Diseases Requiring Transplantation


There are many diseases that affect the kidneys. Some diseases are mild enough that can be treated with medications or medical procedures. Other diseases can progress to the point where the kidneys suffer permanent damage and can no longer suitably filter blood. This is known as kidney failure, and requires the patient to get dialysis or a kidney transplant before his or her life is endangered.

Type 1&2 Diabetes

Diabetes is a disease that leads to high levels of glucose (sugar) in the bloodstream, which forces the kidneys to work harder to filter the blood. Over time, this filtering mechanism begins to fail from being overworked, and waste products begin to accumulate in the bloodstream. This condition is known as End-Stage Renal Disease, and if not treated in a timely manner will lead to complete kidney failure.

Hypertension

Hypertension is the medical term for high blood pressure. This pressure, whatever the cause may be, damages blood vessels over time, and the thin filtering vessels within the kidneys are no exception. After long periods of being exposed to high blood pressure, the kidneys begin to lose their filtering abilities, leading to high levels of waste in the bloodstream.

Acquired Obstructive Nephropathy

Obstructions in the urinary tract may lead to damage to the kidneys’ internal structure. Some causes leading to these obstructions may include: anatomic abnormalities, urethral valve problems, prostate enlargement, prostate cancer, pelvic tumors, kidney stones, and injuries.

Acute Tubular Necrosis

A tubule is a small vessel within the kidney that carries urine to the ureter, while reabsorbing most of its water. Due to their high metabolism, a short obstruction to the tubules’ oxygen supply will result in the death of these tissues. Causes may include kidney poisoning, injury, low blood pressure, septic shock, or blockage of blood vessels. Renal failure occurs if ATN is not treated quickly.

Alport Syndrome

The glomeruli are tiny capillaries in the kidneys that filter waste products from the blood. Alport Syndrome is a genetic disorder that affects the structure of these capillaries, hindering their ability to filter waste, and leading to blood in the urine during childhood. It causes progressive kidney damage because normal tissue is replaced by scar tissue over time, so transplant may be required as early as 15 years of age.

Amyloidosis

Amyloidosis is a condition that leads to certain proteins to become incapable of dissolving in water. These free-floating proteins end up forming accumulations in organs (known as amyloids), and the kidneys are typically the first ones to suffer. When enough buildup has occurred, the kidneys become unable to filter urine properly. If left untreated, amyloidosis will lead to End-Stage Renal Disease.

Analgesic Nephropathy

Analgesics are medications used to alleviate pain, also known as painkillers. The excessive and prolonged use of these medications may lead to damage to the internal structures of the kidney. Painkillers that contain aspirin, phenacetin, paracetamol, ibuprofen, or acetaminophen have been known to cause nephropathy when taken daily for over 3 years. If you consume large amounts of painkillers you should let your doctor know so he may check for kidney damage.

Calcineurin Inhibitor Nephrotoxicity

A calcinerunin inhibitor (CNI) is a class of drug that is used as an immunosuppressant (also known as an anti-rejection medication) for many transplant recipients. They work by blocking the activation of white blood cells in the immune system. Introduced in 1983, it has been gradually substituted for more harmless alternatives, since CNIs have a tendency of being toxic to the kidneys (nephrotoxicity) during prolonged use. Consult with your doctor if you are currently taking ciclosporin, pimecrolimus, or tacrolimus, as these three immunosuppressants are CNIs, and may lead to kidney damage.

Chronic Glomerulonephritis

The glomeruli are capillary structures inside the kidneys that are in charge of the first step in the blood filtration process. Certain genetic diseases, immune system diseases, or many other causes can lead to the inflammation of the glomeruli over time (chronic), which, if untreated, results in the kidney’s failure to effectively filter blood. Early signs include blood or protein in the urine, and hypertension.

Chronic Pyelonephritis

The urinary tract is susceptible to infections from time to time. Repeated occurrences over time may cause the infection to reach further up the ureters, until it reaches the kidney itself. When this happens, the nephrons (the functional units of the kidney) will get inflamed, obstructing the kidney’s ability to perform its functions. Possible outcomes include an abscess around he kidney, septic shock, kidney failure, or death if not treated promptly.

Chronic Reflux Nephropathy

In a normal urinary system the urine drains from the kidneys into the bladder via two tubes called the ureters. Urine is stored in the bladder until the person is ready to go to the bathroom. Certain anatomical anomalies at birth, injuries, or infections may affect this normal flow of urine, forcing it from the bladder back up into the kidneys. This retrograde flow of urine can cause hypertension, protein in the urine, chronic renal failure, and end-stage kidney disease.

Congenital Obstructive Uropathy

In a normal urinary system the urine drains from the kidneys into the bladder via two tubes called the ureters. Urine is stored in the bladder until the person is ready to go to the bathroom. Some people may be born with anatomic anomalies that obstruct the normal flow of urine, causing a buildup in the kidneys. This blockage may cause kidney dysfunction.

Focal Glomerular Sclerosis

The glomeruli are the capillary structures inside the kidneys that are in charge of the first step in the blood filtration process. This disease consists of the scarring of the glomerular tissues, although “focal” means only some of them are scarring, not all of them. This scarring disrupts their filtering ability, and leads to large amounts of protein in the urine. FGS may be caused by diabetes, drug use, infections, or AIDS, although many times the cause is unknown.

Goodpastures Syndrome

This is a disease where your immune system mistakenly attacks certain tissues found only in the kidneys and lungs. This will cause bleeding from the lungs and kidney failure if not treated promptly with immunosuppressants. People with this disease generally start coughing up blood, and may feel a burning sensation when urinating months later. Blood in the urine or foamy urine follows shortly thereafter.

Gout

Uric acid is normally found in the bloodstream as the byproduct of the breakdown of certain foods. It is filtered by the kidneys. When uric acid levels are higher than normal, they may crystalize and subsequently collect in joints, tendons, and other tissues. This leads to arthritis-like symptoms with a red, swollen, hot joint that’s tender to the touch. The big toe is the most commonly affected joint, but the crystals may also accumulate in the kidneys, causing kidney stones and urate nephropathy.

Hemolytic Uremic Syndrome

This disease is most commonly triggered by consuming food or water that’s been contaminated with E. coli, which causes bloody diarrhea. Medications or other infections are also possible causes for HUS. This syndrome produces toxic substances in the digestive system which destroys red blood cells, lowers platelet count, and causes acute kidney failure.

Henoch-Schoenlein Purpura

Vasculitis is an inflammation of capillaries and other small blood vessels. HSP is vasculitis of the skin due to an abnormal immune system reaction. This causes purpura: purplish dots usually found on the legs, buttocks, and other parts of the skin. Vasculitis may also occur in kidneys (causing blood and protein loss in urine), joints (triggering aches and arthritis), and intestines (leading to abdominal pain). HSP resolves after several weeks, and often requires no treatment. However, a very small percentage of patients will develop irreversible kidney damage.

Hepatorenal Syndrome

Commonly called HRS, this disease affects patients who already suffer from one of three liver diseases: alcoholic hepatitis, fulminant liver failure, or cirrhosis. It is thought that the liver’s poor condition indirectly obstructs blood flow to the kidneys, constricting their vessels and quickly hampering their ability to remove waste products from the bloodstream. The life expectancy of patients with HRS is very low, unless a liver transplant takes place in a timely manner, which would restore proper blood pressure to the kidneys.

Hypertensive Nephrosclerosis

This disease is common in patients with chronic hypertension. Over time, their high blood pressure creates a thickening layer of cartilage-like material within the kidneys’ arteries. The glomeruli (capillary structures in charge of the first step in the blood filtration process) eventually stiffen, resulting in protein and blood in the urine. Renal failure occurs if the cause of the high blood pressure is left untreated.

IGA Nephropathy

The glomeruli are tiny kidney capillary structures in charge of the first step in the blood filtration process. With this disease, the glomeruli become inflamed and damaged by the accumulation of Immunoglobulin A (IgA), an antibody usually found in mucosal fluids. The inflammation of the glomeruli may cause the kidneys to leak blood and protein into the urine. Over several years, IgA nephropathy leads to total kidney failure. Henoch-Schoenlein Purpura is similar to IgA nepropathy, but with additional symptoms.

Lithium Toxicity

Lithium may be prescribed by psychiatrists as a mood-stabilizing drug. People who consume this drug should have regular tests performed to measure their kidney function because it has a tendency to impede concentration of urine in the kidneys, leading to excessive loss of water through urine. Lithium overdose (toxicity) may cause kidney failure, among many other symptoms.

Malignant Hypertension

Also known as a “hypertensive emergency”, malignant hypertension occurs when sudden high blood pressure causes organ failure noticed by abrupt hemorrhaging in one or both retinas of the eye. Kidneys normally constrict and dilate their blood vessels to auto-regulate blood pressure independently from the rest of the body. However, this ability weakens after several years of hypertension, making the kidneys vulnerable to spikes in blood pressure. This may cause blood and protein in the urine, or total kidney failure.

Medullary Cystic Kidney Disease

This is an inherited disorder where the affected person suffers from scarring (fibrosis) of kidney tissue. This scarring replaces the functional cells of the kidney with unnecessary connective tissue, impairing the kidney’s ability to reabsorb water and other nutrients from urine. This loss of water leads to excessive urination, low sodium levels in the body, constant thirst, and salt cravings. Some individuals also develop cysts (pockets filled with fluid) in the medulla of the kidneys. Kidney failure eventually develops, requiring dialysis or a kidney transplant.

Membranous Glomerulonephritis

The glomeruli are tiny capillary structures in the kidney in charge of the first step in the blood filtration process. The walls of the glomeruli allow small molecules through (water, salts and sugars) and block large molecules (proteins). This disease is caused when the immune system mistakenly attacks these walls, opening large pores between them, allowing protein to “leak” through. Swelling of the skin through accumulation of fluids (edema) is a common symptom.

Nephritis

The nephron is the functional unit of the kidney. Its job is to first filter out waste from the blood (in the glomeruli), and then reabsorb necessary nutrients from this waste (in the tubules), creating urine. A normal kidney has from 800,000 to 1.5 million nephrons. Nephritis is an umbrella term to describe a condition where the nephrons become inflamed, hindering their ability to either filter out waste from the blood (glomerulonephritis), or reabsorb important nutrients from the filtered waste (interstitial nephritis). There are other, less common forms of nephritis.

Nephrolithiasis

This is the medical term for kidney (nephro-) stones (-lithos). They are formed when the concentration of minerals in the urine is too high, leading to crystallization of calcium and other minerals in the kidney. Sometimes these stones will flow out in the urine. If they grow above 3mm, they may become lodged in the ducts within the kidney or ureters, causing great pain, nausea and vomiting. Treatment includes pain management and anti-inflammatory drugs to promote passing the stone in the urine. Severe cases may require surgical intervention to prevent urine congestion, and subsequent damage to the kidney.

Hereditary Oxalosis

Oxalate is a chemical found in the body and in food. A normal body clears excess oxalate through urine. People with hereditary oxalosis produce too much of this chemical, leading to kidney stones, which tend to be severe and damage the kidneys at an early age. After kidney damage has occurred, the oxalate is no longer expelled in urine, and it accumulates in joints, bone, and bone marrow.

Polycystic Kidney Disease

The nephron is the functional unit of the kidney, where waste is filtered from the blood and then useful nutrients are reabsorbed from this waste. PKD is a genetic disease where small fluid-filled sacs (cysts) form in the walls of some nephrons. These cysts grow by accumulating more fluid over time, constricting nearby nephrons and obstructing their function. When multiple cysts grow large enough (hence the name polycystic), they begin to compromise kidney function, resulting in eventual kidney failure. Polycystic kidneys can grow extremely large in size, and they may also affect the liver and pancreas.

Progressive Systemic Sclerosis

Sclerosis occurs when the body replaces normal organ tissue with connective tissue, often stiffening the affected structure and preventing its normal function. In PSS, the cause for this sclerosis is thought to be an autoimmune reaction, where the patient’s immune system attacks the body, triggering buildup of connective tissue, replacing functioning organ cells. PSS affects the skin at first, making it appear scaly red and tight. PSS eventually affects the heart, lungs, digestive tract, and kidneys, causing sclerosis in the small vessels of these essential organs and ultimately impairing their function.

Rapid Progressive Glomerulonephritis

The glomeruli are microscopic structures in the kidney in charge of filtering waste products from the blood. This disease consists of a rapid deterioration of their function, where at least 50% of their filtration rate is lost within 3 months. The cause is often caused by autoimmune problems, where the immune system attacks the delicate glomerular tissues. The damage results in large amounts of blood in the urine, protein in the urine, high blood pressure, and low urinary output. If left untreated, full kidney failure follows within a few months.

Renal Artery Stenosis

Renal artery stenosis occurs when the artery that supplies the kidney with blood becomes narrower, decreasing the blood supply to the kidney, and increasing blood pressure in the rest of the body. The primary symptom is high blood pressure that can’t be controlled with drugs. With low blood supply, the kidney may become atrophied, and eventually go into kidney failure. This narrowing may occur due to accumulation of plaque inside the vessels, or abnormal growths occurring in these vessels (fibromuscular dysplasia).

Renal Artery Thrombosis

This occurs when the artery that connects to the kidney becomes constricted due to a blood clot (thrombus). The event occurs suddenly, and it often blocks blood supply completely. The main symptom is a sudden pain on the side of the abdomen, fever, and vomiting. If detected early, your doctor may dissolve the blood clot by using “clot-busting” medication, more often known as thrombolytics. If not treated promptly, the kidney will die and may need to be surgically removed. Renal artery thrombosis is very rare.

Renal Cell Carcinoma

This is the most common form of kidney cancer. It forms in the tubules: microscopic structures that transport waste products. This type of cancer often goes undetected until it’s in an advanced stage due to its lack of symptoms. The most successful treatment is complete removal of the affected kidney (nephrectomy), or using drugs to activate the immune system to attack cancerous cells (immunotherapy). Chemotherapy is not very successful against renal cell carcinoma. Cancer that forms in the ureter or the renal pelvis is not considered renal cell carcinoma.

Retransplant Graft Failure

Some patients’ graft may fail after receiving a kidney transplant. This could be due to organ rejection or damage caused to the kidney during transplant, since the kidney may be without oxygen supply for several hours. Organ rejection may occur when the recipient’s immune system attacks the kidney, mistaking it for an unwanted foreign object. An increase in anti-rejection medications usually resolves this issue. When the transplanted kidney fails, the patient will need to go back on dialysis, or receive another kidney transplant.

Sarcoidosis

Granulomas occur when our immune system cannot destroy a foreign organism; instead of eliminating the foreign body, it creates a collection of cells around it to wall it off from our bodies. Sarcoidosis is an inexplicable collection of these granulomas, most often occurring in the lungs, skin, and lymph nodes, but can also occur in other organs, like the heart or kidneys. Prognosis varies, some cases go into remission on their own, some take years and eventually clear up, and some lead to scarring in the lungs and death. When occurring in the kidneys, sarcoidosis can lead to kidney failure.

Sickle Cell Anemia

This is an inherited disease where a genetic mutation alters the shape of red blood cells. Instead of maintaining their normal disc shape, many blood cells assume a c-shape (or crescent shape), hence the name sickle. Sickle cells tend to be sticky and stiff, leading to blockages in blood flow, and reduced oxygen supply, followed by pain or organ damage. Sickle cell can lead to many complications, such as strokes, gallstones, spleen malfunctions, leg ulcers, and kidney failure, among others. There are many treatments for sickle cell anemia, but a bone marrow transplant is the only known cure.

Systemic Lupus Erythematosus

This is a disease where the immune system attacks the body’s tissues, mistaking them for foreign objects. Lupus can attack the heart, lungs, joints, skin, kidneys, blood vessels, liver, and nervous system. There is no known cause for lupus, although it is believed that genetics play a role. Lupus is often mistaken for other illnesses, so diagnosis can be elusive. Some of the main symptoms are a rash on the nose and cheeks, red scaly patches on the skin, joint pain, anemia, inflammation around the heart or lungs, and blood and protein in the urine. These last two symptoms are a sign of possible kidney failure.

Urolithiasis

This is the medical term for kidney stones that form anywhere in the urinary system. They differ from “Nephrolithiasis” (above) as this term is used to refer to stones that are either in the kidney, or elsewhere in the urinary system. For more information, see Nephrolithiasis.

Wegeners Granulomatosis

Granulomas occur when our immune system cannot destroy a foreign organism; instead of eliminating the foreign body, it creates a collection of cells around it to wall it off from our bodies. With Wegner’s Granulomatosis, the immune system mistakenly forms granulomas along the walls of the blood vessels of the lungs, windpipe, nose, throat, and kidneys, causing inflammation. These swollen blood vessels deliver insufficient oxygen to the affected organs, damaging them over time. Damage to the lungs and kidneys can be fatal. Early symptoms include nosebleeds, infections, coughing up blood, and blood in urine.

Wilm's Tumor

A cancerous growth in the kidney, Wilm’s Tumor mostly affects children. The tumor usually forms in only one kidney, but may rarely occur in both. Some children are genetically predisposed to grow this tumor, so they should be checked every 3 months by a doctor until they turn 8 years old. The earlier this tumor is treated, the higher the likelihood of a positive outcome. Some symptoms observed with people with Wilm’s tumor are an abnormally large abdomen, abdominal pain, fever, nausea, and sometimes blood in the urine.