Crampy Abdominal Pain Research Articles

An Unusual Case of Terminal Ileitis and Stricture that Mimicked Crohnʼs Disease

Purpose: Chronic inflammation in the terminal ileum (TI) suggests a cause for the patient's symptoms, especially when the clinical suspicion is Crohn's disease. Non-steroidal anti-inflammatory drugs, lymphoid hyperplasia, intestinal infections, lymphoma, infections and ulcerative colitis (UC) are some of the other causes of terminal ileitis. This is an unusual case of a 41-year-old multiparous female with past medical history of small bowel obstruction and colitis who presented with a 5-day history of crampy abdominal pain, nausea, vomiting, and diarrhea. On the CT scan of the abdomen she was found to have small bowel obstruction with segmental enteritis in close proximity to the terminal ileum. She was started on IV antibiotics and steroids, kept NPO and NG tube was placed to relieve the obstruction. Due to little improvement in the obstruction, she underwent exploratory laparotomy with resection of the narrowed small bowel segment with side to side anastomosis and appendectomy. Patient was started on temporary parenteral feeding, and continued on IV steroids for management of suspected inflammatory bowel disease. Strangely, the pathology results for the small bowel resection specimens showed presence of endometriosis in both the appendix and terminal small bowel segment without any evidence of inflammatory bowel disease. Her steroids were soon tapered off and with tolerance of oral feeds patient was discharged from the hospital with outpatient gynecology follow-up for management of endometriosis. Ileal endometriosis should be carefully considered in the differential diagnosis of Crohn's disease in menstruating females, especially the ones who are nulliparous and have dysmenorrhea, dyspareunia, dyschezia, menometrorrhagia, or other peri-menstrual symptoms. This case exemplifies the importance to not mistake endometriosis for Crohn's disease as they each have different therapeutic management.

Hickam's Dictum in a Patient With Diarrhea

Question: A 49-year-old woman with a history of 2 cadaveric renal transplants was referred for colonoscopy for evaluation of 4 weeks of bloody diarrhea with associated crampy abdominal pain. Renal transplants occurred in 1998 and 2005 for end-stage renal disease related to glomerulonephritis and subsequent chronic allograft nephropathy. Other past medical history was notable for recurrent urinary tract infections with bilateral ureteral stents placed 6 months ago, immune thrombocytopenic purpura, and hypertension.

35-Year-Old Man With Fever and Abdominal Pain

dicine, te o L.T.H., nt achesA 35-year-old man came to our emergency department with fever (temperature, 38.4 C), a 2-day history of crampy intermittent abdominal pain localized to the right lower quadrant, and loose nonbloody stools. His medical history was notable for acute promyelocytic leukemia, for which he was receiving induction chemotherapy. The abdominal pain was not associated with positional changes, eating, bowel movements, or other factors. The patient had last received chemotherapy 10 days previously. A review was negative for mouth sores, nausea, or vomiting or for recent travel. The patient had no history of abdominal surgery, and family history was noncontributory. On physical examination, the patient was febrile (temperature, 38.4 C), with pulse rate of 132/min, supine blood pressure of 108/56 mm Hg, and respiratory rate of 20 breaths/min. He appeared fatigued but in no acute distress. Skin was without rashes, and the port-a-catheter site didnot seem to be infected. Abdominal examination revealed normoactive bowel sounds and moderate tenderness to palpation in the right lower quadrant, without guarding or rigidity. The perirectal area was without lesions, and digital rectal examination was not performed. Results of the remainder of the complete multisystem examination were unremarkable. Laboratory studies revealed the following values (reference ranges shown parenthetically): hemoglobin, 12.3 g/dL (13.5-17.5 g/dL); leukocyte count, 0.1 10/L (3.5-10.5 10/L); absolute neutrophil count (ANC), 91 cells/mL (1500-8000 cells/mL); platelet count, 127 10/L (150-450 10/L); sodium, 138 mEq/L (135-145 mEq/L); potassium, 3.6 mEq/L (3.65.2 mEq/L); chloride, 105 mEq/L (100-108 mEq/L); bicarbonate, 27 mEq/L (22-29 mEq/L); serum urea nitrogen, 22 mg/dL (8-24 mg/dL); creatinine, 0.8 mg/dL (0.8-1.3 mg/dL); calcium, 8.3 mg/dL (8.9-10.1 mg/dL); aspartate aminotransferase, 76 U/L (8-48 U/L); alanine aminotransferase, 51 U/L (7-55 U/L); alkaline

Su2019 Comparative Effectiveness of Antibiotic Therapy for Small Intestinal Bacterial Overgrowth – A Systematic Review

3 phase 3 studies. In all studies, eligible patients were ≥10 years old who presented with an HAE attack considered to be moderate (affected activities of daily living) or severe (prevented activities of daily living). All patients provided written informed consent and the appropriate IRB approved the studies at each site. Pre-treatment information collected in some or all of the studies included affected anatomic location, accompanying symptoms, relevant medical history, physical examination, laboratory evaluations, and pain assessments—using the McGill Short Form Pain Questionnaire and a Visual Analog Scale (VAS) pain score. Results: Across all studies, 310 patients (40 patients 9-17 years old; 270 patients ≥18 years old) were treated for 1249 HAE attacks affecting any anatomic location; abdominal symptoms were reported in 698 attacks (56%). Approximately 51% of these attacks only affected the abdomen. Data on accompanying symptoms were available for 166 attacks with abdominal symptoms. Distention (74% of attacks), cramping (72%), and nausea (65%) were reported most frequently; vomiting (20%) and diarrhea (13%) were less common. Based on available data from the McGill Pain Questionnaire (N=502 abdominal attacks), patients were most likely to describe their abdominal pain as tender, aching, or cramping. In 23% of 569 abdominal attacks, white blood cell (WBC) counts were elevated (mean ± standard deviation: 15.1 ± 11.27 X 109/L); WBC elevations were more likely to be associated with severe attacks (64% of severe abdominal attacks had elevated WBC vs 34% of moderate attacks). In 183 patients with abdominal attacks, 23% had a surgical history of appendectomy and 16% had a history of cholecystectomy. Conclusions: During HAE attacks, patients often experience gastrointestinal symptoms in the absence of peripheral signs of angioedema. Gastroenterologists should consider HAE in the differential diagnosis for patients with recurrent, unexplained crampy abdominal pain associated with nausea with or without an elevated WBC.

5-ASA Induced Recurrent Myopericarditis and Cardiac Tamponade in a Patient with Ulcerative Colitis

A 20-year-old woman with recently diagnosed moderate left-sided ulcerative colitis (UC) presented to the emergency department (ED) for chest pain. Two months earlier, she had been evaluated in gastroenterology clinic for bloody diarrhea and crampy left lower quadrant abdominal pain. Stool tests for infectious causes were negative. Colonoscopy demonstrated diffuse mucosal erythema, linear ulcerations, and friability that extended continuously from the rectum to the descending colon (Fig. 1), histologically reported as acute and chronic colitis with crypt architectural distortion, consistent with inflammatory bowel disease. No granulomas or viral inclusion bodies were seen. Institution of sulfasalazine 500 mg four times daily and mesalamine enemas significantly improved her symptoms. Three weeks later, she presented to the ED with pleuritic chest pain, aggravated by lying flat and relieved by leaning forward. She denied nausea, vomiting, coryza, or cough. She was afebrile, with a heart rate 116 beats per minute and blood pressure 102/67 mmHg. Physical exam was notable for a friction rub heard loudest over the left sternal border, mild epigastric tenderness, and 1? pitting edema to the mid-shin. Laboratory examination revealed leukocytosis with neutrophilia (WBC 16.8 k/ll, 82.8 % neutrophils), elevated troponin, creatine kinase-myocardial band (CK-MB), erythrocyte sedimentation rate (ESR), and C-reactive protein. ECG revealed diffuse ST elevation. Echocardiography revealed a small pericardial effusion with normal systolic function (Fig. 2), which was also noted in the CT angiogram report, with no evidence of pulmonary embolism or other chest pathology. The patient was diagnosed with acute myopericarditis. Direct fluorescent antibody test for respiratory viruses, blood cultures, HIV antibody screen, PPD, and autoantibodies (including anti-nuclear antibody, anti-double-stranded DNA, and others) were all negative. Although viral myopericarditis could not definitively be excluded (since not all implicated viruses can be tested for), the main concern was for a reaction to sulfasalazine or, less likely, rectal mesalamine. Sulfasalazine was thus discontinued, mesalamine enemas were switched to hydrocortisone enemas, and she was treated with ibuprofen and colchicine, with symptom resolution. Low-dose balsalazide was then cautiously initiated. Five days later, the patient returned with recurrent severe pleuritic chest pain. She was hypotensive (85/52 mmHg) and tachycardic (117 bpm). Her ECG showed diffuse T wave flattening without electrical alternans. Bedside echocardiogram revealed a large pericardial effusion with hemodynamic compromise consistent with cardiac tamponade (Fig. 2). She underwent emergent pericardiocentesis with drainage of 300 ml of serosanguinous fluid. Fluid analysis and cultures were unrevealing. She was again treated with ibuprofen and colchicine, with discontinuation of the balsalazide, accompanied by marked improvement of her chest pain. Hydrocortisone enemas were prescribed with a plan for immunomodulator I. Sonu R. Wong M. E. Rothenberg Department of Medicine, Stanford University Medical Center, Stanford, CA, USA

PEComa of the colon resistant to sirolimus but responsive to doxorubicin/ifosfamide

A 23-year-old male presented with a three-week-history of crampy abdominal pain and melaena. Colonoscopy revealed a friable mass filling the entire lumen of the cecum; histologically, it was classified as perivascular epithelioid cell tumor (PEComa). An magnetic resonance imaging scan showed, in addition to the primary tumor, two large mesenteric lymph node metastases and four metastatic lesions in the liver. The patient underwent right hemicolectomy and left hemihepatectomy combined with wedge resections of metastases in the right lobe of the liver, the resection status was R0. Subsequently, the patient was treated with sirolimus. After 4 mo of adjuvant mammalian target of rapamycin inhibition he developed two new liver metastases and a local pelvic recurrence. The visible tumor formations were again excised surgically, this time the resection status was R2 with regard to the pelvic recurrence. The patient was treated with 12 cycles of doxorubicin and ifosfamide under which the disease was stable for 9 mo. The clinical course was then determined by rapid tumor growth in the pelvic cavity. Second line chemotherapy with gemcitabine and docetaxel was ineffective, and the patient died 23 mo after the onset of disease. This case report adds evidence that, in malignant PEComa, the mainstay of treatment is curative surgery. If not achievable, the effects of adjuvant or palliative chemotherapy are unpredictable.

Lymphocytic Colitis in a Two Year Old Presenting with Chronic Diarrhea

Lymphocytic colitis (LC) is a subtype of microscopic colitis characterized by chronic, non-bloody, watery diarrhea, normal colonoscopy, and abnormal histopathological findings defined by >20 intraepithelial lymphocytes per 100 enterocytes. Defined in 1976, it has traditionally been a disease of adulthood with the mean age of diagnosis of >50 years. Over the past decade, numerous reports have suggested that LC may present at any age with increasing incidence in children. We describe a 2-year-old girl with LC who presented with chronic diarrhea. To our knowledge, she is the youngest described patient with LC. A 2-year-6-month-old girl presented with episodic crampy abdominal pain, nonbloody, nonmucoid, watery diarrhea and decreased growth velocity. These cycles occurred every 1-2 months since 18 months of age. Stool cultures, C difficile, ova and parasites were negative. Celiac screen, RAST for food allergies, and standard blood tests were all within normal limits. CT abdomen was normal. EGD and colonoscopy appeared normal. Histopathology of esophagus, stomach, duodenum, and terminal ileum were normal. However, biopsies of all sections of colon and rectum showed marked intraepithelial lymphocytosis within the lamina propria that was consistent with LC. She was started on sulfasalazine (25 mg/kg/day) with initial success marked by decreased stooling and decrease in abdominal pain. The mother discontinued the medication and symptoms returned almost immediately. Three weeks after discontinuation of sulfasalazine, patient returned to clinic due to increased symptoms. She was restarted on sulfasalazine (40 mg/kg/day) with near complete resolution of abdominal pain, having 1-2 formed stools daily, and two-month interval weight gain of 1.5 kg. The cause of LC is unknown although bacteria, viruses, non-steroidal anti-inflammatory drugs, or autoimmunity have been implicated as inciting factors. Treatment is aimed at decreasing diarrhea symptoms and inflammation. Bulking agents improve diarrhea and anti-inflammatory agents such as sulfasalazine and corticosteroids have been used with success. While LC is a rare disease in children, it should be considered in pediatric patients with chronic non-bloody diarrhea.

Profound neurological injury in a patient with atypical hemolytic uremic syndrome

Dear Editor, Atypical HUS (aHUS) is a thrombotic microangiopathy (TMA) characterized by complement-mediated end-organ injury. While patients with aHUS may present similarly to patients with thrombotic thrombocytopenic purpura (TTP), they have different underlying pathophysiologies, and this may explain differing responses to plasma-based therapy. It can be challenging to differentiate aHUS from TTP. It had been presumed in the past that profound renal injury is due to aHUS, while neurologic injury was felt to be more consistent with TTP. Although kidney injury may be the overriding feature in aHUS, it has been recognized that multiple end organs may be affected including the brain, heart, lung, and GI tract [1, 2]. We report a patient with aHUS and profound neurological symptoms that recovered following eculizumab therapy. An otherwise healthy 66-year-old female was admitted to the medical ICU after presenting with nausea, crampy abdominal pain, and intermittent bloody diarrhea following consumption of a salad at a local restaurant 9 days prior. A Shiga toxin assay was negative. Although not thrombocytopenic on admission, over the 36 h of hospitalization, platelet count decreased to 100×10/μL platelet, and on peripheral blood smear, she was noted to have 3+ schistocytes per high power field. She quickly became anuric and developed mental status changes. Given her deteriorating condition, she was transferred to our institution. On admission, she was intubated for airway protection. Laboratory studies showed findings consistent with a microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. ADAMTS13 activity level was 68 %. On day 3 of plasma exchange, she experienced a generalized tonic– clonic seizure. Following the seizure, despite being off all sedating medications, she remained unresponsive but maintained gag and corneal reflexes. CT of head was unremarkable and an MRI demonstrated only a focal 3×3-mm area of increased FLAIR signal abnormality in the left superior temporal gyrus cortex. EEG was suggestive of a moderate, nonspecific encephalopathy. Lumbar puncture was unremarkable. Despite an extensive neurological evaluation, no etiology of her mental status changes other than her TMA was identified. On day 4, therapy with eculizumab was initiated. Eculizumab is a humanized monoclonal antibody against CS that blocks terminal compliment activity. Therapy consisted of induction infusion administered at 900 mg iv weekly for 4 weeks followed by maintenance therapy at 1,200 mg biweekly. Following her third weekly infusion, she became independent of hemodialysis, though still unresponsive with a tracheostomy for airway protection. On week 7 of therapy, she rather abruptly awoke and became verbal. She gradually improved neurologically and has been making tremendous strides in her rehabilitation. She is currently continuing biweekly eculizumab therapy and is now functionally independent, approaching her pre-illness state. Results of her mutation studies demonstrated the presence of a heterozygous mutation G. Salem (*) Department of Internal Medicine, The Ohio State University Comprehensive Cancer Center, Starling Loving Hall, Room 365, 320 West 10th Avenue, Columbus 43210, USA e-mail: Galena.Salem@osumc.edu

A Complicated Case of Perforated Diverticulitis with Septic Embolization

Introduction: Acute diverticulitis occurs when fecal matter obstructs the orifice of a diverticulum and local infection ensues. Inflammation can result in reduced tissue perfusion leading to focal necrosis and perforation. A small perforation may be walled off by omentum and present as a localized abscess. Disruption of tissue and vascular integrity may result in the siphoning of gas into the vascular system. Rarely intravascular gas leads to thrombosis and septic emboli. Case Report: We are reporting an interesting case of a 54-year-old male with history of alcohol abuse presented with one week of fever, chills, diffuse, crampy abdominal pain and diarrhea. He was febrile with leukocytosis, elevated LFT's and hyperbilirubinemia. CT of the abdomen revealed mesenteric gas with likely source arising from sigmoid colon and small areas of hypoenhancement within the spleen possibly areas of infarct. Antibiotics were administered. Patient's condition continued to deteriorate with high temperatures, diarrhea and increasing leukocytosis. On seventh day of hospitalization, CT scan revealed gas in the mesenteric vein extending into the portal vein and its branches. The mesenteric venous gas was traced back and appeared to originate from the sigmoid colon. The liver and spleen revealed areas of infarction. Clinically he appeared to have perforated diverticulitis with the ominous sign of venous gas and septic emboli. He was transferred to a referral hospital where he had exploratory laparotomy with anterior resection of the sigmoid colon and a prolonged postoperative antibiotic course. On follow-up, he was healthy and had no gastrointestinal complaints. Discussion: This patient presented with classic findings of acute diverticulitis and CT of the abdomen revealed mesenteric vein air contiguous with sigmoid colon extending to the portal vein and its branches and infarcts in the liver and spleen. Hepatic portal venous gas is a rare entity and along with septic emboli is an ominous sign suggestive of severe mortality. It is usually noted in bowel ischemia and in rare cases it can also be seen with complicated colonic diverticulitis. Conclusion: In conclusion, it is important to be aware of septic emboli and venous thrombosis as a complication of diverticulitis with perforation. The findings of venous gas, septic emboli or venous thrombosis necessitate urgent surgery intervention. Since diverticulitis is such a common entity, complications should be anticipated and physicians should be aware of rare complications such as septic emboli leading to liver and splenic infarcts. Air in the hepatic portal venous system is always an ominous sign and requires immediate surgical intervention.Table: No Caption available.

PO5 GIARDIASIS IN SUSPECTED CELIAC DISEASE

Background and Objectives: Celiac disease (CD) is an immunemediated enteropathy induced by gluten ingestion in a genetically susceptible patient. Giardia lamblia is the most common human parasite with a worldwide distribution and fecal-oral way of transmission. Giardia lamblia has a cosmopolitan distribution. Infected children may have acute or chronic diarrhea, crampy abdominal pain, anorexia, malasorption and poor weight gain and may be misdiagnosed as celiac disease. Diagnosis is usually made by finding the characteristic cysts in stool specimens or by duodenal aspiration. In most cases histology reveals a dense accumulation of the parasites on the surface of the duodenal mucosa with no or only slight inflammation. In rare cases, a dense inflammatory infiltrate with severe mucosal atrophy and increased count of intraepithelial lymphocytes may be seen. If in such cases the amount of parasites is low, the histological picture may mimic celiac disease. We report the case of a child 2 years old who presented poor growth, recurrent aphtous stomatitis, abdominal pain and recurrent diarrhea. No family history of CD. Material and Methods: In suspected CD, were assayed the IgA anti-endomysium antibodies (EmA-IgA) that resulted doubt and the IgA anti-tissue transglutaminase antibodies (TgA-IgA) that were found weakly positive (17.50UA; normal value 16UA). He was referred to our hospital for further diagnostic. We performed the search for genetic susceptibility of CD; the antibodies assay was repeated after 3 months. These investigations documented the presence of HLADQ2, positivity of EmA-IgA and an increase value of TgA-IgA (20.70UA). In the meantime symptoms worsened, so we proceeded performing the esophagogastroduodenoscopy and duodenal biopsies. The macroscopic appearance of duodenal mucosa was of villous atrophy, but microscopic examination showed a parasitic duodenitis, with the presence of microorganisms like Giardia lamblia. Then was started medical therapy with metronidazole per os for 10 days, and the child continued to eat gluten. Results: Abdominal pain and diarrhea disappeared gradually but rapidly; the parasitological examination was negative on 3 consecutive samples (at 3 weeks after eradication therapy); EmA-IgA and TgAIgA were negative at 1 month after eradication. The follow up to 1 year had confirmed that the increase in IgA was secondary to giardiasis, and that the symptoms were due to the histological damage of the parasite. Conclusions: When investigating a patient with suspected celiac disease (CD), several other conditions must be considered, including potential infection with Giardia lamblia. In doubtful cases like our patient, are of fundamental support an accurate assessment and appropriate follow up.

Hemorrhagic Colitis as a Manifestation of Wegenerʼs Granulomatosis: Think Outside the Box

Case Report: A 37-year-old African American male presented to our institution with a 2 week history of diffuse crampy abdominal pain associated with frequent watery bowel movements and low grade fever. On presentation he was tachycardic, febrile and abdominal exam was unremarkable. Blood work revealed normocytic anemia, leucocytosis, coagulopathy, elevated ESR, CRP and hypokalemia and was started on intravenous antibiotics. Chest X-ray at admission revealed multiple cavitary lesions. Echocardiogram, stool studies, pancultures, serum Quantiferon, HIV test and urinalysis were unrevealing. CT chest showed lung opacities suspicious for septic emboli or neoplastic process and was started on anticoagulation that precipitated rectal bleeding while awaiting lung biopsy. Colonoscopy revealed large, deep, skipped, exudative ulcerations throughout the colon, pathology significant for chronic inflammation with crypt distortion. Due to the persistence of fever on antibiotics, steroids were started for possible autoimmune etiology. CT guided lung biopsy revealed suppurative granulomas with small vassel vasculitis, highly suspicious for Wegener's granulomatosis (WG) in the setting of elevated C-ANCA. Repeat colonoscopy for rectal bleeding revealed improvement in ulcerations with antibiotic and steroid use. Thoracoscopic lung biopsy revealed thrombotic arteriopathy, vascular intimal thickening indicative of vasculitis. He was discharged home on steroids having refused cyclophosphamide. He was asymptomatic at 6 months follow-up. Discussion: We present a unique case of a patient with pulmonary nodules and hemorrhagic colitis consistent with WG. Intestinal manifestations as an initial presentation of WG are rarely reported. As intestinal involvement is rare in WG, an important differential is inflammatory bowel disease (IBD), though lung manifestations are rare in IBD. Although our initial suspicion was Crohn's disease based on colonoscopic findings and pathology results, further investigation of the patient's lung pathology confirmed a systemic vasculitic process consistent with WG. Conclusion: Combined pulmonary and intestinal findings carry a small list of differentials, of which vasculitis including WG should be high on the differential.

Diffuse Nodular Lymphoid Hyperplasia of Intestine in Selective IgG 2 Subclass Deficiency, Autoimunne Thyroiditis, and Autoimmune Hemolytic Anemia in 60 Year Old Male

Purpose: Diffuse Nodular Lymphoid Hyperplasia (DNLH) of the intestine is a rare lymphoproliferative disorder of uncertain etiology. We present a rare case of DNLH associated with IgG2 subclass deficiency, autoimmune thyroiditis and autoimmune hemolytic anemia. A 60 year old Turkish male presented to GI clinic with watery diarrhea and crampy abdominal pain for 1 month. He had a history of treated prostate cancer. He denied any family history, alcohol, tobacco, or illicit drugs. He denied any sick contacts, or recent travel. One week prior, he was seen in the ER for similar complaints where he was anemic (hemoglobin 7.4 g/dl, mean corpuscular volume 103). CT abdomen revealed extensive lymphadenopathy. He was discharged with a one week course of ciprofloxacin and metronidazole, and scheduled for follow up in the GI clinic. At follow up iron deficiency anemia was found. Assays for vitamin B12, folate, methylmalonic acid and homocysteine were within normal range. Lactate dehydrogenase and reticulocyte count was high, haptoglobin low, and direct coomb's test positive with warm antibody. Gastrointestinal bacterial and parasitic infections were ruled out. Tissue transglumatinase antibody IgA and anti-parietal cell antibody were negative. Thyroid stimulating hormone was 12.6, with elevated thyroglobulin and thyroid peroxidase antibody. EGD and colonoscopy showed nodular mucosa in duodenal bulb and rectosigmoid respectively. Small bowel series followed by capsule endoscopy was performed. Bone marrow and abdominal lymph node biopsy was negative for malignancy. Gallium scan was negative for any metastatic disease. An increased risk (2-10%) of malignancy, mainly gastrointestinal lymphoma has been reported in patients with DNLH and hypogammaglobulinemia. These lymphoid aggregates could be an independent response to antigenic stimuli in presence of deranged immunoglobulin function. However, the relationship is unclear. Because of the risk of malignant transformation prophylactic examinations and small bowel studies are recommended by some authors. Diffuse nodular lymphoid hyperplasia in itself is a rare benign condition of unknown etiology. In symptomatic patients, workup should be directed towards underlying hypogammaglulinemia, infections and possible malignancy. Eradication of infection could lead to resolution of symptoms. Patients should undergo prophylactic surveillance endoscopy to rule out complications and to establish extent of disease. However, there are no clear cut guidelines for surveillance.

Acute appendicitis mimicking intestinal obstruction in a patient with cystic fibrosis

Cystic fibrosis (CF) is an inherited disease of the secretory glands caused by mutations of the cystic fibrosis transmembrane regulator (CFTR) gene. The clinical manifestations of CF are repetitive lung infections, biliary cirrhosis, pancreatic abnormalities, and gastrointestinal disorders. We report a 21-year-old Taiwanese man with CF who had abdominal pain for 2 days. The diagnosis of CF had been confirmed by peripheral blood analysis of the CFTR gene 5 years before admission. He presented to the emergency department with nausea, vomiting, abdominal distension, and crampy abdominal pain, which is atypical for acute appendicitis. The physical examination and a series of studies revealed intestinal obstruction, but acute appendicitis could not be ruled out. After conservative treatment, together with empiric antibiotics, the refractory abdominal pain and leukocytosis with a left-shift warranted surgical intervention. A diagnostic laparoscopy revealed a swollen, hyperemic appendix, a severely distended small intestine, and serous ascites. The laparoscopic procedure was converted to a laparotomy for open disimpaction and appendectomy. He was discharged on the eighth postoperative day. The histologic examination of the appendix was consistent with early appendicitis. In conclusion, acute abdominal pain in adult CF patients is often associated with intestinal obstruction syndrome. The presentation of concurrent appendicitis may be indolent and lead not only to diagnostic difficulties, but also a number of therapeutic choices.

An Adolescent With Fever, Jaundice, and Abdominal Pain: An Unusual Presentation of Kawasaki Disease

A 16-year-old boy presented with a 6-day history of fevers and myalgias and a 4-day history of diffuse crampy abdominal pain. On admission, his sclerae were icteric and he had diffuse abdominal tenderness. Erythrocyte sedimentation rate was elevated to 40; the γ-glutamyl transferase level was elevated to 168 U/L; indirect bilirubin was 5.6 mg/dL; and direct bilirubin was 3.3 mg/dL. During the next 2 days, he developed many stigmata of Kawasaki disease (KD), including conjunctivitis, desquamating rash, mucosal changes, swelling of the hands and feet, and lymphadenopathy. KD is commonly seen in young children but can also occur in adolescents and adults. Providers should be aware that these age-groups are at risk for KD and may present with atypical symptoms. Delays in diagnosis can put these adolescents at increased risk of coronary artery aneurysms, and, accordingly, a high index of suspicion is essential.

Intestinal Amyloidosis with Intractable Diarrhea and Intestinal Pseudo-obstruction

We report herein a case of intestinal amyloidosis with grave prognosis that caused intractable diarrhea and intestinal pseudo-obstruction, alternately in spite of intensive conservative treatment. A 44-year-old woman was admitted for fever, diarrhea, and crampy abdominal pain which had been continuned during 6 months. Abdomen CT scan showed edematous wall thickening of the small bowel and right colon, and colonoscopic biopsy revealed amyloid deposition in the mucosa. Monoclonal light chains in serum and/or urine were not detected and highly elevated serum amyloid A was shown. In spite of intensive treatment including oral prednisolone and colchicine, diarrhea and intestinal pseudo-obstruction developed alternately, general status rapidly got worsened and died after two months.

Severe stricturing Crohn's disease of the duodenum: A case report and review of surgical options

INTRODUCTIONDuodenal Crohn's disease is a rare clinical entity that occurs in 0.5–4.0% of patients with Crohn's disease. A unique case of Crohn's disease of the upper gastrointestinal tract characterized by multiple strictures within the duodenum and jejunum is described in our review. PRESENTATION OF CASEA 41-year-old male presented with a 2-month history of intermittent, crampy abdominal pain accompanied by nausea, bilious emesis, early satiety, anorexia and weight loss. Physical examination revealed fullness in the epigastric region. Imaging demonstrated strictures in the proximal and distal duodenum with dilatation of the intervening segments. There was also gross dilatation of the proximal jejunum, which was followed by a 9cm strictured segment. There was no evidence of acute Crohn's disease. Although a Whipple's resection was initially considered as a form of operative intervention given the extent of disease within the duodenum, the discovery of unexpected disease intra-operatively presented a surgical dilemma. In this case, strictureplasty, surgical resection and bypass were used to treat the patient. DISCUSSIONDiffuse stricturing of the proximal gastrointestinal tract is a rare manifestation of Crohn's disease. Although imaging can aid in surgical planning, intra-operative decision-making to deal with unexpected findings will remain an important aspect of the management of this entity. CONCLUSIONThe fundamental goal of the surgical management of strictures secondary to Crohn's disease is to relieve obstruction while maximizing bowel conservation. A variety of operative techniques are currently described for the management of duodenal Crohn's disease and are reviewed in this case report.

Infliximab and Methycillin Resistant Staphylococcus aureus in Ulcerative Colitis

Purpose: History: A 16 year old female presented with bloody diarrhea and a recent diagnosis of ulcerative colitis (UC). Medications included mesalamine since diagnosis and steroids over the past two weeks. Once the steroids were discontinued, symptoms escalated to 15 episodes of bloody diarrhea daily with nighttime stooling, crampy abdominal pain, and subjective fevers. Physical Exam and Lab Studies Exam revealed a well-nourished female in moderate distress due to abdominal pain. Her abdomen was tender in the left lower quadrant, bowel sounds were present, no guarding nor rebound tenderness. Exam was otherwise unremarkable. Abnormal labs included WBC 23,360, Hgb 10.4 g%, ESR 36, platelets 364,000, Albumin 2.8g%, CRP 37.4, and IBD-7 panel consistent with UC. Stool was positive for C. difficile toxin A. Hospital Course The patient was started on methylprednisolone 20 mg IV q12 hours and metronidazole 500 mg IV q8 hours. Repeat C. difficile toxin was negative; however, symptoms had not improved, so methylprednisolone was gradually increased to 60 mg IV q6 hours. She had some improvement, but methylprednisolone wean was not tolerated, with increasing abdominal pain, bloody diarrhea, and fever up to 102F. Flexible sigmoidoscopy ruled out CMV infection and C. difficile colitis. Due to persistent severe colitis, infliximab was infused at a dose of 5 mg/kg. Within 24 hours, the patient noticed significant improvement in symptoms; however, she also noticed a 2 mm, pinpoint, erythematous, pruritic lesion on her right leg. Four more identical lesions appeared on her extremities and all evolved into 2 cm, mildly-tender, red-ringed, pus-filled lesions with necrotic centers. Pathologic evaluation was inconsistent with neutrophilic dermatosis, and culture of the lesion grew methycillin resistant staphylococcus aureus (MRSA). Discussion The degree of immunesuppression was likely the turning factor for the appearance of MRSA. The high dose and prolonged course of steroids were probably the most important factors; however, infliximab may have prompted the appearance of the abscesses due to its mechanism of action. Infliximab's effect on NADPH oxidase may impair the oxidative burst, increasing the susceptibility to catalase-positive infections such as MRSA.

Multiple Granular Cell Tumors of the Ascending Colon: A Case Report and Review of the Literature

Purpose: Introduction: Granular cell tumor (GCT) is a very uncommon entity that rarely affects the GI tract. It is usually detected incidentally on routine colonoscopy in asymptomatic patients. Because of its morphological pattern and its submucosal location, it can often be mislabeled as a malignant lesion. Due to its low incidence no clear standards of therapy have been described. We report an unusual form of GCT of the ascending colon diagnosed and treated by endoscopic ultrasound (EUS) resulting ultimately in right hemicolectomy. Case: A 32 year-old woman presented with crampy abdominal pain, diarrhea and hemoccult positive stools. Initial colonoscopy revealed four firm submucosal nodules in the cecum and ascending colon. Chase biopsies reported nonspecific chronic mucosal inflammation without visualization of submucosal tissue. Due to the submucosal location of the masses a full workup for carcinoid was sent with negative results. A follow up colonoscopy six months later showed no changes in the submucosal nodules. Repeat biopsies with jumbo forceps were non-diagnostic. EUS revealed 5 well defined hypoechoic nodular structures in the right colon. Submucosal resection of the two smaller and most distal lesions was performed. The same evening, the patient is diagnosed with acute ruptured viscus. Exploratory laparotomy with right hemicolectomy was performed identifying perforation points at one of the mucosectomy sites. Pathology reports from EUS revealed a submucosal mass strongly positive for S-100 with no clear necrosis or mitotic activity clearly suggestive of GCT. Surgical pathology confirmed the same diagnosis. Discussion: GCT are rare and typically benign tumors. Schwan cells are currently the most acceptable derivation site. It might occur anywhere in the body with a GI incidence close to 1%. The most common site in the GI tract is the esophagus, followed by the stomach, colon and perianal region. With < than 2 cm in diameter, and frequently reported as solitary masses, GCTs are often diagnosed as incidental findings during routine endoscopy. Positive immunohistochemical markers such as S-100 and Neuron Specific Enolase strongly suggest the diagnosis. Rate of malignancy depends on tumor size (usually > 4 cm) and adjacent tissue spreading (muscularis propia). EUS is a very effective tool in evaluating submucosal lesions. Although endoscopic resection is the preferred treatment, surgery has also been considered for cases where malignant features are present. It is imperative for the gastroenterologist to include GCT in the differential diagnosis of submucosal tumors of the colon.

Combined Jejunal and Colonic Fecal Bacteriotherapy in Patients with Recurrent Clostridium Difficile Infection (RCDI)

Purpose: Recent epidemiological trends suggest an increase in frequency of Recurrent Clostridium Difficile infection (RCDI) in the United States (5-35% per Emerging Infectious Diseases 2008; 14: 929-31). RCDI is generally treated with additional courses of various antibiotics. However, subsets of patients continue to suffer from persistent abdominal pain, diarrhea and Clostridium Difficile infection. Fecal instillations from healthy donors have been administered through enemas and nasogastric tube with significant clinical efficacy. A large number of patients, however, have concerns about regurgitation and lack of retention of fecal instillation from healthy donors. To overcome this problem, we have administered fecal bacteriotherapy from healthy donors to jejunum and colon of 3 patients with RCDI. Methods: The study subjects were three women (ages 85, 53 and 31 yrs) with RCDI for >6-months, and >5 recurrences. Patients had received all standard RCDI treatments, including the 2010 guideline recommendations from the Infectious Diseases Society of America (IDSA), but still had persistent crampy abdominal pain, chronic diarrhea and weight-loss. On colonoscopy, the severity of disease ranged from severe left-sided colitis in one-case to mild right-sided colitis in the other two. Fecal samples from healthy donors were collected, homogenized, liquefied, and processed in the microbiology lab. Varying amounts of fecal instillations were administered to RCDI patients during colonoscopy and enteroscopy. Results: Outcome: all three patients had complete resolution of symptoms within a few days after administration of fecal bacteriotherapy. Follow-up: all three patients have been symptom-free and off antibiotics for the last 3-22 weeks. Conclusion: These observations suggest fecal bacteriotherapy administered in the jejunum and colon is a highly effective treatment option in RCDI patients. Combined jejunal and colonic instillation of fecal bacteriotherapy achieves prompt resolution of RCDI symptoms. Additional controlled trials are required for further validation of this therapy.

Intestinal Spirochetosis as a Presenting Symptom of Undiagnosed HIV Infection

Purpose: A 47-year-old male presented with a 2-month history of diarrhea, averaging 3-20 bowel movements per day. The stools were watery, non-mucoid, malodorous, and primarily postprandial in nature. There was no fecal urgency, tenesmus, or incontinence. He developed rectal bleeding and he reported chronic, generalized abdominal pain following meals. Despite an excellent appetite, he had frequent nausea and lost approximately 40 pounds in weight over the preceding two months. He had no fever, but did note night sweats. He denied any history of pancreatic disease, exotic travel, antibiotic use, sick contacts, or anal intercourse. Stools studies were unrevealing for fecal leukocytes, C. difficile toxin, bacterial culture (including AFB), ova and parasite examination (including Giardia antigen), Cryptosporidium/Isospora, and qualitative fecal fat. Stool pH was 6.5, arguing against carbohydrate malabsorption. Celiac serologies, B12, folate, zinc, and fat-soluble vitamin levels were all normal. CBC demonstrated pancytopenia. HIV serology and confirmatory Western blot were positive. EGD with duodenal biopsies was normal. Colonoscopy revealed subtle erythema in the ascending and descending colon, with biopsies demonstrating active colitis and colonic spirochetosis. Results: Histological findings of intestinal spirochetosis are often an incidental finding on colonic biopsies. Intestinal spirochetes constitute a heterogeneous group of bacteria predominantly associated with low socioeconomic status, especially in developing countries. HIV infected individuals and homosexuals are at increased risk of colonization by these organisms. In the United States and other developed countries, 30-40% of cases have been reported in HIV/AIDS patients, as well as in Hepatitis C patients. The clinical course typically includes prolonged bouts of watery diarrhea, weight loss, and vague crampy abdominal pain occasionally associated with rectal bleeding. Symptoms are notably relieved with fasting. Conclusion: Definitive diagnosis of spirochetosis is histologic. Treatment is supportive in most cases, however in patients with severe symptoms, a 2 week course of metronidazole has been proven to be effective.Figure. 60X: Silver Stain showing spiral shaped organisms coating the colonic mucosa consistent with Brachyspira.