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• W2331210318 abstract "Exocrine pancreatic insufficiency is common in patients with celiac disease (1,2), is thought to be related to the intestinal mucosal damage (3,4), and is usually reversible when the patient is placed on a gluten-free diet (1,5,6). Irreversible pancreatic insufficiency is rare and has been reported in elderly patients with celiac disease (7) and post mortem studies of patients with celiac disease (8). It is believed to be caused by prolonged lack of pancreatic stimulation and malnutrition (9,10). It is well known that prevalence of celiac disease is increased in the patients with insulin-dependent diabetes mellitus (IDDM), ranging from 3% to 7%(11–13). Adults with celiac disease commonly have liver enzyme abnormalities (14,15), and celiac disease is common among patients with idiopathic hypertransaminasemia (16,17). It is believed that the liver enzyme abnormalities are caused by hepatic steatosis (15,18). This case report describes a 17-year-old girl with the unusual combination of celiac disease, permanent pancreatic insufficiency, diabetes mellitus, and hepatic and pancreatic steatosis. CASE REPORT A 17-year-old Jewish Ashkenazi girl was admitted to the hospital because of severe weight loss and diarrhea. She was the only daughter of a healthy mother and a father suffering from diabetes mellitus type II and ischemic heart disease. A maternal grandmother had inflammatory bowel disease. The patient was born at term (birth weight, 3 kg) and, from 1 year of age, had experienced abdominal pain, abdominal distension, and diarrhea. She had menarche at 13 years of age with regular menses, and because complaints were intermittent, medical evaluation was not done. Three months before the patient's admission, she complained of vomiting, abdominal pain, abdominal distension, diarrhea, hematochezia, tenesmus, and a 14-kg weight loss. Physical examination revealed mild dyspnea, distended abdomen, and pitting edema +3 of legs. Sexual development was assessed as Tanner stage 5, and the rest of the physical examination was unremarkable. The laboratory results were as follows, and control ranges, when abnormal, are given in parentheses. Erythrocyte sedimentation rate (ESR) was 8 mm in the first hour, hemoglobin level 11.5 g/dL (12.0–16.0 g/dL), white blood cell count 14,100 cells/mm3 (72% neutrophils, 19% lymphocytes), mean corpuscular volume 73.9 fL (78–102 fL), platelet count 678,000 cells/mm3 (150,000–400,000 cells/mm3), glucose 463 mg/dL (70–105 mg/dL), urea level 13 mg/dL, creatinine level 0.7 mg/dL, total protein 5.0 g/dL, albumin level 2.9 g/dL (4.0–5.3 g/dL), calcium 8.1 mg/dL (8.4–10.2 mg/dL), uric acid 2.2 mg/dL, aspartate aminotransferase 64 IU (5–45 IU), alanine aminotransferase 52 IU (5–45 IU), gamma-glutamyltranspeptidase 187 IU (5–24 IU), total cholesterol 160 mg/dL, triglycerides 378 mg/dL (40–128 mg/dL), prothrombin time 61% (INR 1.37), partial thromboplastin time 21.3 seconds, and fibrinogen 286 mg/dL. The patient's urine sample tested positive for glucose and negative for blood and protein. Results from repeated stool studies for bacteria, parasites, and clostridium toxin were negative. Stool for occult blood tested positive. Serology for hepatitis A, B, C was negative, as was serology for cytomegalic and Ebstein-Barr viruses. Immunoglobulins were within normal range, and anti DNA, anti nuclear factor, anti cytosolic, anti centromer, anti islet cell, and anti insulin antibody levels were either negative or in the normal range. Alpha-fetoprotein and ceruloplasmin serum levels were normal. Antigliadin and antiendomysial antibodies were positive. An enlarged hyperechogenic pancreas without calcifications and an enlarged hyperecogenic liver, consistent with fatty infiltration, were shown by abdominal ultrasonography. Abdominal computed tomography (CT) demonstrated focal fatty infiltration of the liver and an enlarged pancreas consistent with fatty infiltration (Fig. 1). Upper gastrointestinal series were normal, including the terminal ileum. Gastrointestinal endoscopy revealed edema of the duodenum and small intestinal biopsy revealed complete villous atrophy, marked intraepithelial lymphocytic infiltration, and marked infiltration of the lamina propria consistent with celiac disease. Colonoscopy and large intestinal biopsy results were normal.FIG. 1.: Computed tomography shows severe hepatic and pancreatic steatosis.At the time of diagnosis, steatorrhea of 18 g/d stool fat was found. Pancreatic insufficiency was diagnosed by abnormal pancreatolauryl test and zero stool elastase-1 level. Result of the sweat test was normal, and none of the common cystic fibrosis mutations present in the Jewish population were identified. The patient was given total parenteral nutrition for 3 weeks, followed by a gluten-free diet, pancreatic enzymes, and subcutaneous insulin. On gluten-free diet, the patient's liver enzyme abnormalities disappeared, antiendomysial antibodies turned negative, but steatorrhea persisted with undetected stool elastase-1. On repeated abdominal CT 1 year later, the liver was normal, but there was complete pancreatic atrophy (Fig. 2). The patient still requires pancreatic enzyme supplementation and subcutaneous insulin despite adherence to a gluten-free diet.FIG. 2.: Computed tomography, 1 year after initiation of therapy, shows normal liver and pancreatic atrophy.DISCUSSION Pancreatic insufficiency in celiac disease is thought to be caused by reduced secretion of enterokinase due to the mucosal damage and is usually reversible on a gluten-free diet (1,5,6). Coexistence of congenital pancreatic insufficiency and celiac disease was recently reported (19), and irreversible pancreatic insufficiency in an adolescent was attributed to a combination of chronic understimulation of the pancreas and malnutrition (20). However, in a study of 52 patients with celiac disease, at least mild to moderate pancreatic insufficiency was completely independent of nutritional status (21). Fatty infiltration of the pancreas is commonly seen in other diseases involving the pancreas. It is an integral part of Shwachman syndrome (22,23) and is commonly seen in cystic fibrosis (24). In cystic fibrosis, the most common result of imaging of the pancreas is complete lipomatosis of the pancreas, followed by atrophic pancreas and cystic pancreas (24). Although ultrasonography, CT, and magnetic resonance imaging (MRI) were used for imaging the pancreas in patients with cystic fibrosis (24), MRI was able to demonstrate lipomatosis in all patients with Shwachman syndrome (n = 13). Ultrasonography was unable to demonstrate the pancreas in 3 patients (23). Pancreatic lipomatosis is also common in patients with diabetes mellitus (25,26) and has been reported in Johanson-Blizzard syndrome (27), in conjunction with long standing liver disease (28), and as an isolated finding (29,30). Furthermore, pancreatic lipomatosis, fibrosis, and atrophy are commonly found in patients with diabetes mellitus (26). Therefore, the pancreatic steatosis in our patient could be related to celiac disease and pancreatic insufficiency, to the patient's diabetes (25,26), or it could be an incidental finding (29,30). To the best of our knowledge, demonstration of pancreatic infiltration evolving into atrophy on CT has not been reported previously. Hepatic steatosis seems to be common when liver enzyme abnormalities are present in patients with celiac disease (15,18). The combination of abdominal pain, diarrhea, and liver enzyme abnormalities, with no evidence for inflammatory bowel disease, should bring celiac disease high on the differential diagnosis. In two recent reports, approximately 9% of patients with idiopathic hypertransamiasemia were found to have celiac disease (16,17). The abnormal transaminase level disappears on a gluten-free diet (14,15,18). In one series, 60 of 67 patients with celiac disease and abnormal liver enzymes had normal levels on a gluten-free diet (15). Steatosis was found in 5 patients, and chronic active hepatitis in 2 of 7 patients with celiac disease with persistent elevation of transaminases. Steatosis is usually reversible on a gluten-free diet (18,31–33), even when accompanied by liver failure (34). Because liver biopsy is recommended only if enzyme abnormalities persist while the patient is on a gluten-free diet, a liver biopsy was not performed in our patient. The association of celiac disease and IDDM is well established (11–13). Nevertheless, in a recent study (13), despite prevalence of 6.5% of celiac disease in patients with IDDM, the risk of IDDM was not increased in celiac disease. In our case, the lack of autoantibodies associated with IDDM suggests that the cause of diabetes may have been the longstanding celiac disease and continued pancreatic damage. This may be similar to pancreatic damage associated with chronic pancreatitis, where after 10 years of follow-up, 50% of the patients will need insulin therapy (35). In summary, we describe a patient with celiac disease with severe pancreatic and hepatic steatosis. Pancreatic insufficiency persisted, and the evolution to pancreatic atrophy and diabetes mellitus was documented for the first time, to the best of our knowledge. Future imaging of the pancreas in patients with celiac disease and pancreatic insufficiency may reveal the prevalence of pancreatic steatosis and atrophy in these patients." @default.
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• W2331210318 title "Celiac, Fatty Liver, and Pancreatic Insufficiency" @default.
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