Polycystic Kidney Disease An inherited disorder polycystic kidney

Polycystic Kidney Disease An inherited disorder, polycystic kidney disease is characterized by multiple, bilateral, grapelike clusters of fluid-filled cysts that grossly enlarge the kidneys, compressing and eventually replacing functioning renal tissue.

• This disorder appears in 02 distinct forms. • The infantile form – causes still birth/ early neonatal death. • A few infants with this disease survive for 2 years and then develop fatal renal. Heart, liver, or respiratory failure. • The adult form – Onset of the adult form is insidious but commonly becomes obvious between ages 30 and 50. • Renal deterioration in the adult form of this disorder commonly leads to renal failure.

Causes • The infantile form appears to be inherited as an autosomal recessive trait. • The adult form appears to be inherited as an autosomal dominant trait.

Clinical manifestations • Infantile polycystic disease include Pronounced epicanthal folds, Pointed nose, Small chin, Huge bilateral masses on the flanks, Floppy, low-set ears (Potter facies) • These are symmetrical and tense.

• Nonspecific early effects of adult polycystic disease include, Hypertension, Polyuria, Symptoms of UTI. Later signs & symptoms include, Lumbar pain, Widening girth, Swollen / tender abdomen.

• Advanced problems may include recurrent hematuria, life – threatening retroperitoneal bleeding, proteinuria, and colicky abdominal pain. • Both kidneys are grossly enlarged and palpable.

Diagnostic Tests • IVP • Ultra sound & CT scan – Show kidney enlargement and the presence of cysts • Urinalysis • Creatinine clearance tests

Treatment Although polycystic kidney disease cannot be cured, careful management of associated UTIs and secondary hypertension may prolong life. Ø Adult polycystic kidney disease discovered in the asymptomatic stage requires careful monitoring, including urine cultures and creatinine clearance test. Ø When urine culture detects infection, prompt and vigorous antibiotic treatment is necessary even for asymptomatic infection

Ø As renal impairment progresses, selected pts may undergo dialysis, transplantation, or both. Ø Nephrectomy is recommended only for severe infection or bleeding.

Nursing Interventions • Carefully assess the pt’s lifestyle and his physical and mental state; determine how rapidly the disease is progressing. • Provide supportive care to minimize any associated symptoms. • Refer the young adult pt or parents of infants with this disorder for genetic counseling.

Pt Education • Explain all diagnostic procedures to the pt or his family. • Stress to the pt the need to take medication exactly as prescribed, even if symptoms are minimal / absent. • Explain that cystoscopic procedures pose a serious risk of infection and that he should avoid them.