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About oxalosis

What is oxalosis?

Hyperoxaluria occurs when you have too much oxalate in your urine. Oxalate is a natural chemical in your body, and it's also found in certain types of food. But too much oxalate in your urine can cause serious problems.

Hyperoxaluria can be caused by inherited (genetic) disorders, an intestinal disease or eating too many oxalate-rich foods. The long-term health of your kidneys depends on early diagnosis and prompt treatment of hyperoxaluria.

Oxalosis happens after the kidneys fail in people who have primary and intestinal causes of hyperoxaluria, and excess oxalate builds up in the blood. This can lead to oxalate deposits in blood vessels, bones and body organs.

What are the symptoms for oxalosis?

Severe or sudden back pain symptom was found in the oxalosis condition

Often, the first sign of hyperoxaluria is a kidney stone. Symptoms of a kidney stone can include:

  • Severe or sudden back pain
  • Pain in the area below the ribs on the back (flank) that doesn't go away
  • Blood in the urine
  • Frequent urge to urinate
  • Pain when urinating
  • Chills or fever

When to see a doctor

Kidney stones in childhood are uncommon. Kidney stones that form in children and teenagers are likely to be caused by an underlying condition, such as hyperoxaluria.

All young people with kidney stones should see a doctor for a thorough evaluation, including a test that measures oxalate in the urine. Adults with recurrent kidney stones also should be evaluated for oxalate in the urine.

What are the causes for oxalosis?

Hyperoxaluria occurs when there is too much of a substance called oxalate in the urine. There are several types of hyperoxaluria:

  • Primary hyperoxaluria. Primary hyperoxaluria is a rare inherited (genetic) condition present at birth. In this type, the liver doesn't create enough of a certain protein (enzyme) that prevents overproduction of oxalate, or the enzyme doesn't work properly. Excess oxalate is eliminated through your kidneys, in your urine. The extra oxalate can combine with calcium to create kidney stones and crystals, which can damage the kidneys and cause them to stop working (renal failure).

    Kidney stones form early and most often cause symptoms during childhood or adolescence. Because of the very large amounts of oxalate produced, the kidneys of many people with primary hyperoxaluria fail by early to middle adulthood. But renal failure can occur as early as infancy, while others with primary hyperoxaluria never develop kidney failure. To date, experts have identified three different genetic causes of primary hyperoxaluria.

  • Oxalosis. Oxalosis occurs if you have primary hyperoxaluria and your kidneys fail. Because your body can no longer eliminate the extra oxalate, it starts accumulating — first in your blood, then in your eyes, bones, skin, muscles, blood vessels, heart and other organs. This can cause multiple problems.
  • Enteric hyperoxaluria. Several intestinal diseases, including Crohn's disease and short bowel syndrome as a result of surgical procedures, increase the absorption of oxalate from foods, which can then increase the amount of oxalate excreted in the urine.
  • Hyperoxaluria related to eating high-oxalate foods. Eating large amounts of foods high in oxalate can increase your risk of hyperoxaluria or kidney stones. Ask your doctor or dietitian for a list of high-oxalate foods. Avoiding high-oxalate foods is particularly important if you have enteric hyperoxaluria.

What are the treatments for oxalosis?

Treatment depends on the type, symptoms and severity of hyperoxaluria and how well you respond to treatment.

Reducing oxalate

To reduce the amount of calcium oxalate crystal formation in your kidneys, your doctor may recommend one or more of these treatments:

  • Medications. Prescription doses of vitamin B-6 can be effective in reducing oxalate in the urine in some people with primary hyperoxaluria. Oral preparations of phosphates and citrate help prevent the formation of calcium oxalate crystals. Other medications, such as thiazide diuretics, also may be considered, depending on which other abnormalities are present in your urine.
  • High fluid intake. If your kidneys are still functioning normally, your doctor will likely tell you to drink more water or other fluids. This flushes the kidneys, prevents oxalate crystal buildup and helps keep kidney stones from forming.
  • Dietary changes. In general, paying attention to your diet is more important if you have enteric or dietary hyperoxaluria. Your doctor may recommend changes to your diet including restricting foods high in oxalates, limiting salt, and decreasing animal protein and sugar (high fructose corn syrup). Dietary changes like these may help to lower the levels of oxalate in your urine. But dietary restrictions may not be effective for all people with primary hyperoxaluria. Follow your doctor's recommendations.

Kidney stone management

Kidney stones are common in people with hyperoxaluria, but they don't always need to be treated. If large kidney stones are causing pain or blocking urine flow, you may need to have them removed or broken up so they can pass in the urine.

Dialysis and transplantation

Depending on the severity of your hyperoxaluria, you may eventually lose kidney function. Kidney dialysis may help temporarily, but it doesn't keep up with the amount of oxalate produced. A kidney transplant or kidney and liver transplant can cure certain inherited types of hyperoxaluria (primary hyperoxaluria).

What are the risk factors for oxalosis?

Oxalosis is a rare metabolic disorder caused by the overproduction of oxalate in the liver. Genetic mutations that lead to the deficiency of enzymes involved in glyoxalate fixation or reutilization cause the accumulation of oxalate. The accumulation first occurs in the kidney and causes stones, which eventually lead to renal failure. After renal failure, oxalate accumulates first in the blood and then in the eyes, bones, skin, muscles, blood vessels, heart, and other organs.

Risk factors
1. Low consumption of water: Low water content in the body concentrates the urine compositions, which induces the formation of crystals of oxalate, and leads to kidney failure.
2. Consumption of oxalate-rich food: Food ingredients such as tomato, spinach, rhubarb, almonds, cashews, miso soup, grits, baked potatoes with skin, beets, cocoa powder, okra, bran cereals, shredded wheat cereals, french fries, raspberries, stevia sweeteners, sweet potatoes contain high amounts of oxalate. Consumption of these items in excess is risky to the patients.
3. Enteric infections: Microbial infections are treated with antibiotics, commonly delivered orally. Oral antibiotics may disrupt the gut microbiome, which contains bacteria that reduces oxalate. It poses the risk of increased absorption of oxalate from the GI tract.

Symptoms
Severe or sudden back pain,Pain in the area below the ribs on the back (flank) that doesn't go away,Blood in the urine,Frequent urge to urinate,Pain when urinating,Chills or fever
Conditions
Hyperoxaluria,Oxalosis
Drugs
Lumasiran (Oxlumo),Prescription doses of vitamin B-6,Oral preparations of phosphates and citrate,Thiazide diuretics, also may be considered, depending on which other abnormalities are present in your urine

Is there a cure/medications for oxalosis?

Oxalosis is a rare inherited metabolic disorder caused by the overproduction of oxalate, which leads to the accumulation of an excessive amount of calcium oxalate. It is caused by a deficiency of enzymes involved in the metabolism of glyoxylate. Primarily calcium oxalate accumulates in the kidneys causing kidney stones and eventually leading to kidney failure. Followed by renal failure, the accumulation occurs first in blood and then in the eyes, bones, skin, muscles, blood vessels, heart, and other organs. There are three types, each caused by different genetic mutations.

Treatment
1. Large Fluid Consumption: It helps dilute the urine reducing crystallization of oxalate, through frequent urination.
2. Large Daily Doses of Vitamin B6
3. Magnesium Supplements
4. Oxalate-Reduced Diet: Tomato, spinach, rhubarb, almonds, cashews, miso soup, grits, baked potatoes with skin, beets, cocoa powder, okra, bran cereals, shredded wheat cereals, french fries, raspberries, stevia sweeteners, sweet potatoes are rich in oxalate and must be avoided.
5. Urinary Tract Endoscopy: Kidney stones of the size of up to 2 centimeters can be removed by ureteroscopy.
6. Renal dialysis: It is a procedure that allows the removal of excessive oxalate when the kidney fails to excrete oxalate crystals. A limitation of dialysis is its incapability to keep pace with the production of oxalate.
7. Kidney Transplant: Renal failure is addressed by a kidney transplant. However, recurrent failure leads to graft failure upon repeated transplantation.
8. Kidney-Liver Transplant: After the confirmation of oxalosis due to a deficiency of relevant hepatic enzymes, liver transplantation along with kidney transplantation, followed by first renal failure, is a better option.

Symptoms
Severe or sudden back pain,Pain in the area below the ribs on the back (flank) that doesn't go away,Blood in the urine,Frequent urge to urinate,Pain when urinating,Chills or fever
Conditions
Hyperoxaluria,Oxalosis
Drugs
Lumasiran (Oxlumo),Prescription doses of vitamin B-6,Oral preparations of phosphates and citrate,Thiazide diuretics, also may be considered, depending on which other abnormalities are present in your urine

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