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- What is the etiopathogenesis of FCPD?
The etiopathogenesis of FCPD remains elusive; however, various theories have
been proposed. FCPD has been reported predominantly from developing countries
where malnutrition is widespread. However, the cause and effect relationship
between the two is not established. Besides malnutrition, consumption of cassava
(Manioc esculenta) as a staple food in some part of the tropics was shown to be
associated with chronic pancreatitis and FCPD. The alkaloids linamarin and
lotaustralin present in cassava produce cyanide compounds which are detoxified
by sulfur-containing amino acids. These amino acids are deficient in individuals
with malnutrition; therefore, accumulation of cyanogens result in chronic pancre-
atitis. In addition, oxidative stress and genetic factors (SPINK 1) have also been
incriminated. Increased secretion of a putative peptide termed as pancreatic stone
protein has also been suggested for the development of pancreatic calcification.- What are the clinical manifestations of FCPD?
The classic triad of FCPD comprises of abdominal pain, steatorrhea, and diabe-
tes. Hyperglycemia is usually severe but is not accompanied with ketosis.
Microvascular complications are common; however, macrovascular complica-
tions are rare. This dichotomy is possibly due to lack of hypertension and ath-
erogenic lipid profile.- Why is ketosis uncommon in FCPD?
Diabetic ketosis is uncommon in patients with FCPD and has been reported in
<15 % of patients. Despite severe hyperglycemia, ketosis is less common
because of the presence of residual β-cell function, loss of α-cell function
(decreased glucagon), reduced availability of non-esterified fatty acids due to
lack of subcutaneous fat, and carnitine deficiency associated with
malnutrition.- What are the causes of pancreatic calcification?
Pancreatic calcification is seen in patients with chronic alcoholic pancreatitis,
primary hyperparathyroidism, FCPD, kwashiorkor, cystic fibrosis, hereditary
pancreatitis, and pancreatic tumor. Large intraductal calcification is character-
istic of FCPD, while small intraductal and parenchymal calcification is a fea-
ture of chronic alcoholic pancreatitis.- How to manage a patient with FCPD?
Patients with FCPD require management for hyperglycemia, exocrine pancre-
atic insufficiency, and chronic abdominal pain. For glycemic control, majority12 Diabetes in the Young