Cola-colored urine Flashcards
Case
CC: cola-colored urine
CAA 9/M , sees you for the first time with the chief complaint of
coke-colored urine
HPI: On day of consult, presented with gross hematuria, periorbital
edema, and scanty urine.
ROS:
(+)fever 37.9
(-)rashes
(-)easy bruisability
(-) epistaxis
(-)gum bleeding
(-) melena/hematochezia
(+)malaise
(+)headache
PMHx: no known history of trauma
No known history of intake of any substance or drug
History of cough 3 weeks ago, diagnosed to have URTI,
was treated with intake of co-amoxiclav
FMHx: no known relatives with bleeding disorders, or kidney
problems
PE: BP 130/90 HR 100 RR 23 Temp 37.7c
ambulatory, Weight for age below -1 , Length for age below -1
anicteric sclerae, pink palpebral conjunctiva, no
cervicolymphadenopathies
adynamic precordium, regular rhythm, no murmur
soft abdomen, normoactive bowel sounds, (+) CVA tenderness,
right
Pink nailbeds, (+) bipedal edema
SMR 1 (Pubic hair, penis, testes)
Laboratory
CBC:
Hgb 10.0 (LOW) 12.0- 15.0 g/dL
Hct 35 (LOW) 36 – 48%
RBC 5 3.5 – 5.5 ml/UL
MCV 65 (LOW) 80-100 FL
MCH 18 (LOW) 25-35 PG
RDW 12 11 – 16 FL
WBC 9.0 4.5 -11.0 K/UL
Segmenters 55 40-74
Lymphocytes 45 14-46
Monocytes 0 4-13
Platelet Count 350 150 – 450 K/UL
PBS: normochromic, normocytic
Urinalysis:
Color amber
Turbidity Sl. Hazy
Sp. Gravity 1.010
pH 6.0
Glucose Neg
Ketones Neg
Protein ++
Bilirubin neg
Urobilinogen neg
Casts RBC casts, hyaline casts
RBC Too numerous to count
WBC 14
Epi cells 2
Other lab exams done:
ASO titer : 300units/mL (normal=200units/mL)
Anti-DNAse B: 180 (normal= 170 units/mL)
C3: 60 (normal= 88-252 mg/dL)
I. Primary Working Impression:
Acute glomerulonephritis (post-streptococcal type)/ PSGN
What are the basis of your diagnosis?
On the basis of:
History
1. Acute onset of nephritic syndrome (gross hematuria,
edema, hypertension, oliguria).
2. Symptoms occurred 1-2 weeks after an antecedent
URTI.
3. This disease is not familial.
4. Common in children 5-12
PE
1. Hypertensive (130/90)
2. Edema (periorbital and bipedal)
3. CVA tenderness/flank pain
Labs
1. Urinalysis
a. Hematuria is grossly appreciated (amber in
color, RBC is TNTC)
b. Sp. Gravity shows diluted urine
(hypovolemia)
c. Proteinuria (+2: is always present, but rarely
exceeds +3)
d. PSGN frequently presents with RBC casts,
and WBC
e. Sample is a clean catch sample (epi cells are
within normal range)
2. Mild, normochromic anemia
3. Elevated ASO titers
4. Elevated antiDNAse B
5. Depressed C3
6. Renal exams (LM: diffuse proliferation; IF: granular IgG,
C3; EM: subepithelial humps)
What are your differentials?
—–1.IgA nephropathy—–
Rule in:
*Presentation of nephritic
syndrome
*Proteinuria is not markedly
increased
*Presence of hypertension
(30-50% in IgA nephropathy)
Rule out:
*Usual age 10-35 (index
patient is 9)
*IgA nephropathy rarely
presents with oliguria
*IgA nephropathy follows viral
syndromes; our patient had
bacterial URTI
*Serum IgA should be elevated
IgA Nephropathy vs PSGN
Light microscopy:
IgA Nephropathy –> Focal
proliferation
PSGN –> Diffuse
Immunoflorescence:
IgA Nephropathy –> Diffuse mesangial IgA
PSGN –> Granular IgG
Electron microscopy:
IgA –>Mesangial deposits
PSGN –>Subepithelial
humps
—–2.Goodpasture Syndrome—–
Rule in:
presents with nephritic syndrome
Proteinuria is not markedly increased
Rule out:
Usual age is 15-30
flank pain is rare
Hypertension is rare in
goodpasture
Anemia is IDA in goodpasture
Pulmonary hemorrhage is
seen
There should be note of anti-
GBM antibody
—–3. SLE Nephritis—–
Rule in:
Nephritis
with an antecedent URTI 1-3 weeks prior
Rule out: systemic features (rashes) of HSP are not present
—-4. Trauma—–
rule in: hematuria
Rule out: Trauma-related hematuria
does not present with
hypertension or
proteinuria/edema
There is no known history of
trauma
—–5. Urinary lithiasis—–
Rule in: hematuria
Rule out: Flank pain is usually very
prominent (renal colic)
What are the diagnostics?
Management
Diagnostics:
Urinalysis
CBC
PBS
ASO titer
Anti-DNAse B
C3
Treatment?
Mostly supportive
1. Fluid restriction
2. Diuretics (Furosemide 2mkdose q6-q12)
3. Antihypertensives (calcium channel blocker:
Nifedipine 0.5mkdose q4-6)
4. Sodium intake restriction
Use of penicillin antibiotics (10days) is recommended to limit
spread of the nephritogenic organisms. This, however, does not
affect natural course of the existing infection.
Prognosis
95% recovery
Hypertension and proteinuria resolves by 4-6 wks after onset
Hematuria may persist up to 1-2 yrs
C3 levels should normalize after 6-8 weeks
Recurrences are extremely rare
Acute complications of this disease result from hypertension and
acute renal dysfunction. Hypertension is seen in 60% of patients
and may be associated with hypertensive encephalopathy in 10%
of cases. Other potential complications include heart failure,
hyperkalemia, hyperphosphatemia, hypocalcemia, acidosis,
seizures, and uremia.
What are the manifestations of Nephritic syndrome?
CH 511: ACUTE GLOMERULONEPHRITIS (AGN)
CM (Nephritic syndrome):
1. Tea/cola-colored urine
2. Facial or body edema (pleural effusion, pulmonary
edema, HF)
3. Hypertension
4. Oliguria
What is POST STREPTOCOCCAL GLOMERULONEPHRITIS (PSGN)?
POST STREPTOCOCCAL GLOMERULONEPHRITIS (PSGN)
Etiology
- Nephritogenic strain of GABHS
- Disease of children, 5-12 yo (peak 6-7yo), uncommon
<3yo
- Molecular mimicry: streptococcal Ag elicit circulating
Ab which react with glomerular Ag»_space; complement
activation»_space; immunologic renal injury
- Exemplifies immune complex mediated reaction
o Involves activation of classic and alternative
complement pathways
o Localized in the glomeruli due to negatively
charged capillary wall, mesangial trapping,
hydrodynamic forces
Patho
What are the clinical manifestations of PSGN?
CM
1. Latency period – Hx of infection (1-2 wks: pharyngitis
or 3-6 wks: streptococcal pyoderma)
2. Acute onset of nephritic syndrome (gross hematuria,
periorbital edema, hypertension, renal insufficiency)
PLUS:
a. 4 phases of APSGN “LOrd OF Da Rings”:
each phase 7-21d
i. Latent phase
ii. Oliguric phase
iii. Diuretic phase
iv. Resolution phase/ period of
convalescence
b. elevated ASO
c. hypocomplementenemia (N c1 and c2, low
c3 and other panels)
3. nonspecific sx – malaise, lethargy, abdominal pain, or
flank pain
What are the diagnostics for PSGN?
Dx
1. UA – hematuria, dysmorphic RBC, RBC casts, WBC,
proteinuria, PMN leukocytes
2. Dec C3 - >90% decrease in acute phase, returns to N by
6-8wks after onset
3. BUN, crea – renal insufficiency, assess renal fxn
4. CBC – mild normocytic, normochromic anemia:
dilutional anemia secondary to fluid overload from
oliguria
5. Documentation of streptococcal infection
a. ASO titer – throat infection
b. Anti-deoxyribonuclease B (anti DNAse B) –
pyoderma: single best Ab titer to document
cutaneous strep infection
c. Rising Ab titers to streptococcus Ag
What are the findings in light microscopy, IF and EM of PSGN?
Light microscopy:
Enlarged,
bloodless,
glomeruli, diffuse
mesangial cell
proliferation,
increased
mesangial matrix
IF:
“lumpy-bumpy” granular deposits
of Ig and complement (C3) on GBM and in the mesangium
EM:
Electron dense deposits “subepithelial humps” on the
epithelial side of the GBM
Management of PSGN
Mgt
1. Abx – early systemic abx do not eliminate the risk of GN, to ensure eradication of pathogen
- Penicillin G 1.2 M U/dose x 10d to limit the spread of nephritogenic strains
- Alt: Erythromycin 50mkd po q12 x 10d
- Fluid limitation – mainstay of tx: 400ml/BSA + UO in 24h
- Strictly monitor I&O, weigh daily. Readjust fluid limit daily
- Sodium restriction - <2g Na/d
- Diuresis (Furosemide 1-2 mkdose q6-12h IV)
- Titrate daily based on clinical status - Antihypertensives – short-acting Ca channel blockers
for acute BP control then diuretics once BP controlled
- For short-acting ACEI, WOF hyperK - monitoring
Prognosis of PSGN
Prognosis
Time course of resolution:
The acute phase (incl C3) generally resolves within 6-8 wks
Proteinuria and HTN normalize by 4-6 wks after onset
Persistent microscopic hematuria can persist for 1-2 yrs after
onset
Oliguria, azotemia –> gross hematuria –> HTN –> C3 –> persistent proteinuria –> microscopic hematuria –> intermittent or orthostatic proteinuria
Complications:
1. Hypertensive encephalopathy – MC cause of death
- BOV, severe headache, altered mental status, new seizure
- Posterior reversible encephalopathy syndrome (PRES)
- Dx: brain MRI – parietooccipital area
2. HF – due to HTN or hypervolemia
More favorable outcome in children. Complete recovery in >95%
0.5-2% may progress to RPGN, reaching ESRD within weeks to
mos 2nd attacks unusual. Hx of recurrent nephritis should prompt
investigation for chronic renal ds
What is the other name of IgA Nephropathy?
BERGER DISEASE
IG A NEPHROPATHY/ BERGER DISEASE
- MC form of primary GN in the world
- MC chronic glomerular ds in children
- Ab-mediated glomerular ds
- Immune deposits (IgA) localize to the mesangium
- Differentiated from APSGN by: lack of latent phase, N
c3
- Synpharyngitic: gross hematuria often occurs after 1-
2d of URTI or GIT infection
- M>F
Pathophysiology of IgA nephropathy
Patho
- Galactose-deficient IgA1 act as autoAg that trigger
production of Ab and formation of immune complexes
deposited in the renal mesangium –> glomerular injury
by proinflammatory cytokine release, chemokine, and
macrophage migration to the kidney –> glomerular
deposition
What are the clinical manifestations?
CM
1. Recurrent Gross/microscopic hematuria and/or proteinuria
- May present with nephrotic or nephritic syndrome, or combined nephritic-nephrotic syndrome
- Onset within 1-2 days of viral URTI
What are the 3 syndromes?
3 syndromes:
1. Recurrent macroscopic (synpharyngitic) hematuria
- Asymptomatic microscopic hematuria and variable
proteinuria - HSP
What are the diagnostics for IgA Nephropathy?
Dx
1. UA – hematuria
2. Proteinuria <1000 mg/d
3. Normal C3
4. Serum IgA – no diagnostic value (inc in only 15%)
What are the findings in light microscopy, IF and EM of IgA Nephropathy?
IgA Nephropathy/Berger Disease
Light microscopy:
Focal and segmental
mesangial proliferation and
increased mesangial matrix
in the glomerulus
IF:
Diffuse IgA deposits in the
mesangium with C3 complement
EM:
Mesangial deposits
management of IgA nephropathy
Mgt
1. ACEI, ARBs - BP control and Mgt of proteinuria
2. fish oil (omega-3-FAs) – decrease rate of renal
progression
3. CS – if ACEI and ARBs are ineffective
Prognosis of IgA nephropathy
Prognosis
- 40% renal function progressively worsens
- 30% benign course with chronic microscopic
hematuria, N crea, proteinuria <1g/d
- 20% ESRD after 20 yrs of clinically apparent ds
- HTN common, malignant HTN in 5%
- Poor prognosis: persistent HTN, renal dysfunction,
heavy/prolonged proteinuria
What is Henoch-Schonlein Purpura Nephritis?
HENOCH-SCHONLEIN PURPURA (HSP) NEPHRITIS
- Idiopathic systemic immune complex-mediated
vasculitis with IgA deposits in the small BV walls
- Any mucosal infection or food Ag may trigger the inc
production of pathogenic IgA
- Etiology unknown
- More freq in children 2-8 yo; M>F
Patho
- IgA deposition in glomeruli
What are the clinical manifestations of HSP Nephritis?
CM
1. Preceded by URTI (1-3 wks)
2. LG fever, fatigue
3. Tetrad of HSP:
a. Palpable purpuric rashes on dependent
parts of the body – hallmark
b. Arthritis or arthralgia
c. Abdominal pain
d. Renal ds (25-50%)
4. Hematuria + proteinuria – within first 3 mos of ds
a. Majority have isolated hematuria
b. Resolve spontaneously
How is HSP Nephritis diagnosed?
Dx
1. UA – asymptomatic microscopic hematuria to severe
progressive GN
2. CBC – anemia, mod thrombocytosis and leukocytosis
3. Elevated ESR
4. Elevated IgA and IgM
5. ANA, ANCA, RF (-)
6. Anticardiolipin and antiphospholipid ab (+)
7. N C3 – distinguishes HSP from APSGN
Findings of HSP Nephritis in light microscopy, IF and EM
Light microscopy:
Leukocytoclastic vasculitis/angiitis
with IgA C3, and fibrin deposition
IF: Deposition of polymeric IgA in
the glomeruli
EM: Mesangial deposits
What is the management of HSP nephritis?
- UA - Done weekly during active ds then once monthly for 6 mos (if all N, GN unlikely)
- Mild: resolves spontaneously
- Moderate to severe ds:
a. CS (prednisone 1mkd x 3 mos)
b. ACEI
c. Followed by azathioprine or mycophenolate
if persistent - Refer to nephro – if with proteinuria, renal
insufficiency, HTN - Adequate hydration, bland diet
- Pain control with Pct
Prognosis of HSP nephritis
Prognosis
2-5% risk of CKD
Older children and adolescents have more severe ds
Majority do not have severe renal ds/ serious sequelae
What is HUS?
HEMOLYTIC UREMIC SYNDROME (HUS)
- One of the MC cause of ARF in young children
- Usually preschool and school-aged children, MC <4yo
Etiology
- MC form due to Shiga toxin-producing E.coli 0157:H7
(STEC)
- SLE, HELLP syndrome, genetics, drugs (calcineurin
inhibitors, cytotoxic agents, clopidogrel, ticlopidine,
quinine)
Patho
- Toxin initiates endothelial cell injury which leads to microvascular injury
- Microangiopathic anemia due to mechanical damage to RBCs as they pass through the altered vasculature
- Thrombocytopenia due to intra-renal platelet adhesion or damage
What is the triad of HSP nephritis?
Triad
a. Microangiopathic hemolytic anemia
b. Thrombocytopenia
c. Renal insufficiency
Other clinical manifestations of HUS
- Triad
a. Microangiopathic hemolytic anemia
b. Thrombocytopenia
c. Renal insufficiency - Hx of bloody AGE within the preceding 3 weeks
- Sudden onset of pallor, irritability, weakness, lethargy, oliguria
- Usually 5-10d after initial illness
- Dehydration, petechiae, hepatosplenomegaly, marked
irritability - CV: HTN, pericarditis, myocardial dysfunction, arrythmia
- E’ abN – hyperK, hypoNa
- CNS – encephalopathy, sz
- GI – inflammatory colitis, ischemic enteritis, bowel perforation, intussusception, bleeding
Laboratory criteria of HUS
The following re both present at some time during the illness:
1. Anemia (acute onset) with microangiopathic changes (schistocytes, burr cells, helmet cells) on PBS
2. Acute renal injury: hematuria, proteinuria, elevated creatinine levels (>1.5 mg/dL in >13 years old or ≥50% increase over baseline
Classification of HUS
—–PROBABLE—–
meets lab criteria but with no clear history of diarrhea in the preceedig 3 weeks or
onset within 3 weeks of diarrhea, and meets the lab criteria except that microangiopathic changes are not confirmed
—–CONFIRMED—–
meets the laboratory criteria AND began within 3 weeks of diarrhea
