Nephrology COPY Flashcards
(224 cards)
1
Q
You are seeing a 4 year old girl who was treated for impetigo 2 weeks ago. She now presents with brown coloured urine. Her vitals show an HR of 100, T 37, BP 160/100, RR 20. She has a gallop rhythm and crackles bilaterally. Her liver edge is 1cm below the costal margin. Has hematuria with RBC casts and 3+ protein on urinalysis. Based on the most likely etiology, what is the pathophysiology?
Interstitial fibrosis and microangiopathic thrombosis
Immune complex deposition in glomeruli
Altered podocyte function at the glomerular basement membrane
generalized proliferative glomerular inflammation
A
Immune complex deposition in glomeruli
this patient has post-strep glomerulonephritis
Group A strep antigen deposition and immune complex formation –> Immune complex leads to complement deposition, mesangial cell proliferation and leukocyte infiltration
PSGN leads to decreased glomerular filtration rate (GFR) –> salt and water retention –> fluid overload and hypertension
Volume overload results in :
- gallop rhythm (S3/S4 heart sound)
-pulmonary congestion (crackles)
-hepatic congestion (hepatomegaly)
2
Q
10-year-old boy with 3 UAs showing proteinuria and 0-2 RBCs/HPF. Exam is normal with normal blood pressure. What is your next step?
First morning UA
24 hour urine protein collection
Creatinine, BUN and lytes
Renal US
A
First Morning UA
this is likely orthostatic proteinuria, obtain first morning UA for 3 consecutive days
3
Q
Benign causes of proteinuria?
A
Orthostatic (teen boys)
Transient (fever, exercise, dehydration, cold exposure, or stress)
4
Q
Pathologic causes of proteinuria?
A
Tubular (Fanconi, RTA, ATN, AIN, CAKUT, cystinosis)
Glomerular (minimal change, focal segmental, membranous nephropathy)
Glomerulonephritis (proteinuria with hematuria, edema, HTN)
5
Q
Features of orthostatic proteinuria?
A
tall teen boys
proteinuria bc kidney protein excretion increases in the upright position
absence of proteinuria on first morning UA
other lab work is normal:
normal Cr, albumin
normal BP
No hematuria
6
Q
Investigations for orthostatic proteinuria?
A
3 consecutive first morning urinalysis (should not show proteinuria)
7
Q
treatment of orthostatic proteinuria?
A
reassurance
8
Q
what are transient causes of proteinuria?
A
fever, exercise, dehydration, cold exposure, or stress
repeat urinalysis once acute trigger resolved
9
Q
features of transient proteinuria?
A
normal Pro/Cr ratio + intermittent (not persistent) proteinuria
10
Q
what is tubular proteinuria?
A
Alpha 1 and beta 2 microglobulins are low molecular weight proteins that are resorbed in the proximal tubule
damage to the tubules results in urinary loss of these proteins
if these proteins are found in the urine, there is proximal renal tubular injury (Eg. proximal RTA)
11
Q
what is Fanconi syndrome?
A
Fanconi syndrome is these 4 signs of proximal tubular dysfunction:
- Tubular proteinuria
- Glucosuria
- Proximal renal tubular acidosis (RTA) with bicarbonate wasting
- Phosphaturia
12
Q
What are glomerular causes of isolated proteinuria (no hematuria suggestive of GN)?
A
- Minimal Change disease (most common cause of nephrotic syndrome)
- Focal segmental glomerular sclerosis
○ Idiopathic or secondary - Membranous Nephropathy (rare in children)
13
Q
what are the causes of focal segmental glomerular sclerosis?
A
- Idiopathic
- secondary to infection (Hep B, C, HIV), autoimmune disease, drugs, malignancy, or chronic hyperfiltration (e.g. obesity, hypertension, heart failure)
14
Q
what is nephrotic syndrome? (4 features)
A
increased permeability of the glomerular filtration barrier leading to nephrotic-range proteinuria (frothy urine), hypoalbuminemia, hyperlipidemia and edema
2021 Diagnostic criteria:
- Hypoalbuminemia (serum albumin <30) OR Edema
- Proteinuria (first morning urine >200 mg/mmol or >3+ dipstick)
15
Q
what defines “nephrotic-range” proteinuria?
A
> 3.5g/L protein
urine protein/creatinine ratio of >0.02 g/mmol or >200mg/mmol
24 hr urine protein >40 mg/m^2/hr. OR >1000mg/m2/day
16
Q
what is the most common cause of nephrotic syndrome in children?
A
Minimal change disease
17
Q
typical age group for minimal change disease?
A
school age typically (1-10yrs)
(if adolescent with nephrotic syndrome, think about another cause such as lupus nephritis)
(if age group abnormal or pts don’t respond typically to steroids, then nephro will do renal biopsy to look for alternative diagnosis to minimal change disease)
18
Q
what is the pathogenesis of minimal change disease?
A
Altered podocyte function at the glomerular basement membrane (results in protein passing into the urine = proteinuria, albuminuria)
Minimal change disease is the most common cause of nephrotic syndrome
19
Q
Treatment for minimal change disease?
A
Prednisone x 4-6 weeks then taper over next ~6 weeks
80% of children will respond (protein to creatinine ratio returns to normal)
Albumin 25% if signs of hypovolemia – oliguria, AKI, hyponatremia, prolonged cap refill time, tachycardia, abdo discomfort, hypotension, or severe edema
20
Q
80-90% of patients with minimal change disease will experience one or more relapse. What prognostic factor identified the patients with better outcomes?
A
if kids relapse, we treat them again with steroids
kids that respond to steroids have better outcomes
(steroid resistant kids have worse outcomes)
21
Q
Lab findings in nephrotic syndrome
A
nephrotic range proteinuria on UA
Hypoalbuminemia (pee out protein)
Edema (from lack on oncotic pressure)
Hyperlipidemia (low oncotic pressure stimulates cholesterol synthesis, LDL and TG)
22
Q
Why are nephrotic patients immunosuppressed and at risk for infections?
A
the following are lost in the urine resulting in immunosuppression:
IgG (incr risk of encapsulated organisms such as STREP PNEUMO - hence we give them extra vaccine against Strep
pneumo)
Factor B (part of complement system; phagocytosis)
Edema and pleural/peritoneal effusions allow for bacterial proliferation –> spontaneous bacterial peritonitis (SBP)
often treated with steroids which reduce immune system
23
Q
Treatment of nephrotic syndrome?
A
These pts are edematous –> Identify if intravascularly dry (tachycardic, elevated Cr) or volume overloaded (HTN)
fluid + salt restrict
25% albumin if dry
ACEi if fluid overloaded
NO DIURETICS (at risk for thromboembolism)
24
Q
complications of nephrotic syndrome?
A
spontaneous bacterial peritonitis
- from stagnant fluid collection in abdomen (usually strep pneumoniae)
thromboembolism (eg DVT, Renal Vein Thrombosis)
- Hypercoagulable from loss of natural anti-coagulants (anti-thrombin III, and Protein C + S)
- think of RVT if patient with nephrotic syndrome develops gross hematuria –> do Doppler renal US
Infection
- from urinary losses of compliment proteins and Ig
- if fever, culture and cover for strep pneumo and gram neg (predominantly E coli)
Hyperlipidemia (part of tetrad)
Hypothyroid (loss thyroid binding globulin in urine)
25
treatment of nephrotic syndrome
oral steroids: Prednisone 60mg/m2/d x 6 weeks (may require another course if relapse)
if frequent relapse, maintenance steroids or immunosuppressive agent (Eg. Cyclosporine, Rituximab)
When off high dose steroids, children with nephrotic syndrome should be vaccinated with the 23 valent pneumococcal vaccine and get the yearly flu shot.
26
side effects of high-dose steroid course?
Cushingoid appearance
Weight gain
HTN
Diabetes
osteoporosis
skin thinning
pancreatitis
gastritis
WBC/neut demargination (neutrophils detach from the blood vessel walls and circulate freely in the bloodstream, labs show high WBC/neut)
27
90% of nephrotic syndrome is caused by minimal change disease or focal segmental glomerulosclerosis. what are the red flags that it is a secondary cause of nephrotic syndrome?
Secondary causes include GN, lupus, drugs (eg NSAIDs), hyperfiltration states (obesity), malignancy (leuk/lymphoma), infection (hepatitis B, C, HIV)
Red Flags (refer to nephro):
- Under 1yr
- Over 12yrs
- Hypertensive
- Gross Hematuria (not related to renal vein thrombosis which is complication of nephrotic synd)
-AKI that is not pre-renal
- Low C3
- Arthritis or rashes
- Steroid-resistance (persistence of proteinuria after 4 weeks of steroid therapy)
28
Child with proteinuria, 3+ peripheral edema, ascites, and coming in with fever and abdominal distension and abdominal pain. He undergoes paracentesis and there are bacteria seen. What is the most common bacteria causing this presentation?
A. Escherichia coli
B. Streptococcus pneumoniae
C. Bacteroides fragilis
D. Enterococcus faecalis
Strep pneumo
nephrotic pts are at risk for encapsulated organisms bc they are losing Ig in their urine (this is also why we give an extra vaccine against strep pneumo)
29
patient with nephrotic syndrome presents with abdo pain and fever - what is it and how do you treat?
Spontaneous Bacterial Periontinitis
usually caused by strep pneumo
dx: peritoneal fluid culture via paracentesis
Tx: IV Vanco + 3rd gen cephalosporin
30
A girl with nephrotic syndrome is admitted. She has no respiratory distress. She has pitting edema, ascites, and pleural effusions. On labs, has a sodium 125 and albumin was 22. What do you do for management?
Fluid restrict
0.9 nacl
Albumin 1g/kg
3% saline
Albumin 1g/kg per Dr Ding (with lasix)
the hypoNa and pleural effusions are indicating intravascularly deplete
fluid restriction won't work fast enough
31
10 month old male had a VCUG after his second febrile UTI. VCUG showed Grade 2 reflux on VCUG. What is the most appropriate management?
a) DMSA
b) No intervention is necessary
c) Prophylactic antibiotics for one year
d) Follow up VCUG in 1 year
Follow up VCUG in 1 year
if low grade vesicoureteral reflux, do VCUG and KUB q12mo (natural course is to improve) + teach parents that fever may indicate UTI
Only grade 4 or 5, or significant renal anomaly require prophylactic antibiotics
32
what is VUR?
vesicoureteral reflux = retrograde passage of urine from the bladder to the ureter and kidney
"vesica" is latin word for bladder
33
what causes vesicoureteral reflux?
improper closure of ureterovesical
junction (where ureter meets bladder) - "Primary"
high voiding pressure in bladder (from posterior urethral valves, neurogenic bladder, bladder dysfxn) - "Secondary"
34
Presentation of VUR?
Antenatally: hydronephrosis
Postnatally: febrile UTI can lead to workup revealing VUR
35
Grading of vesicoureteral reflux (VUR) based on VCUG findings?
Grade I - fills ureter
Grade II - fills ureter and collecting system
Grade III - fills ureter and collecting system, mild dilation
Grade IV - grossly dilates ureter and collecting system, blunted calices of kidney
Grad V - massive dilation and ureteral tortuosity
36
treatment of Grade 1 - 3 VUR?
if low grade vesicoureteral reflux:
1. VCUG and KUB q12mo (natural course is to improve)
2. counsel the parents about assessing for UTI early in febrile illnesses and the importance of early antibiotics in this setting to prevent renal scarring
37
treatment of high grade VUR?
Grade IV or V vesicoureteral reflux, or significant anomalies (e.g. grade III VUR in solitary kidney) --> prophylactic antibiotics are considered
3-6 mo of Trimethoprim/Sulfamethoxazole, Nitrofurantoin or Cephalexin
Stop the ppx abx if pt grows bug resistant to abx
38
which febrile patients needs to be screened for UTI?
preverbal children (<3 yrs) with unexplained fever
verbal children with sx (dysuria, urinary frequency, hematuria, abdo pain, back/flank pain or new daytime incontinence)
39
Investigations to send in UTI work up?
midstream urine for UA and Ucx if toilet trained (girls sit backwards helps spread labia and reduce contamination)
otherwise urine cath or suprapubic aspirate
Bagged urine sample for UA ONLY (initial screen)
Do not need BCx unless hemodynamically unstable
Monitor renal function if complicated UTI or aminoglycosides >48h
40
UA findings in UTI?
positive nitrites on UA strongly suggests UTI (unless gram + org which doesn't metabolize nitrite)
positive leukocyte esterase (indirect measure pyuria) – false neg w/ low concentration of WBC (microscopy can be helpful)
Pyuria >5 WBC/HPF 73% sens and 81% spec for dx of UTI
41
Pathogens causing UTI?
Escherichia coli, Klebsiella pneumoniae, Enterobacter species, Citrobacter species, Serratia species or, in adolescent females only, Staphylococcus saprophyticus.
42
what colony count on urine culture indicated UTI?
note: Ucx MUS be pre Abx (1 dose abx sterilizes urine)
Clean catch: > 10^8 CFU/L
In and out: > 5x10^7 CFU/L (CPS) (>1x 10^7 CFU/L AHS)
Suprapubic: Any growth
Mixed growth is usually contamination
43
treatment of UTI?
PO cefixime 8 mg/kg/day daily x 7-10 days
if requiring hospitalization or structural kidney anomaly = IV gentamicin* 5mg/kg/d or CTX 50mg/kg/d until able to switch to PO
*gent is more narrow than CTX; must monitor gent levels and Cr/urea
if <2mo, IV abx initially
teenage girls with cystitis (afebrile UTI) = 2-4 days of oral abx
44
follow up after febrile UTI?
if <2yrs, KUB US to identify structural abN within 2 wks of UTI
VCUG in MALES or NCG in FEMALES:
- 2 or more febrile UTIs or,
- if US suggestive of VUR, renal anomalies or obstructive uropathy
nuclear cystogram (NCG) = less radiation but not good for post urethral valves (hence not for first imaging of males)
voiding cystourethrogram (VCUG) = best study for diagnosing VUR and urethral anatomy (eg PUV)
use NCG in place of VCUG in follow-up studies for both sexes.
45
what defines a "complicated" UTI?
Hemodynamically unstable
Elevated Cr
Bladder or abdominal mass
Poor urine flow
Not improving clinically within 24h of abx
46
management of complicated UTI?
Renal and bladder ultrasound (RBUS) to look for obstruction or abscess
IV abx (rather than oral) until child is clearly improving
47
3-year-old girl is seen for brown-coloured urine. She was treated with topical antibiotics for impetigo three weeks ago. She presents now with periorbital edema, bilateral crackles in the Basilar lung fields. HR 130, RR 30, BP 160 systolic. Urine analysis shows haematuria with red blood blood cell casts and 3+ protein. What lab finding would confirm the MOST likely diagnosis?
Antinuclear antibody
anti-DNase B
Serum IgA
Antineutrophil cytoplasmic antibodies
anti-DNase B (one of the tests for post-strep glomerulonephritis)
48
what is glomerulonephritis?
Gross hematuria (with red cell casts)
Edema
Hypertension (salt and water retention).
- Additional features: AKI (oliguria, uremia, elevated creatinine) and proteinuria
49
what is the most helpful investigation for distinguishing the etiology of glomerulonephritis?
C3
50
Key hematuria features of glomerulonephritis?
brown, tea-coloured, Coca-Cola coloured urine
red cell casts
51
General work up for glomerulonephritis?
extended lytes, CBC, Cr/urea, Albumin, C3, C4, ASOT
consider: ANA, ANCA, anti-dsDNA, Hep B/C, HIV
Kidney biopsy if proteinuria, AKI
52
what is the most common cause of glomerulonephritis?
post-infectious glomerulonephritis (post-strep GN)
53
what causes post-infectious glomerulonephritis?
Group A strep ("post-strep" GN) most commonly
(staph aureus, mycoplasma, viral/fungal/parasitic)
54
Timeline of post-strep glomerulonephritis?
1-2 weeks after strep throat/URTI
3-6 weeks after skin infection (eg impetigo)
55
Lab features of post infectious glomerulonephritis?
Low C3, normal C4
ASOT elevated (but not in skin infections)
Anti-DNAse B antibody (measures the amount of antibodies produced by the body in response to a group A streptococcus)
56
Treatment of post-strep glomerulonephritis?
supportive (1% require dialysis)
fluid and salt restriction to control BP and edema
treat strep infection (to prevent spread but will not help with nephritis)
57
Follow up and anticipatory guidance for post-strep GN?
*prove that is it post-infectious GN by repeating C3 in 8 weeks to see that it normalizes (now can be certain that post-infectious GN)
If not, consider renal biopsy for C3 glomerulopathy
gross hematuria can last 10 days (micro can last 1-2 yrs)
unlikely to recur
58
what is a red flag that presumed post-strep glomerulonephritis is the incorrect diagnosis?
if creatinine is rising rapidly this may be indicative of a rapidly progressive glomerulonephritis (Anti-GBM = crescentic GN)
--> requires renal biopsy
59
what are the type of glomerulonephritis that cause low C3?
Post-infection GN
C3 Glomerulopathy
Lupus Nephritis
60
what are the types of glomerulonephritis with normal C3?
IgA nephropathy
Thin-basement membrane disease
Anti-Glomerular Basement Membrane (GBM) disease (both isolated kidney and with pulmonary hemorrhage "Goodpasture syndrome")
Alport
IgA Vasculitis (HSP)
61
Epidemiological features of IgA Nephropathy?
>10yrs
M>F
Asian ethnicity most common
62
Presentation of IgA nephropathy?
asymptomatic microscopic hematuria
Gross hematuria DURING illness ("synpharyngitic")
- 1-2 day latency period (vs post-infectious strep where symptoms present 1-2 weeks after URTI)
+/- proteinuria
+/- elevated creatinine
Less likely to have edema or HTN (rest of GN triad)
63
pathophysiology of IgA nephropathy?
immune complex mediated
antibodies deposit into mesangium -> mesangial cell proliferation, pro-inflam cytokines and glomerular injury over time
64
Investigations in IgA nephropathy?
immunoglobulin A (IgA) only elevated in 10%
NORMAL complement
kidney biopsy
-will show glomerular IgA deposits along mesangial cells (will be the same findings as biopsy of IgA vasculitis aka HSP patients as they are on a continuum)
AKI, hematuria, proteinuria
65
Management of IgA nephropathy?
no treatment required unless:
ACEi/ARB if proteinuria or HTN
(steroids if nephrotic range proteinuria)
66
Prognosis of IgA nephropathy?
CKD/ESRD in 20-40%!
higher risk for CKD if proteinuria or hypertension, or scarring/crescents on kidney biopsy
67
A 7 year old boy presents with purpuric rash and bilateral knee swelling with severe abdominal pain. CBC and Creatinine normal. Urinalysis shows 1+ proteinuria and 5-10 RBC. What is next step in management:
A. Tylenol/NSAIDs and monitor
B. IVIG
C. Nephro consult
D. start steroids
68
What is IgA Vasculitis?
IgA Vasculitis (Henoch-Schönlein Purpura)
=small vessel vasculitis
69
Presentation of Henoch-Schönlein Purpura?
palpable purpura, abdominal pain, arthritis/arthralgia, hematuria, proteinuria
usually follows URTI
Memory aid HSP
H = Huge joint (arthritis)
S = Stomach hurts (intense colicky pain from intussusseption)
P = Purpura
70
Diagnosis of Henoch-Schönlein Purpura?
palpable purpura (on lower body/bum) + at least one of:
- abdo pain
- arthritis/arthralgia
- renal: hematuria or proteinuria
- biopsy showing IgA deposition (Renal Biopsy shows proliferative glomerular nephritis; Skin Biopsy shows Leukocytoclastic vasculitis )
71
Work up for HSP?
urinalysis (if normal, serial urinalysis)
biopsy (if renal involvement)
72
timeline of resolution of HSP?
resolves over weeks
rash appears in crops in gravity/pressure dependent areas (Eg lower body) - rash can take WEEKS to resolve and new crops are not concerning
must be monitored for renal invovlement with serial UA and BPs
73
What are complications and monitoring in HSP?
Renal disease (HTN; CKD) - serial urinalysis and BPs
- looking for hypertension or proteinuria
- UA/BP q1wk for 1st month,
- UA/BP q2wk for 2nd and 3rd month
- UA/BP once at 6mo and at 1 year mark
- annual review if renal involvement
Intussusception (intense, colicky abdominal pain; target sign on AUS)
74
treatment for HSP?
Supportive
NSAIDs for joint pain (NOT if renal invovlement)
Steroids if severe abdo/joint pain
(if renal disease, immunosuppressive agents such as cyclosporine, tacrolimus, mycophenolate)
75
Which patients with HSP need to be referred to nephrology?
Persistent proteinuria
HTN (confirmed)
Gross hematuria x 5 days
Complications of HSP:
HTN
Elevated Creatinine
Nephrotic syndrome: ++proteinuria, hypoalbuminemia, hyperlipidemia, edema
Acute Nephritic syndrome (AKI + GN triad (gross hematuria, HTN and edema))
76
patient with rising creatinine, proteinuria, gross hematuria as well as shortness of breath, cough, hemoptysis, pulmonary infiltrates on chest X-ray. What is the diagnosis?
Pulmonary Hemorrhage + Glomerulonephritis = Goodpasture Syndrome
GN is from Anti-GBM antibodies
Kidney biopsy shows crescentic glomerulonephritis
77
Treatment of Anti-GBM glomerulonephritis?
Plasmapheresis +steroids or immunosuppresion in rapidly progressive GN or pulmonary hemorrhage (Goodpasture dz)
78
Epidemiology in lupus nephritis?
F>M in adolescence, in childhood equal F:M
79
Features of lupus nephritis? (8 categories)
Glomerulonephritis (hematuria with red cell casts, edema and HTN), proteinuria, AKI
Joint pain/arthritis
Serositis (hepatitis, pleuritis, carditis)
Mucous membrane ulceration
Rash: malar or discoid, photosensitivity
Fatigue, fever, weight loss
CNS: seizures, psychosis
Cytopenias
80
Diagnosis of lupus nephritis?
kidney biopsy!
(send sample for immunofluorescence, wire loop deposits)
ANA, anti-dsDNA, anti-Sm, anti-phospholipid Ab
LOW C3 and C4
81
Treatment of lupus nephritis?
Initial therapy:
○ Mycophenolate mofetil or Cyclophosphamide
○ High dose steroids with a taper
Maintenance therapy:
○ Mycophenolate mofetil or azathioprine
○ Steroids
○ ACE inhibitors
○ Hydroxychloroquine for relapse prevention and to manage skin manifestations
○ Sunlight: Avoidance of sun exposure as can trigger relapse
82
What type of glomerulonephritis is presents as "rapidly progressive"?
ANCA-associated vasculitides
Includes granulomatosis with polyangiitis (GPA) (previously known as Wegener’s), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (eGPA)– previously known as Churg-Strauss
83
3 week old with fever and poor feeding presenting to ED with positive urinalysis, started on cefixime. Improving after 24 hrs, culture pre abx positive 10^8 CFU for E.coli resistant to TMP-SMX, cephalosporins and sensitive to gent, ciprofloxacin. What will you do next?
A) Repeat UA and UCx
B) Change to nitrofurantoin
C) Change to IV gent
D) Change to po ciprofloxacin
Change to IV gent
cipro cannot be used in infants
84
6-year old female with persistent microscopic hematuria (20-40 RBC/hpf) without proteinuria. On family history, father has hearing loss and history of kidney transplant secondary to renal failure. Her physical examination, including blood pressure, is normal. What is the inheritance pattern on the underlying condition?
Autosomal dominant
X-linked recessive
Autosomal recessive
Sporadic
X linked recessive (answer per Dr. Ding)
Females get x-linked Alport’s but phenotype is much milder than boys. this pt only has microscopic hematuria
85
What is Alport's?
disease of collagen type IV that effects the basement membrane of the glomerular capillary as well as causes:
sensorineural hearing loss
Eye problems
Platelet defects
86
features of Alport's?
think eyes, ears, kidney
Anterior lenticonus or other ocular problems
Sensorineural hearing loss (early onset deafness)
Hematuria (classically episodes of gross hematuria on a background of microscopic hematuria)
Glomerulonephritis, proteinuria, CKD
100% ESRD in males with X-linked
87
genetics of Alport's
85% are X-linked COL4A5 mutations
15% are autosomal recessive
88
treatment of Alport's?
ACEi
89
4 year old with one week of periorbital and peripheral edema. Has 4+ protein on urinalysis and albumin at 14.
What other lab finding are they likely to have?
Elevated antithrombin III
Low C3
High total cholesterol
Low C4
High total cholesterol
this pt has nephrotic syndrome which is tetrad of nephrotic range proteinuira, hypoalbuminemia, hyperlipidemia and edema
they actually lose antithrombin III (natural anticoagulant) in urine which is only of the reasons they are hypercoagulable
90
Six-year-old boy with a history of well-controlled, chronic kidney disease who has short stature and slow growth velocity. What is the reason for his poor growth?
Growth hormone insensitivity
CKD related osteodystrophy
Chronic metabolic acidosis.
Poor nutrition
CKD related osteodystrophy
poor linear growth suggest bone/mineral disorder.
Poor nutrition and chronic metabolic acidosis also contribute to poor growth
91
Lab findings of CKD-related osteodystrophy?
Hyperphosphatemia (kidneys not excreting phos)
Hypocalcemia (kidneys not turning Vit D to active calcitriol, thus decreased calcium absorption)
Hyperparathyroidism (PTH causes Ca and PO4 release from bones, compensates until ESRD)
High alkaline phosphatase (ALP)
92
Presentation of CKD-related osteodystrophy?
similar to Vit D deficiency (Rickets)
- poor growth, short stature
- frontal bossing
- rachitic rosary
- weak
- bowed legs
93
4 year old with one week of periorbital and peripheral edema. Has 4+ protein on urinalysis and albumin at 14.
What other lab finding are they likely to have?
Elevated antithrombin III
Low C3
High total cholesterol
Low C4
High total cholesterol
part of the tetrad of nephrotic syndrome
94
A three-year-old girl is seen for brown coloured urine. She had previously been treated with topical antibiotics for impetigo three weeks ago. She presents now with periorbital edema, bilateral crackles at the lung bases. HR 130, RR 30, BP 160 systolic. Urine analysis shows haematuria with red blood blood cell casts and 3+ protein. There is a systolic murmur as well. What is the best next step in management?
Restrict, sodium and fluid
IV antibiotics
IV Furosemide
Iv enalapril
IV Furosemide
first step in management in post-infectious GN is sodium and fluid restriction however this patient is overloaded (HTN) so need IV furosemide
95
5 yo boy, 12h post tonsillectomy develops confusion, nausea, and seizures. Na 121, serum osmolality 260. What is most consistent with his diagnosis?
Urine Na <20
Urine osmolality < serum osmolality
Excess urine output
Urine osmolality > serum osmolality
96
You are seeing a new referral, 12 year old girl with known congenital solitary kidney with compensatory hydronephrosis. Her UA is normal. Exam otherwise normal. What is the most appropriate investigation
No further investigation needed
eGFR
Repeat Urinalysis
VCUG
97
Baby has history of antenatal hydronephrosis on prenatal imaging. Post natal imaging at 2 weeks shows persistent unilateral pelviectasis with measurement of APD 5 mm. Next best step?
Repeat US in 6 months
MAG3
VCUG
Repeat US in 6 months
right side of chart
98
You have a patient who is 8 years old who had HUS at 2 year old. She needed a transfusion then, but no renal support was needed. Recovered completely. Now she’s due for an annual well child check up. What investigation should you do?
UA
Serum Creatinine
Nothing
CBC
UA
Annual BP and UA for declining kidney function, proteinuria and hypertension
99
How does STEC-HUS present?
Diarrhea progressing to BLOODY diarrhea
triad:
1. microangiopathic hemolytic anemia (MAHA)
2. thrombocytopenia (rapidly progressive *cardinal feature*)
3. AKI (ranges all the way to anuria and dialysis)
100
Exposure leading to Shiga-toxin Hemolytic Uremic Syndrome (HUS)?
*undercooked meat*
contaminated apple juices, spinach and various farm products
101
pathophysiology of Hemolytic Uremic Syndrome (HUS)?
clots in the blood vessels leading to the kidney
RBCs get sheared by the clots --> hemolytic anemia
AKI from clots limiting renal perfusion --> elevated Cr/urea --> uremia
102
What causes hemolytic uremic syndrome?
Shiga-toxin producing E coli (STEC) most commonly!
103
Lab features of STEC-HUS?
hemolysis: anemia, high LDH, low haptoglobin, high bili
thrombocytopenia: low platelets
AKI: high Cr/urea
UA: may have hematuria, proteinuria
104
treatment of HUS?
hydrate 1x maintenance fluids D5NS (no K)
replace diarrheal output 1:1 with bicarb half normal saline
essential to maintain blood flow to kidneys and keep them voiding
q12h labs: CBC, lytes, Cr/urea, LDH, type and screen up to date
105
medications we avoid in HUS?
no NSAIDs in renal injury
no antibiotics without ID involvement, could make STEC worse
106
complications of HUS?
*Dialysis (up to 50% require dialysis)
*ischemic stroke*
*hypertensive encephalitis*
*pRBC transfusion requirement (never transfuse plt unless intracranial bleed or other severe bleed)
bacteremia (e coli translocate through leaky gut to bloodstream)
cardiomyopathy
107
prognosis and monitoring in HUS?
5% remain dependent on dialysis
50% will be left with some degree of kidney damage
yearly BP and UA to assess for HTN and proteinuria
108
3y boy with fever and dysuria. No vomiting, no abdominal pain, no diarrhea. Past medical history significant for obstructive uropathy and recurrent UTIs. Looks unwell. VBG gas pH 7.26 CO2 30 bicarb 13.6, Na 138 K 3.2 Cl 116, Cr 30. What is diagnosis?
Starvation ketosis
Addison's disease
Distal renal tubular acidosis
Acute kidney injury
Distal renal tubular acidosis
Metabolic acidosis (pH 7.26, bicarb 13), mild hypokalemia, normal Na
AG = Na - (HCO3 + Cl) = 8 NAGMA
non-anion gap met acidosis: can only be kidney or GI (no diarrhea)
RTA with hypokalemia
109
what is renal tubular acidosis?
kidney unable to either
- resorb bicarbonate or
-excrete ammonia
Think of RTA with normal anion gap metabolic acidosis and no diarrhea
Typically patients also have high chloride
110
You have identified metabolic acidosis on a gas (low pH, low bicarb, CO2 low trying to compensate). how do you calculate the anion gap?
Anion Gap: Na - (Cl + HCO3)
Normal is 10 +/- 2
111
what are the causes of non-anion gap metabolic acidosis? how do you sort out which cause it is?
Renal or GI losses of bicarb
Urine Anion Gap = (Na + K) - Cl
Used to determine if renal or GI etiology of non-anion gap met acidosis
If urine anion gap neGUTive, this represents HIGH ammonium, appropriate kidney compensation, and usually GI losses of bicarb
no change or high UAG = renal
112
Interpret the urine anion gap to determine the etiology of non-anion gap metabolic acidosis?
If urine anion gap neGUTive, this represents HIGH ammonium, appropriate kidney compensation, and usually GI losses of bicarb
no change in UAG = renal loss of bicarb (proximal RTA)
positive (high) UAG = impaired renal excretion of H+ (distal RTA and type 4 hyperkalemic RTA)
113
Interpret the urine pH to determine the etiology of non-anion gap metabolic acidosis?
* Urine pH >5.5 – distal RTA
* Urine pH <5.5 + low K – proximal RTA
* Urine pH <5.5 + HIGH K – hyperkalemic RTA (Type IV), i.e. low aldosterone, aldosterone insensitivity, renal failure or obstructive uropathy
** in proximal RTA, when bicarb is given there is no longer a steady state and there will be more bicarb loss - thus urine pH will increase
114
What is proximal RTA?
Proximal (Type II) RTA is impaired bicarb resorption
memory aid: BI-carb is like 2, thus type 2
bicarb wasting = met acidosis
to balance out acidosis (H+), body eliminate K+ to maintain electoneutrality --> HYPOkalemia
urine pH < 5.5 since lots of H+ still in urine. in Type II they are acidotic bc of bicarb wasting in the urine
115
What is distal renal tubular acidosis?
Distal RTA (Type I) = met acidosis, hypokalemia, hypercalciuria, urine pH >5.5
kidney normally excretes acid by forming ammonium in the distal tubule
distal tubule dyfxn = unable to excrete hydrogen --> metabolic acidosis
holding onto H+ in the blood, so kidney eliminate K+ and Ca+ to maintain electroneutrality --> HYPOkalemia in the serum and hypercalciuria (more calcium excreted in the urine, but bone release maintains serum ca+ levels)
urine is more alkalotic since not much H+ going into urine (pH >5.5)
memory aid: 1 H+ ion thus problem is with excreting acid in type 1
Also more likely to have nephrocalcinosis / stones
Causes:
- Drugs (amphotericin, lithium)
- Autoimmune (Sjogrens, SLE, PBC)
- SCD
- Obstructive uropathy
- primary (defective pumps/channels/exchangers)
116
what is type IV RTA?
Aldosterone related
- can be aldosterone deficiency
- aldosterone resistance
- pseudohypoaldosteronism
- hyporeninemic hypoaldosteronism
- medications
Causes:
Hyperkalemic metabolic acidosis
recall aldos is for incr BP (thus increases Na resorption and K excretion)
NO aldosterone = NO K excretion -->HYPERkalemic
117
Causes of type IV RTA?
Causes of Hypoaldosteronism (Type 4)
1. Adrenal Gland Disorders
a. Congenital Adrenal Hyperplasia (CAH)
b. Addison's Disease
2. Secondary Causes
a. Obstructive Uropathy (ex. PUV)
b. Pyelonephritis
c. Meds: Septra, ACEi, Cyclosporine, Prolonged heparin, Spironolactone
118
Define and list causes of anion gap metabolic acidosis?
Non-Anion Gap Metabolic acidosis means anion gap (Na - (Cl + HCO3) is around 10 +/- 2
Causes: "KULTIS:
-Ketosis
-Uremia
-Lactic acidosis
-Toxic alcohols (ethanol, methanol, propylene glycol)
- Iron
- Salicylates
119
8 year old girl had a femur fracture. Post-op, admitted to hospital. On clear fluids, PO Morphine and was started on IV fluids D5 ½ NS 65ml/hr (weight = 25kg).
Developed irritability and new onset headaches.
Labs showed Na 128.
What are the most likely findings on further investigations?
A- Low urine osmolality, high serum osmolality
B- High urine osmolality, high serum osmolality
C- Low urine osmolality, low serum osmolality
D- High urine osmolality, low serum osmolality
Low urine osmolality, low serum osmolality
Low serum osm means true hyponatremia
Low urine osm means peeing out excess water
thus problem is excessive intake of free water
HypoNa Cheat Sheet
Urine osm <100 = excessive intake free water
Urine Na <20 = hypovolemic
Urine Na >20 = SIADH
120
Girl with HSP, hand swelling, and mild proteinuria. What is your management
UA weekly until the rash resolves then once monthly x 6 month
Refer to nephrology (consensus)
Start steroids
UA weekly until rash and proteinuria resolve
UA weekly until rash and proteinuria resolve
Looking for Renal disease (HTN; CKD) in HSP pts - serial urinalysis and BPs (sample protocol)
- looking for hypertension or proteinuria
- UA/BP q1wk for 1st month,
- UA/BP q2wk for 2nd month
- UA/BP once at 6mo and at 1 year mark
- annual review if renal involvement
121
You are seeing a 16yo boy for 2 week history of intermittent cough, knee, and ankle pain, with 2d history of hematuria. Exam shows small bilateral knee effusion. Urinalysis has 3+ blood and protein. Blood work shows Hb 110, WBC 12.3, platelets 210, albumin 32, creatinine 160, C3 and C4 low. What is the most likely diagnosis?
Systemic lupus erythematosus
IgA nephropathy
Post infectious glomerulonephritis
Henoch Schoenlein Purpura
SLE- giveaway is the low C3/C4, systemic features
IgA - normal complement
HSP - 90% <10yrs, also has normal complement
PSGN - normal C4
122
5-year-old with 2 day history of tea-colored urine and foot/leg swelling. Normal vitals, except BP around 125/60. Two weeks ago had pharyngitis. What is the most likely lab finding?
Normal Creat
Low serum albumin 25
Low C3
Urine proteinuria 4g/L
Low C3
(key feature of post-strep GN)
can get AKI so normal cr is wrong
+++proteinuria is more feature of nephrotic syndrome
123
10 year old female with new-onset tea-coloured urine. Her physical exam is normal. She has a normal C3 and C4. What is the most likely diagnosis?
a) Post-streptococcal glomerulonephritis
b) Membranoproliferative glomerulonephritis
c) IgA nephropathy
d) Systemic Lupus Erythematosus
IgA nephropathy (normal complement)
124
4-year-old girl with 3 days of abdominal pain, cough, and low-grade fever. She is diagnosed with a viral URTI. At the time of diagnosis, she has a urinalysis with 1 g/L protein (2+ protein). There are no WBCs, leukocytes, or RBCs. What is your next step in management?
a) Repeat urinalysis in 1 week
b) Reassure and no further treatment
c) Treat with oral cefixime
d) Abdominal ultrasound
Repeat urinalysis in 1 week - need to PROVE that it resolves to make diagnosis of transient proteinuria
125
Described a child who developed hematuria several weeks after a nonspecific respiratory illness. What lab test result would be most likely with your expected diagnosis?
Increased creatinine
Low albumin
Low C3
low C3
126
6-year-old girl, has been toilet trained for the past 2 years, presents with 2 episodes of UTI in the past 6 months. What is the most likely cause?
Vesicoureteral reflux
Constipation
Infrequent voiding
Inadequate fluid intake
Constipation
127
Contraindication to circumcision:
A) VUR
B) bilateral cryptorchidism
C) penoscrotal hypospadias
D) family history of bleeding
hypospadias
128
A previously well 3 year old boy presents with one week of progressive edema. His BP is 125/80. There is moderate ascites and significant periorbital edema. Urinalysis shows 3+ protein and no blood. Labs show Na 126, K 4.4, Cl 106, albumin 19. CBC and Creatinine are normal. Besides starting prednisone 2mg/kg, what medication is the most appropriate to start?
Nifedipine
3rd generation cephalosporin
Albumin
Furosemide
Furosemide (Safest choice, hypertensive and overloaded)
WRONG ANSWERS:
Nifedipine - treat the hypertension by treating the overload with the diuretic (lasix)
Cephalosporin - ?Can prophylax spontaneous bacterial peritonitis
Albumin - he is just going to continue to pee out the protein, indication for albumin is anasarca and pre-renal AKI
129
Hydronephrosis diagnosed prenatally, postnatal scan shows widespread dilation of the calyces with an APD of 17mm. What is the next imaging test?
Intravenous pyelogram
VCUG
Mag3 renal scan
VCUG
APD >17mm means referral to urology then VCUG
130
School aged child with mildly elevated creatinine and hypertension. u/a has blood and 2+ protein. C3, c4 and CBC are normal. What is most likely diagnosis?
IgA Nephropathy
Post streptococcal glomerulonephritis
Lupus
Membranoproliferative glomerulonephritis
IgA Nephropathy
normal complement, school age kids
131
A 7 year old boy presents with pretibial purpuric rash and left ankle swelling. CBC and Creatinine normal. Urinalysis shows 2+ proteinuria and 5-10 RBC. What is next step in management:
Renal ultrasound
UA in one week
nephro consult
start steroids
UA in one week
The most important laboratory test to obtain with HSP is the urinalysis
Because renal disease is the leading cause of morbidity in HSP, following up serial urinalyses for urine protein-creatinine ratios (if urine protein is detected) is warranted for at least 6-12 months after diagnosis, with weekly monitoring during first month and biweekly in 2nd month after initial presentation.
Most often HSP is a self-limiting illness, with resolution of symptoms within 4 to 6 weeks of onset.
One-third of patients experience a recurrence or relapse, usually within 1 year of initial presentation. However, long-term follow-up is important because complications such as hypertension and chronic kidney disease have been observed up to 10 years after an HSP episode.
132
12yo boy, with normal renal function, malaise and burgundy urine. FhX of hyperchol, for which he is on prasuvastatin. Normotensive, mild edema. No Abdo pain.
UA red, ph 6 SG 1.035, protein >5g/L, Leuk esterase: negative (0-2 WBC), RBC: 4+ (5-10/hpf). No casts. Cr 160 (baseline 53)
Rhabdo
RVT
ATN
Renal Vein Thrombosis
Although statins can cause rhabdomyolysis, the presentation would be positive urine dip but <5 RBC per HPF on urinalysis microscopy
ATN: typically show muddy brown casts
133
4yo girl post strepglomerular neprhritis 4 days ago. Cr up (44→87), normal lytes, no headache. 1Kg weight gain since diagnosis. Blood pressure elevated 120/88. Sacral edema and perioorbital edema. What do you do NEXT?
Ace inhibitor
Lasix
Spironolactone
Calcium channel blocker
Lasix
treatment for PSGN:
Fluid & salt restriction to control blood pressure and edema, +/- furosemide for hypertension management and other anti-hypertensive medication can be considered
134
Baby with e.coli uti. Has an abdomen mass. Minimal urine output. Best test to make the diagnosis?
Ultrasound
Vcug
CT
Suprapubic aspirate
Ultrasound - need to differentiate mass type
if this was hydronephrosis, would do VCUG to assess degree of hydronephrosis after
135
3 year old presenting with periorbital edema. Normotensive, minimal abdominal distension. Urinalysis shows 3+ protein, remainder of urinalysis unremarkable. What complication is the child most likely to experience?
DVT
Acute renal failure
CHF
Encephalopathy
DVT
thrombosis is a complication of nephrotic syndrome
136
12yo boy has a pre-camp physical examination which was normal. Urinalysis showed a specific gravity of 1.010, protein 2+, negative for leukocytes, blood, or nitrites. What is the best next step?
Urine culture
24hr protein
Urine ACR
Morning urinalysis
Morning urinalysis
healthy adolescent male with orthostatic proteinuria
137
7 year old with 4 days of right scrotal pain. The scrotum is swollen and teste is slightly tender. There is a 5 mm tender firm mass in the upper region. What do you do?
NSAID
Scrotal scintigraphy
Antibiotics
Consult surgery
NSAIDs
mass indicates this is torsion of the APPENDIX testes, for which NSAIDS are the tx.
Usually get an US with Doppler first to distinguish from testicular torsion
parking lot: ?consult surgery to distinguish from torsion as safest option
138
Child with CP, g-tube feeds and reflux undergoes a fundoplication. He resumes the same feeds 1000ml via Gtube that he was on previously plus 500cc of water. Two weeks after his surgery he experiences irritability, and retching. Na 129, Cr 35, serum osmol 263 (low). Urine Na 147 and urine osm 1043
Which of the following explains his hyponatremia?
A. Cerebral Salt wasting
B. Excess free water
C. Inadequate salt intake
D. SIADH
SIADH
HypoNa Cheat Sheet
Urine osm <100 = excessive intake free water
Urine Na <20 = hypovolemic
Urine Na >20 = SIADH
139
What findings would you expect in a distal [type one] RTA:
A. Glycosuria
B. Hypercalciuria
C. Hyperkalaemia
D. Elevated urine anion gap
Hypercalciuria
Elevated urine anion gap could also be true
- in distal RTA there is a positive urine net charge (so positive anion gap) due to LOW excretion of NH4CL
Negative UAG → High NH₄⁺ excretion → Kidneys are appropriately responding to acidosis.
Positive UAG → Low NH₄⁺ excretion → Kidneys are not excreting enough acid
140
5 yo girl with 2 episodes of difficult urination. Otherwise well. On exam, she has a red donut shaped protrusion from her urethra. There are no bruises, abrasions or lacerations. What is the diagnosis?
1. Sarcoma
2. Urethral prolapse
3. Foreign body
4. Sexual abuse
Urethral prolapse (donut shaped)
141
5 year old girl diagnosed with nephrotic syndrome. Started on prednisone 2mg/kg/day. 2 days later she develops right leg pain. You note ascites and periorbital edema on exam. She has swelling to both legs but right much more than left. No redness, no fever. Systolic blood pressure 105. Investigations show urine positive for 3+ protein and serum albumin 19. Most appropriate treatment?
A. Furosemide
B. Albumin
C. Third generation cephalosporin
D. Unfractionated heparin
Unfractionated heparin for thrombosis (complication of nephrotic syndrome)
142
Child was seen for bilateral cryptorchidism and on examination found to have abdominal wall laxity as well as bilateral abdominal masses. What is the most likely etiology of the masses:
Hydronephrosis
Wilms tumor
Multicystic dysplastic kidneys
Polycystic kidneys
Hydronephrosis (seen in Prune Belly Syndrome)
143
what is prune belly syndrome?
Triad:
Abdominal muscle laxity from deficient musculature
Urinary tract dilation
Bilateral cryptorchidism
144
Tx of prune belly syndrome?
UTI prophylaxis
Potential surgical intervention to facilitate adequate bladder emptying
Orchiopexies for cryptorchidism
Delayed abdominal wall reconstruction for cosmesis
145
prognosis of prune belly?
ESRD will occur in ⅓ of patients
146
Most common pathogen in Spontaneous Bacterial Peritonitis?
Strep pneumo
147
13 year old boy with a blood pressure of 130s/80s (average of 3 measurements taken at one visit) you are provided with charts and determine this is between 95% and 95%+12 mmHg. What do you do?
Repeat measurements at 3 more visits
ABPM
Urinalysis, Creatinine, lytes, EKG, renal ultrasound
Urinalysis, Creatinine, lytes.
Repeat measurement at 3 more visits
148
Statin- brown urine with hemoglobin and protein .- rhabdo or ATN
Statin-Induced Rhabdomyolysis With Myoglobinuria
149
Teen with Testicular mass on L, irregular, better appreciated when standing
Varicocele
spermatocele
Inguinal hernia
Varicocele - testicular mass on the LEFT in teenage boy, that is more prominent with standing/valsalva, “irregular” may indicate "bag of worms" palpation that is characteristic
150
Testicular pain in 10yo male, ultrasound with doppler shows increased flow and enlargement of the epididymis. Has blue dot on exam
Oral antibiotics and urine culture
NSAIDs and bed rest
NSAIDs and bed rest
blue dot sign from torsion of the APPENDIX testis (diff than testicular torsion)
151
Hypoalbuminemia - no urine protein. What is next best test to do?
alpha-1 antitrypsin in stool
Serum trypsinogen
Echo
Urine Protein:Creatinine ratio
alpha-1 antitrypsin in stool - useful test to assess for protein losing enteropathy (malabsorptive process)
152
Below what systolic blood pressure would a 3yo be considered hypotensive?
64
70
76
84
70 + 2x age = 76
153
12 year old girl with 3 weeks of fever, malaise, and polyarthralgias. No arthritis. Protein and blood in her urine, normal creatinine. Leukopenia, lymphopenia, normal platelets. ESR 75. What’s the most likely diagnosis?
Systemic JIA
SLE
Rheumatic fever
Post strep GN
SLE (Lupus Nephritis)
no major criteria for rheumatic fever
systemic JIA would have arthritis
154
Kid with nephrotic syndrome started on steroids. Presents with abdominal pain, distended abdomen with dullness over the flanks bilaterally, peritoneal signs. Which test will give you the diagnosis?
CT abdomen
Paracentesis and culture
Abdominal U/S
Surgical exploration? Or ?laparotomy
Paracentesis and culture
for Spontaneous Bacterial Peritonitis
155
Child presents with brown urine. UA with hematuria and proteinuria. Hypertensive to sBP 160. C3 and C4 are both normal. What is likely diagnosis?
IgA nephropathy
MPGN
PSGN
SLE
IgA Nephropathy
156
Kid nephrotic syndrome. Most likely consequence?
-DVT
-encephalopathy
-acute renal failure
-heart failure
DVT
157
15yo M with fever x 24 hours, one sided scrotal pain and swelling, with dysuria and pyuria. Urinalysis shows WBC- best management?
Refer to urology
Treat him with antibiotics
Treat him and sexual partners with antibiotics
Treat him and sexual partners with antibiotics
A teen with epididymitis is typically due to STI causative organism, therefore you must treat both the teen and their partner
158
Antenatal hydronephrosis, postnatal ultrasound at 3w shows one sided mod-severe hydronephrosis, no mentioned urological abn. Baby looks well and peeing well. Next step?
Urology consult before discharge
VCUG
DMSA
reassure
Urology consult before discharge (based on chart)
(will need VCUG)
159
Undescended testes at 8 month, what to do next
Refer to surgery
ultrasound
Refer to surgery
160
Kid with hematuria - no mention of viral illness - C3 normal
Alports
MPGN
PIGN
Alports
(C3 and PIGN have abN C3)
161
Below what systolic blood pressure would a 3yo be considered hypotensive?
64
70
76
84
76
70 + (age x 2) = 5%ile for age
162
What is important to know in order to determine if BP is within normal range?
a) Weight
b) Ethnicity
c) Height
d) Age
Height
163
16 year old girl comes to your office. Her BMI is 27 and she has stage 1 hypertension. No protein present on urinalysis. What is the next step in managing her high blood pressure?
1) Beta blocker
2) Calcium channel blocker
3) Lifestyle
4) ACEi
Lifestyle
confusing bc you need to do lifestyle interventions in order to make dx for stage 1
overall tx for stage 1 is lifestyle
164
Teen girl with hypertension and BMI 30. What is best way to determine long term damage from the HTN?
A. ECG
B. Echo
C. Serum creatinine
D. Fundoscopy
ECHO
looking for LVH (complication of HTN)
fundoscopy would identify retinopathy (complication of HTN), but LVH is more common
165
A child with grade IV unilateral VUR presents with second UTI. Most recently she had a pyelonephritis that was cultured and resistant to TMP-SMX and nitrofurantoin. You are thinking of starting prophylaxis. What do you start?
a) Cefixime
b) TMP-SMX
c) Ciprofloxacin
d) No prophylaxis indicated
Cefixime
stop the abx if they culture bugs resistant to those bugs
166
Most common cause of hypertension in a newborn?
a. Hydronephrosis
b. Coarctation of aorta
c. Renovascular
d. CAH
Renovascular
Most common cause of secondary hypertension is renal parenchymal and renovascular (parenchymal is most common) (34-79%)
HTN Causes:
Neonate:
Renovascular: RA thrombus, RA stenosis, RV thrombus, CAKUT, acquired renal disease; Coarct; Meds
Infant: Renal parenchyma, renovascular, coarct, CLD (BPD)
1-6y: same + essential, endocrine
6-12y: renal parenchymal, renovascular, essential, coarct, endocrine, iatrogenic, OSA
12-18y: essential, renal parenchymal, drugs (steroids, OCP), renovascular, endocrine, OSA, pregnancy
167
10 year old boy presents with 12 hours of scrotal pain. He has focal tenderness at the upper pole of the testis with a focal blue discolouration, and there is some edema. What is the best next step?
a. Ultrasound
b. Analgesia and scrotal support
C. Urology consult
Doppler US may be useful to distinguish between testicular torsion and appendix torsion
blue dot sign = appendix torsion
168
13 year old girl presents to the ED with a generalized tonic clonic seizure. Her parents say she’s been drinking a lot of water recently. Her labs: Na 118, Cl 86, osm 262, Urine Na 20, serum urine osm 68. What’s the MOST likely diagnosis?
a. T1DM
b. Psychogenic polydipsia
c. SIADH
d. Adrenal insufficiency
Psychogenic polydipsia
HypoNa Cheat Sheet
Urine osm <100 = excessive intake free water
Urine Na <20 = hypovolemic
Urine Na >20 = SIADH
169
4 month girl with FTT.
Gas: pH 7.24, CO2 30, HCO3 16
BW: Na 138, Cl 111, K 5, PO4 2.1, glucose 4
What’s the most likely diagnosis?
a. Fanconi syndrome
b. RTA
c. Mitochondrial disorder
d. CF
RTA
this is Type IV given HYPERkalemia
170
Children with Turners have which renal abnormality at rates higher than healthy population?
A. horseshoe kidney
B. MCDK
C. Vesicoureteral reflux
Horseshoe kidney
Horseshoe kidneys occur in about 7% of Turners patients and 30% will have renal anomalies
171
Kid with Burkitt’s Lymphoma and TLS. Urine pH 7.0. What’s next.
A. Hemodialysis.
B. Potassium
C. ACE inhibitor
D. Rasbiricase
rasburicase
=enzyme that degrades uric acid. Used in patients with established TLS with high uric acid levels or those at high risk for TLS (Burkitt's is high risk)
172
Which of the following is seen in distal RTA?
a. Hyperkalemia
b. Hyponatremia
c. Hypophosphatemia
d. Hypercalciuria
Hypercalciuria
(K and Ca are excreted in the urine to try to compensate for the electronegative gradient caused by inability to excrete daily acid load)
you would see HYPOkalemia
173
7yo M with recent URTI who presents with respiratory distress and BP 150/110. Most likely diagnosis?
a. anxiety
b. pneumonia
c. Henoch-schonlein purpura
d. post strep GN
post strep GN - most likely given PRIOR URTI and new hypertension with respiratory distress (fluid overload causing pulmonary edema vs effusions)
174
11y old presents with a 4 day hx of tea colored urine .He had an upper respiratory tract infection 10 days ago. There is no hx of urgency or dysuria. Normal PE. Blood pressure 110/75. Urine analysis: red blood cells, 0-2 WBC and 1+ protein.
a. cystitis
b. Hypercalcuria
c. IGA nephropathy
d. Post infectious GN
Post infectious GN - parapharyngitic with URTI 10d ago
175
Most common renal stone in children
a) Calcium oxalate
b) Cystine
c) Urate
d) Struvite
Calcium Oxalate: most common stone type, due to inadequate calcium and fluid intake
Struvite Stones: associated with UTI
Uric Acid Stone: can be secondary to genetic cause or from diet (organ meats and shellfish)
Cysteine Stone: rare inherited genetic condition increasing the amount of cysteine in the urine
176
4 year old girl with glomerulonephritis, hypertension, and vomiting x3 days. Most probable test to determine diagnosis
a) C3
b) Renal Biopsy
C3
177
A child has constipation, vomiting and cause of low Glc, Low K, Low Na, high Cl non anion gap Met acidosis. No FTT. Urine pH 7.5.
a) CF
b) RTA
distal RTA
* Urine pH >5.5 – think about distal RTA
* Urine pH <5.5 + Low K – think about proximal RTA
* Urine pH <5.5 + High K – think about hyperkalemic RTA (Type IV), i.e. low aldosterone, aldosterone insensitivity, renal failure or obstructive uropathy
178
Kid with elevated BP confirmed by ambulatory monitoring. what’s next?
a) Renal US
b) Treat with nifedipine
c) Recheck in a month
d) Ambulatory monitoring
Renal US - part of the list of investigations we do for pts with confirmed hypertension
179
Child with distal RTA who doesn’t take his meds. 2 months after last visit, presents with bilateral leg weakness and doesn’t want to walk. What does he have?
a. Chronic acidosis
b. Hypokalemia
c. Hypocalcemia
d. Hypomagnesemia
Hypokalemia - can be associated with periodic limb weakness
180
7 y.o boy presents with left-sided night time flank pain. An US reveals left-sided hydronephrosis. what do you do next?
a. VCUG
b. Nuclear scan with lasix washout
c. DMSA scan
d. CT abdomen with contrast
VCUG
181
School aged child with pH 7.15, HCO3 9, Na 138, Cl 121, PO4 0.7, K 3.0. Also has urine pH of 5 and glucose in urine. What do you check? Which test is most likely to reveal the diagnosis?
a. Leukocyte for cystine
b. Urine ca/cr ratio
Leukocyte for cystine
Cystinosis (most common cause of Fanconi syndrome, RTA)
AR lysosomal storage d/o
Cystine accumulates in the lysosome
Low phosphate, low uric acid, low glucose, type 2 RTA, poor growth, FTT, hypophosphatemic rickets, deposits in the eye (keratopathy and photophobia), benign IIH, hypoTSH, hepatomegaly, polyuria/polydipsia, muscle weakness, normal cognition
Diagnosis: WBC cysteine level
Tx: fluid & electrolyte supplementation and cysteamine
182
(photo of) labial adhesions in an 18mo girl. What to do?
a. Estrogen cream to the affected area
b. Abdo US
c. Call CAS
d. Refer to urology
Estrogen cream to the affected area BID
Asymptomatic—no treatment is necessary if adhesion only involves small portion of labia and does not affect urine stream; adhesion may resolve when estrogen production increases at puberty
Symptomatic—adhesions should be treated if they affect urination, are associated with recurrent infections, or symptomatic
183
6 yr American African girl presented with blood in underwear. She has some trouble urinating, no fever, no other symptoms. On exam there is a red mass coming out of vagina..
a. Urethral prolapse
b. Cancer
Cancer
this is Sarcoma Botryoides (highly malignant rhabdomyosarcoma)
- mass that protrudes from vaginal, "bunch of grapes"
- 2-6 yr olds
- higher incidence of rhabdosarcomas in black patients
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Patient presents with edema, abdominal distension, and proteinuria. Patient also has fever and is found to have spontaneous bacterial peritonitis. What is the MOST likely pathogen causing the SBP.
a. Steptococcal pneumoniae
b. E. Coli
c. Enterococcus
d. Listeria
strep pneumo
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Normal range of proteinuria for >24 mo?
<150mg in 24 hour sample
PCR <20mg/mmol in first morning urine
ACR <3mg/mmol in first morning urine
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what defines nephrotic range proteinuria?
PCR >200mg/mmol
PCR >40mg/m2/hr (>3g/1.73m2/day) in 24 hr sample
1.73m^2 is average body surface area for an adult
187
How does torsion of the appendix testes present?
tender mass on upper pole of testes
blue dot sign (can see the mass through scrotum)
testicular pain and scrotal swelling
2-10yrs old
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investigation of torsion of the appendix testes present?
Doppler US will show increased flow and enlargement of the epididymis
Used to distinguish from testicular torsion
189
treatment of torsion of the appendix testes present?
NSAIDs x 5 d
Best rest 24hr
inflam resolves in 3-10 days
190
presentation of testicular torsion?
acute pain and swelling of scrotum
ABSENT cremasteric reflex (elevation of the testis in response to stroking of the upper inner thigh)
“high-riding, transverse lie” testis in scrotum
Nausea and Vomiting
>12yrs
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treatment of testicular torsion?
manual detorsion within 4-6 hours
do NOT delay for ultrasound
prompt surgical exploration and detorsion
scrotal orchidopexy on BOTH sides to prevent torsion of testes in the future
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typical age presentation of testicular torsion, torsion of appendix testis and epididymitis?
testicular torsion >12yrs
appendix torsion 2-10yrs
epididymitis <2yr or >12yrs
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features distinguishing epididymitis from torsion?
gradual onset of pain (torsion is acute onset)
dysuria, pyuria (since often from STI in teens)
PRESENT cremasteric reflex (absent in torsion)
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195
What is a varicocele?
abnormal dilation of veins in scrotum
congenital anomaly but not diagnosed until puberty (when increased blood flow)
196
Presentation of varicocele?
"bag of worms" scrotal mass
LEFT side usually
Teenage boy
more PROMINENT with STANDING/Valsalva
usually painless (<10% have dull ache)
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Treatment of varicocele?
surgery if significant difference in L and R scrotal side
198
what typically causes acute interstitial nephritis?
AIN = usually drug sensitivity reaction causing a high creatinine and associated with fever and rash
"renal" AKI
199
DDX pre-renal AKI?
hemorrhage, GI losses
Sepsis (decreased vascular resistance)
3rd spacing: nephrotic syndrome, liver failure
impaired renal autoregulation (hypercalcemia, ACEi, NSAIDs, calcineurin inhibitors (tacrolimus or cyclosporine)
200
DDX renal AKI?
acute interstitial nephritis (AIN), nephrotoxin, ischemic tubular injury
Glomerulonephritis
Hemolytic Uremic Syndrome (HUS)
Renal vein thrombosis (RVT)
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DDX post-renal AKI?
"Obstructive" AKI
BOTH kidneys need to be obstructed to get AKI
UPJ, ureteric, bladder, or uretheral obstruction
UPJ is where Ureter meets kidney (renal Pelvis)
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Difference in timeline between throat/URTI infection and skin infection and onset of PIGN symptoms?
URTI --> 1-3 weeks until development of PIGN
Skin infection --> 3-6 weeks until PIGN
203
C3 and C4 in PIGN
C3 - low
C4 - normal
In real life, the lack of C3 being low does not mean it's not a PIGN
204
When to do bloodwork to re-check C3 in PIGN?
8-12 weeks after illness
Should normalize by 8 weeks.
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Treatment of strep in PIGN?
Controversial
However recent evidence shows that treatment of positive GAS culture is indicated in acute poststreptococcal glomerulonephritis
Penicillin is the drug of choice for nephrogenic Strep, preferably Pen G IM
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If fluid overloaded and hypertensive in glomerulonephritis, even if hemodynamically stable (on the exam) - what treatment is best (per Dr Ding)
Use Lasix
Don't just choose salt and water restriction
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Serum IgA positivity in IgA nephropathy?
Elevated IgA in only 10% of patients
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When treating hypertensive emergency, how quickly do you want to drop BP?
By 25% of planned reduction over the first 8 hours, the rest over the next 12 hours
Eg if trying to drop from 180 to 140, you would drop by 10 in the first 8 hours, then by 30 over the next 12 hours
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Plasma vs urine metanephrines as best test for pheo workup?
Plasma metanephrines
210
Antihypertensive infusions: name the side effects/risks of each one
Nitroprusside (vasodilator)
Labetalol (beta and alpha blocker)
Nicardipine (CCB)
Nitroprusside: risk of cyanide toxicity esp in liver and renal failure
Labetalol: Use caution in cardiogeneic shock, heart block, ASTHMA, diabetes
Nicardipine; increased ICP, IOP
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Causes of proximal RTA?
Isolated
- inherited
- meds (eg carbonic anhydrase inhibitors)
Fanconi's
- acquired - meds (ifosfamide, aminoglycosides, cidofovir), heavy metals, renal disease
- genetic (mitochondrial, galactosemia, tyrosinemia, cystinosis, Dent's, Wilson's)
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Easy ways to distinguish types of RTA?
Serum potassium
- normal- low in Proximal RTA
- LOW in Distal RTA
- HIGH in type 4 RTA
Urine pH
- HIGH > 5.5 in distal RTA
- <5.5 in proximal and type IV RTA
Change to urine pH if you give bicarb?
- INCREASES to > 7 in proximal RTA
Urine anion gap
- normal (negative urine net charge) in Proximal RTA
- low (positive urine net charge) in Distal RTA and Type IV RTA
Urine K (TTKG)
- normal-increased in proximal RTA
- increased in distal RTA
- Decreased in type IV RTA
Urine citrate
- low in Distal RTA only
Nephrocalcinosis / stones
- present in distal RTA only
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What is Bartter syndrome?
Genetic mutation causing defect in the NKCC2 transporter = where furosemide acts
Presentation
- impaired NaCl absorption --> polyuria, polydipsia, volume depletion (low BP)
- hypokalemia
- metabolic alkalosis
- normal or mildly decreased Mg
- normal to increased urine Ca
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What is Gitelman syndrome?
Genetic mutation causing defect in the nCC transporter = where thiazide acts
Presentation:
- mild volume depletion
- hypokalemia
- metabolic alkalosis
- hypomagnesemia
- hypocalciuria
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Key differences between Bartter and Gitelman syndromes?
BP low in Bartter
Mg lower in Gitelman syndrome
Urine calcium normal to high in Bartter, low urine calcium in Gitelman
Bartter - presents infancy to childhood
Gitelman - presents in adulthood
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Causes of positive UA for blood but negative microscopy
Hemoglobinuria
Myoglobinuria
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Reasons to refer PIGN to Nephrology?
Elevated creatinine
Nephrotic range proteinuria
Persistent proteinuria beyond resolution of gross hematuria
Hypertension
C3 does not recover
218
How long does gross hematuria persist in IgA nephropathy vs PIGN?
IgA Nephropathy - usually gross hematuria lasts <72 hours
PIGN - usually gross hematuria persists 1-2 weeks
219
Best approach (per Nephrology ;) ) to maintenance fluids:
Insensible + urine output + other losses = Maintenance
220
Insensible fluid calculation?
400ml / m2
221
What is the expected metabolic compensation for acute respiratory acidosis?
What about chronic respiratory acidosis?
Acute:
HCO3:CO2 = 1:10
Bicarb should increase by 1 for every 10 increase in CO2
Chronic:
HCO3:CO2 = 3:10
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What is the expected metabolic compensation for acute respiratory alkalosis?
What about chronic respiratory alkalosis?
Acute HCO3:CO2 = 2:10
Bicarb should decrease for every 10 that CO2 decreases by
Chronic
HCO3:CO2 = 2:4
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Best choice of anti-hypertensive for PIGN if needed?
Typically amlodipine
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