Cystic Renal Disease Flashcards
A 3 year old presents with a history of polyuria and UTIs. On exam you note a high BP. U/S shows large cystic kidneys, hepatosplenomegaly. What is the likely diagnosis?
ARPKD: large kidneys, micro cysts, progressive renal failure, history of polyuria, UTIs, hepatic fibrosis
You see a child with widely spaced eyes with epicanthial folds, a broad nasal bridge, low set ears, and a receding chin. What is the name of this sequence? What disease is associated with it?
Potter sequence: widely spaced eyes with epicanthial folds, a broad nasal bridge, low set ears, and a receding chin, pulmonary hypoplasia, limb deformities ( club feet, hip dysplasia).
It is associated with ARPKD.
Which cystic kidney disease is not associated with kidney stones?
a) ADPKD
b) medullary sponge
c) multi cystic
d) simple renal cysts
e) c & d
e) multi cystic and simple renal cysts
The diagnosis of ADPKD is best made by:
a) history
b) PE
c) renal U/S
d) urinalysis
c) renal U/S
True or false? ADPKD diagnosis can only be made in adults.
False - can be diagnosed in kids or adults
Which of the following is a non-genetic form of cystic renal disease?
a) ADPKD
b) ARPKD
c) simple renal cysts
d) medullary sponge kidney
e) nephronopthisis
c) simple renal cysts, d) medullary sponge kidney can also be correct
In ADPKD which of the following organs does NOT develop cysts?
a) brain
b) liver
c) pancreas
d) spleen
a) brain
Which of the following cystic kidney diseases is associated with hepatic fibrosis?
a) ADPKD
b) ARPKD
c) simple renal cysts
b) ARPKD results in hepatic fibrosis. ADPKD results in hepatic cysts.
Which of the following statements is true of Potter Syndrome?
a) it is a genetic disease
b) describes physical characteristics and findings in an infant resulting from low amniotic fluid
b)
Which cystic disease occurs after abnormal nephrogenesis (ureteral bud) leading to atresia or absence of the bud?
a) cystic renal dysplasia
b) multi-cystic dysplastic kidney
c) nephrophthsis
d) simple renal cysts
b) multi cystic dysplastic kidney
Which of the following is often associated with antenatal obstruction of the urinary tract?
a) cystic renal dysplasia
b) multi cystic dysplastic kidney
c) simple renal cysts
a) Cystic renal dysplasia is often associated with antenatal obstruction of the urinary tract; posterior urethral valves, UPJ.
Which cystic disease most commonly leads to paediatric ESRD?
a) ADPKD
b) medullary sponge kidney
c) nephronophthisis
d) simple renal cysts
c) nephronopthisis
Which cystic kidney disease is associated with skin lesions?
a) ADPKD
b) Tuberous sclerosis complex
c) von hippel-lau
b) Tuberous sclerosis complex
Neurocutaneous presentation: cognitive delay, skin findings, vascular lesions.
