Mucosal Genomics Implicate Lymphocyte Activation and Lipid Metabolism in Refractory Environmental Enteric Dysfunction

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Microscopic enteritis: Bucharest consensus

Microscopic enteritis (ME) is an inflammatory condition of the small bowel that leads to gastrointestinal symptoms, nutrient and micronutrient deficiency. It is characterised by microscopic or sub-microscopic abnormalities such as microvillus changes and enterocytic alterations in the absence of definite macroscopic changes using standard modern endoscopy. This work recognises a need to characterize disorders with microscopic and submicroscopic features, currently regarded as functional or non-specific entities, to obtain further understanding of their clinical relevance. The consensus working party reviewed statements about the aetiology, diagnosis and symptoms associated with ME and proposes an algorithm for its investigation and treatment. Following the 5^th International Course in Digestive Pathology in Bucharest in November 2012, an international group of 21 interested pathologists and gastroenterologists formed a working party with a view to formulating a consensus statement on ME. A five-step agreement scale (from strong agreement to strong disagreement) was used to score 21 statements, independently. There was strong agreement on all statements about ME histology (95%-100%). Statements concerning diagnosis achieved 85% to 100% agreement. A statement on the management of ME elicited agreement from the lowest rate (60%) up to 100%. The remaining two categories showed general agreement between experts on clinical presentation (75%-95%) and pathogenesis (80%-90%) of ME. There was strong agreement on the histological definition of ME. Weaker agreement on management indicates a need for further investigations, better definitions and clinical trials to produce quality guidelines for management. This ME consensus is a step toward greater recognition of a significant entity affecting symptomatic patients previously labelled as non-specific or functional enteropathy.     

Steroid Pulse Therapy for Protein‐losing Enteropathy after the Fontan Operation

A 19‐year‐old male with Fontan circulation developed protein‐losing enteropathy associated with acute enteritis. Although his central venous pressure was in the normal range, subcutaneous high molecular heparin injection and oral predonisolone administration were not effective. We initiated intravenous high‐dose methyl‐predonisolone (15 mg/kg/day) for 3 days followed by oral predonisolone (0.5 mg/kg/day) for 4 days and repeated the course in 2 weeks. The serum protein and albumin increased to the normal level at 2 months after pulse therapy. The patient has not shown any recurrence of such protein‐losing enteropathy for 2 years without any steroid agents.     

Autoimmune Enteropathy in an Adult with Autoimmune Multisystemic Involvement

We describe the case of a 58-year-old woman with autoimmune enteropathy associated with thyroiditis, gastritis, transitory neutropenia, sicca syndrome and severe axonal polyneuropathy of autoimmune origin. Enterocyte autoantibodies were not detected. However, predisposition to autoimmune disease was indicated by the presence of high titres of anti-gastric parietal cell, anti-thyroglobulin, anti-thyroid peroxidase and anti-neutrophil antibodies. CD4+ and CD8+ lymphocytes were equally distributed in the lamina propria of the small intestine, but CD8+ cells were highly represented among intraepithelial lymphocytes.     

Enteropathy in Niemann-Pick disease

Three consecutive cases of Niemann-Pick disease with predominant enteropathy were seen. The diagnosis was confirmed by the demonstration of typical foamy cells in the bone marrow, small intestinal mucosa, and liver. The enteropathy was apparent in steatorrhoea, xylose malabsorption, protein loss etc. The hypothesis of foamy cell infiltration of the lamina propria of the small intestinal mucosa as a cause of malabsorption is proposed.     

Gliadin- and immunoglobulin-containing cells of small intestinal lamina propria in childhood coeliac disease

One hundred twenty-five small intestinal biopsies of coeliac children and controls were investigated prospectively for gliadin binding by specific immunofluorescent staining of lamina propria cells, using a TRITC rabbit IgG antigliadin-conjugate. In parallel, sera were investigated for serum gliadin antibodies by a red cell immunosorbent fluorescence test (RIFT). There was no epithelial or reticulin staining found with antigliadin. Exclusively in coeliac disease, i.e. in all active coeliacs, and in 71% of coeliacs on a gluten-free diet, gliadinand immunoglobulin-containing cells were detected. Among these, there was a relatively higher proportion of IgM- and IgG-containing cells, compared to IgA. They are possibly involved in a local B-cell reaction to gliadin. Their absolute numbers per mucosal tissue unit were small. There was no correlation found between serum gliadin antibody titres of different immunoglobulin classes, and the respective local gliadin- and immunoglobulin-containing cells. It is concluded that lamina propria cells are not a main source of serum gliadin antibodies. Pathogenetic and diagnostic consequences of these data await further clarification.Supported by Deutsche Forschungsgemeinschaft Gr 278/6 and Ste 305/1     

Abdominal computed tomography in refractory coeliac disease and enteropathy associated T-cell lymphoma

AIM: To evaluate computed tomography (CT) findings, useful to suggest the presence of refractory celiac disease (RCD) and enteropathy associated T cell lymphoma (EATL). METHODS: Coeliac disease (CD) patients were divided into two groups. Group I: uncomplicated CD (n = 14) and RCD type I (n = 10). Group II: RCD type II (n = 15) and EATL (n = 7). RESULTS: Both groups showed classic signs of CD on CT. Intussusception was seen in 1 patient in group I vs 5 in group II (P = 0.06). Lymphadenopathy was seen in 5 patients in group II vs no patients in group I (P = 0.01). Increased number of small mesenteric vessels was noted in 20 patients in group I vs 11 in group II (P = 0.02). Eleven patients (50%) in group II had a splenic volume < 122 cm3 vs 4 in group I (14%), 10 patients in group I had a splenic volume > 196 cm3 (66.7%) vs 5 in group II (33.3%) P = 0.028. CONCLUSION: CT scan is a useful tool in discriminating between CD and (Pre) EATL. RCD II and EATL showed more bowel wall thickening, lymphadenopathy and intussusception, less increase in number of small mesenteric vessels and a smaller splenic volume compared with CD and RCD I.     

An Unusual Treatment for Protein Losing Enteropathy

We present the case of a 22‐year‐old man with a Fontan circulation who suffered from intractable protein losing enteropathy for an 8‐year period necessitating several hospital admissions for recurrent pleural effusions and edema. Despite trying several recognized medical therapies to alleviate his protein losing enteropathy, his condition did not improve until the introduction of loperamide for troublesome diarrhea. Following this, his symptoms and serum albumin improved dramatically and he has not required any further hospital admissions at 22 months follow up.