Lecture 45 - Pediatric Pathology Flashcards
CF is an autosomal _______, most common lethal genetic disease in ______ (race).
It can result at birth with ______ ileus (dilated bowel full of meconium).
Recessive
Caucasians
Meconium ileus
Remember that Hirschprung’s disease is an Aganglionosis (neural crest cells failed to migrate) in BOTH ______ (Auerbach’s) and _______ (Meisner’s) plexuses.
Look for marked increase of ______ activity in nerve branches and ______ nerve trunks.
Myenteric
Submucosal
Acetylcholinesterase
Hypertrophic nerve trunks
Though it’s not very common (about 1/10,000 births), ______ _____ atresia is often considered when a newborn presents with jaundice bc it is the most common cause of death from Liver disease in early childhood.
Extrahepatic Biliary Atresia
The best thing that has reduced incidence of SIDS is putting the baby to sleep on their _____ and avoiding ______.
SIDS is thought to be an issue with ____ ____ abnormalities.
Premature birth, low birth weight, ____ (sex), twins, and hx of SIDS are risk factors.
Back
Co-sleeping (not sleeping with parents)
Male
_______ (aka _______) is the term used to describe a tumor-like condition in which there is normal looking tissue but in the wrong place.
_______ is the term used to describe a tumor-like condition in which there is disorganized growth of native tissue (the right cells, but the wrong arrangement/architecture).
Heterotopia (aka Choristoma)
Hamartoma
_______ are the most common neoplasms of infancy.
_______ are neoplasms that may encroach on the mediastinum or axillary nerves.
_______ teratomas occur due to midline migration of germ cells and occur more frequently in _____ (sex).
While the first two are always ______ (malignant or benign), the third one is only mostly (75%).
Hemangiomas
Lymphangiomas
Sacrococcygeal teratomas
Benign
Childhood malignancies can arise from Constitutional chromosomal abnormalities or Acquired chromosomal abnormalities. What’s the difference?
Constitutional –> both the child AND the tumor have the abnormality (so basically in all the cells - e.g. Trisomy 21).
Acquired –> just the tumor has the abnormality.
Are childhood cancers likely to arise from environmental factors?
No, they have obviously much shorter duration of environmental exposure than adults.
Childhood malignancies, unlike adult, are more likely to spontaneously ______ or differentiate.
Regress
People who had cancer in childhood are much more likely to develop a secondary malignancy later in life (either from recurrence or damage from treatment).
______ is the most common secondary tumor, followed by Leukemia and CNS tumors.
Sarcomas
Burkitt’s lymphoma is associated with a mutation in ___-___.
Neuroblastoma is associated with a mutation in ___-____.
How does number of copies of these genes correspond to prognosis?
Are these Oncogenes or Tumor Suppressor Genes?
Burkitt –> C-myc
Neuroblastoma –> N-myc
More copies –> worse prognosis
Oncogenes
Neuroblastomas are one of the most common extracranial solid tumors of childhood. They typically arise in children under the age of _____, and they most often result from ____ ____ cells originating in the _____ or Paravertebral autonomic ganglia.
5
Neural crest
Adrenal
Neuroblastomas (“small round blue cells” and characteristic _____ on histology) tend to differentiate, but they can persist and metastasize. They spread via ______ or ______ route, so they often spread to the _____ and _____.
Rosettes
Hematogenous
Lymphatic
Brain
Liver
How does the age of the child correspond to Neuroblastoma prognosis?
What does 4S staging of Neuroblastoma mean?
Younger –> better
4S means mets to Skin and Liver, but NOT to bone –> better prognosis than other stage 4.
Neuroblastomas produce ______. They can be measured directly in serum or their metabolites _____ and _____ can be measured in Urine.
Catecholamines
VMA
HVA
