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CLINICAL CHALLENGES: 18 YEAR OLD MALE WITH BLOODY DIARRHEA
[GRAND ROUNDS]
KIM, HELEN J. MD; NEWMAN, BEVERLEY MD; KELJO, DAVID J. MD, PHD

Department of Pediatrics, Division of Gastroenterology, Department of Radiology, University of Pittsburgh School of Medicine and Children's Hospital of Pittsburgh.
Reprint requests: David J. Keljo, MD, PhD, Gastroenterology Department, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh PA 15213. E-mail: [Ссылки доступны только зарегистрированным пользователям ].
Submitted for publication Aug 19, 2004; last revision received Mar 11, 2005; accepted Apr 5, 2005.
CT: Computed tomography; IBD: Inflammatory bowel disease

CASE PRESENTATION
An 18-year-old boy presented to the emergency department with a 2-week history of diarrhea and fatigue. His diarrhea was watery and nonbloody, occurring several times a day. A diagnosis of viral gastroenteritis had been made by his primary care physician. Four days before admission, his diarrhea became more frequent, accompanied by nonbloody, nonbilious emesis, and worsening fatigue. His parents noted that he looked pale and had low-grade temperatures. He denied night-time stooling but admitted to having 1 episode of fecal incontinence. He denied abdominal pain, night sweats, weight loss, jaundice, rash, easy bruising, or bleeding. There was no recent travel or unusual exposures. Medical history was notable for psoriasis. Family history was negative for inflammatory bowel disease or celiac disease but was positive for a maternal grandfather with a “ruptured colon.” There was no family history of bleeding or clotting disorders.

Physical examination revealed a pale, well-grown boy in no distress. He was fever free, with tachycardia to 119 beats/min and hypertension with a blood pressure of 148/72 mm Hg, and he had a normal respiratory rate. His weight plotted to the 90th percentile and height plotted to the 75th percentile for age. Head, ears, eyes, nose, and throat examination showed no scleral icterus but revealed an ulcer on the left tonsil. Cardiac examination revealed a I/VI systolic murmur along the left sternal border. His abdomen was not distended and not tender with no hepatomegaly; however, a spleen tip was palpable. A rectal examination revealed gross blood with no fissures or tags. On skin examination, there were no bruises, petechiae, or rashes.

The patient was admitted to the hospital for further evaluation. While hospitalized his diarrhea became grossly bloody with mucus.

Initial laboratory results included a white blood cell count of 22,300 cells/mm3 with a differential of 44% neutrophils, 36% bands, 9% lymphocytes, 7% monocytes, 1% eosinophils, 1% metamyelocytes, and 2 % myelocytes. Hemoglobin was 8.3 g/dL, hematocrit 25.8 %, with a low mean corpuscular volume of 63 and elevated red cell volume distribution width of 19.4%. Platelet count was 38,000/mm3. The reticulocyte count was 4.5 %. The peripheral smear revealed red blood cell fragments, ovalocytes, teardrop cells, and hypochromia. Blood urea nitrogen level was 13 mg/dL, and creatinine was 1.5 mg/dL. Liver function tests revealed elevated alanine aminotransferase of 1030 IU/L, aspartate aminotransferase of 746 IU/L, alkaline phosphatase of 133 IU/L, gamma-glutamyltransferase of 73 IU/L, total bilirubin of 0.4 mg/dL, and albumin of 2.9 g/dL. The prothrombin time was elevated at 19.8 seconds, with an activated partial thromboplastin time of 29.4 seconds, and an elevated international normalized ratio of 1.8. The lactate dehydrogenase was 1118 IU/L, and uric acid was 8.8 mg/dL. Erythrocyte sedimentation rate was 24 mm/h. Urinalysis revealed 2 red blood cells, trace ketones, small amount of protein, and 26 hyaline casts.

Evaluation for infectious enteritis with stool cultures, ova, and parasites, and Clostridium difficile toxin proved negative. Blood cultures were negative. Tests for infectious hepatitis including a hepatitis panel and cytomegalovirus antigen were negative. Epstein-Barr virus titers were consistent with a remote prior infection. Serial complete blood count and liver function tests revealed that the low platelet count and elevated transaminases returned toward normal levels. Renal function remained normal throughout the admission. An opthalmologic examination was negative for Kayser-Fleischer rings, and serum ceruloplasmin and copper levels, as well as 24-hour urinary copper levels, were within normal limits.

Abdominal ultrasonography with Doppler scanning revealed no flow in the left portal vein, but patent and normal flow direction in the right portal, main portal, and splenic veins. There was a mildly enlarged spleen, nonspecific gallbladder wall thickening, and a moderate amount of free fluid within the abdomen. The kidneys and liver were otherwise normal. Because there was evidence of possible thrombosis in the portal vein by ultrasound examination, initial therapy with vitamin K was discontinued, and fresh frozen plasma was not administered.

A computed tomography (CT) angiogram was obtained to further evaluate for possible thrombosis in the portal system and to inspect the liver and colon. This imaging revealed extensive subocclusive thrombosis of the portal and mesenteric venous systems, patchy enhancement of the liver, and colonic changes suggestive of colitis (Figure). There was no suggestion of appendicitis, ruptured appendix or other source of abdominal sepsis.



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Figure. Images from the portal venous phase of a contrast enhanced CT scan. A, Almost complete thrombosis of the intrahepatic portal venous branches (arrows). Liver has patchy heterogeneous appearance with peripheral geographic areas of low attenuation. B, Subocclusive thrombus of portal confluence and medial splenic vein (black arrows). Splenic flexure of colon has thickened, edematous walls (white arrows) with prominent mucosal enhancement. C, Enlarged superior mesenteric vein with subocclusive thrombus (black arrow). Walls of descending and transverse colon (white arrows) are thickened and edematous with prominent mucosal enhancement.

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The patient was evaluated for known primary causes of hypercoagulable states. The evaluation was negative for factor V Leiden and prothrombin G20210A mutations. There was normal activated protein C resistance. Low antithrombin III, protein C activity, and protein S activity were believed to be caused by liver disease or the recent thrombosis itself. There was no evidence of an antiphospholipid antibody syndrome, with normal tissue thromboplastin inhibition indexes, negative hexagonal lipid neutralization test, normal anticardiolipin and antithyroid antibody levels, and normal dilute Russell viper venom time. The patient was heterozygous for the methylenetetrahydrofolate reductase thermolabile variant (C677T) but had normal homocysteine levels. Flow cytometry was negative for paroxysmal nocturnal hemoglobinuria as a cause of thrombosis.

A colonoscopy was performed. At colonoscopy the colon was found to be diffusely inflamed and edematous with mucosal ulceration and friability, indicating a pancolitis. The terminal ileum was not inspected. Biopsy specimens revealed acute colitis with inflammatory pseudomembranes, organizing submucosal thrombi, and crypt abscess formation. No viral inclusions, organisms, or granulomas were appreciated. There were no chronic changes at this point. Concern was raised about a thrombotic/ischemic colitis.

The patient was transferred to the intensive care unit for optimal management of anticoagulation in the face of active gastrointestinal bleeding. Heparin was used for anticoagulation.

A sigmoidoscopy with biopsy was performed on a later date to further delineate the underlying diagnosis. These biopsy specimens of the left colon showed architectural distortion and lamina propria lymphoplasmacytic inflammation with gland lift-off, acute cryptitis, and crypt abscesses. These features of chronic mucosal injury with active inflammation were more suggestive of chronic inflammatory bowel disease. The colitis was treated with prednisone and sulfasalazine. The patient was eventually discharged on prednisone, mesalamine (Asacol), and a 6-month course of warfarin. Repeat abdominal ultrasound examination approximately 3 months after the initial ultrasound examination revealed patent portal vessels with no evidence of thrombus. On follow-up visits, the patient's ulcerative colitis was noted to be under good control, with resolution of abdominal pain and bloody diarrhea. The final diagnosis was ulcerative colitis with secondary hypercoagulable state and portal and mesenteric vein thrombosis.
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