8

Pancreatic diseases(excluding cystic fibrosis)

JACQUES SARLES MD
Professorof Paediatrics
Hapita

dEnfants

de la Timone,

13005-Marseille,

France

Except for cystic fibrosis, which is the most frequent genetic disorder in the Caucasian
population, diseasesof the exocrine pancreas are relatively uncommon in children.
However, they are many and varied in terms of their pathogenesis and clinical manifestation. They can be classifiedas: (1) congenital anatomical abnormalities, (2) congenital
secretory insufficiencies, and (3) pancreatitis. In all of these diseases,when pancreatic
insufficiency (whether partial or complete) is present, the nutritional statusof the patients
must be investigated regularly, and pancreatic enzymes as well as nutritional supplementations must be prescribedas soon asmalnutrition is present,or even prophylactically.
The preservation of good nutritional statusis the guaranteeof a better prognosis.
Key words: pancreas;pancreaticinsufficiency; nutrition; children.

Discounting cystic fibrosis-the

most frequent genetic disorder in the
Caucasian population-diseases
of the exocrine pancreas are relatively
uncommon in children. They are, however, many and varied in terms of
their clinical manifestation and pathogenesis. They can be classified into:
(1) congenital
anatomical
abnormalities,
(2) congenital
secretory
insufficiencies, and (3) pancreatitis (Table 1) (Sarles, 1993; Durie, 1996;
Lerner et al, 1996). They do not always induce pancreatic insufficiency (PI)
and/or malnutrition, so nutritional maintenance or catch-up may be the
focus of medical concern or may be completely unnecessary. In this
chapter, we will review all these diseases (except for cystic fibrosis),
emphasizing the nutritional aspects when appropriate.

CONGENITAL ANATOMICAL

ABNORMALITIES

An annular pancreas consists of a flat band of pancreatic tissue encircling

the second portion of the duodenum. It is a rare abnormality, more
frequently associated with Downs syndrome. A neonatal occlusion may be
the initial presentation when very tight, but the condition usually causes
various dyspeptic disorders, peptic ulcer symptomatology or even acute
pancreatitis later on in life. Barium meals or upper gastrointestinal tract
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J. SARLES
Table 1. Classification of pancreatic diseases in children.
Congenital

anatomical

abnormalities

Formation abnormalities
Annular pancreas
Pancreas divisum
Hypoplasia and agenesis
Situation abnormalities
Heterotopic pancreas
Congenital

secretory

insufficiencies

Isolated enzyme deficiencies

Lipomatosis
Shwachman-Diamond syndrome
Johanson-Blizzard syndrome
Mitochondrial cytopathies
Pearson syndrome
Pancreatitis

Acute pancreatitis
Chronic pancreatitis
Chronic obstructive pancreatitis
Calcic litbiasis - nutritional (tropical) pancreatitis
- familial hereditary pancreatitis (accessory form)
Proteic lithiasis - cystic fibrosis
- familial hereditary pancreatitis (main form)
- idiopathic pancreatitis
Chronic inflammatory pancreatitis

endoscopy make the diagnosis, and surgical bypass is the recommended

approach. Malnutrition can be the result of chronic vomiting or PI (Sarles
et al, 1993). In the first case, surgical repair should lead to a rapid catch-up.
However, the possibility of PI (perhaps due to chronic pancreatitis) should
be evaluated and, when present, compensated for by pancreatic enzyme
replacement therapy.
Pancreas divisum and heterotopic pancreas may be the cause of acute
pancreatitis but have not been shown to be associated with PI or malnutrition.
Agenesis of the pancreas is extremely rare and is usually incompatible
with life. Hypoplasia may induce PI when it reduces functional exocrine
tissue to a level less than l-2% of normal.
CONGENITAL

SECRETORY

INSUFFICIENCIES

Isolated enzyme deficiencies

Isolated deficiencies of exocrine pancreatic enzymes have been described,
but all of them are extremely rare. The enzymes affected include lipase,
lipase-colipase, colipase, trypsinogen and amylase (Durie, 1996). When
lipid digestion is involved, the main symptom is malnutrition with chronic
diarrhoea, and steatorrhoea is readily documented (Sheldon, 1964). Both of
the children with trypsinogen deficiency described by Townes presented
with growth failure, hypoproteinaemia
and oedema. Finally, starch

PANCREATIC

DISEASES

801

ingestion induces diarrhoea in children with amylase deficiency, but there

is no evidence that the condition is permanent (Lowe and May, 195 1).
Pancreatic enzyme replacement therapy is very effective in all these
patients.
Pancreatic

lipomatosis

Two syndromes have been described, which have in common an extensive

fatty replacement of the pancreatic acinar tissue with normal ductal
architecture. Pancreatic function studies confirm the presence of markedly
impaired enzyme secretion but preserved ductal function (Hill et al, 1982;
Jones et al, 1994).
The PI of Shwuchman-Diamond
syndrome is associated with haematological alterations (neutropenia, red cell hypoplasia, thrombocytopenia and
lympho- or myeloproliferative
malignancies), skeletal abnormalities (short
stature, thoracic dystrophy, metaphyseal dysostosis) and various other
manifestations (psychomotor delay, hypotonia, hepatomegaly, elevated
transaminase levels, dental abnormalities, endocardial fibrosis, renal
tubular acidosis and diabetes mellitus) (Aggett et al, 1980). PI in this
syndrome may improve with time in 50% of the patients (Hill et al, 1982).
In Johanson-Blizzard
Syndrome, PI is associated with aplasia of the
nasal alae, deafness, hypothyroidism,
absent permanent teeth and scalp
defects. Developmental delay and genitourinary, cardiac and anorectal malformations may also occur. Death in infancy due to severe malabsorption is
not infrequent. Whether patients with this syndrome show improvement of
pancreatic function with advancing age has not been determined (Durie,
1996).
Pearsons syndrome
In this syndrome, there is exocrine pancreatic dysfunction and a refractory
sideroblastic anaemia with variable degrees of neutropenia and thrombocytopenia. Qualitative pancreatic function tests reveal depressed acinar
function as well as reduced fluid and electrolyte secretion. The pancreas is
of a normal size but shows an acinar atrophy with fibrosis. It is now clearly
established that Pearsons syndrome results from a mitochondrial
respiratory enzyme defect (Cormier et al, 1990). This explains the nonMendelian transmission of the disorder and the presence of abnormalities in
the oxidoreduction status, which are of great help in making the diagnosis.
PANCREATITIS
Acute pancreatitis
Acute pancreatitis is the most frequent form of pancreatitis in children
(Sarles, 1993). It occurs in a previously normal organ and involves a
spectrum of inflammatory
lesions in the pancreas and peri-pancreatic

802

J. SARLES

tissues. The condition may be recurrent, but if the primary cause and
complications such as pseudocysts are eliminated, clinical, morphological
and functional restitution occurs. Exceptionally, when scarring of the main
duct persists, chronic pancreatitis may result caudal to the stenosis. The
main causes of acute pancreatitis in children are abdominal trauma, general
disorders, infections and drugs, but between 20% and 25% of cases remain
idiopathic.
During the acute phase of acute pancreatitis, there is a generalized hypermetabolic response that enhances caloric needs. In contrast, putting the
pancreas to rest is commonly advised, although whether this can change
the course of the disease has never been established (Steinberg and Tenner,
1994). These two conflicting approaches can induce a nutritional depletion
leading to malnutrition and septic complications. Thus maintaining a good
nutritional status despite the patients inability to be fed orally seems to be
of great importance. Artificial nutrition provides the best way of attaining
this goal (Manrlendra and Kirby, 1995). In moderate forms of acute
pancreatitis, enteral feeding can be tried, preferably using elemental diets.
In severe forms, total parenteral nutrition is required. A high-energy, highnitrogen supplement is recommended.
Lipid infusion has several
advantages (low osmolarity, high energy value and inhibition of gastric
acid secretion), whereas carbohydrates have numerous drawbacks (high
osmolarity, carbon dioxide overproduction and an increased need for
insulin, inducing lipogenesis and glyconeogenesis).
Chronic

pancreatitis

Chronic pancreatitis is defined by the presence of chronic inflammatory

lesions characterized by destruction of the exocrine parenchyma, fibrosis
and, at least in the late stages, destruction of the endocrine parenchyma. It
is frequently complicated, during the first stages of its evolution, by painful
exacerbation, which may be confused with recurrent acute pancreatitis.
Different forms of chronic pancreatitis may be classified as follows (Sarles
et al, 1989, 1992):
1.

2.

3.

Chronic obstructive pancreatitis

is caused by occlusion of the
pancreatic ducts prior to the onset of pancreatitis. In children, occlusion
is mainly a result of congenital anomalies or, exceptionally, of scars
from necrosis or necrotic pseudocysts. Calcification is never observed.
Proteic Zithiasis is the main cause of chronic pancreatitis in children
since it is represented by cystic fibrosis and the main form of hereditary
pancreatitis. Calcification
may appear secondarily (especially in
hereditary pancreatitis) but is not the rule.
Culcic lithiusis (mainly resulting from excessive alcohol intake in
adults) is responsible in children for rare forms of hereditary
pancreatitis and for nutritional (tropical) pancreatitis. Diabetes is
particularly frequent and precocious in this form of the disease.

Chronic pancmatitis sooner or later results in pancreatic insufficiency,

which leads to failure to thrive, Calorie intake must be increased to

PANCREATIC

DISEASES

803

120- 150% of the recommended daily allowance through an unrestricted fat

diet with pancreatic enzyme supplementation. The guidelines for the
prescription of pancreatic enzymes are the same as those for cystic fibrosis
(see Chapter 9). Moreover, fat-soluble vitamin deficiencies occur in
pancreatic insufficiency. The recommended daily doses are 5ooO- 10 000 IU
vitamin A, 400-8001U
vitamin D and 50-2001U vitamin E. Vitamin K
10 mg weekly is recommended only for toddlers. Finally, diabetes must be
investigated regularly and, when present, treated by insulin.

CONCLUSION
In all the diseases of the exocrine pancreas that induce pancreatic
insufficiency (whether partial or complete), the nutritional status of the
patient must be checked regularly, and pancreatic enzymes as well as
nutritional supplementations must be prescribed as soon as, or even before,
malnutrition is present. The preservation of a good nutritional status
guarantees a better prognosis.

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Childhood

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Cormier V, Riitig A, Quartino AR et al (1990) Widespread multitissue deletions of the mitochondrial

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*Durie P (1996) Inherited and congenital disorders of the exocrine pancreas. Gustroenterologist
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Hill RE, Durie PR, Gaskin KJ et al (1982) Steatorrhea and pancreatic insufficiency in Shwachmans
syndrome. Gastroenterology
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Jones NL, Hofley PM & Durie PR (1994) Pathophysiology of the pancreatic defect in Johanson
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