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Abnormal medullary pyramid morphology

MedGen UID:
1368733
•Concept ID:
C4476730
•
Anatomical Abnormality
Synonym: Abnormality of medullary pyramid morphology
 
HPO: HP:0025361

Definition

A structural anomaly of the pyramid of the adult kidney, cone-shaped structures with a broad base adjacent to the renal cortex and the narrow apex that is termed papilla. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormal medullary pyramid morphology

Professional guidelines

PubMed

Posttraumatic hypothermia in the treatment of axonal damage in an animal model of traumatic axonal injury.
Koizumi H, Povlishock JT
J Neurosurg 1998 Aug;89(2):303-9. doi: 10.3171/jns.1998.89.2.0303. PMID: 9688127

Recent clinical studies

Etiology

Mitochondrial membrane protein-associated neurodegeneration (MPAN).
Hartig M, Prokisch H, Meitinger T, Klopstock T
Int Rev Neurobiol 2013;110:73-84. doi: 10.1016/B978-0-12-410502-7.00004-1. PMID: 24209434
Brainstem representation of vestibular evoked myogenic potentials.
Heide G, Luft B, Franke J, Schmidt P, Witte OW, Axer H
Clin Neurophysiol 2010 Jul;121(7):1102-8. Epub 2010 Mar 3 doi: 10.1016/j.clinph.2010.02.007. PMID: 20202897
Brain-stem compression in vertebrobasilar dolichoectasia. A multimodal electrophysiological study.
Passero S, Rossi S, Giannini F, Nuti D
Clin Neurophysiol 2001 Aug;112(8):1531-9. doi: 10.1016/s1388-2457(01)00597-1. PMID: 11459694
Ultrasound findings in renal parenchymal disease: comparison with histological appearances.
Page JE, Morgan SH, Eastwood JB, Smith SA, Webb DJ, Dilly SA, Chow J, Pottier A, Joseph AE
Clin Radiol 1994 Dec;49(12):867-70. doi: 10.1016/s0009-9260(05)82877-6. PMID: 7828393
Embolization of intramedullary spinal arteriovenous malformation fed by the anterior spinal artery with monitoring of the corticospinal motor evoked potential--case report.
Katayama Y, Tsubokawa T, Hirayama T, Himi K, Koyama S, Yamamoto T
Neurol Med Chir (Tokyo) 1991 Jul;31(7):401-5. doi: 10.2176/nmc.31.401. PMID: 1720218

Diagnosis

A case of diffuse kidney hyperechogenicity in early childhood associated with biallelic PKHD1 variants.
Krall P, Faundes V, Gálvez C, Cavagnaro F
Pediatr Nephrol 2024 Sep;39(9):2633-2636. Epub 2024 Mar 19 doi: 10.1007/s00467-024-06348-y. PMID: 38502226
Brainstem representation of vestibular evoked myogenic potentials.
Heide G, Luft B, Franke J, Schmidt P, Witte OW, Axer H
Clin Neurophysiol 2010 Jul;121(7):1102-8. Epub 2010 Mar 3 doi: 10.1016/j.clinph.2010.02.007. PMID: 20202897
Ultrasonography of discoid adrenals in Potter's syndrome: report of three cases.
Volberg FM, Dillard R, Sumner T
Am J Perinatol 1989 Jul;6(3):326-8. doi: 10.1055/s-2007-999605. PMID: 2659015
Congenital absence of pyramids and its significance in genetic diseases.
Chow CW, Halliday JL, Anderson RM, Danks DM, Fortune DW
Acta Neuropathol 1985;65(3-4):313-7. doi: 10.1007/BF00687014. PMID: 3976367
Sonographic features of ATN and of acute rejection in renal allografts.
Cruz C, Hricak H, Eyler WR, Levin NW, Uniewski M
Proc Eur Dial Transplant Assoc 1980;17:413-7. PMID: 7017682

Therapy

A review of the natural progression of medullary sponge kidney and a novel grading system based on intravenous urography findings.
Forster JA, Taylor J, Browning AJ, Biyani CS
Urol Int 2007;78(3):264-9. doi: 10.1159/000099350. PMID: 17406139
MRI-FLAIR images of the head show corticospinal tract alterations in ALS patients more frequently than T2-, T1- and proton-density-weighted images.
Hecht MJ, Fellner F, Fellner C, Hilz MJ, Heuss D, Neundörfer B
J Neurol Sci 2001 May 1;186(1-2):37-44. doi: 10.1016/s0022-510x(01)00503-2. PMID: 11412870

Prognosis

A case of diffuse kidney hyperechogenicity in early childhood associated with biallelic PKHD1 variants.
Krall P, Faundes V, Gálvez C, Cavagnaro F
Pediatr Nephrol 2024 Sep;39(9):2633-2636. Epub 2024 Mar 19 doi: 10.1007/s00467-024-06348-y. PMID: 38502226
Mitochondrial membrane protein-associated neurodegeneration (MPAN).
Hartig M, Prokisch H, Meitinger T, Klopstock T
Int Rev Neurobiol 2013;110:73-84. doi: 10.1016/B978-0-12-410502-7.00004-1. PMID: 24209434
Unusual sonographic features of ARPKD.
Okumura M, Bunduki V, Shiang C, Schultz R, Zugaib M
Prenat Diagn 2006 Apr;26(4):330-2. doi: 10.1002/pd.1410. PMID: 16491512
Ultrasound findings in renal parenchymal disease: comparison with histological appearances.
Page JE, Morgan SH, Eastwood JB, Smith SA, Webb DJ, Dilly SA, Chow J, Pottier A, Joseph AE
Clin Radiol 1994 Dec;49(12):867-70. doi: 10.1016/s0009-9260(05)82877-6. PMID: 7828393
Neonatal schizencephaly: comparison of brain imaging.
Chamberlain MC, Press GA, Bejar RF
Pediatr Neurol 1990 Nov-Dec;6(6):382-7. doi: 10.1016/0887-8994(90)90005-l. PMID: 2127354

Clinical prediction guides

A case of diffuse kidney hyperechogenicity in early childhood associated with biallelic PKHD1 variants.
Krall P, Faundes V, Gálvez C, Cavagnaro F
Pediatr Nephrol 2024 Sep;39(9):2633-2636. Epub 2024 Mar 19 doi: 10.1007/s00467-024-06348-y. PMID: 38502226
A taxonomy for brainstem cavernous malformations: subtypes of medullary lesions.
Catapano JS, Rumalla K, Srinivasan VM, Lawrence PM, Larson Keil K, Lawton MT
J Neurosurg 2023 Jan 1;138(1):128-146. Epub 2022 May 20 doi: 10.3171/2022.3.JNS22626. PMID: 35594887
Mitochondrial membrane protein-associated neurodegeneration (MPAN).
Hartig M, Prokisch H, Meitinger T, Klopstock T
Int Rev Neurobiol 2013;110:73-84. doi: 10.1016/B978-0-12-410502-7.00004-1. PMID: 24209434
Unusual sonographic features of ARPKD.
Okumura M, Bunduki V, Shiang C, Schultz R, Zugaib M
Prenat Diagn 2006 Apr;26(4):330-2. doi: 10.1002/pd.1410. PMID: 16491512
Congenital absence of pyramids and its significance in genetic diseases.
Chow CW, Halliday JL, Anderson RM, Danks DM, Fortune DW
Acta Neuropathol 1985;65(3-4):313-7. doi: 10.1007/BF00687014. PMID: 3976367

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