Treatment of polycystic kidney disease (PKD) depends on the extent of cyst formation and other associated conditions.
Management of autosomal recessive PKD
In the autosomal recessive form (ARPKD) initial tests include:
- Evaluation for dehydration or fluid retention
- Assessment of hepatic or splenic enlargement and other signs of liver involvement
- Respiratory testing, such as chest examination, flow rates and pulse oximetry, with radiographs if required
- Renal function tests
- Serum electrolyte levels
- Urinalysis which includes:
- urine concentration
- urine protein assay and estimation
- urinary infection
- hepatic function tests such as
- transaminases
- bile acids
- serum albumin
- 25-OH cholecalciferol
- Coagulation studies
- Fat-soluble vitamin levels
Other complications which should be diagnosed and treated include:
- hypertension monitoring, preferably at home
- food intake charts
- weight and height gain charts
- nutritionist assistance as indicated
In infants, immediate care may include:
- Respiratory care
- stabilization of breathing
- respiratory assistance by mechanical ventilation
- in severe limitation of diaphragmatic movements by the grossly enlarged kidneys, nephrectomy may be unavoidable, though many researchers do not see much use in this procedure. This will require dialysis and, in future, a living kidney transplant.
- Renal insufficiency - peritoneal dialysis may be required if the neonate shows oliguria or anuria
- Monitoring and correction of hyponatremia and dehydration, with feedings through nasogastric or gastronomy tubes
- Supplemental feedings as indicated, with or without growth hormone treatment
Surveillance
Further surveillance includes:
- Monitoring and treatment of hypertension with angiotensin-converting enzyme (ACE) inhibitors or ARB inhibitors
- Treatment of anemia with iron and erythropoietin-stimulating agents (ESA)
- Treatment of kidney disease including dialysis or kidney transplant
- Hepatic support
- Monitoring nutrient absorption
- Synthetic bile acids if fat-soluble vitamin levels are low, or weight gain is below acceptable levels
- Monitoring for ascending cholangitis
- Treatment of esophageal varices with endoscopic banding or sclerotherapy
- Hepatic transplants or combined liver-kidney transplants for severe and progressive portal hypertension
- Prevention of other complications
- Immunization against relevant pathogens such as pneumococcal vaccine and palivizumab to children below 2 years with chronic pulmonary disease or history of premature birth
- Antibiotic prophylaxis in patients at risk for ascending cholangitis (first or recurrent episodes)
Drugs to avoid
- Sympathomimetics
- Nephrotoxic drugs such as NSAIDs and aminoglycosides
- Hepatotoxic drugs and alcohol
Risk-based screening of siblings by high-resolution ultrasound of the liver and kidney along with blood pressure measurement, and molecular genetic testing if possible and indicated, may sometimes be useful in early diagnosis and treatment and to relieve stress.
Management of autosomal dominant PKD
Treatment of ADPKD involves the following aspects:
- Treatment of pain with analgesics or antidepressants
- Surgical treatment includes aspiration of cysts and denervation of sclerosis, cyst fenestration, and renal
- Complications of cyst surgery include hematuria and cyst hemorrhage.
- Treatment of renal calculi
- Treatment of cysts associated with infection, often with cotrimoxazole or fluoroquinolones
- Ruling out malignancy should be a top priority in such cases
- Controlling the progress of renal disease by:
- lowering hypertension, with dietary sodium restriction and ACE inhibitors
- controlling hyperlipidemia
- restriction of dietary protein
- acidosis control
- preventing high phosphate levels
- Preventing or treating aneurysmal rupture using surgical clipping at the aneurysmal neck or sometimes, endovascular platinum coils
- Replacement of the thoracic aorta when dilatation of the aortic root crosses acceptable limits
Surveillance should be carried out for:
- blood pressure
- screening for aneurysms in high-risk patients
- screening for heart or aortic defects in high-risk patients
Drugs to avoid
- nephrotoxic drugs for a prolonged time
- caffeine
- estrogens
- smoking
Screening should be advised for adult relatives to enable early detection and treat complications.
Pregnancy with ADPKD
Monitor for:
- Rises in blood pressure
- UTIs
- Oligohydramnios
- Intrauterine growth restriction of the fetus
- Fetal kidney defects
References
Further Reading