A Long-Term Follow-Up Study With Special Reference to Renal Involvement

7/29/2019 A Long-Term Follow-Up Study With Special Reference to Renal Involvement

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AN APHYLACTO ID PURPURA (SCH bN LE IN -HENOCHSYNDROME )

A Long -te rm Fo llow -up S tudy w ith Spec ia l R e fe renceto R ena l Invo lvem en t

By Ra lph J. P . W ed gw oo d, M.D. , * an d Marsha l l H . Klaus , M .D .NA I I I YLACTOI D purpura is a genera l ized

d isease charac te rized by a sp ec ificsk in lesion , usua lly accom pan ied b y jo in tsy n l1 )ton )S and often by gas tro in tes tina liiianifestatioiis. The spec ific sk in lesion , ex -te iis ive lv (Iesc ribe (l b y de cons istso f a rog re55 iO f l from urtica ria, th roug h al)lanch ing p ink or red rn acu lo papu la r e ru p-tion to th e classic pu rpuric les io n fromwh ich the d isease has derived its nam e.H is to lo g ically the sk in lesion is tha t o f anacute in flan irna tory exud ate aroun d thesm all v esse ls o f the co rium ra th er than ac-tua l he liio rrhage . F or th is reaso n G airdner1pre fe rs the te rm S chon le in -H enoch Syn-droiiie.

T he na iiie s o f S ch#{246}n le in a fl(l Henoch a rea ttached to the d isease l)ecause of the irea rly , classic an d com ple te d esc rip tion ofits iiianv m anifesta tio ns.2 The d iseaseSCC II15 to have IWC I1 1 1)rS lttl cha lleng eto O sle r w ho w ro te ex ten sive ly on th ev isce ra l m an ifesta tions. C er ta in ly these an-tho rs reco g iiized th at the sk in les ions a rel)tIt a m ino r (a lth ough a t tim es the on ly ) ob -se rvab le manifesta t ion of a genera lized d is -ease . A s gastro in tes tina l and jo in t m an i-fes ta tions a re 1 )0 th frequen tly p resen t, itseem s of little va lue to separa te the d iseasein to 2 svn(lrO IT IeS m ere ly becau se of thePrec l011 i i f l 11 it inv o lvem en t o f one sy stem orano ther .

From th e 1)epa r tments of P ed ia tric s an d P a th o l-og v, \V es tc rn Rese rve Univers i ty , and the B ab iesand Child ren s ho sp ita l, U n iv ersity H osp ita ls,Cleveland, Oh io .

Th is w ork w as in part supp orted by resea rchgrant 1 l-164 3 from the National Ins titu tes o fhealth , U . S . Pub lic Ilealth S erv ice .

(Subm itted A ug . 26, 195 4; rev ision acceptedApri l 1 :3 , 1955 .)O ADDRESS: 2065 Adclhcrt Road, C lev e land 6 ,Ohio .

The e tio lo gy of th e d is ea se is un know n .H ow eve r , because o f the w id e -sp readvascu lar lesions , and becau se of the frequ en toccurrence o f p reced ing in fec tio n , o f tenw ith be ta h em oly tic strep tococc i, it ha sbeen su ggested tha t the d isease rep resen tsa h ypersen sitiv ity sta te and b elon gs inth e group of so -ca lled co llag en d iseases .

T he pu rp ose o f th is pape r is to de term in eth e frequen cy and na tu re o f seq ue lae to th eacu te ph ase o f the d isease.

P AT IE NT M AT ER IA LBe tween 1939 and 1953 , 36 ch ild ren w ith

anaphv lac to id purpu ra w ere adm itted to B ab iesand Child ren s H osp ita l. Tw en ty -s ix ch ild renw ho cou ld be located in 1954 w ere a live andcons id ered w ell. T he age at on se t o f svm p-tom s rang ed from 13 m on th s to 1 23 y ears andusua lly w as b etw een 2 and 4 years (F ig . 1 ).S even teen of th e pa tien ts w ere m ale and 9w ere fem ale. T h is sex ra tio o f approx im ately2 : 1 is s im ila r to the ra tio repo rted b y o th er ob-se rvers.1 A ltho ugh 20 p er cen t o f the pa tien tsadm itted to th e hosp ita l du rin g th ese years

8


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10 15NUMBER OF CH ILDREN

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OR IG INAL ART ICLES 19 7w ere N eg ro , no N egroes w ere fo und am ong the3 6 p atien ts w ith anaphy lac to id purpura , a d is -c repancy w hich m ay ref lec t the d ifficu lty inrecogn iz ing purpu ra o n th e neg ro id sk in ra th e rthan an ac tua l d iffe rence in the a ttack rate .In a ll o the r resp ects the ch ild ren w ere de-r iv ed from a w ide ly varied rac ial background .

N o o th er m em bers o f the imm ed iate fam ilie so f the ch ild ren in the se ries have been know nto have h ad an aphy lac to id purpu ra or sym p-tom s sugges tiv e of it; n o r w as there , in these

PURPURA0%

a lle rgen ic facto rs as spec ific foo ds have beendesc r ibed iii the lite ra tu re #{ 176} as causativeagen ts .

A s m igh t b e suspec ted from th e defin ition ofthe d isease , all o f the ch ild ren h ad purpu ra(F ig . 2 ). T he rash in vo lved the low er ex trem i-tie s in a ll p atien ts; ha lf a lso had invo lvem en to f the arm s and one-th ird had lesions on thetru nk . T he sk in les io ns con sis ted no t o n ly ofpurpu ra , b u t a lso ur tica ria , e ry them a, edem a,an d sm all red p in po in t m acu les o r p ap u les .

50 % 100%

ARTHRALG IAABDOMINAL

PAIN

FEVERPROTE INUR IA

VOMIT INGM ICROSCOP ICHEMATUR IA

BLOOD INSTOOL *

famil ie s , an y su ggestio n of an u nusual in -c iden ce o f any of the so -ca lled co llagen d is-eases .

T h irteen of the tw en ty -six ch ild ren had ah isto ry of u pper resp ira to ry in fec tion prio r too r occas iona lly concom itan t w ith the onse t o fpurpu ra . O f 9 ch ild ren in w hom throa t cu ltu reswere ob tain ed , 5 harbo red be ta h em o ly ticstrep tococc i. T h is is in agreem en t w ith o th erpub lish ed reports . In a thorou gh search tofind an alle rg ic d ia thesis in the ch ild ren , 4 g avea fam ily h isto ry of a lle rgy bu t w ere w ithou ta lle rg ic m an ifesta tio ns them selves , and ano ther4 by h isto ry had had m inor m an ifes tation s sug-g es tive of alle rgy (h iv es , d ru g erup tions , andI)0S 5 i11e asthm a). Iii iio ne of the ch ild ren co u ldany spec ific a lle rgen be im plica ted as th e pre -c ip itating fac to r in th e d isease . H ow ever, such

S ix teen of the tw en ty -s ix had m odera te sw ell-ing of one or m ore jo in ts, usua lly the knees,ank les, o r w ris ts . A ltho ugh there w as usua llysom e lim ita tio n of m otio n o f these jo in ts be-cause of m ild to m od erate pa in , the in vo lve-m en t genera lly w as n o t severe ; hea t, red nessand exqu is ite tend erness w ere usu ally absen t.

Tw elv e o f the tw en ty -s ix ch ild ren had sym p-tom s dur in g the acu te p hase re fe rab le to thegastro in tes tina l tract, 1 1 had abdom ina l pa in ,and 7 had ep isodes o f em esis . T ests fo r occu ltb lood in the stoo l w ere pos itive in 3 o f 7 ex -am ined . Tw o o f th ese ch ild ren had grossm elena . O ne of these (C ase 19) w as o pera tedupon fo r in tussuscep tion and a sec tion ofg an grenou s bow el rem ov ed . T he o ther (C ase17) had an exp lo ra to ry lap aro tom y for poss ib lein tes tina l ob struc tion just 1 )rio r to the onse t o f


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19 8 WEDGWOOD - ANA PHY LACTO ID PU RPURA

FIG . 5 . 1 )uratio n of in itia l p hase of the d isea se .

FIG. :3. W BC (luring acute phase.

ptlrp(lra. A t opera tion hyp erem ic an d edem atousbow el w as seen .

A t the tim e o f th e in itia l adm is sion to thehosp ita l 9 o f th e ch ild ren w ere feb rile . F ourhad hypertension (C ases 8 , 1 7 , 19 , 2 4), w h ichill 1 ch ild (C ase 1 9) w as assoc ia ted w ith con- u 1sio ns and respo nded to treatm en t w ithm agnesium su lp ha te. P hys ica l ex am in ation ,ap ar t from ev iden ce of u pper resp ira to ry in fec -tio ns, sk in les io ns, and jo in t invo lvem en t, w asno t rem arkab le . L ym phadenopa thv w as nom ore comm on than is seen in the gene ra l ped i-a tric pop u la tion . T he sp leen w as pa lpab le inon ly 2 ch ild ren . N o ev id en ce of heart d iseasew as found .

A ll o f the ch ild ren , excep t fo r 3 w ho w erem ild ly anem ic , w ere found on laborato ry ex-am ina tio n to h av e norm al red b lo od ce ll cou n ts,hem og lo b in , and hem atocrits . T he wh ite b loodcoun ts (F ig . 3 ) varied from 5800 to 22 ,00 0 /mm .. E leva ted w hite cou n ts cou ld n o t all bea ttribu ted to ex is tin g in fection . E osino ph ilia (4to 10 per cen t on d iffe ren tia l) w as found in 5p atien ts. T he sed im en ta tio n ra te (co rrec ted\V in trob e) w as eleva ted (above 15 mm ./h r.) in12 ch ild ren , borderline (10 to 15 mm .,/h r.) in 4and no rm al (be low 10 mm . h r.) in 4 of 20 ch il-d ren on whom the exam in a tion w as perfo rm ed .E lev ated sed im en ta tion ra tes w ere no t co r-re la ted w ith th e ex is ten ce of in fect ion . Atou rn ique t tes t p er fo rm ed in 12 ch ild ren w asp ositive in o n ly 3 . S tud ies o f th e b leed in g andc lo tting m echan ism s w ere genera lly w ith in

123456M INUTESFic. 4. B leed in g tim e during acu te phase .

n orm al lim its . B leed ing tim es (F ig . 4 ) w ereabn orm al in o n ly 5 of the 21 pa tien ts tested .C lo t retractio n w as no rm al in 12 ch ild ren ex -am in ed and p la te le t coun ts w ere w ith in n or-m al lim its in 1 8 ch ild ren o n w hom th is ex am -in a tion w as perfo rm ed . R ou tine ur in alyses re -vea led p ro tein u ria and m ic rosco p ic hem a tu riain 6 ch ild ren , inc lud ing the 4 w ith hy perten -s io n . P ro te inur ia a lone w as found in 3 o th ers.

T he duratio n of the in itial sym ptom s rangedfrom 6 to 180 day s (F ig . 5 ). U su ally thein itia l m an ife sta tion s o f th e d isease sub sided in4 w eek s. T hose ch ild ren in w hom the illn essran a pro trac ted course gen erally h ad a sev erefo rm o f the d is ea se , ch aracte r ized by rem is-sion s and exacerba tions . D ur in g the acu tephase som e of the ch ild ren rece ived an tib io tic sfo r the ir in fec tions and som e ace ty l sa licy licacid fo r an alg esia . A few of the ch ild ren w ereg iv en an tih is tam in e w ith ou t p rov en e ffec ts ;


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OR IG INA L ART ICLES 19 9none w as g iven cortisone or ad ren ocortico trop ichormone .

M E TH OD SThe 26 ch ild ren w ho cou ld b e lo ca ted w ere

in terv iewed and exam ined by the au th ors . A tth is tim e a fu ll h is to ry w as taken w ith spec ia lem phas is on th e in terva l s ince th e ir acu te d is-ease . C om ple te phys ical exam ina tion w as per-fo rm ed . A 12-hou r co llec tion o f u rine , p rese rvedw ith thym ol or fo rm alin , w as ex am ined bythe Add is techn ic . U rina ry pro te in ex cretionw as m easured w ith T such iyas reagen t in aS hevky-S ta ffo rd tube . U rea n itrogen de ter -m ina tions w ere carried ou t o n b loo d ob ta in ed(lu rin g fas tin g . A bnorm al A ddis coun ts , if yen-fled by su bsequ en t ex am ina tion w ere causefo r adm ission of the ch ild to B ab ies and Chil-d ren s H o sp ita l fo r m ore com p le te rena lstud ies. D uring ho sp ita liza tion A ddis coun tswer e rep eated and m ultip le u rea clea rance an dpheno lsu lfonph tha le in excre tion tes ts w ere pen-fo rm ed . In trav en ous pve logram s and rena l con-cen tra t ion an d d ilu tion s tud ies w ere d one inadd ition to de te rm ina tions of the usu al b lo odchem ica l va lues . C are fu l ob se rva tion w as m adeof the b lood pressu re of the p atien ts w hile a trest.

RESULTSIn th e Il lajority of p atien ts 2 or m ore

years had e lapsed b etw een the o nse t o f d is -ease and the fo llow -up s tud ies (F ig . 6 ). O nech ild h ad b een fo llow ed 15 years , ano th er8 years. O n ly 2 ch ild ren h ad been fo llow edless than 1 year.

A ll bu t 1 of th e ch ild ren h ad been freefro nl sym ptom s sugges tive of anaphy lac to id In 1 ch ild (C ase 9) w ho hadbeen fo llow ed fo r on ly 6 m onths , the re hadbeen severa l recurrences of the rash an d 1ep isod e of g ross hem atun ia .

O ne ch ild had b een fo llow ed in card iacclin ic b ecause of a pro long ed PR in terv a lin the electrocardiogram. The PR intervalhad rem ain ed unchanged fo r 2 years andthe p atien t had show n no o th er ev id en ceof ca rd iac abn orm a lity ; th us, the elec tro -ca rd iog raph ic f ind in g w as consid e red to bea no rm al va rian t. M o st o f the ch ild ren h adexp er ienced the usua l co n tag io us d iseaseso f ch ildhood w ithou t d ifficu lty .

On phy sica l ex am ina tion the he igh t and

w eig h t o f th e ch ild ren fe ll w ith in the nor-m a! ran ges defined by the H arvard S choo lof Pub lic H ea lth g row th curves. B lo od pn es-su re s w ere n orm a l in a ll bu t 2 p atien ts( C ases 2 1 , 2 4 ) . H ow ev e r, a fte r be ing co n-f ined to bed in th e ho sp ita l th e b lo od pres-su res in the se 2 ch ild ren w ere w ith in thenorm a l range . The rem a inde r o f the p hys ica lexam in a tion w as en tirely no rm al in a ll th ech ild ren . O n fu ndu scop ic exam in atio n th e rew as no abnorm ality o f the re tina l vessels .There w as no ev idence of o rgan ic hea rtd isease in an y of the ch ild ren . A lthoughseveral grad e 1 so ft basila r systo lic m u rm u rsw ere h ea rd , the se a ll d im in ish ed w ith exe r-c ise o r re sp ira tion and w ere thu s con side redfunct ional .

B lood u rea n itrogen de te rm ined on 23ch ild ren va ried be tw een 9 an d 18 m g ./1 00m l. w ith a m ean of 1 4 m g ./1 00 m l. As umma ry of u rina ry find ings is sh ow n in F ig -u res 7 , 8 , and 9 . In these f ig u res dup lica teo r m u ltip le d ete rm ina tion s a re rep re sen tedby a m ean v alue . T en of the ch ild ren h adhematur ia w ell over the lim it o f norm al(6 00 ,00 0 rbc /12 12 J 8 the averageof 2 o r m ore Addis cou n ts show ed hem atu riain ex cess o f a m illio n red ce lls p er 12 hours.In 12 ch ild ren a urin a ry ex cre tion of 10 0 ,000or m ore casts pe r 12 h ours w as fou nd . Insom e of these ch ild ren red ce ll casts in ad -d itio n to hy alin e an d g ranu lar ca sts w ereseen . In o n ly 2 ch ild ren (C ases 23 , 24 ) w asan av erage p ro te inu ria in ex cess o f 10 0m g./12 hr. o bse rv ed . T he 1 2-hou r excre tionof w hite ce lls w as w ide ly variab le , pa rticu -la rly w ith regard to sex , an d in te rp re ta tio nof th ese co un ts w a s impossib le.

In gene ra l, a s can be seen in F igu re s 7,8 , and 9 , the ch ild ren fell in to 2 d istinc tg ro ups in re spec t to th eir A dd is cou n ts . S ix -teen o f th e ch ild ren o n fo llow -up h ad urinew h ich can be consid ered w ith in n orm a llimits. Ten of the children (C ases 11 , 14 ,16 , 17 , 18 , 1 9 , 2 1 , 23 , 24 , 25 ) had u rin ewh ich , on the basis o f th e ave rage of re -p ea ted A dd is coun ts , m ust be con side redabno rm al by curren t cn iter ia .1 1 4 T he ab -n orm a lity in vo lv ed th e u rina ry exc retio n ofexcessiv e n um bers o f red ce lls and casts ; in


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fin d ing . O n repea ted A dd is co un ts , w ith theexcep tion of 1 ch ild (C ase 17) w ho hadm ark ed inc rease in h em a tu ria concom i-tan t w ith ph a ryn g itis cau sed b y be tah em o ly tic strep tococc i, the na tu re and de-g ree o f th e ab norm a lity rem a ined rem ark -a bly c onsiste nt.

0 20406080#{174} CASTS /XI000

on ly 3 w as pro teinu ria# {176 } co nsis ten tly in -c reased to a s ig n ifican t deg ree, and ev en inthese (C ases 17 , 23 , 24) it w as no t a strik ing

0 Six ch ild ren (C ases 16 , 17 , 18 , 2:3 , 24 , 26 ) hadincreased p ro te in uria in 1 o r m ore spec im ens onfo llow -up . how ever, in 3 (C ases 16 , 18 , 26) p ro .te inu ria w as no t cons isten tly abn orm al.

20 0 \ V EDGWOOD - ANA PHYLACTO ID PURPURA

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WEDGWOOD - ANAPHY LACTO ID PURPURA02

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F ic . 9 . I o te inuria found a t fo llow -up : E xpressedas m g./12 hr.

T he 10 ch ild ren w ith abn orm al u rine in -dude 6 m ales and 4 fem ales , a sex ra tio o f1 .5 : 1 , a ra tio no t s ign if ican tly d iffe ren t fromth e sex ra tio o f the en tire g roup . Th ese 1 0ch ild ren have been fo llow ed fo r an averageof 4. 3 y ears , s im ila r to the average o f 4 .8years fo r the group w ith norm al u rine .Sev en of the ten h av e been fo llow ed m orethan 3 years from th e on se t o f the in itial d is -ease. T he abnorm a l u rin ary find in gs cann o tb e con side red pa rt o f the acu te phase o f thed isease in any of th e ch ild ren ; the 1 ch ildw ith co n tinu ed purp ura (C ase 9 ) is no t in -e lu ded in th is g roup .Th ere w as a d istinc t co rre la tion be tw eenth e age o f the ch ild at th e o nse t o f th edi sease and the later finding of urinary a b-norm ality (T ab le II). Whi l e 9 of the 13 (69per cen t) over the age of 6 years a t th e tim eo f o nse t o f the d isease had abnorm al u rine

fo llow -up exam ina tio n , on ly 1 o f 13(8 p e r cen t) und er the age of 6 y ea rs w hen

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OR IG INA L ART IC LES 20 3among th e 16 who h ad no exacerb a tion s.

M ore com p le te s tud ies w ere p erfo rm edon the 1 0 ch ild ren w ith abn orm a l u rin es(T ab le IV ). In g ene ra l th ese s tud ie s re-v ealed no strik ing o r un ifo rm im pairm en to f rena l fun ction . F ou r of the ch ild ren h adav erag e urea c learan ces be low 70 per cen to f no rm al an d 1 ch ild fa iled , d u ring p ro -lo ng ed flu id depriv a tion , to co ncen tra te h isu rin e abo ve a spec ific g rav ity o f 1 .015 . In -tnavenous py elog ram s w ere ob ta ined o n8 o f these ch ild ren , and in each case the rew as n o v isib le abnorm ality o f the urina ryt racts .

TABLE IVFoL i ow-uI STUD IES

C ase U rea h igh es t um - Present Length of (earance* U rineb er .lge 1o l lo l1 - uJ) (% ) Sp . G r.j11 10 9/ P1 7 1 1.50 1.03014 8 6/1 6/1 105 1.03816 H 9/ 12 6 t) /I 89 1.03517 14 5/ 1 6 6/1 86 1.01518 1 4/ 1 4 /1 180 1.03619 13 8/1 S 0/U 1 1.03 14 5/ 1 4 11/1 64 1.04023 13 10 /1st I 9/U 56 1.024 13 7/1 6/U 67 1.0405 15 /1 3 0/1 68 1. 040

* Ave r ag e of 4 to 8 m ax im a l clearanc es of 1 ho ureach ..t Fo llow in g f lu id d epr ivat ion o f 1 to 3 0 hours.

DISCUSS IONA t least 10 of the 26 ch ild ren in th is

fo llow -u p s tudy have urine tha t m ust beco nside red abnorm a l. T he urin ary ab nor-mali ty is c ha ra cte riz ed by hem atun ia an dcy lind ru ria ra the r th an pro te inun ia . T heleng th o f fo llow -up preclu des the abno rm alu rin e b ein g con side red pa rt o f the acu tephase o f the d isea se . T he ab norm a lity w asfou nd m ost o ften in ch ild ren w ho w ereover the age o f 6 years a t the tim e o f onse to f p u rp ura, and w ho had hem a tu ria o rp ro tein u ria du ring the acu te ph ase o f thei l lness. I t is gene ra lly accep ted th a t ana -phy lac to id p urpura occurs m ore comm onlyin m a le s than fem a le s.1 9 T he sim ila r sex

ra tio in ch ild ren w ith abn orm a l u rine su g-g es ts tha t, w h ile the re is a sex d ifferen tia -tio n in the a ttack ra te o f th e p urp un ic m an i-fe sta tion s, the re is no m ark ed sex d iffe ren -tia tion in the a ttack ra te o f the su bsequ en turin ary ab no rm a lity .

R ena l m an ife sta tio ns o f anaphy lacto idp urpura h av e b een recog n ized sin ce th ed isease w as firs t de scr ibed . Heno ch ,3O sle r,5 and m ore recen tly G a ird ne r b e-liev ed th is to be the m ost se r ious com plica-tio n . P a tho log ic stu d ie s o f th e k id neys frompatien ts du rin g th e acu te p hase o f thed isease have show n ea rly ch an ges o f su b -acu te g lom eru lo nephn itis .1 Ind eed , inv iew o f the w idesp read vascu la r les ionsth ro ughou t the bo dy , it sho u ld be ex-p ec ted tha t such rena l invo lvem en t w ou ldbe foun d . B ecau se o f th e k now n v ascu larinvo lvem en t, and the na tu re o f the u rina ryab norm a lity , th is seq ue l to th e d isea se inthe 10 ch ild ren in th is se rie s seem s to fitbe st in to the c las sifica tion o f laten t n ep hn itis .T h e urine , w h ile ab norm a l, co n ta ined few erfo rm ed elem en ts and le ss p ro te in thanis u sua lly seen in chron ic o r subacu ten ephritis , and non e of the ch ild ren as ye tshow s cons titu tion al m an ife sta tions o f ren alin su ff iciency .

T he re is no t eno ugh d ata in the lite ra tu reto eva lua te the p rog nos is fo r th e se c lii!-dren , w ho , a fte r a p ro long ed pe riod o ffo llow -u p , appa ren tly m an ifes t a la ten tphase o f the d isea se . I t seem s m ost like lyth a t the h em atu ria m u st hav e ex istedth ro ughou t the y ears fo llow in g the in itia lm an ifes ta tion s of th e a ttack o f pu rpura .T he m ajo rity o f r t5 15 desc ribe ag rave ou tlook fo r th e nephritic seque lae o fth is d isea se w ith in on ly a y ea r o f its onset.W e have em p loyed th e te rm laten t ne -p h ritis adv is ed ly to sugg est tha t th e ren alle sion m ay e ithe r h ea l o r p rog re ss . In fo l-low ing th ese ch ild ren w e h av e taken ca reto avo id d iscuss in g any se riou s p rog nosis ino rd er to m in im ize th e im press ion of d is-ab ility w h ich the d iscuss ion o f ren al d is-ea se can produ ce so easily . H ow ev er , theknow n p ath o log ic f ind in gs o f anaphy lac -to id pu rpu ra , the p robab le p ro lon ged dura -


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20 4 WEDGWOOD - ANA PHYLACTO ID PURPURAtion of th e h em atu ria , the dec reased ureaclearances fou nd in 4 , and the d ecreasedab ility to con cen tra te u rin e fou nd in 1 o fthese 10 ch ild ren , su ggest a t lea st a po ten -tially se riou s na tu re fo r th ese no t u ncom -m on ren al seq ue lae.

T he re is no know n the rap y fo r th e rena lseque lae o f anap hy lac to id pu rp ura . A d -ren ocortico trop ic h orm on e an d co rtiso nehave b een u sed bu t d o no t app ea r to p re-v en t o r am e lio rate the u rina ry abn orm a li-t ies;8 16 and th ere is su ggestive ev idenceth at in som e fo rm s o f rena l d isea se theseh orm on es m ay be 7W heth e r th e rena l sequ elae can be pre-v en ted b y m ore rigo rou s trea tm en t in th eacu te p hase (e .g ., p ro lo nged b ed rest, ad e-qua te an tib io tic th erapy fo r in fec tions ) isa lso unknown . B ecause the m ajo r ity o fch ild ren w ho dev elo p th is com plicatio n a reo ve r th e ag e of 6 y ea rs d u ring the acu tea ttack , an d have a t th at tim e e ithe r hem a-tuna o r p ro tein un ia , such preven tive th en -apy , if use( l, sho u ld pro bab ly b e d irec tedp ar ticu la rly to th is g roup . A s w ith a ll ch il-d ren w ho have laten t rena l d isea se , in ter-cu rren t in fec tio n sho u ld b e trea ted v igo r-ously ; on e of the ch ild ren in th is se ries( C ase 17) had a pron oun ced an d sud deninc rease in h em atu n ia during and imm ed i-ate ly subsequen t to a be ta hem oly tic s trep -toco cca l in fec tion .

M any adu lt p atien ts w ith ch ron ic ne-phritis g ive no h isto ry o f acu te g lom eru -lonephn itis and curren t ped ia tric sugges t tha t few , if any , ch ild ren w ith theclassic po st-s trep to co cca l acu te g lom eru-lo nephritis p ro g ress to a ch ro n ic phase . O new ond ers w he the r som e o f the pa tien ts w hohave chron ic rena l d is ea se in adu lt lifem igh t h av e had a m ild ep isod e of anaphy-lac to id pu rp ura# {176} in ch ild hood w hich hadbeen th ough t at th e tim e to be in sign if i-can t, o r e lse h ad passed unno ticed . P e r-haps th is gene ralized d isease, w h ich is

o E ig h t o f the ten ch ild ren w ith abno rm al u rinele ft the u su al pedia tric age gro up prior to the find -ing of u rina ry abnorm ality a t a tim e w hen th eyw ere th ough t by the ir fam ilie s to b e en tire ly w ell.

ch a rac ter ized and recog n ized by its sk inles ion , can ex ist in a fo rm w ith v isce ra lm an ife sta tio ns alo ne .

SUMMARYA long-te rm fo llow -up stu dy of 26 ch il-

d ren w ith anaphy lac to id purpu ra (S ch# {246}n-lein -H enoch Synd rom e) is p re sen ted . Th eave rage len g th o f fo llow -up w as 4% years .O f th ese 26 ch ild ren , 1 0 w ere fo und toh av e an app a ren t la ten t nephritis ch aracte r-ized by h em a tu ria and cy lindu n ia , bu tw ith ou t m arked pro te inun ia. N in e o f theten ch ild ren w ere ove r th e age o f 6 y ea rs a tth e tim e of onse t o f pu rpu ra ; 6 had p ro -te in un ia o r h em a tuna d uring the acu tephase o f the d isease. I t is sugg ested th atth is ren al seq ue la o f anap hy lac to id pur-p u ra m ay con stitu te o ne o f the o rig ins o fch ron ic rena l d isea se o f u nknow n e tio l-ogy w hich o ccu rs in adu lt p atien ts .

ACKNOWLEDGMENTT he au tho rs a re ind eb ted to the m em -

be rs o f the h ouse sta ff o f B ab ie s and C h il-drens Hosp ita l fo r the ir ass is tan ce in th iss tudy .

REFERENCES1 . G a irdne r , D . : T he S ch# {246}n le in -H eno ch Syn -

d rom e . Q uart. J. M ed ., 17 :9 5 , 1 94 8 .2 . H eno ch , E . : U eber e ine eigen thum liche

Form von Pu rp ura . B e rl. k lin . W chn sch r .,11 :641 , 18 74 .

3 . H enoch , E . : V orlesun gen ilbe r K inder-k rank he iten , 1 0 th E d . A . B erlin , H irsch -w a ld , 18 99 , p . 8 39 .

4 . S ch #{ 24 6} nl ei n, J. L . : A lIg . u . spec . P a th . u .The rap ., H en isau , L it.-C om p t., 1 1:48 ,3 rd E d ., 183 7 .

5 . O sler , W . : T he v iscera l le sion s o f pu rpu raand a llied co nd itio ns. B rit. M . J. , 1 :517 ,1914.

6 . S he ldo n, J. H . : P urpu ra N ecro tica . A rch .D is. C h ild hood , 2 2 :7 , 1 947 .

7 . L ev itt, L . M ., and B urbank , B . : G lom eru -loneph n itis as a com plica tion of theSch#{246 }n le in -H enoch Syndrom e. N ew E ng-land J. M ed ., 24 8 :53 0 , 19 53 .

8 . W edgw ood , R . J. P ., and Jan ew ay , C . A .:S erum com plem en t in ch ild ren w ithco llagen d isea se s. PEDIATR ICS , 11 :569 ,1953 .


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OR IG INAL ART IC LES 20 59. Philpo tt, N I. G . : T he Sch# {246}n le in -H enoch

Syndrome in ch ildho od w ith p articu la rreference to the occuren ce of neph ritis .A rch . D is. C h ildho od , 27 :48 0 , 19 52 .

1 0 . A ckro yd , J. F. : A lle rg ic purp ura , inc lud ingpurpu ra due to food , d rugs , an d in fec -tions . Am . J. Med . , 14 :605 , 195 3 .

11 . L y ttle , J. D . : The A ddis sed im en t coun tin norm al ch ild ren . J. C lin . Inv estiga -tion , 12 :87 , 1933 .

12 . G iles, M argare t D . : T h e A ddis coun t inth e prognos is o f acu te nephritis in ch ild -h ood . A rch . D is . C h ildhood , 22 :2 32 ,1947 .

13 . A dd is , T . : C lom eru la r N ephritis : D iagno-sis and Trea tm en t, 1 st E d . N ew York ,M acm illan , 1948 .

14 . H am , T . H ., E d ito r: A Syllabus of L abo-ra to ry Ex am in ation s in C lin ical D iagno-sis, 1 st E d . C am bridg e , H arvard , 1953 .

15 . F erra ri, M ., L ap ido , C ., and y Sch er-schemer , J. : Sm nd rom es ren ales en e lp#{2 41} rpura reum ato ide . A n . F ac. m ed .\Ion tev ic leo , 38 :239 , 1953 .

16 . Ph ilpo tt, N I. C ., and B riggs , J. N . : T rea t-m ea t o f the H enoch-S ch#{246}n lein Syndrom ew ith adrenocor tico trop ic ho rm one andcor tisone . A rch . D is . C h ildhood , 2 8 :57 ,1953 .

17 . \Iichaels , L ., and W alte rs , C . : In c reasedh em a tunia in nephritis du rin g co rtison eand ad renocortico trop ic ho rm one ad-minis t r a t ion . A rch . D is. C h ildhood , 28 :213, 1953.

18 . R ub in , M . I . : D istu rb ances o f the k idn ey ,in M itche ll-N e lso n T ex tbook of Ped i-atrics, 5 th E d ., W aldo E . N elson , E d ito r.P h ilade lph ia , S aunders , 19 50 , p . 1217 .

1 9 . G oe ttsch , E lv ira : N ephn itis and n ep hros is ,ill B renn em anns P rac tice o f P ed iatric s ,III. H agers tow n , P rio r , 1948 , chap . 28 ,p. 7.

SPAN ISH ABSTRACTP i rpu ra A nafilac to ide ( S Ind rom e d eSchon le in -H eno ch ) : E stu d io con

re fe ren c ia e spec ia l aC om p li ca ci #{ 2 4 3} n R e n al

La p# {241}rpu ra anafilacto ide es una enfe rm edadgenera lizada carac ten izada por lesion es espec ifi-ca s d e Ia p ie l, genera lm en te ag regadas des in tom as ar ticu la res y a m enudo g astro -in testina les ; se le aso cian los nom b res deS chon le in v H enoch po r Ia d esc ripc i#{2 43}n c l# {225 }sicay com ple ta que es tos au to res h ic ie ron de lr adecimiento. S e desco noce la e tio lo g la pero

se supone que es t# {225 }ond icion ada p or un estadode h ipersensib ilidad y qu e p erten ece a! g rupod e las llam adas enfe rm ed ad es co l#{225}g en as .

Lo s au to res p resen tan es te trab ajo parase#{2 41}a la r Ia p resenc ia y na tu ra leza de las secu e lasa Ia fase aguda de l padec im ien to , segd n lasobservacion es sobre 26 n iflos es tud iados , d e 13m eses a 1 2 y m edio aflo s d e ed ad , s iend o 17n inos y 9 n iflas , nad ie de Ia raza negra . N ing#{241}nfam ilia r ce rcan o d e estos n ino s presen t#{243} iiip iirpura anafilac to id e n i n ingu no de lo s llam adospadec im ien tos co l#{225}genos .

T rece d e lo s ve in tis# {2 33} is ni#{241 }osad ec ie ro n in -fecci# {243 }nde las v Ias resp irato r ias a ltas inm ed iata -m en te an tes 0 b ien con com itan tem en te a Iain iciac i#{2 43}n d e Ia p# {241 }rpu ra ; 4 niflO s m o stra ronan teceden tes fam ilia res d e a le rg ia y o tros 4p ad ecIan a lerg ia en s i m ism os . D uran te Ia faseagu da , 12 de los 26 im os p resen ta ron sIn tom asg as tro in testina les (11 d o lo r abdom ina l y 7ep isod io s d e em es is; 2 co n m elena abundan tese opera ron pens#{225}ndose en invag in ac i#{243}n re -sec#{225}ndo le a l p r im ero una p orc i#{2 43}n d e in tes tinogangrenad o pero sin con flrm arse e l d iagn#{243 }sticoen e l o tro ) . A lgun os de es to s n if lo s rec ib ie ronan tih is tam #{23 7}n icos sin m in gn efec to .

L o s da tos de lab ora to rio y ev o luc i#{243}n sepresen tan en las f ig u ras 3 , 4 , 5 y 6 . A tod osse les ob serv# {243 } d esde e l pun to de v is ta de lascom plicac iones rena les, resum en d e cuyos da tosse encuen tran en las figu ras 7 , 8 y 9 . D eacuerdo con Ia cuen to de A dd is los 26 im osse d iv ide ron en dos gru pos ; 10 presen ta ro n enfo rm a defin itiva m an ifestac iones de les ionrena l, q ue no puede co nsidera rse com o p artede Ia fase agud a de Ia enfe rm ed ad . S e ob servOuna co rrelac iO n de fln ida en tre Ia edad de l n i#{241 }oa! in icia rse e l pad ec im ien to si su grad o de lesiO nrena l, ca rac ter izada por hem atu ria y c ilind ru riapero sin pro tein uria acen tu ad a: n ueve con m i-c iac iO n despu #{233}s de lo s 6 aflos de ed ad (6 co npro te inuria y hem atu ria duran te Ia fase agu dade l padec im ien to ) y sO lo u no an tes de esta ed ad ;esta d ife renc ia es estad Is ticam en te s ign ifica tiva .Tam b i# {233 }n se encon trO relac iO n , aunque noab so lu ta , en tre Ia p ro te inuria y hem atu riainiciales y su p ersistenc ia en las consu ltas derev is iO n . N o se enco n trO re lac iO n aparen te n ien tre Ia in tensidad o durac iO n de Ia fase in ic ia lde l padec im ien to y las anorm alidades ren alessubsecuen tes n i tam poco en tre Ia exacerbac iO nde Ia pO rp ura duran te Ia fase ag uda y anorm ali-d ad es urina rias en Ia rev is iO n de los casos. E ntan to que se o bservO d iferen c ia de l sexo en


11/11

20 6 WEDGWOOD - ANA PHYLACTO ID PURPURAcuan to a Ia inc idenc ia d e las m an ifes tacion espurpO ricas, no la hubo en Ia in ciden cia de lacom plicac iO n rena l subsecuen te .

E l p ronO stico d e estos n i#{241}os n o puedeva lo ra rse ; Ia m ayorIa de los au to res lo con-s ide ran g rave para las secu elas nefrIticas . N oex iste tra tam ien to efec tivo p ara las com plica -c iones rena les; en caso de ex is tir deben tra ta rseen#{233} rg icam en te las in fecc iones in tercu rren tes.

N uestros au to res cons id e ran q ue las secue lasrena les de Ia pO rp ura anafilac to ide p uedencons titu ir u na de las cau sas de p ad ec im ien tosrena les c rO nicos de e tio logIa descon oc ida enlos ad ulto s.

IN TER LINGUA ABSTRACTPurpura A n aphy lac to id e ( Syndrom e deSch#{246}n l e in -Henoch ) : Stud io de C on -

tinu atio n a L ong e D uran tia co nR eferen tia S pec ia l aInvo lv im en to R en a l

Le s tud io h ic p resen ta te esseva in te rp rend itep ro de term in ar le frequen tia e na tu ra de lseq ue las de l phase acu te de p urpura an a-phylactoide.

B en lue le e tio log ia d el m orb o non escogno sc ite, le occurren tia in illo de ex tenselesiones vascu la r e de frequ en te in fec tionespreceden te , sp ec ia lm en te con s trep to co cco shem oly tic be ta , pa re jus tifica r le these quepurpu ra anaphy lac to ide rep resen ta un s ta to deh ypersen sib ilita te e p ertine a l g rupp o de l s i-app ella te m o rbos co llag en ic.

Le p ro to co llo s de l H osp ita l p ro In fan te s eJ uven iles a C leve lan d , O h io , reve la p ro le anno sab 1939 a 1953 un to ta l de 36 adm issiones d ecasos de purpu ra anaphy lacto ide . In 19 54 ilesseva po ss ib ile trov ar 26 de is te pa tien tes .Ille s om nes se trovava in b on sta to de san ita tee in b r to ta lita te fo rm a le base de l p resen terepor to .

Le e tate de l pa tien tes a ! tem po re de l dec la ra -tion de l m orb o esseva in te r 13 m enses e 12 e

3 ann os con un m ajo r concen tratio n in te r 2 e4 annos. D e accordo co n reportos pe r a lte reau to re s, c irca duo te rtio s d el p atien te s e ssevam ascu los . B en que le co n tem poranee popu la -tion to ta l d el ho sp ita l inc ludeva 20 pro cen toneg ros , nu lle neg ro essev a tro va te in te r le 3 6pa t ien tes de pu rp ura an ap hy lac to id e. Is ted iffe ren tia es po ssib ilem en te deb ite a l d iff i-cu ltate de d isce rn er pu rpu ra sup er u n pe llenegro ide .

In le fam ilia s de l pa tien te s nu lle a lte re oc -curren tia de p urpura anaphy lac to ide essevaco nsta ta te , n i U n frequen tia inusual d e u llea ltere m orbo co llagen ic .

In le m ajo rita te d e l pa tien te s duo annos op lu s h ab ev a passa te in te r le p r im e dec la ra tionde l m o rb o e le p re sen te stud io . In un caso lein te rva llo habeva ess ite 15 annos , in u n a lte re8 an nos . I llo h ab ev a ess ite m inus que un annoin so lo 2 casos e am ontava pro le in teg re se rie aUn lon gor m ed ian de 4 e 3 anno s.

C on le so ! excep tion de un caso ob se rv ateso lm ente duran te 6 m enses , om ne le pa tien te sin is te s tu d io habeva essite sin sym ptom as m d i-ca tive de purpu ra an ap hy lac to ide .

In 10 casos-6 m ascu los e 4 fem in in as-n ostrecon stata tion es in d icav a u n laten te neph n itischara cten isate per hem atur ia e cy lm nduria sedsin p ron un cia te p ro tein u ria . Is te g rupp o in -c lud ev a 9 pa tien tes de p lus que 6 anno s dee tate a ! tem po re de l d ec la ra tion d el m orbo . Jibinc lu deva 6 casos de pro te inu ria o hem a tu riaduran te b e p hase acu te d el m orb o .

I l pa re po ssib ile qu e is te typ o de seq ue larena l de pu rpura anaphy lac to ide cons titue u nde l o rig ines de chro n ic m o rbo rena l a e tio lo g iaincog noscite que o ccu rre in p atien tes adu lte .L e h isto r ia d e tab casos in clu de fo rsan u n lev eep iso d io juven il d e purpu ra anaphy lacto ideque a ! tem pore d e su o ccurren tia e ssev a con-s id erate com o m n sign ifica tiv e o non essevarecognosc ite de l to to . P ossib ilem en te istem orbo , que es cha rac ter isa te e recognosc ite p ersu besio nes d erm a tic , p o te ex iste r in un fo rm acon m an ife sta tiones exc lusiv em en te v is ce ral.

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