R ev ist a d a S o c ie d ad e B r as ile ir a d e M e d ic in a T r o p ic al 2 9 ( 3 ) :2 1 9 - 2 2 8 ,m ai- j u n , 1 9 9 6 .
A RH GO S
PO LYCYSTIC HYD A TID DISEASE IN BRA ZIL. REPO RT O F
FIV E N EW HUM A N CASES A N D A SHO RT REV IEW O F
O THER PUBLISHED OBSERVATIONS
A. D’A lessandro, M A P. M onies and A.N. Raick
T his p a p e r d e s c r ib e s f i v e a d d i t i o n a l B r az ilian h u m an c as e s o f p o ly c y s t ic h y d at id d is e as e d u e to Echinococcus vogeli, r ev iew s t h e p r e v io u s c as e s r e p o r t e d in B r az il, in c lu d in g o n e r e p o r t o fE . oligarthus ( 2 0 in total) , a n d s o m e e p id e m io lo g ic al as p e c t s o f this d is e as e w h ic h is n o lo n g e r a c u r io s it y b u t r at h e r a p r o b le m t h at is n o t m e d ic ally e as y to h an d le . Its p r e s e n c e s h o u ld b e e x p e c t e d in a n y r u r al a r e a o f t he N ew W o rld w h e r e h u m a 7 i s h a v e n o t e lim in at e d w ild fe lid s /c an id s , b u s h dog s, p a c a s , ag o u t is a n d o t h e r w ild r o den t s.
K e y - w o r ds: P o ly c y st ic h y d at id d is e as e . Echinococcus vogeli. B r az il.
E c h i n o c o c c u s g r a n u lo s u s is kno w n to b e end em ic in hum ans, ung ulates and d o m estic d o g s in Uruguay , A rgentina, Chile, the A nd ean z o ne o f Peru and Rio G rand e d o Sul, Brazil. H o w ev er, the p arasite p ro b ab ly is p resent in ev ery co untry in the co ntinental A m ericas5, but sp o rad ic hum an cases are rare o r se en in im m igrants fro m end em ic areas o f the sam e o r ano ther co untry . Th e reaso n fo r this is no t clear, b ut it m ay b e d ue to ep id em io lo g ical circum stances that p rev ent transm issio n, o r to the b ehav io r o f the strain o f the p arasite p resent in no n-end em ic areas.
In ad d itio n, hyd atid s sho w ing a p o ly cy stic m o rp ho lo g y , rather than unilo cular, had b een o b serv ed in hum ans and anim als and w ere c a ta lo g e d as a lv e o la r , m u ltic y s tic o r m ultilo cular hyd atid s and interp reted as d ue to E. g r an u lo s u s , E. m u lt ilo c u lar is (cy cle canid - sm all ro d ent, in the arctic z o nes) o r m o re recently to E. o lig ar t h r u s (w ild cat-ag o uti cy cle, m o stly in tro p ical areas). H o w ev er, these d ia g n o s e s w e r e n o t s u b s ta n tia te d b y exp erim ental infectio ns in carniv o res to o b tain th e a d u lt w o rm s w h ic h a re e a s y to d ifferentiate.
D ep artm ent o f T ro p ical M ed icine,T ulan e U niversity S chool o f Pub lic H ealth and T rop ical M ed icine, N ew O rleans, LA, USA , Fu n d ação S erv iços d e Saúde Púb lica, M inistério de S aúd e, Belém , PA an d D ep artam en to d e Pato log ia, U niversid ade d e Brasilia, Brasilia, DF, Brasil.
A d d r e s s to : D r. A . D ’A lessand ro. D ep artm en t o f Tropical M ed icine, Tulane U niversity S cho o l o f Pub lic H ealth and T ro p ical M ed icine, 1 5 0 1 C anal S treet, N ew O rleans, LA 7 0 1 1 2 , USA . Fax: 5 0 4 5 8 7 - 7 3 1 3 .
R eceb id o p ara p u b licação em 2 0 / 0 6 / 9 5 .
Fo rtunately, w hile w o rking in Co lo m bia, w e w ere ab le to infect d o g s and cats w ith p o ly cy stic hyd atid m etacesto d es o f hum an and anim al o rigin and d em o nstrated the p resence in th e c o u n tr y o f th e tw o n e o tr o p i c a l E c h in o c o c c u s sp ecies E. o lig ar t h u s and E. v o g e li (Ev ). W e d escrib ed the larv al characteristics o f Ev, also p o ly cy stic, in the o nly im p o rtant kno w n ho st, the p aca (C u n ic u lu s p a c a ) . The ad ult p arasite had b een d escribed b y Rausch an d Be rn ste in 26 in a b u sh d o g (S p e o t h o s
v e n at ic u s ) fro m Ecuad o r and , as they had
p red icted d ue to the fo o d habits o f the bush d o g, the interm ed iate ho st w as fo u nd to b e the p aca.
A s m e n tio n e d a b o v e , e x p e r im e n ta l infectio ns d em o nstrated that the m etacesto d es o f the tw o neo tro p ical sp ecies w ere b o th p o ly cy stic. Th e d ifferential m o rp ho lo g ical characteristics w ere stud ied and d escribed . It w as fo u nd that the sh ap e, the relativ e p ro p o rtio n o f hand le and guard / blad e and the size o f the ho o klets o f the p ro to sco lices w ere o f p articular im p o rtance7 8 28 29.
Since the o rig inal d escrip tio n o f p o ly cy stic hyd atid d isease (PH D ) in 1979 inv o lv ing 13 cases in 4 co u ntries, no less than 63 hum an cases d ue to Ev (arid also E. o lig ar t h u s ) hav e b e e n fo und in 10 co untries, includ ing Brazil.
D A le s s an d r o A , M o r ae s M A P, R aic k A N . P o ly c y st ic h y d at id d is e as e in B r az il. R e p o rt o f f i v e n e w h u m an c as e s a n d a s h o r t r e v ie w o f o t h e r p u b lis h e d o bs e r v at io n s . R ev ist a d a S o c ie d ad e B r as ile ir a d e M e d ic in a T r o p ic al 2 9 :2 1 9 - 2 2 8 , m ai- ju n , 1 9 9 6 .
D ’A le s s an d r o A , M o r ae s M A P, R aic k A N . P o ly c y st ic h y d at id d is e as e in B r az il. R e p o rt o f fiv e n e w h u m an c as e s a n d a s h o r t r e v ie w o f o t h e r p u b li s h e d o bser v at io n s. R ev ist a d a S o c ie d ad e B r as ile ir a d e M e d ic in a T r o p ic al 2 9 :2 1 9 - 2 2 8 , m ai- ju n , 1 9 9 6 .
P o l y c y s t ic h y d a t i d i n f e c t i o n s in B r a z i l i a n a n i m a l s . Brum p t and Jo y e u x 2 fo und in the liv er and sp leen o f 1 o f 4 ag o utis (D a s y p r o c t a a g o u t i = D . l e p o r i n a ) c o l l e c t e d in A lburquerque, Lins, State o f Sao Paulo , a p o ly cy stic hyd atid that they d escribed as the m e t a c e s t o d e o f a n e w s p e c i e s o f E c b i n o c o c c u s : E . c r u z i r a th e r th a n E . o l ig a r t h r u s . Based o n a careful study o f the d ifferential characteristics o f the 2 neo tro p ical sp ecies, Ev and E . o l ig a r t h r u s , fro m d ifferent o rig ins and the o rig inal slid es o f E. c r u z i, it w as arriv ed to the co nclu sio n that the ro stellar ho o klets fro m p ro to sco lices o f E. o l ig a r t h r u s and E . c m z i co rresp o nd ed in fo rm , altho ug h tho se fro m the latter w ere slightly g reater in leng th. The d ifference w as co nsid ered o f no taxo no m ic sig nificance and therefo re they p lac e d E . c r u z i in sy no ny m y w ith E. o l i g a r t h r u s26.
The o ccu rrence o f the larv al stag e o f E c b i n o c o c c u s sp p in the A m ericas b y sp ecies o f ho st and co untry w ere sum m arized 8. In Brazil, o ther m etacesto d es co llected in ago utis n e a r B e le m w e re a ls o f o u n d to b e E . o l i g a r t h r u sb ut the rep o rt o f E . g r a n u l o s u sin a Braz ilian ag o u ti b y Lutz lacks su fficient info rm atio n to allo w a d efinitiv e taxo no m ic o p inio n. In ad d itio n to these o bserv atio ns, E. o l i g a r t h r u s has b e e n rep o rted in v ario us co untries and in a few sp ecies o f m am m als; P r o e c h i m y s sp p . (sp iny rat), and o nce in C u n i c u l u s p a c a , and in D i d e l p h i s m a r s u p i a l i s (o p o ssu m ). M o re recently Ev w as fo und in S y l v i l a g u s f l o r i d a n u s (w ild rab b it) fro m V enez uela16 and in p acas fro m Para State (M o raes, this rep o rt); in A cre State21; in Bo liv ia12 and in Ecuad o r1 u .
The carniv o res rep o rted naturally infected w ith E. o l i g a r t h r u s are the fo llo w ing w ild cats- .Felis y a g o u a r o u n d i , F. c o n c o l o r , F. o n c a , F. p a r d a l i s , F. g e o f f r o y iand F. c o lo c o lc P ; sev eral o f these cats hav e ex ten siv e g eo g rap hic rang es. The w id est is that o f the co ug art, F e lis c o n c o l o r , w hich o ccu rs in no rthen British Co lum bia, Canad a, and so uthw ard to Tierra d el Fu eg o . So m e, su ch as the o c elo t and the jaguar, w hich fo rm ely w ere p resent in SW the US seem to hav e d isap p eared fro m this co untry . O n the co ntrary , the b u sh d o g, S p e o t h o s v e n a t i c u s (Fig ure 1), d istributed fro m Panam a to n o rth en A rg entina, and the d o m estic d o g, are the o nly anim als that have b een rep o rted naturally infected w ith Ev and
the p aca is the o nly im p o rtant interm ed iate ho sts ('Figure 2).
F i g u r e 1 - B u s h d o g , S p eo tho s v en aticu s. P h o t o c o u rt e s y o f t h e L o s A n g e l e s Z o o .
F i g u r e 2 - A d u l t p a c a , C u niculus p aca. P h o t o c o u rt e s y o f D r. I g n a c i o B o r re r o , U n i v e rs i d a d a e l V alle , C a li, C o lo m b i a .
It sho u ld b e m en tio n ed that PH D transm issio n has b een rep o rted in z o o s w here b u sh d o gs serv ed as a so urse o f infectio n fo r nutrias, M y o c a s t o r c o y p u si9, and a g ro up o f primates: gorillas, o rangutans and chim p anz ees15 23. This info rm atio n sho uld b e useful to alert z o o v eterinarians to d etect infectio ns in cap tiv e b u sh d o g s, using antip arasitic d rugs to reco v er the ad ult w o rm s and in p rim ates, u sing u ltraso u nd p ro ced u res to d etec t the m etacesto d es.
D ia g n o s is . It seem s p ertinent in this sho rt rev iew to m entio n ho w the d iag no sis o f PHD is carried o ut in hum ans.
D A le s s an d r o A , M o r ae s M A P, R aic k A N . P o ly c y st ic h y d at id d is e as e in B r az il. R e p o rt o f f i v e n e w h u m an c as e s a n d a s h o r t r e v ie w o f o t h e r p u b li s h e d o bser v at io n s. R ev ist a d a S o c ie d ad e B r as ile ir a d e M e d ic in a T r o p ic al 2 9 :2 1 9 - 2 2 8 , m ai- ju n , 1 9 9 6 .
procedures-, x ray, sho w ing po lycystic tumors, usually w ith so m e calcificatio ns 2-3cm o r mo re in diam eter w ith irregular bo rders, but th e y m ay h a v e d iv e r s e f o rm s an d co nfiguratio ns. Ultraso und, CT and MRI are more so phisticated and exp ensiv e pro cedures but useful fo r diagno sis19 22. The m etacesto d e is usually present simultaneo usly in various organs o f the up p er and lo w er abdo m en, the c h e s t, and th e o rb it ( D ’A le ssan d ro , unp ublished ).
2. The patient lives o r has lived in rural areas w ith abundant w ild life, and usually is familiar w ith pacas.
3. Sero lo g ical tests. IHA and Im m uno blo t usually are po sitiv e in cases diagno sed by o ther means. Very recently, Go ttstein et al 199513 have o btained a purified Ev antigen, Ev2, allo w ing d iscrim inatio n o f no n- E c h i n o c o c c u s i n fe c t io n s and E. g r a n u lo s u s infectio ns fro m Ev. O nly so m e alv eo lar hydatid cases co uld no t be discriminated. How ever, the distributio n o f E . m u lt ilo c u lar is is ho lartic rather than neo tro p ical so its d istributio n d o es no t o v erlap in these regio ns.
4. Parasito lo g ical characteristics o f PHD o btained by bio psy, patho lo gical specimens o r necro p sy: a) the shap e, pro po rtio ns o f the parts o f the p ro to sco lex ho o klets (better seen in sq u ash p re p a ra tio n s ) an d b ) th e m o rp ho lo gy o f the bo d y o r w alls o f the hydatid in tissue sectio ns stained w ith H & E and PAS6 29.
C ase R ep o rts
C a s e 1. (M VS (A 93-87). A 31-y ear-o ld
Braz ilian fem ale agricultural w o rker, b o rn in G o ias State but living in M ato G ro sso at the tim e o f p resentatio n, w as ad m itted to the teaching ho sp ital o f Brasilia in O cto b er 1987 c o m p la in in g o f an e n la r g e d a b d o m e n , diarrhea, increasing jaund ice, and g eneraliz ed itching since a few m o nths. She had b een d iag no sed as hav ing viral hep atitis o ne m o nth earlier in ano ther ho sp ital. A t ad m issio n she w as fo und to b e in p o o r g eneral health, w ith a histo ry o f a 12kg w eig ht lo ss during the p rev io us w eeks. She w as m arked ly jaund iced , and the p h y sic al exam rev ealed p alm ar ery them a and signs o f g eneraliz ed skin lesio ns d ue to scratc hing . Th e ab d o m en w as d istend ed and the liv er w as hard and p alp able
20cm b elo w the rig ht co stal margin. No ascites o r ab d o m in al c o lla te ra l c irc u latio n w e re d e te c te d . M ain la b o r a to r y f in d in g s at ad m issio n includ ed : to tal bilirubin, 33mg/ dl w ith the d irect fractio n o f 21mg/ dl; alkaline p ho sp hatase, 57U; A ST 36U; ALT 40U; to tal p ro tein 6.8gr/ dl, album in 3.2gr/ dl; creatinine 0.7/ dl. The W BC co u nt w as no rm al but 11% eo sino p hils w as rep o rted . The ab d o m en ultraso und sho w ed enlarg ed liv er and sp leen w ith a cav erno us m ass in the p o rta hep atis, d ilated intrahep atic b ile d ucts, signs o f p o rtal thro m bo sis, and a calcified no d u le in the liver (2cm x 2cm ). W ith the d iag no sis o f o bstructiv e ja u n d ic e an d p o r ta l h y p e r te n s i o n , an exp lo rato ry lap aro to m y w as carried o ut o ne m o nth after ad m issio n. Exam inatio n o f the p o rta hep atis sho w ed enlarg ed , recanaliz ed u m bilical v eins. The g allblad d er ap p eared shriv eled and w itho u t calcu li. W hen the co m m o n bile d uct w as o p ened , m ultip le sm all v e s i c l e s w e r e s e e n ( F ig u r e 3 ) . A n intrao p erativ e cho lang io g ram rev ealed the left hep atic bile d uct to b e o b stm cted . The right
F i g u r e 3 - Ech in o co ccu s v o g eli c y s t o b t a i n e d f r o m t h e li v e r a n d t h e b i li a ry d r a i n o f c a s e 1 . N o t e t h e d iv e rs i t y o f s i z e a n d t h e p r e s e n c e o f b r o o d c a p s u le s , f r e e a n d w it h in t h e cy s t s . B a r = 1 c m .
D ’A le s s an d r o A , M o r ae s M A P, R aic k A N . P o ly c y st ic h y d at id d is e as e in B r az il. R e p o r t o f f i v e n e w h u m an c as e s a n d a s h o r t r e v ie w o f o t h e r p u b li s h e d o bs e r v at io n s . R ev ist a d a S o c ie d ad e B r as ile ir a d e M e d ic in a T r o p ic al 2 9 :2 1 9 - 2 2 8 , m ai- ju n , 1 9 9 6 .
hep atic b ile d uct w as im p erm eable and the cy stic d uct w as fibro tic. A cho lecy stecto m y w as p erfo rm ed and external d rainag e o f the biliary sy stem w as established . Tw o days fo llo w ing surg ery an ep iso d e o f b leed ing fro m the surgical excisio n and the externaliz ed drain w as o b serv ed , and fluid fro m the d rain co ntained p arasitic cysts. Tw o w eeks after su rg ery the p atient e x p e rie n c e d m assiv e hem atem esis, hy p o v o lem ic sho ck, and d ied the fo llo w ing day. A t au to p sy num ero us v esicles w ere o b serv ed o n the surface and in sectio ns o f the p arenchy m a and the p o rta hep atis o f the liv er (Fig ure 4). The p arasite w as aliv e in so m e areas and d ead in o thers. The
F i g u r e 4 - Ech in o co ccu s v o g eli s h o w n i n a l i v e r s e c t i o n o f c a s e 1 . T h e p o ly c y s t ic a p p e a r a n c e o f t h e l e s i o n c a n b e c l e a rl y s e e n a s w e l l a s t h e i n v a s i o n o f t h e p a r e n c h y m a b y t h e m e t a c e s t o d e . B a r = 2 0 c m .
lam inated m em b rane w as thic k and the g erm inal thin, sho w ing few calc areo u s co rp u scles. Bro o d cap su les and p ro to sco lices w ith ty p ical Ev ho o klets w ere freq uently seen. (Fig ure 5 and 6). Betw een the ho st fibro us t i s s u e r e a c t i o n a n d th e p a r a s i t e , an accum ulatio n o f granular m aterial rep resenting d ead eo sino p hils w as freq u ently seen. In sev eral areas p ro liferatio n o f the lam inated m em b rane w as ev id ent,at tim es hav ing a cerebro id ap p earance. In so m e p ro to esco lices sev eral abno rm al lo o king ho o klets w ere seen (Fig ure 7).
C a s e 2 . (OA F, Brasilia Univ ersity, BU-
29023) Year 1976. A 45 y ear-o ld fem ale fro m Curralinho , rural area o f Para State, w ith no c lin ic al histo ry av ailab le. A liv er b io p sy c o n tain ed a p o ly c y stic hy d atid w ith a lam inated m em brane sho w ing a cereb ro id ap p earanc e (Fig ure 8). Th e p arasite w as
F i g u r e 5 - T is s u e s e c t i o n ( H & E ) o / 'Ech in o co ccu s vogeli o f c a s e 1 . T h i c k l a m i n a t e d m e m b r a n e ( a r r o w h e a d ) , t h in g e r m i n a l m e t n b r a n e ( m e d i u m l e n g t h a rr o w ) , g r a n u l a r m a t e ri a l b e t w e e n t h e l a m i n a t e d m e m b r a n e a n a t h e l i v e r ( l o n g l e n g t h a rro w ) , b r o o d c a p s u l e s a n d p ro t o s c o l i c e s . B a r = 2 0 0 p i m .
F i g u r e 6 - U n s t a i n e d s q u a s h p r e p a r a t i o n o f f i x e d p ro t o s c o l i c e s o f c a s e 1 , s h o w i n g l a r g e a n d s m a l l h o o k l e t s
o f E. v o g eli. B a r - 2 0 } i m .
m o stly d ead and surro und ed b y histio cy tes arrang ed in a p alisad e fashio n. Pro to sco lices and ho o klets co nfo rm ed to tho se o f Ev.
C as e 3- ( DM B, BU- 28000). Year 1976. A 67
y ear-o ld m ale fro m M acap a, A m ap a State. Tum o rs o f the liv er and o f co lo n w ere excised after 7 y ears o f illness. The m etacesto d e w as d ead b u t it sho w ed Ev h o o k le ts and p ro liferating , cerebro id lam inated m em brane.
C as e 4 . ( MSS, BU -13539). Year 1971. A 51
D A le s s an d r o A , M o r ae s M A P, R aic k A N . P o ly c y st ic h y d at id d is e as e in B r az il. R e p o rt o f f i v e n e w h u m an c as e s a n d a s h o r t r e v ie w o f o t h e r p u b li s h e d o bser v at io n s. R ev ista d a S o c ie d ad e B r as ile ir a d e M e d ic in a T r o p ic al 2 9 :2 1 9 - 2 2 8 , m ai- ju n , 1 9 9 6 .
F i g u r e 7 - U n s t a i n e d s q u a s h p r e p a r a t i o n o f f i x e d p ro t o s c o l i c e s o f c a s e 1 , s h o w i n g n o r m a l a n d a b n o r m a l
b o o k le t s ( a r ro w s ) o f E. v o g eli. B a r = 2 0 u m .
F i g u r e 8 - T is s u e s e c t i o n ( H & E ) . C o n v o l u t e d l a m i n a t e d m e m b r a n e w it h in t h e l u m e n o f a c y s t ( c a s e 3 ) g i v e n t h e a p p e a r e n c e o f a c e r e b r o i d f o r m a t i o n . B a r = 2 0 u m .
p o ly cy stic lesio ns fro m the liv er and o m entum w ere rem o v ed . This is the o nly info rm atio n that w as o btained .
C as e 5 . (BSR, stud ied b y Mario M o raes in
1987). A 56 y ear-o ld agricultural w o rker fro m Parag o m inas, Para State. In 1980 he had b e e n to ld that he w as infected w ith hyd atid d isease. The p atient d ied in 1987 and an auto p sy co uld no t b e p erfo rm ed . H o w ev er, sp ecim ens av ailable fro m p rev io us b io p sies sho w ed he w as infected w ith p o ly cy stic hyd atid s w ith a lam inated m em b rane sho w ing a cerebro id ap p earance. Pro to sco lices and Ev ho o klets w ere seen. This p atient had b ro ug ht w ith him
an Ev -infected p ac a to d em o nstrate the p arasitic lesio ns o f the anim al.
In summary, fiv e new cases o f PHD are rep o rted . In 4 the charac teristic s o f the m etacesto d e and, in particular, the p ro to sco lices’ ho o klets ind icate that the infectio n w as d ue to Ev. The first p atient d ied o f co m p licatio ns after surg ery carried o ut to allo w the bile to drain. Little clinical info rm atio n w as av ailable fro m the o ther 4 cases ex c ep t that the illness that o rig inated the surg ery to m ake a d iag no sis d em o nstrated that it w as a PH D and that the p arasite inv o lv ed in at least 3 c ases w as Ev d ue to the m o rp ho lo g y o f the ho o klets. W hether the cereb ro id ap p earance o f the lam inated m em b rane o f the 4th case is sufficient to ind icate that it is d ue to Ev is a p o int that sho uld b e co nfirm ed b y the stud y o f m o re E. o lig ar t h r u s in hum ans.
A sum m ary o f the data o f the new and p rev io usly rep o rted hum an cases in Braz il is p resented in Tab le 1.
D ISCU SSIO N
A s stated earlier, it is und erstand able that PHD w as no t d iag no sed until recently . Ev w as d escribed in 197226 in the b ush d o g , b ut the m etacesto d e w as no t reco g niz ed until the o bserv atio ns m ad e w ith C o lo m bian m aterial7 29. W ith the d ifferential criteria established , it b ec am e p o ssib le to reco g niz e that Eo w as the o rig in o f an Echino co ccu s heart infectio n fo u nd o u tsid e the en d em ic area o f E. g r a n u lo s u s in Brazil4 6. A lso that c ases 2 and 3 in the p resent rep o rt, reco g niz ed in 1976 as p o ly cy stic and therefo re n o t d ue to E.
g r an u lo s u s , w ere no t p u blished b y M o raes up
until no w , in the p resent rep o rt. They w ere b o th Ev. Rep o rts o f all Braz ilian c ases, includ ing tho se p resented in co ng resses (as new , o v erlo o ked , o r p rev io usly unrep o rted ), are sum m ariz ed in Tab le 1.
So far, in Brazil, there hav e b e e n 20 PHD cases rep o rted , 9 m ales and 11 fem ales; the m ean ag e w as 41 w ith a rang e o f 12 to 76 y ears. O nly 7 w ere y o u ng er than 30 . Three cases w ere asy m p to m atic indiv id uals; 2 w ith g ro up s o f ro und ed calcificatio ns in the liver and lung (co nsid ered p ro bab ly d ead and calcified m etacesto d es o f PH D ) and the p atient w ith E. o lig ar t h r u s in the heart, w ho d ied o f tetanus and at auto p sy sho w ed this p arasite as an accid ental finding.
D ’A le s s an d r o A , M o r ae s M A P, R aic k A N . P o ly c y st ic h y d at id d is e as e in B r az il. R ep o rt o f f i v e n e w h u m an c as e s a n d a s h o r t r e v ie w o f o t h e r p u b li s h e d o bs e r v at io n s . R ev ist a d a S o c ie d ad e B r as ile ir a d e M e d ic in a T r o p ic al 2 9 .2 1 9 - 2 2 8 , m ai- ju n , 1 9 9 6 .
The lo catio n o f the p arasites w as: liv er alo ne (9 cases), liv er and lung (5 cases), base o f the m esentery (1 case) and cysts in the liv er and o ther o rg ans in the last 4. Fiv e o f these 20 cases d ied as a c o n seq u en c e o f the PHD w hich had cau sed liv er cirrho sis, o bstructiv e jaund ice, p o rtal hy p ertensio n and o r eso p hag eal v arices. O f the 20 cases, 11 w ere d efinitely d ue to Ev and o n e to E. o lig ar t h r u s b ec au se the ty p ical characteristics o f the ho o klets; in 7, ho w ev er, ho o klets w ere no t se en and in 2 there w ere no p ath o lo g ic al sp ecim ens av ailable fo r m icro sco p ic exam inatio n (o nly rad io lo gical). A ltho ugh the typical characteristics o f the E. o lig ar t h r u s m etacesto d e is w ell estab lished , o nly o n e case o f a d ead E. o l i g a r t h r u s in a m a n ’ s h e a r t h a s b e e n d escribed sho w ing no t o nly ho o klets but its cy stic ap p earance, no t d ifferent fro m that sho w n in anim als.
It is c lear that Ev is fo u nd m uch m o re freq u ently in hum ans than is E. o lig at t h r u s . Th e fo rm er is transm itted fro m the d o m estic d o g , w hich is a g o o d ho st fo r Ev but no t fo r E. o lig arthrus; so far as has b een o b serv ed , the latter d ev elo p s to sexu al m aturity and p ro d uces eg g s o nly in w ild cats (Felid ae) o f v ario us sp ecies8.
F o r tu n a te ly , m o s t o f th e B r a z ilia n inv estig ato rs hav e b een aw are o f the stud ies o f PH D , and hav e reco g niz ed its p resence in Brazil. In 1992 it w as fo u nd an end em ic area o f hum an transm issio n in A cre State22. The p a tie n ts w e re th o r o u g h ly s tu d ie d an d lo ng itud inal o bserv atio ns m ad e p o ssib le the ev aluatio n o f beneficial resp o nse to albend az o le treatm ent in 4 o f 6 p atients20. O n the o ther hand , 1 y ear o f m eb end az o le treatm ent w as inefficient to av o id the d eath o f o n e p atient w ho had lo ng lasting lesio ns in the liv er w ith o b structio n o f the biliary sy stem and p o rtal hy p ertensio n11. The sam e failure w as o b serv ed in tw o o f the M eneg helli p atients treated w ith alb end az o le20.
Su rg ical treatm ent w as effec tiv e in rem o v ing the m etacesto d e fro m the b ase o f the m esenterium and the p atient w as rep o rted cured (c ase 4 o f M eneg helli)21. In 5 o f the 20 Braz ilian PHD cases, their fate w as unrep o rted . In o u r ex p erien c e and in the study o f the results rep o rted b y o thers, surgical treatm ent sho u ld b e rec o m m end ed w h en the m etacesto d e is sm all, lo caliz ed and has no t resp o nd ed to m ed ical treatm ent. Rad ical cures
m ay b e exp ected in these cases, b u t p ro bably no t w hen the PHD inv o lv es a larg e p o rtio n o f the liv er o r o ther o rgans. H o w ev er, m o re o bserv atio ns fo r p ro lo ng ed p erio d s o f tim e are necessary to assess the chances o f p erm anent im p ro v em ent o r cure in eac h case.
W hen the biliary sy stem is inv o lv ed , o r cirrho sis has d ev elo p ed w ith sp leno m eg aly and eso p hag eal v arices, surg ery has accelerated the d eath o f the p atient d ue to co m p licatio ns. H o w ev er, if the itching d ue to the jau nd ice is v ery tro u bleso m e, d rainag e o f the biliary tract is usually required .
Surgery has b een u sed su ccessfu lly as a p alliativ e m ean o f d im inishing the effects o f large num bers o f cysts, w hich interfere w ith no rm al p hy sio lo g ical activ ity o f the sto m ach and o ther o rgans, b y rem o v ing as m any cysts fro m the m esenteries o r tho se em erg ing fro m o rg ans as p o ssib le. In ad d itio n, the ab senc e o f intrap erito neal m asses allo w s the p atient to s le e p m o re c o n f o r ta b ly ( D ’A le s s a n d r o , um p ublished ).
In sum m ary , m ed ic al treatm ent w ith alb end az o le has b een u sed w ith g o o d results altho ug h really it is to o early to assess lo ng lasting cures. H o w ev er, alb end az o le sho uld b e used and ev aluated o n the p atient’s w ell being . In g eneral, Ev seem s to b e less ag g ressiv e than
E. m u lt ilo c u lar is and w e kno w o f asy m p to m atic
p atients w ho had the infectio n fo r sev eral d ecad es.
The ep id em io lo g ical info rm atio n o btained fro m the Braz ilian cases is v alu able. The A cre p atients no t o nly w ere fam iliar w ith p acas but also had se e n p arasitic v esic les in their abd o m en. A s in Co lo m bia, the v iscera o f the p acas are fed to d o g s. The co nd itio ns in A cre, at least, seem fav o rab le fo r stu d ies o f transm issio n o f Ev d ue to the nu m b er o f infected p erso ns rep o rted and u nrep o rted 20. In this p lace it has no t b e e n estab lished ho w the p acas g et the infectio n, b ut A cre is w ithin the g eo g rap hical d istributio n o f the b u sh d o g. In 19869 it w as rep o rted 13 sp ecific p laces w ere the bush d o g had b een sen in Brasil includ ing the states o f M inas G erais, M ato G ro sso , Santa Catalina and São Paulo , and these p laces w ere sho w n in a m ap (Fig ure 9). A lso u nkno w n is the freq u en c y o f in fe c tio n in p ac as, in d o m estic d o g s and in hum ans (w hich co u ld b e a s s e s s e d b y u s in g th e l a te s t a v a ila b le sero lo g ical tests).
D ’A le s s a n d ro A , M o ra e s M A P , R a ic k A N . P o ly cy s t ic h y d a t id d is e a s e in B ra z il. R e p o rt o f f i v e n e w h u m a n c a s e s a n d a s h o rt re v iew o f o t h e r p u b l i s h e d o bset v at io t is . R ev is t a d a S o c ie d a d e B ra s ile ira d e M e d i c in a T ro p ica l 2 9 : 2 1 9 - 2 2 8 , m a i - j u n , 1 9 9 6 .
In C o lo m bia, p eo p le fro m the enz o o tic area co u ld no t p ro v id e info rm atio n o n the p resence o f the b u sh d o g until a p ho to o f the anim al w as p resented . Then m o st p eo p le reco g niz ed the anim al. The lo cal nam e in the Co lo m bian O riental p lains is “z o rro g u ache” and in Brazil the lo cal nam e is “cacho rro d o m ato v inag re”3.
A surv ey o f a lim ited rand o m sam p le o f ad ult p acas (w eig hing m o re than 10kg ) sho uld b e su fficient to assess the p rev alence o f Ev infectio n, co nsid ering that the freq u ency in C o lo m b ia w as 30% in ad ult anim als, 16% in subad ults and n o ne in the ju v eniles w eig hing less than 5.5kg 8.
A ltho ugh Ev infects hum ans and anim als m o re freq u en tly than E. o li g a rt h ru s the g eo g rap hical d istributio n o f w ild cats is w id er than that o f the b u sh d o g 8. Therefo re, the p resence o f PHD o utsid e the rang e o f the b ush d o g m ay b e d ue to E. o liga rt hrus . H o w ev er, it has b e e n p o stulated that anim als o ther than the b u sh d o g and p acas m ay b e inv o lv ed in the transm issio n o f Ev6.
A ctually, b o th p assibilities m ay p lay a ro le. To answ er this q u estio n the co llab o ratio n o f
P *
z o o s is req u ired b y th e treatm ent w ith p raz iquantel o f canid s/ felid s that hav e b een cap ture in the w ild as w ell as tho se w h ic h has b e e n in captiv ity , in p articular fo r a sho rt tim e. Lo ng lasting cap tiv ity m ay hav e an effec t o n s o m e p a r a s ite s w h ic h are e lim in a te d sp o ntaneo usly .
It is im p o rtant to d eterm ine the sp ecies inv o lv ed in a case o f PHD b ecau se, ap p arently, Ev is su scep tib le to m ed ical treatm ent w ith alb end az o le altho ug h, as stated earlier, lo ng term o b serv atio ns o f rep o rted su c c esse s require p ro lo ng ed p erio d s o f o b serv atio n. A num b er o f rep o rts are enco u rag ing , at least in regard to the im p ro v em ent o f the p atient’s health.
RESUM O
E s t e t ra b a lh o d e s c re v e c i n c o n o v o s c a s o s h u m a n o s b ra s ile iro s d e d o e n ç a h id ã t ic a p o lic ís t ica , c a u s a d a p o r Ech in o co ccu s vogeli e f a z u m a re v is ã o d e c a s o s a n t e rio re s re la t a d o s n o B ras il, i n c lu in d o u m re la t o s o b re E. oligarthrus ( n u m t o t al d e 2 0 ) e a lg u n s a s p ect o s e p id e m io ló gico s d e s t a d o e n ç a , q u e j ã n ã o é m a is u m a c u ri o s id a d e m as , s e m d ú v id a ,
D ’A le s s an d r o A , M o r ae s M A P, R aic kA N . P o ly c y st ic h y d at id d is e as e in B r az il. R e p o r t o f fiv e n e w h u m an c as e s a n d a s h o r t r e v ie w o f o t h e r p u b li s h e d o bs e r v at io n s . R ev ist a d a S o c ie d ad e B r as ile ir a d e M e d ic in a T r o p ic al 2 9 :2 1 9 - 2 2 8 , m ai- ju n , 1 9 9 6 .
u m p r o b l e m a d e d ifí c il m an e j o d o p o n t o d e v ista m é d ic o . S u a p r e s e n ç a p o d e o c o r r e r em. t o d as a s ã r e a s r u r ais d o N o v o M u n do , o n d e o h o m e m a i n d a n ã o e le m in o u o s f e l i n o s e c a n i n o s s e lv ag e n s , c a c h o r r o d o m at o , p a c a , c u t ia e o u t ro s r o e d o r e s selv ag en s.
P alav r as - c h av e s : D o e n ç a h id ã t i c a p o lic ís t ic a. Echinococcus vogeli. B ras il.
A C K N O W LED G M EN TS
W e thank Dr. Ro bert I Rausch (D ep artm ent o f C o m p arativ e M ed icine, Univ ersity o f W ashingto n, Seattle, WA .) and Dr. M D ale Little (D ep artm ent o f Tro p ical M ed icine, Tulane U niv ersity Sc h o o l o f Pu b lic H ealth and Tro p ical M ed icine, N ew O rleans, LA.) fo r rev iew ing the m anuscrip t and o ffering useful sug g estio ns.
REFEREN C ES
1. A munarriz M. Estúdios sobre patalogias tropicales en la A mazonia Ecuatoriana. V icariato A postolico de A guarico, Ed. CICAME, Pompeya, N apo, Region A mazônica Ecuatoriana. 1991
2. Brum pt E, Jo yeux Ch. D ecription d’un nouvel echinocoque: E c h in o c o c c u s c r u z i n.sp. A nnales de Parasitologie Humaine et C om paree 2:226-2 3 1 ,1 9 2:226-2 4 .
3. Cabrera A, Yepes J. M amíferos Sul A mericanos. Ediar editors. 2nd. edition, Buenos A ires, I960.
4. Chapadeiro E, Lopes ER, M esquita PM . Prymary hydatid cyst of the heart. Journal o f Tropical M edicine 6 8 :9 - 1 1 ,1 9 6 5 . Portugese version published in Revista do Instituto de M edicine Tropical de São Paulo 6:1 7 1 - 1 7 3 ,1 9 5 4 .
5. D ’A lessandro A, H enao H, Cuello C. Un caso colom biano autoctono de hidatidosis poliquistica multiple de higado, pericárdio, pulmones, pleura y corazon. A cta M edica del Valle 9 :28- 35,1978.
6. D ’A lessandro A, Ramirez LE, Chapadeiro E, Lopes E, M esquita PM Second recorded case of human inf ection by E c h in o c o c c u s o lig ar t h r u s . The A m erican Journal o f Tropical M edicine and Hygiene 5 2 :2 9 - 33,1995.
7. D ’A lessandro A , Rausch RL, Cuello C, A ristizabal N. E c h in o c o c c u s v o g e li in man, w ith a review of polycystic hydatid disease in Colom bia and neighboring countries. The A merican Journal of Tropical M edicine and Hygiene 28; 303- 317, 1979.
8. D ’A lessandro A, Rausch RL, M orales GA, Collet S, A ngel D. E c h in o c o c c u s infections in Colombian
anim als. The A m erican Jo urnal o f Tropical M edicine and Hygiene 3 0 :1 2 6 3 - 1 2 7 6 ,1 9 8 1 .
9 . D ru w a P. P e r r o d e g r u l l e i r o , d e r Sudamerikanische W adhund-ein Rätsel fur die H undeforschung. In Z eitschrift des Kölner Z oo 2 5:71- 90,1982.
10. Ferreira MS, N ishioka SA, Rocha A, D ’A lessandro A. E c h in o c o c c u s v o g e li polycystic hydatid: rep ort of tw o Brazilian cases outside the A mazon region. Transactions o f the Royal Society o f Tropical M edicine and Hygiene 89:286- 287,1995.
11. Ferreira MS, Rocha A, G onçalves EG, Carvalho AM, N ishioka SA, A ndrade NB. Um caso de hidatidose policística autóctona de M inas G erais, Brasil. Revista da Sociedade Brasileira M edicina Tropical 20:181- 186,1987.
1 2 . G ard n e r SL, R au s ch RL, C am ach o O C J. E c h in o c o c c u s v o g e li Rausch and Bernstein, 1972, from the paca, C u n ic u lu s p a c a L. (Rodentia: D asyproctidae), in the D epartam ento de Santa Cruz, Bolivia. Journal Parasitology 74:399- 402, 1988.
1 3 .G o tts te i n B, D ’ A l e s s an d ro A , R au s ch RL. Im m u n o d iag n o s is o f p o l y cy s ti c h y d atid d is ease/ p o l y cy stic e ch in o co cco s is d ue to E c h in o c o c c u s v og eli. The A m erican Journal of Tropical M edicine and Hygiene 5 3 :558- 563,1995.
14. G uderian R, Kerrigan KR, C hico ME, G uevara A, Hidalgo G. Hidatidosis humana en el oriente ecuatoriano: informe de los prim eros dos casos. Revista M edica V ozandes 4:5 7 - 6 0 ,1 9 9 0 .
15. H oward EB, G endron AP. E c h in o c o c c u s v o g e li infection in higher primates at the Los A ngeles Z oo. In : M ontai RJ, Migaki G (eds)The com parative pathology of zoo animals. The symposia o f the N ational Z oological Park. Smithonian Society. W ashington, D C. Smithonian Institution Press 379- 382,1980.
16. Lopera RD, M elendez RD, Fernandez I, Sirit J, Perera MP. O rbital hydatid cyst o f E c h in o c o c c u s o lig ar t hr u s in a human in V enezuela. Journal Parasitology 75:4 6 7 - 4 7 0 ,1 9 8 9 .
1 7 .M e l e n d e z RD , Y e p e z M S, C o ro n ad o A . E c h in o c o c c u s o lig ar t h r u s cysts of rabbits in V enezuela. Journal Parasitology 70:1004- 1005, 1984.
18. M eneghelli UG. C alcificações hepáticas múltiplas d eco rrentes de d oença hidática policística. Revista G oiana de M edicina 31: 5 3 - 6 0 ,1 9 8 5 .
D ’A le s s an d r o A , M o r ae s M A P, R aic k A N . P o ly c y st ic h y d at id d is e as e in B r az il. Report- o f fiv e n e w h u m an c as e s a n d a s ho r t r e v ie w o f o t h e r p u b lis h e d o bser v at io n s. R ev ist a d a S o c ie d ad e B r as ile ir a d e M e d ic in a T r o p ic al 2 9 :2 1 9 - 2 2 8 , m ai- ju n , 1 9 9 6 .
manifestations and treatm ent w ith albendazole of a patient from the Brazilian A mazon region. A rquivos de G astroenterologia de São Paulo 2 3 :1 7 7 - 1 8 3 ,1 9 8 6 .
20. M eneghelli UG, M artinelli A LC, Bellucci AD, V illanova M G . Po ly cy stic hyd atid d isease ( E c h i n o c o c c u s v o g e l i) . T re a tm e n t w ith albendazole. A nnals o f Tropical M edicine and Parasitology 8 6 :1 5 2 - 1 5 6 ,1 9 9 2 .
21. M eneghelli UG, M artinelli ALC, Llorach Velludo MAS. Cisto de E c h in o c o c c u s v o g e li em fígado de paca (C u n ic u lu s p a c a ) originária do Estado do A cre, Brasil. Revista da Sociedade Brasileira M edicina Tropical 2 2 :1 5 3 - 155,1990.
22. M eneghelli UG, M artinelli ALC, Llorach V elluto MAS, Bellucci LE, Barbo MLP. Polycystic hydatid disease (E c h in o c o c c u s v og eli) . Clinical, laboratory and m orphological findings in nine Brazilian p atients. Jo urnal o f H ep atology 14:203- 210,
1992.
23- O ’G rady JP, Yaeger CH, Esra GN, Thomas W. Ultrasonic evaluation of echinococcosis in four lowland gorillas. Journal o f A merican V eterinary M edical A ssociation 181:1348- 1350,1982.
24. Pacheco M DK, Leite Pinto RN, Silva Souza LC, A raujo Pereira LI. D oença hidática policístiea. Relato de um caso p roced ente do Pará. Revista da S o cied ad e Brasileira de M ed icina T ro p ical 19(sup p l):67,1986.
25. Passos DMM, Pinho A lves M, Creuv O, Freitas G, M atushita JP, M archiori E. Echinococose alveolar
hepática. (D escrição de um caso). Radiologia Brasileira 15:129- 132,1982.
26..Rausch RL, Bernstein JJ. E c h in o c o c c u s v o g e li sp.n. (Cestoda:Taeniidae) fron the bush dog, S p eo t ho s v e n at ic u s (Lund) Z eitschrift fur Tropenmedizin und Parasitologie 23:25- 34,1972.
27. Rausch RL, D ’A lessandro A, O hbayashi M. The taxonom ic status o f E c h in o c o c c u s c r u z i Brumpt and Joyeux (Cestoda:Taeniidae ) from an agouti (Rodentia: D asyproctidae) in Brazil. Journal of Parasitology 7 0 :2 9 5 - 302,1984.
2 8 . R au s ch RL, D ’ A l e s s an d ro A , R au s ch V R. Characteristics of the larval E c h in o c o c c u s v o g e li Rausch and Bernstein 1972, in the natural intermediate host, the paca, C u n ic u lu s p a c a L. (Rodentia: D asyproctidae) The A m erican Journal Tropical M edicine and Hygiene 30:1043- 1052,
1981.
2 9 . R au s ch RL, R au s ch V R, D ’ A l e s s an d ro A . D is cri m i n a ti o n o f th e l arv al s tag e s o f E c h in o c o c c u s o lig ar t hr u s (D iesing, 1863) and E. v o g e li Rausch and Bernstein, 1972 (Cestoda: Taeniidae). The A m erican Journal o f Tropical M edicine and Hygiene 2 7 :1 1 9 5 - 1 2 0 2 ,1 9 7 8 .
30. Timerman A, A ndrade DR, H utzler RU, M arinho IS, Uliana SRB.Terapêutica da hidatidose hepática co m albendazole: relato de 1 caso. In : Resumos do IX Congresso Brasileiro de H epatologia São Paulo, abstract 8 5 ,1 9 8 6 .
