Pediatric Disorders

Question 1

What are some examples of transcervical (ascending) perinatal infections?

Answer 1

Beta hemolytic streptococcal (bacterial) and herpes (viral); may cause pneumonia, sepsis, meningitis; associated with chorioamnionitis and funisitis.

Question 2

What are transcervical perinatal (ascending) infections?

Answer 2

Infections that spread through the cervicovaginal canal in utero or during birth. Fetus can also gain infection from amniotic fluic.

Question 3

What are some exampls of transplacental (hematological) perinatal infections?

Answer 3

Viruses, parasites, some baterial. You have TORCH (toxoplasma, rubella, CMV, herpes), and T pallidum. Usually present with fever, encephalitis, chorioretinitis, hepatosplenomegaly, pneumonia, myocarditis, anemia.

Question 4

What causes respiratory distress?

Answer 4

Most common casue is respiratory distress syndrome. Excessive maternal sedation during delivery, fetal head injury during deliery, aspiration of blood of amniotic fluid, intrauterine hypoxia.

Question 5

What are the risk factors for respiratory distress syndrome (RDS)?

Answer 5

Mainly premature birth (<36 wks), but also maternal diabetes, C-section before labour, twins, male infants.

Question 6

What is the pathogenesis of RDS (hyaline membrane disease)?

Answer 6

Immature lungs cannot create enough surfactant (made by type II pneumocytes). Alveoli collapse and infant tires from breathing. Hypoxia leader to epithelial/endothelial damage leading to formation of hyaline membranes.

Question 7

What are treatments for RDS?

Answer 7

Corticosteroids for the mother if early delivery is unavoidable (increases surfactant synthesis), supportive ventlilation, aerosolized natural or recombinant surfactant.

Question 8

What is sudden infant death syndrome?

Answer 8

Sudden and unexpected mysterious death <1 yrs of age. It is the leading cause of death during infancy in developed countries. Most babies are during age 2-4 months.

Question 9

What are some maternal factors for SIDS?

Answer 9

Youth <20 yrs, unmarried, short intergestational periods, low SDS, smoking, and drug abuse.

Question 10

What are some infant factors for SIDS?

Answer 10

Prematurity, low birth weight, male, multiple births, not 1st sibling, prior SIDS.

Question 11

What are some environmental factors for SIDS?

Answer 11

Prone sleeping position (sleeping on belly), sleeping in soft surface, hyperthermia, postnatal passive smoking.

Question 12

What are heterotopia or choristoma?

Answer 12

Microscopically normal cells or tissues present in abnormal locations. (pancreatic tissue)

Question 13

What are hamartomas?

Answer 13

Excessive but focal overgrowth of cells and tissues native to the organ in which it occurs (pulmonary hamartoma of cartiladge, smooth muscle, respiratory epithelium.

Question 14

What are teratomas?

Answer 14

Monster tumour, from all 3 germ layers; may be benign, lesions of indeterminate malignant potential or frankly malignant. Usually sacrococcygeal ,in the testes, ovaries, mediastinum, retroperitoneum, head/neck.

Question 15

What are the two types of benign tumours?

Answer 15

Hemangiomas and lymphangiomas.

Question 16

What are hemangiomas?

Answer 16

Usually capillary or cavernous, usually on the skin as well as within internal organs. Most common during infancy.

Question 17

What are lymphangiomas?

Answer 17

In cystic or cavernous spaces; usually in skin, deeper regions of neck, axilla, mediastinum, retroperitoneum, abdominal.

Question 18

What are the 3 types of small round blue cell malignant tumours in childen?

Answer 18

Neuroblastomas, retinaoblastomas, wilms tumours.

Question 19

What are neuroblastomas?

Answer 19

Originating from the neural crest and may have deletion in chromosome 1 (N-MYC). May arrise in adrenal glands.

Question 20

What is the chance of surviving to 5 years with a neuroblastoma?

Answer 20

55%

Question 21

What are retinoblastomas?

Answer 21

It is a malignant eye tumour and often congenital but may undergo spontaneous regression.

Question 22

Familial retinoblastoma is usually characterized by what?

Answer 22

Multiple focal points and bilateral (both eyes).

Question 23

Sporadic retinoblastomas are usually characterized by what?

Answer 23

Unilateral and unifocal.

Question 24

What is the pathogenesis of retinoblastomas?

Answer 24

Mutation in tumour supressor gene and retinoblastoma gene on ch13; both copies must be knocked out.

Question 25

What are Wilms tumours?

Answer 25

Most common type of primary tumour of the kidney in children (nephroblasoma). Often associated with other congenital malformations.

Question 26

How do Wilms tumours present?

Answer 26

Abdominal mass;

Question 27

What is the prognosis of wilms tumour patients?

Answer 27

90% long term survival.

Question 28

What are other imporant malignant in infants/children?

Answer 28

Leukemia, rhabdomyosarcoma (skeletal muscle), hepatoblastoma, CNS.