31. Kidney Disease in Children

Question 1

with hematuria and either systemic s/s, or otherwise asymptomatic presentation, should I be thinking glomerular or non-glomerular?

Answer 1

glomerular. (glomerulonephritis)

urine will be dark/coke colored, RBC casts, acanthocytes.

Question 2

with hematuria and localized s/s of disease, should I be thinking glomerular or non-glomerular?

Answer 2

non-glomerular. acronym is TTICCHSS

urine will be pink/red, with normal RBCs

Question 3

what does TTICCHSS stand for?

Answer 3

non-glomerular causes of hematuria:

Trauma, Tumors, Infection, Cystic dz, Congenital Obstruction, HyperCa, Stones, Sickle Cell

Question 4

Ddx of glomerulonephritis in children?

Answer 4

(remember Peeing blood Means Lupus and hemATurIgA)
-post-infectious (most common)
-membranouproliferative GN
-lupus
-Alport
-Thin Basement Membrane
-IgA nephropathy
Also: henoch schonlein purpura

Question 5

Ddx of glomerulonephrosis in children?

Answer 5

Minimal change (most common)
FSGS
Lupus (if secondary)

Question 6

presentation of patient with MCD?

Answer 6

periorbital edema, post-URI. edematous, proteinuria, urine microscopy negative, high cholesterol.

Question 7

what is the most common cause of anasarca in children?

Answer 7

nephrotic sx
(anasarca = generalized edema)

Question 8

If there is dysfunction at the proximal tubule, what might we see in the patient? (in general, and the most significant problems that will result)

Answer 8

overall decrease in reab of water, Na, bicarb, Ca, phosphate, uric acid, glucose, AAs.
sig problems: poor growth, metabolic acidosis, rickets

Question 9

most common causes of dysfunction at the prix tubule?

Answer 9

metabolic or congenital causes

Question 10

if there is dysfunction at the LOH, what symptoms will we see?

Answer 10

(Bartter’s Sx: like being on chronic furosemide)

• polyuria, polydipsia, salt craving
• dehydration, RAAS activation, high aldo
• hypoK, metabolic acidosis
• HyperCa (loss of paracellular Ca)
• stones, Ca deposits, renal failure

Question 11

what can cause dysfunction at the LOH?

Answer 11

Bartter’s Sx: auto recessive loss of function.

Question 12

dysfunction at the DCT will cause what?

Answer 12

• Wasting of Na, Cl -> hypokalemia and metabolic alkalosis
• HypoMg because of Mg wasting
• HyperCa because of increased Ca reabsorption

Question 13

kids with dysfunction at the DCT crave what?

Answer 13

pickle juice, V8! because they are Mg-deficient and these contain Mg

Question 14

hyper function at the DCT of the Na-Cl symporters can lead to what symptoms? Tx?

Answer 14

Salt and fluid retention
HTN with low renin
HyperK, metabolic acidosis
Tx = thiazides

Question 15

dysfunction at the collecting duct will cause what?

Answer 15

(Liddle’s Sx)
GOF mutation of the ENaC channel. sodium retention, HTN with low renin
hypokalemia and metabolic alkalosis

Question 16

what symptoms will we see with nephrogenic DI?

Answer 16

(tubular non-responsiveness to ADH)
polyuria, polydipsia
hypernatremic dehydration if no access to free water

Question 17

Nephrogenic DI: if medication-caused, what are the meds?

Answer 17

lithium, tetracycline

Question 18

MultiCystic Dysplastic Kidney (MCKD) is an example of what kind of disease?

Answer 18

cystic/dysplastic disease. usually do not have hereditary implications

Question 19

How does MCKD appear grossly?

Answer 19

like a collection of grapes, no identifiable renal tissue

Question 20

MCKD: unilateral or bilateral?

Answer 20

UNI, because bilateral is not survivable

Question 21

MCKD: what is the cause?

Answer 21

embryological: the ureteric bud does’t find the mesonephric duct it is supposed to join with. the tissue becomes cystic.

Question 22

MCKD: symptoms?

Answer 22

unilateral kidney function, if bad kidney is big and cystic, may have GERD or feeding problems. may be asx

Question 23

what kind of disease is ARPKD, ADPKD? what are the problems?

Answer 23

inherited cystic kidney diseases

kidneys form normally, but tubular cystic changes form gradually and kidneys progressively lose function.

Question 24

Prune Belly syndrome is an example of what? occurs in males or females?

Answer 24

Genitourinary Malformation

males only

Question 25

Prune Belly: what happens, how does it present?

Answer 25

weak abdominal wall musculature, undescended testes, weak muscles around the urinary tract (low urine pressure, infections, renal failure)

Question 26

cloacal anomaly: males or females? how does it present?

Answer 26

females only
failure of urorectal septum to develop, urethra/vag/colon may be interconnected
one or more tracts may be obstructed

Question 27

bladder outlet obstruction in utero can result in what?

Answer 27

if severe, Potter’s syndrome (renal failure, pulmonary hypoplasia, limb deformities). may result in oligohydramnios, hydronephrosis, dysplasia