Uremia


Uremia is the condition of having high levels of urea in the blood. Urea is one of the primary components of urine. It can be defined as an excess in the blood of amino acid and protein metabolism end products, such as urea and creatinine, which would normally be excreted in the urine. Uremic syndrome can be defined as the terminal clinical manifestation of kidney failure. It is the signs, symptoms and results from laboratory tests which result from inadequate excretory, regulatory, and endocrine function of the kidneys. Both uremia and uremic syndrome have been used interchangeably to denote a very high plasma urea concentration that is the result of renal failure. The former denotation will be used for the rest of the article.
Azotemia is a similar, less severe condition with high levels of urea, where the abnormality can be measured chemically but is not yet so severe as to produce symptoms. Uremia describes the pathological and symptomatic manifestations of severe azotemia.
There is no specific time for the onset of uremia for people with progressive loss of kidney function. People with kidney function below 50% and over 30 years of age may have uremia to a degree. This means an estimated 8 million people in the United States with a GFR of less than 60 mL/min have uremic symptoms. The symptoms, such as fatigue, can be very vague, making the diagnosis of impaired kidney function difficult. Treatment can be by dialysis or a kidney transplant, though some patients choose to pursue symptom control and conservative care instead.

Signs and symptoms

Classical signs of uremia are: progressive weakness and easy fatigue, loss of appetite due to nausea and vomiting, muscle atrophy, tremors, abnormal mental function, frequent shallow respiration, and metabolic acidosis. Without intervention via dialysis or kidney transplant, uremia due to renal failure will progress and cause stupor, coma, and death. Because uremia is mostly a consequence of kidney failure, its signs and symptoms often occur concomitantly with other signs and symptoms of kidney failure.
Area affectedSigns and symptoms
Central nervous systemdiurnal somnolence, night insomnia, memory and concentration disorders, asthenia, headache, confusion, fatigue, seizures, coma, encephalopathy, decreased taste and smell, hiccups, serositis
Peripheral nervous systempolyneuritis, restless legs, cramps, peripheral neuropathy, oxidative stress, reduced body temperature
Gastrointestinalanorexia, nausea, vomiting, gastroparesis, parotitis, stomatitis, superficial gastrointestinal ulcers
Hematologicanemia, hemostasis disorders, granulocytic, lymphocytic and thrombocytic dysfunction
Cardiovascularhypertension, atherosclerosis, coronary artery disease, pericarditis, peripheral and pulmonary edema
Skinitching, skin dryness, calciphylaxis, uremic frost
Endocrinologygrowth impairment, impotence, infertility, sterility, amenorrhea
Skeletalosteomalacia, β2-microglobulin amyloidosis, bone disease
Nutritionmalnutrition, weight loss, muscular catabolism
Otheruremic fetor
Immunitylow response rate to vaccination, increased sensitivity to infectious diseases, systemic inflammation

Glomerular filtration rate measures the amount of plasma in millilitres being filtered through the kidneys each minute. As the GFR decreases, the prognosis worsens. Some of the effects can be reversed, albeit temporarily, with dialysis.
GFR Effects
100–120Normal GFR
<60Uremic symptoms may be present, reduced well-being
30–60Cognitive impairment
55Fatigue and reduced stamina
<50Insulin resistance
<30Increasing likelihood of symptoms
≤15Kidney failure

Residual syndrome

People on dialysis acquire what is known as "residual syndrome". Residual syndrome is a non-life-threatening disease which is displayed as toxic effects causing many of the same signs and symptoms that uremia displays. There are several hypotheses why residual syndrome is present. They are: the accumulation of large molecular weight solutes that are poorly dialyzed ; the accumulation of protein-bound small molecular weight solutes that are poorly dialyzed ; the accumulation of dialyzable solutes that are incompletely removed ; indirect phenomena such as carbamylation of proteins, tissue calcification, or a toxic effect of hormone imbalance ; and the toxic effects of dialysis itself. Dialysis increases life span, but patients may have more limited function. They have physical limitations which include impairment of balance, walking speed, and sensory functions. They also have cognitive impairments such as impairment in attention, memory, and performance of higher-order tasks. Patients have been maintained longer than three decades on dialysis, but average mortality rates and hospitalizations are high. Also, patient rehabilitation and quality of life is poor.

Causes

Conditions causing increased blood urea fall into three different categories: prerenal, renal, and postrenal.
Prerenal azotemia can be caused by decreased blood flow through the kidneys or by increased production of urea in the liver via a high protein diet or increased protein catabolism.
Renal causes can be attributed to decreased kidney function. These include acute and chronic kidney failure, acute and chronic glomerulonephritis, tubular necrosis, and other kidney diseases.
Postrenal causes can be due to decreased elimination of urea. These could be due to urinary outflow obstruction such as by calculi, tumours of the bladder or prostate, or a severe infection.

Diagnosis

A detailed and accurate history and physical examination will help determine if uremia is acute or chronic. In the cases of acute uremia, causes may be identified and eliminated, leading to a higher chance for recovery of normal kidney function, if treated correctly.

Blood tests

Primary tests performed for the diagnosis of uremia are basic metabolic panel with serum calcium and phosphorus to evaluate the GFR, blood urea nitrogen and creatinine as well as serum potassium, phosphate, calcium and sodium levels. The principal abnormality is very low GFR. Uremia will demonstrate elevation of both urea and creatinine, likely elevated potassium, high phosphate and normal or slightly high sodium, as well as likely depressed calcium levels. As a basic work up a physician will also evaluate for anemia, and thyroid and parathyroid functions. Chronic anemia may be an ominous sign of established renal failure. The thyroid and parathyroid panels will help work up any symptoms of fatigue, as well as determine calcium abnormalities as they relate to uremia versus longstanding or unrelated illness of calcium metabolism.

Urine tests

A 24-hour urine collection for determination of creatinine clearance may be an alternative, although not a very accurate test due to the collection procedure. Another laboratory test that should be considered is urinalysis with microscopic examination for the presence of protein, casts, blood and pH.

Radioisotope tests

The most trusted test for determining GFR is iothalamate clearance. However, it may be cost-prohibitive and time-consuming. Clinical laboratories generally calculate the GFR with the modification of diet in renal disease formula or the Cockcroft-Gault formula.

Other

In addition, coagulation studies may indicate prolonged bleeding time with otherwise normal values.

Mechanism

Uremia results in many different compounds being retained by the body. With the failure of the kidneys, these compounds can build up to dangerous levels. There are more than 90 different compounds that have been identified. Some of these compounds can be toxic to the body.
Solute groupExampleSourceCharacteristics
Peptides and small proteinsβ2-microglobulinshed from major histocompatibility complexpoorly dialyzed because of large size
Guanidinesguanidinosuccinic acidarginineincreased production in uremia
Phenolsρ-cresyl sulfatephenylalanine, tyrosineprotein bound, produced by gut bacteria
Indolesindicantryptophanprotein bound, produced by gut bacteria
Aliphatic aminesdimethylaminecholinelarge volume of distribution, produced by gut bacteria
Polyolsunknowntightly protein bound
UcleosidespseudouridinetRNAmost prominent of several altered RNA species
Dicarboxylic acidsoxalateascorbic acidformation of crystal deposits
Carbonylsglyoxalglycolytic intermediatesreaction with proteins to form advanced glycation end-products

Uremic toxins

Uremic toxins are any biologically active compounds that are retained due to kidney impairment. Many uremic salts can also be uremic toxins.
Urea was one of the first metabolites identified. Its removal is directly related to patient survival but its effect on the body is not yet clear. Still, it is not certain that the symptoms currently associated with uremia are actually caused by excess urea, as one study showed that uremic symptoms were relieved by initiation of dialysis, even when urea was added to the dialysate to maintain the blood urea nitrogen level at approximately 90 mg per deciliter. Urea could be the precursor of more toxic molecules, but it is more likely that damage done to the body is from a combination of different compounds which may act as enzyme inhibitors or derange membrane transport. Indoxyl sulfate is one of the better characterized uremic toxins. Indoxyl sulfate has been shown to aggravate vascular inflammation in atherosclerosis by modulating macrophage behavior.
ToxinEffectReferences
UreaAt high concentrations : headaches, vomiting, fatigue, carbamylation of proteins
CreatininePossibly affects glucose tolerance and erythrocyte survival
CyanateDrowsiness and hyperglycemia, carbamylation of proteins and altered protein function due to being a breakdown product of urea
Polyols Peripheral neuropathy
PhenolsCan be highly toxic as they are lipid-soluble and therefore can cross cell membranes easily
"Middle molecules"Peritoneal dialysis patients clear middle molecules more efficiently than hemodialysis patients. They show fewer signs of neuropathy than hemodialysis patients
β2-MicroglobulinRenal amyloid
Indoxyl sulfateInduces renal dysfunction and cardiovascular dysfunction; associated with chronic kidney disease and cardiovascular disease
ρ-cresyl sulfateAccumulates in and predicts chronic kidney disease