Message from the Principal Investigator

 Kidney diseases in children as individual diseases do not afflict a large number of patients. However, they pose various issues, including the extremely long period of the duration of the disease, the complications peculiar to pediatric kidney disease and the side effects of medication, and the big impact they have on the children’s growth and development. The need is great for factfinding in Japan, information communication through guidelines and various clinical practice guides, provision of information materials for patients and internet resources, all of which should be designed to lead to early diagnosis and appropriate treatment.

Our research

 Our research group is studying the following intractable renal diseases and specific pediatric chronic diseases as designated by the MHLW of Japan:
・Alport Syndrome (Designated Intractable Disease Registration Number 218)
・Galloway-Mowat syndrome (No. 219, as above)
・Epstein syndrome (No. 287, as above)
・Nail-patella syndrome/LMX1B-associated nephropathy (No. 315, as above)
・Branchio-oto-renal syndrome (No. 190, as above; in this research this is not treated as a disease for investigation, but in collaboration with the research team on intractable hearing disorders, research support on the renal disease and its prognosis is to be provided)
・Congenital anomalies of the kidney and the urinary tract (Specific pediatric chronic disease)
・Congenital nephrotic syndrome (as above)
・Nephronophthisis (as above)
・Bartter and Gitelman syndromes (as above)
・Idiopathic nephrotic syndrome in children (as above)
・Lowe syndrome (as above)

 In association with the Japanese Society for Pediatric Nephrology, the Japan Pediatric Society, the Japan Society of Nephrology, together with other bodies concerned, we will engage in the following activities:
1.Revision and diffusion of diagnostic criteria and severity classification as approved by academic societies
2.Editing, revision and diffusion of evidence-based clinical practice guidelines and patient guidelines, produced as approved by academic societies
3.Cohort creation and prognosis survey of diseases profiled through national epidemiological survey

we will continue to investigate chronic kidney disease (CKD)

 As well as conducting research into individual diseases, we will continue to investigate chronic kidney disease (CKD), which is the pathophysiology common to kidney diseases. Irrespective of what the primary disease is, CKD necessitates dialysis or organ transplant when kidney function deteriorates. Notably, we regard CKD in children as pediatric CKD, as distinct from adult CKD: pediatric CKD exhibits background factors, primary disease and complications specific to children. Since 2010, we have been conducting cohort studies, revealing a wide range of facts about the state of the disease. We will continue to pursue this research thread and hope to shed light on the condition of patients after they reach adulthood.

 Pediatric renal disease is a typical chronic disease. Patients carry the disease into adulthood. Therefore, we need to improve transitioning to adult care, that is, healthcare to be provided after patients reach adulthood. We hope to create a model program for Kanagawa Prefecture, so that we can ensure that each patient receives healthcare in the most suitable environment after becoming an adult.

 Through these research activities, we aim to carry on with our effort to deliver benefit to pediatric patients of kidney disease by resolving the many issues in pediatric renal disease mentioned at the opening of my message.

Kenji Ishikura
Principal Investigator
Study Group on the development of
clinical practice and research systems
for rare and refractory renal diseases in pediatric nephrology