Transcript Morning Report

Morning Report
Jieli Li
03/28/05
Chief Complaint
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Generalized edema x 1 week
HPI
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46 y/o AAM with hx of htn, Hep C, syphillis
presented to Urgent Care with generalized
edema x 1 week
Pt noticed progressive lower extremity edema,
then scrotal edema, as well as tightness in
abdomen. + facial edema as well
Pt was seen by PMD and started on HCTZ
last week without any relief
HPI cont.
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+ 2 pillow orthopnea
+ 1 episode of PND and wheezing recently
+ occasional wheezes x 1 yr
+ SOB with exertion
+ occasional cough with yellow phlegm
Baseline exercise capacy excellent
No CP, f/c/s, no diarrhea/constipation
No dysuria, no hemauria
PMH
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Htn – dx’d 1 yr ago
Hep C – never treated
Syphillis – treated in 1989
Depression
Hyperlipidemia
Bilateral leg fractures in the past
Meds & Allergies
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Meds:
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Atenolol 50 qd
HCTZ 12.5 qd
Simvastatin 10 qhs
Ascorbic acid 500 qd
Alleve prn (OTC)
Allergy:
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NKDA
Social and Family History
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SH:
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Single, lives at Midnight Mission
Hx of incarceration x 37 months until Nov 2004, HIV neg
in 2000
Hx of cocaine, MJ, no IVDU
Hx of heavy etoh use, quit 40 months ago
Hx of tobacco (1/2 pk per day x 20 yrs), quit 2 yrs ago
FH:
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Mother: DM & htn
Father: CAD with triple bypass
Physical Exam
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VS: 96.8, 73, 20, 178/99, 0/10
Gen: obese AAM with anasarca, NAD, AAO x 4
HEENT: PERRLA, EOMI, op moist and intact, no
lesions
Heart: distant heart sounds, no murmurs appreciated
Lungs: cta bilaterally, no crackles/wheezes
Abd: obese, mildly distended, NT, na bs, no hsm
PE cont.
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GU: + large scrotal edema, non-tender, testes
palpable and intact, no masses
Ext: 2-3+ pitting edema bilateral legs, generalized
anasarca, + patchy flesh-colored papular lesions
bilateral shins, faint pulses bilaterally
Lab Studies
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UA
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Spec grav 1.04
PH 6.5
Protein > 600
Glucose neg
Ketones neg
Bilirubin neg
Small occult blood
Urobilinogen 0.2
Spot protein/Cr: 2967/396 = 7.5
24 hr urine protein: 12.1g
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Nitrite neg
LE neg
RBC 4
WBC 8
Hyaline casts 40
Lab Studies cont.
15.2
8.6
356
44.8
141 106
27
93
4.57 31.6 1.4
Alk Phos 66
ALT 14
Total bili 0.7
Alb 1.1
Total cholesterol 411
Trig 157
HDL 121
LDL 259
Glomerulonephritis Panel
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RPR 1:1
MHA-TP: 4+
ESR: 89
HIV neg
C3 and C4: nl
RF: neg
ASO: nl
SPEP:
hypogammaglobulinemia
Cryoglobulin neg
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Hep A ab R, IgM NR
Hep B surface Ag NR
Hep B core Ab NR
HCV RNA 1,010,000
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HIV neg
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Renal u/s
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Right kidney 14.6 cm, left kidney 13 cm
No definitive abnormalities although there is
very mild increased cortical echogenicity
Mildly enlarged prostate without bladder
outlet obstruction
Hospital Course
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Pt was admitted to GMED for workup of his
nephrotic syndrome
Hep C induced MPGN vs FSGS vs membranous GN
was high on the differential
Pt was started on lasix, titrated up to 40 po bid
eventually for his anasarca
He was placed on low salt diet
ACEI was held during diuresis, Cr improved to
baseline (1.1)
Hospital Course cont.
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GI was consulted for possible Hep C treatment after
HCV RNA came back > 1 million
Pt’s proteinuria was followed by serial protein/Cr
ratio and 24 hr urine protein
Renal biopsy showed minimal change disease
confirmed by EM
This was believed to be 2/2 hx of NSAIDS
By the time of discharge, pt has only trace protein on
UA, he did not receive any further tx
1 week follow up
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At the renal clinic f/u one week after
discharge, pt’s proteinuria has dropped from
12 g/day to 0.3 g/day. He has lost nearly 100
lbs on diuresis (back to baseline wt). He is no
longer taking NSAIDs.
Nephrotic Syndrome
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Defined by presence of:
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heavy proteinuria (> 3g/24hrs)
Hypoalbuminemia (< 3.0 g/dL)
Peripheral edema
Isolated heavy proteinuria is more likely to be due to
secondary focal glomerulosclerosis
Urinary sediment:
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few cells or casts
Lipiduria (oval fat bodies)
Oval Fat Bodies
Etiology
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In children
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In adults
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Minimal change disease is predominant
Systemic disease related: 30%
Primary renal disorders: 70%
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Membranous nephropathy
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Focal glomerulosclerosis
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Minimal change disease
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Amyloidosis
In elderly
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Increased incidence of amyloidosis and decreased incidence of SLE
Etiology cont.
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Although nephrotic syndrome can develop in
patients with postinfectious GN,
membranoproliferative GN, and IgA
nephropathy, most commonly these disorders
present with a “nephritic” picture, i.e., RBC
and cellular casts in UA
Minimal Change Disease
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90% of nephroitic syndrome in children under
the age of 10
50% of cases in older children
In adults, can occur as an ideopathic condition
or be associated with:
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NSAIDs
Cancers as a paraneoplastic phenomenon, most
often Hodgekin’s Disease
Minimal Change Disease
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Light Microscopy
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Either normal or reveals only mild mesangial cell
proliferation
EM
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Diffuse fusion of the epithealial cell foot
processes
Minimal Change Disease
Focal Glomerulosclerosis (FGS)
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35% of all cases of nephrotic syndrome in the U.S.
> 50% of cases among African Americans
Can occur as an ideopathic condition or be
associated with:
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HIV disease
reflux nephropathy
Healed previous glomerular injury
NSAIDs
Massive obesity
Diagnostic Considerations for FGS
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Sampling error in renal biopsy may lead to
misclassification of FGS as minimal change disease
Steroid-resistance in minimal change disease pts
should raise suspicion for FGS
Primary FGS usually presents with acute onset
nephrotic syndrome, tx is corticosteroids.
Secondary FGS usually presents with slowly
increasing proteinuria, nephrotic syndrome is rare.
Tx is ACEI.
Focal Glomerulosclerosis
Collapsing FGS
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A histologic variant usually associated with HIV
infection
Tendency to collapse and sclerosis of the entire
glomerular tuft, rather than segmental injury
Often severe tubular injury with proliferative
microcyst formation and tubular degeneration
Often with rapidly progressive renal failure
Optimal therapy is uncertain
Collapsing FGS
Membranous Nephropathy
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Basement membrane thickening with little or no
cellular proliferation or infiltration
Presence of electron dense deposits across the
glomerular basement membrane
Can occur as ideopathic condition or be associated
with:
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Hep B
Autoimmune diseases
Thyroiditis
Carcinoma
Certain drugs (e.g., gold, penicillamine, captopril and
NSAIDs)
Membranous Nephropathy
Amyloidosis
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4-17% of nephrotic syndrome
Increased frequency among elderly
Two major types:
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Primary amyloid (AL)
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A light chain dyscracia
Fragments of monoclonal light chains form the amyloid
fibrils
Secondary amyloid (AA)
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Acute phase reactant serum amyloid A forms the amyloid
fibrils
Assoc with chronic inflmmatory diseases such as RA or
osteomyelitis
Amyloidosis
Pathophysiology
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Proteinuria
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Increased filtration of macromolecules across the
glomerular capillary wall
Commonly due to abnormalities in podocytes
Increased loss of:
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Albumin
Clotting inhibitors
Transferrin
Hormone binding proteins (e.g., Vit D binding
protein)
Pathophysiology
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Hypoalbuminemia
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Presumably 2/2 proteinuria
Unclear why hepatic synthesis can not
compensate sufficiently
Edema
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Marked hypoalbuminemia leading to movement
of fluid into the interstitial space by decreasing
plasma oncotic pressure
Primary renal sodium retention in collecting
tubules
Pathophysiology
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Hyperlipidemia and lipiduria
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Decreased plasma oncotic pressure stimulates
hepatic lipoprotein synthesis
Diminished clearance may also play a role
Impaired metabolism is primarily responsible for
nephrotic hypertriglyceridemia
Oval fat bodies are thought to be degenerated
renal tubular epithelial cells containing
cholesterol esters
Complications of Nephrotic Syndrome
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Protein malnutrition
Hypovolemia
Acute renal failure
Urinary loss of hormones
Hyperlipidemia and the potential for
accelerated atherosclerosis
Thrombosis
Increased susceptibility to infection
Protein malnutrition
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Loss in lean body mass due to proteinuria
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May be masked by concurrent edema
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May be compounded by GI symptoms of
anorexia and vomiting 2/2 bowel edema
Hypovolemia
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Often as a result of overdiuresis in those with
a serum albumin < 1.5 g/dL
Occurs more often in children
Acute Renal Failure
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Can be seen in:
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Minimal Change Disease
Collapsing FGS
Crescentic glomerulonephritis superimposed upon
membranous nephropathy
Mechanism not well understood
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Hypovolemia
Interstitial edema
Ischemic tubular injury
NSAIDs
Thromboembolism
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Increased incidence of arterial and venous
thromboemboli, particularly DVT and renal vein
thrombosis
Mechanism not well understood
Renal vein thrombosis is most often found with
membranous nephropathy
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Can present acutely with flank pain, gross hematuria and
ARF or
Indolent disease without symptoms, suspected only when
PE occurs
Infection
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Before abx became available, this used to be
the leading cause of death in children with
nephrotic syndrome
Pneumococcal infections, esp peritonitis were
most common
Mechanism is not well understood
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Low levels of IgG may play a role
Proximal tubular dysfunction
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Often associated with advanced disease
Can result in:
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Glucosuria
Aminoaciduria
Phosphaturia
renal tubular acidosis
Vitamin D deficiency
Thyroid dysfunction – due to loss of thyroxinebinding globulins
Diagnosis
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24 hour urine collection
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Total protein to creatinine ratio on spot urine
specimen
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> 3 g/day
Correlates with daily protein excretion in g/1.73
m2 of body surface area
In history, should look for hx of DM, SLE,
HIV, drugs such as NSAIDs, gold,
penicillamine
Serologic Studies
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Certain serologic tests may preclude the need
for renal biopsy:
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SPEP/UPEP
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ASO
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Presence of a paraprotein should be followed by fat
pad or rectal biopsy to look for amyloidosis
poststreptococcal glomerulonephritis
Cryoglobulins
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Mixed cryoglobulinemia, commonly 2/2 Hep C
Renal Biopsy
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In adults, renal biopsy is usually required to
determine diagnosis
Contraindications:
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Uncorrectable bleeding diathesis
Uncontrolled hypertension
Small kidneys generally indicative of chronic irreversible
disease
Multiple bilateral cysts or renal tumor
Hydronephrosis
Active renal or perirenal infection
Uncooperative patient
Management
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Proteinuria
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ACEI / ARB
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To lower intraglomerular pressure, which may reduce
protein excretion and slow the rate of disease
progression
Potential adverse effects include ARF and
hyperkalemia
Evidence is unclear on protein restriction
Management
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Edema
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Dietary sodium restriction
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Edema is due to primary renal sodium retention in
most cases
Diuretics
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Proceed slowly to prevent acute hypovolemia
Generally there is lesser natriuresis than in normal
patients because of hypoalbuminemia and albuminuria
Serial body weight is important in guiding the titration
of diuretics
Management
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Hyperlipidemia
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Usually reverse with resolution of the renal disease
In case of persistent nephrosis, dietary modification is
usually of little value and a statin is usually required
Hypercoagulability
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Some have suggested prophylactic anticoagulation in
membranous nephropathy due tot high incidence of
thromboemboli
In others, if unexplained thrombosis occurs, they should
be put on heparin followed by warfarin for as long as the
nephrotic syndrome persists