Difference between revisions of "Pediatric pathology"

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=Gastrointestinal pathology=
=Gastrointestinal pathology=
==Cystic fibrosis==
{{Main|Pediatric gastrointestinal pathology}}
*Abbreviated ''CF''.


===General===
GI is a big part pediatric pathology and therefore gets its own article.
*Genetic.
*May lead to meconium ileus.


===Microscopic (large bowel)===
Among others, things discussed include:
Features:<ref name=pmid710839>{{cite journal |author=Neutra MR, Trier JS |title=Rectal mucosa in cystic fibrosis. Morphological features before and after short term organ culture |journal=Gastroenterology |volume=75 |issue=4 |pages=701–10 |year=1978 |month=October |pmid=710839 |doi= |url=}}</ref>
*Cystic fibrosis.
*Crypt enlargement.
*Aganglionosis (Hirschsprung disease).
 
*Meconium peritonitis.
Notes:
*''Not'' intracellular and extracellular accumulation of mucus. (?)
 
==Aganglionosis==
*[[AKA]] Hirschsprung disease.
===General===
*Congenital.
*Fixed by surgery.
 
Pathology:
*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>
 
Notes:
*Most common reason for litigation in paediatric pathology.<ref>GT. 19 January 2011.</ref>
 
===Microscopic===
Features:<ref name=pathcon_hirschsprung/>
*Ganglion cells missing in submucosal plexus and myenteric plexus.
*+/-Submucosal fibrosis.
 
===Stains===
*Acetylcholinesterase: abundant, disorganized, nerve fibers.
*CD117. (???)
 
Images:
*[http://66.244.141.33/colorectal-Hirschsprung-disease Hirschsprung disease - collection (66.244.141.33)].
*[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==
===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:
*[http://www.pathologyoutlines.com/caseofweek/case2008106image2.jpg Meconium peritonitis - gross (pathologyoutlines.com)].
 
==Necrotizing enterocolitis==
===General===
*Disease of the newborn.
*Diagnosed by imaging.
 
===Microscopic===
Features:
*Large spaces.
 
Images:
*[http://en.wikipedia.org/wiki/File:Neonatal_necrotizing_enterocolitis,_gross_pathology_20G0021_lores.jpg NEC - gross (WP)].
*[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==
===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:
*[http://eulep.pdn.cam.ac.uk/pathbase2/Search_Pathbase/factsheet.php?image_number=3297 Islet cell hyperplasia - mouse (cam.ac.uk)].


=Cardiovascular pathology=
=Cardiovascular pathology=

Revision as of 15:29, 1 February 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.

Syndromes

Noonan syndrome

  • Many different problems.[1]

Cardiac

Angelmann syndrome

  • AKA happy puppet syndrome.

General

  • Loss of a gene on 15q.
    • May be due to genetic imprinting disorder, i.e. only maternal gene imprinting pattern is present (due to loss of the paternal chromosome).[2]
  • Mental retardation.

Notes:

  • Loss of the maternal imprinting pattern on 15q leads to Prader-Willi syndrome.[3]

Gastrointestinal pathology

Main article: Pediatric gastrointestinal pathology

GI is a big part pediatric pathology and therefore gets its own article.

Among others, things discussed include:

  • Cystic fibrosis.
  • Aganglionosis (Hirschsprung disease).
  • Meconium peritonitis.

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. ↑ URL: http://www.ncbi.nlm.nih.gov/omim/163950. Accessed on: 13 January 2011.
  2. ↑ URL: http://www.ncbi.nlm.nih.gov/omim/105830. Accessed on: 28 January 2011.
  3. ↑ URL: http://www.ncbi.nlm.nih.gov/omim/176270. Accessed on: 28 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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