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Long-term Follow-up
to Renal
By Ralph
NAIIIYLACTOID
Involvement
and The However,
vascular Marshall
J. P. Wedgwood,
purpura is a
M.D.,*
H. Klaus,
of
and
M.D.
is unknown. wide-spread
of the frequent
generalized a specific by joint exa eruplesion its is that around from name. of an the ac-
etiology
lesions,
the of
disease the
characterized usually and often The (Iescribe(l from or classic disease the skin red has accompanied by specific by urticaria,
by
because
because
it
often has
in
teiisivelv of tion
which
represents diseases.
l)lanching
purpose phase
paper
frequency
nature disease.
Histologically
acute inflanirnatory
the For
corium this
rather reason
than Gairdner1
PATIENT
Between
MATERIAL
1953, were in 36 Twenty-six 1954 age months 2 and at to were of cent during were onset 123 children with to Babies children alive of years (Fig. and svmpand 1). 9 obyears admitted
1939
and Hospital.
Syn-
The
attached early, its
SCCII15
afl(l l)ecause
Henoch
of The
are
their of disease
well.
4 years
manifestations.2
IWCI1 1
Seventeen of the were female. This the anare 2: 1 is similar servers.1 to the Although to the
to thors l)tIt
Osler
wrote that
reported
by other
of the these patients
visceral
the
skin
a minor
at times of
obdismaniit
z
LU -J
C-) Li
admitted
MALE 6 FEMALE
disease of the or
Precl011iifl11it another.
From
involvement
system
04
LU
the
1)epartments
\Vestcrn Childrens
Ohio.
Cleveland,
health, April
O
work was in part supported 1l-1643 from the National U. S. Public Ilealth Service.
Aug.
1955.)
(Submitted
1:3,
26,
1954;
revision Road,
ADDRESS:
2065
Adclhcrt
Cleveland
6,
Ohio.
1. Age
at onset
of
symptoms.
196
ORIGINAL
were Negro,
no
ARTICLES
the allergenic described agents. As might the (Fig. ties of trunk. purpura, and small in the disease, 2). all arms The but red The be suspected all rash and skin also patients; of the involved half one-third lesions urticaria, pinpoint also had consisted erythema, macules or from children the had the definition had lower lesions not factors
iii
Negroes may on
have as
been
36
patients
anaphylactoid
purpura,
causative of
crepancy recognizing
than an actual difference In all other respects the rived No of the from other children a widely members
in
involvement
0%
PURPURA ARTHRALGIA
ABDOMINAL PAIN
50%
100%
FEVER PROTEINURIA
VOMITING
MICROSCOPIC HEMATURIA BLOOD IN STOOL
10 NUMBER
* BENZIDINE TEST PERFORMED Fic. IN ONLY 2. Initial
15 OF CHILDREN
7 PATIENTS and signs.
20
25
symptoms
families,
cidence of
any
any
suggestion of
of
an
unusual
in-
the
so-called
collagen children infection with the throat beta with had prior onset
disa to of
Sixteen ing of ankles, some cause ment and toms and blood amined. melena.
twenty-six had moderate more joints, usually the Although of to was tenderness motion moderate not there of severe; were phase 1 1 had of emesis. were positive children referable abdominal Tests in for 3 of these the heat, usually had pain, was joints
Of 9 children This
in whom agreement
a thorough search to in the children, 4 gave but were and eruptions, without another sugand
allergy minor
stool
history
of
manifestations
Two of these children One of these (Case 19) intussusception bowel removed. an exploratory obstruction just
I)0S5i11e asthma). Iii iione of the any specific allergen be implicated cipitating factor in the disease.
and a section of The other (Case for to the possible onset of 1)rior
laparotomy
198
WEDGWOOD
ANAPHYLACTOID
PURPURA
z8
LU
-J
z
LU
-J
I C)
.Li
I C)
0
LU
LU
#{174} 5 8
FIG. :3. WBC
ptlrp(lra.
II WBC
17
20 and
X 1000
acute phase.
over
#{174} o
Fic.
normal abnormal
4.
123456
I
and
MINUTES
Bleeding time Bleeding only 5 of the during times 21 acute (Fig. patients phase. 4) were tested. limits. in
(luring
bowel
hyperemic
and
edematous
At
hospital had
ill
the
time
9 of
of the
the (Cases
initial
admission
were febrile.
to the
Four
children
hypertension
1 child
(Case
and
19)
responded
was
Clot retraction was normal in 12 children examined and platelet counts were within normal limits in 18 children on whom this examination was performed. including alone Routine urinalyses re-
u1sions
vealed
in sion.
proteinuria
Proteinuria
microscopic
the was 4 with found in
hematuria
hyperten3 others.
magnesium apart tions, not more atric only was from skin common population. 2 children. found.
6 children,
of upper joint
seen in
remarkable.
Lymphadenopathv
the
was
general
palpable
pediin disease
The
spleen evidence
The duration of the initial symptoms ranged from 6 to 180 days (Fig. 5). Usually the initial manifestations of the disease subsided in 4 weeks. Those children in whom the illness
ran a protracted course generally had a severe
of heart
form children,
were and normal hematocrits. varied white on from counts infection. differential)
of
and
the
disease,
exacerbations. of the
characterized
During received some acetyl and children
by
the
remisacute salicylic
All
mildly amination
of the
anemic,
except
found
for
on blood The 5800 could was
3 who
laboratory cell white to not found
were
excounts, blood
some
antibiotics
red
infections
to existing
w
0 -J4 C.)
borderline (below
to 15 mm.,/hr.)
dren on whom the examination Elevated sedimentation rates related tourniquet positive clotting with test in only the existence in were performed 3. Studies
U
.
,
I
A
was and within
FIG.
0 I
5.
8-12
6 mos.
children bleeding
I d
disease.
over
of the
mechanisms
generally
ORIGINAL
none hormone. was given cortisone or adrenocorticotropic
ARTICLES weight
ma!
fell
the
within
Harvard
the Blood
norpnes-
ranges
defined
School
M ETHODS
The
interviewed
of Public
be by the their taken located authors. with acute was preserved by a examined special disperwere At
growth
26
could was
time
to bed
in these
presthe
emphasis
since
range.
physical in all
vessels.
the
funduscopic no
any
there heart
Although
Addis technic. measured with tube. were fasting. by subsequent of the for and Hospital During repeated carried
Urinary protein excretion Tsuchiyas reagent in Urea out on Addis examination child more urea to Babies complete Addis clearance tests and the were renal nitrogen blood counts, were and deterobtained if yencause Chilrenal counts and pencon-
abnormality
There
disease
several
was
in
evidence
of the
of
children.
organic
Abnormal
1 soft these
systolic thus
were cise
all diminished
admission
nitrogen
determined
phenolsulfonphthalein formed.
centration
findings
in Figduplicate
ures or
by
7,
multiple
a mean
determinations
value. well of the
12
are
the limit J
represented
children of had normal average
hematuria
(600,000
rbc/12
8 the
RESULTS
In
years ease child
the
had and had than
Illajority
of
patients
2 or
the onset (Fig. years, been of 6).
more
disOne
showed hematuria cells per 12 hours. excretion was cell red of 100,000 found. casts casts 23, excess 24) of
excretion particu-
In in adwere was
100
another followed
of these
8 years.
less All
fronl
2 children
but
symptoms
children
of
had
been
free
an mg./12 of larly 1
of
suggestive
In been
white
cells
interpretation
recurrences
counts
impossible. be seen fell in into Figures 2 distinct counts. had Sixurine normal 11, 14,
had urine 7,
In 8, and
general, 9, the
as can children
prolonged
in the electrocardiogram.
unchanged
The
for
PR
interval
and
2 years
which can be considered within limits. Ten of the children (Cases 16, 17, 18, 19, 21, which, on the basis peated abnormal normality excessive Addis counts, 23, of 24, the must
25)
of
reabof in
Most
usual
of the
contagious difficulty.
considered
On
physical
examination
the
height
and
casts;
200
\VEDGWOOD
ANAPHYLACTOID
PURPURA
12
z8
LU
-J
10
w
6
4
0 -J
I C)
U-
0 LU
U0
I
0 2468and YEARS over
0 I 2345678 RBC/ 12 hrs. XI00,000 and over
I0
z
LU -J I
8
6
C)
U-
0
LU
left).
Length
of follow-up
found
right). Expressed
Hematunia
as
RBC
10
Cylindruria as casts
found at 10 per
12
hours.
#{174}
only creased these
0
20406080 CASTS/
XI000
3 was to (Cases Six children proteinuria#{176} a significant 17, 23, 24) 16, degree, it was 17, consistently and not even a striking 26) had on pro. inin
On
repeated
Addis
counts,
with
the
(Cases
beta
de-
proteinuria in 1 or more specimens however, in 3 (Cases 16, 18, 26) was not consistently abnormal.
gree ably
of the consistent.
abnormality
remained
remark-
ORIGINAL
P11OO1/lliNJtI
ARTICLES
Z 2
,
201
t 2
2
4 x to
2
.p
2
S
#{241}wnw
cOIX3ff,11
,,
t,.
-a
C,
c()IXW)
cO1X3UW
-C
O1X3FJ.fl
C (1/wwiqoJ;uzf -C
ZZ
ZZ
vunuwo4,f
-H
I +
I +
+ +
+ +
v:4n7uw.)JJ
I -I-
I +
I -F
I -f-+-+
1)fl.)7J$
I -f-
I -1- I
a
C
.a
biajzwo1 I I I -f#{149} I I
I -I- I I I 4- I I I I -f-
-I-+
I I -I- I I I I
.E
a
a
-a
aiOJ7Z)UiULOpq
.a
C
I+I+++I
1+1
1+1+
+++I+I
III
C
1vJw?J,oJtqlu!or
I+++I
I++I++I+++
++++I+++I
C =
CC C
vJnd4nJ+++++++++++++++++
+++++++++
II II II
ii3
1#{176}Vi
tq
+ +
,zzz
z
++
z1z
.
I
I +++
U
I
C
CC
vo!PJJUI
++I
I++I++++Iz
ICI
II
I I I I I I
,,
(#{149}I7)
,, a,
,,
,,
,,
,,
,,
,,
dn-iwjoj
Jo
V713vd7
.),x.,,O,,)
i4oz7vq4.)?vz
I ++
I +
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I ++
I ++
I +
I I I I
vJ1
Flija 1.
+ + +++++++++++++++++
+ ++
+
+ +
a
C
(flvp)avqJanafoncn7n4nq
:
,
:
, ,,
t:
;
0
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,
I
:
p
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.
-a
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.C C
()o1v?flv
C .
C
o
+
II II II I +i++
z
-C
JqazdtD3 .
202
WEDGWOOD
ANAPHYLACTOID
the disease this
PURPURA
began its acute group. that be this over phase This (X2 subsequent expected the age. can also be seen at chilSeven of nine age was difference 10.4, to be p urinary more of 6 years found is <
in 0.01)
z
LU
statistically
abnormalities
significant
0 -J
onset
disease.
proteinuria, or both of the disease were There initial nine was on children also followwith a the of proteinuria
hematuria
The
dude
10 children
6 males and
abnormal
a sex
urine
ratio
inof
4 females,
not
been
different group.
for the an average normal
from 10
average of 4.8
reported TABLE
sex 4.3
ratio
have years, for the
of the
similar group
These
children years
RELATIONSHIP
OF URINARY
LATE
with
urine.
Seven
than
ease.
of the
3 years
ten
from
have
the
been
onset
followed
of the initial
more
disSubsequent Initia I Urinary Ilematuria 4 Findings Normal 4
The
abnormal
urinary
zndznys
be considered part of the acute disease in any of the children; with continued purpura (Case correlation eluded in this group. There was a distinct the age of disease and
normality per of cent) onset of over the
Prokinuria
6
Urine
9) is not between
abnormal
Urine
3
13
the child at the onset of the later finding of urinary II). While
the age disease had of 6 years
the abtime
urine
normal
(Table
urines the found tion renal There tween initial quent was ously,9 tions on
on routine of the
only age
II
AGE ON AT ONSET
13
of 6 years
RELATIONSHIP URINARY
BETWEEN FINI)INGS
AND
FoLLow-UP
(ri,zc
Age at Oruel Normal
on Folithe-up
- - __________
Abnormal 1 9 11 4
follow-up.
10 children
tfl(ler6VarS Over
(X=10.5
6 ye:irs
p<O.Ol).
group
ORIGINAL
among
ARTICLES
ratio
gests
203
the
16
who
had studies
no
in children
that, while
with
there
abnormal
is a sex
urine
differentia-
sugmani-
More
on
complete
the
10 IV).
no
children In
striking or
Four
with general
of
urines rehad
cent
tion tiation
urinary
in the
rate is no
purpunic sex
(Table
vealed
of renal
festations,
differen-
uniform
impairment
in the
of the
subsequent
function.
the
below
children
70 per
abnormality.
average
urea
clearances
of urine
and
1 child
failed, gravity
were
prohis Inon
there urinary
Renal purpura disease Osler,5 lieved tion. patients disease acute view throughout of
the
longed
tnavenous
deprivation, a specific
pyelograms children, and abnormality
to concentrate
described.
Henoch,3
and more recently this to be the most Pathologic during have the the shown widespread body, renal of the studies the acute early
Gairdner beserious complicakidneys phase changes Indeed, vascular it should lesions be exwould vascular urinary
in
8 of these
was no
in
each of
case the
of the
visible
tracts.
TABLE FoLiow-uI IV
STUDIES
glomerulonephnitis.1
Case
Urea
highest
\umber 11 14 16 17 18 19 1 23 24
l5
*
Present .lge 10 8 H 14 1 13 14 13 15
of
Length
1ollol1-uJ)
of
(learance*
(%)
1.50 105 89 86 180 13 64 56 67 68 clearances
7 2 6 6 4 S I 2 3
1.030 1.038 1.035 1.015 1.036 1.03 1.040 1.07 1.040 1.040 of 1 hour
to the disease series seems to of latent nephnitis. contained fewer less protein
or
fit
and none of
than
subacute
usually
chronic
411/1
nephritis,
and
the
children
as
yet
13 10/1st
shows constitutional insufficiency. There is not enough the after the the of for within employed either the to evaluate dren, who, of the
Average
8 maximal
each.
.t Following
fluid
deprivation
of 1
to 30 hours.
DISCUSSION
At follow-up considered
mality cylindruria
is
hematuria
existed initial a of
purpura.
least
10 study
of
the have
26 urine The
by
in must
this be
and The
throughout
manifestations
abnormal.
characterized
abnor-
rt5 nephritic
describe sequelae
rather
than
of follow-up precludes being considered part of the most the purpura, It is than age disease. often and during generally
occurs purpura
the of
of its onset. latent nethat the In taken prognosis of renal anaphylacduraof of renal folcare in disdisthe
advisedly
progress. have
children
of 6 years
of
illness.
hematuria
proteinuria
phylactoid
the anasex
However, prolonged
in
males
females.19
similar
204 tion
clearances
WEDGWOOD of the to serious renal hematuria, found concentrate suggest for nature sequelae. is no known therapy of anaphylactoid hormone
used been
in
visceral
10 children,
these
SUMMARY
A long-term follow-up dren with anaphylactoid lein-Henoch average length study of purpura 26 chil(Sch#{246}n-
cortisone
Syndrome) is presented. The of follow-up was 4% years. 10 were nephritis and proteinunia. over the of found characterto but
do the is of be
not urinary
or
16
by
hematuria
cylindunia, age
in
hormones
may renal
without marked ten children were the phase this pura chronic
ogy
sequelae
of or the
purpura; during It is
teinunia
rest,
suggested
therapy
Because
infections)
majority
sequela constitute
of
and or
if use(l,
at that such
time
either
be
proteinunia,
preventive
As with all
ACKNOWLEDGMENT
The bers
drens study.
probably group.
directed chil-
are for
to assistance
the and
memChilin this
particularly
of Babies
dren current
ously;
who
one
have infection
of
latent should
the
renal be
children
disease, treated
in this
intervigorseries
( Case
increase
ately
17) in
had
a pronounced during
a beta to
and and
hemolytic
sudden immedistrep-
REFERENCES
1. Gairdner, drome. 2. Henoch, D. : The Sch#{246}nlein-Henoch SynQuart. J. Med., 17:95, 1948. E. : Ueber eine eigenthumliche Form von Purpura. Berl. klin. Wchnschr., 11:641, 1874.
E. : Vorlesungen ilber Kinder-
hematunia
subsequent
tococcal
Many
infection.
adult patients with chronic ne-
phritis lonephnitis
suggest
give that
of
acute
glomeruwith
glomeru-
pediatric children
acute
3.
Henoch,
classic
post-streptococcal
lonephritis
to a chronic
phase.
wonders whether some of the patients have chronic renal disease in adult might lactoid been cant, haps have had a mild episode purpura#{176} in childhood thought at the time to
krankheiten, 10th Ed. A. Berlin, Hirschwald, 1899, p. 839. 4. Sch#{246}nlein, J. L. : AlIg. u. spec. Path. u. Therap., Henisau, Lit.-Compt., 1 1:48, 3rd Ed., 1837. 5. Osler, W. : The visceral lesions of purpura and allied conditions. Brit. M. J., 1:517, 1914. 6. Sheldon, J. H. : Purpura Necrotica. Arch. Dis. Childhood, 22:7, 1947. 7. Levitt, L. M., and Burbank, B. : Glomerulonephnitis as a complication of the
o Eight of the ten children with abnormal urine left the usual pediatric age group prior to the finding of urinary abnormality at a time when they were thought by their families to be entirely well.
Sch#{246}nlein-Henoch Syndrome. New England J. Med., 248:530, 1953. 8. Wedgwood, R. J. P., and Janeway, C. A.: Serum complement in children with collagen diseases. PEDIATRICS, 11:569, 1953.
ORIGINAL
9.
ARTICLES
se supone de de las Los que est#{225} condicionada y que enfermedades presentan este y naturaleza del padecimiento, 26 por pertenece trabajo un
205
estado
10.
11.
12.
G. : The Sch#{246}nlein-Henoch childhood with particular reference to the occurence of nephritis. Arch. Dis. Childhood, 27:480, 1952. Ackroyd, J. F. : Allergic purpura, including purpura due to food, drugs, and infections. Am. J. Med., 14:605, 1953. Lyttle, J. D. : The Addis sediment count in normal children. J. Clin. Investigation, 12:87, 1933. Giles, Margaret D. : The Addis count in the prognosis of acute nephritis in childhood. Arch. Dis. Childhood, 22:232,
in
Philpott, Syndrome
NI.
a! grupo para
se#{241}alarIa presencia a Ia fase aguda observaciones meses a 12 ninos familiar piirpura padecimientos
Ning#{241}n
ni ninguno
col#{225}genos.
1947.
13. Addis, T. : Clomerular Nephritis: Diagnosis and Treatment, 1st Ed. New York, Macmillan, 1948. Ham, T. H., Editor: A Syllabus of Laboratory Examinations in Clinical Diagnosis, 1st Ed. Cambridge, Harvard, 1953. Ferrari, M., Lapido, C., and y Scherschemer, J. : Smndromes renales en el p#{241}rpura reumatoide. An. Fac. med. \Iontevicleo, 38:239, 1953. Philpott, NI. C., and Briggs, J. N. : Treatmeat of the Henoch-Sch#{246}nlein Syndrome with adrenocorticotropic hormone and cortisone. Arch. Dis. Childhood, 28:57, 1953. \Iichaels, L., and Walters, C. : Increased
hematunia in nephritis during cortisone
Trece
fecci#{243}nde
de
ina Ia 4
respiratorias
altas
inmediatamostraron
14.
Ia
familiares
15.
alergia en si mismos. Durante 12 de los 26 imos presentaron (11 emesis; dolor 2 con
gastrointestinales episodios de
16.
se operaron pens#{225}ndose sec#{225}ndole al primero una gangrenado en el Los presentan se se les complicaciones encuentran con definitiva que Ia fase no aguda puede de en acuerdo se divideron forma renal, de otro). datos en pero Algunos de las sin de laboratorio figuras el las
conflrmarse estos
antihistam#{237}nicos
sin mingn
17.
6. A todos
vista cuyos 8 los de como y 26 de 9. datos
and
Dis.
hormone Childhood,
ad28:
213, 1953.
18. M. I. : Disturbances of the kidney, Mitchell-Nelson Textbook of Pediatrics, 5th Ed., Waldo E. Nelson, Editor. Philadelphia, Saunders, 1950, p. 1217. Goettsch, Elvira: Nephnitis and nephrosis, ill Brennemanns Practice of Pediatrics, III. Hagerstown, Prior, 1948, chap. 28, p. 7. Rubin, in
presentaron
19.
Se observO
una
renal, pero ciaciOn
correlaciOn
caracterizada sin proteinuria
deflnida
por los
entre
si su
Ia edad
grado nueve de edad Ia fase
del
ni#{241}o
a! iniciarse
el padecimiento acentuada:
de lesiOn con
y cilindruria
SPANISH
ABSTRACT
mi-
despu#{233}s de y hematuria
Piirpura Anafilactoide ( SIndrome de Schonlein-Henoch ) : Estudio con referencia especial a Complicaci#{243}n Renal
La
cas
y sOlo uno
de esta
es estadIsticamente encontrO relaciOn, Ia se proteinuria en las de relaciOn anormalidades entre Ia fase Ia revisiOn diferencia aguda de y persistencia encontrO y las ni tampoco durante en o duraciOn
entre su No
p#{241}rpura anafilactoide caractenizada Ia piel, articulares se v Henoch que Se le por estos desconoce generalmente y asocian a de
Ia intensidad padecimiento
Ia descripci#{243}n autores
y r
completa
adecimiento.
observO
del
sexo
en
206
WEDGWOOD
de
en
PURPURA con un major concentration inter 2 e De accordo con reportos per altere circa duo tertios del patientes esseva
Ben que le contemporanee popula-
cuanto a Ia incidencia purpOricas, no la hubo complicaciOn El valorarse; sideran existe ciones pronOstico Ia grave tratamiento renales; las autores de una crOnicos Ia de para renal mayorIa las efectivo en caso de
las manifestaciones Ia incidencia de ni#{241}os no los para existir autores nefrIticas. las deben que de las complicatratarse secuelas pueden lo puede
la
infecciones consideran
intercurrentes.
tion total del hospital includeva 20 pro cento negros, nulle negro esseva trovate inter le 36 patientes de purpura anaphylactoide. Iste differentia es possibilemente debite al difficultate de discerner purpura super un pelle
negroide.
pOrpura las de
In
currentia en
le familias
de ni
Un
del
purpura
patientes
frequentia
nulle
inusual
oculle o le
padecimientos
anaphylactoide
esseva
constatate,
altere In
patientes
le prime
duo In un
INTERLINGUA
Purpura tinuation Anaphylactoide
ABSTRACT
plus
del
morbo
e le presente
habeva
studio.
annos, minus pro
Sch#{246}nlein-Henoch
a Longe
):
e amontava
le integre
Referentia
Involvimento
Le pro sequelas
phylactoide.
Special
Renal
esseva e de del in frequente con justificar representa pertine al acute
a
interprendite natura del ananon de extense es purpura morbo illo
Con
solmente
hic
in iste
cative de purpura anaphylactoide. In 10 casos-6 masculos e 4 femininas-nostre constatationes indicava un latente nephnitis
charactenisate per hematuria e
le le
etiologia occurrentia
cylmnduria
sed
hypersensibilitate
gruppo
sin pronunciate proteinuria. Iste gruppo includeva 9 patientes de plus que 6 annos de etate a! tempore del declaration del morbo. Jib includeva 6 casos de proteinuria o hematuria durante be phase acute del morbo. Il pare possibile que iste typo de sequela
renal de origines purpura de que anaphylactoide chronic occurre de purpura morbo in renal patientes constitue a etiologia adulte. un del
collagenic.
Le
protocollos
a
del
un
Hospital
Ohio, revela
pro
pro
Infantes
le annos
e
de il
J uveniles
Cleveland,
incognoscite
total de 36 anaphylactoide.
Le historia
episodio
de
juvenil
tab casos de
include
forsan
anaphylactoide
esseva possibile trovar 26 de iste Illes omnes se trovava in bon stato e in reporto. br totalitate del morbo forma patientes esseva le base del
que
a! tempore
como
su occurrentia
esseva
siderate recognoscite
Le etate
tion del
a! tempore inter 13
del menses
morbo, que es characterisate e recognoscite per su besiones dermatic, pote exister in un forma con manifestationes exclusivemente visceral.