Difference between revisions of "Pediatric pathology"

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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.
=Syndromes=
==Noonan syndrome==
*Many different problems.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/163950 http://www.ncbi.nlm.nih.gov/omim/163950]. Accessed on: 13 January 2011.</ref>
===Cardiac===
*May be associated [[cardiomyopathy]]: DCM, RCM.


=Gastrointestinal pathology=
=Gastrointestinal pathology=

Revision as of 13:23, 13 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.

Syndromes

Noonan syndrome

  • Many different problems.[1]

Cardiac

Gastrointestinal pathology

Aganglionosis

  • AKA Hirschsprung disease.

General

  • Congenital.
  • Fixed by surgery.

Pathology:

  • Parasympathetic ganglion cells in intramural and submucosal plexuses - not present.[2]

Microscopic

Features:[2]

  • 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.[5]

Associations:[6]

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

Williams syndrome

  • Supravalvular stenosis.[7]

Neuropathology

Hypoxic-ischemic encephalopathy

  • Abbreviated HIE.

General

  • Autopsy adds some information.
  • Two-tone liver - suggests prior injury.[8]
  • 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.[9]
    • 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.[10]

Possible findings in HIE

Hemorrhagic lesions:[11]

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

White matter lesions:[11]

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

Grey matter lesions:[11]

  • 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.[12]
  • 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. 2.0 2.1 URL: [[1] [2]]. Accessed on: 11 January 2011.
  3. URL: http://pathology.mc.duke.edu/research/PTH225.html. Accessed on: 11 January 2011.
  4. URL: http://cueflash.com/decks/Pathology_Pediatrics. Accessed on: 11 January 2011.
  5. URL: http://www.thechildrenshospital.org/wellness/info/parents/20830.aspx. Accessed on: 4 January 2011.
  6. URL: http://www.thechildrenshospital.org/wellness/info/parents/20830.aspx. Accessed on: 4 January 2011.
  7. URL: http://www.ncbi.nlm.nih.gov/omim/194050. Accessed on: 11 January 2011.
  8. 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.
  9. Grafe MR, Kinney HC (February 2002). "Neuropathology associated with stillbirth". Semin. Perinatol. 26 (1): 83–8. PMID 11876572.
  10. URL: http://emedicine.medscape.com/article/973501-overview. Accessed on: 7 January 2011.
  11. 11.0 11.1 11.2 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/.
  12. 12.0 12.1 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