BACKGROUND Crohn’s disease(CD)can affect the entire gastrointestinal tract.Proximal small bowel(SB)lesions are associated with a significant risk of stricturing disease and multiple abdominal surgeries.The assessment...BACKGROUND Crohn’s disease(CD)can affect the entire gastrointestinal tract.Proximal small bowel(SB)lesions are associated with a significant risk of stricturing disease and multiple abdominal surgeries.The assessment of SB in patients with CD is therefore necessary because it may have a significant impact on prognosis with potential therapeutic implications.Because of the weak correlation that exists between symptoms and endoscopic disease activity,the“treat-to-target”paradigm has been developed,and the associated treatment goal is to achieve and maintain deep remission,encompassing both clinical and endoscopic remission.Small bowel capsule endoscopy(SBCE)allows to visualize the mucosal surface of the entire SB.At that time,there is no recommendation regarding the use of SBCE during follow-up.AIM To investigate the impact of SBCE in a treat-to-target strategy in patients with CD.METHODS An electronic literature search was conducted in PubMed and Cochrane library using the following search terms:“capsule endoscopy”,in combination with“Crohn’s disease”and“treat-to-target”or synonyms.Two authors independently reviewed titles and abstracts identified by the search strategy after duplicates were removed.Following the initial screening of abstracts,all articles containing information about SBCE in the context of treat-to-target strategy in patients with CD were included.Full-text articles were retrieved,reference lists were screened manually to identify additional studies.RESULTS Forty-seven articles were included in this review.Two indexes are currently used to quantify disease activity using SBCE,and there is good correlation between them.SBCE was shown to be useful for disease reclassification in patients who are suspected of having or who are diagnosed with CD,with a significant incremental diagnostic yield compared to other diagnostic modalities.Nine studies also demonstrated that the mucosal healing can be evaluated by SBCE to monitor the effect of medical treatment in patients with CD.This review also demonstrated that SBCE can detect post-operative recurrence to a similar extent as ileocolonoscopy,and proximal SB lesions that are beyond the reach of the colonoscope in over half of the patients.CONCLUSION SBCE could be incorporated in the treat-to-target algorithm for patients with CD.Randomized controlled trials are required to confirm its usefulness and reliability in this indication.展开更多
BACKGROUND Hermansky-Pudlak syndrome(HPS)is a rare autosomal recessive disorder characterized by oculocutaneous albinism,platelet storage pool deficiency and systemic complications associated with ceroid deposition in...BACKGROUND Hermansky-Pudlak syndrome(HPS)is a rare autosomal recessive disorder characterized by oculocutaneous albinism,platelet storage pool deficiency and systemic complications associated with ceroid deposition in the reticuloendothelial system.HPS types 1 and 4 are associated with Crohn’s disease(CD)-like gastrointestinal disorders,such as granulomatous enterocolitis or perianal disease.Cases of colitis can be particularly severe and,before the use of anti-tumor necrosis factor alpha(TNFα)therapy had become common,were reported as showing poor responsiveness to medical treatment.CASE SUMMARY We present the case of a 51-year-old albino woman who presented with acute severe colitis that led to the diagnosis of HPS.Histologic findings of biopsy samples showed chronic inflammation with deep ulcerations,and granulomas without caseous necrosis.Molecular genetic analysis confirmed HPS type 1,with a homozygous 27 base-pair deletion in exon 20 of the HPS1 gene.Once the patient’s bleeding diathesis was corrected by platelet transfusion,the granulomatous colitis responded dramatically to a medical treatment regimen that included corticosteroids,azathioprine and infliximab;this regimen is similar to that used in CD treatment.Although it remains unclear if the granulomatous enterocolitis in HPS is due to ceroid deposition or reflects the co-existence of CD and HPS,the fact that this case of HPS-related granulomatous colitis responded to the same therapeutic approach used in CD suggests that this type of colitis may result from HPS patients’genetic susceptibility to CD.CONCLUSION We report a case of severe colitis that led to the diagnosis of HPS,which was responsive to azathioprine and infliximab.展开更多
文摘BACKGROUND Crohn’s disease(CD)can affect the entire gastrointestinal tract.Proximal small bowel(SB)lesions are associated with a significant risk of stricturing disease and multiple abdominal surgeries.The assessment of SB in patients with CD is therefore necessary because it may have a significant impact on prognosis with potential therapeutic implications.Because of the weak correlation that exists between symptoms and endoscopic disease activity,the“treat-to-target”paradigm has been developed,and the associated treatment goal is to achieve and maintain deep remission,encompassing both clinical and endoscopic remission.Small bowel capsule endoscopy(SBCE)allows to visualize the mucosal surface of the entire SB.At that time,there is no recommendation regarding the use of SBCE during follow-up.AIM To investigate the impact of SBCE in a treat-to-target strategy in patients with CD.METHODS An electronic literature search was conducted in PubMed and Cochrane library using the following search terms:“capsule endoscopy”,in combination with“Crohn’s disease”and“treat-to-target”or synonyms.Two authors independently reviewed titles and abstracts identified by the search strategy after duplicates were removed.Following the initial screening of abstracts,all articles containing information about SBCE in the context of treat-to-target strategy in patients with CD were included.Full-text articles were retrieved,reference lists were screened manually to identify additional studies.RESULTS Forty-seven articles were included in this review.Two indexes are currently used to quantify disease activity using SBCE,and there is good correlation between them.SBCE was shown to be useful for disease reclassification in patients who are suspected of having or who are diagnosed with CD,with a significant incremental diagnostic yield compared to other diagnostic modalities.Nine studies also demonstrated that the mucosal healing can be evaluated by SBCE to monitor the effect of medical treatment in patients with CD.This review also demonstrated that SBCE can detect post-operative recurrence to a similar extent as ileocolonoscopy,and proximal SB lesions that are beyond the reach of the colonoscope in over half of the patients.CONCLUSION SBCE could be incorporated in the treat-to-target algorithm for patients with CD.Randomized controlled trials are required to confirm its usefulness and reliability in this indication.
文摘BACKGROUND Hermansky-Pudlak syndrome(HPS)is a rare autosomal recessive disorder characterized by oculocutaneous albinism,platelet storage pool deficiency and systemic complications associated with ceroid deposition in the reticuloendothelial system.HPS types 1 and 4 are associated with Crohn’s disease(CD)-like gastrointestinal disorders,such as granulomatous enterocolitis or perianal disease.Cases of colitis can be particularly severe and,before the use of anti-tumor necrosis factor alpha(TNFα)therapy had become common,were reported as showing poor responsiveness to medical treatment.CASE SUMMARY We present the case of a 51-year-old albino woman who presented with acute severe colitis that led to the diagnosis of HPS.Histologic findings of biopsy samples showed chronic inflammation with deep ulcerations,and granulomas without caseous necrosis.Molecular genetic analysis confirmed HPS type 1,with a homozygous 27 base-pair deletion in exon 20 of the HPS1 gene.Once the patient’s bleeding diathesis was corrected by platelet transfusion,the granulomatous colitis responded dramatically to a medical treatment regimen that included corticosteroids,azathioprine and infliximab;this regimen is similar to that used in CD treatment.Although it remains unclear if the granulomatous enterocolitis in HPS is due to ceroid deposition or reflects the co-existence of CD and HPS,the fact that this case of HPS-related granulomatous colitis responded to the same therapeutic approach used in CD suggests that this type of colitis may result from HPS patients’genetic susceptibility to CD.CONCLUSION We report a case of severe colitis that led to the diagnosis of HPS,which was responsive to azathioprine and infliximab.