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Clinicopathologic Conference OS 214 - Renal Module. Group IV Tan-Tanchuling-Te-Teo-Tindoc-Tugano-Urquiza-Uy-Velasco-Ventigan-Ventura-Verdolaga-Villanueva-Villanueva-Visperas-Yabut-Yambot-Yap-Yap. General Data. R.V. 45 y/o, F Separated Filipino, Roman Catholic From Port Area Manila - PowerPoint PPT Presentation
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ClinicopathologicConferenceOS 214 - Renal ModuleGroup IV
Tan-Tanchuling-Te-Teo-Tindoc-Tugano-Urquiza-Uy-Velasco-Ventigan-Ventura-Verdolaga-Villanueva-Villanueva-Visperas-Yabut-Yambot-Yap-Yap
General Data
R.V.
45 y/o, F
Separated
Filipino, Roman Catholic
From Port Area Manila
CC: Bipedal Edema
History of Present IllnessOverview
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LBMGeneralized Body WeaknessPalpitationsEasy FaltigabilityEdema
History of Present IllnessOne Month Prior to Consult
Enzyme/Hormone Panel↑ ALT↑ TSH↔ FT4↓ FT3
UrinalysisColor: Yellow
pH: 6.8Slightly Turbid
Albumin +3↑ WBC↑ RBC
Trichomonas
CBC↔ Hemoglobin↔ Hematocrit
↑ WBC↑ Neutrophils
↓ Lymphocytes↔ Platelets
LBM (2 episode, 1 cup/ episode)Generalized Body Weakness/Palpitations/Easy Fatigability
Prescribed with silymarin 140 mg BID for 1 monthNorfloxacin 400 mg BID for 7 days
History of Present IllnessTwo Weeks Prior to Consult
Generalized Body Weakness/Palpitations
UrinalysisProteinuria
↑ WBC Prescribed with Metronidazole 500 mg TIDCiprofloxacin 500 mg BID
Developed Edema on the 3rd day of intake of antibiotics
Consulted another physician and was prescribed with:Potassium Citrate / Diltiazem / Spironolactone / Methylprednisone / Furosemide
Laboratory Work-up was requested
Laboratory Work-up was requested
CBC↔ Hemoglobin↔ Hematocrit↔ Platelets
↑ WBC↑ Neutrophils
↓ Lymphocytes
UrinalysisColor: L. Yellow
pH: 6.0Slightly Turbid
(-) Glucose(-) CrystalsProtein +4
↑ WBC↑ RBC
+ Cast (Coarse/Fine)Bacteria – Moderate
Blood Chemistry↔ ALT↔ AST↔ BUN
↔ Creatinine ↔ Total Protein
↔ Globulin↔ Sodium
↔ Potassium↓ Albumin
↑ ALP
Tumor Markers↔ CEA↔ AFP
SerologyHBsAg – non-reactive
AntiHCV – non-reactive
Thyroid Hormone↔ FT3↔ FT4↑ TSH
Radiological Findings
UTZLiver normal size with solid mass
(3.5x3.3x3.9cm) in left lobenormal portal vein, tributaries,
intrahepatic and CBDGallbladder normal findings
Pancreas and Spleen normal
KUB X-RayNormal Kidneys
Urinary Bladder Normal
Patient was lost to follow up
Seek Consult with a GI consultant
Advised a CT Scan with Triple IV Referred to a Nephrologist
24 hr Urine↑ Protein
↑ Total Volume↑ Protein Excretion
Lipid Profile↑ Cholesterol↑ Triglyceride
↑ LDL↑ VLDL↓ HDL
Prothrombin Time9.5/11.90/>100%/INR 0.78
Normal
Patient was lost to follow up
Day of Consult
History
Review of Systems(+) Hair Loss (thinning)
No joint painsMalar rash
Family History(+) DM - Mother
No intake of NSAIDS and other nephrotoxic drugs or previous Blood Transfusion
Personal/Social HistoryWorked as a photographerClaims Ex-husband was
promiscuous hence separatedNo vices
OB/Gyne HistoryG1P1 (1001)
Physical Exam
BP: 110/80HR: 76 bmp
Bipedal Edema Grade 2Periorbital Edema
LaboratoryANA Negative
Anti dsDNA Negative↔ BUN
↔ Creatinine↔ Sodium
↔ Potassium↓ C3
↓ Albumin↓ Calcium
CBC↔ WBC↔ MCV↔ MCH
↔ Neutrophils↔ Lymphocytes
↔ PlateletsPT: NoramalPTT: Normal
↓ Hgb↓ Hct
Laboratory Results
UrinalysisColor: Yellow
SG: 1.020Slightly Turbid
↔ WBC↔ RBC
(-) Glucose(+) Cast (Waxy/Fine
Granular)Bacteria – Moderate
Protein +4
Non-obstructing Nephrolithiasis in left
kidneyPelvocaliectasis in
right kidney,normal urinary
bladder
Radiologic Findings X-Ray/UTZ
.
Differential Diagnosis
Nephrotic Syndrome
Proteinuria Hyperlipidemia Edema
Minimal change disease
Membranous Glomerulonephritis
Focal Segmental Glomerulonephritis
Membranoproliferative Glomerulonephritis
IgA Nephropathy
Post-streptococcal Glomerulonephritis
Nephrotic Syndrome
Membranous GlomerulonephritisIgA Nephropathy
Rule in: Nephrotic syndrome(10%) Onset following GI infection/UTI Lethargy(if renal function is
impaired)Rule out:x Frank hematuria following
infection(most common presentation)
x Normal to increased C3 complement
Rule in: Nephrotic syndrome(80%) Microscopic hematuria(~50%) Peak incidence 30-50 y/o Non-spcific complaints – fatigue,
malaiseRule out:x Hypertension – not characteristic
but absence makes it unlikelyx Normal C3 Complementx Edema usually generalized
Nephrotic Syndrome
Minimal change disease
Membranous Glomerulonephritis
Focal Segmental Glomerulonephritis
Membranoproliferative Glomerulonephritis
IgA Nephropathy
Post-streptococcal Glomerulonephritis
Nephrotic Syndrome
Minimal change disease
Focal Segmental Glomerulonephritis
Rule in: Proteinuria Hypoalbuminemia hyperlipidemia Benign urinary sediments – casts Dependent edema Facial edema (Periorbital) Microscopic Hematuria (<15%)
Rule out:x More common in childrenx Normal C3 Complement
Rule in: Nephrotic syndrome(~60%) RBC’s and leukocytes in urine
Rule out:x Gross hematuria is more
commonly seenx Hypertension is found as a
presenting feature in one third of patients
x Normal C3 Complement
Nephrotic Syndrome
Minimal change disease
Focal Segmental Glomerulonephritis
Membranoproliferative Glomerulonephritis
Post-streptococcal Glomerulonephritis
Nephrotic Syndrome
Rule in: Nephrotic syndrome Hematuria(may be microscopic) Low C3 Complement Periorbital or dependent edema Fatigue
Rule out:x Can’t be ruled out
Rule in: Hematuria Malaise Weakness Low C3 Complement
Rule out:x Gross hematuria is more
commonly seenx Hypertensionx No history of infection weeks
prior to edema (no latent period)
Membranoproliferative Glomerulonephritis
Post-streptococcal Glomerulonephritis
Nephrotic Syndrome
Membranoproliferative Glomerulonephritis
Post-streptococcal Glomerulonephritis
Nephrotic Syndrome
Membranoproliferative Glomerulonephritis
Type I Type II
Rule in: Nephrotic syndrome Hematuria(may be microscopic) Low C3 Complement Periorbital or dependent edema Fatigue
Rule out:x Can’t be ruled out
Rule in: Hematuria Low C3 Complement
Rule out:x More Nephriticx Red cellsx Red call castsx Hypertensionx No history of infection weeks
prior to edema (no latent period)
Primary Impression
Nephrotic Syndrome2○ Membranoproliferative Glomerulonephritis Type I
Nephrotic Syndrome
Membranoproliferative Glomerulonephritis Type I
Idiopathic
Hepatitis C and B
Cryoglobulinemia
Infective endocarditis
Visceral abscesses
SarcoidosisSjögren syndrome
Scleroderma
Systemic lupus erythematosus
Leukemia
Lymphoma
Carcinoma
HIV Nephropathy
Nephrotic Syndrome
Membranoproliferative Glomerulonephritis Type I
Hepatitis C and BInfective endocarditis Scleroderma
No Evidential Findings in the History and PE
Negative for Serologic Tests
No Evidential Findings in the History and PE
Nephrotic Syndrome
Membranoproliferative Glomerulonephritis Type I
No Evidential Findings in the History and PE
No Lymphadenopathy
No other Evidential
Findings in the History and PE
No Evidential Findings in the History and PE
SarcoidosisSjögren syndrome Lymphoma Leukemia
AnemiaNo other
evidential findings in the History and
PE
Nephrotic Syndrome
Membranoproliferative Glomerulonephritis Type I
No Evidential Findings in the History and PE
Liver MassNo weight lossNegative CEANegative AFP
No Malar RashNegative ANA
Negative Anti-dsDNA
Visceral abscesses Systemic lupus erythematosusCarcinoma
Nephrotic Syndrome
Membranoproliferative Glomerulonephritis Type I
More Common in Type I EBV Infection
Promiscuous HusbandAnemia
Predisposition of repeated Infection
Cryoglobulinemia Idiopathic HIV Nephropathy
Primary Impression
Nephrotic Syndrome2○ Membranoproliferative Glomerulonephritis Type I
2○ to EBV Infectious Mononucleosis vs. HIV Infection
Pathophysiology
HIV Nephropathy
Epstein-Barr Virus
Liver massMPGN Type 1
Nephrotic Syndrome
Cryoglobulin
Low C3 Complement
MPGN Type 1
Nephrotic SyndromeHypothyroid state
HypocalcemiaHyperlipidemia
Proteinuria
Decrease in TBG
Easy fatigability; weakness
Nephrolithiasis due to increased
excretion of calcium
Hypoalbuminemia leading to Edema
Increase in Cholesterol,
Triglycerides, LDL, VLDL
Decrease in HDL
Most have low CD4 counts <200 and advanced HIV
Mechanism is not well understood but in vitro study suggested HIV can infect glomerular endothelial cells, mesangial cells and tubular cells.
Presents with renal insufficiency with heavy proteinuria (>10g), few red or white cells in urine sediment and hypoalbuminemia, nl to large hyperechogenic kidneys on US. HTN and peripheral edema is not common.
Rapid decline in renal function due to severe glomerular injury and marked damage to the tubular cells
In histology, tendency to collapse and sclerosis of the entire glomerular tuft rather than segmental injury.
HIV Infection
EBV Infectious Mononucleosis
Epstein-Barr virus (EBV) is ubiquitous. It is estimated that more than 90% of adult humans demonstrate serologic evidence of a prior infection with EBV.
Most cases of IM are due to EBV, but the vast majority of EBV infections do not result in infectious mononucleosis.
The most common mode of transmission of EBV is through exposure to infected saliva from asymptomatic individuals, often as a result of kissing.
Most patients with Epstein-Barr virus (EBV) infectious mononucleosis are asymptomatic
Prodromal symptoms consisting of 1-2 weeks of fatigue, malaise, and myalgia are common.
ManagementConfirmatory Diagnostics
HIV InfectionRapid HIV tests
Test for HIV antibodies with an enzyme-linked immunosorbent assay (ELISA).
Next steps if rapid test positive Confirm reactive tests with Western blot or immunofluorescent
assay (IFA).
Nonreactive tests with high suspicion for acute HIV infection should be followed up with a virologic test such as HIV RNA assay (viral load).
Viral load is very high (>100,000 copies/mL) in acute HIV infection.
If virologic test is positive, then repeat antibody testing in 3 months after seroconversion.
Patient should undergo the heterophile test to diagnose EBV with IM.
Human serum is absorbed wth guinea pig kidney.
Heterophile titer is defined as greatest serum dilution that agglutinates sheep, horse or cow erythrocytes.
A titer of greater than or equal to 40-fold is diagnostic of acute EBV infection in a person with symptoms of IM with atypical lymphocytes.
The patient can also undergo the monospot test which is slightly more accurate and more commercially available.
The patient can also choose to undergo EBV-specific serologic studies which is considered the “gold standard”.
EBV Infection
Renal Biopsy
Liver Biopsy
Biopsy
ManagementTherapeutics
Treatment of nephrotic syndrome involves:
1. Specific treatment of the underlying morphologic entity
2. General measures to control proteinuria if remission is not achieved through immunosuppresssive therapy and other specific measures
3. General measures to control nephrotic complications
Nephrotic Syndrome
Corticosteroids
Immunoregulators
Diuretics
Anticoagulation to prevent thromboembolic complications
Hypolipidemic agents may be used, but if the nephrotic syndrome cannot be controlled, there will be persistent hyperlipidemia.
In secondary nephrotic syndrome, angiotensin-converting enzyme (ACE) inhibitors and/or angiotensin II receptor blockers are used. These may reduce proteinuria by reducing the systemic blood pressure and by reducing intraglomerular pressure.
Nephrotic Syndrome
Diet
For patients with nephrotic syndrome, their diet should provide adequate energy (caloric) intake and adequate protein (1-2 g/kg/d). Supplemental dietary protein is of no proven value.
A diet with no added salt will help to limit fluid overload.
Management of hyperlipidemia could be of some importance if the nephrotic state is prolonged.
Nephrotic Syndrome
1/3 of patients with Type 1 MPGN will have spontaneous remission, 1/3 will have progressive disease, and 1/3 will have a disease process that will wax and wane but never completely disappear.
The goals of treatment:
reduce symptoms
prevent complications, and
slow the progression of the disorder.
Type 1 MPGN
There is no clearly defined treatment for Type 1 MPGN.
After ruling out secondary causes, it consists of corticosteroid therapy (there is no standard dosage for adults) and antiplatelet drugs (aspirin, 500–975 mg/d orally, plus dipyridamole 225 mg/d orally). The rationale for antiplatelet therapy is that platelet consumption is increased in MPGN and may play a role in glomerular injury.
Prednisone – low dose, alternate-day may have salutary effect on improving renal function.
However studies for this have been limited to children.
Type 1 MPGN
a host of other forms of immunosuppressive and anti-coagulant treatment has been used in the management of type I MPGN.
Ex. dipyridamole, aspirin, and warfarin with and without cyclophosphamide
A study using acetylsalicylic acid with dipyridamole demonstrated a slight decrease in urine protein excretion by 3 years without differences in renal function.
Important to treat the associated diseases, if any.
Treatment with inhibitors of the renin-angiotensin system is prudent if there is proteinuria.
Type 1 MPGN
Clinical course is generally self-limited
Does not usually require specific therapeutic intervention beyond the use of aspirin or acetaminophen for antipyresis and mild pain relief
Short courses of corticosteroids have been used to hasten symptomatic recovery in cases in which the symptoms are severe or refractory.
Excessive physical activity during the first month should be avoided.
Antiviral agents (like acyclovir) have not been demonstrated to significantly accelerate resolution of symptoms or prevent complications of the disease.
EBV-associated IM
Six classes of antiretroviral agents currently exist, as follows:
Nucleoside reverse transcriptase inhibitors (NRTIs)
Nonnucleoside reverse transcriptase inhibitors (NNRTIs)
Protease inhibitors (PIs)
Integrase inhibitors (IIs)
Fusion inhibitors (FIs)
Chemokine receptor antagonists (CRAs)
HIV – Antiretrovirals