A	C98983	Melnick-Fraser Syndrome	Branchio-oto-renal Syndrome	Melnick-Fraser Syndrome	An autosomal dominant inherited syndrome caused by mutations in the EYA1, SIX1, and SIX5 genes. It is characterized by the abnormal development of the second branchial arch, resulting in the formation of branchial cleft cysts and fistulae, hearing loss and ear malformations, and renal abnormalities.	A hereditary disorder caused by mutations in the EYA1 gene, which is characterized by hearing loss, structural defects of the ear, branchial fistulas or cysts, and early, progressive chronic renal failure.	C3101	C98983		C98983	C123272	Pediatric Nephrology Terminology		
C	C26847	Polyarteritis Nodosa	Polyarteritis Nodosa	Classic Polyarteritis Nodosa|PAN|Panarteritis Nodosa|Periarteritis Nodosa	A necrotizing vasculitis that affects medium-sized arteries, it is usually idiopathic, and may be triggered by viral infections (Hepatitis B, also others). A multisystem disease, it most commonly affects skin (nodules, livedo reticularis) and peripheral nerves; higher morbidity is associated with gastrointestinal, central nervous system, cardiac, and/or other organ involvement. Anti-neutrophil cytoplasmic antibodies (ANCA) are typically negative.	A necrotizing vasculitis that affects medium-sized arteries, it is usually idiopathic, and may be triggered by viral infections (Hepatitis B, also others). A multisystem disease, it most commonly affects skin (nodules, livedo reticularis) and peripheral nerves; higher morbidity is associated with gastrointestinal, central nervous system, cardiac, and/or other organ involvement. Anti-neutrophil cytoplasmic antibodies (ANCA) are typically negative.	C26912	C26847		C26847	C123272	Pediatric Nephrology Terminology		
C	C123055	Immune Complex Mediated Membranoproliferative Glomerulonephritis	Immune Complex Mediated Membranoproliferative Glomerulonephritis	Membranoproliferative Glomerulonephritis Type I|Mesangiocapillary Glomerulonephritis Type 1	Glomerulonephritis characterized by mesangial proliferation, endocapillary proliferation, and glomerular capillary wall remodeling with immune complex deposits from classical complement pathway activation.	Glomerulonephritis characterized by mesangial proliferation, endocapillary proliferation, and glomerular capillary wall remodeling with immune complex deposits from classical complement pathway activation.	C34644	C123055		C123055	C123272	Pediatric Nephrology Terminology		
C	C123185	P3 Hydronephrosis	P3 Hydronephrosis		Postnatal Hydronephrosis with the following clinical findings: 1) calyceal dilation and the ureter are the same as those in UTD P2, 2) the renal parenchymal is thinned, has increased echogenicity and/or has decreased corticomedullary differentiation, or 3) the bladder is abnormal (wall thickening, ureterocele, posterior urethral dilation). Cases in which there are parenchymal abnormalities but the APRPD is less than 15 mm, are classified as UTD P3. (Adapted from: Hiep T. Nguyen, Carol B. Benson, Bryann Bromley, Jeffrey B. Campbell, Jeanne Chow, Beverly Coleman, Christopher Cooper, Jude Crino, Kassa Darge, C.D. Anthony Herndon, Anthony O. Odibo, Michael J.G. Somers, Deborah R. Stein; Multidisciplinary consensus on the classification of prenatal and postnatal urinary tract dilation (UTD classification system); Pediatric Urology; December 2014 Volume 10, Issue 6, Pages 982-998)	Postnatal Hydronephrosis with the following clinical findings: 1) calyceal dilation and the ureter are the same as those in UTD P2, 2) the renal parenchymal is thinned, has increased echogenicity and/or has decreased corticomedullary differentiation, or 3) the bladder is abnormal (wall thickening, ureterocele, posterior urethral dilation). Cases in which there are parenchymal abnormalities but the APRPD is less than15 mm, are classified as UTD P3. (Adapted from: Hiep T. Nguyen, Carol B. Benson, Bryann Bromley, Jeffrey B. Campbell, Jeanne Chow, Beverly Coleman, Christopher Cooper, Jude Crino, Kassa Darge, C.D. Anthony Herndon, Anthony O. Odibo, Michael J.G. Somers, Deborah R. Stein; Multidisciplinary consensus on the classification of prenatal and postnatal urinary tract dilation (UTD classification system); Pediatric Urology; December 2014 Volume 10, Issue 6, Pages 982-998)	C26796	C123185		C123185	C123272	Pediatric Nephrology Terminology		
C	C114688	Urolithiasis	Urolithiasis	Urinary Stones	Stone(s) within the urinary tract.	Stone(s) within the urinary tract.	C3430	C114688		C114688	C123272	Pediatric Nephrology Terminology		
D	C123224	Branchio-oto-renal Syndrome	Branchio-oto-renal Syndrome	Branchiootorenal Syndrome	A hereditary disorder caused by mutations in the EYA1 gene, which is characterized by hearing loss, structural defects of the ear, branchial fistulas or cysts, and early, progressive chronic renal failure.	A hereditary disorder caused by mutations in the EYA1 gene, which is characterized by hearing loss, structural defects of the ear, branchial fistulas or cysts, and early, progressive chronic renal failure.	C28193	C123224		C123224	C123272	Pediatric Nephrology Terminology