AS%&A! S"ST') /iuretics Spironolactone &nsafe %

TH' S3I( L %arotene Safe A

'(/O%!I(' S"ST') Hor5one reparations StanoDolol &nsafe %

I(8'%TIO( Strepto5ycin Safe A%'(T!A ('!-O&S S"ST') Anticon6ulsants Succini5ides &nsafe A

(&T!ITIO( A(/ OO/ Sulbutia5ine Safe /

Sulfado>ine Safe

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents Sulindac Safe %

TH' '"' Sulphaceta5ide &nsafe %

SulphadiaDine &nsafe Associated Cith acute

attacks of porphyria$Sulphadi5idine &nsafe Associated Cith acuteattacks of porphyria$

Sulphado>ine &nsafe Associated Cith acuteattacks of porphyria$

Sulpha5etho>aDole &nsafe Associated Cith acuteattacks of porphyria$

.AST!OI(T'STI(A S"ST') SulphasalaDine &nsafe %

)&S%&O=S3''TA and @OI(T /IS'AS' Anti=.out Agents SulphinpyraDone &nsafe &

I(8'%TIO( Sulphona5ides &nsafe A

'(/O%!I(' S"ST') Anti=/iabetic Agents Sulphonylureas &nsafe A

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics Sulpiride &nsafe %

%'(T!A ('!-O&S S"ST') Anticon6ulsants Sulthia5e &nsafe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics Sultopride &nsafe /

A(A'STH'SIA Su>a5ethoniu5 Safe %

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics Su>a5ethoniu5 Safe A

I(8'%TIO( Tala5picillin Safe )AI.(A(T /IS'AS' and I))&(OS&!'SSIO( Ta5o>ifen &nsafe

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics Te5aDepa5 Safe

!'SI!ATO!" S"ST') Antihista5ines Terfenadine &nsafe

A(A'STH'SIA Tetracaine Safe %

I(8'%TIO( Tetracyclines %ontentious S

TH' '"' Tetracyclines %ontentious S



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%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics TetraDepa5 &nsafe /

!'SI!ATO!" S"ST') Theophylline &nsafe

A(A'STH'SIA Thiopentone (a &nsafe A

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics ThioridaDine &nsafe

'(/O%!I(' S"ST') Hor5one reparations Thiouracil Safe

'(/O%!I(' S"ST') Hor5one reparations Thyro>ine Safe

)&S%&O=S3''TA and @OI(T /IS'AS' (on=Steroidal Anti=Infla55atory Agents Tiaprofenic Acid Safe

I(8'%TIO( Ticarcillin Safe %A!/IO-AS%&A! S"ST') /iuretics Tienilic Acid Safe /

%'(T!A ('!-O&S S"ST') Analgesics (A!%OTI%S Tilidate &nsafe

TH' '"' Ti5olol )aleate Safe /

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents Ti5olol )aleate Safe /

I(8'%TIO( TinidaDole &nsafe /

TolaDa5ide &nsafe Associated Cith acuteattacks of porphyria$

%A!/IO-AS%&A! S"ST') Anti=hypertensi6e Agents TolaDoline Safe A

Tolbuta5ide &nsafe Associated Cith acuteattacks of porphyria$

%A!/IO-AS%&A! S"ST') Trane>a5ic Acid Safe

%'(T!A ('!-O&S S"ST') sychoanaleptics Tranylcypro5ine &nsafe %

%'(T!A ('!-O&S S"ST') sychoanaleptics TraDodone H% &nsafe %

Triacetyloleando5ycin Safe

Tria5terene Safe%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics TriaDola5 Safe %

%A!/IO-AS%&A! S"ST') /iureti cs Trich lor5eth iaDide %ontent ious S

%'(T!A ('!-O&S S"ST') Hypnotics, Sedati6es and An>iolytics TrifluoperaDine Safe

.AST!OI(T'STI(A S"ST') Tri5ebutine )aleate Safe /

!'SI!ATO!" S"ST') Antihista5ines Tri5epraDine Tartrate Safe /

%A!/IO-AS%&A! S"ST') Tri5etaDidine H% Safe /

I(8'%TIO( Tri5ethopri5 &nsafe %'(T!A ('!-O&S S"ST') sychoanaleptics Tri5ipra5ine %ontentious &

!'SI!ATO!" S"ST') Antihista5ines Tripelenna5ine Safe

%'(T!A ('!-O&S S"ST') Anticon6ulsants Tro>idone &nsafe

A(A'STH'SIA Tubocurarine Safe %

)&S%&O=S3''TA and @OI(T /IS'AS' )uscle !ela>ants and Anti=Spas5odics Tubocurarine Safe

-alproate &nsafe

%'(T!A ('!-O&S S"ST') Anticon6ulsants -alpro5ide %ontentious /&

I(8'%TIO( -anco5ycin Safe

%'(T!A ('!-O&S S"ST') sychoanaleptics -eralipride &nsafe /

%A!/IO-AS%&A! S"ST') %alciu5 %hannel lockers -erapa5il &nsafe

I(8'%TIO( -ibra5ycin &nsafe

%'(T!A ('!-O&S S"ST') sychoanaleptics -ilo>aDine H% &nsafe %

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( -inblastine %ontentious &/

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( -incristine %ontentious &/

(&T!ITIO( A(/ OO/ -ita5ins Safe



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%y1te"/Area %u2 Area Dru3 U1ea24ty Te1t 'o""e#t1%A!/IO-AS%&A! S"ST') Anticoagulants Warfarin (a Safe

I(8'%TIO( Kido6udine Safe

)AI.(A(T /IS'AS' and I))&(OS&!'SSIO( Kido6udine Safe

TH' S3I( Kinc reparations *Topical+ Safe

TH' '"' Kinc Sulphate Safe A

%'(T!A ('!-O&S S"ST') sychoanaleptics Kuclopenthi>ol &nsafe %


A Guide fosdsasdr People With Porphyria

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Transcript of A Guide fosdsasdr People With Porphyria