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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
Renal pyramid echogenicity in ureteropelvic junction obstruction: correlation between altered echogenicity and differential renal function.
Chavhan G, Daneman A, Moineddin R, Lim R, Langlois V, Traubici J
Pediatr Radiol 2008 Oct;38(10):1068-73. Epub 2008 Jul 17 doi: 10.1007/s00247-008-0943-5. PMID: 18633607
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
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

Diagnosis

Brain MRI features and scoring of leukodystrophy in adult-onset Krabbe disease.
Cousyn L, Law-Ye B, Pyatigorskaya N, Debs R, Froissart R, Piraud M, Federico A, Salvatore S, Cerase A, Macário MC, Durães J, Kim SH, Adachi H, Audoin B, Ayrignac X, Da Y, Henderson R, La Piana R, Laule C, Nakamagoe K, Raininko R, Schols L, Sirrs SM, Viader F, Jastrzębski K, Leclercq D, Nadjar Y
Neurology 2019 Aug 13;93(7):e647-e652. Epub 2019 Jul 23 doi: 10.1212/WNL.0000000000007943. PMID: 31337714
Assessment of the corticospinal fiber integrity in mirror movement disorder.
Solmaz B, Özyurt MG, Ata DB, Akçimen F, Shabsog M, Türker KS, Dalçik H, Algin O, Başak AN, Özgür M, Çavdar S
J Clin Neurosci 2018 Aug;54:69-76. Epub 2018 Jun 12 doi: 10.1016/j.jocn.2018.06.001. PMID: 29907388
Heart-Shaped Bilateral Medullary Pyramidal Infarction as a Pathognomonic Finding of Anterior Spinal Artery Occlusion.
Searcy S, Akinduro OO, Spector A, Yoon JW, Brown BL, Freeman WD
Neurocrit Care 2018 Jun;28(3):388-394. doi: 10.1007/s12028-017-0406-7. PMID: 28484930
Periodic limb movements in syringomyelia and syringobulbia.
Nogués M, Cammarota A, Leiguarda R, Rivero A, Pardal A, Encabo H
Mov Disord 2000 Jan;15(1):113-9. doi: 10.1002/1531-8257(200001)15:1<113::aid-mds1017>3.0.co;2-y. PMID: 10634249
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

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
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
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 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
Broadening the phenotype of the TWNK gene associated Perrault syndrome.
Fekete B, Pentelényi K, Rudas G, Gál A, Grosz Z, Illés A, Idris J, Csukly G, Domonkos A, Molnar MJ
BMC Med Genet 2019 Dec 18;20(1):198. doi: 10.1186/s12881-019-0934-4. PMID: 31852434Free PMC Article
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
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
Segmental "hypoplasia" of the kidney (Ask-Upmark).
Arant BS Jr, Sotelo-Avila C, Bernstein J
J Pediatr 1979 Dec;95(6):931-9. doi: 10.1016/s0022-3476(79)80279-6. PMID: 501498

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