Peds Exam 3

Front 1

which is more common, ADPKD or ARPKD?

Back 1

Think about it one is dominant PKD and the other recessive. ADPKD is more common

Front 2

When does ADPKD usually present?

Back 2

in 4th or 5th decade

Front 3

associated with bilateral enlargement of the kidneys and multiple cysts in both the cortex and medulla. Hepatic cysts present in most cases, as well as splenic and pancreatic cysts

Back 3

ADPKD

Front 4

Marked enlargement of bilateral kidneys and innumerable cysts throughout the cortex and medulla
Often progresses to tubular atrophy and interstitial fibrosis and even renal failure
Liver involvement ALWAYS present in the form of hepatic fibrosis, cysts, cholangitis, biliary dysgenesis

Back 4

ARPKD

Front 5

If you find multiple cysts on a babies kidneys what other organ must you check?

Back 5

LIVER. ARPKD wil always have liver involvement

Front 6

In ARPKD, liver involvement is ALWAYS present in what forms?

Back 6

1. hepatic fibrosis
2. cysts
3. cholangitis
4. biliary dysgenesis

Front 7

How do we diagnose ARPKD?

Back 7

By ultrasound, may be dx prenatally if cysts present

Front 8

What are the 4 classifications of ARPKD distinguished by? what are they?

Back 8

distinguished by age
1. perinatal
2. neonatal
3. infantile
4. juvenille

Front 9

what is the Tx for ARPKD?

Back 9

1. supportive, including managing renal htn
2. dialysis if necessary
3. Renal transplantation!

Front 10

a simple cyst found on the kidney. we usually just leave it there.

Back 10

acquired cystic disease

Front 11

what are two cysts types associated with systemic disease?

Back 11

1. Tuberous sclerosis
2. Von Hippel Lindau

Front 12

what are the Sx of UTI in neonate/infants?

Back 12

vomiting, fever, irritability, feeding problem

Front 13

What are the sxs of UTI in 2-6 yo?

Back 13

GI sx
and pain
dysuria
frequency
malodorous urine

Front 14

How do we dx a UTI in peds?

Back 14

1. urinalysis: + leukocyte esterase, >3-5 WBC per HPF (diagnostic), + nitrites, + blood
2. Urine culture with sensitivities
3. Elevated serum WBC

Front 15

What on urinalysis is dx of uti?

Back 15

1. >3-5 WBC per HPF
2. + blood
3. + leukocyte esterase
4. + nitrites

any WBC in urine of baby is uti until proven otherwise. Do a catheter, clean catch not so feasible at younger ages

Front 16

what are the pathogens likely to cause UTI in kids?

Back 16

MC = e. coli
other gram negative bacteria ("peck") -- Proteus, Enterobacter, citrobacter, Klebsiella

Front 17

what is first line therapy for UTI in kids?

Back 17

3rd generation cephalosporin for 7-14 days
ex: cefixime, cefdinir, ceftibuten

May also consider Bactrim/Septra (SMX-TMP_

Front 18

what drugs do we not want to use for tx of UTI in kids?

Back 18

Amoxicillin, amoxicillin-clavulanate and 1st gen cephaolopsorins should not be used due to increasing rates of resistance

Front 19

oral therapy for UTI is considered for whom?

Back 19

>2 mo who can tolerate po

Front 20

what 5 factors would lead us to hospitalize a child for an UTI?

Back 20

1. Less than 2 mo old
2. clinical urosepsis (toxic appearance, hypotension)
3. inability to take po, vomiting
4. immunocompromised pt
5. failure of outpt. therapy

Front 21

what is 1st line tx for parenteral tx of UTI?

Back 21

3rd or 4th generation cephalosporins
*Ceftriaxone "Vit. R"

Front 22

what are parenteral tx options for UTI tx?

Back 22

1. 3rd of 4th cephalosporin
2. aminoglycoside (gentamicin) usually in combo with ampicillin
Use total of 10-14 day course combo of IV and PO

Front 23

which patients need renal US as follow up to UTI?

Back 23

1. <2yo with hx of febrile UTI
2. Any child requiring hospitalization/parenteral thearpy
3. UTI in a male child
4. Recurrent febrile UTI

Front 24

WHen would we do a VCUG ?

Back 24

should be performed in any patient with evidence of hydronephrosis on renal US

Front 25

what lab study should we do on someone with hydronephrosis?

Back 25

VCUG - voiding cystourethrogram

Front 26

abnormal backflow of urine from the bladder to the ureter to the kidney due to incompetence of the vesicoureteral junction

Back 26

Vesicoureteral reflux

Front 27

what is the MC cause of primary VUR?

Back 27

due to inadequate closure of the UVJ

Front 28

what is the cause of secondary VUR?

Back 28

due to abnormall high bladder pressure which results in failure of closure of the UVJ due to:
1. anatomic- posterior urethral valve
2. physiologic - neurogenic bladder

Front 29

fills and grossly dilates ureter and collecting system, blunting of calyces, tortuosity of ureters
what VUR grade?

Back 29

Grade 4

Front 30

fills and mildly dilates the ureter and collecting system with blunting of calyces
what VUR grade?

Back 30

grade 3

Front 31

reflux fills the ureter without dilitation
what VUR grade?

Back 31

grade 1

Front 32

massive reflux and gross dilitation, significant ureteral tortuosity
what VUR grade?

Back 32

grade 5

Front 33

-fills the ureter and collecting system, no dilitation
what VUR grade?

Back 33

grade 2

Front 34

30-60% of pts at initial diagnosis of VUR also have evidence of?

Back 34

renal scarring
my be congenital or effect of recurrent infection

Front 35

what is the first choice for UTI prophylaxis in children <2 mo?

Back 35

Amoxicllin
even though we never use it as a first line thearpy

Front 36

In children >2 months old what can we use for UTI prophylaxis?

Back 36

TMP-SMX, nitrofurantoin or cephalexin

Front 37

what is the MCC of bladder outlet obstruction in males?

Back 37

posterior urethral valve

Front 38

Obstructing membranous folds within the lumen of the posterior urethra

Back 38

posterior urtethral valve

Front 39

what is the MCC of ascites in the newborn period?

Back 39

urinary ascites, from rupture of the renal pelvis as a possible cause

Front 40

if posterior urethral valve is not dx by US in utero how might the infant present?

Back 40

with poor urinary output
failure to thrive
urosepsis
grunting/straining while voiding

Front 41

how do we dx posterior urethral valve?

Back 41

1. US in utero or
2. VCUG

Front 42

what will VCUG show if there is posterior urethral valve?

Back 42

will show dilated and elongated poseriror urethra during voiding phase

Front 43

what is the Tx for posterior urethral valve?

Back 43

preliminary decompression
valve ablation (surgery)

Front 44

when might we see acute post- sreptococcal gomerulonephritis (AGN)?

Back 44

Following GABH pharyngitis or impetigo-pydoerma by 2-21 days

Front 45

What is the MOA in which GABH srep is nephritogenic?

Back 45

activated the completment system and causes glomerular rpoliverative and inflammatory response. This causes a decrease in volume of glomerular filtrate and capacity to excrete salt and water which then causes an expansion of extracellular fluid (edema)

Front 46

what are the signs and sx of AGN?

Back 46

Asymptomatic gross hematuria
Proteinuria with or without RBC casts
Tea or cola colored urine
Late sx: HTN, Heart failure, encephalopathy, oliguria

Front 47

Diagnosis of AGN is made by what information?

Back 47

1. hx of sore throat
2. hamaturia
3. htn
4. presence of edema

Front 48

what dx test can you run for AGN?

Back 48

serum streptozyme test - tests for 5 different streptococcal antibodies including (ASO) anti-streptolysin

Front 49

how do we treat AGN?

Back 49

by the time the child present w/ sx, glomerular injury has already occured and healing process has begun.
SUpportive sx managment of HTN with CCB, ACE inhibiors and vasodilators
95% of children recover completely

Front 50

Characterized by proteinuria, hypoproteinemia, hypercholesterolemia and edema

Back 50

nephrotic syndrome

Front 51

what is the MCC of idiopathic nephrotic syndrome?

Back 51

Minimal Change dz

Front 52

Pts will present with dependent pitting edema, diarrhea (caused by intestinal edema) and respiratory distress (caused by pulmonary edema or effusion)

Back 52

minimal change disease

Front 53

Defined as the absence of persistent hematuria, absence of hypertension, normal complement levels and normal renal function

Back 53

minimal change disease

Front 54

what tx are most patients responsive to for minimal change dz?

Back 54

most respond to steroids
good sign if they do, favorbale long term outcomes and low risk of chronic renal dz

Front 55

if a child with minimal change dz is not responding to steroid tx what should we do?

Back 55

renal bx is indicated. there may be underlying pathology

Front 56

Acute immunoglobulin A-mediated leukocytoclastic vasculitis
May cause nephritis (28%)

Back 56

Henoch-Schonlein Purpura

Front 57

signs and sx of HSP?

Back 57

arthritis
cutaneous purpura
GI bleed
abdominal pain

Front 58

hematuria and proteinuria often present with HSP if so what do we worry about?

Back 58

nephrotic syndrome may occur
15%risk of developing renal failure just with the hematuria and proteinuria but with nephrotic syndrome this increases to 50%

Front 59

what is the tx for HSP?

Back 59

1. supportive
2. plasmapheresis - used to treat rapidly progressive HSP
3. corticosteroids - may use for arthralgias if indicated

Front 60

Abrupt reduction in kidney function measured by a rapid decline in GFR

Back 60

Acute kidney injury (acute renal failure)

Front 61

what are possible prerenal causes of AKI?

Back 61

1. volume depletion (blood loss or urinary)
2. decreased arterial pressure/volume (heart failure, shock or cirrhosis)

Front 62

what are possible intrarenal cuases of AKI?

Back 62

1. glomerular - acute glomerulonephritis MC post infections
2. Tubular and Interstitial Dz - acute tubular nephrosis- results from ischemia due to decreased renal perfusion
nephrotoxic agents such as gentamicin, contrast(amphtericin B), Myoglbinuria due to rhabdo (tea colored urine very common in athletes)
3. Vascular - thrombosis, HUS, vasculitis

Front 63

what are possible postrenal causes of AKI?

Back 63

bilateral urinary tract obstruction

Front 64

AKI history may reveal sx such as?

Back 64

Vomiting, diarreha, hemorrhage, sepsis, dehydration, blood diarrhea with olgiuria (HUS), pharyngitis or impetigo (PSGN)

Front 65

on physical exam for AKI what would you find?

Back 65

1. tachycardia
2. dry mucous membranes
3. orthostatic BP changes
4. Purpura, malar rash, petichiae (HSP)
5 pyuria with WBC and granular casts or waxy casts
6. hematuria

Front 66

what sort of labs would you order on a patient with suspected AKI?

Back 66

based on hx, sx and index of suspicion order:
1. renal US
2. renal Bx
3. chest xray

Front 67

The NKF-KDOQI defines chronic renal failure as?

Back 67

disorder lasting 3 months or more with kidney damage defined by structureal or functional renal abnormalities, OR GFR <60ml/min per 1.73 m2

Front 68

Between birth and 10yo what are common causes of CRF?

Back 68

congential and obstructive abnormalities MCC
ex: posterior urethral valve, polycistic kidney

Front 69

after 10 yo what are the common causes of CRF?

Back 69

acquired diseases are MCC
ex: focal segmental glomerulosclerosis, HUS, chronic glomerulonephritis

Front 70

WHat are the clinical manifestations of CRF?

Back 70

1. growth failure
2. progressive anemia
3. HTN
4. decreased GRF can result in - sodium and water retention (edema), hyperkalemia, metabolic acidosis

Front 71

decreased GFR in CRF can result in?

Back 71

1. sodium and water retention
2. hyperkalemia
3. metabolic acidosis

Front 72

What is the management for CRF?

Back 72

1. diet
2. loop diuretics
3. iron supplementaion and EPO stimultaing agents (ESA)
4. if reversible tx the cuase

Front 73

When altering diet for CRF management what suggestions would you make to the patient?

Back 73

1. restrict sodium to 1.2 to 1.5 g/day
2. low potassium diet
3. restrict milk and produts due to high phophate content
4. infants require >125% recommended daily caloric intake

Front 74

what is the Tx for end-stage renal failure?

Back 74

1. dialysis
2. renal transplantation

Front 75

Urethral meatus located below and proximal to its normal position

Back 75

hypospadias

Front 76

hypospadias occurs how often?

Back 76

1:500 males

Front 77

Can males with hypospadias be circumcised?

Back 77

NO, they need the foreskin to repair it laer. usually done before 18mo

Front 78

The inability to retract the prepuce after age 3
Severe cases may require circumcision or surgical enlargement of the opening

Back 78

phimosis

Front 79

When the prepuce is retracted behind the coronal sulcus and cannot be returned to its normal position

Back 79

paraphimosis

Front 80

what is the likelihood that a premie baby weighing 900g will have cryptorchidism?

Back 80

100%! perecentage increases with prematurity.

Front 81

Intra-abdominal testis are at a higher risk for what?

Back 81

tumor development
required surgical correction, associated with greater probablilty for fertility.

Front 82

Normal testes that retract into the inguinal canal as a result of an exaggerated cremasteric reflex

Back 82

retractile tetes

Front 83

when testes do not descend we call this?

Back 83

cryptorchidism