How much do you know about Polycystic Kidney Disease

Outline
Polycystic kidney disease (PKD) is a hereditary disorder of renal cyst which causes gradual enlargement of both kidneys. Sometimes it progresses to renal failure. Almost all forms are caused by a familial genetic mutation. Symptoms and signs include pain in flank of abdomen, hematuria, and high blood pressure. Diagnosis is by CT or ultrasonography. Symptomatic treatment is very effective before kidney failure. But when kidney failure, dialysis or transplantation are needed.
Etiology
PKD can be divided into dominant polycystic kidney disease and autosomal recessive polycystic kidney disease.
Autosomal dominant polycystic kidney disease (ADPKD) has an incidence of 1/1000 and accounts for about 5% of patients with end-stage renal disease. Most patients have not clinical manifestations before adulthood, but penetrance of kidney is complete. In contrast, autosomal recessive PKD is rare. Its incidence is 1/10,000. It causes renal failure during childhood frequently.
In 86 to 96% of cases, ADPKD is caused by mutations in the PKD1 gene on chromosome 16, which codes for the protein polycystin 1; the others are caused by mutations in the PKD2 gene on chromosome 4, which codes for polycystin 2.
Symptoms and Signs
ADPKD usually causes no symptoms initially; one half of patients remain asymptomatic, never develop renal insufficiency or failure, and are never diagnosed. Most patients develop symptoms by the end of their 20s.
Symptoms include low-grade flank, abdominal, and lower back pain due to cystic enlargement and symptoms of infection. Acute pain is usually due to hemorrhage into cysts or renal stone.
Fever is common with acute pyelonephritis, and rupture of cysts into the retroperitoneal space may cause a fever that can last for weeks. Hepatic cysts may cause right upper quadrant pain if they enlarge or become infected. Valvular disorders rarely cause symptoms but occasionally cause heart failure.
Symptoms and signs of unruptured cerebral aneurysm can be absent or may include headache, nausea and vomiting, and cranial nerve deficits; these manifestations warrant immediate intervention
Signs are nonspecific and include hematuria and hypertension (each in about 40 to 50%) and, in 20% of patients, proteinuria in the subnephrotic range (< 3.5g/24 h in adults). Anemia is less common than in other types of chronic kidney disease, presumably because erythropoietin production is preserved. In advanced disease, the kidneys may become grossly enlarged and palpable, causing fullness in the upper abdomen and flank.
Treatment
Supportive Measures to Complications
Strict BP control is essential. Typically an ACE inhibitor or angiotensin receptor blocker is used. In addition to controlling BP, these drugs help block angiotensin and aldosterone, growth factors that contribute to renal scarring and loss of renal function. Urinary tract infection should be treated promptly. Hemodialysis, peritoneal dialysis, or kidney transplantation is required in patients who develop chronic renal failure. ADPKD does not recur in grafts. With dialysis, patients with ADPKD maintain higher Hb levels than any other group of patients with renal failure.
Give ACE inhibitors or angiotensin receptor blockers for hypertension and to help prevent renal scarring and dysfunction; treat other complications as they arise, and consider use of tolvaptan.
Traditional Chinese Medicine Treatment

Traditional Chinese medicine treatment is very effective to patients with CRF, especially the patients in early or medium-stage. This treatment can improve clinical symptoms , control the progression of disease, low creatinine and urea nitrogen. To kidney failure which is caused by PKD, we use Cdonopsis pilosula, Atragalus membranaceus, Angelica sinensis. These can romote blood circulation to remove blood stasis and clear away heat and toxic materials. But it has a long period of treatment.