Transcript Nephrotic Syndrome

Chapter 25
Disorders of Renal Function
Dory Roedel Ferraro, DNP, ANP-BC, CBN
College of New Rochelle School of Nursing
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Chapter Outline
• Congenital and hereditary disorders of the kidney
• Disorders of glomerular function
• Tubulointerstitial disorders
• Obstructive disorders
• Malignant neoplasms
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Congenital and Hereditary Disorders
• Kidney development begins in 5th week of gestation
• Urine formation begins at the 9th week and is excreted in
the amniotic cavity and is the main component of
amniotic fluid
• 10% of all people are born with potentially significant
malformations of the urinary system; most acquired
defects during embryonic development
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Disorders of Kidney Development
• Renal agenesis
– complete failure of an organ to develop
– Bilateral agenesis occurs 1 in 3000 births
– Unilateral agenesis is compatible with
life and is usually a coincidental finding
• Renal hypoplasia
– Kidney(s) small is size with fewer
nephrons and calyces
– Can lead to renal failure if bilateral
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Disorders of Kidney Development
• Renal dysplasia
– Maldifferentiated structure in all or part of the kidney
– MDKD-multicystic dysplastic kidney disorder
• generally unilateral
• Hypertension and Wilms tumor occurs in 1 in 333 cases
• Annual follow-up with BP and ultrasound is
recommended
• Alteration in position and form
– Horseshoe kidney-the upper and lower poles of the
kidneys are fused
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Cystic Diseases of the Kidney
• Genetic
– Autosomal dominant polycystic kidney disease
(inherit one defective gene from one parent)
– Autosomal recessive polycystic kidney disease
(inherit two defective genes, one gene from each
parent)
– Nephronophthisis
– Medullary cystic kidney disease
• Acquired
– Simple cysts
– Acquired form of renal cystic disease
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Genetic Cystic Diseases
• Autosomal dominant polycystic kidney disease (ADPKD)
• Inherit one defective gene from one parent
• Most common of all inherited kidney diseases
• Characterized by multiple expanding cysts that
ultimately destroy the kidney
• Diagnosed by ultrasound and screening of family
members
• Treatment is supportive and aimed at delaying disease
progression
• Autosomal recessive polycystic kidney disease (ARPKD)
• inherit two defective genes, one gene from each
parent
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Nephronopthisis and Medullary Cystic
Kidney Disease
• Small and shrunken kidneys with multiple cysts
• Progresses to chronic kidney disease
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Disorders of Glomerular Function
• Acute nephritic syndrome
• Rapid progressive glomerulonephritis
• Nephrotic syndrome
• IgA nephropathy
• Hereditary nephritis
• Chronic glomerulonephritis
• Glomerular lesions in systemic disease
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Glomerular Damage
• Proliferative: number of cells increase.
• Membranous: glomerular basement membrane
thickens.
• Sclerotic: amount of extracellular matrix increases.
• All can decrease the efficiency of filtration.
• Allow blood cells, lipids, or proteins to pass into the
urine.
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Types of Glomerular Diseases
• Nephritic syndromes – proliferative inflammatory
response
• Nephrotic syndromes – increased permeability of the
glomerulus
• Mixed nephritic and nephrotic syndromes
• Definitive diagnosis requires renal biopsy
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Acute Nephritic Syndrome
• Acute inflammatory process that occludes the glomerular
capillary lumen and damages the capillary wall
• Primary (acute postinfectious glomerulonephritis)
• Secondary complicating disorder of systemic disease
(SLE)
• Characterized by sudden onset of hematuria, proteinuria,
diminished GFR, oliguria, signs of impaired renal function
• ECF accumulation, edema, hypertension
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Acute Postinfectious Glomerulonephritis
• Occurs after infection with certain strains of group A βhemolytic streptococci, staph infections, viruses
(measles, mumps, chicken pox)
• Caused by deposition of immune complexes
• Rare in industrialized world
• Excellent prognosis if treated
• Rarely causes CKD
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Rapidly Progressive Glomerulonephritis
• Clinical syndrome characterized by signs of severe
glomerular injury
• May be caused by immunologic disorders
• Goodpasture Syndrome- an uncommon and aggressive
form of rapidly progressive glomerulonephritis
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Nephrotic Syndrome
• A constellation of clinical findings that result from an
increase in glomerular permeability and loss of plasma
proteins in the urine
• Characterized by massive proteinuria and lipiduria
• Hypoalbuminemia, generalized edema and
hyperlipidemia
• At risk for thrombotic complications (DVT, PE)
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Nephrotic Syndrome
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Membranous Glomerulonephritis
• Most common cause of primary nephrosis in adults
• Characterized by diffuse thickening of the glomerular
basement membrane due to deposition of immune
complexes
• Idiopathic
• Associated with autoimmune diseases, infections and
metabolic disorders
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Focal Segmental Glomerulonecrosis
• Characterized by sclerosis
• Accounts for 30% of primary nephrotic syndrome in
adults
• More common in AA
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IgA Nephropathy (Berger Disease)
• Primary glomerulonephritis
• Characterized by the presence of glomerular IgA immune
complex deposits which cause inflammation
• Typically asymptomatic; usually discovered during
routine physical
• May present with gross hematuria
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Glomerular Lesions Associated with
Systemic Disease
• Systemic lupus erythematous glomerulonephritis
– Lupus nephritis
– Renal involvement most common complication of SLE
– Routine screening for hematuria and proteinuria in
SLE patients
• Diabetic glomerulosclerosis
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Diabetic Nephropathy
• Major cause of CKD and the most common cause of renal
failure treated by organ replacement
• Occurs in both types (1 and 2)
• Widespread thickening of the glomerular capillary
basement membrane
• Elevations in blood glucose cause an increase in GFR and
glomerular pressure that lead to enlargement of
glomerular capillary pores
• Clinical manifestations: GFR, microalbuminuria (Iimp.
Predictor of future diabetic nephropathy)
• Changes may be reversed by careful regulation of blood
glucose, ACE inhibitors, cessation of smoking and control
of blood pressure
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Scenario
A woman with diabetes mellitus:
• Has severe edema and frothy, cola-colored urine
• Has difficulty breathing, with crackles in both lungs
• Just finished a course of antibiotics for strep throat
• Is taking corticosteroids for lupus-related arthritis
Question:
• What are three reasons for her renal problems?
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Scenario (cont.)
A woman:
• Has diabetes mellitus and lupus
• Recently had strep throat
• Has severe edema and frothy, cola-colored urine
Question:
• The doctor says the only way to determine what has
caused her glomerular disease is a renal biopsy.
Why?
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Scenario (cont.)
• Urinalysis show that she is producing urine
– With 500 mg protein/day
– Containing blood and RBC casts
– With high level of K+
• Blood tests show
– Hypoalbuminemia and slightly decreased K+
• She has borderline hypertension
Question:
• Does she have nephritic syndrome or nephrotic
syndrome?
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Scenario (cont.)
• Urinalysis show that she is producing urine
– With 500 mg protein/day
– Containing blood and RBC casts
– With high level of K+
• Blood tests show
– Hypoalbuminemia and slightly decreased K+
• She has borderline hypertension
Question:
• Does she have nephritic syndrome or nephrotic
syndrome? (Acute postinfectious glomerulonephritis)
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Nephritic vs. Nephrotic Syndrome
Nephritic Syndrome
• Loss of blood
Nephrotic syndrome
• Loss of protein
• Hematuria
• proteinuria
• Red cell casts
• Urine is frothy
• Low urine volume
• Albumin is lost in urine
• Mild hypertension
• Swelling around eyes
and in ankles
• Proteinuria (mild)
• Hyperlipidemia
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Nephritic/Nephrotic Syndrome in
Differential Diagnosis
Nephritic
• Postinfectious
glomerularnephritis
Nephrotic
• Minimal change
glomerulonephritis
• IgA nephropathy
• Focal segmental
Glomerulosclerosis
• Rapidly progressive
glomerulonephritis
• Membranous
glomerulonephritis
• Secondary causes
– SLE
– DM
– Hep B and C
– HIV
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Tubular and Interstitial Disorders
• Acute tubular necrosis (Chapter 26)
• Tubulointerstitial nephritis (Acute or chronic
inflammation of tubules)
• Pyelonephritis
– Acute pyelonephritis
– Chronic pyelonephritis
• Drug-related nephropathies
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Acute Pyelonephritis
• Acute suppurative inflammation of the kidney caused by
inflammation as a result of bacterial infection
• Escherichia coli causative agent in 80% cases
• Complicated
– Occurs in the presence of structural or functional
abnormalities (outflow obstruction, pregnancy,
neurogenic bladder)
– Predisposing medical conditions
• Uncomplicated
– Most commonly seen in young women
– Abrupt onset with shaking chills, fever, backache,
lower UTI symptoms
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Chronic Pyelonephritis
• Progressive disease involving scarring and deformation of
the calyces
• Recurrent of persistent bacterial infection superimposed
on an obstructive process
• Symptoms similar to acute
• May have insidious onset
• Significant cause of CKD
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Bacterial Invasion of the Kidney
• Ascending infection from the lower urinary tract
• Through the bloodstream (septicemia, infective
endocarditis)
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Chronic Pyelonephritis
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Drug-Related Nephropathies
• Functional or structural changes in the kidney following
exposure to a drug
• Kidney cells are exposed to high levels of toxic
metabolites
• Mechanisms of nephropathy
– Decreasing renal blood flow (diuretics, contrast
media, immunosuppressives, NSAIDs, ASA)
– Directly damaging tubulointerstitial structures
– Producing hypersensitivity reactions (synthetic
antibiotics, furosemide, thiazide diuretics)
– Obstructing urine flow (sulfonamides, Vit C)
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Drug-Related Nephropathies
• Tolerance to drugs varies with age, renal function, state
of hydration, BP and pH of the urine
• Elderly patients are high risk for nephrotoxicity
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Obstructive Disorders
• Hydronephrosis
• Renal calculi
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Obstructive Renal Disorders
• Sudden or insidious
• Partial or complete
• Unilateral or bilateral
• Conditions that cause obstruction: congenital anomalies,
urinary calculi, pregnancy, BPH, scar tissue, tumors,
neurologic disorders)
• Classified according to site, degree and duration of
obstruction
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Consequences of Dilation of the Renal
Tubules or Tract
• Expansion of the kidney with urine (hydronephrosis)
– Increased pressure inside the renal capsule
– Compartment syndrome compresses blood vessels
inside kidney
– Renal ischemia
• Stasis of urine
– Risk of infection
– Stones
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Question
True or false?
Hydronephrosis is categorized as a disorder of glomerular
function.
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Answer
False
Rationale: Hydronephrosis is caused by a urinary
obstruction, so it is considered an obstructive disorder.
The glomerulus is not involved.
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Renal Calculi (Kidney Stones)
• Saturation theory: Urine is supersaturated with
stone components.
• Matrix theory: Organic materials act as a nidus for
stone formation.
• Inhibitor theory: A deficiency of substances that
inhibit stone formation.
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Four Types of Renal Calculi
• Calcium stones (i.e., oxalate or phosphate)
• Magnesium ammonium phosphate stones
• Uric acid stones
• Cystine stones
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Renal Calculi (cont.)
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Clinical Features of Renal Calculi
• Pain
– Renal colic: acute, intermittent and excruciating flank
pain (caused by stretching of the ureter)
– Non-colicky pain: dull, deep ache in the flank or back
(caused by distension of the renal calyces or pelvis)
• Skin cool and clammy
• Nausea and vomiting
• Diagnosis: urinalysis, US, IVP, non-contrast CT scan
• Treatment: pain relief, strain urine, analysis of stone
prevent recurrence
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Malignant Kidney Tumors
• Embryonic kidney tumors
– Wilms tumor (occurs in childhood)
– Also known as nephroblastoma
– Can be unilateral or bilateral
– Sharply demarcated, encapsulated mass which
usually presents as a protruding abdominal mass
– Treated with surgery, chemotherapy
– Long term survival rates 70-96%
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Malignant Kidney Tumors
• Renal cell carcinoma
– Usually occurs in adults (incidence peaks between 55
and 84 yrs)
– Contributing factors: obesity, occupational exposure
to petroleum products, heavy metals, asbestos
– Presenting features include: hematuria, flank pain,
presence of palpable flank mass
– Metastasizes to lung and bone
– Diagnosis: CT scan, US
– Treatment: surgery
– Prognosis: 5 year survival rate is 90%
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Wilms Tumor of the Kidney
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Renal Cell Carcinoma of the Kidney
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