Autosomal Dominant Polycystic Kidney Disease Polycystic kidney disease include ADPKD and ARPKD.Today let us know more about ADPKD first. Most individuals present with complications of the disease. However, increasing numbers are being detected by screening individuals with an affected relative.
Complications associated with renal disease: Impaired urine concentrating capacity is a common early presentation with problems associated with excessive water and salt loss such as nocturia. Loin pain is the most common symptom, reported by about 60% of affected adults. It can be caused by renal haemorrhage, stones and UTIs. However, chronic loin pain, without an obvious identifiable cause beyond the cysts themselves, develops in a proportion of patients. Hypertension is a common presenting feature. 10-15% of affected children are hypertensive and 50% of affected adults with normal renal function. Hypertension is associated with left ventricular hypertrophy.
Bilateral kidney enlargement - abdominal examination may reveal enlarged and palpable kidneys. (If other organs are cystic there may be palpable hepatomegaly and even splenomegaly.) Gross haematuria following trauma is a classic presenting feature of ADPKD. It occurs in 30-50%. Renal colic due to clots in the collecting system can be severe. Usually the bleeding is quite brief, reverting to microscopic levels in a few days.
UTI and pyelonephritis may be presenting features. Renal stones are twice as common as in the general population. Uric acid stones are more common than calcium oxalate stones. Patients sometimes present with renal failure, usually in the fourth to sixth decade of life.
Extra-renal manifestations: Most polycystic liver disease is asymptomatic. Cysts develop in 80% and tend to be larger in women. Symptoms tend to result from mass effect (eg, dyspnoea, early satiety,
gastro-oesophageal
reflux,
low
back
pain,
ascites,
oesophageal varices, obstructive jaundice) or from cyst complications (eg, haemorrhage, infection, torsion or rupture). Male infertility is rare but can be due to cysts in the seminal vesicles and defective sperm motility. Pancreatic cysts can, rarely, cause recurrent pancreatitis. Arachnoid membrane cysts are asymptomatic but increase the risk of subdural haematomas. Intracranial (berry) aneurysms occur in 6% of patients with a negative family history of aneurysms and 16% of those with a positive family history. They may cause cranial nerve palsies or seizures and have a risk of severe morbidity or mortality (35-55%) if they rupture. Mean age of rupture is lower than in the general population (39 years vs 51 years) and may not be associated with abnormal renal function or blood pressure. Aneurysms can also occur elsewhere in the vascular tree (eg, thoracic aorta, cervicocephalic artery, coronary artery) and may cause dissections. Cardiac abnormalities are also reasonably common: mitral
valve prolapse is found in up to 25% and aortic insufficiency can occur with aortic root dilatation. --
Prognosis There is great inter-familial and intra-familial variability in the severity of renal and extrarenal manifestations; however: Around 50% will be in end-stage renal failure and require dialysis or transplantation by the age of 60 in PKD1 and the age of 75 in PKD2. Risk factors for progression include: PKD1 genotype. Multiple pregnancies. Afro-Caribbean race. Male sex. Younger age of onset. Large kidneys.
Hypertension. Biomarkers
which
correlate
with
progression
include
increased urinary sodium secretion and urine osmolality, and lower HDL-C.
Treatments Do you heard Blood Pollution Therapy?Let me tell the Magical of this therapy. Unhealthy blood is the chief culprit of the damage to renal tissues and root cause of lingering kidney disease. If the blood in the body is heavily contaminated, the organs and tissues where the blood flows through will be damaged. After we find the cause of kidney damage- the unhealthy blood, Clear Blood Pollution Therapy is invented to treat kidney disease fundamentally.
If you want to know more about this therapy.Or have any questions about your kidney disease send me an email kidney-treatment@hotmail.com .