With the presence of PKD, cysts develop in both kidneys.
There may be just a few cysts or many, and the cysts may vary from small
to large sizes (as large as a soccer ball). The liver is the other organ
commonly affected as well.
In general, cysts cause problems because of their size
and the space they occupy. Large cysts may cause pain.
ADPKD
Inheritance
The dominant form of the disease (ADPKD) is passed from
one generation to the next by an affected parent. There is no carrier
state with a dominant gene; it does not hide and come out in a later
generation. Each child of an ADPKD parent has a 50 percent chance of
inheriting the disease. When an individual does not have the gene for
ADPKD, he/she does not have the disease and therefore cannot pass the
gene on to the next generation. Scientists have also discovered that
approximately 10% of the PKD patient community become involved through
spontaneous mutation, and not through inheritance. This means that instead
of inheriting the ADPKD gene from a parent with the disease, the gene
mutates by itself for no known reason. It is important to know that
even with a true spontaneous mutation, a newly affected person will
still pass the mutated gene on to his/her children. ADPKD equally affects
men and women, regardless of age, race, or ethnic origin.
Diagnosis
The diagnosis is confirmed by 3 main clinical tests: ultrasound,
computed tomography (CT) or magnetic resonance imaging (MRI).
Ultrasound is the best screening and the most readily
available non-invasive test for ADPKD. Although the majority of ADPKD
patients have cysts by the time they are adults, it is only after the
age of 30 that a negative ultrasound probably means that someone has
less than a 5 percent chance of having ADPKD.
Signs and Symptoms
Early in the disease there generally are no symptoms at
all. Often the first sign of ADPKD is high blood pressure, blood in
the urine or a feeling of heaviness/pain in the back, sides or abdomen.
Sometimes the first sign is urinary tract infection and/or kidney stones.
Kidney stones occur in about 20 percent to 30 percent
of people with ADPKD compared to 8 percent to 10 percent in the general
population.
Although everyone with the ADPKD gene develops kidney
cysts, not everyone progresses to kidney failure, and if they do it's
rarely before the age of 40.
Other associated conditions
5 to 10% of ADPKD patients may develop intracranial aneurysms
(outpouching in a blood vessel in the brain). These aneurysms seem to
cluster in certain families. That is, if a member of your family has
an aneurysm or has ruptured an aneurysm, you may be at a higher risk
of having an aneurysm also. Aneurysms can leak or rupture. In these
events the symptoms can include sudden severe headache, pain in moving
the neck, nausea and vomiting, and even loss of consciousness. All such
symptoms require immediate medical attention.
Treatment
No specific treatment is available at this stage. However
the functions of the kidney can be better maintained by control of blood
pressure. A low protein diet should be considered in the treatment of
failing kidneys. Avoiding large amounts of red meat can help protect
your kidneys. Excessive amounts of salt should also be avoided.
Pain Management
There have been some exciting preliminary results in the
use of laparoscopic surgery to de-roof the cysts and thereby reduce
pain in ADPKD patients. This procedure is conducted only in patients
whose symptoms strongly suggest that their pain is caused by the cysts,
and who have cysts larger than five centimeter in diameter. This procedure
is only to reduce pain, not to preserve kidney function.
Renal
Stone
Introduction
A kidney stone is a solid material deposited in the middle
of the kidney, where the urine collects before it flows into the ureter.
It begins as a small deposit and then gradually builds up into a solid
mass. Either kidney can develop such process. Renal stones occur in
around 5% of population, the recurrence rate is about 40 to 70%. Fifty
percent of patients may have symptom recurrence within 5 years of discovery.
Causes of kidney stone formation
The commonest cause of kidney stone is idiopathic which
means nobody knows why it occurs. Kidney stones may be formed due to
an over concentration of calcium in blood (hypercalcemia), increased
urine calcium concentration (hypercalciuria), high urinary acidity which
decreases uric acid solubility (hyperuricosuria) or due to certain metabolic
disorders (e.g. hyperparathyroidism or abnormal oxalate metabolism resulting
in hyperoxaluria). A rare cause is cystinuria, which is a rare inherited
error of the renal tubular transport system. People living in the warmer
climate may develop kidney stones more often. This may be due to the
loose of body fluid through sweat and less production of urine, which
results in the concentration of calcium in the body. Man is more commonly
affected then woman. An excessive amount of calcium in the urine may
be due to excessive intake of calcium either through food and direct
ingestion of calcium as a supplement. If the outflow of urine is obstructed
for any reason, as in the case of stricture of the urethra or enlargement
of the prostate gland, may increase the production of kidney stones.
Symptoms and signs
A kidney stone may lie dormant for years without the patient
being aware of it. Even big stones lodged in the kidney often produce
no symptoms. When the kidney stone is discovered in its acute phase
generally during the passage from the kidney to the bladder, patient
usually has severe pain that classically begins from the side of back
radiating to groin. The pain is usually stabbing in nature and comes
in waves. In some cases the pain may be accompanied with nausea, vomiting,
chills and sweating. The pain stops whenever the stone stops moving,
after it reaches the bladder or after it is expelled.
Diagnosis
KUB x-ray, IVU x-ray, ultrasound, or a CT scan are the
tests to determine the presence of a kidney stone. Other tests such
as the urinalysis, urine culture, 24-hour urine test, blood tests are
used to and will guide the treatment modalities. There are different
types of kidney stones including calcium oxalate (75%), struvite (15%),
uric acid (8%), and calcium phosphate (5%) and cystine xanthine (1%).
Treatment of kidney stone
Treatment of Kidney Obstruction Complicated
by Infection
This is an urgent medical problem. Hospital admission
is likely.
Urgent relief of the obstruction and antibiotic treatment.
Potent antibiotics may have to be administered intravenously. Because
the kidney excretory function is adversely affected in obstruction,
antibiotics work poorly unless the obstruction is relieved and infected
urine drained from the kidney.
Glomerulonephritis
Introduction
Glomerulonephritis is the term used to describe a group
of kidney diseases where the filtering part of the kidney (glomerulus)
is inflamed. Sometimes the name is shortened to nephritis. In general
there are two type of glomerulonephritis: acute glomerulonephritis and
chronic glomerulonephritis. With acute glomerulonephritis, there is
a tendency for spontaneous recovery. In the case of chronic glomerulonephritis,
progressive damage of kidney tissue occurs which usually resulted in
kidney failure. Signs of the disease may include protein and blood in
the urine and usually high blood pressure. Chronic glomerulonephritis
is the most common cause of chronic kidney failure leading to end stage
renal disease (ESRD). There is another form of glomerulonephritis called
rapidly progressive glomerulonephritis (RPGN). RPGN leads to kidney
failure fairly quickly. This disease is of unknown cause, it is irreversible,
can appear suddenly, and is characterized by decrease in urine output
and worsening of kidney function test.
Causes of Acute Glomerulonephritis.
Patients with acute glomerulonephritis often have evidence
of a recent infection. The most common infection causing glomerulonephritis
is a streptococcal infection of the throat or skin. Other serious bacterial
and viral infections may also be associated with acute glomerulonephritis.
Symptoms and signs of Acute Glomerulonephritis
Acute glomerulonephritis is usually a disease of children,
but can occur at any age. Males have the disease more frequently than
females. It usually occurs about 10 days after the start of an infection
of the throat or skin. The patient will frequently note a fall in the
urine output and the urine looks "smoky or rusty" (often described as
coffee or cola colored). Puffiness over the face, eyelids and hands
occurs due to fluid retention. Shortness of breath and cough can occur.
High blood pressure is common. In addition, systemic and hereditary
diseases have symptoms and signs, which are similar to acute glomerulonephritis.
The diseases in this category include systemic lupus erythematous and
many types of vasculitis. However, the above diseases generally do not
improve and treatment is needed.
Causes of Chronic Glomerulonephritis
There are many causes for the general term of chronic
glomerulonephritis. A wide variety of diseases affecting the glomerulus
(the filtering unit of the kidney) have a common picture. There is usually
a prolonged course, often with years of no symptoms, while progressive
scarring of the kidney is silently occurring. Occasionally, an acute
glomerulonephritis can have a quiet phase after the original illness
and appear many years later as chronic glomerulonephritis.
Symptoms and signs of Chronic Glomerulonephritis
This consists of wide variety of diseases of the kidney
that generally have a longer course. Usually patients do not any symptoms.
During this time, there is progressive damage of kidney tissue. In the
early stages, frequently the only findings are an abnormal urinalysis
(for example, red blood cells, white blood cells and protein in the
urine). High blood pressure can occur. As the disease progresses, there
is swelling of the legs (edema) and persistent high blood pressure.
Signs of chronic kidney failure (uremia) are noted when there is a severe
loss of kidney function. The signs include loss of appetite, nausea
and vomiting, extreme fatigue, difficulty sleeping, itching and dry
skin, muscle cramps, especially at night.
Diagnosis of Glomerulonephritis
The signs and symptoms noted above are the first clues
to the diagnosis. In acute glomerulonephritis, blood tests can indicate
a recent streptococcal infection. Other forms of glomerulonephritis
can be detected by special blood tests that show the type of damage
occurring in the kidney without learning the exact cause of the injury.
Also, cultures of the throat, skin, or other known areas of local infection
can identify the bacteria, which is causing the problem. Examination
of the urine can show the presence of blood, protein, and other elements.
Other blood tests that point out inflammation in the kidney can show
how much kidney function is lost. Often it is necessary to do a kidney
biopsy (removal of a tiny piece of tissue by a special needle). This
is done under local anesthesia to establish the exact diagnosis and
determine the short and long-term outlook for the patient. Sometimes
the biopsy is necessary for the doctor to plan the best possible treatment
for the patient.
Treatment for Glomerulonephritis
There is no specific treatment for acute glomerulonephritis.
This illness will usually heal completely within 3-12 months after onset.
With both acute and chronic glomerulonephritis, it is important to treat
high blood pressure. Uncontrolled high blood pressure can lead to a
rapid decrease in kidney function. Diuretics often are used to control
excess retention of body fluid. In chronic glomerulonephritis, steroids
and other drugs have been used. However, this treatment may not be useful
for treating the cause of chronic glomerulonephritis. A special blood
filtering process (plasmapheresis) has been used in some special types
of glomerulonephritis. Restricting protein, salt and sometimes potassium
in the diet may be a major part in the conservative treatment used by
your doctor. Close follow up with your doctor is necessary so that he/she
can treat the known complications of chronic kidney disease.