Renal Lesions in Congenital Rubella

7/28/2019 Renal Lesions in Congenital Rubella

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1967;40;901-904PediatricsGILLESPIE and A. M. MURPHY

MARGARET A. MENSER, S. E.J. ROBERTSON, D. C. DORMAN, AILSA M.Renal Lesions in Congenital Rubella

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Online ISSN: 1098-4275.Copyright 1967 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005.

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E X PER IEN C E A N D R E A SO N -B R IE FLY R EC O R D ED 90 1Using the num be r o f in fan ts at risk (num -be r o f in fan ts aliv e in the nurse ry a t the b e-gin n in g of e ach p erio d p lu s th ose adm itteddur ing the pe rio d ) a s the d enom ina to r o f th edea th ra te s fo r th e p eriod , th e cum u lativ e re -su its w ere :

go vn-100/395 x 10 0 = 25 % no g ow n - 72 /3 50 x 10 0 = 21% .

S inc e it w as d iffic u lt to ag ree on a un ifo rmdefin ition of non -fa tal b ac te ria l in fections , w edec ided tha t gene ralized fa tal in fec tion s (espe -d aIly m en ing itis) con firm ed by postm o rtemexam in ation , w ould serv e as a crude (bu t fa irlyfirm ) m arke r in th is expe rien ce. Th e m a jo rbacte r ial in fec tions w ere d is tribu ted as fo l-low s: gown-m en ing itis 6 , sep ticem ia w ith -ou t m en ing itis 2 ; n o gow n-m en in g itis 0 ,sep ticem ia w itho u t m en in g itis 2 .

COMMEN TW e repo rt th is exp e rience no t b ecause w e

be lieve tha t w e h av e ob ta ined proof tha t tra -dit ion al gown ing p recau tions in neona tal u n itscan be sa fely abandoned by a ll. N o te, fo r ex -am ple, tha t in our un it al l in fan ts are ho usedin room s (b low er-ven tila ted incub ato rs) w ith -in room s (a lso p re ssu re ven tila ted ), an d th isspec ial c ircum stan ce m ay affo rd a m easu reo f p ro tectio n no t gene rally ava ilab le . W e c iteou r expe rien ce on ly to encou rage o the rs toex am ine tim e honored prac tices w h ich m ayhave the und es irab le e ffect o f keep ing th ephysic ian and h is h igh -r isk n eo na ta l pa tien tapar t , an d to do so cau tio usly an d system a ti-cally.

W IL L IA M A . S i L V E R M A N , M.D .J OH N C . S INCLAIR , M . D .Depar tmen t of Ped ia t r ic sC o lleg e o f Phys icians an d SurgeonsC o lum bia U n ive rsityan dN eon ata l U nit, B ab ie s H osp ita lC o lum bia -P re sby te r ian M ed ica l C en te r

3975 B roadw ayN ew Yo rk , N ew Yo rk 10032

W . A . S . is a C aree r In vestiga to r o f the H ea lthResea rch Coun cil o f th e C ity o f N ew Y ork (C on -tract 1-181) ; J . C . S . is support ed by R esearch C a-re er D ev elop me nt Award of P ub lic H ealth Service(N o . 1K 3 HD -34 , 9 92-01) .

R EF ER ENC E1 . Forfar , J . 0 . and M acC ab e, A . F .: M ask ing

an d gow nin g in nu rse ries for the n ew born

in fan t. E ffec t on staphy lococca l car ria ge an dinfe ctio n . B rit . M ed . J. 1 :76 , 1 95 8 .

Rena l Les ions in Congen ita l Rube llaG regg1 no ted on e in fant w ith renal an d

ure te ric m alfo rm atio ns w hen he firs t de sc r ibedth e effec ts o f cong en ital rub ella in fec tion in1 941 . H ow ev er , the re a re few subseq uen t re -por ts of rena l ab norm a litie s in th is synd rom e .O n ly fou r ch ild ren w ith rena l les ions w erefou nd in Lunds trom s2 ex tens ive p rosp ec tivesu rvey of in fan ts born to m o th ers expo sed torubel la during the 19 51 ep idem ic in Sw ed en .R ecen tly , a rub ella-a ffec ted infan t w ith anab norm a l k id ney and doub le u re te r w as do cu -m en ted by B ellan ti, e t a i. T he p resen t rep ortconcerns a ch ild w ith p ro lon ged rube lla v ir-una an d g ros s ren al ab norm a litie s .

CASE REPORTTh is m ale in fan t we igh ed 3.05 kg wh en born

on A ugust 8 , 1 965 . N o in fo rm a tio n is avai lab lere gard in g h is pa ren ts o r the cou rse of th e p reg -nancy . O n the secon d day o f life a soft G rad e I-I!m id-sys to l ic c ard iac m u rm ur w as aud ib le ove rthe p reco rd ium , h is che st x - ray w as n orm al , andca tara cts w e re no t d ete cte d , a lth ou gh the p ossi-bility of the pre sen ce of sm all lesions w a s no tex clu ded . W hen 18 weeks o ld , a m a tu re cata rac tw as pre sen t in the lef t lens an d the re w as asm aller cen tral po ste rio r co r tica l opac ity in th erig h t lens . H is h ead c ircum ference w as 37 .5 cm ,an d a sev ere degre e of b ila teral d eafnes s w a s sus -pected .

O n Decembe r 14 , 1965 , the in fa n t w a s ad-mit ted to th e hosp ita l w ith a cu te b ro n ch itis , con -junct iv i t is , an d oti t is ex te rna . H e w as pyrex ic andt here w as m in im a l p ro te rnu nia . A cen trifu ged spec-imen of u rine con ta in ed 1 -2 leucocy tes pe r h ig hpow er fie ld and 4-6 g ran u la r cas ts. U rin e cu l-ture show ed no bacte r ial growth bu t p rov ided arub ella v irus iso la te . B e ta -h em oly tic strep tococcuswa s no t p re sen t in the ph a ryn gea l sec retio ns. H isblood u rea wa s 80 m g p er 100 m l, and th e seruma lbumin w as norm al.

O n Jan uary 24 , 19 66 , th e rig h t c ata ract w a sasp ira ted and 1 m onth late r the left catara ct wa strea ted s im ila rly . R ube lla v iru s w a s iso lated fromthe cata rac tous m ate ria l rem ov ed from both ther igh t an d le f t lense s a t ope ratio n (F ig . 1 ).

T he b loo d urea fell gradu ally (F ig . 1 ) and w as52 m g per 1 00 m l o n Feb rua ry 28 , 1 966 . In -travenous p ye lography dem ons tra ted ren al fun c-tion on the r igh t side bu t n ot on th e lef t. A cys to-gram and re tro grad e py elo gram (F ig . 2 ) sh ow ed

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F ic . 1. C lin ical p rog ress o f m ale in f an t w ith cong enital ru bella.

f r ee r e flu x u p b oth u r e t er s d u r in g fi llin g o f t h eb la d d er , som e d ist en sio n of t h e r igh t u r e t er a n dpelv i-caly ceal sy s tem , bu t n o ev idence of a p elv isor caly ce s on tile le f t s ide . A le f t renal sh adowco u ld n ot b e se en .

R u b e lla v ir u r ia p e r sist ed . T h e b lood u r e a w a s51 m g p er 10 0 m l w h en th e in fa n t w a s 8 m on th sold . O n M ay 6 , 1966 , 2 .5 m l o f p oo led hum angam m a-g lobu lin (C om m onw ealth S erum L abo ra-t o n e s# {176 } a tc h N o. 047 w h ich has been show n4 tohav e a titer o f n eutraliz in g an tib ody to rub ellav irus o f 1 : 1024 ) w ere giv en by in tram uscular in -je ction . R ube lla v iru s w as no t iso lated f rom u rineo r pharny g eal secretio ns af te r the in f ant w as ag ed1 0 mon th s .F u ll a s sessm en t of r en a l fu n ct io n w a s c a r r iedou t when th e ch ild w as 13 m on th s o ld . A t th istim e leucocy te s w ere n o t de tected in th e ur ine,th e re w as no pro te inu ria and te s ts o f ren al con -cen trating ab ility and tubu lar fu n ct ion w er e n or -m al. R andom blood urea w as 38 m g per 100 m lbu t th is f e ll to 22 m g per 100 m l f ollow ing in-stitu tion of a low p rotein diet. In trav enou s py -elography again failed to dem on strate any renalf un ctio n on th e le f t side . R enal scannin g p er-f o rm ed on N ov em ber 23, 1 966 (Fig . 3), u sin gHg show ed no f un ctioning renal tissue on th ele f t. T h e righ t k idn ey had a no rm al iso to pe dis-tribution an d m easured 9 cm by 5 cm .

V I R O L O G YS pec im ens w e re co llec ted in sep a r a t e bott les

and processed accord ing to th e m ethod s desc rib edprev iou s ly u sing th e R K ,, co n tinuous lin e o f rab -b it k id ney cells .

R ube lla v irus w as f r equen t ly iso lated f rom

u rine and phary ngeal sec ret ion s until the in f an tw as 10 m on th s o ld (Fig . 1 ). R u be lla v iru s w asalso iso lated f rom t ile ca ta r a c t ou s m a t e r ia l r e -m ov ed at o peration f rom both th e rig h t and le f tle nse s but no t f rom spec im ens of con jun ctiv al S e-c ret ion and aqueous hum o r co lle cted at th e s amet i m e .

Ne u t r a l i z i n g an tibody titers w ere de term in ed inR K 13 cu ltures using th e m e thod d esc ribed b yRe i d , e t a l. S erum co llec ted w hen th e in f an t w as4 m on th s o ld had a tite r o f neu traliz ing an tibodyt o r u b ella v ir u s of 1 : 128 ; at the age of 1 3 m onthsth e t i t er w a s 1 : 51 2 (F ig . 1 ).

N eutraliz atio n w as f ound to occur in sim ilar d ilu -l ion s in a sy st em u sin g th e ch ild s se rum againsto ne of h is ow n rub ella v irus iso late s.

C O M M E N TS ix ch ildren hav e b een reported prev iou sly

w i th r e n a l lesion s p oss ib ly d u e t o con g en it a lrub ella. O f th ese , tw o had poly cy stic k id -ney s, tw o had a bibb ed k id ney w ith dup lica-tion o f the co llectin g sy s tem , and the natureo f the abnorm ality w as no t d escribed in therem ain ing tw o . N ow , a ch ild w ith d eaf ness,m icro cephaly , b ilateral catarac ts, p ro long edrubella v iru ria, an d p r ob a b le a g en esis o f t h ele f t k id ney is reported . I t is suggested thatth is renal le sion m ay be du e to cong en italru bella in f e ction .

D ev e lopm en t o f the k idn ey d ep ends uponfo rm atio n o f a ure teric bud . T h is bud d if f e r-en tiates caudally f rom th e n ephric d uc t at theend o f the f irst m onth o f g es tatio n .6 A s thebu d e lon gates i t a s cen d s t h e p os t er io r a b d om i-nal w all. In the 10 to 15 m m em bry o , prim ary



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Fic. 2 . R e trog rad e p ye log ram .

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EX PER IE N C E A N D R E A SO N -B R IE FLY R E C O R D ED 90 3tub u le s grow out from the p rim itive rena lpe lv is in to the su rro und ing m esoderm al tis su ew here th e kid ney is fo rm ed . If the prim a rytubu les d o no t d iffe ren tia te, a func tio n ing k id -ney fai ls to d eve lop . D ifferen tia tio n w ouldseem to have a rre sted at th is stag e in thep resen t ca se , po ssib ly due to the p re sence ofrubel la v irus 11 1 the em b ryo . I t is o f in te res tthat , ill Co llin ss se rie s of 58 1 p atie llts withun ila tera l re na l agenes is, the ure ter w a sto ta l ly absen t in o n ly 51% .

Som e infan ts w ho are cong en ita l ly in fectedw ith ru be lla v irus ha rb o r and ex cre te the v irusfo r m any m onths afte r b irth . It is known tha tp ers isting ru bella v irus in fec tion m ay con-tribu te to the pa tho gen esis o f fur the r d efe ctspostnata l ly . 10h1 T he presen t in fan t had ru -b ella v in in ia fo r a t lea st 10 m on th s an d , al-th oug h at f irst h is ren al fu nction ap pearedg ros sly im pa ire d , i t g radu ally im pro ved (F ig .1 ) . T h is im pro vem en t in fu nc tion su ggeststh at a slow ly re so lv in g le sio n m ay h ave b eensu per im posed u pon th e ba sic rena l d efec t.

R en al g lom eru ia r and tub u la r ce ll chang eshave rare ly been seen in co n jun ctio n w ithco ngenita l ruh ella3 ,b0 ,121 4 but a re w ell do cu-mented in in fec t ious hep at it is.1 5 In the p re sen tca se , rub ella v iru s m ay have caused a n e -phnit ic pro ces s, in ad d it ion to gro ss structura lre nal abn orm alitie s. T he infan ts urin e con -tam ed leu cocy te s and g ran u la r cas ts bu t oncu ltu re sh ow ed no bacte r ial g row th . T he fa llwh ich w as ob served in th is ch ild s b lood urea

m ay repre sen t a pro gre ssiv e im provem ent inh is ren a l fu nc tion as the v ira l in fec tion w anedand the n ep hritis re so lved .

T h is is th e secon d in fan t to b e reco rd edwith viro log ically -p rov en cong en ita l rub ellaal ld a rena l le sion . A s m o st cen te rs h av e no t

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F ic . 3 . R ena l scan using 1TT Hg .



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90 4 SM A LL V E SSEL C A N N U L A T IO Nin clu ded tes ts of rena l fun ctio n in th e rou tinec lin ica l a ssessm en t o f rube lla -a ffec ted in fan tsand as rena l-ar te ry steno sis has now been re -po rted in th ree in fan ts w ith th e rub e lla sy n -d rom e,1 6 it appears lik ely tha t rena l an d reno-v ascu lar ab norm a litie s m ay have b een ove r-look ed in th e p as t. T h e re fo re , it is recom -m end ed tha t a tten tion be p a id to any c lin ica lo r labo ra to ry ev idence of ren al dys fu nc tion inin fan ts w ith congen ita l rube lla , tha t an a t-tem pt b e m ad e to co rrela te such ev idencew ith the dura tion of activ ity o f ru be lla v irusin th e k id ney , an d th at n ec ropsy an d b iop syspe cim en s b e c arefu lly ev alu ate d fo r ind ica -t ionsof rub ella neph ritis .

S U M M A R YR en al les ion s h ave rarely b een repo rte d in

con gen ita l rub ella . A m ale in fan t w ith c la ssi-ca l rube lla synd rom e de fec ts an d agenesis o fth e le ft k idn ey is desc r ibed . R ube lla v iru s w aspresen t in h is u rine fo r 10 m onths . H is rena lfun ction , a ltho ugh poor in itia lly , im provedpro gress ive ly . It is su ggested tha t con gen italru be lla in fect ion w as resp on sib le no t o n ly forh is rena l d efe ct bu t also for a slow ly reso lv -in g n eph ritis .

M A R G A R E T A . M E N5 E R , M . B . , M.R .A .C .P .C h ild ren s M ed ica l R esea rch Founda tionRoya l A lexan dra H o sp ita l fo r C hild ren

P ost Office Box 34Camperdown, N.S.W. 20 50 . Austra lia

S . E . J. R O B E R T S O N , M .B ., F .R .A .C .P .Royal A lexan dra H osp ita l fo r C hild renD . C . DO RM A N , PH .D .A I L S A M . G I L L E S P I E , M.B .In stitu te o f Pa tho log yRoyal A lexan dra H osp ita l fo r C hild renA . M . M U R P H Y , B .S c .I n s t it u t e o f C lin ica l P a tho logy

an d M edical R esearchLidcombe , N.S.W. 2 141 . Austra lia

W e w ish to th an k A sso cia te P rofe sso r J. McRae ,Radio iso top e U nit, D ep artm ent o f M ed icine, U ni-v ers ity o f S ydn ey , fo r p erm iss ion to pu blish therenal p h o t o s c a n , and D r. D . A rno ld and D r. L ornaT ann en baum fo r th eir he lp w ith th is case .

R E F E R E N C E S1. G regg , N . M cA . : C ongen ita l c ataract fo l low -

ing G erm an m ea sle s in the m oth er. T ran s.O pth al. So c. A u st., 3 :35 , 19 41 .

2 . Lund strom , R . : R ubella d uring pregnancy :Follow -up s tudy o f ch ild ren b orn afte r anep idem ic o f rub ella in Sw eden , 19 51 , w ith

additio nal inv est iga tion s on p rop hylax is andtreatm en t o f m aternal rub ella . A cta P aed iat .( S to ck ho lm ) ( S upp l. 133 ), 51 : 1, 196 2 .

3 . B e llan ti, J . A ., A r t en st ein , N I. S ., O lson , L . C .,B u escher , E . L ., L uh rs, C . E ., and M il-ste ad , K . L . : C ong enita l ru be lla . Am er. J.D is. C hild ., 1 10 :46 4 , 19 65 .

4. M urphy , A . M ., an d R eid , R . R . : N eu tral iz-ing an tib od ies to rube lla v irus in g am m aglob ulin and convalesc en t sera . M ed. J.Aust., 1 :3 69 , 1 967 .

5. Reid , R . R ., M urp hy , A . M ., G ille sp ie , A . M .,D o rm an , D . C ., M en ser, M . A ., H ertzberg ,R ., and H arley , J . D . : Iso la tion of ru bel lavirus from congen ital ca tarac ts rem oved a toperat ion . Med . J. A us t., 1 :54 0 , 196 6 .

6 . Sm ith , D . W . : D ysm orpho lo gy (te rato lo gy) .J. P ed iat. , 69 :11 50 , 1 96 6 .

7 . B lack , D . A . K . : R enal D ise ase . O xford :B la ckw ell Scien t ific P ublicat ion s, p . 9 , 1 963 .

8 . C ollins, D . C . : C ong en ita l un i la te ral ren alagene s ia . A nn . Su rg ., 95 :71 5 , 193 2 .

9. Cooper, L . Z ., and K rugm an, S . : D iagno sisan d m anagem en t: co ng en ita l rub ella . P ED I -ATRI CS, 37:3 35 , 19 66 .

10 . Naeye , R . L ., and B lanc , W . : P ath og en es iso f congen i ta l ru bel la . J.A .M .A ., 19 4 :1 27 7 ,1965 .

11 . M en ser, N I. A ., H arley , j. D ., H ou sego , C . J. ,an d M u rphy , A . M . : Po ssib le ch em ica l fac -to rs in the po stna tal d eve lopm ent o f rub ellacatara cts. L ancet, 2 :77 1 , 1 96 6 .

1 2 . S w a n , C . : A stu dy o f th ree in fan ts dy ing fromcongen ita l defects fo llow ing m atern al ru -b ella in the ear ly s tag es of p reg nan cy . J.Path . B ac t., 56 :28 9 , 1944 .

13 . T #{246}ndu rv , G ., and Sm ith , D . W . : F etal ru -bel la pa tho log y. J. P edia t., 68 :86 7 , 19 66 .

14 . B ayer, W . L ., Sherm an , F . E ., M ich ae ls ,R . H ., Sze to , I. L . F ., and L ew is , j. H .:Pu rpu ra in con gen ita l a nd acq uired ru bel la .N ew E ng . j . M ed ., 273 :13 62 , 1 965 .

15 . C onrad , M . E ., Sc llw artz , F . D ., and Y oung ,A . A . : In fec tious hep atitis-a genera liseddisea se . A s tud y of ren al, ga stro in test ina land h em ato log ic abno rm alit ies . Am er. J.M ed., 3 7 :7 89 , 19 64 .

1 6 . M enser, M . A ., D orm an, D . C ., R ey e, R .D .K .,and Re id , R . R . : R enal-ar tery ste nos is inrub ella . L ancet, 1 :571 , 1 96 7 .

in t ra v e n o u s C u t d o w n -T e c h n ic a l A idin C a n n u la t io n o f S m a ll V e s s e ls

The m ethod fo r perfo rm ing in trav enous cu t-dow n in m o st in s titu tion s is usu ally stan -da rd ized up to the po in t o f th e actu al in se rtio n



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1967;40;901-904PediatricsGILLESPIE and A. M. MURPHY

MARGARET A. MENSER, S. E.J. ROBERTSON, D. C. DORMAN, AILSA M.Renal Lesions in Congenital Rubella

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Renal Lesions in Congenital Rubella