title: The role of immunohistochemistry in idiopathic chronic intestinal pseudoobstruction (CIPO): a case-control study.
abstract: BACKGROUND
  Chronic intestinal pseudoobstruction (CIPO) is classified into enteric visceral myopathies, neuropathies, and/or mesenchymopathies. Although the histology usually permits to highlight pathologic abnormalities of CIPO, it fails in almost a third of cases. The yield of a systematic immunohistochemistry needs to be evaluating.
  
  
  METHODS
  Twenty-one adult patients with idiopathic CIPO [11 females/10 males, median age 23.1 (0.3 to 57) y] were included and compared with 27 control and 10 with mechanical obstruction patients. Comparison between standard histology (hematoxylin and eosin-stained sections) and systematic immunohistochemistry using muscular (smooth muscle alpha-actin, desmin, and smoothelin-A/B), nervous (Hu C/D, Bcl-2, and S100 protein), and mesenchymal (CD117) markers was carried out.
  
  
  RESULTS
  Histology showed neuromuscular abnormalities in 13 out of 21 (62%) patients, consisting of enteric visceral myopathy in 9 (43%) patients, enteric visceral neuropathy in 2 (9.5%), and mixed neuromyopathy in 2 (9.5%). Among the 8 patients who had no histologic structural abnormality, 6 patients (75%) had underlying abnormalities detected with immunohistochemistry: immunostain with Hu C/D detected a hypoganglionosis (<50 ganglion cells/cm) in 6 out of 21 (29%) patients, 4 of them undiagnosed on standard histology; CD117 (c-kit) detected a interstitial cells of Cajal defect in 10 out of 21 (48%) patients, 2 of them with no histologic structural abnormality. Smoothelin-A/B and desmin were useless as normally expressed in all patients with no myopathy; although it was not relevant in ileal samples (86% of abnormal expression in control patients), smooth muscle alpha-actin showed an abnormal expression in 2 CIPO patients (2/21).
  
  
  CONCLUSIONS
  Immunohistochemistry using Hu C/D and CD117 antibodies combined to the standard histology increased the yield of detection of neuromuscular abnormalities in idiopathic CIPO patients.
authors.affiliation: Pôle des Maladies de l'appareil Digestif, Unité de Gastroentérologie et d'assistance Nutritive, Hôpital Beaujon, Clichy, France.
authors.forename: Aurelien
authors.initials: A
authors.lastname: Amiot
authors.forename: Dominique
authors.initials: D
authors.lastname: Cazals-Hatem
authors.forename: Francisca
authors.initials: F
authors.lastname: Joly
authors.forename: Anne
authors.initials: A
authors.lastname: Lavergne-Slove
authors.forename: Michel
authors.initials: M
authors.lastname: Peuchmaur
authors.forename: Yoram
authors.initials: Y
authors.lastname: Bouhnik
authors.forename: Pierre
authors.initials: P
authors.lastname: Bedossa
authors.forename: Bernard
authors.initials: B
authors.lastname: Messing
mesh_terms: D000199:Actins / Q000032:analysis; D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000032:analysis*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D004396:Coloring Agents; D003598:Cytoskeletal Proteins / Q000032:analysis; D003893:Desmin / Q000032:analysis; D051959:ELAV Proteins / Q000032:analysis; D000067778:ELAV-Like Protein 3; D000067779:ELAV-Like Protein 4; D004801:Eosine Yellowish-(YS); D005260:Female; D006416:Hematoxylin; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007418:Intestinal Pseudo-Obstruction / Q000378:metabolism* / Q000473:pathology; D007422:Intestines / Q000737:chemistry* / Q000473:pathology; D008297:Male; D008648:Mesoderm / Q000737:chemistry* / Q000473:pathology; D008875:Middle Aged; D009124:Muscle Proteins / Q000032:analysis; D009130:Muscle, Smooth / Q000737:chemistry* / Q000473:pathology; D009474:Neurons / Q000737:chemistry* / Q000473:pathology; D011237:Predictive Value of Tests; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000032:analysis; D019009:Proto-Oncogene Proteins c-kit / Q000032:analysis; D009418:S100 Proteins / Q000032:analysis; D013194:Staining and Labeling / Q000379:methods; D055815:Young Adult
chemical_list: D000199:Actins; D015415:Biomarkers; D004396:Coloring Agents; D003598:Cytoskeletal Proteins; D003893:Desmin; D051959:ELAV Proteins; D000067778:ELAV-Like Protein 3; D000067779:ELAV-Like Protein 4; C490264:ELAVL3 protein, human; C498010:ELAVL4 protein, human; D009124:Muscle Proteins; D019253:Proto-Oncogene Proteins c-bcl-2; D009418:S100 Proteins; C100762:SMTN protein, human; D019009:Proto-Oncogene Proteins c-kit; D004801:Eosine Yellowish-(YS); D006416:Hematoxylin