Difference between revisions of "Pediatric pathology"

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(more, +hirschsprung)
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The article deals with '''paediatric pathology''', which is quite different than adult pathology.  Many diseases that afflict children are uncommon or unheard of in adults.
The article deals with '''paediatric pathology''', which is quite different than adult pathology.  Many diseases that afflict children are uncommon or unheard of in adults.


==Gastrointestinal pathology==
=Gastrointestinal pathology=
===Aganglionosis===
==Aganglionosis==
*[[AKA]] Hirschsprung disease.
*[[AKA]] Hirschsprung disease.
====General====
===General===
*Congenital.
*Congenital.
*Fixed by surgery.
*Fixed by surgery.
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*Parasympathetic ganglion cells in intramural and submucosal plexuses - not present.<ref name=pathcon_hirschsprung>URL: [[http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970813-0] [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970813-0]]. Accessed on: 11 January 2011.</ref>
*Parasympathetic ganglion cells in intramural and submucosal plexuses - not present.<ref name=pathcon_hirschsprung>URL: [[http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970813-0] [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970813-0]]. Accessed on: 11 January 2011.</ref>


====Microscopic====
===Microscopic===
Features:<ref name=pathcon_hirschsprung/>
Features:<ref name=pathcon_hirschsprung/>
*Ganglion cells missing in submucosal plexus and myenteric plexus.
*Ganglion cells missing in submucosal plexus and myenteric plexus.
*+/-Submucosal fibrosis.
*+/-Submucosal fibrosis.


====Stains====
===Stains===
*Acetylcholinesterase: abundant, disorganized, nerve fibers.
*Acetylcholinesterase: abundant, disorganized, nerve fibers.
*CD117. (???)
*CD117. (???)
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*[http://pathology.mc.duke.edu/research/Histo_course/myent_plexus.jpg Normal myenteric plexus (duke.edu)].<ref>URL: [http://pathology.mc.duke.edu/research/PTH225.html http://pathology.mc.duke.edu/research/PTH225.html]. Accessed on: 11 January 2011.</ref>
*[http://pathology.mc.duke.edu/research/Histo_course/myent_plexus.jpg Normal myenteric plexus (duke.edu)].<ref>URL: [http://pathology.mc.duke.edu/research/PTH225.html http://pathology.mc.duke.edu/research/PTH225.html]. Accessed on: 11 January 2011.</ref>


===Meconium peritonitis===
==Meconium peritonitis==
====General====
===General===
*May be due to a number of causes:
*May be due to a number of causes:
**Aganglionosis (Hirschsprung disease).
**Aganglionosis (Hirschsprung disease).
**Meconium ileus.
**Meconium ileus.


====Microscopic====
===Microscopic===
Features:
Features:
*Brown granular material - '''key feature'''.
*Brown granular material - '''key feature'''.
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*[http://www.pathologyoutlines.com/caseofweek/case2008106image2.jpg Meconium peritonitis - gross (pathologyoutlines.com)].
*[http://www.pathologyoutlines.com/caseofweek/case2008106image2.jpg Meconium peritonitis - gross (pathologyoutlines.com)].


===Necrotizing enterocolitis===
==Necrotizing enterocolitis==
====General====
===General===
*Disease of the newborn.
*Disease of the newborn.
*Diagnosed by imaging.
*Diagnosed by imaging.


====Microscopic====
===Microscopic===
Features:
Features:
*Large spaces.
*Large spaces.
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*[http://cueflash.com/cardimages/answers/thumbnails/7/4/7747308.jpg NEC - micro. (cueflash.com)].<ref>URL: [http://cueflash.com/decks/Pathology_Pediatrics http://cueflash.com/decks/Pathology_Pediatrics]. Accessed on: 11 January 2011.</ref>
*[http://cueflash.com/cardimages/answers/thumbnails/7/4/7747308.jpg NEC - micro. (cueflash.com)].<ref>URL: [http://cueflash.com/decks/Pathology_Pediatrics http://cueflash.com/decks/Pathology_Pediatrics]. Accessed on: 11 January 2011.</ref>


===Pancreatic islet cell hyperplasia===
==Pancreatic islet cell hyperplasia==
====General====
===General===
*Assoc. with maternal diabetes.
*Assoc. with maternal diabetes.


====Microscopic====
===Microscopic===
Features:
Features:
*Marked size variation of pancreatic islets.
*Marked size variation of pancreatic islets.
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*[http://eulep.pdn.cam.ac.uk/pathbase2/Search_Pathbase/factsheet.php?image_number=3297 Islet cell hyperplasia - mouse (cam.ac.uk)].
*[http://eulep.pdn.cam.ac.uk/pathbase2/Search_Pathbase/factsheet.php?image_number=3297 Islet cell hyperplasia - mouse (cam.ac.uk)].


=Cardiovascular pathology=
==Persistent pulmonary hypertension of the newborn==
==Persistent pulmonary hypertension of the newborn==
*Abbreviated PPHN.
*Abbreviated PPHN.
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*Birth asphyxia.  
*Birth asphyxia.  


==Williams syndrome==
*Supravalvular stenosis.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/194050 http://www.ncbi.nlm.nih.gov/omim/194050]. Accessed on: 11 January 2011.</ref>
=Neuropathology=
==Hypoxic-ischemic encephalopathy==
==Hypoxic-ischemic encephalopathy==
{{Main|Neuropathology}}
{{Main|Neuropathology}}
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*The germinal matrix is thought to be intrinsically fragile and is especially so in premature infants.
*The germinal matrix is thought to be intrinsically fragile and is especially so in premature infants.


==References==
=References=
{{Reflist|2}}
{{Reflist|2}}


==External links==
=External links=
*[http://www.surgical-pathology.com/paediatric_pathology.htm Paediatric pathology (surgical-pathology.com)].
*[http://www.surgical-pathology.com/paediatric_pathology.htm Paediatric pathology (surgical-pathology.com)].
*[http://www.spponline.org/ Society for pediatric pathology (spponline.org)].
*[http://www.spponline.org/ Society for pediatric pathology (spponline.org)].


===Cases===
==Cases==
*[http://www.urmc.rochester.edu/pathology_lab_medicine/research_academics/pediatric_pathology/cases.cfm Pediatric pathology cases (rochester.edu)].
*[http://www.urmc.rochester.edu/pathology_lab_medicine/research_academics/pediatric_pathology/cases.cfm Pediatric pathology cases (rochester.edu)].
*Cases from UMPC:
*Cases from UMPC:

Revision as of 22:02, 11 January 2011

The article deals with paediatric pathology, which is quite different than adult pathology. Many diseases that afflict children are uncommon or unheard of in adults.

Gastrointestinal pathology

Aganglionosis

  • AKA Hirschsprung disease.

General

  • Congenital.
  • Fixed by surgery.

Pathology:

  • Parasympathetic ganglion cells in intramural and submucosal plexuses - not present.[1]

Microscopic

Features:[1]

  • Ganglion cells missing in submucosal plexus and myenteric plexus.
  • +/-Submucosal fibrosis.

Stains

  • Acetylcholinesterase: abundant, disorganized, nerve fibers.
  • CD117. (???)

Images:

Meconium peritonitis

General

  • May be due to a number of causes:
    • Aganglionosis (Hirschsprung disease).
    • Meconium ileus.

Microscopic

Features:

  • Brown granular material - key feature.
  • +/-Multinucleated giant cells.
  • Inflammatory infiltrate (PMNs, lymphocytes, plasma cells).

Image:

Necrotizing enterocolitis

General

  • Disease of the newborn.
  • Diagnosed by imaging.

Microscopic

Features:

  • Large spaces.

Images:

Pancreatic islet cell hyperplasia

General

  • Assoc. with maternal diabetes.

Microscopic

Features:

  • Marked size variation of pancreatic islets.
    • Normal islets ~ 150 micrometers (diameter). Hyperplastic islets - up to ~500 micrometers (diameter).

Image:

Cardiovascular pathology

Persistent pulmonary hypertension of the newborn

  • Abbreviated PPHN.
  • Related to patent ductus arteriosus and persistent fetal circulation.[4]

Associations:[5]

  • Meconium aspiration.
  • Anemia.
  • Infection.
    • Pneumonia (severe).
  • Hypoglycemia.
  • Birth asphyxia.

Williams syndrome

  • Supravalvular stenosis.[6]

Neuropathology

Hypoxic-ischemic encephalopathy

Main article: Neuropathology
  • Abbreviated HIE.

General

  • Autopsy adds some information.
  • Two-tone liver - suggests prior injury.[7]
  • HIE in perinatal period may be unique to the specific time of the injury, i.e. the type of hypoxic insults vary by developmental stage.[8]
    • Some hypoxic injuries that are prenatal do not occur after birth.
      • Pontosubicular necrosis is prenatal; the subiculum postnatal (like in adults) is resistant to hypoxic-ischemic insults.
    • Hypoxic-ischemic insults are predominantly in the white matter. (???)
  • HIE is the most common cause of neonatal seizures and often difficult to control with anticonvulsants.[9]

Possible findings in HIE

Hemorrhagic lesions:[10]

  • Germinal matrix & intraventricular hemorrhage.
  • Choroid plexus hemorrhage.
  • Cerebellar hemorrhage.
  • Subpial hemorrhage.

White matter lesions:[10]

  • Periventricular leukomalacia.
  • Subcortical leukomalacia.
  • Telencephalic (cerebral) leukomalacia.

Grey matter lesions:[10]

  • Pontosubicular necrosis.
  • Infarcts of the cerebral cortex, basal ganglia, thalamus, brain stem.

Germinal matrix hemorrhage

  • Arises from the germinal matrix, the tissue from which neurons and glial arise from.[11]
  • The germinal matrix is thought to be intrinsically fragile and is especially so in premature infants.

References

  1. ↑ 1.0 1.1 URL: [[1] [2]]. Accessed on: 11 January 2011.
  2. ↑ URL: http://pathology.mc.duke.edu/research/PTH225.html. Accessed on: 11 January 2011.
  3. ↑ URL: http://cueflash.com/decks/Pathology_Pediatrics. Accessed on: 11 January 2011.
  4. ↑ URL: http://www.thechildrenshospital.org/wellness/info/parents/20830.aspx. Accessed on: 4 January 2011.
  5. ↑ URL: http://www.thechildrenshospital.org/wellness/info/parents/20830.aspx. Accessed on: 4 January 2011.
  6. ↑ URL: http://www.ncbi.nlm.nih.gov/omim/194050. Accessed on: 11 January 2011.
  7. ↑ Elder DE, Zuccollo JM, Stanley TV (July 2005). "Neonatal death after hypoxic ischaemic encephalopathy: does a postmortem add to the final diagnoses?". BJOG 112 (7): 935–40. doi:10.1111/j.1471-0528.2005.00608.x. PMID 15957995.
  8. ↑ Grafe MR, Kinney HC (February 2002). "Neuropathology associated with stillbirth". Semin. Perinatol. 26 (1): 83–8. PMID 11876572.
  9. ↑ URL: http://emedicine.medscape.com/article/973501-overview. Accessed on: 7 January 2011.
  10. ↑ 10.0 10.1 10.2 Riezzo I, Neri M, De Stefano F, et al. (2010). "The timing of perinatal hypoxia/ischemia events in term neonates: a retrospective autopsy study. HSPs, ORP-150 and COX2 are reliable markers to classify acute, perinatal events". Diagn Pathol 5: 49. doi:10.1186/1746-1596-5-49. PMC 2914029. PMID 20626887. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2914029/.
  11. ↑ 11.0 11.1 Ballabh P (January 2010). "Intraventricular hemorrhage in premature infants: mechanism of disease". Pediatr. Res. 67 (1): 1–8. doi:10.1203/PDR.0b013e3181c1b176. PMC 2799187. PMID 19816235. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2799187/.

External links

Cases

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