Renal 2

GFR 60-89

GFR 15-29

A glomerular disorder characterized by severe loss of protein in the urine, typically leading to accumulation of fluid (edema) and low levels of the protein albumin in the blood.

Increased permeability of glomerulus

Can occur at any age. Most common between the ages of 18 months and 4 years

Incidence: Peak incidence in children (3-14), mostly winter and spring, 1~4 weeks following Group A Strep. Infection (skin or pharyngeal)

Etiology: Glomerular trapping of circulating anti-streptococcal immune complexes. (Type 3 hypersensitivity)

Pathology: LM: Enlarged, hypercellular glomeruli with endothelial and mesangial cell proliferation. Acute inflammation. EM: subepithelial immune complexes “humps”. IF: “lumpy-bumpy” deposits of IgG (Ag-Ab complex). and C3 along GBM.

Loss of renal function characterized by a rapid decline in glomerular filtration rate (GFR) over hours to days.

Is often considered to be reversible.

Is manifest clinically by multiple cysts evident at birth.
Autosomal recessive
Results in death shortly after birth

Definition:
The death of tubular epithelial cells accompanied by acute diminution or loss of renal function

Is the most common cause (50% cases) of acute renal failure (ARF).

Causes:
Ischemic ATN (due to tubular hypoperfusion): Hypotension, Obstetric (birth-related) complications, Surgery, etc.
Nephrotoxic ATN: poisons, Hb, myoglobin, drugs, heavy metals

GFR 30-59

Clinical: Fever, malaise, hematuria, smoky colored urine (hemoglobin→hematin), oliguria, mild to moderate ↑BP, mild proteinuria & periorbital edema

Lab:
Nephritic urine with RBC casts.
↑ASO titers (evidence of streptococcal infection)
Serologic evidence of recent infection.
↓ C3

Prognosis:
Children - excellent prognosis (95% recover)
Adults - worse prognosis (60% recover)
Some develop progressive disease.

GFR < 15 or dialysis

Morphology:
LM: Glomeruli are normal
EM: fusion of the podocytes
IF: No Ig or complement deposits detected
Positive fat stain in the glomerulus and tubules

Treatment: Prednisone with ACE inhibitor

Prognosis:
Response dramatically to corticosteroids in 90% of cases

Most Common conditions which affect blood flow to kidney
Shock, congestive heart failure, use of NSAIDs, etc

Proteinuria
Hematuria
Hypertension
Edema
Oliguria
Azotemia
Hypoalbuminemia

Major causes are Diabetes mellitus and hypertension.

Urinalysis: waxy and broad casts, proteinuria (tubule is dilated)

Uremia can be used as a synonym, or can be used to indicate severe azotemia, in which symptoms are produced

Signs and symptoms:

Endocrine and electrolyte findings:
Hypocalcemia and osteomalacia due to Vitamin D3 def. → loss of Ca2+, ↓ absorption of Ca++ in GI tract
Metabolic acidosis (retention of organic acids, ↑ anion gap)
Hyperkalemia (transcellular shift due to acidosis and ↓ K+ excretion)
Hypertriglyceridemia

Cardiovascular findings: uremic pericarditis, hypertension (salt retention, volume overload), congestive heart failure with pulmonary edema (volume overload)

Hemotologic findings:
Anemia (↓ erythropoeitin synthesis → ↓ RBC)
Bleeding (thrombocytopenia)

Infection due to leukopenia and lymphopenia

Skin disorders: pruritus, bruising (thrombocytopenia), pallor (anemia)

Neuromuscular findings: asterixis, headache, fatigue, uremic encephalopathy

GI findings: anorexia, nausea, vomiting, peptic ulcer

Glomerular diseases are characterized by-
Massive proteinuria (>3.5g/24h)
Massive hypoalbuminemia
Hyperlipidemia
Generalized pitting edema
Fatty casts with Maltese crosses

Most common form of childhood kidney cancer.
Usually presents in the first 5 years of life peak age ( 2-4 ).

Manifestations: typically presents with abdominal mass, may cause hematuria, abdominal pain, fever, and anridias.

WAGR (Wilms’ tumor, Aniridia, Genital anomalies, Mental Retardation)

Treatment and Prognosis:
With modern chemotherapy and surgery, the long term survival more than 80%; but the prognosis is worse in those with metastasis and older kids

Metastasis to lung occurs early; liver, bone and brain are rare, lymphatic spread is uncommon

Causes:
Acute renal failure
Nephritis
Renal artery obstruction
Kidney stones
Nephrotic syndrome
Polycystic kidney disease
Diabetic nephropathy
Hypertension
Atherosclerosis

Acute poststreptococcal glomerulonephritis
Goodpasture’s syndrome (with hemoptysis, anti-GBM antibodies)
IgA nephropathy (a.k.a., Berger’s disease, deposition of the IgA antibody in the glomerulus)

Alport’s syndrome (split GBM)

Three major categories:

Prerenal azotemia (MC, conditions which affect blood flow to kidney)
Shock, congestive heart failure, use of NSAIDs, etc

Renal azotemia (Intrinsic renal disease)
Target glomeruli (Glomerulonephritis), tubules (toxic or ischemic acute tubular necrosis (ATN), interstitium (drugs)

Postrenal azotemia (ureteral or bladder conditions)
Prostate hyperplasia, urolithiasis, tumor obstructing
Oliguria (decreased urine production, quantified as less than 400~500 ml/day)

Also known as Nephroblastoma.

Definition: a cancer arising from the embryonic kidney that occurs in young children, especially, in the age range 3-8 years.

Etiology: unknown. But WT1 gene mutation on Chromosome 11 account for 20%, WTX (a gene on the X chromosome) is inactivated in up to 30% of cases.

Target glomeruli (Glomerulonephritis), tubules (toxic or ischemic acute tubular necrosis (ATN), interstitium (drugs)

Definition: a kidney cancer that originates in the lining of the proximal convoluted tubule (within the renal cortex).
Are responsible for 90 ~ 95 % of all primary renal neoplasm.
Most common in the 6th-7th decades of life
Men affected twice as common as women.
Etiology: unknown.

Characterized by the presence of multiple cysts (polycystic) in both kidneys
Autosomal dominant [mutation of PKD1,(85%) or PKD2 (15%) gene] with hypertension
Bilaterally, the kidneys are marked enlarged
Subarachnoid hemorrhage due to ruptured berry aneurysm of the circle of Willis

Is a systemic disease with cysts in other organs such as the liver (which may lead to cirrhosis), pancreas

Clinical manifestations:
Hypertension
Hematuria
Palpable renal mass
Progression to renal failure

Due to proliferative inflammatory response within the glomeruli and damage to the capillary wall.

Glomerular diseases characterized by
Hematuria (RBC casts in urine)
Oliguria
Periorbital puffiness (due to retention of salt)
Mild to moderate proteinuria (<3.5g/24h)
Azotemia [presence of nitrogenous wastes (urea, creatinine, etc) the blood, aka., Uremia]

Signs and Symptoms:
Typically does not produce specific signs or symptoms
Initiation phase (~36hr): Oliguria (<400ml/day)
Maintainace phase: Oliguria, salt & water overload, high BUN, hyperkelemia, metabolic acidosis
Recovery phase: polyuria (>3L/day), hypokelemia

Diagnosis: rule out other reasons for ARF, such as prerenal, postrenal, and renal ARF

Treatment: treatment of the factors that precipitated ATN
Prognosis: Because tubular cells have the capacity to replace themselves, the overall prognosis for ATN is quite good if the cause is corrected

Risk factors: smoking, obesity, family history, von Hippel-Lindau disease, occupational exposure to cadmium

Manifestations:
hematuria, abdominal pain, abdominal mass, 2nd polycythemia; paraneoplastic syndrome; hypertension
fever, and unexplained weight loss

Treatment and Prognosis:
Surgery is the treatment of choice
5 year survival rate is 90%, drops to 30% if metastasis has occurred.

Minimal change diseases (children)
Focal segmental glomerular sclerosis (AIDS, IV drug abuser)
Membranouse glomerulonephritis

Most Common:
Drugs (gold, penicillamine, “street heroin”)
Diabetic nephropathy
Systemic lupus erythematosus (SLE)

A disorder of glomeruli characterized by tissue swelling (edema), high blood pressure, and the presence of red blood cells in the urine.

Proliferative inflammatory response

Most common cause of nephrotic syndrome in children (65%)
Peak incidence 2~6 years of age
Sometimes follows a respiratory infection or vaccination
↑ incidence in patients with Hodgkin lymphoma

Etiology and Pathogenesis:
Probably cellular immune reaction of with T cell production of a lymphokine that destroys the negative charge barrier.

GFR > 90

Azotemia (increased severe BUN and creatinine)
Acidosis (accumulation of sulfates, organic acids and phosphates)
Hyperkalemia (transcellular shift due to acidosis and ↓ K+ excretion)
Body fluids disturbances
early, inability to concentrate urine → polyuria
later, inability to dilute urine → oliguria (<400ml/d) or anuria (<100ml/d)
Hypocalcemia (loss of 1a-hydroxylase enzyme → ↓ active form of vitamin D)
Anemia (↓ secretion of erythropoietin → ↓ RBC)
Hypertension caused by hyperproduction of renin and ↑ volume (due to oliguria)

Other clinical manifestations: Uremia (severe azotemia)
Anorexia, nausea, vomiting, fibrinous pericarditis, bleeding, hypersomnia, etc

Prostate hyperplasia, urolithiasis, tumor obstructing

Common
Hypoalbuinemia
Edema
Lipiduria
Hyperlipidemia