Você está na página 1de 20

NEPHROTIC SYNDROME: I.

Natural History of the Disease


LEWIS A. BARNESS, GRETCHEN H. MOLL and CHARLES A. JANEWAY
Pediatrics 1950;5;486

The online version of this article, along with updated information and services, is located on
the World Wide Web at:
http://pediatrics.aappublications.org/content/5/3/486

PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication,
it has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked
by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village,
Illinois, 60007. Copyright 1950 by the American Academy of Pediatrics. All rights reserved. Print
ISSN: 0031-4005. Online ISSN: 1098-4275.

Downloaded from pediatrics.aappublications.org at Indonesia:AAP Sponsored on September 27, 2013

NEPHROTIC SYNDROME: I. Natural History of the Disease


LEWIS A. BARNESS, GRETCHEN H. MOLL and CHARLES A. JANEWAY
Pediatrics 1950;5;486
Updated Information &
Services

including high resolution figures, can be found at:


http://pediatrics.aappublications.org/content/5/3/486

Permissions & Licensing

Information about reproducing this article in parts (figures, tables)


or in its entirety can be found online at:
http://pediatrics.aappublications.org/site/misc/Permissions.xhtml

Reprints

Information about ordering reprints can be found online:


http://pediatrics.aappublications.org/site/misc/reprints.xhtml

PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it
has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked by the
American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007.
Copyright 1950 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005.
Online ISSN: 1098-4275.

Downloaded from pediatrics.aappublications.org at Indonesia:AAP Sponsored on September 27, 2013

NEPHROTIC
I. Natural
By

LEwis

SYNDROME

History

A. BARNESS,
AND

of the Disease

M.D.,
A.

CHARLES

GRETCHEN

H.

JANEWAY,

M.D.

M.D.,

MOLL,

Boston

HE

nephrotic

syndrome,

albuminuria,
nonprotein
ized

nitrogen

by

has

observed

with

and

which

assist

mainly

and

relatively

exacerbations,

of

in this

occurs

hypoproteinemia,

blood

purpose

pathogenesis
favorable

the

course

It is the

might

and

in

a chronic

recovery.

been

which

lipemia

this

clinic,

treatment.

normal

blood

to review

has

identifying

been

the

of this

eventual
disease,

projected

placed

factors

as it

studies

on its

on establishing

which

of

is character-

instances,

history

and

edema,
levels

and

in many

present

by

normal

pressure,

natural

for

emphasis

on

relatively

and,

the

as a background
and

is manifested

with

remissions

paper

Particular

prognosis

in children,
usually

criteria

contribute

to an

un-

outcome.

Although

similar

material,

possible

changed

considerably

this

analysis.

tant

chronic

studies

have

been

geographic
with

Two

the

recent

disease

made

in other

differences,

and

advent

articles5

of

clinics,14

an

antibacterial

#{176}
have

the

impression

extensiveness

that

the

chemotherapy

reviewed

the

led

literature

of

this

prognosis

had

us to undertake

concerning

this

impor-

of childhood.
METhOD

The
during
of

records
of all Childrens
and Infants
Hospital
patients
with the nephrotic
syndrome
admitted
the period
1926
to 1948
were
examined.
Letters
were
written
to the parents
or physicians

patients

last

hospital

for

known

to be

a follow-up

describe
the
examinations

health
when

alive

visit.

but

When

not

under

this

active

was

status of the children


possible.
If the child

not

treatment,

possible,

asking

the

the

parents

and to give the results


had died, the parents

and

of recent
were

patients

to return

physicians

were

urine

blood
to record
the

asked

to the
asked

and

to

pressure
cause of

death.
The
case

analysis

were

showing

nitrogen

retention

various

therapeutic

procedures.

records

of patients

known

pared

with

which

the

in the records

of the data

made,

over

the

the

course

the

fluctuations

years.

These

to

and

diagnosis

of

for

living

and

findings

lipoid

carried

in

weight,

findings

Criteria
be

was

in

were
well.

was

of

reported
by Wolbach
and Blackfan7
as examples
Criteria
for an unfavorable
prognosis
were
died

a renal

failure

and

in

From the Department


Boston, Mass.
Supported
Health

of

by
the

whom

autopsy

of Pediatrics,

a grant,
United

recommended
States

by

Public

Panel

of

each

pressure

and

of infections

established

by

in

group

had
of

nephrosis.
by a study

definite

Service,

which
Several

: Charts

blood

occurrence

findings

cases

autopsy.

this
ended

these

in

of

were
death,

autopsies

of the records

and

study

the
com-

and

in

have

been

of patients

who

glornerulonephritis.

Medical

the

Health

the
were

and

similar

manner

hematuria,
with

course

of lipoid
established

Harvard

following

prognosis

at

showed

the

correlated

The

made

in

proteinuria,

a favorable

a group

nephrosis

out

School

on

and

The

Hematology,

and

also

Childrens

from

by

a grant

Medical

the

National

from

the

Center,

Institute
Mead

of

Johnson

Company.
Presented
atrics,

Buffalo,

(Received

in

abbreviated
N.Y.,

April

for publication

form
29-30,

April

at

the

Eastern

May

1-2,

13,

1949.)

Area!

1948.

486

Meeting

of

the

American

Academy

of

Pedi-

NEPHROTIC
The
the

distinguishing

frequency

was

made

the

future.

sions

or

features

and
of

duration

the

causes

of

An effort

of each

of

such

death

was

of these

signs

made

groups

to see

487

of cases

as hematuria,

in order

also

SYNDROME

nitrogen

what

steps

to determine

what

were

determined

retention

and

might

be taken

to reduce

of episode

type

by an analysis
hypertension.
the

appeared

of

A study
mortality

to initiate

in

remis-

exacerbations.
DEFINm0N5

For

the

purposes

1. Acute

of this

the

glomerulonephritis-an

a pyogenic

infection,

proteinuria

are

into

report,

in

acute

which

hematuria

present
for a short
glomerulonephritis.

chronic

NEW

CASES

following

OF

definitions

renal
alone

period

NEPHROSIS

associated

time,

then

TABLE

acute

or abrupt

with

edema,

gradually

NEPHRITIS

AND

used:

with

or

of

INFANTS

are

disease,

subside

ADMITTED

nephrosis

Nephrotic

stage

Total

of glomerulonephritis

nephrotic

syndrome

Acute glomerulonephritis
Chronic
glomerulonephritis
Total

acute

and

Total

cases

admitted

2. Chronic
or a pyogenic
and ultimately
the

infection

3. Nephrotic

a. Chronic
and

initially

obscured,

(see

history

edema

as
but

normal
and

poses

stage
of

nuria,

or

phase

161

36

47

63

145

208

52
24

131
34

183

76

165

241

310

449

nature

58

form

with

nephrotic

and

if

the

nephrotic

syndrome

infection,

evidences

of

now

manifested

hematuria

process

with

chronic

without

persistent

pro-

glomerulonephritis

during

the
at

course

been
to the

classified

(as

lipoid

which

diagnosis

disease.

but

For
as

Also

have

persistent

the

nephrotic

or

during

time.

; in others,

the

diagnosis.

appropriate

any

purposes

instances

who

nephrosis
was

lipemia,

at

at autopsy

classified

clinical

activity,

proteinuria,

retention

only

of the
were

of the

normal

nitrogen

apparent

autopsy

regardless
pursuing

by edema,

or

becomes

scarring

glomerulonephritis
have

other

any,

developing

according

of

pyogenic

syndrome

little,

of the disease

are

or

manifest.

of the

glomerular

azotemia,

stage-A

and

becoming

who

glomerulonephritis).

109

11

manifested

nephrotic
signs,

evidence

patients

hypertension

chronic

presenting

showed

of chronic

Total

1939-1948

52

glomerulonephritis*

pressure

clinical

analysis,

acute

form

who

AND

paragraph)-

in the

ultimately

the true

patients

nephrotic

the

and

progress

disease

insidiously,

first

of

blood

cases

laboratory

analysis,

syndrome

nephrosis-A

In some
by

and
except

renal

subsequently

following
either acute glomerulonephritis
by proteinuria,
hematuria
on occasion
elevated
blood pressure.
Edema may or may not be present,
but is not
in those
cases
which
are classified
as the nephrotic
syndrome.

glomerulonephritis

teinuria

essentially

chronic

developing

symptom

antecedent

b. Lipoid

or

or

following

azotemia

TO CHILDRENS

139

by azotemia

outstanding

known

chronic

glomerulonephritis-a

usually

1926-1946

HOSPITALS

1926-1938
Lipoid

onset

hypertension,

of
of

the

for the

the

pur-

albumistage

of

edematous

of the disease.
Since

nephrosis

this
from

study

glomerulonephritis
occasional

was

chronic
were

cases

of

acute

initiated

originally

glomerulonephritis,
arbitrarily

excluded,

glomerulonephritis

in

an

attempt

cases
since
may

of
in

to
the

them

progress

find

out

nephrotic
the
into

how

distinction
the

to

syndrome
nephrotic

seemed

differentiate

following
clear.
syndrome.

lipoid

acute
However,

488

L.

A.

G.

BARNESS,

H.

MOLL

AND

C. A. JANEWAY

RESULTS

Incidence
In the
and

year

23

Infants

these,

148

have

reached
for
a diagnosis
nephritis.
acute

period,

Hospital
been

for

records

statistical

to 1948,

of

have

29

58

with

were

who

children

the

nephrotic

returned

sixty-one
47 for

patients

who

208

as having

One hundred
nephrosis
and

glomerulonephritis

used

1926

diagnosed

followed,

follow-up.
of lipoid

The

from

were

questionnaires

of the 208
the nephrotic
chronic

admitted

entered

the

syndrome
and

patients
stage

hospital

I).

Of

could

not

be

31

fulfill
the criteria
for
of chronic
glomerulo-

glomerulonephritis

to this

Childrens

(Table

and

during

of

the same

183

with

period

were

controls.

NEPHROSIS

--

ACUTE
CHRONIC

NEPHRITIS
NEPHRITIS

#{149}.4 NEPHROTIC

STAGE

CHRONIC

OF

61

CASES

83

CASES

58

CASES

47

CASES

GLOMERULONEPHRITIS

(I)
I)
a
.4.)

0
0

IC

II

YEARS

CHART

1. Age

of onset

of different

forms

Age
In

the

lipoid

nephrosis,

average

years

age

in this

the

of

series.

glomerulonephritis

age

onset
The

of

for

onset,

the

average

is 7.6

as

shown

6.3

* In

in the
standard
or

2.3

onset

syrnptorns.-The
lipoid

nephrosis

nephrotic
error
times

of lomerulonephritis

of
the

the

stage
the

of

average
chronic

difference

standard

error,

is significant.

in
age

all
is

these
the

difference

in

3.5

years,

while

glomerulonephritis

series

is 4.6

of children

with

chronic

glomerulonephritis.*

instances

3.5

yr.
the

2 means

1, averages

Course

glomerulonephritis,
of

control

and

onset

Chart

in acute

almost
of

in childhood.

of- chronic

in the

years

Symptomatology
initial

in

stage

of onset

and

disease

Onset

nephrotic

age

years,

of

of renal

and

age

onset

lipoid

a standard

average

is 0.473,
of

of

with

the
of

is

4.6

difference
nephrosis

nephrosis
deviation
yr.

of the
and

and
of

3.0

yr.

2 means
the

nephrotic

the
yr.;

2.1

Since
is

the
yr.

1.1

stage

NEPHROTIC

SYNDROME
TABLE

ONSET

Symptom

OF

Lipoid

489

II

DISEASE-FIRST

SYMPTOM

NOTED

Nephrotic

Nephrosis

Stage

of Chronic
Total

Glomerulonephritis

Upper

Living

Dead

Lost

Total

Living

Dead

Lost

Total

respiratory

infection

22

14

40

25

12

25

52

44

14

67

42

14

30

81

12

14

11

37

23

14

30

51

Enuresis

Diarrhea

Other

Edema

of eves

%Vt.gain
Pallor
Generalized

rash

11
208

Total

90

nephrotic

stage

around

the

infection
of

two

first

or

was

cases),

three

days

reported

difference

in the

26

nephritis

(81

symptoms

significant

and

of

eyes

45

exists

nephrotic

stage

161

20

insidious.

rapid

increase

before

the

The

between

the

frequency

Patients

with

1. Within

Patients

than

mo.

having

having
remission

Total

number

Average

symptoms

A breakdown
no

in lipoid

statistically

nephrosis

not

with

associated

obvious

infections)

Nephrotic

Stage

of Chronic
.

Glomerulonephritis
Total

45

52

22

74 (55%)

46

11

57(42%)

64

26

38

125

135

Total

Living

Dead

Total

20

22

52

14

ii

25 (60%)

99(56%)

11(26%)

68(38%)

177

after

more

of

no.

90 (67%)

14

13

27 (64%)

117 (66%)

47 (35%)

1 1 (26%)

58 (33%)

44

169(1.3)*

20

21

at least

than

spont.

missions

that

edema

respiratory

III

90

remission

spont.

cases)

indicate

either

upper

remissions:

patients

1 spont.

of first

were

or an
(52

figures

REMISSIONS

Dead

onset
Total

edema

The

SPONTANEOUS

Living

2 mo. of onset

2. More

noted

cases)

glomerulonephritis.

Nephrosis

patients

of

II.

TABLE

Total

(51

appearance

in Table

(Remissions

symptoms

in weight

is shown
of chronic

first

22

of remissions

re-

per

patient.

4! (1.0)*

210 (1.2)*

L. A.

490

Sex
62

BARNESS,

G.

incidence.-Ninety-nine

were

girls.

glomerulonephritis
cidence

renal

Remissions

and

called

spontaneous

Table

III,

boys

135

children

of chronic

C.
with

JANEWAY

lipoid

with

girls.

A.

nephrosis

the

There

were

nephrotic

was,

thus,

stage

boys
of

a somewhat

and

chronic

higher

in-

males.
of

cases

are common
with

children

AND

children

18 were

exacerbations.-All

of

stage

47

among

remissions

74

161

the

and

diseases

MOLL

the

of

were

of these

nephrotic

of

Twenty-nine

H.

the

within

lipoid

nephrotic

syndrome

two months

nephrosis

glomerulonephritis

of the

and

25

demonstrated

had

onset.

of

children

42

this

edema.

type

So-

As indicated

in

with

the

of remission.

IV(A)

TABLE
RESPONSE

TO INFECTION

Nephrotic
Stageof

Nephrosis

I otal

Chronic
Nephritis

Total

patients

Patients

with

Total

patients

Total

no. infections

with

infections

recorded

followed

by

remissions*

Infections

followed

by

exacerbation*

Infections

followed

by

no change

As

or exacerbation

the

disease

any

individual

that

the
Response

to

177

is not

58

116

34

150

298

85

383

to any
cases.

infection,

208

No.

No.

75

25

18

21

93

146

49

44

52

190

77

26

23

27

100

apparently

by Table

apparent

remission

to

remissions

related

in the hospital,

observed

13

No.

indicated

followed

infection.-Any

a response

47

45

of onset.

spontaneous

This
not

among

considered
while

progresses,

remissions

68 instances

15 days

within

patient.

161
recorded

Infections

Remission

no infections

infection

occurring

since,

in

19

frequently

which

occurred

of

a diuresis

less

however,

within

a series

18 experienced

occur

III,

after

two

1 5 days

of

patients

who

between

1 and

for

shows

an

only

months

in

infection

was

had

infections

1 5 days

following

infection.
Remissions,

exacerbations

infection,

and

patients

no

(Table

known

no

change

difference

IV(A)

Of

161

in

in
this

children

the

patients

respect

with

status

exists

may

between

nephrosis,

there

occur

were

lollowing

two

the

298

groups

of

instances

of

infections.

Seventy-five
146

or

marked

In

the

infections

%)

(25

(49%)

of

these

by exacerbations
47 children
; 21

with

were

infections
and

the

followed

77

nephrotic
by

were

(26%)

followed

by

by no change

stage
remissions,

remissions

in status

of glomerulonephritis,
52%

by

within
of

the

there

exacerbations

were
and

1 5 days,

patient.
85 known
27%

by

no

change.
In

apparent
by

Table

IV(B),

that

exacerbations,

upper

the

response

respiratory
whereas

the

to different

infections
rate

of

types

are seldom
remission

of

infection

is contrasted.

followed

is roughly

40%

It is quite

by remissions

and

after

other

most

usually
types

NEPHROTIC
of

infection.

of

the

Again

there

nephrotic

high

in upper

types

of infection.

Recovery
episode

of

is no

syndrome.

from

lipoid
or

significant

difference

It is interesting

respiratory

edema

SYNDROME

infections,

in this

as

many

for

series

of Infection

Type

favorable
occurred

Change

fection

16

96

49

Pneumococcus

23

23

10

Streptococcus*

8
28

6
21

3
15

47
44

respiratory

Almost

for as long as
months
of the

No

% Re-

Death

occurred
from

number

of Chronic

Nephritis

Remis-

Exacer-

sion

bation

Change

10

34

41

25

5
8

2
2

4
10

45
40

phase

without

missions

after

and

1 to

14

to 1 5 months

duration

exacerbations.

prior

The

to death.

of exacerbations

incidence

tension

was

of hypertension,
considered

appropriate

size

the

was

NPN

to

and

No

% Remissions

ultimate

above

was

the

over

and

when
blood

40

the

hematuria

is indicated

pressure

NPN,

these

signs

were

of standard

cc. ; and

classified

of chronic

glomerulonephritis,
the

month

while

tension

while

161

children

seen
under

absent

persistent
Persistent

hypertension
was
in

transient
120,

in 22 and
hematuria

Of

in

when

absent

was

the

had

47

pressure

nephrosis

with

found

the

than

for a period
stage
Only

longer
127

nephrotic

longer

red

nephrosis.

for
and

transient

one

the nephrotic

against

in 14,

than

present

hypertension

of

; azotemia

Because
nephrotic
or elevation
of

and

hypertension

children

was

more

were

is evidence

transient

graphs

sediment.
hypertension

if they

a cuff

than

had
stage

one

no hyperof

hypertension

chronic
in

12

21.

in 28

whereas

nephrosis

33 had

blood

lipoid

hypertension

lipoid

hospital,

observation.

normal

persistent
Azotemia

with

at this

glomerulonephritis,

between

only

2. Hyper-

with

pressure-age

hematuria

as persistent

persistent

between

in Chart

determined

blood
cell per high power
field was found
in spun urinary
children
with infections
may temporarily
show hematuria,
of one month
or longer.
It will be seen that in differentiating

remission

is no correlation

outcome.

blood

pressure

mg./100

there

Findings

azotemia,

exist

edematous

Apparently,

Laboratory
The

Stage

all i3 Str. hemolyticus.

persisted

was

as one

in several

in-

Other

and

as few

OF INFECTION

Nephrotic

bation

of

to other

occurred

episode
of edema
after one to two

Nephrosis

Exacer-

one

after

forms

is seldom

response

Recovery

TO TYPE

sion

blood

which

IV(B)

Remis-

when

more

of patients

OF RESPONSE

Lipoid

the

the

two

the

fever,

phase.

RELATION

between

whether

from one
occurred

TABLE

Upper

response

16 exacerbations.

as

children
even though
there were no remissions
12 months,
though
more frequently
remissions
edematous

in

to speculate

accounts

nephrosis
after

491

nephrotics,

it was

persisted

transient

in

nine

one

nephrotics

in

the

lipoid

nephrosis,

month
nephrotic

in

1 3 and
stage,

in 16.

found

in

41

children

with

transient

hema-

492

A.

L.

tuna

in 43

who

are

and

sole

abnormal

no

living,

records.

in four

ing

; in

who

between

had

noted

in

have

these

AND

four

who

died,

and

in

the

Thus

though

(LEFT

now

has
gross

COLUMNS)

NEPHRITIS

80

(RIGHT

NIT

these

patients

been

lost

as the
from

our

had

per-

glomerulonephritis

no

hematuria

are

least

noted.

Gross

in our

records

was

no

longer

helpful

is rare

OF

sign

in lipoid

in differentiat-

nephrosis.

This

is

be

cx-

SIGNS

(44Lw

CASES

47

HYPERTENSION

the

of

albuminuria

have

who

hematuria

161

COLUMNS)

13

two

been

in five

have

who

of

in
in

45DE40
10

four

stage

SIGNIFICANCE
NEPHROSIS

noted

in

is living,

hematuria

diseases,

was

nephrotic

who

A. JANEWAY

of whom

h#{235}maturia and

child

C.

hematuria

and

transient

died.

MOLL

Gross

77.

four

one

two

H.

is well

children
10

was

in

of whom

finding

hematuria,

hematuria

G.

hematuria

one

Twenty-four

sistent

and

BARNESS,

.45

ALBUMINURIC,

I HYPERTENSION

26LOST)

CASES

(2OLIVING

22DEAD

RETENTION

5 LOST)

MIC

5%

HEMATURIA

46%

50z

20%

21Z

1%

26%

TRANSIENT

CHART

at

variance

plained
from

with
group

of cases

the

cholesterol

275

700
of

in

mg./100
both

above

groups,
this

Values
7.4

19 cases

gm./100

measurements

level
for

with

79

of

glomerulonephritis

to three

the

serum

cc.

and

were

out

made.

of

than

275

was

below

months

protein
were

less
In

signs

students
cases

to 1,300

170

88

of

in

the

than
children

in

which

gm./100
in

the

the

cc.

of the
with

nephrotic

stage.

lipoid

nephrosis

cholesterol

stage

with

first

more

reported.

was

varied

19.

when

may

from

204

In several

to

children

measured,

but

rose

disease.

lipoid
cc.

and

glomerulonephritis

nephrotic
in

16 of these

mg./100

children
4.5

disease

nephrotic

cc. in

the onset

disease.

as acute

cc.

in the
275

MONTH)

the

in the

mg./100

after

of

beginning

mg./100

cases

(>
renal

PERSISTENT

major

other
of

in
more

of

glomerulonephritis

from

cholesterol
one

most

exclusion

of chronic

cc.

cc.,

of

arbitrary

varied

mg./100

Cholesterol

significance

experience

by our

Serum
than

the

in part

MONTH)

(<

Diagnostic

2.

in

nephrosis
119
stage

of
of

ranged
153

patients

chronic

from
in
nephritis,

2.1

to

whom
the

NEPHROTIC
serum

protein

below

4.5

gm./100

ranged

edema

was

present

absent

when

in

above

present

was

edema

from

186

this

at levels

was

3.5

cc. Of

absent

to 7.2

197

of

gm./100

patients

when

the

level.

SYNDROME

whom

serum

protein
cc.

There

was

no

artificially
and

fed.

D,

and

reported
nephrosis.

had

been

finding

half

About

three

have

had

lit
in

to

Fifteen

the

attempt

was

A family
47

made

history

chronic

to find

of allergy

glomerulonephritics

is difficult

onset

A family
diabetes

due

history

206

of

was

nephritics

group

of

diseases,

chronic

children

before

childhood
or

vitamins

the

tonsillectomies

children.

the remainder
of

Twenty

nephrosis

of

fed and

are

the
the

onset

children

glomerulonephritis.

History

hereditary

nephrotic

varying

disease

and

edema

three

supplements

usual

background

in 43 of the

the

to the

of renal

in 23 of the

found

in

to determine

cc.,

and

breast

none.
had

the

a common

was

reported,

nephritics

in either

adequate

had

for

Family

An

levels

were

gm./100

seven

nephrotics

been

received

except

prior

4.5

and

disease
had

received
had

colds.

however,

health

previous

had

fourth

below

in three

of children

fourths

frequent

general,

good

group

one

was

showing

levels

History

of any

of each

approximately

to

of

constant

one

patients

cc.

Previous

Approximately

44

protein

nephrotics

and

of 3 to 4 gm./100

23 of

serum

in nine

5 to 6 gm./100

at levels

cc. with

in

However,

493

161

stage.

estimates

was found

of

in

children.

and

The

significance

allergy

in the

patients,

23

in these

nephrotics,

in 16 of the

of these
general

figures

population.

of tuberculosis

in 20

of

and

patients.
Treatment

Table

V lists

under

64

that

few

in

have

not been

been

evaluated
now

per

milk.*

Removal

of

requiring

teeth

extracted.

but

attempt

this

of renal

gm.

chloride
children.

have

been
of

the

been

protein

test

used,

low-salt

used

for

the

208

methods

of

time

; undoubtedly

of

patients

while

treatment

abscess
carried

of

to perform
hospital

had

one

out.

This

has

proved

with

less

than

rib

the

has

the

child

208

indicates
many

have

for

been

gm.

children

had

one

tonsillectomies

when

edema
as

the

procedure

is minimal.

and

in

action.

for
and

has

been

is necessary,

The

anesthetic

otitis
carious

antrotomy

a tonsillectomy

cyclopropane

salt-free
had

Two

empyema,

When

sodium

drink

Seventeen
a routine

exist.

will

drainage.

performed.
not

therapeutically
0.5

phase.

edematous

requiring

resection

were

operation

recommends

unless

the edematous

cellulitis

indications

the

none

during

drainage

customary

but
diet

to achieve,

empirically
Eleven

the

anesthesia

of

de-

choice

in

the

damage.

(sodium-free
it for

nephrotic

diet
high

is impossible

diuretic

to substitute
sodium

have
number

to withstand

mastoidectomy,

when

is made
of

large

infection-Fourteen

pelvic

Diuretics-No
Lonalac

of

One
one

only

partment

this

were

performed

in
a

myringotomy.

and

which

The

are given

focus

adenoidectomies

enough

vitamins

media

presence

good

favor

day,

Mixed

sinusitis,

hospital.

variations
We

chloride

of treatment

the

carefully.

Diet-Seven
striking.

an

methods

observation

has
milk)

ordinary
per

milk.
day

can

been

found

(Mead

Johnson)

With

Lonalac,

be

achieved,

consistent
.

It has

salt-free
which

is

proved

Mercurial

useful,

bread

and

salt-free

still

more

salt

if the

diuretics
child

butter,
than

is

can
an

excreted

have

be induced
intake

of
by

0.5

many

494

L.

A.

BARNESS,

G.

H.

AND

MOLL
TABLE

DRUGS

AND PROCEDURES
AND

C.

USED

IN TREATMENT

INFANTS

HOSPITALS,

OF NEPHROSIS

High

low salt

and Blood

Blood

protein

Low

Concentrated

serum

albumin,

Concentrated

human

serum

Low protein
Low fat

Immune

Limited

Acacia

fluids

fluids

Removal

salt-poor

globulin

Amino

acids

Bovine

serum

albumin

of Infection
.

Infections

Tonsillectomy
Carious
teeth

and adenoidectom.y
removed

Rib

Measles

resection

Pelvic

Induced

Vaccinia

Mastoidectomy

Chemotherapeutic

abscess

drained

Antrotomy

Drugs

Penicillin

Myringotomy

Cellulitis
.

Substitutes

transfusion

Plasma

salt

Forced

IN CHILDRENS

1926-1948

Blood
protein

High

A. JANEWAY

Sulfadiazine

drained

Sulfathiazole
Sulfapyridine

Diuretics
Mercupurin
Salyrgan
.

Diuretin

Ammonium
Hypertonic
Hypertonic
Aminophyllin
Theophyllne
Theobromine

Sulfanilamide
Streptomycin

#{174}
#{174}
#{174}
chloride
glucose
sucrose
#{174}
#{174}
#{174}

Other Drugs

and

Procedures

Pyramidon
Paracentesis
Thyroid
extract
Decapsulation
of kidney
Ascorbic
acid
Testosterone
propionate

Urea

Rutin

Magnesium

sulfate

Vitamin

J ava tea

A and

Mazola

Carbohydrate

Potassium

chloride

Parathormone

Potassium

nitrate

Liver,

intramuscular

Potassium

citrate

Horse

antiserum

Calcium

chloride

Urinary

oil

fraction

of pneumococcus

antiseptics

Digitalis
Mixed

vitamins

Choline
Pyridoxine

Sweats

been

used

in six children.

stance was
out notable
nesium

sulfate

tea (Kumis
three children
child

over

salts

to two

Ammonium

chloride

there a diuresis.
Hypertonic
glucose
effect. Aminophyllin,
theophylline
was

given

to

14

children

kuching,
an herb used
with equivocal
results,

a period

children.

of nearly

a year

was

without

chiefly
although

at home.

used

in six children

and

in no in-

and sucrose
were used eight times
and theobromine
were used in five.
consistently

in the Orient
it repeatedly
Potassium

salts

demonstrable

effect.

is a diuretic)
was
produced
a diuresis
were

given

to six and

withMagJava

used in
in one
calcium

NEPHROTIC
Blood,

blood

given

derivatives

transfusions

era

of

serum

increasing

has

the serum

human

three

received

stances

albumin

serum,

four

amino

were

cially

measles,

17

been

achieved

by intranasal

from

the patient

frozen

at

with

gargle

method

repeated

secondary

the

ready

and

in

nephrotic

stage

Three

one year

of

is

early

in the

disease

spread

over

easily
drugs

gland

ficial

results.

ures

listed

striking

sub-

and

onset

has

collected

by

washings
u.

are

each

of

and

a typical
measles
in

chemotherapy

the

to

complete

rash.

; two

similarly

prevent

to the

have

disappear-

Three

of the

children,

chronic
nine

essentially

edema

in five in the

developed
to develop

measles.

of

remained

and

espewashings

100,000

almost

no diuresis

a diuresis

the

208

children

effective

late

in the

wall

is thick

in

the

all

nephritics

children

edema

recurred

the

cavity

than

abdominal

released

with

free

from

for

one

two

month

to

used

section
was

in

abdominal

paracentesis.

43.

been

disease.

One

and

edematous,

abdominal
in

thyroid

it at home.
well,

was

time

possible

the

is thin,

parts

of

Of

noted

that

explanation

and

wall

other

It has

the

is that

edema
edema

fluid
fluid

body,

and

is
col-

conse-

by paracentesis.

received

administration

required

one

disease,

procedures.-Desiccated

in the last

given

after

abdominal

the

improvement

of active

than

Later

others

been

1 2 days

proteinuria

are
The

nephrosis
children
failed

have

have

These
broth.

with

attack

had

infection,
inoculation

at 37#{176}C.,and

1 1 experienced

its

Even

use

has

of Table
noted,

were

gland

though
been

in five children
V have

was given
these

been

in the hospital

children

abandoned

without

discontinued.

because

apparent

tried

only

The

use

of

The

time

and,

11

tolerate

lack

effect.
one

of

to

apparently

of

other
because

chemotherapeutic

bene-

measno
drugs

below.

Cause
The

causes

present

study

these,

of these

inoculation.

Fourteen
and three

more

more

Digitalis

is discussed

of

were

globulin;

#{149}

the
in

; several

thyroid

effects

Successful

oropharynx.

measles,

with

of

readiiy

virus.

children

12

their

stage,

performed

body.

is more

Other

serum

follows

bacteriologic

thawed

in

children

1 1

eight,

often

the

more

children

without

1 to 2 cc. of throat

in the
with

children

to

responded
these

was

paracentesis

lects

used

the diuresis.8

paracentesis

quently

then

developed

Paracentesis.-Seventy-four

these

Salt-

cases

Six children

The

frequently

to 2 cc. before

nephrotic

remaining

after

use,

The

albuminuria
in the

the

throat

throughout

who

nephritics

In

for
was

infections

of edema

years.

serum

albumin.

immune

albumin.

the

human

the

elsewhere.8

were

before

serum

half

given

measles

spots

the

are added

chronic
nephrosis.

rinse

inoculations.

children

14

with

of chronic
glomerulonephritis.
7 to 17 days after inoculation

bacterial

the

children

normal

circulating

of approximately

Kopliks

and

of inoculation

spite

of

bovine

diuresis

inoculated

instillation

70#{176}C.until

stage
eruption

ance

were

and streptomycin

nephrotic
macular

Of

children

individuals

having

This

the

six were

one

spontaneous

induced.-Because

penicillin

received

as reported
and

and

groups

208

infections,

in approximately

plasma

acacia

during

variable.

infectkrns

obtained

of the

therapy

increase

appreciably,

given

one

and

a diuresis

level

were

acids,

in both

diuresis

produced

495

substitutes.-Fifty-one

as supportive

children

to initiate

albumin

given

and

Thirty-one

in an attempt

poor

blood

anemia

chemotherapy.

albumin

and

for

SYNDROME

45

of death,
of

161

patients

when

the child

patients

diagnosed

(32%)

have

of Death

died

in the

lipoid
died.

hospital,

nephrosis,

Seventeen

either

are shown
136
died

in Chart

have

been

at

home,

3. In the

followed
or

the

and,
cause

L. A.

496

BARNESS,

G.

H.

MOLL

AND

OF

DEATH

CAUSES
IN

NEPHROTIC

C.

NEPHROTIC
CHRONIC

29

STAGE

19

CASES

ACCIDENTS
OF THERAPY

__

-CARDIAC

OF

GLOMERULONEPHRITIS

CASES

mTnhl
I

JANEWAY

SYNDROME

NEPHROSIS

A.

5,,

FAILURE

-3

RENAL

637

._-

FAILURE
83

f-

INFECTION

-4
27Z

3.

CHART

Comparative

incidence
Note

of

death

was

of cardiac

not

of

different

predominant

ascertained.

Of

decompensation,

one

causes

role

the

of

death

of infection

remaining

of anuria

in

2 forms

in lipoid

patients,

28

following

of

nephrotic

syndrome.

nephrosis.

died

23

sulfapyridine

and

of

infection,

one

three

following

tonsil-

lectomy.

Twenty-two
known
of

of

to have

infection,

of

of

albumin

of

death

causes

nephrosis

are

in

16,

in

which

not

shown

while

children
The

one

administration

The

the

died.

cardiac

Chart

12

in

It is seen

stage

has

been

4.

that

one

of

of

be

the

This

followed

two

One

or

implicated
the

time

the

advent

since

infection

possible

explanation

increase

in

is shown

the

number

in Table

after

1942

among

access

to prompt

VI

medical

recent
of

and

death.

In

of the

also

living

and

followed

increase

patients

which

children

19.

Of

are

these,

edema

five

died

following

the

distance

hospital

three

in

cases,

the

with

lipoid

was

two

of

the

the

organisms

Jied

were

cases
were

in

in the

two

present
23

use of sulfadiazine

the patient
since 1942,

1942,

hospital.
circulatory

tonsillectomy.

have

indicates

patients
peritonitis

E. coli

liberal

in the

who

the

pneumococcal

in two
of

and one death


for

glomerulonephritis
in

among

few deaths
have been accompanied
by infection
when
Of the five hospital
deaths
in children
with nephrosis
deaths,

chronic

pulmonary

infection

Pneumococcemia

was
at

of

ascertained

uremia.

to type

present

was

nephrotic

death

decompensation,

and

according

in

infection

the

of

streptococcus

the

identified.

in

cause

number
come

of deaths

from

considerable

rate

higher

mortality

and

hence

presumably

the

data

at home

may

distances.

both
having

before
less

and
ready

care.
Prognosis

The
The

prognosis
status

of

regarding

the

weighted,

as no

of
these
patients
special

these

diseases

patients
with
attempt

is based

is

briefly

acute

was

on

summarized

and

made

chronic

to

in

from

children

Tables

VII

glomerulonephritis

follow

these

patients

and
in

after

followed

to

date.

VIII.

The

data

Table

discharge

VII

are

from

NEPHROTIC

497

SYNDROME

j,

SULFONAMIDES

[PENILLIN

IS A1SP1S.T1SD.

24

...-.NEW

CASES

CASE

LOAD

DEATHS

EEl
D
t:i

PNEUMOCOCCUS

OTHER

DIED

STREPTOCOCCUS
ESCHERICHI

COLI
INFECTION

AT

HOME

CIRCULATORY

ACCIDENT

CASES

OF

OF

THERAPY

.4,)

w
U)

a
.4)
U.

28

16
YEAR

NEW

4. Causes

CHART

to

rise

that

since

most

deaths

responsible

for

the

hospital.

one

year,

in

of

onset

in the

years
has

The

criteria

used

blood

not

The

with

1942,

occurred

at

period

1939

to

; those

with

onset

death

home
since

have

before
when

that

failure
case

pneumococcus

been

are

and

subdivided

of mortality

load.

which

freedom

Also

was

from

urine

free

into

three

prechemotherapy

sulfonamides
when

well

NPN,

1 938-the

1942,

Note

increasing

of

edema

groups

and

for

era ; those

were

introduced

sulfadiazine

began

for
to

RELATION

Date

OF

DOMICILE

of Onset

Cases

from

OF

PATIENTS

TO

the

be

used

IN

161 CASES

OF LIPOID

192 6-1942
Total

Cases

treatment
for

NEPHROSIS

1943-1948

Deaths

Mortality

Total

Cases

Deaths

purwith

VI

MORTALITY

for

protein

treatment.
F.l3LE

note

formerly

1942.

serum

children

after

and

1926-1948

45 deaths.

steadily

a patient

normal

1942

IN NEPHROSIS

with

despite

considering

onset

DEATHS

nephrosis

in

caused

for

AND

of lipoid

pressure,

elements.

: those

cases

have

fatalities

analysis

infections

161

sulfadiazine

most

formed

abnormal

in
of

recent

normal

poses
of

of death

introduction

NEPHROSIS

Mortality

GreaterBoston

49

(61%)

17

35%

27

(33%)

11%

Elsewhere

31

(39%)

15

48%

54

(67%)

10

19%

the

L. A.

498

BARNESS,

G.

H.

MOLL

C-

C-

U)

U)

CI

U)

0U)

AND

C.

U)

A.

U)

ei

OH

oO

-u

U)

U)

d.e%l

e
0\

U)

\0

c-

U)

e-

z-

4
0

U)
0

U)

.4-

F-

U)
U)
-

U)
ri

0
U)
H

H H

CU

C
V

C)
.

dei

U)

__
__
__

00

UI
C

e.4 U)

EU.
U)

U)

CU

___
___

.4
H

U)

4)

U)

00

C0

C-

C-

U)

U)

4)

-u
00

C-

-u

:
cU

4)

C-4

U)

olo

C-

U)

4)

U)

;-

-u

UI
0

CM

C-.

U)

U)

U)

U)

UI
CU

.-

0
a

0.

.C

-E
.

,_
U)

:-,:
.

(.1
U)

nrn-u

I-

C/)

.0

.-

V...

4)

.C

L)

U)
.

CU

CU
.

uu C

C.)

4)4)
MU

U)
0

-.

4)
CU

NEPHROTIC
Of

161

period

children

1926

to

with

lipoid

the

1938,

nephrosis,

average

deaths,
a relative
mortality
to 1942, the average
case

13
1939

mortality

the

of

10.3

average
One

load

hundred

years : 45

seven

of renal

and approximately

with

the

of these

for

to

1948,

been

followed

or more

) are living

1 7%

10 are among

two

the current

with

patients,

VIII

WITH

LnoID

NEPHROSIS

2 OR MORE

WHO

HAVE

BEEN

YEARS

No.

Patients

with

Major

Signs

HyperEdema

Azotemia

tension

Hematuria

11-lSyr.

6-lOyr.

12

4-5yr.

10

3yr.

10

2yr.

12

the

shown

children

newer

in the

antibiotics,

died

reduced.

been

death.
It remains

the

these

impossible,

for

followed

that

(76%
than

with

mortality).

2 yr.

The

of

the

certain

has

been

supportive

might

have

183

102

with
others

acute

are

have

further

actual

been

followed

(from

mortality

6.8

who

have

measures,

such

as are available

whatever

prognosis

to 3.1

will

children
tolerated

patients
followed
renal disease.

patients

reduced

a favorable

of active

of the

nephritis

are living.
nephrosis
before
chemotherapy
the introduction
of sulfadiazine

follow-up,

nephrotic

to give

of living
Of

rate
a longer

patients

evidence

majority

of chronic

died
crisis

in either

also

since

at the

caused

chronic

be

1942

their
glomer-

during
the year plus the number
of new
Thirty-three
chronic nephritics
have been
glomerulonephritis,

presumed

are likely to return to the hospital. Fifty-three


chronic and 3 died of cardiac decompensa1on

difficulty

become

year

had

however,

load is the number

Case

in
all

Perhaps,

used,

stage

mortality

Almost

at home.

hospital,

nephrotic

are dead, and seven (24%


over-all
prognosis
in lipoid
40%
of the cases. Since

Presumably,

to be

have

have

1943

patients/100/year)

longer
sequent

period

were

period
a relative

Twenty-nine

there

of 3.9/100/year.

well ; 18 (

and

and

sulfonamide
16 deaths,

16-22yr.

two or more years : 22 (76%)


Thus,
it is seen that the
was favorable
in approximately

patients

year,

year

the
with

to recover.

TABLE

Proteinuria

and

In

have

18, however,

_________

Total No.
Patients

In the presulfonamide
per

mortality

nephrosis

may be expected

PATIENTS

died.

patients

a relative

% ) are living

latter

FOLLOWED

Followed

14.6

sulfadiazine-penicillin

lipoid

(41

Of these

OF 62 LIVING

have

was

1 5 deaths,

died ; 44

one half

STATUS

In

64 with

disease.

(28%)

load*

499

of 6.8 patients/100/year.
load was 39 patients
per

children

) have

(42%

evidence

was

45

case

patients/100/year.

case

SYNDROME

to be well

81

have

as only

of the 81 are known


during
the acute illness.

been

those

followed

with

to be well,

sub25

500

L.

ulonephritis
with

A.

or

the

BARNESS,

the

latter

G.

nephrotic

may

live

H.

MOLL
of

syndrome

or longer

10 years

In

the

series

course

lasted

of

62

children

of nephrosis

between

occurred

one

and

between

who

has

years

(2

1.2

after

onset,

three

2 and

95

months
Growth

Growth

and

following

development

recovery,

height-weight

usually

the

patient

anthropometric

children

apparently

psychometric

are

but

or more

years

In all but

a few,

Of those

usually

during

normal

Only

school

children

with

nephrotics

recovery,

active

who

within

two

years.

active

phase

of

disease

died,

death

nephrosis,

but

Development

his

charts.

do average

years)

delayed

assumes

two

6 to 96 months.

and

though

disease.

followed

from

JANEWAY

Disease

been

persisted

A.

glomerulonephritis,

their
of

have

C.

chronic

with

Duration

the

AND

one

work,

the

growth

channel

patient

has

shown

no

attempt

has

and

as plotted
mental

on

standard

retardation.

been

made

The

to do

routine

examinations.
DISCUSSION

Our

observations

Block.4

The

are comparable

Using
lipoid

are

mortality
to those

the

possible.

result

of

to those
our

recorded

criteria

nephrosis

but

similar
among

for

and

stage

diagnoses

reports

hypertension

or

nitrogen

retention.

considerable

time,

even

up to one

Glomerular

changes

diagnosis

of
some

time.

nephrotic

stage

of

different

variants

instances

of

just

as

More

may

tion

by

patients
under

function

or

syndrome
the

newer

who
way

tests

used

recovery

actually

follow-up

study,
The

come

fact

to

intercurrent

past

cases

ended

up

we have

did
of

with

had

in the

not

reduced

group

of

that

the

renal

For
followed

a rela-

glomerulo-

picture,

of

disease.

renal

in the

of

renal

findings

itself.

could

Such

studies

the
for

the

allow

clinical

16 or

one
or

purposes

examination

funcin those

Unfortunately,
with

clinical

have

derangements

autopsy

which

two

certain

a mixed

studies

disease

nephrosis

function.

patients

forms

clinics.9

information
lipoid

on ordinary
nine

to

or

that

cases,

giving

of careful

other

the

glomerulonephritis

in certain

ultimate

been

and

diseases

is clear

nephrosis

anatomic

tubular
had

nephrosis

It

a clinical

five,

symptoms

different

other

and

whom

these

lipoid

for

be made.

in

of
and

nephrosis,

and
or

could

lipoid

as a

to be followed

of chronic

complicate

of

yield

so-called

to rely

as

correlation

a number

as hematuria,

all

two

possible

course

signs

process.

features

infection

as in

actually

physiologic

clinical

and

patients

whether

lipoid

the

either

signs

classify

the

may

from

the

atypical

discuss

It is quite

only

as well

in the

that,

five

we

the

17

difficult,

changed,

had

in

pathologic

sclerosis

with

some

whether

to

concerning

methods
own

place

5 of

been

be

distinction

However,

on an original

vascular

can

succumb
in our

the

in which

prognosis.

information

termine

nalysis.

a poor

be superimposed

exact

of

which

those

and

findings
between

has

to

patients

in

made.

are

syndrome

clinical

differentiation

symptoms

many

fundamental

while

pyelonephritis

nephrotic

same

nephrotic

have

nephritis

the

had

a definite

glomerulonephritis

the

prognosis,

predominate

is not

the

have

such

at autopsy

been

in four

Heyman,3

and

glomerulonephritis

of

before

noted

and

outlined,

cases

However,
year,

had

chronic
of

the

good

This

208

appearance

been

nephrosis

predominated,
at

tively

the

have

lipoid

degeneration

noted

or

as

of chronic

among

autopsy

Murphy,2

as the laboratory

series.
diagnosis

nephrotic

Fourteen

by Schwarz,1

as well

in their

differential

the

reported

patients

of

with
more

are
renal

to de-

apparent
this

initial

routine

un-

years,

pro-

NEPHROTIC
has

teinunia
from

been
in

This

would

normal.
Because

lipoid

the

of

difficulty

optimism.

but

ultimate

patient

serum

tients

poor

program

for
views

It

physician
disease.

On

patient

far

to treat

and

by

infections

the

contacts

the

winter

doses

of

the

deserves

are

to prompt
may

be

of

In

of our

all severe

in

The
cians

or parents

less of what
chronic

disease,

tient

as

results

in prolonged

infectious

and

and

parents.

In

with

diuretics,

found,

may

be

child

ineffective

with

hypertension,

be dangerous

leads

during

or

the

Those

pa-

assume

may
and

environment

as much

There

is noknown

reason

of respiratory

children

during

in prophylactic
and

effectiveness
of the

parents

chemotherapy

the

development

have

occurred

is probably

in

indicated

the

patient

eliminate
to

certainly

the mercunials,
plasma

the

of

edema

foci,

We

such

believe

if
as the

may

to

albumin
given

adenoids,

athomeor

inkeepingpatients

is

infection

in themselves
are

and

child

patient

of

serum

doses

tonsils

both

the
foci

or even

large

paThis

exposures
for

if any

the

edema.

repeated

concentrated

pulmonary

the

effects

and,

to any

of

trauma

dfrfrnental
sorts,

regard-

reaction

appearance

emotional
all

of physicourse

natural

the

to

important

of

this

struggle

of

unwillingness
its natural

affects

submits

diuretics,

phase.

is the
of

profoundly

precipitate

the edematous

lipoid
infection

by cutting.down

E. coli

to run

consequence

but

removal

with
of

so that

therapy

child
is apt

unnecessary

infusions

the
the

its safety
fever,

the

at minimizing

is education

streptomycin

infusions

The

until

the
of

protecting

antibiotics

important

valiant

subtle

of

to

disease

so

at

particularly

or

our
present

etiology,

extent

family,

our

is identified.

The

intravenous

sheltered
-

protect

course

prevention

due

the

which

intravenous

The

and

patients

infections

nephrotic

to considerable

removed.

are

kidney,

degree

therapeutic

frequency

preventing

the

and

in

and

organism

which

addiEinifhes#{233}

these

the

damage

edema,

represent

real

and

the

particularly

and

that

continuous

with

because

own

aimed

bacteremia
fatal

the

fact

one

the

infection,

that

hospitalization,

agents,

plied
are

of

the

death

thoroughly.

Most

penicillin

the

of

our
study

runs

to diminish

be recommended

is administered.

is

and
with

usually

unknown

be
he

sulfonamides

controlling
fact

should

cause

outside

cannot

of
affects

to a considerable

of

threatens

particularly

nephrosis,

past.

this

diseases

which

and

until

which

treatment

can

in the

comments

on

which

major

conditions.

the

to accept

to

exacerbations,

if one

to place

based

hazards

the

use

thus

at least

hazard

kidney

a reasonable

however,

been

program

people

combined

infections,

second

cases

and

death

following

chronic

promptly

symptoms

of

patients,

of

has

treatment

accomplished

but

view

particularly

The

be made

controlled

promptly,

peritonitis.

several

be

with

of

with

involvement,

should

continuous

recognition
instituted

many
of

Since

can

patient

under

recovery

the

patients

remissions

cause

study

special

effort

investigation,

demonstrated

so

any

infections

The

with

footing.

therapeutic

disease.

but

months.

in

his

every

difficult,

clinical
of

of

all

course

of nephrosis

way

from

established
is

as
the

hand

the

types

be treated

major

this

management

in

is infection,

two

should

glomerular

a sounder

that,

possible

as

caused

nephrosis

the

other

the

children,

the

in making
on

little

the

so

damage

do

many

been
of

that

restoration

nephritis.

patients

is clear

can

in

suggests

in childhood,

a chronic

evidence

regarding

experience.

nun
has

purposes

these

between
common

albuminunia

occur

of chronic

chief

imply

observations.10

relatively

which
frank

prognosis

of our

current

will

develop

complete

Rennies

will

infection,

who

One

disease

recovery

from

in two,

always

with

and

501

hypertension

not

to be

proteins

The

and

does

in distinguishing

appears

low

of

in three,

childhood
be in line

nephrosis

lipemia,

the

found

nephrosis

SYNDROME

to

may
in a

as possible.
why

a child

with

nephrosis

should

not

be

ambulatory

as

L. A.

502

long

as the

provided

edema

G.

permits

an adequate

tamed.

remains

The
with

who

as to embarrass
eating,
methods
of treatment

but,

because

more

data

that

of

it is not

but

measure

not

diseases

drome.

He
have

and

for

has

the

periods

seeing

a patient
is well

of

patient

its effect

kidney.

accumulatiort

although

of

understanding

is apt

in those

fluid

becomes

in some

to be transitory.

one

value

nature

patients,
Moreover,

can be absolutely

of measles

it produces

fluid

of the

edema

before

The

can be main-

extracellular

in relieving

are needed,

the

of

above

day out,

irritability

striking

sure

inoculation

as a

remissions

restored

to

all,

guide,

as the

expert,

in many,

and

years

who

nephrotic

nutritionist,

counsellor

or three

depression,

grotesque
at any
normal

the time

so extensively

disease

for two

or

may become
and bacteremia

worth

practitioner

of infectious
and,

day in and

whose
appearance
with peritonitis
edema

resources

be a combination

to live.

often

the

tax

must

psychiatrist

who

urine,

as yet,

the

as possible,

of protein,

of removing

in whom

function

in sodium

amount

be useful

nephrotic

clear

be as low

all patients.

Few
and

in the

to the

A. JANEWAY

or getting
about.
to await a cleaner

may

on renal

is not

and

albumin

C.

should

method

ascites,

excretion

its effects
injurious

diet

breathing
will have

Serum

its rapid

upon

therapeutic

considerable

AND

a considerable

the simplest

children

process.

MOLL

intake,

so great
Better

have

H.

activity.

caloric

Panacentesis

of the disease

BARNESS,

with

frequently

syn-

physiologist

friend

to the

a child

who

vomits

or has

parents,

eats poorly
diarrhea,

at times, and who may become


desperately
moment.
On the other
hand,
the satisfaction

health

and

and patience

activity

after

several

years

of

sick
of

nephrotic

required.

CoNcLusIoNs
The

records

Infants

of 208

Hospitals

patients

of Boston

with

from

The main criteria


found
for
stage of chronic
glomerulonephnitis
for

longer

quently

than

one

distinguishable.

few

month

in children

nephnitis

patients
nephrosis
the

hypercholesterolemia
but

rarely

from

of

with

of age.
the

was

chiefly

and

the

There
disease,

No

seen

in the

Childrens

and

reviewed.

latter

nephrosis
and
of hypertension

disease,

features

which

of the

to differentiate

nephrotic

and

heavy

one

if the

the nephrotic
or azotemia

occurs

two

between

syndrome

due

newer

more

diseases

may

them,

frebe in-

as apparently

evidences

of glomerulo-

at home,

with

and growth
form

from

because

no constitutional
and development

of treatment

has been

the onset
or duration

not

1942,

has

with

become

be elevated
exclude

was

defect

in these
after

not

short

diagnosis,

one

to three

years

by remissions

shortly

after

relatively
common.
Death
before
1942

advent

common,

promptly

children

for

the

involvement.
from

the

less

children,

hypoproteinemia,

of the disease
are
of exacerbations.

treatment

wholly

for

followed

Since

are normal
found

persist

infection,

infection

may
does

of glomenular

may

infection.

death

presumably

is apparently

evidence

in young

edema,

pressure

hematuria

nephrosis

of edema

include

Blood

is usually

Exacerbations

onset

Findings

Microscopic

in lipoid

to an intercurrent
antibiotics,

insidious

four.

proteinunia.

month.

findings

therapy.

and

the infection,
and remission
soon after
Prognosis
is not related
to the number

occurred

the
Other

by the

one

hematuria

and

specific

ages

over

macroscopic

Symptoms
without

years

is characterized

between

though

been

differentiating
between
lipoid
have been the presence

it is desirable

recover

syndrome

have

are clear-cut.

Lipoid

periods,

nephrotic

to 1948

in patients

four

Nonetheless)

if any

usually

over

the

1926

of sulfadiazine
and

has

usually

instituted.
before

the onset

of the

recovery.

satisfactory.

Paracentesis,

transfusion

and

NEPHROTIC
low

salt

able

during

years.

diet

are the

Focal

surgical

most

infections
sources

of

intervention
albumin

serum

common

and

have

supportive

infection

the

lowered

should

induction

503

measures

apparently

is definitely

and

SYNDROME

be

used

the

here.

mortality

eliminated

the

by operative

indicated.

Injections

of

of

are

evaluated

measles

Antibiotics
of

being

are valu-

disease

in recent

procedure

salt-poor

only

when

concentrated

human

as methods

of

inducing

a diuresis.

In this

series

approximately

have

apparently

nephrosis

number

show

vision

persistent

the

of

increased

half

and

of the patients
completely

albuminunia

patients

in the

one
recovered

or

with

with

without

hypertension.

adequate

a clinical

residual
It is hoped

chemotherapy,

this

diagnosis

disease,
that

with

figure

of lipoid

while

a small

closer

super-

can

be

significantly

of

the

Department

future.
ACKNOWLEDGMENT

Fhe
of

authors

wish

Biostatistics

and

of

preparation

to thank
the

Miss

Harvard

Jane

Worcester,

School

of

A.B.,

Public

M.D.,

Health,

for

S.D.,

her

guidance

in the

analysis

of tables.
REFERENCES

J., and Weiner,


S., Lipoid nephrosis:
Observations
over period of 20 years,
Am. J. Dis. Child. 65:355,
1943.
2. Murphy,
F. D., Warlield,
L. M., Grill,
J., and Annis, E., Lipoid nephrosis:
Study of 9 patients,
with special
reference
to those observed
over long period,
Arch. Int. Med. 62:355,
1938.
3. Heyman,
W.,
and Startzman,
V., Lipemic
nephrosis,
J. Pediat.
28: 177, 1946.
4. Block,
W. M., Jackson,
R. L., Stearns,
G., and Butsch,
M. P., Lipoid
nephrosis:
Clinical
and bio1. Schwartz,

H.,

chemical

Kohn.,

studies

of 40 children,

10 with

necropsies,

PEDivnucs

1:733,

1948.

5. Bradley,
S. E., and Tyson,
C. J., Nephrotic
syndrome,
New England
J. Med. 238:233
1948.
6. Farr, L. E., Nephrosis,
Advances
in Int. Med.
1:225,
1942.
7. Wolbach,
S. B., and
Blackfan,
K. D.,
Clinical
and
pathological
studies
on so-called
nephritis

(nephrosis),

8. Janeway,
jection

C. A., and

others,

Diuresis

of

normal

human

serum

61 : 108,

1948.

Physicians

9. Galan,
10.

E., Nephrosis

J.

Rennie,

Med.

J. M. Sc.

Am.

B.,

in children,

Oedematous

16:21,

180:453,

of

han

revisado

nefr#{243}tica de
un

mes

los

records

pnincipales

que

se han

esta

iiltima

pacientes

La nefrosis
mente

entre

colesterolemia
persisten
nuevos,

1 a

ya que

de

Longwood

Comparison
particularly

of response
measles,

to in-

Tn.

A.

Am.

1949.
special

reference

to

prognosis,

J.

Quart.

renal

en

pacientes

por
Los

terapia
y ha

es la causa

Avenue

con

sido

la Enfermedad

sIndroma

para
Ia

nefr#{243}tico vistos

diferenciar

presencia

el principio

entre

de

desde

1926

Ia nefrosis

hipertensi#{243}n

con

nefrOtica

principal

descubnimientos

El

progn#{243}stico

el

advenimiento

por la mayor
de

glomerulonefritis

de muerte.

de

edema

incluyen

y los

especIfica.

mejorado

insidioo

descubnimientos

smntomas

es responsable

Ia etapa

de

hasta

lipoide

1948.

y la etapa

y azotemia

por

m#{225}s
de

enfermedad.

proteinuria.

3 a#{241}ossin

Nefr#{243}tico

encontrado

1 y 4 a#{241}os.Los

Ia infecci#{243}n

paste,

208

se caracteriza

y exacerbaciones

Por otra
y el desorden
300

edades
y fuerte

de

77:328,
with

Natural

cr#{243}nica han

con

lipoide
las

remisiones

de

glomerulonefritis

en

nephrosis:
infection,

ABSTRACT

Smndroma

criterios

tubular

1947.

I. Historia
Se

to

nephnitis

SPANISH

Los

with

and

J. Dis. Child.

Am.

syndrome

260,

1930.

in children
albumin

and

paste

en

niflos

edema,
en nefrosis

no
de

se

relaciona

pequefios,

general-

hipoproteinemia,

hiper-

lipoide

generalmente

con

la sulfadiazina

el

nCimero

y antibi#{243}ticos

de
m#{225}s

de las muertes.
cr#{243}nica el progn#{243}stico

es

todavia

pobre

Você também pode gostar