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Hemolytic uremic syndrome

is a disease of two body systems. Hemolysis describes the destruction

of red blood cells. In hemolytic uremic syndrome, blood within capillaries,the smallest blood vessels in the body, begins to clot abnormally. When red

 blood cells pass through the clogged capillaries, they are sheared apart and

 broken. (hemo=blood +lysis=destruction). The second failed system, kidney

failure (uremia), occurs when urea and other waste products build up in the

 bloodstream because the kidneys cannot filter and dispose of them. (urea=a

waste chemical + emia= in the blood).

Etiology:

Enterohemmorrhagic E. coli

Pregnancy

Pneumonia

AIDS

Medicaton

Signs and Symptoms:

In E. coli-related HUS, gastroenteritis occurs with abdominal

cramping, vomiting and profuse bloody, watery diarrhea. This may cause

significant dehydration, weakness and lethargy, as well as electrolyte

imbalances because of the loss of sodium, potassium, and chloride in

the vomit and diarrhea. These symptoms may resolve before the onset of 

anemia and the kidney failure symptoms of HUS.

The anemia and uremia usually present with weakness, lethargy, and

sleepiness. Purpura or small areas of bleeding in the skin may be seen

 because of low platelet counts.

Treatment:

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HUS in children tends to be self-limiting, and supportive care is often all

that is needed. This may include intravenous fluids for rehydration and

rebalancing of electrolytes like sodium and potassium, which can be lost

with the diarrhea. Blood transfusions are only used for the most severe casesof anemia in which the hemoglobin falls below 6 or 7 g/dL (depending on

age, the normal value is 11-16).Kidney failure may be managed expectantly

(by observation and supportive care), and dialysis is not often required.

Nephrotic syndrome (NS)

is a condition that is often caused by any of a group of diseases that

damage the kidneys' filtering system, the glomeruli. The structure of the

glomeruli prevents most protein from getting filtered through into the

urine. Normally, a person loses less than 150 mg of protein in the urine in a

24-hour period. Nephrotic-range proteinuria, the urination of more than 3.5

grams of protein during a 24-hour period, or 25 times the normal amount, is

the primary indicator of NS.

Etiology:

There are a number of different disorders that can cause NS. Diabetes and, to

a lesser extent, hypertensioncan cause diffuse damage to the glomeruli and

can ultimately lead to NS.The following diseases can cause specific damage to the glomeruli and often

result in the development of heavy proteinuria and in many instances NS:

• Amyloidosis (the stiffening and subsequent malfunction of the kidney

due to fibrous protein deposit in the tissue)

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• Focal segmental glomerular sclerosis (FSGS) (creates scar tissue in

the glomerulus, damaging its protein-repellant membrane)

• Glomerulonephritis (GN)

•

IgA nephropathy (Berger's disease) (deposit of specificimmunoglobulin A causing an inflammatory reaction and leading to

glomerulonephritis)

• Pre-eclampsia (rarely associated with NS, more often associated with

heavy proteinuria)

Signs and Symptoms:

In addition to proteinuria, there are three main symptoms of nephrotic

syndrome associated with protein leaking into the urine:• Hypoalbuminemia (low level of albumin in the blood)

• Edema (swelling)

• Hypercholesterolemia (high level of cholesterol in the blood)

Treatment:

Diuretics

Anti-hypertensive medications

Proper diet

Supportive measures

Polycystic kidney disease (PKD)

is a genetic disorder characterized by the growth of numerous cysts in

the kidneys. The kidneys are two organs, each about the size of a fist,

located in the upper part of a person's abdomen, toward the back. Thekidneys filter wastes and extra fluid from the blood to form urine. They also

regulate amounts of certain vital substances in the body. When cysts form in

the kidneys, they are filled with fluid. PKD cysts can profoundly enlarge the

kidneys while replacing much of the normal structure, resulting in reduced

kidney function and leading to kidney failure.

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Two major inherited forms of PKD exist:

• Autosomal dominant PKD is the most common inherited

form. Symptoms usually develop between the ages of 30 and 40, but

they can begin earlier, even in childhood. About 90 percent of all PKD

cases are autosomal dominant PKD.

The cysts grow out of nephrons, the tiny filtering units inside the kidneys.

The cysts eventually separate from the nephrons and continue to enlarge.

The kidneys enlarge along with the cysts-which can number in the

thousands-while roughly retaining their kidney shape. In fully developed

autosomal dominant PKD, a cyst-filled kidney can weigh as much as 20 to

30 pounds.

People with autosomal dominant PKD also can experience the following

complications:

• Urinary tract infections, specifically in the kidney cysts

• Hematuria (blood in the urine)

• Liver and pancreatic cysts

• Abnormal heart valves

• High blood pressure

• Kidney stones;

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• Autosomal recessive PKD is a rare inherited form. Symptoms

of autosomal recessive PKD begin in the earliest months of life, even

in the womb.Autosomal recessive PKD is caused by a mutation in the autosomal

recessive PKD gene, called PKHD1. We all carry two copies of every

gene. Parents who do not have PKD can have a child with the disease if 

 both parents carry one copy of the abnormal gene and both pass that gene

copy to their baby. The chance of the child having autosomal recessive

PKD when both parents carry the abnormal gene is 25 percent. If only one

 parent carries the abnormal gene, the baby cannot get autosomal recessivePKD but could ultimately pass the abnormal gene to his or her children.

Children with autosomal recessive PKD experience high blood

 pressure, urinary tract infections, and frequent urination. The disease usually

affects the liver and spleen, resulting in low blood cell counts, varicose

veins, and hemorrhoids. Because kidney function is crucial for early

 physical development, children with autosomal recessive PKD and

decreased kidney function are usually smaller than average size.

GLOMERULONEPHRITIS

Glomerulonephritis is an inflammatory disease of the kidneys,

specifically the glomeruli. The glomeruli are the part of the kidneys in

charge of filtering waste from the bloodstream. Glomeruli can becomeinflamed for a variety of reasons. Once inflamed, the glomeruli cannot filter 

waste properly and become leaky, which allows protein and blood to pass

into the urine.

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Symptoms of glomerulonephritis include blood in the urine, foamy

urine, and edema (swelling) of the legs, abdomen and body. As the disease

 progresses and the kidneys become more damaged, additional symptoms

may appear, including abdominal pain, diarrhea, fever , aches, loss of appetite, and shortness of breath. Toward the end stages of the disease, more

serious symptoms can occur, such as excessive urination, nosebleeds, bloody

stool, and vomiting blood. Glomerulonephritis can even progress to kidney

(renal) failure.

Treatment for glomerulonephritis includes blood pressure medication,

steroids, and immunosuppressant drugs. Plasmapheresis (to remove

antibodies against glomeruli from the blood), dialysis, or a kidney transplant

may also become necessary depending upon the cause and severity of the

condition. Changes in diet may also help to alleviate symptoms

KIDNEY STONES

A kidney stone is a hard, crystalline mineral material formed within

the kidney or urinary tract. Kidney stones are a common cause of blood in

the urine (hematuria) and often severe pain in the abdomen, flank, or groin.

Kidney stones are sometimes called renal calculi.

What causes kidney stones?Kidney stones form when there is a decrease in urine volume and/or an

excess of stone-forming substances in the urine. The most common type of 

kidney stone contains calcium in combination with either oxalate or 

 phosphate. About 75% of kidney stones are calcium stones. Other chemical

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compounds that can form stones in the urinary tract include uric acid,

magnesium ammonium phosphate (which forms struvite stones; see below),

and the amino acid cystine.

Dehydration from reduced fluid intake or strenuous exercise without

adequate fluid replacement increases the risk of kidney stones. Obstruction

to the flow of urine can also lead to stone formation. In this regard, climate

may be a risk factor for kidney stone development, since residents of hot and

dry areas are more likely to become dehydrated and susceptible to stone

formation.

Signs and symptoms:

report the sudden onset of excruciating, cramping pain in their low

 back and/or side, groin, or abdomen. Changes in body position do not

relieve this pain. The abdominal, groin, and/or back pain typically waxes

and wanes in severity, characteristic of colicky pain (the pain is sometimes

referred to as renal colic). It may be so severe that it is often accompanied by

nausea and vomiting. Kidney stones also characteristically cause blood in

the urine. If infection is present in the urinary tract along with the stones,

there may be fever andchills. Sometimes, symptoms such as difficulty

urinating, urinary urgency, penile pain, or testicular pain may occur due to

kidney stones.

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Treatment:

Most kidney stones eventually pass through the urinary tract on their 

own within 48 hours, with ample fluid intake. Ketorolac (Toradol), an

injectable anti-inflammatory drug, and narcotics may be used for pain

control when over-the-counter pain control medications are not effective.

Intravenous pain medications can be given when nausea and vomiting are

 present.

For kidney stones that do not pass on their own, a procedure called

lithotripsy is often used. In this procedure, shock waves are used to break up

a large stone into smaller pieces that can then pass through the urinary

system.

Surgical techniques have also been developed to remove kidney stones when

other treatment methods are not effective. This may be done through a small

incision in the skin (percutaneous nephrolithotomy) or through an

instrument known as an ureteroscope passed through the urethra and bladder 

up into the ureter.