Abstract
(01). The literature of fibrocystic disease of the
pancreas is reviewed.
(02). Six proved cases and one unproved case are
described in regard to the clinical picture, treatment,
pathology, bacteriology, biochemistry and
radiology.
(03). The unselected cases represent an incidence
of 1.7% of all hospital admissions among children
and it is emphasised that the disease is therefore
not uncommon.
(04). The evidence points to the disease being inherited
and inheritance by an incomplete dominant
character is tentatively suggested.
(05). The results of pathological examination in
the three post-mortems fully studied are in agreement
with previous work that this disease affects
mucus-secreting glands generally in the body.
(06). The view that inspissation of viscid mucus
in these glands causes the pathological lesions
throughout the body is considered to be consistent
with the findings.
(07). The importance of an accurate history is
stressed.
(08). It is suggested that cases in group 2 be
divided into nutritional and respiratory sub-groups,
depending on the presenting symptom and that cough
does not necessarily indicate infection.
(09). the chances of successful duodenal intubation
at any one attempt are considered to be about one
in three.
(10). Fibrocystic disease of the pancreas as
generally regarded may not be the only condition
(excluding congenital syphilis) in which trypsin is
absent from the duodenal juice in children.
(11). Reference is made to two cases in adults who
have suffered from steatorrhoea since birth, but
or other
who have no signs of pulmonary infection, and in
whom trypsn is absent from the duodenal juice.
(12). It is suggested that these cases may be cases
of fibrocystic disease of the pancreas in which the
lungs are not affected. if this is so, they should
be separated from those cases in which the disease
is systemic in distribution.
(13). Nevertheless absence or virtual absence of
trypsin has been considered essential for diagnosis
in the cases in this study as well as the presence
of the typical clinical picture and steatorrhoea.
(14). Fat in the faeces is well split in this disease
and it is agreed that estimations of the total fat
only are of value.
(15). Unless this disease is borne in mind as the
possible cause of any chronic nutritional and/or
respiratory disturbance in children it will be frequently
misdiagnosed.
(16). The prognosis is considered to be bad.
(17). where is insufficient evidence to show
that variations in the severity of the lesion
determine the prognosis.
(18). Present treatment is considered to be
unlikely to produce more than temporary improvement
and to exert little control over the course
of the disease.
(19). It is suggested that animals, in whom the
lesions of this disease have been produced, be
treated with secretin and the results observed.
(20). If the results are satisfactory, the
treatment should be extended to children, but it
is considered that only treatment from an early
age and throughout life would be efficacious.
