×
Choose Speciality

EXPLORE!

LOGIN

Medullary Cystic Kidney Disease

  762 Views

Dr Anupama Kaul, Lucknow    29 November 2019

  • Genetic study is recommended in patients with suspected ADTKD.
  • Affected individuals should in general be treated according to established CKD guidelines.
  • Children with ADTKD-HNF1B and ADTKD-REN disease are likely to benefit from early management.
  • Liberal water intake, no restriction of salt and avoiding use of diuretics can be useful measures.
  • There is no consensus on the benefits of renin-aldosterone blockade for prevention of renal disease progression.
  • In case of REN gene mutation, patients usually require erythropoietin early and also fludrocortisone to treat hypotension; however, it should not be used in patients with deterioration of glomerular filtration, hypertension hyperkalemia or edema.
  • Renal transplantation is the treatment of choice in ESRD caused by ADTKD, since the disease does not recur in the graft.
  • Renal-pancreatic double transplantation may be considered in patients with mutation in HNF1B and with diabetes.

To comment on this article,
create a free account.

Sign Up to instantly get access to 10000+ Articles & 1000+ Cases

Already registered?

Login Now

Most Popular Articles

News and Updates

eMediNexus provides latest updates on medical news, medical case studies from India. In-depth medical case studies and research designed for doctors and healthcare professionals.