[Protein-losing Enteropathy Caused By Acute Graft-versus-host Disease - a Case-report]
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The authors report the case of a 41-year-old woman who developed an acute graft-versus-host disease following bone marrow transplantation for post-polycythemic myeloid metaplasia; liver, cutaneous and intestinal lesions were present, associated with a severe protein-losing enteropathy. Symptoms, diagnostic procedures and therapy of intestinal graft-versus-host disease are reviewed. The intestinal clearance of alpha-1 antitrypsin was particularly useful to assess the response to medial treatment, which induced a complete remission.Keywords:
Protein losing enteropathy
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Immunosuppression
Intestinal Failure
Short Bowel Syndrome
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Objective To enhance the understanding of the systemic lupus erythematosus (SLE) and chronic intestinal pseudo-obstruction.Methods Clinical manifestations,laboratory and serologic abnormalities,treatment and prognosis of SLE patients with chronic intestinal pseudo-obstruction were analyzed.Results The average age at the onset of disease was 36 years (range from 21~56).The average course from initiation of clinical symptoms to diagnosis was 31 months.Five patients died.Nine patients had renal involvement,8 had cardiovascular system,and 6 had hematological system involvement.Involvement of joints,skin,central nervous system and respiratory system also existed in various frequencies.Two patients with intestinal obstruction as the first presentation were misdiagnosed as ulcerative colitis and enteritis.Two patients were performed surgical intervention,and pathological examination demonstrated mesenteric vasculitis.Conclusion Intestinal obstruction is a life-threatening complication of SLE,usually associated with multisystem activity and high mortality.Mesenteric vasculitis is the major pathogenic basis in patients with intestinal obstruction in SLE.Early diagnosis and treatment with corticosteroid can improve the prognosis.
Intestinal pseudo-obstruction
Systemic vasculitis
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Three children aged between 7 and 11 years, after an acute onset of diarrhoea and vomiting, developed protracted diarrhoea and severe loss of weight. None had been abroad. No significant aetiological agent was found. There was evidence of acute inflammatory disease on proximal small intestinal biopsy, and some evidence of more widespread gut involvement--of the rectum in Cases 1 and 2, and the terminal ileum in Cases 2 and 3. The disease resolved spontaneously and without relapse.
Etiology
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Protein-loosing enteropathy in patients with inflammatory bowel diseases (IBDs) is an uncommon complication, but should be considered in any patient with hypoproteinemia in whom other causes have been excluded such as concomitant hepatic disease, severe malnutrition or proteinuria. The diagnosis is based on determination of fecal alpha-1 antitripsin clearance and stool analysis. Prognosis depends upon the patient and the disease location, severity and complication. Treatment is directed at control of the underlying IBDs but also includes albumin infusion in the most severe cases of hypoalbuminemia and fluid retention, dietary modifications for recovery and maintenance of nutritional status and supportive care to prevent further complication like deep venous thrombosis. Surgery is not curative in Crohn's disease patients, but in severe protein-loosing enteropathy with severe disease, not responding to conventional therapy, it may be the best choice.
Hypoalbuminemia
Protein losing enteropathy
Hypoproteinemia
Concomitant
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Protein losing enteropathy
Colorectal Surgery
Surgical oncology
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Protein losing enteropathy
Lymphoproliferative Disorders
Immunoglobulin A
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Chronic graft-versus-host disease (GVHD) is an increasingly common complication of solid organ transplant recipients. It can affect a wide range of epithelial target tissues including the gastrointestinal tract. A 40-year-old female presented to our hospital with two days of frequent high volume and watery diarrhea. Associated symptoms included nausea, vomiting, and diffuse abdominal pain. Her medical history included type I diabetes mellitus and deceased donor kidney-pancreas transplant. She was maintained on immunosuppression with azathioprine, tacrolimus, and prednisone. Her physical examination was notable for cachexia, diffuse abdominal pain, and a confluent and erythematous macular rash. Laboratory values included a white blood cell count of 0.8 x1000/uL (range 4.0-10), absolute neutrophil count 0.7x1000/uL (range 1.0-11), hemoglobin 6.9g/dL (range 12-18), platelet 109x1000/uL (range 140-440), total bilirubin 12.1mg/dL (range <1.20), direct bilirubin 9.3 (range <0.20), alkaline phosphatase 294U/L (range 30-130), and alanine aminotransferase 124U/L (range 0-34). She was started on intravenous immune gamma-globulin and broad spectrum anti-bacterial, viral and fungal agents. Computerized tomography scan of the abdomen and pelvis with IV contrast (Figure 1) showed bowel wall thickening of the colon moreso than small bowel, consistent with GVHD. She underwent a flexible sigmoidoscopy for definitive diagnosis. Pathology from colonic biopsy (Figure 2, 3) showed severe apoptosis, crypt attenuation, and paucity of inflammation without viral inclusions or neutrophils, pathologically characteristic of GVHD. She was started on high dose steroids and ruxolitinib (janus kinase inhibitor) to help control her GVHD. She had an unfortunate hospital course complicated by severe clostridium difficile colitis, enterococcus bacteremia, and eventually hypoxemic respiratory failure. She was transferred to the medical intensive care unit and transitioned to hospice care where she ultimately died. Risk factors for GVHD include HLA disparity and older age upon organ transplantation. Symptoms of gastrointestinal GVHD are nebulous but can include anorexia, weight loss, and chronic diarrhea. Given the myriad symptoms of GVHD the prudent gastroenterologist must always be aware of this diagnosis. Diagnosis is confirmed via biopsy and scored according to the 2014 revised NIH criteria; she had severe chronic GVHD with an expected two year mortality of 38%.1656_A Figure 1. CT abdomen/pelvis, coronal, white arrow points to diffuse colonic bowel wall thickening1656_B Figure 2. Colon biopsy, black arrow points to apoptotic body1656_C Figure 3. Colon biopsy, black arrows points to crypt attenuation
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We read with interest the article by Yakoub-Agha et al. in the December issue of Transplantation about the role of video-capsule endoscopy (VCE) in diagnosing acute gastrointestinal graft-versus-host disease (aGI-GvHD) (1). The article underlined the importance to include the small intestine in the evaluation of aGI-GvHD, since the development of graft-versus-host disease (GvHD)-related diarrhea is mainly attributed to GvHD in the small intestine (2). In this regard, VEC offers a safe possibility to examine the complete small intestine as the authors concluded. This is important, because during the course of aGI-GvHD, follow-up examinations may be necessary and patients may acquire conditions (bleeding, ulcerations) that render standard endoscopies risky. The shortcoming of VEC is the lack of biopsies. As the authors pointed out, the diagnosis of GvHD is “problematic because of lack of specific symptoms.” This is valid as well for the macroscopic endoscopic findings of aGI-GvHD. They are not specific for GvHD and there is no correlation between the endoscopic observations and the histological findings (3). At our institution, we therefore prefer the push enteroscopy that allows the examination of the small intestine by the endoscopist and to obtain biopsies. With this form of endoscopy, we were able to diagnose GvHD in the small intestine in patients who suffered from diarrhea after allogeneic blood stem cell transplantation lacking pathological findings in previous conventional endoscopies (gastroscopy, colonoscopy). We therefore recommend push enteroscopy of the small intestine in patients with suspected aGI-GVHD. However, the article by Yakoub-Agha et al. proposes a new, interesting, and valuable additional diagnostic tool of diarrhea after allogeneic blood stem cell transplantation. Axel Schulenburg Peter Kalhs Werner Rabitsch Bone Marrow Transplantation Unit Department of Medicine I Medical University of Vienna Vienna, Austria
Enteroscopy
Obscure Gastrointestinal Bleeding
Sigmoidoscopy
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We report the case of a 63-year-old female patient with liver cirrhosis who presented with symptoms of severe hypoalbuminaemia and diarrhoea. After ruling out other causes of hypoalbuminaemia and confirmation of an elevated faecal α-1 antitrypsin clearance, the diagnosis of protein-losing enteropathy (PLE) could be established. Since PLE is a syndrome caused by various diseases, classified into erosive and non-erosive gastrointestinal diseases or lymphatic obstruction, an extensive work-up was necessary, establishing the final diagnosis of Crohn’s disease.
Protein losing enteropathy
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