Acute pancreatitits uncommon manifestation of cystic fibrosis? A case report

The pancreas is one of the primary organs affected by dysfunction of the cystic fibrosis transmembrane
conductance regulator protein. While exocrine pancreatic insufficiency is a well-recognized complication
of cystic fibrosis (CF), symptomatic pancreatitis is often under-recognized and occurs approximately in
20% of patients with CF and almost exclusively in pancreatic sufficient people. This is a case of acute
pancreatitis in a ten-year-old boy in whom cystic fibrosis was suspected. Ten year old boy presented with
stomach pain and vomiting for three days. On physical examination afebrile, conscious, dehydrated,
occupies a forced position in bed. Abdomen was very painful on palpation epigastrically and
periumbilically without organomegaly. Blood tests: Glycose= 8,4..11,4mmol/l, amylase=
147..222..660..800U/L, lipase=141..288..465..501U/L. Urine test positive for ketones, amylase in urine=
481..1302..8667U/L. Ultrasound and CT of abdomen with normal findings. The child was treated with
parenteral rehydration, Amp. Ceftriaxon, Amp. Pantoparzole and subcutaneous somatostatin. Due to the
persistence of elevated pancreatic enzymes, a sweat test was performed with high chloride values of 93..90
mmol/l. Chest X ray with normal findings. Tracheal aspirate was negative. Control laboratory analyzes
with low inflammatory markers, but still with elevated pancreatic enzymes. Due to positive sweat test on
two occasions and the manifestation of acute pancreatitis, CF was suspected and a blood sample was sent
for genetic analysis. Symptomatic pancreatitis is uncommon manifestation in CF because it occurs
exclusively in pancreatic sufficient CF patients. Newborn screening and improved panels of DNA mutation
techniques are revealing more rare and nonclassical pictures of the disease, generally associated with
pancreatic sufficiency and with an increased risk of developing pancreatitis.