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Cronkhite-Canada syndrome polyps infiltrated with IgG4-positive plasma cells.
World J Clin Cases. 2016 Aug 16; 4(8):248-52.WJ

Abstract

Cronkhite-Canada syndrome (CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40-year-old male patient was admitted to our hospital because of diarrhea and hypogeusia associated with weight loss for 4 mo. On physical examination, skin pigmentation, dystrophic nail changes and alopecia were noted. He had no alike family history. Laboratory results revealed low levels of serum albumin (30.1 g/L, range: 35.0-55.0 g/L), serum potassium (2.61 mmol/L, range: 3.5-5.5 mmol/L) and blood glucose (2.6 mmol/L, range: 3.9-6.1 mmol/L). The erythrocyte sedimentation rate was elevated to 17 mm/h (range: 0-15 mm/h). X-ray of chest and mandible was normal. The endoscopic examination showed multiple sessile polyps in the stomach, small bowel and colorectum. Histopathologic examination of biopsies obtained from those polyps showed hyperplastic change, cystic dilatation and distortion of glands with inflammatory infiltration, eosinophilic predominance and stromal edema. Immune staining for IgG4 plasma cells was positive in polyps of stomach and colon. The patient was diagnosed of CCS and treated with steroid, he had a good response to steroid. Both histologic findings and treatment response to steroid suggested an autoimmune mechanism underling CCS.

Authors+Show Affiliations

Ru-Ying Fan, Xiao-Wei Wang, Li-Jun Xue, Jian-Qiu Sheng, Department of Gastroenterology, Beijing Military General Hospital, Beijing 100700, China.Ru-Ying Fan, Xiao-Wei Wang, Li-Jun Xue, Jian-Qiu Sheng, Department of Gastroenterology, Beijing Military General Hospital, Beijing 100700, China.Ru-Ying Fan, Xiao-Wei Wang, Li-Jun Xue, Jian-Qiu Sheng, Department of Gastroenterology, Beijing Military General Hospital, Beijing 100700, China.Ru-Ying Fan, Xiao-Wei Wang, Li-Jun Xue, Jian-Qiu Sheng, Department of Gastroenterology, Beijing Military General Hospital, Beijing 100700, China.Ru-Ying Fan, Xiao-Wei Wang, Li-Jun Xue, Jian-Qiu Sheng, Department of Gastroenterology, Beijing Military General Hospital, Beijing 100700, China.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

27574615

Citation

Fan, Ru-Ying, et al. "Cronkhite-Canada Syndrome Polyps Infiltrated With IgG4-positive Plasma Cells." World Journal of Clinical Cases, vol. 4, no. 8, 2016, pp. 248-52.
Fan RY, Wang XW, Xue LJ, et al. Cronkhite-Canada syndrome polyps infiltrated with IgG4-positive plasma cells. World J Clin Cases. 2016;4(8):248-52.
Fan, R. Y., Wang, X. W., Xue, L. J., An, R., & Sheng, J. Q. (2016). Cronkhite-Canada syndrome polyps infiltrated with IgG4-positive plasma cells. World Journal of Clinical Cases, 4(8), 248-52. https://doi.org/10.12998/wjcc.v4.i8.248
Fan RY, et al. Cronkhite-Canada Syndrome Polyps Infiltrated With IgG4-positive Plasma Cells. World J Clin Cases. 2016 Aug 16;4(8):248-52. PubMed PMID: 27574615.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cronkhite-Canada syndrome polyps infiltrated with IgG4-positive plasma cells. AU - Fan,Ru-Ying, AU - Wang,Xiao-Wei, AU - Xue,Li-Jun, AU - An,Ran, AU - Sheng,Jian-Qiu, PY - 2016/04/23/received PY - 2016/05/24/revised PY - 2016/06/14/accepted PY - 2016/8/31/entrez PY - 2016/8/31/pubmed PY - 2016/8/31/medline KW - Autoimmune mechanism KW - Cronkhite-Canada syndrome KW - Gastrointestinal polyposis KW - IgG4 plasma cells SP - 248 EP - 52 JF - World journal of clinical cases JO - World J Clin Cases VL - 4 IS - 8 N2 - Cronkhite-Canada syndrome (CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40-year-old male patient was admitted to our hospital because of diarrhea and hypogeusia associated with weight loss for 4 mo. On physical examination, skin pigmentation, dystrophic nail changes and alopecia were noted. He had no alike family history. Laboratory results revealed low levels of serum albumin (30.1 g/L, range: 35.0-55.0 g/L), serum potassium (2.61 mmol/L, range: 3.5-5.5 mmol/L) and blood glucose (2.6 mmol/L, range: 3.9-6.1 mmol/L). The erythrocyte sedimentation rate was elevated to 17 mm/h (range: 0-15 mm/h). X-ray of chest and mandible was normal. The endoscopic examination showed multiple sessile polyps in the stomach, small bowel and colorectum. Histopathologic examination of biopsies obtained from those polyps showed hyperplastic change, cystic dilatation and distortion of glands with inflammatory infiltration, eosinophilic predominance and stromal edema. Immune staining for IgG4 plasma cells was positive in polyps of stomach and colon. The patient was diagnosed of CCS and treated with steroid, he had a good response to steroid. Both histologic findings and treatment response to steroid suggested an autoimmune mechanism underling CCS. SN - 2307-8960 UR - https://www.unboundmedicine.com/medline/citation/27574615/Cronkhite_Canada_syndrome_polyps_infiltrated_with_IgG4_positive_plasma_cells_ L2 - http://www.wjgnet.com/2307-8960/full/v4/i8/248.htm DB - PRIME DP - Unbound Medicine ER -
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