Renal Disease Leading to Kidney Failure

Renal Disease

Renal Disease
Leading to
Kidney Failure

Abstract Each year hundreds of thousands of patients begin dialysis due to kidney failure. A review of diseases that cause kidney failure can help build an understanding. Glomerulonephritis, polycystic kidney disease and tumors are some of the many diseases that may affect the kidney. They have similarities between them in terms of symptoms and may have an association with malignancy. The epidemiology, pathology, and pathogenesis of these diseases are of importance to help differentiate between them.

Renal Disease Leading to Kidney Failure

The kidney is a bean shaped organ responsible for regulating blood composition and excretion or urine. A human’s kidney consists of an outer cortex and an inner medulla with about one million tubules or nephrons. Nephrons contain numerous capillaries, which filter the blood entering from the renal artery. Some substances are reabsorbed into the blood while other substances, such as urine or other wastes, remain and are passed. Only one healthy kidney is needed to maintain life; however, when a serious kidney disease affects the normal function of both kidneys renal replacement therapy is needed. Glomerulonephritis (GN) can be an acute or chronic inflammation of the glomeruli of the kidneys (Youngson, 2005). Glomeruli are the blood vessels within the nephrons. GN is not race or gender specific, and is often a silent disease so the incidence of GN remains unknown. It is, however, more commonly found in children and young adults than older adults (Larson, 2003). The patient normally presents with fever, headache, loss of appetite, vomiting and puffiness of the face and body (Youngson, 2005). Acute GN frequently follows infections, especially those of the upper respiratory tract (Davis, 2009). With chronic GN the symptoms develop slowly, and the patient can be asymptomatic for years. The disease then progresses to kidney failure. Edema and hypertension are frequently noted. Laboratory findings typically show proteinuria and hematuria (Mosby, Inc., an affiliate of Elsevier Inc., 2009). GN has been found in patients with malignant tumors and lymphomas. However the relationship between these conditions have yet to be found (Rose, 2009). Sonographically acute GN will present itself in both kidneys. The bilateral renal parenchyma is usually thickened, with normal or slightly reduced echogenicity. No major changes will be noticed with color or power Doppler. From time to time there may be slight hypervascularity. The resistive index (RI) is generally normal in acute GN (Barozzi & Pavlica). On the other hand, chronic GN will present opposite findings. Ultrasound will show uniform shrinking of the kidneys, in which the parenchyma is thinned and becomes hyperechoic. Color or power Doppler will show diffusely decrease vascularization; however, spectra Doppler analysis could show a normal RI. The RI can show an increase in patients with long-standing GN because there is a decrease in renal blood flow and elevation in renal vascular impedance (Barozzi & Pavlica). Post-infectious GN disease generally resolves in 2–4 weeks. Treatments that may be used are doses of an oral steroid or a combination of steroids. Children respond better to steroid treatment than adults. Those who do not respond well, they in addition to steroids are also treated with immunosuppressant drugs (Appel & Kaplan, 2009). Polycystic kidney disease (PKD) is an inherited renal disease that causes an irreversible decline in kidney function (Chapman, Rahbari-Oskoui, & Bennett, 2009). PKD is an inherited trait and can be autosomal dominant or recessive. In genetics, both parents must contribute the recessive trait in order to appear in the offspring. On the contrary, autosomal dominant PKD (ADPKD) is where the trait or abnormal gene only needs to be passed down from one parent in order for the offspring to inherit the disease. ADPKD is the most common genetic cause of chronic kidney disease (Chapman, Rahbari-Oskoui, & Bennett, 2009). It is also one the most common hereditary diseases. Every child of a patient with PKD has a 50% chance of inheriting the disease (Torres & Bennett). ADPKD is not race or gender specific and is the underlying cause of kidney disease in approximately 5% of patients who begin dialysis annually in the United States (Chapman, Rahbari-Oskoui, & Bennett, 2009). Even though ADPKD is not race or gender specific it’s can develop during childhood or adult life. There are two known mutations on two different chromosomes. Patients with polycystin-1 (PKD1) mutations on chromosome 16 will present with symptoms at a younger age. End stage renal disease or death is more commonly associated with this mutation. Patients with polycystin-2 (PKD2) mutations on chromosome 4 will develop renal insufficiency later in life because it progresses more slowly (Chapman, Rahbari-Oskoui, & Bennett, 2009). Patients with either PKD1 or PKD2 will present with similar clinical findings. Patients with ADPKD have symptoms including, but not limited to, hematuria, flank and/or abdominal pain, arterial hypertension and urinary tract infection. Developmeant of renal cysts are visible by imaging studies such as ultrasound. Cystic lesions may also be visible in other organs. These organs include the liver, pancreas, spleen, thyroid, ovary, endometrium and seminal vesicles. In adults, an enlargement of both kidneys and bilateral, asymmetrical cysts of varying sizes are seen with ultrasound. Cysts with thickened walls and internal echoes can be due to hemorrhage or infection (Montemezzi & Gortenuti). Such findings make it harder to diagnoses renal cell carcinoma. There is no specific treatment that has been proven to prevent or delay progression of ADPKD. Hypertension and heart disease are risk factors for patients. About 36% of patients die from heart disease, 24% from infection, and 12 % from a neurological event. Strict blood pressure regulation and statins can be used to possibly prevent progression of disease and reduce cardiovascular mortality. Treatment of infection and pain control therapy can further be administered as possible preventative measures (Chapman, Rahbari-Oskoui, & Bennett, 2009). Renal cell carcinoma (RCC) is an infrequent complication of ADPKD. Diagnosis is difficult to establish because many of the symptoms that occur in patients with ADPKD can also be associated with RCC. Malignancy can be suspected when these signs and symptoms are more severe than usual or there is a rapid growth of a complex cyst (Chapman, Rahbari-Oskoui, & Bennett, 2009). RCC, which originates in the renal cortex, represents 80% to 85% of all primary renal tumors. The second most common is transitional tumors of the renal pelvis making up about 8% of primary renal tumors. Nephroblastoma or Wilms tumor, which is almost exclusively in children, make up approximately 5% to 6% of all primary renal tumors. Other tumors such as oncocytoma, collecting duct tumors, renal sarcomas and lymphomas are considered rare and occur infrequently (Atkins, 2009). They occur in men more than women in between the ages of 50-70. The most frequent symptoms for RCC is hematuria, flank pain and occasionally the presence of a palpable mass (Gortenuti & Montemezzi). When visualized in ultrasound most tumors are solids. There appearance may be isoechoic, hypoechoic, or hyperechoic (Gortenuti & Montemezzi). It may be difficult to differentiate between malignant and benign masses through the use of ultrasound. Findings such as hypervascularization shown by color and power Doppler or high velocity signals detected at the lesion periphery, may indicate malignant tumors. There are four tumor stages to consider when treatment is to be given. Stage I tumor is where the tumor is confined to the renal capsule. In stage II the tumor invades the peripheral fat. Patients with stage I or II are treated surgically with partial or radical nephrectomy. Stage III involves the regional lymph nodes or venous structures. When lymph nodes are affected, reducing the severity of the disease, prevention and/or relief of pain is all that can be done. If there is venous thrombosis the treatment involves radical nephrectomy in addition to thrombectomy. Patients who have stage IV, where the tumor has invaded adjacent organs or distant metastases, surgery is done and the treatment is only palliative like in stage III (Gortenuti & Montemezzi). Patients will ultimately reach end-stage renal disease, where the kidneys can no longer perform the function the body requires. In this case the only option is renal replacement therapy. Hemodialysis, peritoneal dialysis, and renal transplant are options for renal replacement therapy. Renal transplant may be the treatment of choice for patients with end-stage renal disease but not all patients are appropriate candidates for renal transplant. Medication or a patient’s condition may not allow for it. When a patient is a candidate, the graft survival rate is higher when the transplantation is done before dialysis has started. Even if a patient is suitable for the renal transplant there is still the aspect of availability. When a kidney is not available or renal transplantation is not an option there is hemodialysis or peritoneal dialysis (Post & Rose, 2009). Hemodialysis is done with a machine that filters the blood. It requires a continuous and stable access to the blood stream. Arteriovenous fistulas and synthetic arteriovenous fistulas are the most common seen in my lab. They are used to allow for easy access to the blood and the required volume. The access is normally placed on the nondominant upper extremity. There is a higher risk for infection or arterial steal syndrome with lower extremity grafts. These grafts can fail and either another access is placed into the same arm or on the opposite one. For this reason venipuncture to the opposite arm should be restricted to preserve the veins. Peritoneal dialysis catheters are placed into the abdominal cavity. A large volume of fluid is introduced into the abdomen. This will allow the wastes to be transferred to the fluid and later replaced with clean fluid. This method can be done automatically at night or throughout the day. There is a much higher risk for infection in the abdomen using this method of dialysis. Glomerulonephritis, polycystic kidney disease and tumors are three diseases that adversely affect the kidney. These diseases will ultimately lead to end-stage renal disease. When the kidneys can no longer perform the function the body requires the patient must be placed on hemodialysis, peritoneal dialysis, or receive a renal transplant. Having similar signs and symptoms, such as proteinuria, hematuria and flank pain the diseases becomes more difficult to differentiate. Understanding who is affected, what the disease is and how it progresses will allow for better diagnosis and future treatment.

References

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