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Pediatric gastrointestinal pathology (view source)
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===IHC=== | ===IHC=== | ||
*Carcinoembryonic antigen (CEA) +ve.<ref name=Ref_Sternberg4>{{Ref Sternberg4|}}</ref> | *[[Carcinoembryonic antigen]] (CEA) +ve.<ref name=Ref_Sternberg4>{{Ref Sternberg4|}}</ref> | ||
===EM=== | ===EM=== | ||
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===General=== | ===General=== | ||
*Genetic disorder:<ref>{{OMIM|142623}}</ref> | *Genetic disorder:<ref>{{OMIM|142623}}</ref> | ||
** | **5-10% familial; RET gene most commonly mutated. | ||
**Several genes involved. | **Several genes involved. | ||
**Inheritance pattern variable. | **Inheritance pattern variable. | ||
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Pathology: | Pathology: | ||
*Parasympathetic ganglion cells in submucosal plexus (Meissner plexus) and myenteric plexus (Auerbach plexus) - absent.<ref name=pmid17139897>{{Cite journal | last1 = Vorobyov | first1 = GI. | last2 = Achkasov | first2 = SI. | last3 = Biryukov | first3 = OM. | title = Hirschsprung's disease in adults. | journal = Acta Chir Iugosl | volume = 53 | issue = 2 | pages = 113-6 | month = | year = 2006 | doi = | PMID = 17139897 }}</ref> | *Failure of neural crest cell migration | ||
**Parasympathetic ganglion cells in submucosal plexus (Meissner plexus) and myenteric plexus (Auerbach plexus) - absent.<ref name=pmid17139897>{{Cite journal | last1 = Vorobyov | first1 = GI. | last2 = Achkasov | first2 = SI. | last3 = Biryukov | first3 = OM. | title = Hirschsprung's disease in adults. | journal = Acta Chir Iugosl | volume = 53 | issue = 2 | pages = 113-6 | month = | year = 2006 | doi = | PMID = 17139897 }}</ref> | |||
Notes: | Notes: | ||
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Features: | Features: | ||
*Dilated bowel; stuffed sausage-look. | *Dilated bowel; stuffed sausage-look. | ||
Classification: | |||
*Short-segment (75-80%): Rectum, distal sigmoid | |||
*Long-segment HD (10-20%): Beyond splenic flexure | |||
*Total colonic aganglionosis (5-15%): Entire colon.<ref name=pmid25395999>{{Cite web | last = | first = | title = Diagnosis of Hirschsprung's disease with particular emphasis on histopathology. A systematic review of current literature | url = https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/ | publisher = | date = | accessdate = 19 August 2017 }}</ref> | |||
**[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/figure/F0001/ Classification of Hirschsprung's disease according to the aganglionic segment length (https://www.ncbi.nlm.nih.gov)] | |||
Image: | Image: | ||
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Features: | Features: | ||
*Ganglion cells missing in submucosal plexus and myenteric plexus. | *Ganglion cells missing in submucosal plexus and myenteric plexus. | ||
**Increasing ganglia proximally into transition zone | |||
*Hypertrophy of neural plexuses. | |||
**Many nerve trunks > 40 μm | |||
*Abnormal submucosal blood vessels may be seen | |||
*+/-Submucosal fibrosis. | *+/-Submucosal fibrosis. | ||
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**Nerve fibres +ve control. | **Nerve fibres +ve control. | ||
Others | Others<ref>{{Cite book | last1 = Lin | first1 = Fan | last2 = Prichard | first2 = Jeffrey | title = Handbook of practical immunohistochemistry : frequently asked question | date = | publisher = | location = | isbn = 9781493915774 | pages = }}</ref> | ||
* | : | ||
* | *Calretinin ~ -ve, Usually negative in hypertrophied nerve fibers in Hirschsprung’s disease, superior to acetylcholinesterase. | ||
*NSE ~ -ve, Highlights ganglion cells to exclude Hirschsprung’s disease; specific but not very sensitive. | |||
==Meconium ileus== | ==Meconium ileus== | ||
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*Classically due to ''[[cystic fibrosis]]''. | *Classically due to ''[[cystic fibrosis]]''. | ||
*May lead to ''[[meconium peritonitis]]''. | *May lead to ''[[meconium peritonitis]]''. | ||
*Can be mimicked by [[CMV]] infection.<ref>{{cite journal |author=Déchelotte PJ, Mulliez NM, Bouvier RJ, Vanlieféringhen PC, Lémery DJ |title=Pseudo-meconium ileus due to cytomegalovirus infection: a report of three cases |journal=Pediatr Pathol |volume=12 |issue=1 |pages=73–82 |year=1992 |pmid=1313975 |doi= |url=}}</ref> | |||
===Gross=== | ===Gross=== | ||
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*Green. | *Green. | ||
Image | ====Image==== | ||
*[http://library.med.utah.edu/WebPath/jpeg3/PERI175.jpg Meconium ileus (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html]. Accessed on: 3 December 2011.</ref> | *[http://library.med.utah.edu/WebPath/jpeg3/PERI175.jpg Meconium ileus (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html]. Accessed on: 3 December 2011.</ref> | ||
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Images: | Images: | ||
*[http://en.wikipedia.org/wiki/File:Neonatal_necrotizing_enterocolitis,_gross_pathology_20G0021_lores.jpg NEC - gross (WP)]. | *[http://en.wikipedia.org/wiki/File:Neonatal_necrotizing_enterocolitis,_gross_pathology_20G0021_lores.jpg NEC - gross (WP)]. | ||
*[ | *[https://library.med.utah.edu/WebPath/PEDHTML/PED045.html NEC - micro]. | ||
==Autoimmune enteropathy== | ==Autoimmune enteropathy== | ||
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*Lobular cholestasis. | *Lobular cholestasis. | ||
Images | ====Images==== | ||
==Biliary atresia== | ==Biliary atresia== | ||