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1

Causes of Death by Age

<1 

 Congenital malformations, deformations, and chromosomal anomalies

 Disorders related to short gestation and low birth weight

 SIDS

 Maternal complications of pregnancy

2

 1-4 years Causes of death

 Accidents (unintentional Injuries)

 Congenital anomalies

 Assault (homicide)

 Malignant neoplasms

 Heart diseases

3

Causes of death

 5-9 years

 Accidents (unintentional Injuries)

 Malignant neoplasms

 Congenital anomalies

 Assault (Homicide)

 Diseases of the heart

4

Causes of death 10-14 years

 Accidents (unintentional injuries)

 Malignant neoplasms

 Assault (homicide)

 Intentional self-harm (suicide)

 Congenital anomalies.

5

Predisposing factors to birth injuries

– Cephalopelvic disproportion

– Difficult labor (Breech presentation)

– Prematurity

6

Birth Injuries

• Cranial injuries

– Caput succedaneum

– Cephalohematoma

– Skull fractures

– Intracranial hemorrhage

7

Birth • Peripheral nerve injuries

– Brachial palsy

– Facial nerve palsy

8

most common birth injury

Clavicle Fx 

9

22q11.2 Deletion Syndrome

Velocardiofacial Syndrome

 Di George Syndrome 

10

Velocardiofacial Syndrome

– Congenital heart disease (outflow tracts)

– Palatal abnormalities/Facial dysmorphism

– Developmental delay

Deletion of 22q11.2

11

 Di George Syndrome

– Thymic hypoplasia – impaired T-cell immunity

– Parathyroid hypoplasia - hypocalcemia

12

KLINEFELTER‟S SYNDROME

(XXY,XXXY etc.)

– Testicular atrophy • Sterility

– Eunuchoid with:

• Gynecomastia

• Reduced facial, body and pubic hair.

Prone to breast cancer,extragonadal germ cell t., and

autoimmune diseases.

13

ENVIRONMENTAL

– Maternal/Placental Infections 2-3% of birth defects what infections?

• Rubella

• Toxoplasmosis

• Syphilis

• CMV

• HIV

14

– The most common congenital malformations

• Cleft lip/palate

• Neural tube defects

15

Pathogenesis of congenital malformations

1. Timing of the prenatal insult has an important impact on both the occurrence and the type of malformation produced.

2. Genes that regulate morphogenesis may be the target of teratogens.

– Homeobox (HOX) genes

– Sonic hedgehog gene

16

Sonic Hedgehog Gene defect causes

holoprosencephaly

17

Cyclopamine causes

holoprosencephaly

18

Homeobox Gene defect causes

limb, vertebral and craniofacial abnormalities.

19

Valproic acid mimics what 

Homeobox Gene defect

causes valproic acid

20

Retinoic acid (acne) causes

CNS, cardiac and craniofacial abnormalities including cleft lip and palate. May interfere with TGFβ (palatogenesis)

21

Malformations

 Primary structural abnormality with poor formation of tissue due to a localized error that occurs during development.

22

Malformations - examples

• Polydactyly& Syndactyly

• Cleftlip

• Cleftpalate

• Congenital heart disease

23

Disruption

• Structural defect caused by secondary destruction of or interference with a previously normally formed part.

24

Disruption example

Amniotic bands

entanglement followed by the tearing apart or amputation of a normally developed structure.

– Interruption of blood supply leading to infarction, necrosis, and/or resorption of structures distally.

25

Deformation

Localized or generalized compression of the growing fetus by abnormal biomechanical forces.

• Arises later in fetal life than do malformations

26

most common cause of deformation

Uterine constraint

27

Factors for deformations

Factors –

Maternal

» First pregnancy

» Small uterus

» Leiomyomas

Fetal

Multiple fetuses

Oligohydramnios

Abnormal presentation

28

Potter Sequence

29

Oligohydramnios (= lack of amniotic fluid) due to:

• Renal agenesis/ maldevelopment

• Amniotic fluid leak

• Uteroplacental insufficiency

30

Oligohydramnios causes

• Pulmonary hypoplasia

• Amnion nodosum

• Fetal compression

– Worsens pulmonary hypoplasia

– Breech presentation

– Altered facies

– Positioning defects of feet and hands