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AA amyloidosis

AApoAI amyloidosis

AApoAII amyloidosis

AApoAIV amyloidosis

Acquired thrombotic thrombocytopenic purpura (TTP)

Acro-pectoro-renal dysplasia

Acroosteolysis dominant type

Acrorenal syndrome

Action myoclonus-renal failure syndrome

Adenine phosphoribosyltransferase deficiency

Adult-onset Still disease

AFib amyloidosis

AH amyloidosis

AL amyloidosis

Alagille syndrome

Alport syndrome

Alport syndrome, autosomal dominant

Alport syndrome, autosomal recessive

Alport syndrome, digenic

Alport syndrome, X-linked

Alport syndrome, X-linked with diffuse leiomyomatosis

Alström Syndrome

ALys amyloidosis

Amelogenesis imperfecta-nephrocalcinosis syndrome

Aniridia-renal agenesis-psychomotor retardation syndrome

Anterior urethral valve

Anti-glomerular basement membrane disease

Apparent mineralocorticoid excess

AREDYLD (acral-renal-ectodermal-dysplasia-lipoatrophic-diabetes) syndrome

Arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome

Atresia of urethra

Atypical hemolytic uremic syndrome (aHUS)

Atypical hemolytic uremic syndrome (aHUS) due to Methylcobalamin deficiency, type CblC

Atypical hemolytic uremic syndrome (aHUS) with anti-factor H antibodies

Atypical hemolytic uremic syndrome (aHUS) with complement gene abnormality

Atypical hemolytic uremic syndrome (aHUS) with DGKE deficiency

Atypical hemolytic uremic syndrome (aHUS), not further specified

Auto-immune distal renal tubular acidosis

Autoimmune hepatitis

Autoimmune interstitial lung disease-arthritis syndrome

Autosomal dominant polycystic kidney disease (ADPKD)

Autosomal dominant distal renal tubular acidosis (dRTA)

Autosomal dominant hypocalcemia

Autosomal dominant hypophosphatemic rickets

Autosomal dominant intermediate Charcot-Marie-Tooth disease type E

Autosomal dominant polycystic kidney disease type 1 with tuberous sclerosis (ADPKD-TSC)

Autosomal dominant primary hypomagnesemia with hypocalciuria

Autosomal dominant proximal renal tubular acidosis

Autosomal dominant tubulointerstitial kidney disease (ADTKD)

Autosomal recessive distal renal tubular acidosis (dRTA)

Autosomal recessive hypophosphatemic rickets

Autosomal recessive infantile hypercalcemia

Autosomal recessive polycystic kidney disease (ARPKD)

Autosomal recessive proximal renal tubular acidosis

Bardet-Biedl syndrome

Bardet-Biedl syndrome (renal / urinary tract malformation)

Bartter syndrome

Beckwith-Wiedemann syndrome

Beh

Bilateral multicystic dysplastic kidney

Bladder exstrophy

BNAR (Bifid nose, anorectal and renal anomalies) syndrome

BOR (branchio-oto-renal) syndrome

Brachydactyly-arterial hypertension syndrome

Buerger disease

C3 glomerulonephritis

C3 glomerulopathy

C3 glomerulopathy secondary to MGRS

Cat-eye syndrome

Caudal regression sequence

Cerebrorenodigital (Meckel-like) syndrome

CHARGE syndrome

Childhood-onset systemic lupus erythematosus (SLE)

Childhood-onset systemic lupus erythematosus associated TMA (SLE)

Cloacal exstrophy

Collagen type III glomerulopathy

Collagenopathies (inc. Alport syndrome)

Collagenopathy, not further specified

Congenital hydronephrosis

Congenital membranous nephropathy due to maternal anti-neutral endopeptidase alloimmunization

Congenital nephrotic syndrome, Finnish type

Congenital nephrotic syndrome, no genetic cause specified

Congenital nephrotic syndrome-interstitial lung disease-epidermolysis bullosa syndrome

Congenital primary megaureter

Congenital primary megaureter, nonrefluxing and unobstructed form

Congenital primary megaureter, obstructed form

Congenital primary megaureter, refluxing and obstructed

Congenital primary megaureter, refluxing form

Congenital thrombotic thrombocytopenic purpura (TTP)

Cornelia de Lange syndrome

Cranioectodermal dysplasia (Sensenbrenner syndrome)

CREST syndrome

Cryoglobulinemic vasculitis

Cystinuria

De novo thrombotic microangiopathy after kidney transplantation

Dense deposit disease

Dent disease

Dent disease type 1 (CLCN5-related)

Dent disease type 2 (OCRL-related)

Denys-Drash syndrome

Dermatomyositis

Di George syndrome (22q11.2 deletion)

Diffuse cutaneous systemic sclerosis

Distal renal tubular acidosis

Distal renal tubular acidosis with hemolytic anemia

Donnai-Barrow syndrome

Drug-related renal tubular dysgenesis

EAST syndrome

EEC (Ectrodactyly-ectodermal dysplasia-cleft lip/palate) syndrome

Ellis van Creveld syndrome

Eosinophilic granulomatosis with polyangiitis

Epispadias

Exstrophy-epispadias complex

Fabry disease

Faconi syndrome, induced by other heavy metal

Familial hyperaldosteronism

Familial hyperaldosteronism type I (glucocorticoid-sensitive hypertension)

Familial hyperaldosteronism type II

Familial hyperaldosteronism type III

Familial Mediterranean fever

Familial primary hypomagnesemia with hypercalciuria and nephrocalcinosis

Familial primary hypomagnesemia with hypercalciuria and nephrocalcinosis with severe ocular involvement

Familial primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement

Familial primary hypomagnesemia with hypocalcuria

Familial primary hypomagnesemia with normocalciuria and normocalcemia

Familial renal glucosuria

Familial steroid-resistant nephrotic syndrome with adrenal insufficiency

Fanconi syndrome, Cisplatin induced

Fanconi syndrome, drug induced

Fanconi syndrome, heavy metal induced

Fanconi syndrome, induced by other drug

Fanconi syndrome, lead induced

Fanconi syndrome, mercury induced

Fanconi syndrome, monoclonal Ig light chain-associated

Fanconi syndrome, primary

Fetal lower urinary tract obstruction (LUTO)

Fibronectin glomerulopathy

Fraser syndrome

Frasier syndrome

Galactosemia

Galloway-Mowat syndrome

Generalized pseudohypoaldosteronism type 1

Genetic hypertension involving tubular dysfunction

Genetic primary hypomagnesemia

Genetic primary hypomagnesemia with normocalcuria

Genetic steroid-resistant nephrotic syndrome (SRNS)

Giant cell arteritis

Gitelman syndrome

Gitelman-like syndrome

Glomerulopathy as part of a genetic systemic disease

Glomerulopathy as part of a non-genetic systemic disease

Glomerulopathy as part of a syndromic disorder

Glomerulopathy as part of a systemic disorder

Glycogen storage disease due to glucose-6-phosphatase deficiency

Glycogen storage disease due to GLUT2 deficiency

Granulomatosis with polyangiitis (Wegener)

HANAC syndrome

HDR (Hypoparathyroidism-deafness-renal disease) syndrome

Heavy chain deposition disease

Hemolytic uremic syndrome (HUS)

Hereditary amyloidosis with primary renal involement

Hereditary hypophosphatemic rickets with hypercalciuria

Hereditary renal hypouricemia

Hereditary xanthinuria

HNF1B-related autosomal dominant tubulointerstitial kidney disease (ADTKD)

Holoprosencephaly-radial heart renal anomalies syndrome

Hypercalciuria, idiopathic

Hypocomplementemic urticarial vasculitis

Hypokalemic alkalosis, CLDN10 associated

Hypomagnesaemia, drug-induced

Hypophosphatemia, dominant, with nephrolithiasis or osteoporosis

Hypophosphatemic rickets

Hypotonia-cystinuria syndrome

Hypotrichosis-lymphedema-telangiectasia-renal defect syndrome

Hypoxanthine-guanine phosphoribosyltransferase deficiency

Idiopathic multidrug-resistant nephrotic syndrome

Idiopathic non-lupus full-house nephropathy

Idiopathic steroid-resistant nephrotic syndrome

Idiopathic steroid-resistant nephrotic syndrome with sensitivity to second-line immunouppressive therapy

Idiopathic steroid-sensitive nephrotic syndrome

Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance

IgA nephropathy

IgG4-related kidney disease

Imerslund-Gräsbeck syndrome

Immunoglobulin A vasculitis (IgAV, Henoch Schonlein nephritis, PSH)

Immunoglobulin-mediated membranoproliferative glomerulonephritis (MPGN)

Immunotactoid glomerulopathy

Immunotactoid or fibrillary glomerulopathy

Infantile nephronophthisis

Infantile nephropathic cystinosis

Infection-related hemolytic uremic syndrome (HUS)

Infundibulopelvic stenosis-multicystic kidney syndrome

Isolated autosomal dominant hypomagnesemia, Glaudemans type

Jeune syndrome (ciliopathies)

Jeune syndrome (renal / urinary tract malformation)

Joubert syndrome with oculorenal defect

Joubert syndrome with renal defect

Juvenile dermatomyositis

Juvenile nephronophthisis

Juvenile nephropathic cystinosis

Juvenile polymyositis

Kabuki Syndrome

Kallmann syndrome

Karyomegalic interstitial nephritis

Late-onset nephronophthisis

LCAT deficiency

Leigh syndrome with nephrotic syndrome

Lethal fetal brain malformation-duodenal atresia-bilateral renal hypoplasia syndrome

Lethal fetal cerebrorenogenitourinary agenesis/hypoplasia syndrome

Liddle Syndrome

Light and heavy chain deposition disease

Light chain deposition disease

Limited cutaneous systemic sclerosis

Limited systemic sclerosis

Lipoprotein glomerulopathy

Lowe syndrome

Lysinuric protein intolerance

Mayer-Rokitansky-K

Meckel syndrome (ciliopathies)

Meckel syndrome (renal / urinary tract malformation)

Medullary sponge kidney

Megacystis-megaureter syndrome

Megacystis-microcolon-intestinal hypoperistalsis syndrome

Membranous glomerulonephritis

Methylmalonic acidemia (without homocystinuria)

Methylmalonic acidemia, Vitamin B12-responsive

Methylmalonic acidemia, Vitamin B12-unresponsive

Microscopic (including familial) hematuria

Microscopic polyangiitis

Mixed connective tissue disease

Monoclonal gammopathy of renal significance (MGRS)

MUC1-related autosomal dominant tubulointerstitial kidney disease (ADTKD)

Muckle-Wells syndrome

Multicentric carpo-tarsal osteolysis with or without nephropathy

Multicystic dysplastic kidney

MYH9-related disease

Nail-patella syndrome

Neonatal diabetes-congenital hypothyroidism-congenital glaucoma-hepatic fibrosis-polycystic kidneys syndrome

Nephrogenic diabetes insipidus

Nephrogenic syndrome of inappropriate antidiuresis

Nephronophthisis

Nephropathic cystinosis

Nephrotic syndrome without extrarenal manifestations

Nephrotic syndrome, familial, steroid-resistant (SRNS), with sensorineural deafness

Nephrotic syndrome, genetic

Nephrotic syndrome, idiopathic

Nephrotic syndrome, syndromic, not otherwise specified

Nephrotic syndrome-deafness-pretibial epidermolysis bullosa syndrome

Neurofibromatosis type 1

Neurogenic bladder, congenital or acquired

Non-amyloid fibrillary glomerulopathy

Non-amyloid monoclonal immunoglobulin deposition disease

Non-syndromic renal or urinary tract malformation

Noonan syndrome

Ochoa syndrome

OFD (Orofaciodigital) syndrome type 1

Oligomeganephronia

Oncogenic osteomalacia

Osteopetrosis with renal tubular acidosis

Other autoimmune dRTA

Pallister-Hall syndrome

Pauci-immune glomerulonephritis

Pauci-immune glomerulonephritis with ANCA

Pauci-immune glomerulonephritis without ANCA

Phosphoribosylpyrophosphate synthetase superactivity

Pierson syndrome

Polyarteritis nodosa

Polymyositis

Posterior urethral valve (PUV)

Primary biliary cholangitis

Primary hyperoxaluria

Primary hyperoxaluria type 1 (PH1)

Primary hyperoxaluria type 2 (PH2)

Primary hyperoxaluria type 3 (PH3)

Primary hypomagnesemia with refractory seizures and intellectual disability

Primary hypomagnesemia with secondary hypocalcemia

Primary localized amyloidosis

Primary megaureter, adult-onset form

Primary membranoproliferative glomerulonephritis (MPGN)

Primary renal tubular acidosis

Primary systemic amyloidosis

Proliferative glomerulonephritis with monoclonal immune deposits (PGNMID)

Proximal renal tubular acidosis

Prune belly syndrome

Pseudohypoaldosteronism type 1

Pseudohypoaldosteronism type 2

RCAD (Renal cysts and diabetes) syndrome

Relapsing polychondritis

REN-related autosomal dominant tubulointerstitial kidney disease (ADTKD, FJHN type 1)

Renal agenesis

Renal agenesis, bilateral

Renal agenesis, unilateral

Renal artery stenosis, congenital

Renal coloboma syndrome

Renal dysplasia

Renal dysplasia, bilateral

Renal dysplasia, unilateral

Renal hypoplasia

Renal hypoplasia, bilateral

Renal hypoplasia, unilateral

Renal nutcracker syndrome

Renal pseudohypoaldosteronism type 1

Renal tubular dysgenesis

Renal tubular dysgenesis due to twin-twin transfusion

Renal tubular dysgenesis of genetic origin

Renal-hepatic-pancreatic dysplasia

Reynolds syndrome

RHYNS (retinitis pigmentosa, hypopituitarism, nephronophthisis, skeletal dysaplasia) syndrome

Rubinstein-Taybi syndrome

Saldino-Mainzer (conorenal) syndrome

Sarcoidosis

Schimke immuno-osseous dysplasia

Schinzel-Giedion syndrome

Senior-Boichis syndrome

Senior-Loken syndrome

SERKAL syndrome

Severe oculo-renal-cerebellar syndrome

Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS)

Simpson-Golabi-Behmel syndrome

Sjogren Syndrome

Smith-Lemli-Opitz syndrome

Streptoccous pneumoniae-associated hemolytic uremic syndrome (HUS)

Syndromic renal or urinary tract malformation

Systemic Lupus Erythematosus (SLE)

Systemic Lupus Erythematosus associated TMA (SLE)

Systemic sclerosis

Systemic vasculitis associated with glomerulopathy

Takayasu arteritis

Thrombotic thrombocytopenic purpura (TTP)

Thyrocerebrorenal syndrome

Townes-Brocks syndrome

Trisomy 13

Trisomy 18

Tuberous sclerosis complex (TSC)

Tubulointerstitial nephritis and uveitis syndrome

Tubulopathy due to mitochondrial oxidative phosphorylation disorder

Turner syndrome

UMOD-related autosomal dominant tubulointerstitial kidney disease (ADTKD)

Unilateral multicystic dysplastic kidney

Ureteropelvic junction (UPJ) obstruction (bilateral or in solitary kidney)

VACTERL/VATER association

Vesicoureteric reflux (VUR), familial

Vesicoureteric reflux (VUR), high-grade

Von Hippel-Lindau disease (vHL)

WAGR syndrome

Williams syndrome

X-linked hypophosphatemic rickets (XLHR)

Xanthinuria type I

Xanthinuria type II