PKD or PCKD: Signs, Causes & Treatment!

Polycystic Kidney Disease also known as PKD or PCKD is characterized by the growth of multiple cysts in the kidneys as a result of a genetic structural disorder of the renal tubules.

Cysts are non-functioning tubules filled with fluid pumped into them.

These cysts are microscopic or abnormal in size & destroy the neighboring healthy tubules, thereby rendering them functionless.

CLASSIFICATION OF PKD

  • Autosomal dominant polycystic kidney disease (ADPKD):
    a) Associated with large interfamilial & intrafamilial variability.
    b) Occurs as a result in mutation of PKD1 & PKD2 genes. 
  • Autosomal recessive polycystic kidney disease (ARPKD):
    a) Associated as a hereditary renal disease in early childhood.
    b) Occurs as a result in mutation of PKHD1 gene. 

SIGNS & SYMPTOMS

  • High blood pressure
  • Headache
  • Abdominal pain
  • Feeling of fullness in the stomach
  • Excessive urination
  • Blood in the urine
  • Pain in the back
  • Formation of cysts in other organs. 

COMPLICATION

  • Subarchanoid hemorrhage
  • Liver cyst
  • Pancreas cyst
  • Anemia
  • Uremia
  • Increase in RBC
  • Heart valve abnormalities
  • Development of inflammatory sacs in the colon
  • Pregnancy & birth related abnormalities
  • Urinary Tract Infections
  • Kidney stones
  • Chronic Kidney Disease (CKD)
  • End Stage Renal disease (ESRD)
  • Kidney failure. 

CAUSES & RISK FACTORS OF PKD

  • Genetic disorders & familial history
  • Hypertension
  • Male sex
  • Kidney problems
  • Obstruction of the kidney.


DIAGNOSIS OF PKD

  1. Molecular genetic testing also called DNA analysis by linkage analysis or mutation analysis which is available as a clinical method. 
  2. Ultrasound exam of the kidney for production of images of the kidney. 
  3. CT scan & MRI scan for production of renal images & detecting cysts or other abnormalities. 

TREATMENT & MANAGEMENT OF PKD

  • Some medications that are used for treatment of PKD are:
  • Aquaretic drug, like Tolvaptan which acts on the vasopressin receptor & promotes the excretion of fluid, without loss of electrolytes essential to the body.
  • High blood pressure is controlled by drugs of various classes, like ACE inhibitors, CCBs, ARBII blockers etc.
  • Management of pain by using opioid & non-opioid drugs.
  • Some surgical methods for treatment of PKD are:
  • Renal cyst aspiration, draining of the cyst by utilizing a sclerosing agent through a needle.
  • Laparoscopic cyst decortication, involves removal of the cyst by implication of laparoscopic surgery.
  • Neurolysis, done for pain caused due to PKD.
  • Nephrectomy, surgical removal of kidneys.
  • Dialysis
  • Kidney transplant

PREVENTION

  • Some management techniques are;
  • Inhibition of alcohol intake & cessation of smoking that can trigger gene mutation.