Behavioural abnormalities and Mutations in children with CKD

We very commonly see children with CKD and ESRD with behavioural problems and autistic features and CAKUT. Current issue of Kidney International nicely shows the 17q12 deletions but not HNF1B intragenic mutations are associated with neurodevelopmental disorders.

Otherwise, Heterozygous mutations of the HNF1B gene are the commonest known monogenic cause of developmental kidney disease.

Controversies and Management of Cystinosis

Current Issue of Kidney International has an excellent paper on Cystinosis- from diagnosis to management, a must read for all pediatricians and pediatric nephrologists.
Nephropathic cystinosis is an autosomal recessive metabolic, lifelong disease characterized by lysosomal cystine accumulation throughout the body that commonly presents in infancy with a renal Fanconi syndrome and, if untreated, leads to end-stage kidney disease (ESKD) in the later childhood years.

Immunogenicity of HPV vaccine in CKD children

Vaccination is must in all children especially with kidney disorders. CJASN today publishes an excellent study from Baltimore on HPV vaccination.
The study shows that- Antibody response to the quadrivalent recombinant HPV vaccine was robust and sustained in girls and young women with CKD and on dialysis. A less robust response to the vaccine was observed among those with a kidney transplant.

Image Source

Diagnosing CKD early: supAR

It's interesting to read todays's correspondence in NEJM and thoughts that suPAR might be an early marker of CKD in patients in the long run. Though we are still waiting for evidence, and also suPAR in multiple type of renal disorders.