INFANCY
AND
CHILDHOOD
Charles Frey, M.D., and S. Frank Redo, M.D.
7/te New lurk Hospital-Cornell Medical Center, Department of Surgery
525 East 68th Street, New lurk 21, New lurk
(Submitted November 5; accepted for publication December 19, 1962.)
ADDRESS: (CF.) as above.
On the basis of clinical or POstnlOrtenl
PF:ot.vI’Ilu s. July 1963
93
P
ANCREATITIS IN CHILDREN is uncommon.Only 36 cases of acute pancreatitis had
been reported! up to 1959,I while 10 of
pseudocysts of tile pancreas2 and 6 of
cilrOnic pancreatitis had been described
tiurough 1960. In 1961 Blumenthal and
Prob-stein4 collected 74 cases of acute
pancreati-tis in children. Although two-thirds of a!!
adult cases of pancreatitis are attrii)utable to the ingestion of alcohol and to biliary
tract disease, few, if any, cases in chulclren
may be due to tilese causes. \Vitll increased!
knowledge regarding the etiology of
pan-creatitis based! on both clinical and!
experi-nlental observations, the diagnosis of
“idio-1)atilic pancreatitis” has been madle less
frequently.
Eighteen cilildren vithi pancreatitis, Ilot
includlrng those with cystic fibrosis of the
I)1flcreas, were encountered at Tile New
York Hospital between 1932 and 1962. The
case of histories of these patients were
re-viewed to gain a better understanding of
tile etiology and! the factors contributing
to nlortahity of this disease in children.
CLINICAL MATERIAL AND FINDINGS
Eighteen chik!ren with pancreatitis were
seen at The New York Hospital in tile past
30 years. There were 14 i)oys and 4 girls
ranging in age from 1 day to 10 years.
Diag-nosis was made at postmortem examination in 14 cases and by clinical and laboratory
studies during life ill tile remaining 4 cases
(Table I).
Signs and Symptoms
Tile four children who survived gave
evi-dence of an acute inflammatory abdominal
prOce5s associated! \Vitil nausea, voniiting,
pain, and tenderness. These patients were
5, 7, 7, and 10 years of age and presumably
able to give an account of their illness.
Many of tile 14 patients ‘lso came to
au-topsy had! a history of poor oral intake,
feeding proI)lelns, nausea, vonliting,
diar-rilea, abd!Ominal pain , and persistent
inter-current illfectiOn . Less frequently
nsanifes-tations were tacilvcard!ia, disorientation,
jaulldice, or coma. Ill IlOll of these 1)atielltS
‘as tile diagnosis of pancre1titi5
ellter-tamed! Prier to death.
Laboratory Findings
Urinalysis revealed! ahl)urnin in eight
children, seven of whom died. in six
pa-tients, tilree of \‘ilOIll survived, there was fl()
albuminuria. The renlainillg four were
in-fants who dud IlOt IlaVe urine testedl. Blood
urea Ilitrogen determinations were
per-formed ill five patients. Three vith elevated levels died!; one of two with normal values
survived. Serum biliruhill levels were
dc-vated in five children. All five had an ele-ment of obstructive jalllldlice, but, in
adldi-tiOll, four sild)\ved isepatocellular d!arnage
as ind!icated i)y abnormal cepisalin floccula-tion, thymol turhid!ity, or transamillase
studies . Those with coml)ined obstructive
and! hepatocellular changes died. The one
patient with finc!ings of O1)structiOn only
survived. Amylase determinations were
per-formed in the four surviving patients andl
found to be
354,
585, 385, and 446 Somogyiullits.
Steroi(I treated I 4yr F Nausea and vomiting Nephrosis
3 yr 151 Anorexia. jaundice, fever, erythematous rash, coma Cirrhosis
3 6yr M Fatigue, abdominal pain, jaun(lice Cirrhosis
Congenital anoma.
malies
Mumps
4 7 mo F Anorexia, jaundice, ascites, hepatosplenomegaly
S 3yr M Nausea, vomiting, malnutrition, hepatosplenomegaly
6 7yr M Nausea, vomiting, abdominal pain & tenderness
7 10 yr M Nausea, vomiting, abdominal pain & tenderness
8 5yr M Nausea, vomiting, abdominal pain & tenderness
Atretic common duct &
cir-rhosis
Annular pancreas
Mumps
Mumps
Died I)ied I)ied
l)ied
1)ied
Alive
Alive
Alive
i)ied Alive
“I(lK)patl(iC” (infec.
ti()11 8(1(1 malnu-trition)
It 14
Died
Died
l)ied
1)ied I)ied Died
TABLE I
I)ATA ON CASES OF A(’ITTE PANCREATITIS IN INFANTS AND CHILDREN, THE NEV YORK IIOSPITAL-CORNF.LL
MEDICAL CENTER, 19321962’
Etiology Case Age Sex Symptoms & Signs
Postoperative 9 4 yr F Obesity postop., nausea, vomiting, tachycardia
10 7 yr M Cholelithiasis. postop. splenectomy, nausea, vomiting,
abdominal tenderness
Periarteritis nodosa II
Intestinal obstruc- 1’
tion
Associated Disease
5 yr M Nausea, vomiting, hypertension, abdominaltenderness Periarteritis nodosa
Sda M Abdominal distension, vomiting
5urn M Diarrhea, vomiting
:t mo F Diarrhea, marantic
13 mo M Vomiting, jaundice, hepatosplenomegaly
16 1 mo M I)iarrhea, vomiting, dehydration
17 1(Ia M Premature
18 3 la M Peritonitis
Intussusception
Intestinal obstruction
Otitis media, mastoiditis
Pericarditis, syphilis,
malnu-trition, empyema
Ilemorrhagic disease of
new-born, malnutrition
Newborn diarrhea
Toxoplasmosis
Gangrene of ascending colon
Outcome
I)ied
l)ied
Summary: Age: 1 day to 10 yr; Sex: M-14, F-4; died; 14; survived, 4.
findings in the 18 patients, the pancreatitis
encountered was attributed to use of
ste-roids in three cases, congenital anomalies in
tilree, mumps in two, postoperative
prob-lems in two, intestinal obstruction in one,
vascular obstruction in one, and unknown
(possible infection and poor nutrition) in
six.
Autopsy Findings (Table II)
Pancreas: Acute interstitial pancreatitis,
manifested by edema and acute
inflamma-tory cells, was present in six children. Four
children showed fat necrosis and
hemor-rhagic cilanges in addition to edema and
interstitial inflammation. In four patients
tilere was atrophy and fibrosis as well as
acute and chronic interstitial changes. The
pancreatic ductal system was normal in
seven, while in tile remaining seven there
were mucopurulent plugs, stenosis, or
in-spissated eosinopililic secretions.
Liver: Eight patients had pathologic
al-terations of the liver described as fatty
metamorphosis in two, fatty degeneration in
two, cirrhosis in three, and necrosis in one. Renal: Renal pathology was found in
eight children. This was characterized as
glomerulonephritis in two, cholemic
neph-rosis in one, fatty kidney in one, infarct
see-ondary to thromboembolism in one,
pyo-nephrosis in one, abscesses of the kidney in one, and hydroureter in one.
Infection: Heart blood cultures
demon-strated growth in 10 children and no growth in 2; in 2 no samples were obtained.
Gram-negative organisms predominated in the 10
positive cultures; 7 being of the cohi-aero-genes group.
REPRESENTATIVE
CASE REPORTS
PancreaEtis Associated with the
Use of Steroids
Three patients were given ACTH and
adrenal corticoids for cirrhosis in two
in-stances and for the nephrotic syndrome in
the third. These medicaments were
heart Illooel (allure
llemolyticstreptocoecus;
I’rotells vulgaris;
Scm-oi)actem aerog(’nes Aerobacter aemugenes No growth liacteriunl (lklniig(lles Not (lOne Not (l((fle Stapilylocrus tureus Etiology C’ase 1.esions
Pancreas Kidney 1.irer lung
Steroid treated I Acute pancreatitis with fat necrosis
Glomerulo-nephritis Fatty meta-morpliosis ilronchopneu-monia .‘3
Acute & chronic interstitial
pan-creatitis
Acute hemorrhagic pancreatitis
Fatty meta-morphosis (‘holemic nephrosis l”atty meti-morph((sis (‘irrhosis . . (‘ongenital anomalies 4 S
Acute pancreatitis with fat necrosis
Acute & chronic pancreatitis with
atrophy Infarmts Pylonephritii, (‘irrhosis Fatty degen-emation lnfarmts .
Postoperative 9 Acute necrosis Abscess;
hydro-nephrosis
Ascites
Hrotldlopneu-monia
Periarteritis
nodosa
I I Acute interstitial pancreatitis
Glomemulo-nephritis
. . .
Intestinal
obstruc-tion
11 Acute interstitial pancreatitis llydro(lreter .
lironchopneu-monia Malnutrition ((nIl infection 13 14 IS 16 17
Acute interstitial pancreatitis
l”ibrosis
Acute interstitial pancreatitis
Acute interstitial pancreatitis
Acute atrophy . . . . . . . . . Fatty (legen-emation Fibrosis liepatomegaly Necrosis . . Empyema . Interstitial pneumonia Ate!ectasis Atelectasis
18 Acute &chronic pancreatitis . . .
Bronchopneu-monia
TABLE II
POSTMORTEM FINDINGS IN PATIENTS WITH ACUTE PANCREATITIS, NEV O1tK llOSI’ITAL-(’ORNELI. MEI)I(SL
CENTER, 1932-1962
and 2 weeks in the respective children. All
these patients had elevated blood urea
ni-trogen and albuminuria. Two patients had
reduced total proteins with a reversal of
al-bumin-globulin ratio. The following case
history is illustrative of these children.
Case 1
A 4-year-old white girl was admitted to
The New York Hospital on December 10,
1952, because of anasarca. One year earlier ACTH (12.5 mg, q 6ii) had been
adminis-tered for 10 days. Two months prior to
ad-mission, cortisone (200 mg daily) had been
given for 10 days. Examination revealed a
pale, edematous child with orthopnea,
pen-orbital swelling, venous distention, and
as-cites. Her pulse was 108/minute; blood
pressure was 140/60 mm Hg. She had
3-plus albuminunia; calcium in serum, 5.8
mg/100 ml; phospilorus, 6.8 mg/100 ml;
I’roteus vulgaris:
Acm-looter aemogenes:
noi(-Iiemolytimstmeptococcus llenolytie sta)hylococ-((J5 aume(Js I.scl(emiml(Ia (‘(Ii No growth Fsrherir1na (oil
lsmil. (Oil, non-I(eno)iy-tic staph. aureus
Esll. O)li,
non-henu)ly-tim streptococcus
blood urea nitrogen, 56 mg/100 ml; and a
total protein content of 3.06 gm/100 ml,
with 1.45/1.63 albumin-globulin ratio. Her
serum cholesterol was 600 mg/100 ml.
Silortly after admission paracentesis was
performed, with the removal of 7,000 ml
of fluid. Penicillin, tetracycline, and ACTH
(25 mg, q 6h) tilerapy was begun. Eight
days after admission she experienced
con-vulsions lasting up to 2 ilourS. She became
progressively more lethargic and died about
1 month after admission. Postmortem
cx-amination demonstrated glomerulonephnitis, anasarca, fatty metamorphosis of the liver,
bronchopneumonia, and acute pancneatitis with fat necrosis.
Continent: The findlings of pancreatitis in
tiliS cilild agree witil the observations of
Carone and Liebowe and Oppenheimer and
Boitmott7 who at postmortem studies found
dis-(51SC in individuals treated with SterOid!s
than in a random control group of patients
‘ho Ilad! not had! steroid therapy.
Pancreatitis Associated with Congenital Anomalies
Three cilildrefl had congenital anomalies
involving the pancreas as well as tile
pan-creatic and! biliary c!uctal systems. These
were atresia of the pancreatic d!ucts with
a nOrITlal CO1llOll bile duct; atresia of the
COITIOIl bile dlilct with a normal I)1ncretltic
diuct draining into the d!uodenum
sepa-rately; alld! annular pallcreas for which a
duodellojejunostomy had been done 5 years
prior to the onset of pancreatitis. The first
t\V() pitients died!, while tile last survived.
Case 5
One of tile cllildrcn who died was a
3-year-Old! \vllite i)oy \Vitil a history of
feed-ing problems since birth, irritability, and!
mental retardation. He was admitted! to The
New York Hospital (November 15, 1937)
l)ecause of watery d!iarrhea and! cloudy
urine of 1 week’s dluratiOfl. On exammation
ild \VlS irritable, mentally retarded,
under-developed!, and! malnourished. He weighed
8.5 kg. His spleen and liver were palpable
and the anal area excoriated Laboratory
studlies revealed! 3-plus albuminuria,
erytil-rocyte count of 3,300,000, audI leukocyte
coittlt of 41,400. His course in the hospital
was marked! I)V Persistellt vonsiting alld
progressive deterioration. Three d!ays after
ad!mission he died. Postmortem examination
revealed pyonephrosis, pyotireter, fatty de-generation of tile liver, bronclsopneumonia,
and chronic interstitial pancreatitis with
stenosis of the main and accessory
pan-creatic dlucts. The acinar tissue of the
pan-creas was atrophied. Tile hepatic and
com-mon ducts were normal.
Coniment: The und!erlying problem in
this child! was probably malnutrition dIne
to deficiency of pancreatic enzymes. The
obstruction d)f tile pancreatic ducts
tin-diOubtedly led to atrophy of the pancreas.
Pancreatitis Associated with Mumps
Pancreatitis as a complication of mumps parotitis is a benign disease. Both patients
encountered! in our series survivec! without
se(Iuella. The course of tile disease is
illus-trated l)y the following case.
Case 7
A 10-year-old! white boy was admitted to
The New York Hospital on February 15,
1962, Vitll nausea, vomiting, and abdominal
pam. Parotid swelling due to mumps had
subsided 3 days earlier. On examination ile
was a well-developed, well-nourished child
with normal vital signs. Tilere was slight
tenderness in the right lower quadrant and
periumbilical regions. Laboratory studies
revealed a normal urinalysis; hemoglobin
concentration, 12.2 gm; leukocyte count,
9,200; serum amylase, 585 units; calcium in
serum, 12 mg/100 ml; and pilosphorus,
4.3/100 ml. The abdominal tenderness
sub-sided rapidly over the first 24 ilours
follow-ing admission. Tile serum amylase fell to
338 Somogyi units. After 2 more days his
amylase was within normal limits, he was
free of abdominal complaints, and was
dis-cilarged. Two days later he ilad a
recur-rence of abdominal pain, nausea, and
vomit-ing, witil elevation of tile serum amylase.
\Vithin 24 hours these symptoms sul)sided!
and the amylase fell to normal. He has had
no further difficulty.
Comment: Our two patients, like others
reported in the literature, had a short,
i)enign course of pancreatitis that had its
onset shortly after the subsidence of mumps
I1rotitis. These patients are relatively easy to manage and recover without serious corn-plications or sequehla.
Pancreatitis following Abdominal Surgery
Pancreatitis in the postoperative period is
infrequent hut can be a serious
complica-tion of abdominal procedures. Two such
cases were encountered in cilildren at The
New York Hospital, one of wilorn survived.
other biopsy of the left adrenal gland. The
course of the latter patient, who died, is de-scribed below.
Case 9
A 4-year-old girl who had had a 35-lb
(15.9 kg) weight gain during a 6-month
pe-nod was admitted to The New York
Hos-pital for diagnostic evaluation. On
exam-ination tile cilild weighed 28.8 kg, the
pulse was 130/minute, respirations 50/
minute, and blood pressure 150/90 mm
Hg. Laboratory studies revealed
aibumi-nuria; hemoglobin, 14 gm; calcium, 12
mg/100 ml; and phosphorus, 7 mg/100 ml.
Following these determinations, the patient
underwent exploration of the left adrenal
gland and biopsy of the pancreas.
Tachy-pnea, fever, anorexia, and basilar rales in
the left lung were noted postoperatively.
There was purulent drainage from the
wound, and despite all measures the patient died 28 days after surgery. Autopsy revealed
an abscess of the right kidney, congenital
stenosis of the left ureteral orifice with hy-dronephrosis of tile left kidney and bilateral
bronchopneumonia with hydrothorax. There
was hyperplasia of tile eosinophilic cells in
the pituitary gland. The tail of the pancreas
was embedded in a thick mass of
inflam-matory reaction with necrosis. There was a
marked degree of fat necrosis in the
sur-rounding area.
Comment: In this instance, pancreatitis
undoubtedly developed as the result of
in-fection at tile biopsy site. Whether the
a!)-scess in the kidney was secondary to
seed-ing via the blood stream following infection in the pancreas or whether the pancreatic infection was secondary to the renal abscess
cannot be stated. Injury to the pancreas at
the time of abdominal surgery, especially
splenectomy, may give rise to pancreatitis.
Pancreatitis Associated with Periarteritis Nodosa
Thrombosis of pancreatic blood vessels
occurs with periarteritis nodosa.8 One such
case was encountered in Tile New York
Hos-pital.
Case 11
A 5-year-old white boy had developed
angioneurotic edema, hives, back pain,
lethargy, nausea, and vomiting 12 days
prior to admission to The New York
Hos-pita! on Marcil 5, 1962. Examination vealed him to be acutely ill and lethargic
vith facial and peripheral edema. He had
abdominal distention and right upper quad-rant tenderness with rebound tenderness. His temperature was 38.3#{176}C, pulse 120/
minute, respirations 24/minute, and blood
pressure 145/110 mm Hg. Laboratory
studies demonstrated albuminuria. Many
erytilrocytes and leukocytes per high-power
field were found on microscopic
examina-tion of the urine. The hemoglobin
concen-tration was 12 mg/100 ml, leukocytes,
18,000; calcium in serum, 5.8 mg/100 ml;
phosphorus, 11.6 mg/100 ml; and blood urea
nitrogen, 134 mg/100 ml. Treatment with
chioromycetin, digitalis, magnesium
sul-phate, priscoline, gamma-globulin, and
whole blood transfusion was begun, but
there was little improvement. Hypertension increased. Convulsions, fever, pleural
effu-sion, and ascites developed, and ile died
April 26, 1952. Postmortem examination
showed polyarteritis nodosa with glomeru-lonephritis, infarcts of the adrenal gland and spleen, and acute pancreatitis
charac-terized by interstitial edema and
polypmor-phonuclear leukocytic infiltration. There
was also peripancreatic inflammation,
des-quamation of ductal epithelium, and
muco-purulent ductal plugs.
Comment: Pancreatitis in this patent was
probably related to relative hypoxia or
is-chemia as a result of interference with blood
supply secondary to the polyarteritis
no-dosa.
Pancreatitis Due to Intestinal Obstruction
Intestinal obstruction has been postulated
al. There was one patient with pancreatitis
and intestinal obstruction in The New York
Hospital series.
Case 12
A 5-day-old white boy was transferred to
The New York Hospital on the fourth day
of life August 8, 1946, because of intestinal
obstruction. On examination he appeared
dehydrated and had abdominal distention.
At exploration he was found to have
in-tussusception of tile ileum proximal to the
ileocecal valve. An illeocolostomy and
sigmoid colostomy were performed, but
the patient died. Postmortem study
re-vealed intestinal obstruction, peritonitis, bronchopneumonia, atresia of the galiblad-der, and acute pancreatitis. The pancreatic
ducts were filled with mucopurulent plugs
and the stoma was infiltrated by
polymor-phonuclear leukocytes. Cultures of heart
blood grew out proteus vulgaris, aerobacter
aerogenes, and non-hemolytic streptococci.
Comment: The development of
pancre-atitis by intestinal obstruction has been
demonstrated experimentally and clinically by Pfieffer Ct al.b0 and Dreiling et al. This case would appear to fall into this category.
Pancreatitis of Unknown Etiology
In 6 of the 18 patients in The New York
Hospital series, a specific etiology for the
pancreatitis could not be determined.
How-ever, all of them had a history of
malnutri-tion or infection. A brief description of
these cases follows:
Case 13
A 5-month-old white boy was admitted
to The New York Hospital December 1,
1936, because of vomiting and diarrhea of
4 days duration. He had had purulent otitis
media and poor appetite for 4 weeks.
While hospitalized, myringotomies were
performed, but the purulent otitis media
persisted. After a month of nausea,
vomit-ing, diarrhea, and weight loss, death
oc-curred on January 25, 1937. Autopsy
dem-onstrated bilateral otitis media, mastoiditis, pericarditis, myocardial abscess, left fibrino-purulent pleurisy, fatty degeneration of the
liver, and acute pancreatitis. The
pancre-atitis was characterized by the presence of
many interstitial polymorphonuclear
leuko-cytes, large distended ducts filled with
pink-staining homogenous granular
mate-rial, and intralobular fibrosis.
Case 14
A 3-montll-old marantic girl weighing
3 lb 7 oz (1,559 gm) with congenital syphilis
was admitted to The New York Hospital
April 27, 1935, for treatment with mercury
and neoarsphenamine. She developed
diar-rhea and died June 3. Postmortem
examina-tion demonstrated emaciation, osteitis of
the ribs, congenital syphilis, and fibrosis of the liver and pancreas.
Case 15
A 2-month-old white boy was originally
admitted to The New York Hospital
Sep-tember 29, 1948, with hemorrhagic disease
of the newborn. The child fed poorly, then
experienced gagging and regurgitation of
his feedings, requiring parenteral
supple-ments. Persistent poor feedings followed in
spite of penicillin and streptomycin therapy.
The patient became cachectic and died. At
postmortem, pancreatitis and interstitial
pneumonia were noted.
Case 16
A 1-month-old white boy had a 4-day
history of vomiting and diarrhea prompting
admission to The New York Hospital,
Au-gust 24, 1945. On examination the child
was marasmic. Diarrhea persisted, and the
infant died on August 26. Postmortem
ex-amination showed subacute enteritis,
dif-fuse liver necrosis, and acute pancreatitis.
Heart blood culture grew Escherichia coii.
Case 17
A premature 1,520-gm 1-day-old boy died
1 hour after admission to The New York
Hospital February 10, 1943. Postmortem
examination showed encephalitis with areas
of necrosis and calcification secondary to
toxoplasmosis, myocarditis, orchitis,
infiltration with polymorphonuclear
leuko-cytes, and fibrosis.
Case 18
A 3-day-old white boy was admitted to
The New York Hospital May 15, 1948,
because of abdominal distention and
vomit-ing. At operation, on May 16, gangrene of
the ascending colon with perforation of the
cecum was noted. The infant died the day
of surgery. Postmortem examination
dem-onstrated perforation of the cecum and
transverse colon, fibrinous peritonitis,
pneu-monia and focal pancreatitis.
Comment: All of these infants might be
considered to fall into the classification “idiopathic pancreatitis”. However, in every
instance there was malnutrition and/or
in-fection, and it is likely that these contrib-uted to the development of the pancreatitis.
Case 12 might be confused with those
pa-tients with postoperative pancreatitis.
How-ever, this child died on the day of surgery,
and it is highly unlikely that the
pancreati-tis was a postoperative development.
Pan-creatitis in Cases 14 and 17 conceivably
may have been due to syphillis and toxo-.
plasmosis respectively.
COMMENT
As can be deduced from the cases
de-scribed above, pancreatitis in children may
result from several causes. Unfortunately,
in the very young, the process may be
either unsuspected or an accompaniment
of severe widespread involvement of other
organs including the liver and kidneys.
Thus, in 14 of 18 instances, the disease was
not recognized until postmortem
examina-tion. In four of the patients, all over 5 years
of age, the diagnosis of pancreatitis was
made on the basis of signs, symptoms, and
laboratory studies. All of these children
survived following accepted methods of
treatment.
The association of steroid administration
with pancreatitis was documented by
Carone and Liebow6 in 1957 and confirmed
by Nelp,11 Sash,12 and Oppenheimer and
Boitnott.7 That the disease condition for
which the steroid is administered iiay also
be an important factor in the development of pancreatitis is less well understood.
Op-penheimer and Boitnott7 showed that
whereas 15% of children with renal disease
had evidence of pancreatitis at autopsy,
the incidence increased to 40% in a similar
group of children with renal disease who
received steroids. A group of leukemic
chil-dren without steroid therapy showed no
pancreatitis at postmortem. In a comparable series of leukemic children who did receive
steroids, 16% showed evidence of pancreati-tis at autopsy.7 It is interesting to speculate
why children with renal disease incur pan.
creatitis even without steroid administra-tion, while leukemics do not. The cases are
too few to permit conclusions, but there is
the suggestion that renal disease may
pre-dispose to pancreatitis. In the three
chil-dren at The New York Hospital who had
received steroids, one had kidney disease
and the other two had liver disease. Liver
disease, like kidney disease, may predispose
to the development of fatal pancreatitis in
the setting of steroid administration. The
mechanism of steroid pancreatitis is not
understood. Alteration of protein metabo-lism and the frequent appearance of
eosino-philic plugs in the lumina of pancreatic
ducts and acini noted at postmortem study
require further inquiry.
Congenital anomalies may cause
pancre-atic duct obstruction leading to the devel-opment of pancreatitis.13 Tilis is probably rare in association with annular pancreas,
despite the fact that this did appear to be
the case in one of our patients.
Mumps pancreatitis was recognized as
an entity following Farnhour’s report14 in
1922. Of 12 cases of mumps pancreatitis
seen at The New York Hospital since 1932,
only 2 occurred in children under the age
of 10 years. Manifestations of pancreatitis usually occur 3 to 4 days after the subsid-ence of the parotitis. In our two cases the
diagnosis was made on the basis of history,
clinical findings, and elevation of the serum
amylase. Some doubt has been cast on the
pan-creatitis in tile absence of an elevated
serum lipase5 because serum amylase values are usually elevated in 80 to 90% of patients
with the disease.’5 One of our patients in
whom the diagnosis of mumps pancreatitis
was made is of interest in this regard. On
admission serum amylase values were
dc-vated. These fell to normal within a few
days, and he became asymptomatic. Two
days later he had an exacerbation of his
previous symptoms and serum amylase
levels became elevated once more.
Postoperative pancreatitis is less frequent in children than in adults, probably because operations involving the biliary tract,
stom-acll, and pancreas are less frequently
per-formed in tile former. The mechanism for
development of pancreatitis in this
situa-tion is probably direct trauma to the
pan-creas at the time of surgery. This form of
pancreatitis can probably be avoided by
taking care not to injure the pancreas when operations are performed on it or adjacent
structures.
The one instance of polyarteritis nodosa
in our series bears out the experimental
observations that vascular necrosis and
thrombosis of arteries and arterioles
supply-ing tile pancreas can augment or initiate
17 Cecil and Loeb have
stated that 65% of patients with periarteritis
nodosa suffer from abdominal pain and that
tile attacks of pain often simulate those of
llemOrrllagic pancreatitis . The patilologic lesion in periarteritis nodosa and produced
experimentally by Rich and Duff involves
fibrinoid alteration and hyalinization of the
media of medium sized vessels and in some
cases small arterioles. Changes in pancre-atitis associated witil this disease may be
produced by immunologic reactions,
prob-ably mediated by necrosis and thrombosis of arteries and arterioles.
Intestinal obstruction is known to
pro-duce elevation of the serum amylase.#{176}
Hemorrhagic pancreatitis in dogs has been
produced by Pfieffer et ai.b0 by closed
duo-denal loop obstruction. Dreiling et al.
suggested that afferent loop obstruction
following subtotal gastrectomy could
pro-duce pancreatitis and documented this with
case reports. The newborn infant with
in-tussusception of the ileum and obstruction
may represent a variation on the proposal
of Dreiling et a!.
In six of the cases in our series, the eti-ology of pancreatitis remains unclassified.
These would appear to fall in the
“idio-pathic” group. However, in all of these
children there was a history of
malnutri-tion and/or infection. Information based on
experimental, postmortem, and clinical stud-ies suggest that malnutrition, specifically
inadequate protein intake complicated by
or secondary to infection, is capable of
producing pancreatic lesions.
Veghelyi et al.20 noted that infants on a
diet lacking in protein had decreased
cx-cretory function of the pancreas and
de-veloped “fatty” liver. Based on autopsy
studies, pancreatic failure developed within
7 to 14 days in the face of a poor protein
intake, and “the younger the subject, the
earlier the failure.” In these cases, the cells of the acini of the pancreas had decreased
cytoplasm and few granules. The pancreas
in children who survived for longer periods
with continued poor intake of protein
con-tamed eosinophilic plugs blocking the
lumina of dilated acini. Fibrosis appeared
at an even later stage. Experimental work
in rats by Vegheli et a!.#{176}and Wachstein
and Meisel21 and subsequently by
Mc-Phedran and Lucas22 confirmed these
find-ings in those animals with a diet lacking
protein or methionine.
Ethionine, a methionine analogue, is
re-ported to interfere with phospholipid
trans-port of trypsinogen across the cell
mem-brane barrier.23 Accumulation of large
amounts of trypsinogen within tile cell is
reported to lead to activation of some
trypsin within the acinar cell leading to
its destruction.23 Whether the pancreatic
changes seen in ethionine pancreatitis can
be produced by methionine lack alone is
open to question.2326 Ethionine pancreatitis
may not represent a simple methionine
lack, for large amounts of methionine are
Kwashiorkor,2 a protein deficiency dis-ease, produces pancreatic lesions similar to
those noted in protein and methionine
de-222 28 The history of the
develop-ment of kwashiorkor strikingly resembles
that of the “idiopathic” pancreatitis seen in
tile children in our series, thus emphasizing
the importance of the role of malnutrition and infection in the etiology of pancreatic
fibrosis and atrophy. According to Trowell
and Jelliffe,29 in kwashiorkor there is a his-tory of poor protein intake, then a
“respira-tory infection, or attack of diarrhea, or
fever leads to a grave reduction in food
intake and the child becomes manifestly
ill.” The child “may never seem to recover
from his infection.” Lesions noted at
post-mortem examination of children dying of
this disease are atrophy and fibrosis of tile
pancreas, and fatty liver often associated
with cirrhosis.2’ 29
In considering factors affecting mortality in patients with pancreatitis, the association of liver and renal disease is evident in The New York Hospital series. In 11 of 14 chil-dren wilo died, renal and/or liver disease
was demonstrated at postmortem study.
The appearance of liver or renal
dysfunc-tion in patients with pancreatitis augurs a
poor prognosis.28’ 30-37
Whether pancreatic disease is capable
of producing renal and/or hepatic lesions
is less clearly established. Liver changes
may result from pancreatic enzymes
made-quate to digest protein in sufficient amount
to supply hepatic demands, producing fatty
liver or 20, 33, 34 Renal changes may
follow septicemia, shock, hypovolemia,
hy-poproteinemia, or the destructive effect of
circulating pancreatic enzymes, producing
tubular cllanges similar to tilose associated
with gram negative septicemia or ethionine
pancreatitis.
SUMMARY
Eighteen children with acute pancreatitis,
not including those with cystic fibrosis of
the pancreas, were encountered at The
New York Hospital from 1932 to 1962.
Steroid administration, congenital
anom-a!ies, mumps, operative trauma, periarteritis
nodosa, intestinal obstruction, and
malnu-trition and infection were thought to be
factors in the etiology of acute pancreatitis in these patients. The ages of the children
ranged from 1 day to 10 years. Fourteen of
tile children were male and four female.
Fourteen patients died. Significant
post-mortem findings included kidney and renal
lesions in addition to pancreatitis. Survivors
were children 5 to 10 years of age whose
pancreatitis was attributable to mumps,
operative trauma, and annular pancreas.
Liver and renal dysfunction in patients
with pancreatitis appeared to augur a poor
prognosis. All patients thought to have
“idiopathic” pancreatitis had a history of
poor nutrition and/or infection, which
prob-ably are etiologic factors in the develop-ment of pancreatitis in these children.
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