Tuesday, September 22, 2015

NEPHROTIC SYNDROME

A. Physiological Proteinuria

Every day, 10 to 15 kg of serum proteins pass through the kidney, but only 100 to 150 mg are excreted in the urine in 24 hours
The glomerular capillary wall opposes to the filtration effect of these proteins, and the proximal tubule reabsorbs the great amount of filtered proteins
In the normal state, 60% of urinary proteins excreted from plasma proteins: they are mainly low weight proteins molecular (lysozyme, β-2 macroglobulin); the remaining 40% come from the tubular secretion uromodulin (Tamm-Horsfall protein) and urothelium of the urinary tract


Quantity and study methods

Urine dipstick
It is the most widely use method. It detects the presence albumin when it exceeds 50-100 mg/L
This method has a downside: It does not detect light chains immunoglobulin, or other low weight proteins molecular
Spot urine with 24h urine collection
unlike urine dipstick, will accurately measure myeloma light chains
Another possibility is increasingly used to determine the relative proteinuria / creatinine eliminating (N <0.15; abnormal value > 0.2)
In the same individual, the collection must be tested the same times, preferably on the first urine in the morning
The existence of hematuria or pyuria abundant may impede the interpretation of proteinuria
Qualitative analysis
Microalbuminuria: Specific to albumin
Electrophoresis of urinary protein: characterizes the origin of proteinuria
Laboratory result
-Normal albuminuria < 30 mg/24h; Microalbuminuria: 30-300 mg/24h; Albuminuria > 300 mg/24h
-Normal proteinuria: 100-150 mg/24h; Clinical proteinuria > 500 mg/24h

B. Nephrotic Syndrome

Introduction

The nephrotic syndrome is not a specific glomerular disease, but a constellation of clinical findings that result from an increase in glomerular permeability and loss of plasma proteins in the urine
Definition: Nephrotic syndrome: Proteinuria > 3 to 3.5g/24h and Albuminemia < 30 g/L
(Children: Proteinuria > 50mg/kg/24h and Albuminemia < 30g/L)
Nephrotic syndrome occurs predominantly in children ages 2 to 6 yr and in adults of all ages
Syndrome Nephrotic vs Syndrome Nephritic
Ferri's Clinical Advisor

Pathophysiology

Functional: lose negative charge: idiopathic nephrotic syndrome
Organic: all other glomerular nephropathies
Edema
Hypoprotidemia → Decrease oncotic pressure → water leakage into the interstitial: edema Hypovolemia → Stimulate secretion of RAAS (Renin Angiotensin Aldosterone System) → sodium and water retention

Pathophysiology by Lee-Ellen C. Copstead-Kirkhorn, Jacquelyn L. Banasik

Etiology

NS in Children are mostly idiopathic (90%): Idiopathic nephrotic syndrome or primary nephrotic syndrome
Minimal-change disease: Good prognosis / Corticosensible
Focal segmental glomerulosclerosis: Steroid-resistant / Can develop to CKD 35%
Membranoproliferative glomerulonephritis: Corticosensible
In this 3 causes: tubules overload with lipid vacuoles (Oval fat bodies)
NS in adult
Primary nephrotic syndrome (60%)
Membranous nephropathy
Focal segmental glomerulosclerosis*
Membranoproliferative glomerulonephritis
Secondary nephrotic syndrome (40%)
Diabetes mellitus*
Amyloidosis
Systemic lupus erythematosus
Robbins & Cotran Pathologic Basis of Disease

Diagnosis

1. Clinical examination
Common symptom to all NS
Edema* (Account for weight gain)
  • Bilateral/ symmetric/ white/ soft/ painless/ pitting
  • Supine position: Eye orbits/ hands/ lower back (morning)/ lower limbs
  • +/- Anasarca (pleural effusion, ascites, pericardial effusion)
  • Pulmonary edema is exceptional in the absence heart failure
Oliguria: if the onset is rapid
Frothy of foamy urine due to a massive albuminuria
Note: Idiopathic nephrotic syndrome for child
Most frequency at 2 years/ boy > girl
Generalized edema syndrome + frequent abdominal pain

Pathophysiology: The Biologic Basis for Disease in Adults and Children

Investigation

Spot urine with 24h urine collection
Nephrotic syndrome if proteinuria > 3g/24h (or > 50mg/kg/24h for children)
Serum protein electrophoresis

Hypoprotidemia < 60g/L and hypoalbuminemia < 30g/L
Urinalysis urine sediment
The presence of hematuria, cellular casts, and pyuria is suggestive of nephritic syndrome
Evaluation and complication
Hemostasis: Prothrombin time/ D-dimer/ fibrinogen/ coagulation factors
Infectious: CBC-CRP/ urinalysis/ chest x-rays
Fat metabolism: Cholesterol/ LDL/ HDL/ triglyceride
Complement system: C3 - C4 - CH50 (children*)
Hematology: CBC (haemoconcentration)
Investigation for etiology
Renal biopsy
Indication for children (6)
Age < 1 year or > 10 years
Associated with hematuria, HTN or AKI (Parenchymal)
Extra-renal signs (arthralgia, purpura, etc.)
Low complement system (↓ C3 or immunological abnormality)
Resistance to corticotherapy
Family history of nephropathy
In adult, Renal biopsy is required unless
Diabetes
Suspected Amyloidosis → Perform salivary gland biopsy first
Hereditary glomerulonephropathy already diagnosed
Obvious drug etiology (NSAID and lithium salts)
Others etiologic test for adult
Glycaemia: Diabetic nephropathy
Cryoglobulinemia (Rapidly progressive glomerulonephritis)
Antinuclear antibody (ANA) +/- anti-double stranded DNA (dsDNA): SLE
Serology HBV and HCV

Complications

Acute Complications
Acute renal failure
Serum creatinine > 135μM or clearance < 60mL/min
Mechanism:
Functional: hypovolemia, iatrogenic*
Organic: acute tubular necrosis
Vascular: renal vein thrombosis
Thrombo-embolism
Most likely the proteins required for hemostasis are lost in the urine, stimulating hepatic synthesis of clotting factors
Consider renal thrombosis if back pain or hematuria presented in nephrotic syndrome
Infections (bacterial*)
Urinary leakage of Ig and complement proteins lead to immunodeficiency
Risk and increased severity of infection
Medication overdose
Albumin serve as carriers for molecules of low water solubility including some drugs. In time of hypoalbuminemia, the free active fragments of those medications are increased leading to overdose of prescription. Therefore decrease prescription dose
The Merck Manual of Diagnosis

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