peds - Indiana University Bloomingtonmedsci.indiana.edu/c602web/602/c602web/docs/peds/peds_slides.pdf · Under 1 year congenital malformations/chromosome abn. ... Malformations Deformations

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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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Amyloidosis

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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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Intrauterine Infections: Hematogenous

Surfactant

Lamellar bodiesIn pneumocytes

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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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Retinoblastoma

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Wilm’s Tumor

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