1003 Adult nephrotic syndrome - no histology Immunological Glomerulopathy 52254009 1019 Nephrotic syndrome of childhood - steroid sensitive - no histology Immunological Glomerulopathy 445119005 1026 Congenital nephrotic syndrome (CNS) - no histology Immunological Glomerulopathy 48796009 1035 Congenital nephrotic syndrome (CNS) - Finnish type - no histology Immunological Glomerulopathy 197601003 1042 Congenital nephrotic syndrome (CNS) - Finnish type - histologically proven Immunological Glomerulopathy 197601003 1057 Congenital nephrotic syndrome (CNS) - diffuse mesangial sclerosis Immunological Glomerulopathy 48796009 1061 Congenital nephrotic syndrome (CNS) - focal segmental glomerulosclerosis (FSGS) Immunological Glomerulopathy 236384008 1074 Denys-Drash syndrome Immunological Glomerulopathy 236385009 1088 Congenital nephrotic syndrome (CNS) - congenital infection Immunological Glomerulopathy 48796009 1090 Minimal change nephropathy - no histology Immunological Glomerulopathy 44785005 1100 Minimal change nephropathy - histologically proven Immunological Glomerulopathy 44785005 1116 IgA nephropathy - no histology Immunological Glomerulopathy 236407003 1128 IgA nephropathy - histologically proven Immunological Glomerulopathy 236407003 1137 Familial IgA nephropathy - no histology Immunological Glomerulopathy 445404003 1144 Familial IgA nephropathy - histologically proven Immunological Glomerulopathy 445404003 1159 IgA nephropathy secondary to liver cirrhosis - no histology Immunological Glomerulopathy 282364005 1163 IgA nephropathy secondary to liver cirrhosis - histologically proven Immunological Glomerulopathy 282364005 1171 IgM - associated nephropathy Immunological Glomerulopathy 236411009 1185 Membranous nephropathy - idiopathic Immunological Glomerulopathy 197590001 1192 Membranous nephropathy - malignancy associated Immunological Glomerulopathy 197590001 1205 Membranous nephropathy - drug induced Immunological Glomerulopathy 197590001 1214 Membranous nephropathy - infection associated Immunological Glomerulopathy 197590001 1222 Mesangiocapillary glomerulonephritis type 1 Immunological Glomerulopathy 75888001 1233 Mesangiocapillary glomerulonephritis type 2 (dense deposit disease) Immunological Glomerulopathy 59479006 1246 Mesangiocapillary glomerulonephritis type 3 Immunological Glomerulopathy 236409000 1251 Idiopathic rapidly progressive (crescentic) glomerulonephritis Immunological Glomerulopathy 236398000 1267 Primary focal segmental glomerulosclerosis (FSGS) Immunological Glomerulopathy 236403004 1312 Focal segmental glomerulosclerosis (FSGS) secondary to obesity - no histology Immunological Glomerulopathy 236403004 1320 Focal segmental glomerulosclerosis (FSGS) secondary to obesity - histologically proven Immunological Glomerulopathy 236403004 1331 Diffuse endocapillary glomerulonephritis Immunological Glomerulopathy 3704008 1349 Mesangial proliferative glomerulonephritis Immunological Glomerulopathy 35546006 1354 Focal and segmental proliferative glomerulonephritis Immunological Glomerulopathy 83866005 1365 Glomerulonephritis - secondary to other systemic disease Immunological Glomerulopathy 36171008 1377 Glomerulonephritis - histologically indeterminate Immunological Glomerulopathy 36171008 1383 Systemic vasculitis - ANCA negative - histologically proven Immunological Glomerulopathy 46956008 1396 Systemic vasculitis - ANCA positive - no histology Immunological Glomerulopathy 46956008 1401 Granulomatosis with polyangiitis - no histology Immunological Glomerulopathy 195353004 1417 Granulomatosis with polyangiitis - histologically proven Immunological Glomerulopathy 195353004 1429 Microscopic polyangiitis - histologically proven Immunological Glomerulopathy 239928004 1438 Churg-Strauss syndrome - no histology Immunological Glomerulopathy 82275008 1440 Churg-Strauss syndrome - histologically proven Immunological Glomerulopathy 82275008 1455 Polyarteritis nodosa Immunological Glomerulopathy 155441006 1 / 7
1464 Anti-Glomerular basement membrane (GBM) disease / Goodpasture's syndrome - no histology Immunological Glomerulopathy 236506009 1472 Anti-Glomerular basement membrane (GBM) disease / Goodpasture's syndrome - histologically proven Immunological Glomerulopathy 50581000 1486 Systemic lupus erythematosus / nephritis - no histology Immunological Glomerulopathy 68815009 1493 Systemic lupus erythematosus / nephritis - histologically proven Immunological Glomerulopathy 68815009 1504 Henoch-Schönlein purpura / nephritis - no histology Immunological Glomerulopathy 191306005 1515 Henoch-Schönlein purpura / nephritis - histologically proven Immunological Glomerulopathy 191306005 1527 Renal scleroderma / systemic sclerosis - no histology Immunological Glomerulopathy 89155008 1536 Renal scleroderma / systemic sclerosis - histologically proven Immunological Glomerulopathy 236502006 1543 Essential mixed cryoglobulinaemia - no histology Immunological Glomerulopathy 239947001 1558 Essential mixed cryoglobulinaemia - histologically proven Immunological Glomerulopathy 239947001 1562 Cryoglobulinaemia secondary to hepatitis C - no histology Immunological Glomerulopathy 30911005 1570 Cryoglobulinaemia secondary to hepatitis C - histologically proven Immunological Glomerulopathy 30911005 1589 Cryoglobulinaemia secondary to systemic disease - no histology Immunological Glomerulopathy 30911005 1591 Cryoglobulinaemia secondary to systemic disease - histologically proven Immunological Glomerulopathy 30911005 3604 Nephrotic syndrome of childhood - steroid resistant - no histology Immunological Glomerulopathy 800991000000107 3615 Nephrotic syndrome of childhood - no trial of steroids - no histology Immunological Glomerulopathy 445119005 3749 Glomerulonephritis - no histology Immunological Glomerulopathy 36171008 1602 Primary reflux nephropathy - sporadic Tubulo-Interstitial Disease 197764002 1618 Familial reflux nephropathy Tubulo-Interstitial Disease 522551000000101 1625 Congenital dysplasia / hypoplasia Tubulo-Interstitial Disease 204949001 1639 Multicystic dysplastic kidneys Tubulo-Interstitial Disease 82525005 1641 Dysplasia due to fetal ACE-inhibitor exposure Tubulo-Interstitial Disease 519331000000100 1656 Glomerulocystic disease Tubulo-Interstitial Disease 253864004 1660 Congenital pelvi-ureteric junction obstruction Tubulo-Interstitial Disease 373584008 1673 Congenital vesico-ureteric junction obstruction Tubulo-Interstitial Disease 373585009 1687 Posterior urethral valves Tubulo-Interstitial Disease 253900005 1694 Syndrome of agenesis of abdominal muscles - prune belly syndrome Tubulo-Interstitial Disease 5187006 1706 Congenital neurogenic bladder Tubulo-Interstitial Disease 445387007 1710 Bladder exstrophy Tubulo-Interstitial Disease 61758007 1723 Megacystis-megaureter Tubulo-Interstitial Disease 253904001 1734 Oligomeganephronia Tubulo-Interstitial Disease 18417009 1747 Renal papillary necrosis - cause unknown Tubulo-Interstitial Disease 90241004 1752 Acquired obstructive uropathy / nephropathy Tubulo-Interstitial Disease 522591000000109 1768 Acquired obstructive nephropathy due to neurogenic bladder Tubulo-Interstitial Disease 522591000000109 1775 Obstructive nephropathy due to prostatic hypertrophy Tubulo-Interstitial Disease 522591000000109 1781 Obstructive nephropathy due to prostate cancer Tubulo-Interstitial Disease 522591000000109 1799 Obstructive nephropathy due to bladder cancer Tubulo-Interstitial Disease 522591000000109 1809 Obstructive nephropathy due to other malignancies Tubulo-Interstitial Disease 522591000000109 1813 Idiopathic retroperitoneal fibrosis Tubulo-Interstitial Disease 197808006 1821 Retroperitoneal fibrosis secondary to malignancies Tubulo-Interstitial Disease 236017004 1832 Calculus nephropathy / urolithiasis Tubulo-Interstitial Disease 95566004 1845 Calcium oxalate urolithiasis Tubulo-Interstitial Disease 444717006 2 / 7
1850 Enteric hyperoxaluria Tubulo-Interstitial Disease 37497004 1866 Magnesium ammonium phosphate (struvite) urolithiasis Tubulo-Interstitial Disease 444690001 1878 Uric acid urolithiasis Tubulo-Interstitial Disease 267441009 1884 Tubulointerstitial nephritis - no histology Tubulo-Interstitial Disease 428255004 1897 Tubulointerstitial nephritis - histologically proven Tubulo-Interstitial Disease 428255004 1907 Familial interstitial nephropathy - no histology Tubulo-Interstitial Disease 28689008 1911 Familial interstitial nephropathy - histologically proven Tubulo-Interstitial Disease 28689008 1924 Tubulointerstitial nephritis associated with autoimmune disease - no histology Tubulo-Interstitial Disease 428255004 1930 Tubulointerstitial nephritis associated with autoimmune disease - histologically proven Tubulo-Interstitial Disease 428255004 1948 Tubulointerstitial nephritis with uveitis (TINU) - no histology Tubulo-Interstitial Disease 428255004 1953 Tubulointerstitial nephritis with uveitis (TINU) - histologically proven Tubulo-Interstitial Disease 428255004 1969 Renal sarcoidosis - no histology Tubulo-Interstitial Disease 37061001 1976 Renal sarcoidosis - histologically proven Tubulo-Interstitial Disease 37061001 1982 Aristolochic acid nephropathy (Balkan / Chinese herb / endemic nephropathy) - no histology Tubulo-Interstitial Disease 236514003 1995 Aristolochic acid nephropathy (Balkan / Chinese herb / endemic nephropathy) - histologically proven Tubulo-Interstitial Disease 236514003 2005 Drug-induced tubulointerstitial nephritis - no histology Tubulo-Interstitial Disease 439990003 2014 Drug-induced tubulointerstitial nephritis - histologically proven Tubulo-Interstitial Disease 439990003 2022 Nephropathy due to analgesic drugs - no histology Tubulo-Interstitial Disease 59400006 2033 Nephropathy due to analgesic drugs - histologically proven Tubulo-Interstitial Disease 59400006 2046 Nephropathy due to ciclosporin - no histology Tubulo-Interstitial Disease 519481000000106 2051 Nephropathy due to ciclosporin - histologically proven Tubulo-Interstitial Disease 519481000000106 2067 Nephropathy due to tacrolimus - no histology Tubulo-Interstitial Disease 519491000000108 2079 Nephropathy due to tacrolimus - histologically proven Tubulo-Interstitial Disease 519491000000108 2080 Nephropathy due to aminoglycosides - no histology Tubulo-Interstitial Disease 519501000000102 2098 Nephropathy due to aminoglycosides - histologically proven Tubulo-Interstitial Disease 519501000000102 2108 Nephropathy due to amphotericin - no histology Tubulo-Interstitial Disease 519511000000100 2112 Nephropathy due to amphotericin - histologically proven Tubulo-Interstitial Disease 519511000000100 2120 Nephropathy due to cisplatin - no histology Tubulo-Interstitial Disease 53556002 2131 Nephropathy due to cisplatin - histologically proven Tubulo-Interstitial Disease 53556002 2149 Nephropathy due to lithium - no histology Tubulo-Interstitial Disease 4390004 2154 Nephropathy due to lithium - histologically proven Tubulo-Interstitial Disease 4390004 2165 Lead induced nephropathy - no histology Tubulo-Interstitial Disease 519521000000106 2177 Lead induced nephropathy - histologically proven Tubulo-Interstitial Disease 519521000000106 2183 Acute urate nephropathy - no histology Tubulo-Interstitial Disease 236496000 2196 Acute urate nephropathy - histologically proven Tubulo-Interstitial Disease 236496000 2203 Chronic urate nephropathy - histologically proven Tubulo-Interstitial Disease 190829000 2219 Radiation nephritis Tubulo-Interstitial Disease 7725007 2226 Renal / perinephric abscess Tubulo-Interstitial Disease 3321001 OR 80640009 2235 Renal tuberculosis Tubulo-Interstitial Disease 44323002 2242 Leptospirosis Tubulo-Interstitial Disease 77377001 2257 Hantavirus nephropathy Tubulo-Interstitial Disease 102455002 2261 Xanthogranulomatous pyelonephritis Tubulo-Interstitial Disease 38898003 3 / 7
2274 Nephropathy related to HIV - no histology Tubulo-Interstitial Disease 90708001 2288 Nephropathy related to HIV - histologically proven Tubulo-Interstitial Disease 90708001 2290 Schistosomiasis Tubulo-Interstitial Disease 236706006 2300 Other specific infection Tubulo-Interstitial Disease 40733004 3627 Renal cysts and diabetes syndrome Tubulo-Interstitial Disease 446641003 3636 Chronic urate nephropathy - no histology Tubulo-Interstitial Disease 190829000 3662 Hypercalcaemic nephropathy Tubulo-Interstitial Disease 33763006 3670 Retroperitoneal fibrosis secondary to peri-aortitis Tubulo-Interstitial Disease 49120005 3689 Retroperitoneal fibrosis secondary to drugs Tubulo-Interstitial Disease 236015007 2316 Diabetic nephropathy in type I diabetes - no histology Type 1 Diabetes Mellitus 421893009 2328 Diabetic nephropathy in type I diabetes - histologically proven Type 1 Diabetes Mellitus 421893009 2337 Diabetic nephropathy in type II diabetes - no histology Type 2 Diabetes Mellitus 420279001 2344 Diabetic nephropathy in type II diabetes - histologically proven Type 2 Diabetes Mellitus 420279001 2359 Chronic hypertensive nephropathy - no histology Vascular Disease 38481006 2363 Chronic hypertensive nephropathy - histologically proven Vascular Disease 38481006 2371 Malignant hypertensive nephropathy / accelerated hypertensive nephropathy - no histology Vascular Disease 65443008 2385 Malignant hypertensive nephropathy / accelerated hypertensive nephropathy - histologically proven Vascular Disease 65443008 2392 Ageing kidney - no histology Vascular Disease 445108007 2407 Ischaemic nephropathy - no histology Vascular Disease 519581000000107 2411 Ischaemic nephropathy / microvascular disease - histologically proven Vascular Disease 519581000000107 2424 Renal artery stenosis Vascular Disease 302233006 2430 Atheroembolic renal disease - no histology Vascular Disease 51677000 2448 Atheroembolic renal disease - histologically proven Vascular Disease 51677000 2453 Fibromuscular dysplasia of renal artery Vascular Disease 2900003 2469 Renal arterial thrombosis / occlusion Vascular Disease 236488005 2476 Renal vein thrombosis Vascular Disease 15842009 1279 Familial focal segmental glomerulosclerosis (FSGS) - autosomal recessive - no histology Hereditary Disease 445388002 1280 Familial focal segmental glomerulosclerosis (FSGS) - autosomal recessive - histologically proven Hereditary Disease 445388002 1298 Familial focal segmental glomerulosclerosis (FSGS) - autosomal dominant - no histology Hereditary Disease 444977005 1308 Familial focal segmental glomerulosclerosis (FSGS) - autosomal dominant - histologically proven Hereditary Disease 444977005 2756 Alport syndrome - no histology Hereditary Disease 399340005 2760 Alport syndrome - histologically proven Hereditary Disease 399340005 2773 Benign familial haematuria Hereditary Disease 236421001 2787 Thin basement membrane disease Hereditary Disease 236418003 2804 Medullary cystic kidney disease type I Hereditary Disease 444699000 2815 Medullary cystic kidney disease type II Hereditary Disease 445503007 2827 Uromodulin-associated nephropathy (familial juvenile hyperuricaemic nephropathy) Hereditary Disease 46785007 2836 Nephronophthisis Hereditary Disease 204958008 2843 Nephronophthisis - type 1 (juvenile type) Hereditary Disease 444830001 2858 Nephronophthisis - type 2 (infantile type) Hereditary Disease 444558002 2862 Nephronophthisis - type 3 (adolescent type) Hereditary Disease 444749006 2870 Nephronophthisis - type 4 (juvenile type) Hereditary Disease 446989009 4 / 7
2889 Nephronophthisis - type 5 Hereditary Disease 446991001 2891 Nephronophthisis - type 6 Hereditary Disease 447335007 2901 Primary Fanconi syndrome Hereditary Disease 236466005 2917 Tubular disorder as part of inherited metabolic diseases Hereditary Disease 197744007 2929 Dent disease Hereditary Disease 444645005 2938 Lowe syndrome (oculocerebrorenal syndrome) Hereditary Disease 79385002 2940 Inherited aminoaciduria Hereditary Disease 522601000000103 2955 Cystinuria Hereditary Disease 85020001 2964 Cystinosis Hereditary Disease 190681003 2972 Inherited renal glycosuria Hereditary Disease 226309007 2986 Hypophosphataemic rickets X-linked (XL) Hereditary Disease 82236004 2993 Hypophosphataemic rickets autosomal recessive (AR) Hereditary Disease 90505000 3000 Primary renal tubular acidosis (RTA) Hereditary Disease 1776003 3016 Proximal renal tubular acidosis (RTA) - type II Hereditary Disease 24790002 3028 Distal renal tubular acidosis (RTA) - type I Hereditary Disease 236461000 3037 Distal renal tubular acidosis with sensorineural deafness - gene mutations Hereditary Disease 236461000 3044 Nephrogenic diabetes insipidus Hereditary Disease 111395007 3059 Lesch Nyhan syndrome - hypoxanthine guanine phosphoribosyl transferase deficiency Hereditary Disease 10406007 3063 Phosphoribosyl pyrophosphate synthetase (PRPPS) superactivity Hereditary Disease 35759001 3071 Alagille syndrome Hereditary Disease 31742004 3085 Bartter syndrome Hereditary Disease 71275003 3092 Gitelman syndrome Hereditary Disease 3188003 3102 Liddle syndrome Hereditary Disease 71275003 3118 Apparent mineralocorticoid excess Hereditary Disease 237770005 3125 Glucocorticoid suppressible hyperaldosteronism Hereditary Disease 237743003 3139 Inherited / genetic diabetes mellitus type II Hereditary Disease 237604008 3141 Pseudohypoaldosteronism type 1 Hereditary Disease 43941006 3156 Pseudohypoaldosteronism type 2 (Gordon syndrome) Hereditary Disease 15689008 3160 Familial hypocalciuric hypercalcaemia Hereditary Disease 237885008 3173 Familial hypercalciuric hypocalcaemia Hereditary Disease 237885008 3187 Familial hypomagnesaemia Hereditary Disease 80710001 3194 Primary hyperoxaluria Hereditary Disease 17901006 3207 Primary hyperoxaluria type I Hereditary Disease 65520001 3211 Primary hyperoxaluria type II Hereditary Disease 40951006 3224 Fabry disease - no histology Hereditary Disease 16652001 3230 Fabry disease - histologically proven Hereditary Disease 16652001 3248 Xanthinuria Hereditary Disease 190919008 3253 Nail-patella syndrome Hereditary Disease 236527004 3269 Rubinstein-Taybi syndrome Hereditary Disease 45582004 3276 Tuberous sclerosis Hereditary Disease 7199000 3282 Von Hippel-Lindau disease Hereditary Disease 46659004 3295 Medullary sponge kidneys Hereditary Disease 236443009 5 / 7
3305 Horse-shoe kidney Hereditary Disease 41729002 3314 Frasier syndrome Hereditary Disease 445431000 3322 Branchio-oto-renal syndrome Hereditary Disease 290006 3333 Williams syndrome Hereditary Disease 63247009 3346 Townes-Brocks syndrome Hereditary Disease 523411000000105 3351 Lawrence-Moon-Biedl / Bardet-Biedl syndrome Hereditary Disease 232059000 3367 Mitochondrial cytopathy Hereditary Disease 240096000 3379 Familial nephropathy Hereditary Disease 236419006 3658 Renal coloboma syndrome Hereditary Disease 446449009 3731 Primary hyperoxaluria type III Hereditary Disease 828971000000101 2718 Autosomal dominant (AD) polycystic kidney disease Cystic Disease 28728008 2725 Autosomal dominant (AD) polycystic kidney disease type I Cystic Disease 253878003 2739 Autosomal dominant (AD) polycystic kidney disease type II Cystic Disease 253879006 2741 Autosomal recessive (AR) polycystic kidney disease Cystic Disease 28770003 2794 Cystic kidney disease Cystic Disease 82525005 2495 Hepatorenal syndrome Other 51292008 2509 Renal amyloidosis Other 48713002 2513 AA amyloid secondary to chronic inflammation Other 274945004 2521 AL amyloid secondary to plasma cell dyscrasia Other 23132008 2532 Familial amyloid secondary to protein mutations - no histology Other 66451004 2545 Familial amyloid secondary to protein mutations - histologically proven Other 66451004 2550 Familial AA amyloid secondary to familial Mediterranean fever / TRAPS (Hibernian fever) - no histology Other 367528006 2566 Familial AA amyloid secondary to familial Mediterranean fever / TRAPS (Hibernian fever) - histologically proven Other 367528006 2578 Myeloma kidney - no histology Other 32278006 2584 Myeloma cast nephropathy - histologically proven Other 32278006 2597 Light chain deposition disease Other 373604002 2606 Immunotactoid / fibrillary nephropathy Other 73305009 2610 Haemolytic uraemic syndrome (HUS) - diarrhoea associated Other 373421000 2623 Atypical haemolytic uraemic syndrome (HUS) - diarrhoea negative Other 373422007 2634 Thrombotic thrombocytopenic purpura (TTP) Other 78129009 2647 Haemolytic uraemic syndrome (HUS) secondary to systemic disease Other 111407006 2652 Congenital haemolytic uraemic syndrome (HUS) Other 444976001 2668 Familial haemolytic uraemic syndrome (HUS) Other 373420004 2675 Familial thrombotic thrombocytopenic purpura (TTP) Other 373420004 2681 Nephropathy due to pre-eclampsia / eclampsia Other 90708001 2699 Sickle cell nephropathy - no histology Other 13886001 2702 Sickle cell nephropathy - histologically proven Other 13886001 3461 Kidney tumour Other 126880001 3474 Renal cell carcinoma - histologically proven Other 254915003 3488 Transitional cell carcinoma - histologically proven Other 408642003 3490 Wilms tumour - histologically proven Other 302849000 3501 Mesoblastic nephroma - histologically proven Other 307604008 6 / 7
3517 Single kidney identified in adulthood Other 824131000000108 3529 Chronic kidney disease (CKD) / chronic renal failure (CRF) caused by tumour nephrectomy Other 236425005 3538 Chronic kidney disease (CKD) / chronic renal failure (CRF) due to traumatic loss of kidney Other 236425005 3540 Chronic kidney disease (CKD) / chronic renal failure (CRF) due to donor nephrectomy Other 236425005 3572 Haematuria and proteinuria - no histology Other 53298000 AND 29738008 3643 Chronic renal failure due to systemic infection Other 90688005 3712 Isolated haematuria - no histology Other 53298000 3720 Isolated proteinuria - no histology Other 29738008 3380 Acute kidney injury Acute Renal Failure 14669001 3398 Acute kidney injury due to hypovolaemia Acute Renal Failure 14669001 3403 Acute kidney injury due to circulatory failure Acute Renal Failure 14669001 3419 Acute kidney injury due to sepsis Acute Renal Failure 14669001 3426 Acute kidney injury due to rhabdomyolysis Acute Renal Failure 14669001 3435 Acute kidney injury due to nephrotoxicity Acute Renal Failure 236428007 3442 Acute cortical necrosis Acute Renal Failure 197650009 3457 Acute pyelonephritis Acute Renal Failure 36689008 2482 Cardiorenal syndrome Cardiorenal syndrome 445236007 3555 Chronic kidney disease (CKD) / chronic renal failure (CRF) - aetiology uncertain / unknown - no histology Unknown 236425005 3564 Chronic kidney disease (CKD) / chronic renal failure (CRF) - aetiology uncertain / unknown - histologically proven Unknown 236425005 3691 Renal failure Unknown 42399005 3708 Chronic renal failure Unknown 90688005 7 / 7