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Pediatric pathology (view source)

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[[Image:Wilms tumor.jpg|thumb|right|250px|[[Gross pathology|Gross]] image of a [[Wilms tumour]], a tumour common in pediatric pathology. (WC/AFIP)]]
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=
=Syndromes=
==DiGeorge syndrome==
{{Main|DiGeorge syndrome}}
==Noonan syndrome==
==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>
*Many different problems.<ref name=omim163950>{{OMIM|163950}}</ref>
*Mutation in ''PTPN11 gene''.
**This gene is also implicated in multiple [[granular cell tumour]]s.


===Cardiac===
===Cardiac===
*May be associated [[cardiomyopathy]]: DCM, RCM.
*May be associated with [[cardiomyopathy]]: [[DCM]], [[RCM]].
 
==Angelmann syndrome==
*[[AKA]] happy puppet syndrome.


=Gastrointestinal pathology=
==Aganglionosis==
*[[AKA]] Hirschsprung disease.
===General===
===General===
*Congenital.
*Loss of a gene on 15q.
*Fixed by surgery.
**May be due to genetic imprinting disorder, i.e. only maternal gene imprinting pattern is present (due to loss of the paternal chromosome).<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/105830 http://www.ncbi.nlm.nih.gov/omim/105830]. Accessed on: 28 January 2011.</ref>
*Mental retardation.


Pathology:
Notes:
*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>
*Loss of the maternal imprinting pattern on 15q leads to Prader-Willi syndrome.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/176270 http://www.ncbi.nlm.nih.gov/omim/176270]. Accessed on: 28 January 2011.</ref>


===Microscopic===
=Gastrointestinal pathology=
Features:<ref name=pathcon_hirschsprung/>
{{Main|Pediatric gastrointestinal pathology}}
*Ganglion cells missing in submucosal plexus and myenteric plexus.
*+/-Submucosal fibrosis.


===Stains===
GI is a big part pediatric pathology and therefore gets its own article.
*Acetylcholinesterase: abundant, disorganized, nerve fibers.
*CD117. (???)


Images:
Among others, things discussed include:
*[http://66.244.141.33/colorectal-Hirschsprung-disease Hirschsprung disease - collection (66.244.141.33)].
*[[Cystic fibrosis]].
*[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>
*[[Aganglionosis]] (Hirschsprung disease).
*[[Meconium peritonitis]].
*[[Necrotizing enterocolitis]].


==Meconium peritonitis==
=Pulmonary pathology=
===General===
==Respiratory distress syndrome==
*May be due to a number of causes:
*Abbreviated ''RDS''.
**Aganglionosis (Hirschsprung disease).
**Should not be confused with ''[[acute respiratory distress syndrome]]'' (ARDS).
**Meconium ileus.
*Previously known as ''hyaline membrane disease''.


===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===
===General===
*Disease of the newborn.
Associations:
*Diagnosed by imaging.
*Prematurity.
*Maternal diabetes.<ref name=pmid22094826>{{Cite journal  | last1 = Hay | first1 = WW. | title = Care of the Infant of the Diabetic Mother. | journal = Curr Diab Rep | volume =  | issue =  | pages =  | month = Nov | year = 2011 | doi = 10.1007/s11892-011-0243-6 | PMID = 22094826 }}</ref>


===Microscopic===
Etiology:  
Features:
*Not enough lung surfactant -> alveolar collapse with exhalation -> increased work of breathing.
*Large spaces.


Images:
Complications of oxygen therapy:<ref name=Ref_PCPBoD8_244>{{Ref PCPBoD8|244}}</ref>
*[http://en.wikipedia.org/wiki/File:Neonatal_necrotizing_enterocolitis,_gross_pathology_20G0021_lores.jpg NEC - gross (WP)].
*Retinopathy of prematurity.
*[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>
*[[Bronchopulmonary dysplasia]].
 
==Pancreatic islet cell hyperplasia==
===General===
*Assoc. with maternal diabetes.


===Microscopic===
===Microscopic===
Features:
Features:<ref name=Ref_PCPBoD8_243>{{Ref PCPBoD8|243}}</ref>
*Marked size variation of pancreatic islets.
*Proteineous (cellular) debris (hyaline membranes) line alveoli and respiratory bronchioles.
**Normal islets ~ 150 micrometers (diameter).  Hyperplastic islets - up to ~500 micrometers (diameter).


Image:
Note:
*[http://eulep.pdn.cam.ac.uk/pathbase2/Search_Pathbase/factsheet.php?image_number=3297 Islet cell hyperplasia - mouse (cam.ac.uk)].
*Similar to ''[[diffuse alveolar damage]]''.


=Cardiovascular pathology=
=Cardiovascular pathology=
==Congenital heart disease==
{{Main|Congenital heart disease}}
This is a huge topic.
==Persistent pulmonary hypertension of the newborn==
==Persistent pulmonary hypertension of the newborn==
*Abbreviated PPHN.
*Abbreviated PPHN.
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White matter lesions:<ref name=pmid20626887/>
White matter lesions:<ref name=pmid20626887/>
*Periventricular leukomalacia.
*[[Periventricular leukomalacia]].
*Subcortical leukomalacia.
*Subcortical leukomalacia.
*Telencephalic (cerebral) leukomalacia.
*Telencephalic (cerebral) leukomalacia.
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***Image: [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2799187/figure/F1/ Germinal matrix (nlm.nih.gov)].<ref name=pmid19816235/>
***Image: [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2799187/figure/F1/ Germinal matrix (nlm.nih.gov)].<ref name=pmid19816235/>
*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.
Grading:
*Grade 1 = confined to germinal matrix.
*Grade 2 = ventricular hemorrhage.
*Grade 3 = distortion of ventricle.
*Grade 4 = disruption of white matter.
===Periventricular leukomalacia===
Features:<ref name=pmid12416551>{{Cite journal  | last1 = Rezaie | first1 = P. | last2 = Dean | first2 = A. | title = Periventricular leukomalacia, inflammation and white matter lesions within the developing nervous system. | journal = Neuropathology | volume = 22 | issue = 3 | pages = 106-32 | month = Sep | year = 2002 | doi =  | PMID = 12416551 }}</ref>
*Multifocal [[necrosis]] of the cortical white matter adjacent to the lateral ventricles.
*Usually symmetric.
=Pediatric tumours=
Many pediatric tumours have a "primative" histologic appearance and can be grouped into the category ''[[small round cell tumour]]'', which is covered in the article having that name and gives an overview of that grouping.
==Wilms tumour==
:[[AKA]] nephroblastoma.
{{Main|Wilms tumour}}
Most common abdominal solid organ malignancy in children.  A good starting point if you're considering this entity is the ''[[small round cell tumours]]'' article.
==Rhadomyosarcoma==
:Commonly abbreviated ''RMS''.
{{Main|Rhabdomyosarcoma}}
This covers RMS.  A good starting point if you're considering this entity is the ''[[small round cell tumours]]'' article.
==Hepatoblastoma==
{{Main|Hepatoblastoma}}
A good starting point if you're considering this entity is the ''[[small round cell tumours]]'' article.
==Lymphoma==
{{Main|Lymphoma}}
In reference to malignancies, these are very common in children.
==Neuroblastoma==
{{Main|Neuroblastoma}}
A good starting point if you're considering this entity is the ''[[small round cell tumours]]'' article.
=Dermatopathology=
{{Main|Dermatopathology}}
==Juvenile xanthogranuloma==
{{Main|Juvenile xanthogranuloma}}
=Soft tissue lesions=
{{Main|Soft tissue lesions}}
The histomorphology can look very malignant when viewed through the context of adult [[soft tissue pathology]].<ref>{{Ref PCPBoD8|252}}</ref>


=References=
=References=
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*[http://www.sppg.ch/ Swiss pediatric pathology group (sppg.ch)].
*[http://www.sppg.ch/ Swiss pediatric pathology group (sppg.ch)].


[[Category:Weird stuff]]
[[Category:Pediatric pathology]]
Michael
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