Module 10: pediatric renal disorders

Question 1

Polycystic kidney disease (autosomal dominant )-

defect in the formation of ______= cyst formation =_____ obstruction, ____ fibrosis, loss of _____

Answer 1

epithelial cells

=renal parenchyma obstruction, interstitial fibrosis, loss of nephrons

Question 2

what genes =polycystic kidney disease

Answer 2

pkd1, pkd2 present in childhood/ adulthood

Question 3

what may develop from Polycystic kidney disease (autosomal dominant) (4)

Answer 3

htn, heart valve disease, cerebral and aortic aneurysms

Question 4

Hemolytic uremic syndrome characterized by ___anemia, ___, ___impairment

Answer 4

hemolytic anemia, thrombocytopenia, renal impairment

Question 5

Most common cause of AKI in young children (<4)

commonly exposed by (3)

Answer 5

Hemolytic uremic syndrome

unpasteurized drinks, contaminated meat/veggies, animals

Question 6

Hemolytic uremic syndrome associated with ___ and ___ agents

Answer 6

bacterial and viral agents

Question 7

Escherichia coli 0157:H7 and 0104:H4 strains associated with

Answer 7

Hemolytic Uremic Syndrome

Question 8

HUS Patho
Shiga toxin from Ecoli absorbed from intestines into blood, binds to leukocytes and is transported to kidneys=
lysis of_____ endothelial cells
separation of endothelial cells from _____,
activation and aggregation of___
activation of ____

Answer 8

lysis of glomerular capillary endothelial cells
separation of endothelial cells from basement membrane
activation/aggregation of platelets
activation of coagulation cascade

Question 9

HUS Patho
what causes the glomerular arterioles to swell
glomerular swelling =RBC ____ and ___ that are removed by the ____

Answer 9

occlusion d/t plts and fibrin clots
RBC damage and fragmented
removed by the spleen

Question 10

HUS Patho

there is a corresponding decrease in GFR w a child who has ___ and ____

Answer 10

hematuria and proteinuria

Question 11

HUS Patho
___are found in the microcirculation
plts cluster in damaged vessels =___

Answer 11

thrombi

thrombocytopenia

Question 12

HUS preceded by GI illness w diarrhea known as ____

atypical HUS is not associated w diarrhea, known as ___

Answer 12

D+HUS

D-HUS

Question 13

D+HUS occurs __-__wks after GI illness
child may be symptoms free up to 5 days then experiences sudden 4 sx
also can experience 7 sx

Answer 13

1-2 wks
pallor, bruising/purpura, irritability, oliguria
fever, anorexia, v/d, abd pain, jaundice, FVO

Question 14

D+HUS
seizures and Lethargy indicate ___involvement
renal failure may occur _days-_wks after onset of sx
renal failure= (4)

Answer 14

CNS
2days-2wks
metabolic acidosis, uremia, ^K, htn