AUBF 18: RENAL DISEASES

Renal disease

kidney disease

immunologic disorders

Most glomerular disorders result from __________ throughout the body, including the kidney

Immune complexes

___________formed as a result of immunologic reactions and increased serum immunoglobulins, such as immunoglobulin A (IgA), circulate in the bloodstream and are deposited on the glomerular membranes.

complement, neutrophils, lymphocytes, monocytes, and cytokines

Components of the immune system, including ____________ are then attracted to the area, producing changes and damage to the membranes

exposure to chemicals and toxins that also affect the tubules,

disruption of the electrical membrane charges as occurs in the nephrotic syndrome,

deposition of amyloid material from systemic disorders that may involve chronic inflammation and acute-phase reactants, and

the basement membrane thickening associated with diabetic nephropathy

Nonimmunologic causes of glomerular damage include

Glomerulonephritis:

inflammation of the glomeruli within the kidney

refers to a sterile, inflammatory process that affects the glomerulus and is associated with the finding of blood, protein, and casts in the urine

sterile

Glomerulonephritis refers to _________

acute; chronic ; nephrotic; renal failure

It is rapidly progressive from _____ it will develop to ______, and from chronic it will develop to _______syndrome, and eventually it will lead to ________

Acute Poststreptococcal

Glomerulonephritis

Rapidly Progressive (Crescentic) Glomerulonephritis

Goodpasture Syndrome

Wegener Granulomatosis

Henoch-Schönlein Purpura

Membranous Glomerulonephritis

Membranoproliferative Glomerulonephritis

Chronic Glomerulonephritis

Immunoglobulin A Nephropathy

Glomerulonephritis

1.Acute post-streptococcal glomerulonephritis

disease marked by the sudden onset of symptoms consistent with damage to the glomerular membrane

post infection of sequelae

Deposition of immune complexes, formed in conjunction with Group A Strep infection on glomerular membranes

ETIOLOGY of Acute post-streptococcal glomerulonephritis

These may include fever, edema (most noticeably around the eyes), fatigue, hypertension, oliguria, and hematuria.

symptoms of Acute poststreptococcal glomerulonephritis that is usually occur in CHILDREN and YOUNG ADULTS

group A streptococcus

Symptoms usually occur in children and young adults following respiratory infections caused by certain strains of __________ that contain M protein in the cell wall

M protein in the cell wall

group A streptococcus contains ?

nephrogenic strains of streptococci

During the course of the infection, these _________form immune complexes with their corresponding circulating antibodies and become deposited on the glomerular membranes. The accompanying inflammatory reaction affects glomerular function.

hematuria proteinuria oliguria accompanied by red blood cell (RBC) casts dysmorphic RBCs hyaline granular casts white blood cells (WBCs).

Primary urinalysis or laboratory findings include marked

Blood urea nitrogen (BUN)

may be elevated during the acute stages but, like the urinalysis, returns to normal

positive anti-group A streptococcal enzyme

Demonstration of ___________tests provides evidence that the disease is of streptococcal origin

Elevated ASO titer

provides evidence it is of streptococcal origin

2.Rapidly progressive glomerulonephritis

A more serious form of acute glomerular disease

POORER prognosis, often terminating in renal failure

Deposition of immune complexes from systemic immune disorders on the glomerular membrane

ETIOLOGY of Rapidly progressive glomerulonephritis

Crescentic glomerulonephritis

other term for Rapidly Progressive Glomerulonephritis

systemic lupus erythematosus (SLE).

Symptoms are initiated by DEPOSITION OF IMMUNE COMPLEXES in the glomerulus, often as a complication of another form of glomerulonephritis or an immune systemic disorder such as ________

systemic lupus erythematosus

most common immune disorder

Bowman's space

Damage by MACROPHAGES to the capillary walls releases cells and plasma into ______________, and the production of crescentic formations containing macrophages, fibroblasts, and polymerized fibrin, causes permanent damage to the CAPILLARY TUFTS

Proteinuria (markedly elevated protein levels) RBC cast (very low glomerular filtration rates)

Some forms may demonstrate increased fibrin degradation products, cryoglobulins

deposition of IgA immune complexes in the glomerulus

Initial laboratory results are similar to acute glomerulonephritis but become more abnormal as the disease progresses, including

3.Goodpasture syndrome

the etiology of this is Attachment of a cytotoxic antibody formed during viral respiratory infections to glomerular and alveolar basement membrane

rapidly progressive glomerular nephritis

Morphologic changes to the glomeruli resembling those in ______________are seen in conjunction with the AUTOIMMUNE DISORDER termed Goodpasture syndrome.

antiglomerular basement membrane antibody

Attachment of an autoantibody to the basement membrane followed by complement activation that produces capillary destruction

Can be detected in patient serum

hemoptysis and dyspnea

followed by the development of hematuria

Initial pulmonary complaints are

hemoptysis

bloody sputum

dyspnea

difficulty breathing

proteinuria, hematuria, and the presence of RBC casts.

Urinalysis results include

true

Progression to chronic glomerulonephritis and endstage renal failure is common in Goodpasture's syndrome

4.Wegener's granulomatosis

causes a granuloma-producing inflammation of the small blood vessels primarily of the kidney and respiratory system.

Anti-neutrophilic cytoplasmic autoantibody [ANCA] binds to neutrophils in vascular walls producing damage to small vessels in the lungs and glomerulus

etiology of Wegener's granulomatosis

hematuria, proteinuria, RBC casts, and elevated serum creatinine and BUN

Patients usually present first with pulmonary symptoms and later develop renal involvement, including

ethanol or formalin/formaldehyde-fixed neutrophils

Testing for ANCA includes incubating the patient's serum with either _________and examining the preparation using indirect immunofixation to detect the serum antibodies attached to the neutrophils.

indirect immunofixation

ANCA is detected by the use of

p-ANCA

If the neutrophils are fixed in ETHANOL, the antibodies form a perinuclear pattern called _________

c-ANCA

When the neutrophils are fixed with FORMALIN/FORMALDEHYDE, the pattern is granular throughout the cytoplasm called

patients usually present pulmonary symptoms and later develop to renal involvement.

symptoms of Wegener Granulomatosis

5.Henoch-Schönlein Purpura

Occurs primarily in CHILDREN after UPPER RESPIRATORY infections following viral infections; a decrease in platelets [difference with good pasture] disrupts vascular integrity

appearance of raised, red patches on the skin

presence of hemoptysis and dysenteric stools

As its name implies, initial symptoms include the

blood in the sputum and stools, may be present (HEMOPTYSIS)

Respiratory and gastrointestinal symptoms, including

Renal involvement

is the most serious complication of the disorder and may range from mild to heavy proteinuria and hematuria with RBC casts

hematuria, proteinuria with RBC casts (mild to heavy)

LAB FINDINGS

50% of patients

Complete recovery with normal renal function is seen in more than

glomerulonephritis and renal failure

In other patients, progression to a more serious form of __________ may occur

6. Membranous Glomerulonephritis

the etiology of this is THICKENING of the glomerular membrane following IgG IMMUNE COMPLEX DEPOSITION associated with systemic disorders

systemic lupus erythematosus, Sjögren syndrome, secondary syphilis, hepatitis B, gold and mercury treatments, and malignancy

Disorders associated with membranous glomerulonephritis development include

microscopic hematuria Proteinuria (elevated urine protein excretion that may reach concentrations similar to those in the nephrotic syndrome)

Laboratory findings include

7.Membranoproliferative Glomerulonephritis (MPGN)

the etiology of this is Cellular proliferation affecting the capillary walls or the glomerular basement membrane

Many of the patients are children, and the disease has a POOR PROGNOSIS

Membranoproliferative glomerulonephritis (MPGN) Type I:

shows increased cellularity [thickening of membrane] in the subendothelial cells of the mesangium, causing thickening of the capillary walls.

nephrotic syndrome

➢ Type I patients progress to _______

Membranoproliferative glomerulonephritis (MPGN) Type II:

displays extreme dense deposits in the basement glomerular membrane.

chronic glomerulonephritis

Type II patients progress to

hematuria, proteinuria, and decreased serum complement levels

The laboratory findings vary, but ________are usual findings. There appears to be an association with autoimmune disorders, infections, and malignancies.

8.Chronic Glomerulonephritis

the etiology of this is Marked decrease in renal function resulting from glomerular damage precipitated by other renal disorders

TRUE

In Chronic Glomerulonephritis progression to chronic glomerulonephritis and end-stage renal disease may occur

fatigue, anemia, hypertension, edema, and oliguria.

Gradually worsening symptoms include

hematuria proteinuria glucosuria as a result of tubular dysfunction, and many varieties of casts, including broad casts.

Examination of the urine reveals

A markedly decreased glomerular filtration rate GFR

increased BUN and creatinine and electrolyte imbalance

9.Immunoglobulin A Nephropathy or IgA nephropathy

the etiology of this is Deposition of IgA on the glomerular membrane resulting from increased levels of serum IgA

The disorder is most frequently seen in children and young adults

Berger disease

Immunoglobulin A Nephropathy or IgA nephropathy is also known as

YES

IgA a large immunoglobulin that's why it will be deposited in the glomerulus, and will not be filtered out. If you have an increase IgA in the plasma, the IgA will then be deposited in the filter [glomerulus]

20

A patient with this disorder may remain essentially asymptomatic for ___ years or more; however, there is gradual progression to chronic glomerulonephritis and end-stage renal disease.

hematuria (following an infection or strenuous exercise)

LAB FINDINGS of IgA nephropathy

yes

Recovery from the macroscopic hematuria is spontaneous; however, asymptomatic microhematuria and elevated serum levels of IgA remain

10.Nephrotic Syndrome

is marked by MASSIVE proteinuria (greater than 3.5 g/day), LOW levels of serum albumin, HIGH levels of serum lipids, and pronounced edema

Disruption of the shield of negativity and damage to the tightly fitting podocyte barrier resulting in massive loss of protein and lipids

etiology of nephrotic syndrome

yess

Acute onset of the disorder can occur in instances of circulatory disruption producing SYSTEMIC SHOCK that DECREASE the pressure and flow of blood to the kidney.

Increased permeability of the glomerular membrane

________________ is attributed to damage to the shield of negativity and the podocytes that produces a less tightly connected barrier. Such damage facilitates passage of high-molecular-weight prote

albumin

is the primary protein depleted from the circulation

hypoalbuminemia

marked DECREASE in serum albumin concentration

appears to stimulate INCREASED lipid production by the liver

lower oncotic pressure

The______ in the capillaries resulting from the DEPLETION of plasma albumin INCREASES fluid loss into the interstitial spaces, which, accompanied by sodium RETENTION, produces the EDEMA

Depletion of immunoglobulins and coagulation factors

It is à risk of infection and coagulation disorders.

tubular and glomerular damage

Both ___________ occurs, and the nephrotic syndrome may progress to chronic renal failure

Heavy proteinuria Microscopic hematuria Renal tubular cells Oval fat bodies Fat droplets Epithelial, Fatty and waxy casts

LAB FINDINGS

11.Minimal change disease

produces little cellular change in the glomerulus, except for some damage to the podocytes and the shield of negativity, allowing for increased protein filtration.

Disruption of the podocytes occurring primarily in children following allergic reaction, immunizations and possession of the human leukocyte antigen-B12 (HLA-B12) antigen

origin is non-specific

etiology of Minimal change disease

lipid nephrosis

Minimal change disease also known as

children

Minimal change disease occur primarily in ________ that is the most nephrotic syndrome in __________

Heavy proteinuria Transient hematuria Normal BUN and CREATININE results Fat droplets

lab findings of Minimal change disease that is present in edema

yep

Although the etiology is UNKNOWN at this time, allergic reactions, recent immunization, and allergic reactions, recent immunization, and possession of the human leukocyte antigen-B12 (HLA-B12) antigen have been associated with this disease. The disorder RESPOND WELL to corticosteroids, and prognosis is generally good, with frequent complete remissions.

12. Focal Segmental Glomerulosclerosis

Often seen in association with abuse of heroin and analgesics and persons with AIDS.

Affects only certain numbers and areas of glomeruli, and the others remain normal.

Disruption of the podocytes in certain areas of glomeruli associated with heroin and analgesic abuse and acquired immunodeficiency syndrome

etiology of Focal Segmental Glomerulosclerosis

immunoglobulins M and C3

Immune deposits, primarily ____________, are a frequent finding and can be seen in undamaged glomeruli

Moderate to heavy proteinuria Microscopic hematuria

lab findings of Focal segmental glomerulosclerosis

Tubular Disorders

Disorders affecting the renal tubules

Acute Tubular Necrosis (ATN)

primary disorder associated with damage to the renal tubules

Damage to the renal tubular cells caused by ischemia or oxic agents

the etiology of this Acute Tubular Necrosis (ATN)

RTE cells ; toxic substances

Damage to the _________may be produced by DECREASED blood flow that causes a lack of oxygen presentation to the tubules (ischemia) or the presence of __________in the urinary filtrate.

shock, trauma (such as crushing injuries), and surgical procedures

Disorders causing ischemic ATN include

Shock

A condition in which the circulatory system fails to provide sufficient circulation to enable every body part to perform its function; also called hypoperfusion.

cardiac failures, sepsis involving toxigenic bacteria, anaphylaxis, massive hemorrhage, and contact with high-voltage electricity

Examples of conditions that may cause shock are

aminoglycoside antibiotics, the antifungal agent amphotericin B, cyclosporine, radiographic dye, organic solvents such as ethylene glycol, heavy metals, and toxic mushrooms

Exposure to a variety of nephrotoxic agents can damage and affect the function of the RTE cells. Substances include

hemoglobin and myoglobin

filtration of large amounts of _________is also nephrotoxic.

mild proteinuria microscopic hematuria

the presence of RTE cells and RTE cell casts containing tubular fragments consisting of three or more cells. As a result of the tubular damage, a variety of other casts may be present, including

hyaline, granular, waxy, and broad.

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Hereditary and Metabolic Tubular Disorders