Difference between revisions of "Pediatric pathology"

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{{Main|Wilms tumour}}
{{Main|Wilms tumour}}
Most common abdominal solid organ malignancy in children.
Most common abdominal solid organ malignancy in children.
=Dermatopathology=
{{Main|Dermatopathology}}
==Juvenile xanthogranuloma==
*Abbreviated as ''JXG''.
*[[AKA]] nevoxanthoendothelioma.
===General===
*Benign skin thingy in children and infants.
===Microscopic===
Features:
*Dermal histiocytes:
**Abundant cytoplasm - may not be xanthomatous.
*+/-Touton giant cell - '''key feature'''.
Images:
*[http://www.healthcare.uiowa.edu/dermatology/DPT/Hist%5CJXG003.jpg JXG (uiowa.edu)].<ref>URL: [http://www.healthcare.uiowa.edu/dermatology/DPT/Path-Index.htm http://www.healthcare.uiowa.edu/dermatology/DPT/Path-Index.htm]. Accessed on: 3 February 2011.</ref>
Notes:
*Must prove they are non-Langerhans cell histiocytes.
===IHC===
*Langerhans cell markers: CD1a, CD207 -- both should be negative -- '''key feature'''.
*Histiocyte markers: CD68, CD163 -- both should be positive.


=References=
=References=

Revision as of 17:02, 3 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

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.
  • Necrotizing enterocolitis.

Cardiovascular pathology

Congenital heart disease

This is a huge topic.

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

  • 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.

Pediatric tumours

Wilms tumour

Most common abdominal solid organ malignancy in children.

Dermatopathology

Juvenile xanthogranuloma

  • Abbreviated as JXG.
  • AKA nevoxanthoendothelioma.

General

  • Benign skin thingy in children and infants.

Microscopic

Features:

  • Dermal histiocytes:
    • Abundant cytoplasm - may not be xanthomatous.
  • +/-Touton giant cell - key feature.

Images:

Notes:

  • Must prove they are non-Langerhans cell histiocytes.

IHC

  • Langerhans cell markers: CD1a, CD207 -- both should be negative -- key feature.
  • Histiocyte markers: CD68, CD163 -- both should be positive.

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/.
  12. URL: http://www.healthcare.uiowa.edu/dermatology/DPT/Path-Index.htm. Accessed on: 3 February 2011.

External links

Cases