1. Progress Report Nephrology WG September 2015 Gavin Becker

2. WG members Roles (Chair/co- chair/managing editor, etc) NameAffiliations Chair (Co-chair):Gavin BeckerRoyal Melbourne Hospital Australia Co-chairKunitoshi IsekiUniversity of the Ryuku Japan Managing editor(s)Megan Cumerlato Yoshifumi Ubara (commenced June 2015) Toranomon Hospital Japan

3. Progress report (1) ・ Code hierarchy has been confirmed and continues to be reviewed with regard to structure and shoreline changes between the Foundation and the JLMMS ・ We suspect that overlaps and redundancies still exist. Has your WG confirmed the hierarchy of the ICD-11 Foundation and Linearization? Have you found any problem with the hierarchy of the Foundation and/or the Joint Linearization for Mortality and Morbidity Statistics in ICD-11 Beta Version?

4. Progress report (2) ・ We provided referenced definitions for major entities, but were not happy with revisions from WHO intern(s). ・ Original definitions should stand and lower order diagnoses could be defined if wished. Has your WG completed the input of all definitions in the areas that fall under your WG's responsibility? Do the completed definitions meet the required standards?

5. Progress report (3) ・ Yes, as ICD-10 was used as the basis for the development of ICD-11, mapping should be appropriate. ・ Many concepts have been altered and new ones have been added to reflect the changes in terminology. In the areas under your WG's responsibility, are all ICD-10 entities able to be mapped to ICD-11?

6. Progress report (4a) We have liaised with Endocrinology to include Incipient Nephropathy and Overt Nephropathy within proteinuria/albuminuria: might require a little more finesse. Overlaps and redundancies with Rare/genetic/ multisystem diseases are difficult: in some renal manifestations are predominant, in others they are not : we are not clear how this resolves but for now have included the renal diseases within Renal ICD If there are overlapping entities that have moved to another TAG or WG, has your WG confirmed the move with the other TAG or WG? Or is there a plan to confirm it sometime in the future?

7. Progress report (4b) We have assumed that diseases that are centred in the kidney/ureter/bladder/urethra belong in Renal ICD – the cause (e.g. a specific organism/infection) will belong with another TAG or Workgroup. Multiple coding for a single patient e.g. Nephrotic syndrome: associated with: Membranous Glomerulonephritis: associated with: Hepatitis B infection. Here 2 Renal Tag (clinical, pathology) and 1 (Infection) TAG codes are all relevant. If there are overlapping entities that have moved to another TAG or WG, has your WG confirmed the move with the other TAG or WG? Or is there a plan to confirm it sometime in the future?

8. Next steps (1) Remaining issuesExpected outcome Strength of diagnosisDo we need “biopsy proven” vs “clinically suspected”? A larger problem for Oncology Laboratory vs clinical diagnosesSome diseases are defined by laboratory findings e.g. chronic kidney disease grades 1-5. When possible (and not in this example) we have tried to keep isolated pathology findings with laboratory codes A major problem is the rapid increase in known genetic mutations – which is causing redefinition of the clinical diseases e.g. nephronophthisis (AR), medullary cystic disease (AD), familial hyperuricemic nephropathy (AD). In none is the genetic abnormality always found - in fact most commonly it is not. This is a moving target ICD must be aware of, and have a strategy/ convection to handle e.g. organ/clinical diagnosis: gene suspected/proven

9. Next steps (2) Plans in 2015-2016 – Once again review ICD Renal to eliminate any remaining mistakes, ambiguity. – Re-examine definitions for accuracy – Respond to field trial outcomes