Path-Pediatric Diseases

Front 1

What is the leading cause of death in 1-15 yr olds?

Back 1

Injury

Front 2

What is the leading cause of infant death?

Back 2

Congenital anomalies account for 20% and short gestation/low birth weight account for 17%

Front 3

HOw can risk of cleft palate/lip be lowered?

Back 3

By administration of prenatal folic acid

Front 4

What causes disruptions in fetal development?

Back 4

Amniotic bands that constrict certain body parts, often hands and feet

Front 5

What is the Potter sequence?

Back 5

It is renal agenesis followed by oligohydramnios (lack of amniotic fluid). This leads to fetal compression and deformation.

Front 6

What is an ascending (transcervical) infection?

Back 6

One that reaches the baby via the cervix from the vagina (ie Strep agalactiae, HSV)

Front 7

What is the most common cause of neonatal meningitis?

Back 7

Strep agalactiae acquired by ascending infection from mothers vagina

Front 8

What is a transplacental infection?

Back 8

Infection reaching fetus via the placenta

Front 9

What does TORCH stand for and what does it mean?

Back 9

It is an acronym for toxoplasma, rubella, CMV, and HSV. These are the transplacental infections.

Front 10

What effects does toxoplasma infection have on a fetus?

Back 10

Serious brain damage due to hydrocephalus. Also chorioretinitis that leads to blindness

Front 11

What are the effects of T. pallidum infection on a fetus?

Back 11

Hutchinson's teeth, Mulberry molars, perforated palate, and saddle nose.

Front 12

What is the most dangerous effect of neonatal herpes?

Back 12

Neonatal herpes encephalitis

Front 13

What is the most common factor associated with fetal growth retardation?

Back 13

Maternal factors like preeclampsia, narcotic and alcohol abuse, and cigarette smoking (most common)

Front 14

What is the biggest risk factor for neonatal respiratory distress syndrome?

Back 14

Prematurity, for this reason it is important to keep the baby in there as long as possible

Front 15

What is the defect in RDS?

Back 15

Insufficient lung surfactant. This can be remedied by administration of steroids

Front 16

T/F Steroids administered early on in pregnancy will not harm a fetus

Back 16

False, they can only be administered toward the end of pregnancy term

Front 17

If a premature baby exhibited bloody stools, abdominal distension, and circulatory collapse, what is the likely dx?

Back 17

Necrotizing enterocolitis due to intestinal ischemia

Front 18

How would you treat necrotizing enterocolitis in infants?

Back 18

All you can do is remove the necrosed intestinal section, but usually pt will die

Front 19

What is the age associated with greatest risk of SIDS?

Back 19

1-12 months of age

Front 20

How can you reduce risk of SIDS?

Back 20

Breast feed, immunize, and make infant sleep on their back with nothing in the crib.

Front 21

What are the 2 causes of hydrops fetalis?

Back 21

Immune due to Rh or ABO incompatibility, or non-immune due to Turner syndrome or trisomies 21 and 18

Front 22

What is the most common cause of hydrops fetalis?

Back 22

Chromosomal anomalies like Turner syndrome or trisomies 18 and 21

Front 23

What is the most common autosomal recessive disease in NW European populations?

Back 23

Cystic fibrosis

Front 24

Which gene is defective in cystic fibrosis?

Back 24

The gene that codes for the CFTR protein, which is a Cl channel

Front 25

What is the primary defect in cystic fibrosis?

Back 25

Abnormal transport of Cl ion across epithelia

Front 26

Where does the CFTR protein not inhibit function of Na channels?

Back 26

In sweat glands, for this reason CF pts have very salty sweat (early diagnostic feature)

Front 27

What is the effect of CF in sweat glands as compared to respiratory epithelia?

Back 27

THe opposite thing happens, in sweat glands Cl ion can't get into the cell. In resp epithelia Cl ion can't escape the cell.

Front 28

What are the most important clinical manifestations of CF?

Back 28

Recurrent and chronic pulmonary infections, pancreatic insufficiency, and diabetes.

Front 29

What is the most common cause of death in CF pts?

Back 29

Pulmonary complications (ie severe chronic bronchitis, bronchiectasis, lung abscesses, Pseudomonas infection)

Front 30

What is the most common tumor of infancy?

Back 30

Hemangiomas, usually in the skin of face or scalp. Cause port wine stains.

Front 31

What is the most common germ cell tumor of childhood?

Back 31

Sacrococcygeal teratomas

Front 32

What is the second most common cause of death in kids 4-14 yrs old?

Back 32

Malignant tumors such as neuroblastoma, retinoblastoma, ans Wilms tumor

Front 33

What developmental origin do neuroblastomas typically have?

Back 33

Neural creast origin, 75% happen in abdomen

Front 34

What is a Homer-Wright pseudorosette?

Back 34

An arrangement of tumor cells around a central space, found in neuroblastomas

Front 35

What genetic mutation occurs in retinoblastomas?

Back 35

Loss of function due to mutation in RB1 gene

Front 36

What is a Wilms tumor?

Back 36

A nephroblastoma, most common primary tumor of the kidney in children.