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8/10/2019 Pediatrics 1950 BARNESS 486 503
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1950;5;486PediatricsLEWIS A. BARNESS, GRETCHEN H. MOLL and CHARLES A. JANEWAY
NEPHROTIC SYNDROME: I. Natural History of the Disease
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The online version of this article, along with updated information and services, is located on
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PrintIllinois, 60007. Copyright 1950 by the American Academy of Pediatrics. All rights reserved.by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village,it has been published continuously since 1948. PEDIATRICS is owned, published, and trademarkedPEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication,
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1950;5;486PediatricsLEWIS A. BARNESS, GRETCHEN H. MOLL and CHARLES A. JANEWAY
NEPHROTIC SYNDROME: I. Natural History of the Disease
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Online ISSN: 1098-4275.Copyright 1950 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005.American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007.has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked by thePEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it
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48 6
N E P H R O T IC S Y N D R O M E
I. N atu ra l H is to ry of the D isease
B y L E w is A .
B A R N E S S , M. D . ,
G R E T C H E N H. M O L L ,
M. D . ,
A N D C H A R L E S A . J A N E W A Y , M . D .
Bos ton
T H E nep hro tic synd rom e, w h ich occurs m ain ly in ch ild ren , is m an ifes ted by edem a,
a lbu m in uria , lipem ia and hyp opro te inem ia , u sua lly w ith re la tive ly n orm al leve ls o f
nonpro tein n itrogen in th e b lood and re la tiv ely no rm al b lood pressu re , and is charac ter -
ized by a chron ic cou rse w ith exacerba tions , rem issio ns and , in m any instances , even tua l
recovery . It is the purpose of th is paper to rev iew the n atu ral h is to ry of th is d isease , as it
has been ob se rv ed in th is clin ic, a s a backg rou nd fo r p re sen t and pro jec ted stud ie s on its
p ath ogenesis an d trea tm en t. P a rticu lar em ph as is has been p laced o n estab lish in g crite ria
w hich m igh t assist p rogn osis and o n iden tify ing the facto rs w hich con tribu te to an u n-
favorab le ou tcom e.
A lthoug h sim ilar s tu d ies have been m ade in o th er c lin ics,14 the ex tens iv en ess o f th is
m ate ria l, possib le geograph ic d ifferen ces , and an im press ion tha t the prog nos is had
changed cons iderab ly w ith th e adv en t o f an tibac te ria l chem otherap y led us to und ertak e
th is ana lys is. T w o recen t a rtic les5 {176}ave rev iew ed the lite ra tu re con cern ing th is im p or-
tan t
ch ron ic d isease of ch ildho od .
M E T h O D
T he reco rds of a ll C h ild ren s and In fan ts H osp ita l pa tien ts w ith th e nephro tic syn drom e ad m itted
d uring the period 19 26 to 19 48 w e re e xam ine d . L et ters w ere w rit ten to the p are n ts or p hys icia ns
o f pa tien ts la st k no w n to b e aliv e bu t no t und er act ive trea tm ent , ask ing th e pa tien ts to retu rn to the
h osp ita l fo r a fo llow -up visit. W hen th is w as n ot p os sib le , the p are n ts an d phy sic ian s w e re a ske d to
d escribe the hea lth sta tus o f the ch ild ren and to
give
the resu lts o f recen t u r in e and b lo od pressu re
exam ina tions w hen p oss ib le . If the ch ild h ad d ied , the p aren ts w e re ask ed to re cord th e cau se of
death .
T he ana ly sis o f the d ata in th e
rec ord s w a s car ried out in the fo l low ing m ann er : C har ts o f eac h
ca se w e re m ad e, show in g the f luc tua tion s in w e igh t, p ro tein uria , he m a tur ia , b lo od p res sure an d
nitrog en reten t ion o ver the y ears. T he se find ings w ere c orrela ted w ith th e o ccu rre nce of in fec tio ns an d
var iou s thera pe u tic pro ced ure s. C r iter ia fo r a fav orab le pro gno sis w e re e stab lished b y s tud y of the
records of
pa tien ts k no w n to b e liv in g an d w e ll. T he cou rse a nd find ing s in th is group w ere com -
p are d w ith the co urse and fin d in gs in a g rou p of sim ila r ca ses w hic h h ad e nde d in de ath , a nd in
w h ich the d iagn osis of lip o id ne phrosis w as m a de a t au top sy . S e veral o f th ese au top sie s h ave bee n
reported by W olbach and B lack fan7 as exam ples o f lipo id nephros is.
C riter ia fo r an un favorab le p rogn osis w ere es tab lished by a s tudy of the records o f pa tien ts w h o
die d a rena l fa ilu re an d in w ho m autop sy sho w e d de fin i te g lorneru lo nep hri tis .
F rom th e D ep ar tm en t o f P ed iatric s, H arvard M edica l Schoo l an d T he C hild ren s M edica l C en ter ,
B os to n , M ass.
S up po rted by a gra n t, rec om m e nde d by the P an el on H em ato log y , fro m the N atio na l In sti tu te o f
H e alth o f th e U n ite d S ta tes P ub lic H eal th S erv ice , and also b y a gra n t from the M e ad Joh nso n
C o m p a n y .
P resen ted in ab brev ia ted form at the E a ste rn A re a! M e etin g o f the A m er ican A ca dem y o f P ed i-
atrics , B u ffa lo , N .Y ., A pril 2 9-30 , M ay 1-2 , 19 48 .
(R ece ived fo r
pub lica tio n A pril 1 3 , 194 9 .)
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N E P H R O T IC S Y N D R O M E 4 87
T h e d is tin gu ish in g fea tu res o f each o f th ese g roup s of cases w ere de te rm in ed by an ana lys is o f
the frequ en cy and d ura tion of such sig ns as he m a tur ia , n i tro gen reten t ion an d hyp ertens ion . A study
w as m ade o f the ca use s o f de ath in o rde r to see w h at step s m ight b e tak en to red uce the m o rtal ity in
the fu tu re. A n effo rt w as a lso m ad e to de te rm ine w hat
type
of ep iso de appeared to in itia te rem is -
sio ns or ex ace rba tion s.
D E F I N m 0 N 5
F or the pu rpo se s of th is rep ort , th e fo llow ing d efin itions a re u sed :
1. A c ut e g lo m er ul on ep h ri ti s- an acu te rena l d isease, w ith acu te o r abru p t onse t u sua lly fo llow in g
a py oge nic in fec tio n , in w hic h he m aturia a lon e or assoc iate d w ith ede m a , hy pe rten sio n , azo tem ia and
pro tein uria are
presen t fo r a sh ort
per iod of t im e, the n gra du ally sub sid e o r sub seq ue n tly progress
i nt o c hr on ic g lo m er ul on ep hr it is .
T A B L E I
N E W C A S E S
O F N E P H R O SIS A N D
N E P H R IT IS A D M IT T E D T O C H IL D R E N S A N D
IN FA NT S H OS P IT AL S 1926-1946
1 92 6-1 938 193 9-1 94 8 T ota l
L ip oid n eph ros is
N eph ro t ic stage of g lom eru lon eph ritis
52
11
10 9
36
16 1
47
T o ta l nephro tic syndro m e
63
14 5
20 8
A c ut e g lo m e ru lo ne ph ri ti s
C h ro ni c g lo m e ru lo ne ph ri ti s
52
24
13 1
34
18 3
58
T o ta l acu te and chron ic
76
16 5
24 1
T ota l cases adm itted
13 9
31 0
44 9
2.
C h ro ni c g lo m er ul on ep h ri ti s- a chron ic ren a l d isease fo llow in g e ith e r acu te g lom eru loneph ritis
or a
py og enic in fec tio n o r de velop ing in sid ious ly , m anifes ted
b y p ro te inuria , hem atu ria on occas ion
and u ltim ate ly b y azo tem ia and e lev ated b lood pressu re . E dem a m ay or m ay no t b e presen t, b u t is no t
the o u tstand ing sym ptom exc ep t in tho se cases w hich a re clas sif ied as th e
n ep hro tic sy ndrom e.
3. N ep hro tic synd ro m e (se e first pa rag rap h)-
a. C h ron ic g lom eru lon ep hritis in the neph ro tic s ta ge -A fo rm of the ne phro tic synd rom e w ithou t
k no w n an tece de n t h is to ry of a cu te g lo m e ru lone ph ritis* or pyo ge nic in fec tion , w ith pe rsisten t pro -
te inu ria and ede m a as the pre sen tin g sign s, a nd w ith o the r ev ide nce s o f chron ic g lo m e ru lo ne phr itis
in itia lly ob scu red , b u t u ltim ately be com in g m a nifest.
b.
L ipo id
nephros is -A fo rm of th e n eph ro t ic syn dro m e m a nife ste d by ed em a, pro teinur ia , lip em ia,
es sen tia l ly n orm al b lood p res sure a nd li ttle , if any , h em atu ria or n itro gen rete n tion at an y tim e.
In som e cases the tru e na tu re o f th e d isease pro cess becom es apparen t on ly a t au topsy ; in o th ers ,
b y lab orato ry and clin ic al ev id en ce de velo p ing d uring the co urse of th e d isease . F or the purposes of
a nalysis, patien ts w ho sho w e d glom eru lar sca rrin g at au to psy w e re class ifie d as in stan ces of the
n ep hro tic stage of chro n ic g lom eru lo nephritis regard less o f the c lin ica l d iagnos is. A lso fo r th e
pur -
p ose s o f a nalysis , p atien ts w h o a re n ow pu rsu ing norm a l ac tiv i ty , b u t w ho h ave pe rsis ten t alb um i-
nu ria , h ype rte nsion o r a zo tem ia, h av e be en class ifie d (as lipo id n eph ros is or the ne phro tic stage of
ch ron ic g lom eru lo nep hri tis) . ac cord in g to the d iag nos is
w hich
w as app rop ria te d uring the e dem atous
p hase o f the d isease .
* S inc e th is stud y w as in itia ted o rig ina lly in an atte m p t to find ou t ho w to differen t ia te lipo id
ne ph rosis fro m c hro n ic g lom eru lon eph rit is , cases o f th e
nephro tic synd rom e fo llow ing acu te
glo m e ru lo ne phr itis w ere arb it rari ly exc lud ed , s inc e in th em th e d istinction se em ed cle ar. H ow eve r,
o cca sio nal c ase s o f acu te g lo m eru lo nep hr itis m ay p rog res s in to the n ep hro tic synd rom e.
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N EPH R OSI S
-
A C U T E N E P H R IT IS
C H R O N IC N E P H R ITIS
{149 }.4 N E PH R O T IC S T A G E O F
C HR ON IC G LO ME RU LO NE PH RIT IS
6 1 C A S E S
83
C A S E S
58 C A S E S
47 CASES
I
I
a
.4 .)
0
0
z
I 2 3 4 5 6 7 9
IC II a
488 L . A. BARNESS, G . H . M O L L A N D C . A . JA N E W A Y
R E S U L T S
Incidence
In th e
23
year pe rio d , from 1 926 to
194 8 , 20 8 ch ild ren
w ho en te red th e C h ild ren s
an d In fan ts H o sp ita l w ere d iag nosed as hav ing th e nephro tic sy ndrom e (T ab le I) . O f
these, 148 have been fo llow ed , 29 have re tu rned q uestion na ire s an d
31
cou ld no t be
reached fo r fo llow -u p . O ne hun dred six ty -o ne o f the 20 8 pa tien ts fu lf ill the c rite r ia fo r
a d iagno sis o f lip o id n ep hrosis an d 47 fo r the n ephro tic stage o f ch ron ic g lo m eru lo -
n ep hritis . T h e reco rd s o f 58 p a tien ts w ith chron ic g lom eru lo nephritis and of 183 w ith
acu te g lo m eru loneph ritis w ho w ere adm itted to th is hosp ita l d u ring th e sam e pe riod w ere
u sed fo r sta tis tica l con tro ls .
Y E A R S
C H A R T 1 . A ge o f on set of d iffere n t fo rm s of rena l d ise ase in c h ildho od .
A ge of O n se t
In lipo id n ep hros is , the ag e of onse t, a s show n in C hart 1 , averages 3 .5 years , w h ile
the ave rage age o f o nse t fo r th e neph ro tic stag e o f- ch ro n ic g lom eru lon ep hritis is 4 .6
years in th is se ries. T he average age of o nse t in the co n tro l se ries o f ch ild ren w ith chron ic
g lom eru lo nephritis is 7 .6 years, and 6 .3 years in acu te g lom eru lon ephritis .*
S ym ptom ato log y an d C ou rse
in itia l sy rn pto rn s.-T he onset in a lm ost a ll in stances o f lipo id neph ro sis an d the
* In l ipo id ne ph ros is th e ave rag e a ge of onset is 3 .5 yr. w ith a stand ard de v ia tio n o f 2. 1 yr . ;
in the nephrotic stage of c hro n ic g lom eru lon eph ritis , th e av erag e is 4 .6 yr. 3 .0 yr . S in ce th e
s tan dard error of the d ifferen ce of the se 2 m e ans is 0 .4 73 , an d th e d iffe ren ce of the 2 m e ans is 1.1 yr.
or
2.3 times
th e s tand ard e rro r, the d ifferen ce in ag e o f on set of ne phrosis a nd the nep hro tic s tage
of o m e ru lone ph ritis is sig n ifican t.
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*
A v era ge no . o f rem issions p er pa tie n t.
N E PH R O T IC S Y N D R O M E 489
T A B L E II
O N SE T O F D IS EA S E-F IR ST S Y M P T O M N O TE D
S y m p tom L ip oid N eph ro sis
N ep hro tic S tage o f C hro n ic
Glomeru lonephr i t is
Total
L iv in g D ead
L o st T o tal
%
L iv ing D ead L os t T o tal %
U pp er res pira to ry
infect ion 2 2 14 4 40 2 5 4 7 1 12 2 5 52
E d e m a o f ev es 4 4 14 9
6 7 42 6 5 3 14 3 0 81
%Vt.g ain 12 14 11 37 23 6 8 0
14 30 51
Pallor 1
0 1 2 1 1 0 1 2 4 4
G ene ral ized ra sh 3 1 1 5 3 1 0 0
1 2 6
Enuresis 0 1 0 1 1 0 0 0 0 0 1
Diarrhea
0 0 2 1
0 0 0 0 0 2
Other
6 1 0 7
4 2 2 0 4 9 11
Total 90 45 26 161
20 2 2
20 8
nephro tic s tage of nephr itis w as ins id ious . T he firs t sym ptom s no ted w ere e ith e r edem a
around th e eyes (81 cases), rap id inc rease in w eig h t (51 cases) o r an up per resp ira to ry
in fec tion tw o or th ree day s befo re th e appearance of ed em a (52 cases ) . A b reak d o w n
of fir st sym pto m s reported is show n in T ab le II. T h e figures ind ica te tha t n o s tatis tica lly
sig n ifican t d if fere nce ex ists b etw ee n
the frequ en cy of f irs t sym ptom s in lip o id neph ro sis
and in the nephro tic stage of chron ic g lo m eru lonephritis.
T A B L E III
S P O N T A N E O U S R EM IS S IO N S
(R em is sion s n o t asso cia ted w ith ob vio us in fec tion s)
.
Nephrosis
N ep hro tic S tage of C hro n ic
.
Glomeru lonephr i t is
L iv ing D e ad T o tal L iv in g D ea d T ota l
T o t a l
T ota l patien ts 90 45 1 35
20 2 2 5 2 17 7
P a tie n ts w ith re m issions :
1 . W ith in 2 m o. of on set 5 2 22 7 4 (5 5% ) 1 4 ii 2 5 (60 % ) 9 9(56% )
2 . M ore th an 2 m o. af ter
onset
4 6 11 5 7(42% ) 4 7 11(26% )
6 8 ( 3 8 %)
T o tal pa tien ts h av ing at leas t
1 sp on t. rem ission
6 4 2 6 9 0 ( 67% ) 14 1 3 27 (64 % ) 1 17 (6 6% )
P atien ts hav ing m ore th an 1
spo nt. rem is sio n
3 8 9 4 7 (35% ) 6 5 1 1 (2 6% )
58 (33 % )
T o tal n um ber of spo nt. re-
missions
1 25 44 1 69(1.3)* 20 21 4 (1.0)*
2 10 ( 1. 2) *
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49 0 L . A . B A R N E S S , G . H . M O L L A N D C . A . JA N E W A Y
Se x
inc idence .-N ine ty -n in e o f the 161 ch ild ren w ith lipo id nep hros is w ere b oys and
62 w ere g irls . T w en ty -n in e o f the 4 7 ch ild ren w ith the neph ro tic s tag e of ch ron ic
g lom eru lo nephritis w ere boys and 18 w ere g irls. T here w as , thus , a som ew h at h igh er in -
c idence of these rena l d iseases am o ng m ales .
R em iss io ns and exacerba tions .-A ll
cases
of the neph ro tic syn drom e h ad edem a. S o-
called sp on taneo us rem ission s are com m on w ith in tw o
m onths of the onse t. A s ind ica ted in
T ab le III , 7 4 of 1 35 ch ild ren w ith lipo id neph ros is and 25 of 42 ch ild ren w ith th e
neph ro tic stage o f ch ron ic g lo m eru loneph ritis d em on stra ted th is type o f rem ission .
T A B L E
IV A)
R E S P O NS E T O IN F E C T IO N
Nephro t ic
.
Nephrosis
Stageof
.
Chronic
.
I
otal
Nephr i t is
T otal pa tien ts 16 1 4 7 2 08
P atien ts w ith no infe ctio ns reco rde d
45 1 3
58
T otal pa tien ts w ith in fect ion s rec ord ed
11 6 34
15 0
T otal no . in fec tions 2 98
85 38 3
N o. No . % N o.
In fe ctions fo llow ed by re m is sio ns*
75
2 5 18 2 1 9 3
In fec tio ns fo llow ed b y ex ace rba tio n* 14 6 4 9 4 4 52
19 0
Infe ctions fo llow ed by n o cha ng e
77
26 2 3 27 100
*
R em ission o r ex ace rba tion w ith in
1 5 days of onse t.
A s the d isease p rogresses , spo n taneous rem iss ions app aren tly occur less f requen tly fo r
any ind iv idua l p a tien t. T h is is no t in d icated by T ab le III , h ow ev e r, w h ich sho w s on ly
tha t the rem ission s no t rela ted to an y app aren t in fec tion occurred a fte r tw o m onth s in
68 ins tances am ong
17 7
fo llow ed cases .
R espon se to in fec tion .-A ny rem iss ion occurr ing w ith in 1 5 days of an in fec tion w as
cons id e red a resp onse to in fec tion , sin ce , in a se ries o f 19 pa tien ts w ho had in fec tion s
w hile observed in
th e
hosp ita l, 18 ex perienced a d iu res is be tw een
1
an d 1 5 days fo llow in g
in fec t ion .
R em iss io ns, ex acerba tions o r no change in th e pa tien ts s ta tu s m ay occur lo llow ing
in fec tio n , and no m arked d iffe rence in th is respec t ex ists b etw een the tw o group s of
p a tien ts (T ab le IV (A )
)
. O f 1 61 ch ild ren w ith n ep hrosis , th ere w ere 2 98 instances o f
k now n infec tions .
S even ty -five (25 % ) of these in fec tion s w ere fo llow ed by rem iss io ns w ith in 1 5 d ay s,
146 (49% ) by ex acerba tions and 77 (26% ) by no chan ge in sta tus o f the p atien t.
In th e 47 ch ild ren w ith th e nephro tic s tage of g lom eru lonephritis, the re w ere 85 k now n
in fec tion s ; 21 w ere fo llow ed by rem ission s, 52% by exacerba tions and 27 % by no
change .
In T ab le IV (B ), th e resp onse to d iffe ren t typ es of in fection is co n trasted . It is q u ite
appa ren t tha t up pe r resp ira to ry in fectio ns are se ldom fo llow ed by rem is sions an d usu ally
by exacerba tions , w hereas the ra te o f rem iss io n is rough ly 4 0% afte r m ost o the r typ es
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N E P H R O T IC S Y N D R O M E 49 1
of in fec tion . A g a in th ere is no sign if ican t d iffe rence in response be tw een the tw o fo rm s
of the nephro tic syndrom e. It is in te res tin g to specu la te w he th er fever, w h ich is se ldom
h ig h in up pe r re sp irato ry in fec tions, accoun ts fo r the m ore favorab le respon se to o the r
typ es o f in fec tion .
R ecov ery fro m lip o id neph ro sis in th is se rie s o f pa tien ts occu rred a fter a s few as one
ep iso de of edem a or af te r as m any as 1 6 exace rba tions . R ecov e ry occurred in seve ra l
ch ild ren even th oug h the re w ere no rem issio ns fro m one ep iso de of ed em a fo r a s lo ng as
12 m on ths, th oug h m ore frequen tly rem is sions o ccu rred a fter one to tw o m onth s o f the
ed em atou s p hase .
T A B L E IV (B )
R E L A T IO N O F R E S P O N S E T O T Y P E O F IN FE CT IO N
T y pe of
In fec t ion
L ip oid N eph ros is
N e phro tic S tage o f C h ron ic N ep hrit is
R em is- E xacer - N o
sio n ba tion C ha nge
% R e-
missions
R em is- E x acer- N o
s ion b ation C h ang e
% R e-
missions
U pp er resp ira to ry in-
fect ion
P n e u m o c o c c u s
Streptococcus*
O ther
16 96 4 9
2 3 23 10
8 6 3
28 21 1 5
10
41
47
44
2 34 6
3 6 3
5 2 4
8 2 10
5
25
45
40
* A lm ost a ll
i3
S tr . h e m ol yt ic us .
D eath occurred a fte r 1 to 1 4 exacerba tio ns. T he ed em atou s p hase w ithou t rem ission
pe rs is ted fro m
1
to 1 5 m on ths p rio r to dea th . A ppa ren tly , the re is no corre la tion be tw een
th e n um ber and du ra tion of exacerba tio ns and u ltim ate ou tcom e.
L abo ra to ry F in d ing s
T he inc idence o f h ype rten sion , azo tem ia , and hem a tu ria is in d ica ted in C ha rt 2 . H yper-
tension w as co nsid ered to ex ist w h en the b lood pressu re de term in ed w ith a cu ff o f
appropria te size w as ab ove th e b lo od pres su re o f s tan da rd p re ssu re -ag e grap hs
;
azo t em i a
w hen the N P N w as ove r 40 m g./1 00 cc . ; and h em atu ria w h en m ore than on e red
b lo od ce ll pe r h igh pow er fie ld w as fou nd in spun u rina ry sed im en t. B ecause n ep hro tic
ch ild ren w ith in fec tions m ay tem porarily show hem a tu ria , h yp ertension or eleva tio n of
b lo od N P N , these sig ns w ere c la ssif ied as pe rs is ten t on ly if th ey w ere p re sen t fo r a pe rio d
of on e m o n th or lo nge r .
It w ill be seen th at in d iffe ren tia tin g b etw een lip o id nephrosis an d the n ep hro tic stage
o f ch ron ic g lom eru lon ep hritis , pe rsis ten t hy pe rtens ion is ev idence ag ain st neph ro sis . O n ly
one of the 1 61 ch ild ren w ith lipo id nephrosis had hyp ertension fo r lo nge r th an o ne
m on th w hile seen a t th is h osp ita l, 3 3 had tran sien t hy pe rtens ion an d 1 27 had n o hyp er-
tension w hile un der ob serva tion . O f th e 47 ch ild ren w ith the n ep hro tic s tag e of chro n ic
g lo m eru lo neph ritis , no rm a l b lood p re ssu re w as fo und in
14 , trans ien t hypertens io n in 12
an d pe rsis ten t hy pe rtensio n in
21 .
A zo tem ia w as trans ien t in
28 nephro tics, pe rsis ted lo nger than one m onth in 1 3 and
w as absen t in 1 20 , w hereas it w as trans ien t in n ine n ep hro tics in the nephro tic s tage ,
pe rs is ten t in 2 2 and absen t in 16 .
P ersis ten t hem a tu ria w as foun d in 41 ch ild ren w ith lipo id n ephrosis , transien t hem a-
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49 2 L .
A . B A R N E S S , G . H . M O L L A N D C . A . JA N E W A Y
tuna in 43 an d no hem atu ria in
77 .
G ross h em atu r ia w as no ted in
f ive
of these pa tien ts
w h o are liv in g , o ne of w hom is w ell an d fou r o f w ho m now have a lbu m inu ria as th e
so le abno rm al f ind ing ; in fo ur w h o d ied , and in fo ur w ho have been lo st f rom o ur
reco rds . T w en ty -fo u r ch ild ren in the neph ro tic stage o f g lo m eru lo neph ritis had p er-
s is ten t hem atu ria , 1 0 had transien t h#{2 35}m atu ria and in 1 3 no hem atu ria w as no ted . G ross
h em a tur ia w as no ted in on e c h ild w ho is liv in g , in tw o w h o are no long er in ou r record s
and in four w ho have d ied . T hus hem atu ria has been th e leas t h elp fu l sign in d if feren tia t-
ing be tw een these tw o d iseases, thoug h g ross h em atu r ia is ra re in lipo id neph rosis . T h is is
SIG N IF IC AN C E O F S IG N S
N E PH R O SIS (LE F T C O LU M N S ) 161 C A SE S ( 4 4 L w .45 A LB U M IN U R IC , I H Y PE R TE N SIO N
4 5D E 4 0 2 6L O S T )
10 N E PH R IT IS (R IG H T C O LU M N S) 47 C A SE S (2O L IV IN G , 2 2 D E A D 5 LO ST )
80 H YP E R T E N S IO N N IT R ET E N T IO N M IC H E M A TU R IA
5% 46
50 z
1
26 20 21Z
C
0 T R A N SI EN T
(
M O N T H )
C H A R T 2. D iag no stic sig n ifican ce of m ajo r sig ns of re na l d isea se .
at variance w ith the experience of m os t o th e r studen ts o f the d isease and m ay b e cx -
p lained in p ar t b y ou r a rb itra ry exc lusion of cases b eg inn ing as acu te g lom eru lon ep hritis
from the group of cases o f chron ic g lo m eru lonephr itis in th e nephro tic s tag e .
Serum cho les tero l va ried from 17 0 to 1 ,3 00 m g ./1 00 c c. in lip o id n eph ros is w ith m ore
than 275 m g./100 cc . in 79 ou t o f 8 8 ca ses in w hic h the ch ole ste ro l w as repor ted .
C hole ste ro l in
19 cases o f g lom eru loneph ritis in the n ephro tic s tage varied from 204 to
700 m g./100 cc ., w ith m ore than 2 75 m g ./10 0 cc . in 16 of these 1 9 . In severa l ch ild ren
of b o th groups , cho les tero l w as be low 27 5 m g./10 0 cc . w h en firs t m easu red , bu t rose
abo ve th is leve l one to th ree m onths afte r the onset o f the d isease.
V a lues fo r the serum pro te in in the ch ild ren w ith lipo id nephro sis ran ged fro m 2 .1 to
7 .4 gm ./100 cc . and w ere less than 4 .5 gm ./1 00 cc . in 119 of 153 pa tien ts in w hom
m easurem en ts w ere m ade . In ch ild ren in the nephro tic s tage of chron ic nephritis, th e
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N E P H R O T IC S Y N D R O M E 4 93
se ru m pro te in ranged from 3.5 to 7 .2 gm ./1 00 cc . w ith 23 of 44 pa tien ts show ing leve ls
be low 4 .5 gm ./1 00 cc . O f 19 7 pa tien ts in w hom serum pro te in leve ls w ere rep orted ,
edem a w as p resen t in 18 6 w hen the se rum p ro te in w as be lo w 4 .5 g m ./100 cc ., and w as
absen t w hen abo ve th is leve l. H ow ev er , in n ine nephro tic s and seven n ep hritic s edem a
wa s presen t a t lev els o f 5 to 6 gm ./100 cc . an d in th ree nephro tics an d th ree nephr itic s
edem a w as absen t at leve ls o f 3 to 4 gm ./1 00 cc.
P rev ious H is to ry
T he re w as no constan t f ind in g of an y prev iou s d isease in e ithe r g ro up of ch ild ren .
A p pro x im a te ly on e ha lf o f each group o f ch ild ren had b een breast fed and the rem ain de r
artific ia lly fed . A b ou t th ree fourths had rece ived adequa te supp lem en ts o f v itam ins A
and D , and approx im ate ly o ne fou rth had received none . T w en ty o f th e ch ild ren are
rep orted to h av e had frequen t co lds. F ifteen had h ad tons illec tom ies befo re th e onse t
o f neph rosis . lit genera l, h ow ev er , ex cep t fo r the usua l ch ild hood d iseases, th e ch ild ren
h ad b een in g ood h ea lth p rio r to the onse t o f nephrosis o r ch ron ic g lom eru loneph ritis .
F am ily H isto ry
A n a ttem pt w as m ad e to find a com m on h ered ita ry back groun d in these ch ild ren .
A fam ily h isto ry o f a llergy w as fo und in 43 of the 161 nephro tic s , and in 1 6 o f th e
4 7 c hro n ic g lo m e ru lone ph ritic s in the neph ro tic stage . T he s ign if ican ce o f these f igu re s
is
d ifficu lt to de term in e due to the vary in g es tim ates o f a lle rg y in the genera l p opu la tio n .
A
fam ily h is to ry o f ren al d isea se w as foun d in
23 pa tien ts, o f tubercu los is in 2 0 a nd of
d iabe tes in 23 of the 20 6 pa tien ts.
T rea tmen t
T ab le V lists 64 m etho ds of trea tm en t w hich have been u sed fo r the 2 08 pa tien ts w hile
u nder ob serva tion in the h osp ita l. T he large num b er o f m etho ds of trea tm en t ind ica tes
tha t few have been g ood en ough to w iths tan d the test o f tim e ; u ndoub ted ly m any h av e
no t b een ev a lua ted ca re fu lly .
D iet-S even varia tions in d ie t have been used , b u t none has proved therapeu tically
strik in g . W e now favo r a h igh p ro te in low -sa lt d iet w ith less th an 0 .5 gm . sod ium
ch lo ride pe r day , bu t th is is im poss ib le to ach ieve , u n le ss th e ch ild w ill d r ink salt- free
m ilk .* M ixed v itam ins a re g iven em pir ica lly d uring the edem a tou s p hase .
R em ova l o f focus o f in fec tion -F our teen of th e 20 8 edem atous ch ild ren h ad o titis
m ed ia requ ir in g m yrin go tom y. E lev en had ce llu litis req u iring dra inage . T w o had car io us
te eth ex trac ted . O n e m asto idec tom y , o ne rib re sectio n fo r em py em a , o ne an tro tom y fo r
sinus itis , and one p e lv ic abscess d ra in ag e w ere p erfo rm ed . S ev en teen tons illec tom ies and
adeno idec tom ies w ere carr ied ou t. T h is has no t been a rou tine pro cedu re and has been
p erfo rm ed on ly w hen the cu stom ary ind ica tions ex ist. W hen a ton sillec tom y is necessary ,
an a ttem p t is m ad e to perfo rm the opera tion w hen ed em a is m in im al. T he anesthes ia de-
p artm en t o f th is hosp ita l recom m end s cyc loprop an e as th e anesthe tic o f cho ice in th e
presen ce of rena l dam age .
Diure t ic s -No
d iu re tic has been fou nd cons isten t in ac tion . M ercuria l d iu re tics have
*
L on alac (sod iu m -f ree m ilk) (M ead Joh nso n) .
It
ha s prove d u sefu l, if the ch ild can b e ind uce d
to su bs titu te it fo r ord in ary m ilk . W ith L ona lac , s alt -fre e bre ad and salt- fre e bu tter , a n in ta ke o f 0 .5
g m . so d iu m c hlo rid e per d ay can be ac h ie ved , w h ich is s till m o re sal t th an is ex creted b y m an y
nep hro tic ch i ld re n .
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494 L . A . B A R N E S S , G . H . M O L L A N D C . A . J A N E W A Y
T A B L E V
D R U G S A N D P R O C E D U R E S U S E D IN T R E A T M E N T O F N E P H R O S IS IN C H IL D R E N S
A N D IN F A N T S H O S P ITA L S , 1926-1948
B loo d and B lood Subs titu te s
H i g h
pro te in low sa lt B lood transfus io n
H igh pro tein P lasm a
L ow sa lt C o nce n tra ted serum a lbu m in , s alt -po or
Lo w
pro tein C o nce n trate d hu m a n seru m
L o w fa t Im m u ne g lob u lin
L im ited flu ids A c acia
Forced f lu id s A m in o ac ids
B o vin e seru m album in
R e m o v a l o f I nf ec ti on
. . In fec tio ns Indu ced
T on sillecto m y an d a den oid ectom .y
C arious teeth re m o ved V ac cin ia
M as to idec tom y M e asle s
R ib r es ec ti on
. .
C hem o the rap eutic D ru gs
P elv ic abscess d ra ined
A ntro to m y P en ici llin
M yringo tom y S ulfad iaz ine
C ellu litis d ra ined Su lfath iazo le
. .
Sulfapyr id ine
Diure t ic s Sulfan i lamide
Mercupur in
# {174 } S trep tom ycin
Salyrgan #{174}
. .
O t h e r D rugs an d Procedures
Diuret in #{174}
A m m onium chlo ride P yram idon
H y perton ic g lu cose P aracen tes is
H yperton ic sucrose T h yro id ex tract
Aminophyll in
# {174 } D ecapsu la tion of k idney
Theophyllne
#{ 174} A sco rb ic acid
T h e o b r o m i n e #{17 4} T es to ste rone p rop ion ate
U rea R utin
M a gn esiu m su lfa te V ita m in
A
an d M azo la o il
J ava tea C arb ohy drate frac tion o f p n eu m o co c cu s
P o tass ium c hlo rid e P a rathorm o ne
P otass ium n itra te L ive r, in tram uscu lar
P o tass ium c itra te H orse an t ise rum
C a lciu m ch lor ide U rina ry an tise p tics
Dig i ta l is
M ix ed vita m ins
C hol ine
Pyridoxine
S w e a t s
been used in six ch ild ren . A m m o nium ch lo rid e w as u sed in six ch ild ren and in n o in -
stance w as the re a d iu res is . H y pe rton ic g lucose and suc rose w ere used e igh t tim es w ith -
o u t no tab le e ffec t. A m in oph y llin , theop hy llin e an d theob ro m ine w ere used in five . M ag -
n esiu m su lfate w as g iven to
14 ch ild ren w ith ou t con sis ten tly dem o nstrab le e ffec t. Java
te a (K um is k uch ing , an he rb used ch ie f ly in the O rien t is a d iu re tic ) w as u sed in
th ree ch ild ren w ith equ ivoca l re su lts , a lthou gh it repea ted ly p ro duced a d iu re sis in o ne
ch ild over a p erio d of n ear ly a year a t hom e. P o tass ium sa lts w ere g iven to six an d ca lc iu m
sa lts to tw o c hild re n.
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N E P H R O T IC S Y N D R O M E 495
B lood , b lood d eriva tive s a nd b loo d subs titu te s.-F ifty -o ne of th e 2 08 ch ild ren w ere
g iven tran sfu sion s fo r anem ia and as sup portive th erapy during in fec tion s, be fo re the
era of chem otherap y . T h irty -on e ch ild ren in bo th gro ups rece ived no rm al h um an serum
albu m in in an a ttem pt to in itia te d iu resis and increase th e c ircu la ting se rum album in . S a lt-
poo r serum a lbu m in h as p rod uced a d iu re sis in app ro x im a te ly ha lf th e cases w itho u t
inc rea sing th e serum a lb um in leve l ap prec iab ly , a s repo rted els ew here.8 S ix ch ild ren w ere
g iven h um an seru m , four w ere g iven p lasm a and six w ere g iven im m une serum glob u lin ;
th ree rece iv ed am in o ac ids , o ne acac ia an d one b ov ine a lb um in . T h e effec ts o f these sub -
s tan ces w ere v ar iab le .
in fec tk rn s ind uced .-B ecau se spon tan eous d iu re sis frequen tly fo llow s in fec tion , espe -
c ially m eas les , 17 ch ild ren w ere inocu la ted w ith m eas les v irus . Success fu l inocu la tion has
been ach iev ed by in tran asal in stilla tion of ap prox im ate ly 1 to 2 cc . o f th roa t w ash ing s
ob ta ined from ind iv id ua ls w ith K oplik s spo ts in th e oropharyn x . T hese are co llec ted by
hav ing th e p atien t ga rg le and rinse th e th roa t w ith bac te rio log ic b ro th . T he w ash ings are
frozen a t -
70 #{1 76} C . n til ready fo r use, th en thaw ed at 3 7#{ 176 }C ., nd 100,000
u . each o f
pen ic illin and strep tom y cin a re add ed to
2 cc . be fo re inocu la tion .
T h is m e tho d of inocu la tio n w as used in
12
ch ild ren w ith neph ro sis an d in five in th e
nep hro tic s tag e o f ch ron ic g lom eru lo nephritis . F ourteen ch ild ren dev elo ped a ty p ical
m acu la r e rup tio n 7 to
17
days a fte r in ocu la tion an d th ree fa iled to d ev elo p m eas les in
sp ite o f repea ted in ocu lation s. T he ch ild ren have been g iv en chem otherapy to prev en t
secondary bac teria l in fec tions th ro ughou t the ir a ttack of ac tive m eas les .
O f th e 14 ch ild ren w ho deve lop ed m eas les , 1 1 experienced a lm ost com p le te d isap pear-
ance of edem a an d a lbum inuria 1 to 1 2 d ays af te r onse t o f the rash . T hree ch ild ren , a ll
ch ro n ic n ep hritics in the n ep hro tic stage , h ad no d iu re sis ; tw o of the ch ron ic n ep hritic s
in the neph ro tic stag e re spon ded w ith a d iu re sis sim ila rly to the n in e ch ild ren w ith
neph ros is . T hree of these 1 1
ch ild ren h ave rem ain ed essen tia lly edem a free fo r tw o
y ears . In th e rem ain ing eig h t, p ro te inuria and edem a hav e recurred from o ne m o nth to
one yea r a f ter th e d iu re sis .8
{ 149 }
Paracen te s i s . -Seven ty - four of the 2 08 ch ild ren req u ired abdo m ina l pa racen te sis . O f
these p aracen te sis w as p e rfo rm ed m o re than on e tim e in 43 . It ha s been n o ted tha t
parace n te sis is o fte n m ore ef fec tive
la te in the d isea se . O ne possib le ex p lana tion is tha t
ea r ly in th e d isease th e ab dom ina l w a ll is th ick and ed em a tous, and the ed em a flu id is
sp read over the bo dy . L a te r in th e d isease , the abd om ina l w all is th in , edem a flu id co l-
lec ts m o re eas ily in the abdom ina l cav ity th an in o ther parts o f th e bo dy , and conse-
quen tly is m ore read iiy relea sed by pa racen te sis .
O ther dru gs an d procedures.-D es icca ted
th y ro id g lan d w as g iven in the ho sp ita l to
11
ch i ld ren ; severa l o the rs rece iv ed it a t hom e. E ven though these ch ild ren app aren tly to lerate
thyro id g land adm in is tra tion w ell, its u se has b een abando ned because of lack of bene-
f ic ial re su lts . D ig italis w as u sed in fiv e ch ild ren w itho u t ap pa ren t effec t. T he o th er m eas-
u re s lis ted in th e la st sectio n of T ab le V have been tr ied on ly o ne tim e and , because no
strik ing im p rovem ent w as no ted , w ere d iscon tinu ed . T he use of chem otherapeu tic d rug s
is d iscussed b elow .
C ause o f D eath
T he causes o f death , w hen th e ch ild d ied in the h osp ital, a re sho w n in C h art 3 . In the
p resen t stud y of 161 pa tien ts d iag nosed lip o id nephrosis , 13 6 have been fo llo w ed an d ,
o f the se , 4 5 pa tien ts
( 3 2 %) have d ied . S ev en teen e ithe r d ied a t hom e, o r the cause
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49 6
L . A . B A R N E S S , G . H . M O L L A N D C . A . JA N E W A Y
C A U S E S O F D E A T H
IN N E P H R O T IC S Y N D R O M E
N E PH R O S IS N E PH R O T IC S T A G E O F
C HR ON IC G LO ME RU LO NE PH RIT IS
29
C A S E S 1 9 C A S ES
7 m T n h l _
AC C I D EN T S
5,,
. I O F T H ER A PY
O -C AR D IA C F A ILU R E
-3
R E N A L
._-
63 7
FAILURE
83
f- IN F E C T IO N -4
27 Z
C H A R T 3 . C om pa rative inc ide nce of d if fere n t ca use s o f d ea th in 2 form s o f ne phro tic synd rom e.
N o te pre do m in an t ro le of in fect ion in lip o id ne phrosis.
of d ea th w as no t asce rta ined . O f th e rem ain ing
28
pa t ien ts ,
23
d ied o f in fec tion , th ree
of ca rd iac decom p en sa tion , o ne of anu ria fo llo w ing su lfapyrid ine and one fo llow in g to nsil-
lectomy.
T w enty -tw o of the ch ild ren in th e neph ro tic stage of chron ic g lo m eru lonephritis a re
kno w n to have d ied . T he cause of dea th has been ascerta ined in
1 9 . O f th ese , five d ie d
of in fec tion , o ne of ca rd iac deco m pensation , o ne of pu lm onary edem a fo llow in g th e
ad m in istra tion of alb um in a nd 1 2 in urem ia.
T he causes o f death acco rd ing to ty pe of in fec tion am o ng the p a tien ts w ith lipo id
nep hro sis a re sh ow n in C har t 4 . P ne um oco cce m ia or pn eum oc occ al p eriton itis w as p resen t
in 16 , w h ile the s trep tococcus w as im plica ted in tw o an d E . co li in tw o of th e 23 cases
in w h ich infect ion w a s presen t a t the tim e of dea th . In th ree cases , the o rgan ism s w ere
n o t iden tif ied . It is seen tha t since the adv en t o f the libe ra l u se o f su lfad iaz ine in
1 9 4 2 ,
few dea ths h av e b een accom p an ied b y in fec tion w hen the pa tien t Jied in the ho sp ita l.
O f th e f ive h osp ita l death s in ch ild ren w ith n ep hro sis since 19 42 , tw o w ere circu lato ry
d eaths, tw o fo llow ed in fec tion and o ne dea th fo llo w ed tons illec tom y .
O ne po ssib le exp lan a tio n fo r recen t inc rea se in the num ber o f dea ths at hom e m ay
be th e inc rea se in th e n um be r of p atien ts w ho h ave com e fro m c ons ide rab le d istan ce s.
T his is show n in T ab le V I w hich a lso ind ica tes the h ig her m or ta lity rate bo th befo re and
af ter 194 2 a m o ng c hild ren liv ing a d ista nce a nd he nc e presu m a bly h av ing les s read y
access to p rom pt m ed ica l and ho sp ita l ca re .
P rognos is
T h e prog nos is o f these d iseases is b ased on the da ta from ch ild ren fo llow ed to da te .
T he sta tus o f th ese pa tien ts is b rie fly sum m arized in T ab les V II and V III. T he d a ta
regard in g the pa tien ts w ith ac u te a nd ch ron ic g lom eru lon eph ritis in T ab le V II are
w eig h ted , as no spec ia l a ttem pt w as m ade to fo llow these pa tien ts a fte r d ischarge from
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24
.-.N E W C AS E S
..-
A SE L O A D
0
S U L F O N A M ID E S [PEN LL IN
I S A 1 S P 1S . T1 S D . j,
D EA T H S
E E l P N E U M O C O C C U S
D S T R E P T O C O C C U S
t i E S C H E R IC H I C O L I
0
O T H E R IN F E C T IO N
Q D IE D A T H O M E
. C I R C U L A T O R Y
0 A C C ID E N T O F T H E R A P Y
16 2 8
Y E A R
NEPHROTIC SYNDROME 49 7
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w
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2
N E W C A S ES O F N EP H R O S IS A N D D EA T H S IN N EP H R O S IS 1926-1948
C H A R T 4 . C auses o f de ath in 1 61 ca ses o f lip o id n ep hro sis w ith 4 5 d ea ths. N o te failu re o f m ortality
to ris e sin ce in tro du ctio n of su lfa d ia zin e in 19 42 , d esp ite stead ily in crea sin g case loa d . A lso n ote
tha t m os t de ath s in rec en t yea rs h ave oc curred a t h om e a nd tha t pn eum oc occ us w hich w as form er ly
re spo nsib le for m os t fa ta litie s has n o t c aused dea th since 19 42 .
the hosp ita l. T he cr ite ria used fo r cons id er in g a pa tien t w ell a re freedom from edem a for
on e year , no rm a l b lood pre ssu re , no rm a l se rum N P N , an d urine free o f p ro te in and
abnormal fo rm ed e lem en ts . T he ch ild ren have b een su bd iv ided in to th ree g roups fo r pur-
p oses o f ana lysis : those w ith on set be fo re 1 938 -th e prechem o th erap y era ; th ose w ith
onse t in the pe riod 193 9 to 19 42 w hen su lfonam ides w ere in troduced fo r the trea tm en t
o f in fec tio ns ; th ose w ith onse t a fte r 1942 , w hen su lfad iazine began to b e used fo r the
t reatment .
F . 3L E V I
R E L A TIO N O F D O M IC IL E O F P A T IE N T S T O M O R T A LIT Y IN 16 1 C A S E S O F L IP O ID N E P H R O S IS
Date of Onset 192 6 -1942
1943-1948
C ase s fro m T otal C ases
D e aths M orta lity
T o tal C a ses D ea ths M o rtal ity
GreaterBoston
49 (61 % ) 1 7 3 5%
27 (33 % )
3 11 %
E l se w h e r e
3 1 (39 % ) 15 48 % 5 4 (67 % ) 10 19 %
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49 8
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N E P H R O T IC S Y N D R O M E 49 9
O f 1 61 ch ild ren w ith lipo id n ephrosis , 4 5 (2 8% ) have d ied . In the p re su lfonam id e
p eriod 1 926 to 1938, the av e rage case load* w as 1 4 .6 p atien ts pe r y ea r and the re w ere
13 death s, a rela tive m o rta lity o f 6 .8 p atien ts/1 00 /y ear . In th e su lfonam id e pe riod
1939 to 194 2 , the ave rage case lo ad w as 39 pa tien ts pe r y ea r , w ith 1 6 dea ths, a re la tive
m orta lity o f 10 .3 pa tien ts /100 /year. In the su lfad iaz ine -pen ic illin p er io d 1 943 to 194 8 ,
th e ave rage case lo ad w as 64 w ith 1 5 d ea th s, a rela tive m orta lity o f 3 .9 /10 0 /yea r.
O ne hun dred sev en ch ild ren w ith lipo id nephros is have b een fo llow ed tw o o r m ore
yea rs : 4 5 (42 % ) have d ied ; 4 4 (41 % ) are liv ing and w e ll ; 18 ( 1 7% ) a re liv ing w ith
ev idence of rena l d isea se . O f these la tte r 1 8 , ho w ev er , 10 a re am on g th e cu rren t pa tien ts ,
and approx im ate ly o ne ha lf o f th ese m ay b e ex pected to recove r .
T A B L E V III
S T A T U S O F 62 L IV IN G P A T IE N T S W IT H L n oID N E P H R O S IS W H O H A V E B E E N
F O L L O W E D
2
O R M O R E Y E A R S
F ollo w ed fo r
T o tal N o .
Patients
N o. P atien ts w ith M a jor S ign s
_ _ _ _ _ _ _ _ _
P ro teinur ia E de m a
H yper-
tension
A z ote m ia H e m a tur ia
16-22yr .
9 3 0 2 0 0
11-lS yr. 9
0 0
1 0
0
6-lO y r. 1 2
0 0 0 0 0
4-5yr . 1 0 1 0 0 0 0
3yr. 1 0 6 3
0 0 0
2yr. 12 7 7 0 0 0
T w en ty -n in e ch ild ren in the neph ro tic stag e o f ch ron ic n ep hritis have been fo llo w ed
tw o or m ore years : 22 (7 6% ) are dead , an d sev en (24% ) a re liv in g.
T hus, it is seen th at the ov er-a ll p rogn osis in lipo id neph ro sis be fo re ch em othe rap y
w as favorab le in app ro x im a te ly 40 % o f th e cases . S ince the in trod uc tion of su lfad iaz ine
and the new er an tib io tic s , the m orta lity rate h as been red uced fu rth er (from 6 .8 to 3. 1
pat ien ts/100 /year)
.
P resum ab ly , in a long e r fo llow -u p , ac tua l m ortality w ill a lso be
sh ow n to be red uced . A lm ost a ll o f the neph ro tic ch ild ren w ho have d ied since 1942
h av e d ied a t ho m e . P e rhaps, had ce rta in supp ortive m easu res , su ch as a re av ailab le a t the
h osp ital, b een used , th ese pa tien ts m ig h t h av e to le ra ted w ha teve r cr is is cau sed the ir
death .
It rem a ins im p ossib le, h ow eve r, to g ive a favorab le p rog nosis in e ithe r ch ro n ic g lo m er-
*
C a se
load is the num b er of liv ing pa tien ts fo llow ed dur in g the year p lu s th e n um ber of new
p atien ts fo r th at yea r w ith e v id enc e of ac tiv e rena l d isease . T h irty -th ree ch ron ic nephritics have been
fo l low ed (76% m o rta lity ). O f 1 83 pat ien ts w ith acu te g lo m e ru lone ph ritis , 81 ha ve bee n fo l low ed
longer
than
2 y r. T he m ajo rity o f the 10 2
others
ar e
p r e su m e d
to be w ell as o n ly those w ith sub-
seq u en t
dif f icu l ty
are like ly to retu rn to the h osp ita l.
Fif ty - th ree
o f the 81
are k no w n
to be w e ll, 25
h ave b ec om e
ch ro n ic and
3 died
o f c ard ia c
d e c o m p e n sa1 on d uring
the acu te illne ss .
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500 L . A . B A R N E S S, G . H . M O L L A N D C . A . JA N E W A Y
ulone phr itis o r th e n eph ro t ic sy nd rom e of chron ic g lom eru lo nephritis, thou gh ch ild ren
w ith the la tter m ay live 10 yea rs
or longer w ith the ir d isease .
Duration of Di sease
In the se ries o f 62 ch ild ren w ho have been fo llow ed tw o or m ore years w ith recov ery ,
the co urse of nephro sis has pers isted from 6 to 96 m onths . In a ll bu t a few , ac tiv e d isease
las ted be tw een o ne an d th ree years (2 1 .2 years ) . O f th ose n ep hro tic s w ho d ied , d ea th
occurred be tw een 2 and 95 m o nths a fte r onset, b u t usua lly w ith in tw o years.
G row th and D ev elop m en t
G row th and deve lopm ent usua lly a re de lay ed during th e active ph ase of n ep hros is, bu t
fo llow ing reco very , the p atien t assum es h is norm al g row th channe l as p lo tted on s tand ard
h eigh t-w e igh t an th ropom etric charts. O n ly one pa tien t has show n m enta l re ta rda tion . T he
ch ild ren app aren tly do average sch oo l w o rk , and no a ttem p t has b een m ade to do ro u tine
psy ch om etric e xam ina tio ns.
D I S C U S S I O N
O u r ob serva tion s a re sim ila r to th ose repo rted by S chw arz ,1 M urphy ,2 H eym an ,3 and
B lock .4 T he m o rta lity am on g ou r pa tien ts a s w e ll as th e labo ra to ry and clin ica l find ings
are co m parab le to th ose recorded in the ir se ries.
Using
the c rite ria fo r d iffe ren tia l d iagnos is as ou tlined , the d iffe ren tiation be tw een
lipo id nephrosis and the neph ro tic stage of chron ic g lom eru loneph ritis has been d ifficu lt,
bu t po ss ib le. F our teen d iag noses am ong 208 cases have h ad to be changed , eithe r as a
resu l t
o f au top sy repor ts o r the ap pearan ce o f such sym ptom s and s ig ns as h em atu r ia ,
hy pe rten sion or n itro gen reten tio n . H o w eve r , m an y pa tien ts had to be fo llow ed fo r a
consid e rab le tim e , even up to one yea r, be fo re a d efin ite d istin ctio n co u ld be m ade .
G lom eru la r chang es h av e b een n o ted a t au topsy in 5 of 17 p atien ts in w h om a clin ic al
d iagn osis o f lipo id n ephrosis h ad been m ad e. H o w eve r , in a ll o f the se five , tub u la r
degenerat ion
predom ina ted , and in fou r o f the five a typ ica l signs an d sym p to m s h ad been
no ted at som e tim e. T h is is no t th e p lace to d iscuss w he ther lip o id nephrosis and the
nep hro tic stage of ch ron ic g lom eru lo nep hri tis are actual ly tw o d iffe ren t d isea ses or tw o
differen t var ian ts of the sam e fun dam en tal pa tho lo g ic p ro cess. It is c lea r tha t ce rta in
in stances of the nephro tic syndro m e w hich w e c lassify as lipo id n ep hros is h av e a re la -
t ively
go od pro gno sis , w h ile th ose in w h ich the fea tu re s o f ch ro n ic g lo m eru loneph ritis
predom ina te have a po or p ro gnos is. It is qu ite possib le tha t in ce rta in cases, g lom eru lo -
nephritis m ay be sup erim po sed o n an orig ina l lip o id neph ro sis , g iv ing a m ixed p ic tu re ,
just a s py elo nep hri tis o r v asc u la r sclero sis m a y com plicate o th er fo rm s o f re nal d isea se .
M o re ex ac t in fo rm a tion con ce rn ing the p hysio log ic and ana tom ic de ran gem en ts in the
n ep hro tic synd ro m e can co m e on ly from th e corre la tio n of ca re fu l stud ies o f rena l fun c-
tion by the n ew er m etho ds w ith the c lin ical course and u ltim ate au topsy f in d ings in those
pa tien ts w h o succum b to in te rcu rren t in fec tion o r to the d isease itse lf. S uch s tud ies a re
und er w ay in our o w n as w ell as in a num b er of o ther c lin ics.9 U nfo rtuna te ly , the ren al
fun ctio n te sts used in the past d id n o t y ie ld in fo rm atio n w hich cou ld a llo w o ne to de -
term ine w h eth er
som e cases o f so -ca lled lipo id n ephros is w ith c lin ica l o r apparen t
recov ery actu ally ended up w ith redu ced rena l func tion . Fo r th e purpo ses o f th is in itia l
fo llo w -up stud y , w e h av e h ad to re ly on ord ina ry c lin ica l ex am ina tion w ith rou tine un -
n alys is. T h e fac t th at, in the group o f n in e p atien ts fo llo w ed fo r 16 or m ore years, p ro -
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N E P H R O T IC S Y N D R O M E 5 01
te inun ia
has been found in th ree , an d hy perten sion in tw o , su ggests tha t c lin ica l recovery
from ne ph rosis in
ch ild hood d oes no t alw ays im ply com p le te res to ra tio n of the k idney to
norm al. T h is w ou ld be in line w ith R enn ie s observa tions .10
B eca use of the d ifficu lty in d is tin gu ish in g be tw een the tw o types of cases and becau se
lipo id neph ros is appears to be re la tive ly com m on in ch ildhoo d , a ll pa tien ts w ith edem a,
lipem ia , low serum p ro te in s and a lbum inun ia shou ld b e trea ted w ith a reasonab le degree
of op tim ism . T he d isea se w ill nun a ch ron ic cou rse w ith rem ission s and exace rba tions,
bu t u ltim ate recovery w ill occur in m any ch ild ren , pa rticu la rly if o ne can pro tec t th e
p a tien t fro m infec tion , w h ich h as been the m ajo r cau se o f dea th in the p as t. T h ose pa-
tien ts w ho deve lop frank ev iden ce of g lom eru la r invo lv em en t, how ever, u sua lly assu m e
the poor p ro gnos is o f ch ron ic nephritis.
O n e of ou r ch ie f pu rpo ses in m ak ing th is stud y has b een to p lace o ur o w n th erapeu tic
program for these pat ien ts on a soun de r fo o ting . T h e fo llow ing co m m ents rep resen t ou r
c urre n t v ie w s reg ard ing
the m anagem ent o f neph ros is based on th is study an d our p resen t
experience . It is c lea r tha t, a s in so m any chro n ic d iseases o f un know n etio logy , the
phy sic ian can d o little in th e w ay of trea tm en t w hich affec ts the rea l course of the
d isease. O n the o th er hand h is the rapeu tic p rog ram shou ld be a im ed a t p ro tec ting the
pa tien t so fa r as possib le fro m any spec ia l hazard s w h ich he ru ns an d a t m in im iz ing the
dam age caused b y th e d isease . S in ce the m ajo r cause o f d ea th in pa tien ts w ith lip o id
nephrosis is in fec tion , every effo rt shou ld be m ade to d im in ish the freq uency o f in fec tion
an d to trea t estab lish ed in fections prom ptly and tho rough ly . T he prev en tion of resp ira to ry
in fect ion s is
d ifficu lt, bu t can b e accom p lished to a cons ide rab le ex ten t by cu tting .d ow n
the con tac ts o f the p a tien t w ith p eop le ou ts ide the fam ily , p ar ticu la rly ch ild ren d uring
the w in te r m onths . T he con tin uous use o f su lfonam ides o r o f an tib io tic s in p rophy lac tic
doses d ese rves inv estiga tio n , bu t canno t be recom m end ed u n til its sa fe ty and effec tiv en ess
a re dem ons tra ted und er con tro lled cond itions . M o st im portan t is educa tio n of the paren ts
to p rom p t recogn ition of sym ptom s of in fec tion , pa rticu la rly fever, so tha t chem otherapy
m ay be ins titu ted pro m ptly , thus con tro lling bac te rem ia an d p reven tin g the d ev e lo pm ent
o f periton itis . In v iew o f the fac t tha t fa ta l in fec tions du e to E . co li have o ccurred in
severa l o f o ur pa tien ts , com b in ed pen ic illin and strep tom y cin therapy is p robab ly in d ica ted
in a ll severe in fec tions , a t leas t un til the organ ism is iden tified .
T h e secon d haza rd w hich th rea ten s th e nephro tic ch ild is the unw illing ness o f ph ysi-
c ians or p aren ts to accep t the fac t tha t the d isease is ap t to run its na tu ra l course reg ard -
l ess
of w ha t trea tm en t is adm in is te red . T he con sequen ce o f th is n atu ral reac tion to an y
ch ron ic d isease , p articu la rly one
w h ich so pro fo und ly affec ts th e ap pearance of th e pa-
tien t as nephros is, is a co n tin uous an d v alian t s tru gg le to e lim in ate th e edem a. T h is
resu lts in p ro lo nged ho sp ita liza tion , w hich subm its the p a tien t to repea ted expo sures to
in fec tiou s agen ts , and leads to cons id erab le unnecessa ry em otiona l traum a for b o th ch ild
and paren ts. In add iE in i hes# {233 } sub tle bu t im po rtan t d rfrnen ta l e ffec ts the pa tien t is
p lied w ith d iu retic s, w ith in travenous in fu sions of a ll so rts , and , if any foc i o f in fec tion
a re foun d , th ese are rem oved . T he d iu re tic s , ce r tain ly the m ercun ials , m ay in them se lv es
d a m a g e
the k id ney , in traven ous in fusion s of concen tra ted p lasm a or even serum album in
m ay b e ineffec tive or m ay prec ip ita te pu lm onary edem a if la rge doses a re g iv en to a
ch ild w ith hyp er tens ion , and th e rem ov a l o f fo c i, su ch as the tons ils an d adeno ids , m ay
be dange rous du rin g th e ed em ato us p hase . W e b elieve
inkeep ingpat ien ts
a t h o m eo r in a
she lte red en v ironm en t a s m uch as p ossib le.
T h ere is nok now n reason w h y a ch ild w ith neph rosis shou ld no t b e am b ula to ry as
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50 2 L . A . B A R N E S S , G . H . M O L L A N D C . A . JA N E W A Y
long as the edem a pe rm its activ ity . T he d iet sh ou ld be as lo w in so d ium as po ssib le ,
p rov id ed an adequa te ca lo r ic in tak e , w ith a con side rab le am oun t o f p ro te in , can b e m a in -
tam ed . P anacen te sis rem a ins th e s im ple st m e tho d o f rem ov ing ex trace llu la r flu id in th ose
ch ild ren w h o have co nsiderab le asc ite s, and in w hom the accu m ulatio rt o f flu id becom es
so g rea t a s to em barra ss ea ting , b rea th in g o r ge tting ab ou t.
Better
m eth ods o f trea tm en t w ill hav e to aw ait a c leane r u nde rstand in g o f the n atu re
o f the d isea se p ro cess. S erum a lbum in m ay b e use fu l in re liev ing edem a in som e pa tien ts ,
b u t, b ecause of its rap id ex cretion in the urine , its e ffec t is ap t to be transito ry . M oreover,
m ore da ta up on its e ffec ts on ren a l fu nc tion a re needed , be fo re on e can be ab so lu tely su re
th at it is no t in ju r ious to the neph ro tic k id ney . T he va lue o f m easle s in ocu la tion as a
the rapeu tic m easure is n o t c lea r as ye t, a lthough it p rodu ces s trik ing rem iss io ns in m any ,
bu t no t a ll pa tien ts .
F ew d iseases
tax the resources o f th e prac titio ner so ex tensive ly as th e nep hro tic syn -
; drom e. H e m ust be a co m bin atio n of in fectio us d isease ex pe rt, nu trition ist, phy sio log ist
and psych ia trist fo r th e pa tien t an d , ab ove all, gu ide , co unsello r and frien d to th e p aren ts ,
w ho h ave to live . d ay in an d d ay ou t, fo r tw o or th ree yea rs w ith a ch ild w ho ea ts poo rly
and often has periods of irritab ility o r depression , w ho frequ en tly vom its o r has d iar rhea ,
w ho se appea rance m ay beco m e gro te squ e at tim es, and w h o m ay becom e despe ra te ly sick
w ith pe riton itis and bacte rem ia a t any m o m en t. O n the o the r h an d , the sa tisfac tion of
see ing a pa tien t resto red to norm al health and activ ity a fte r severa l years o f neph ro tic
edem a is w e ll w o rth the tim e and pa tien ce req u ired .
C o N cL u s I o N s
T he reco rds o f 2 08 p a tien ts w ith the n ep hro tic sy nd rom e seen in the C h ild ren s and
In fan ts H osp ita ls o f B os ton from 192 6 to 1 948 have been rev iew ed .
T h e m a in crite ria fo und fo r d iffe ren tia ting b etw een lip o id n ep hro sis and the neph ro tic
stage o f ch ron ic g lom eru lo nephn itis have b een the p re sence o f hyp e rtensio n or azo tem ia
fo r long er than one m o nth in pa tien ts w ith the la tter d isea se , w h ich o ccurs m ore fre -
qu en tly in ch ild ren ov er fou r y ea rs o f ag e . O the r fea tu res o f the tw o d isease s m ay be in -
d is tin gu ishab le . N on ethe less ) it is d es irab le to d if fe ren tia te be tw een them , as apparen tly
few if an y pa tien ts recov er fro m the n ep hro tic sy ndrom e if the ev idences o f g lom eru lo -
neph n itis a re c lear-cu t.
L ipo id nephrosis is ch aracte r ized by the insid io us o nse t o f ed em a in you ng ch ild ren ,
usua lly b e tw een the ages of one an d four. F ind ings inc lude edem a, hy popro te inem ia ,
hy pe rch o le stero lem ia an d heavy pro te inu n ia . B loo d pre ssu re m ay be e leva ted fo r sho rt
periods , bu t ra re ly over one m on th . M icroscop ic hem atu ria does no t exc lude th e d iag nosis ,
tho ug h m acrosco p ic hem a tu ria is usu a lly ev iden ce o f g lo m enu lar in vo lv em en t.
S ym ptom s an d fin d in gs in lipo id neph ro sis m ay pe rsis t fo r from o ne to th ree years
w itho u t spec ific the rap y . E xace rba tion s w ith in fec tion , fo llo w ed b y rem iss ions shortly a fte r
the in fec tion , and rem issio n soo n a fter th e o nse t o f th e d isea se are re la tive ly com m on .
P ro gno sis is no t re lated to th e num ber o r du ra tion of ex ace rb atio ns. D eath b e fo re 1 9 4 2
w as ch ie fly du e to an in tercu rren t in fectio n . S in ce 194 2 , w ith the adven t o f su lfad iaz ine
and the n ew er an tib io tic s, dea th from in fec tion has beco m e less com m on, and has usua lly
occurred a t hom e , p re su m ab ly becau se treatm en t w as no t p rom p tly institu ted .
T he re is app aren tly no con stitu tion a l de fec t in these ch ild ren b efo re the on se t o f th e
d isease , an d gro w th and d eve lopm en t a re norm a l af te r recov e ry .
N o fo rm of treatm en t h as been fou nd w ho lly sa tisfac to ry . P aracen te sis , transfus ion and
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N E P H R O T IC S Y N D R O M E 503
low sa lt d ie t a re th e m o st com m on sup portive m easu re s used he re . A n tib io tic s a re v alu -
ab le dur in g in fec tion s an d have app aren tly low ered the m orta lity o f th e d isease in recen t
years . F oca l sources o f in fection shou ld be e lim ina ted by opera tive pro cedu re on ly w hen
surg ica l in te rven tion is defin ite ly ind ica ted . In jection s of sa lt-poor concen tra ted hu m an
serum a lbum in and the induc tion of m easles a re b e in g eva lua ted as m ethods of induc ing
a d iu re si s.
In th is se r ies app ro x im a te ly on e h alf o f th e pa tien ts w ith a c lin ical d iagno sis o f lipo id
n ep hros is have apparen tly reco vered com p le te ly w ithou t residua l d isease , w hile a sm all
n u m b er
show pers isten t alb um inun ia o r hy perten sion . It is h oped tha t w ith close r super-
v ision of
the pa tien ts and w ith ad eq ua te ch em o th erapy , th is figu re can b e s ign ifican tly
inc reased in the fu tu re .
A C K N O W L E D G M E N T
Fhe au thors w ish to thank M iss Jane W orceste r, A .B ., M .D ., S .D ., o f the D epartm en t
o f B ios ta tistic s o f the H arvard Schoo l o f Pub lic H ea lth , fo r h er gu id ance in the ana lys is
and p repara tion o f tab les .
R E F E R E N C E S
1. S ch w ar tz , H ., K o hn .,
J ., and W einer, S ., L ipo id nephro sis : O bserva tions over period o f 20 years,
A m . J. D is. C h ild . 65 :355 , 1943 .
2 . M u rph y , F . D ., W a rlie ld , L . M ., G rill, J., and A nn is, E ., L ipo id nep hros is : S tudy of 9 pa tien ts ,
w ith specia l referen ce to those observed o ver long period , A rch . In t. M ed . 62 :355 , 193 8 .
3 . H ey m a n, W ., an d S tartzm an , V ., L ipe m ic ne ph ros is, J. P ed iat. 28 : 177, 1946 .
4 . B lock , W . M ., Ja ck son , R . L ., S te arn s, G ., an d B u tsc h , M . P ., L ip o id ne phrosis: C lin ica l an d b io -
c hem ic al stud ies of 40 ch ild ren , 1 0 w ith nec rop sies , P E D ivn uc s 1 :733 , 1 94 8 .
5 . B rad ley , S . E ., and T yso n , C . J ., N eph ro tic syn drom e, N ew E ngland J. M ed . 238 :233 an d 260 ,
1948 .
6. F arr , L . E ., N eph ros is, A d va nce s in In t. M ed . 1 :22 5 , 1 94 2 .
7. W olb ach , S . B ., a nd B la ckfan , K . D ., C lin ic al a nd patho log ical stu d ie s on so-cal led tub u la r
nep hri tis (ne ph ros is), A m . J . M . S c. 1 80:453 , 1 93 0 .
8 . Janew ay , C . A ., an d o thers, D iu resis in ch ild ren w ith neph ros is : C om pariso n o f resp onse to in -
jection of norm a l hu m an seru m a lbu m in an d to in fec tion , p articu larly m ea sle s, T n . A . A m .
P h ysic ian s 61 : 10 8 , 194 8 .
9. G ala n , E ., N ep hro sis in c h ild re n , A m .
J.
D is. C hild . 77 :32 8 , 1 94 9 .
10 . R enn ie , J. B. , O ede m a tou s sy nd rom e o f n ep hnitis w ith spe cia l refere nce to p rog no sis, Q ua rt. J.
M e d . 16 :21 , 194 7 .
S P A N IS H A B S TR A C T
S mn dro ma N efr {2 43}tico
I. H istor ia N a tu ra l d e la E n ferm ed a d
S e h an rev isad o los reco rds de 20 8 pa cie n te s co n sIndrom a nefr#{ 24 3} tic o v isto s de sde 19 26 h asta 1 948 .
L o s crite rios pn in cipa les qu e se han encon trado para d ife renc ia r en tre Ia nefrosis lip o ide y la e tapa
nef r#{ 24 3} tica d e g lo m e ru lone frit is c r#{ 24 3} nica h an s ido Ia p resenc ia d e h iper ten si# {2 43} n y azo tem ia po r m #{ 22 5} se
u n m es e n p acien te s co n e sta i ltim a e nfe rm eda d .
L a n efrosis lip o ide se cara cte riza por el p r inc ip io ins id io o de e de m a en n iflo s p equ efios, gen era l-
m en te en tre las eda des d e 1 y 4 a# {2 41} os .L os d esc ubn im ien tos in clu yen ed em a, h ipo pro teinem ia, h ip er-
c o les tero le m ia y fue rte pro tein uria . L os sm ntom as y lo s de scu bn im ien tos en nef ros is lipo ide ge ne ralm en te
persis ten de 1 a 3 a #{ 241 }o s in terap ia esp ecI fic a . E l pro gn #{ 24 3} stic o no se re lac ion a co n el n C im e ro de
re m isio nes y exa cerbac ion es y ha m e jorado con e l ad ven im ien to de la su lfad iazina y an tib i# { 243 }tico s m #{ 22 5} s
n uev os, ya que Ia in fec ci# {2 43} n es responsab le po r la m ayor pas te de las m u er tes .
P or o tra p aste , en Ia eta pa ne frO tica de g lom eru lon efr itis cr#{ 24 3} nic a el pro gn #{ 243 } stic o es to dav ia pob re
y e l deso rden rena l es la causa p rinc ipa l de m uerte .
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