peds - indiana university...
TRANSCRIPT
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Pathology and Pathogenesisof HIV Infection
PneumocystisJeroveci
H&E GMS
HistoplasmosisCryptococcusneoformans
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Cryptosporidium
Toxoplasmosis
Cytomegalovirus
Jacobson, M. A. N Engl J Med 1997;337:105-114
Clavel, F. et. al. N Engl J Med 2004;350:1023-1035
Complex of HIV-1 Reverse Transcriptase with an RNA-DNA Duplex
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Pediatric Pathology
Causes of Death Related with Age
Under 1 year congenital malformations/chromosome abn.prematurity/low birth weightSIDS
Other: maternal pregnancy complications, placenta/cord/membrane complcations, RDS, accidents, sepsis, intrautering hypoxia, asphyxia, circulatory system dis.
1-4 years accidentscongenital malformations/chromosome abn.malignant neoplasmshomicide
5-14 years accidentsmalignant neoplasmshomicidesuicide
15-24 years accidentshomicidesuicidemalignant neoplasmsheart disease
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W E IG HT
gm
GESTATIONAL AGE (Wks)
ASSESSING INTRAUTERINE GROWTH AND DEVELOPMENT
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Intrauterine Growth
Growth Periods
1-9 weeks embryonic9-13 weeks fetal
Growth: hyperplasiahypertrophy
Factors: fetalplacentalmaternal
Small for Gestational Age Fetuses
Low birth weight infants are over-represented among cases of infant death:
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20-22 Weeks
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Near Term
Malformations
Deformations
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Disruptions
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Sequences
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Causes of Congenital Malformations
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RBP retinol binding proteinCRBP cytoplasmic retinol binding proteinCRABP cytoplasmic/cytosol retinoic acid binding proteinRARE retinoic acid response element
Birth Injuries IntrauterineInfections:Ascending
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Respiratory Distress Syndrome
Treatment: surfactantsteroidsventilation (PEEP, O2)
Prevention: Amniotic fluid testing“fetal lung maturity testing”
Outcome: UncomplicatedComplications of RDS
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RDS: Prevention: Fetal Lung MaturityTesting
Fetal Hydrops
Immune
Non-Immune
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Phenylketonuria
Autosomal recessive1:10,000 – 1:25,000 live birthscarrier rate in US: 2-3%
Spectrum of Disease
Classic PKU
Benign Hyperphenylalaninemia
Maternal PKU
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Galactosemia
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Cystic Fibrosis
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CFTR Mutations and PhenotypeClassification:
I. Defective Protein SynthesisII. Abnormal Protein Folding,
Processing, TraffickingIII. Defective RegulationIV. Decreased ConductanceV. Reduced AbundanceVI. Altered Regulation of Separate
Ion Channels
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Sudden Infant DeathSyndrome
Factors Associated with SIDS
Maternal Infant
Young (<20 yrs) PrematurityUnmarried Low Birth WtShort pregnancy interval Male sexLow SE group Multiple birthSmoking Not 1st siblingBlack or Native Amer. SIDS in prior
sibling
SIDS: Pulmonary congestion and Hemorrhage
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Pediatric Neoplasia
Adults vs Children
skin hematologiclung nervousbreast soft tissueprostate bonecolon kidney
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Hemangioma
Port WineStain
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Lymphangioma
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SacrococcygealTeratoma
0-4 5-9 10-14
leukemia leukemia hepatocellular Ca neuroblastoma neuroblastoma soft tissue sarcomas retinoblastoma retinoblastoma osteosarcomaWilm’s tumor hepatocellular Ca thyroid Ca hepatoblastoma rhabdomyosarcoma Hodgkin’s Diseasebrain ewing sarcoma/PNET
lymphoma
Pediatric Malignancy by Age
Neuroblastoma
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