Fibrocystic disease of the pancreas

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.