CLINICAL CHALLENGES AND IMAGES IN GI An Increasingly Uncommon Presentation of a Common Disease Justin Noroozian,1 Jason Welch,2 and Scott Larson2 1
School of Medicine, and 2Department of Gastroenterology and Hepatology, University of Texas Medical Branch at Galveston, Galveston, Texas
Question: A 46-year-old, white, male, prisoner of 16 years, presented with coagulopathy and lower extremity edema. He had no remarkable medical history, reporting overall good health until 2 years before admission. At that time, he had experienced gradual onset of loose stools, which have persisted intermittently since. He felt this to be worse with certain meals, particularly those high in carbohydrates. He reported an associated, unintentional weight loss of 30 pounds in the previous year. He denied any melena, hematochezia, hematemesis, abdominal pain, skin rashes, bleeding events, easy bruising, or other complaints throughout the course of his illness. There was no family history of bleeding diatheses. At the time of referral, he was on oral iron supplementation, which he stated was for anemia, a cause of which had not been identified. His first visit encounter with our institution was through the hematology tele-medicine clinic 1 month before hospital admission, at which time repeat coagulation laboratory tests were recommended to his prison unit. When these laboratory tests were confirmatory, he was admitted to the hospital for additional workup. His physical examination revealed mild, nonpitting, bilateral lower extremity edema, but was otherwise entirely normal. At the time of admission, his prothrombin time (PT) was >100 s, with an International Normalized Ratio (INR) of >13.5. Clotting factors II, VII, and X were all decreased, suggesting a vitamin K deficiency. Because he resides in prison, the hematology team suggested exclusion of rodenticide poisoning; thus, serum warfarin and qualitative brodifacoum were tested and found to be normal. His complete blood count showed a hemoglobin of 11.8 g/dL, mean corpuscular volume (MCV) of 79 fL, and platelet count of 244,000/L. Iron indices found a serum iron of 129 mg/dL, total iron binding capacity of 357 mg/dL, percent saturation of 36%, and serum ferritin of 23 ng/mL. A peripheral smear noted normochromic, normocytic red blood cells with the presence of target cells. A complete metabolic panel yielded an albumin of 2.7 g/dL and total protein of 5.8 g/dL, but was otherwise unremarkable. His 25-hydroxy-vitamin D level was 6 ng/mL, vitamin B6 was 10.3 ng/mL, and prealbumin was 13.2 mg/dL, all decreased. Additional laboratory tests, including D-dimer, fibrinogen, fibrin degradation products, lupus anticoagulant, factor inhibitor assay, hepatitis serologies, human immunodeficiency virus screening, vitamin B12, folate, and thyroid function tests were all normal. A malabsorptive process was suspected, and abdominopelvic CT with intravenous contrast was obtained, showing mildly prominent and fluid-filled loops of small bowel, with hyperemic mucosa. The critical finding of jejunoileal fold pattern reversal was also seen (Figure A–C). What is the cause of his coagulopathy and abnormal chemistries? Look on page 1291 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The authors disclose no conflicts. © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2015.01.046
Gastroenterology 2015;148:1290–1291
CLINICAL CHALLENGES AND IMAGES IN GI Answer to Image 3 (page 1290): Celiac Disease
Jejunoileal fold pattern reversal on CT imaging is highly specific (nearing 100%) and moderately sensitive (59%) for celiac disease.1,2 In this patient, a celiac reflexive panel was ordered as soon as malabsorption was suspected; however, results were not immediately available. Tissue transglutaminase immunoglobulin (Ig)A antibody was 184 U (>31 U ¼ positive) and deamidated gliadin peptide antibody IgA was 191 U (>31 U ¼ positive). Before results of these tests, esophagogastroduodenoscopy was performed. The distal duodenum exhibited characteristic scalloping of the mucosa, with a paucity of folds, and a mosaic pattern (Figure D). Multiple biopsies were taken from the distal duodenum. Histology (Figure E and F) exhibited the presence of severe villous atrophy and numerous intraepithelial lymphocytes, confirming the diagnosis of celiac disease. The patient was given a single dose of intravenous vitamin K, with rapid correction of his prothrombin time. He was discharged with strict instructions for adherence to a gluten-free diet, which is quite difficult in the prison system. Although he did not return to our care, laboratory tests available for review 1 month after discharge indicated normalization of his PT/INR, hemoglobin, and MCV. Review of his documented vital signs indicated a weight gain of 15 lbs from the last recorded weight at our hospital. Presenting symptoms of celiac disease have changed drastically in the last several decades, with the decreasing proportion of patients experiencing diarrhea (and subsequent malabsorption) accounting for most of this change.3 The discovery of reliable serologic markers, combined with an improved understanding of the disease, have allowed for a “casefinding” strategy, which often leads to diagnosis of the disease in much earlier stages. Indeed, it has been shown that the time from symptom onset to diagnosis of celiac disease has decreased significantly.3 Nonetheless, there remain populations (eg, prisoners, underprivileged) in which the diagnosis is made at a much more advanced stage of disease.
References 1. 2. 3.
Lomoschitz F, Schima W, Schober E, et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. Eur Radiol 2003;13:890–896. Tomei E, Marini M, Messineo D, et al. Computed tomography of the small bowel in adult celiac disease: the jejunoileal fold pattern reversal. Eur Radiol 2000;10:119–122. Rampertab S, Pooran N, Brar P, et al. Trends in the presentation of celiac disease. Am J Med 2006;119:355.e9–355.e14.
For submission instructions, please see the Gastroenterology web site (www.gastrojournal.org).
1291
School of Medicine, and 2Department of Gastroenterology and Hepatology, University of Texas Medical Branch at Galveston, Galveston, Texas
Question: A 46-year-old, white, male, prisoner of 16 years, presented with coagulopathy and lower extremity edema. He had no remarkable medical history, reporting overall good health until 2 years before admission. At that time, he had experienced gradual onset of loose stools, which have persisted intermittently since. He felt this to be worse with certain meals, particularly those high in carbohydrates. He reported an associated, unintentional weight loss of 30 pounds in the previous year. He denied any melena, hematochezia, hematemesis, abdominal pain, skin rashes, bleeding events, easy bruising, or other complaints throughout the course of his illness. There was no family history of bleeding diatheses. At the time of referral, he was on oral iron supplementation, which he stated was for anemia, a cause of which had not been identified. His first visit encounter with our institution was through the hematology tele-medicine clinic 1 month before hospital admission, at which time repeat coagulation laboratory tests were recommended to his prison unit. When these laboratory tests were confirmatory, he was admitted to the hospital for additional workup. His physical examination revealed mild, nonpitting, bilateral lower extremity edema, but was otherwise entirely normal. At the time of admission, his prothrombin time (PT) was >100 s, with an International Normalized Ratio (INR) of >13.5. Clotting factors II, VII, and X were all decreased, suggesting a vitamin K deficiency. Because he resides in prison, the hematology team suggested exclusion of rodenticide poisoning; thus, serum warfarin and qualitative brodifacoum were tested and found to be normal. His complete blood count showed a hemoglobin of 11.8 g/dL, mean corpuscular volume (MCV) of 79 fL, and platelet count of 244,000/L. Iron indices found a serum iron of 129 mg/dL, total iron binding capacity of 357 mg/dL, percent saturation of 36%, and serum ferritin of 23 ng/mL. A peripheral smear noted normochromic, normocytic red blood cells with the presence of target cells. A complete metabolic panel yielded an albumin of 2.7 g/dL and total protein of 5.8 g/dL, but was otherwise unremarkable. His 25-hydroxy-vitamin D level was 6 ng/mL, vitamin B6 was 10.3 ng/mL, and prealbumin was 13.2 mg/dL, all decreased. Additional laboratory tests, including D-dimer, fibrinogen, fibrin degradation products, lupus anticoagulant, factor inhibitor assay, hepatitis serologies, human immunodeficiency virus screening, vitamin B12, folate, and thyroid function tests were all normal. A malabsorptive process was suspected, and abdominopelvic CT with intravenous contrast was obtained, showing mildly prominent and fluid-filled loops of small bowel, with hyperemic mucosa. The critical finding of jejunoileal fold pattern reversal was also seen (Figure A–C). What is the cause of his coagulopathy and abnormal chemistries? Look on page 1291 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The authors disclose no conflicts. © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2015.01.046
Gastroenterology 2015;148:1290–1291
CLINICAL CHALLENGES AND IMAGES IN GI Answer to Image 3 (page 1290): Celiac Disease
Jejunoileal fold pattern reversal on CT imaging is highly specific (nearing 100%) and moderately sensitive (59%) for celiac disease.1,2 In this patient, a celiac reflexive panel was ordered as soon as malabsorption was suspected; however, results were not immediately available. Tissue transglutaminase immunoglobulin (Ig)A antibody was 184 U (>31 U ¼ positive) and deamidated gliadin peptide antibody IgA was 191 U (>31 U ¼ positive). Before results of these tests, esophagogastroduodenoscopy was performed. The distal duodenum exhibited characteristic scalloping of the mucosa, with a paucity of folds, and a mosaic pattern (Figure D). Multiple biopsies were taken from the distal duodenum. Histology (Figure E and F) exhibited the presence of severe villous atrophy and numerous intraepithelial lymphocytes, confirming the diagnosis of celiac disease. The patient was given a single dose of intravenous vitamin K, with rapid correction of his prothrombin time. He was discharged with strict instructions for adherence to a gluten-free diet, which is quite difficult in the prison system. Although he did not return to our care, laboratory tests available for review 1 month after discharge indicated normalization of his PT/INR, hemoglobin, and MCV. Review of his documented vital signs indicated a weight gain of 15 lbs from the last recorded weight at our hospital. Presenting symptoms of celiac disease have changed drastically in the last several decades, with the decreasing proportion of patients experiencing diarrhea (and subsequent malabsorption) accounting for most of this change.3 The discovery of reliable serologic markers, combined with an improved understanding of the disease, have allowed for a “casefinding” strategy, which often leads to diagnosis of the disease in much earlier stages. Indeed, it has been shown that the time from symptom onset to diagnosis of celiac disease has decreased significantly.3 Nonetheless, there remain populations (eg, prisoners, underprivileged) in which the diagnosis is made at a much more advanced stage of disease.
References 1. 2. 3.
Lomoschitz F, Schima W, Schober E, et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. Eur Radiol 2003;13:890–896. Tomei E, Marini M, Messineo D, et al. Computed tomography of the small bowel in adult celiac disease: the jejunoileal fold pattern reversal. Eur Radiol 2000;10:119–122. Rampertab S, Pooran N, Brar P, et al. Trends in the presentation of celiac disease. Am J Med 2006;119:355.e9–355.e14.
For submission instructions, please see the Gastroenterology web site (www.gastrojournal.org).
1291
