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Morning Report
Jieli Li
03/28/05
Chief Complaint
Generalized edema x 1 week
HPI 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. + 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 Htn – dx’d 1 yr ago Hep C – never treated Syphillis – treated in 1989 Depression Hyperlipidemia Bilateral leg fractures in the past
Meds & Allergies Meds:
Atenolol 50 qd HCTZ 12.5 qd Simvastatin 10 qhs Ascorbic acid 500 qd Alleve prn (OTC)
Allergy: NKDA
Social and Family History SH:
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: Mother: DM & htn Father: CAD with triple bypass
Physical Exam 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.
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 UA
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
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 RPR 1:1 MHA-TP: 4+ ESR: 89 HIV neg C3 and C4: nl RF: neg ASO: nl SPEP:
hypogammaglobulinemia Cryoglobulin neg
Hep A ab R, IgM NR Hep B surface Ag NR Hep B core Ab NR HCV RNA 1,010,000
HIV neg
Renal u/s
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 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. 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
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 Defined by presence of:
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: few cells or casts Lipiduria (oval fat bodies)
Oval Fat Bodies
Etiology In children
Minimal change disease is predominant In adults
Systemic disease related: 30% Primary renal disorders: 70%
Membranous nephropathy Focal glomerulosclerosis Minimal change disease Amyloidosis
In elderly Increased incidence of amyloidosis and decreased incidence of SLE
Etiology cont. 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 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: NSAIDs Cancers as a paraneoplastic phenomenon, most
often Hodgekin’s Disease
Minimal Change Disease
Light Microscopy Either normal or reveals only mild mesangial cell
proliferation
EM Diffuse fusion of the epithealial cell foot
processes
Minimal Change Disease
Focal Glomerulosclerosis (FGS) 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: HIV disease reflux nephropathy Healed previous glomerular injury NSAIDs Massive obesity
Diagnostic Considerations for FGS 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 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 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: Hep B Autoimmune diseases Thyroiditis Carcinoma Certain drugs (e.g., gold, penicillamine, captopril and
NSAIDs)
Membranous Nephropathy
Amyloidosis 4-17% of nephrotic syndrome Increased frequency among elderly Two major types:
Primary amyloid (AL) A light chain dyscracia Fragments of monoclonal light chains form the amyloid fibrils
Secondary amyloid (AA) Acute phase reactant serum amyloid A forms the amyloid fibrils Assoc with chronic inflmmatory diseases such as RA or
osteomyelitis
Amyloidosis
Pathophysiology Proteinuria
Increased filtration of macromolecules across the glomerular capillary wall
Commonly due to abnormalities in podocytes Increased loss of:
Albumin Clotting inhibitors Transferrin Hormone binding proteins (e.g., Vit D binding
protein)
Pathophysiology Hypoalbuminemia
Presumably 2/2 proteinuria Unclear why hepatic synthesis can not compensate
sufficiently Edema
Marked hypoalbuminemia leading to movement of fluid into the interstitial space by decreasing plasma oncotic pressure
Primary renal sodium retention in collecting tubules
Pathophysiology Hyperlipidemia and lipiduria
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 Protein malnutrition Hypovolemia Acute renal failure Urinary loss of hormones Hyperlipidemia and the potential for
accelerated atherosclerosis Thrombosis Increased susceptibility to infection
Protein malnutrition
Loss in lean body mass due to proteinuria
May be masked by concurrent edema
May be compounded by GI symptoms of anorexia and vomiting 2/2 bowel edema
Hypovolemia
Often as a result of overdiuresis in those with a serum albumin < 1.5 g/dL
Occurs more often in children
Acute Renal Failure Can be seen in:
Minimal Change Disease Collapsing FGS Crescentic glomerulonephritis superimposed upon
membranous nephropathy Mechanism not well understood
Hypovolemia Interstitial edema Ischemic tubular injury NSAIDs
Thromboembolism 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 Can present acutely with flank pain, gross hematuria and
ARF or Indolent disease without symptoms, suspected only when
PE occurs
Infection 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 Low levels of IgG may play a role
Proximal tubular dysfunction Often associated with advanced disease Can result in:
Glucosuria Aminoaciduria Phosphaturia renal tubular acidosis Vitamin D deficiency Thyroid dysfunction – due to loss of thyroxine-
binding globulins
Diagnosis 24 hour urine collection
> 3 g/day Total protein to creatinine ratio on spot urine
specimen 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 Certain serologic tests may preclude the need
for renal biopsy: SPEP/UPEP
Presence of a paraprotein should be followed by fat pad or rectal biopsy to look for amyloidosis
ASO poststreptococcal glomerulonephritis
Cryoglobulins Mixed cryoglobulinemia, commonly 2/2 Hep C
Renal Biopsy In adults, renal biopsy is usually required to
determine diagnosis Contraindications:
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 Proteinuria
ACEI / ARB 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 Edema
Dietary sodium restriction Edema is due to primary renal sodium retention in
most cases Diuretics
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 Hyperlipidemia
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
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