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Cortical sclerosis

MedGen UID:
870710
Concept ID:
C4025164
Pathologic Function
HPO: HP:0005652

Definition

Sclerosis (abnormal hardening) of cortical bone, characterized by increased radiodensity. [from HPO]

Term Hierarchy

Conditions with this feature

Dentin dysplasia-sclerotic bones syndrome
MedGen UID:
377618
Concept ID:
C1852201
Disease or Syndrome
Dentin dysplasia-sclerotic bones syndrome is a rare, genetic odontologic disease characterized by the clinical, radiographic, and histologic features of dentine dysplasia and osteosclerosis of all long bones, with heavy cortical bone and narrowed or occluded marrow spaces. There have been no further descriptions in the literature since 1977.
Craniodiaphyseal dysplasia, autosomal dominant
MedGen UID:
382678
Concept ID:
C2675746
Disease or Syndrome
Craniodiaphyseal dysplasia (CDD) is a severe bone dysplasia characterized by massive generalized hyperostosis and sclerosis, especially involving the skull and facial bones. Progressive bony encroachment upon cranial foramina leads to severe neurologic impairment in childhood (summary by Brueton and Winter, 1990). The sclerosis is so severe that the resulting facial distortion is referred to as 'leontiasis ossea' (leonine facies), and the bone deposition results in progressive stenosis of craniofacial foramina (summary by Kim et al., 2011).
Osteopetrosis, autosomal recessive 9
MedGen UID:
1841123
Concept ID:
C5830487
Disease or Syndrome
Autosomal recessive osteopetrosis-9 (OPTB9) is characterized by increased bone density and bone fragility, as well as renal failure. Vision may be compromised due to compression of the optic nerve secondary to osteopetrotic stenosis of the optic nerve canal (Xue et al., 2022). For a general phenotypic description and discussion of genetic heterogeneity of autosomal recessive osteopetrosis, see OPTB1 (259700).

Professional guidelines

PubMed

Banwell B, Bennett JL, Marignier R, Kim HJ, Brilot F, Flanagan EP, Ramanathan S, Waters P, Tenembaum S, Graves JS, Chitnis T, Brandt AU, Hemingway C, Neuteboom R, Pandit L, Reindl M, Saiz A, Sato DK, Rostasy K, Paul F, Pittock SJ, Fujihara K, Palace J
Lancet Neurol 2023 Mar;22(3):268-282. Epub 2023 Jan 24 doi: 10.1016/S1474-4422(22)00431-8. PMID: 36706773
Abboud H, Probasco JC, Irani S, Ances B, Benavides DR, Bradshaw M, Christo PP, Dale RC, Fernandez-Fournier M, Flanagan EP, Gadoth A, George P, Grebenciucova E, Jammoul A, Lee ST, Li Y, Matiello M, Morse AM, Rae-Grant A, Rojas G, Rossman I, Schmitt S, Venkatesan A, Vernino S, Pittock SJ, Titulaer MJ; Autoimmune Encephalitis Alliance Clinicians Network
J Neurol Neurosurg Psychiatry 2021 Jul;92(7):757-768. Epub 2021 Mar 1 doi: 10.1136/jnnp-2020-325300. PMID: 33649022Free PMC Article
Thompson AJ, Banwell BL, Barkhof F, Carroll WM, Coetzee T, Comi G, Correale J, Fazekas F, Filippi M, Freedman MS, Fujihara K, Galetta SL, Hartung HP, Kappos L, Lublin FD, Marrie RA, Miller AE, Miller DH, Montalban X, Mowry EM, Sorensen PS, Tintoré M, Traboulsee AL, Trojano M, Uitdehaag BMJ, Vukusic S, Waubant E, Weinshenker BG, Reingold SC, Cohen JA
Lancet Neurol 2018 Feb;17(2):162-173. Epub 2017 Dec 21 doi: 10.1016/S1474-4422(17)30470-2. PMID: 29275977

Recent clinical studies

Etiology

You X, Sun X, Yang C, Fang Y
Medicine (Baltimore) 2017 Dec;96(49):e9058. doi: 10.1097/MD.0000000000009058. PMID: 29245313Free PMC Article
Kotnis N, James SL
Skeletal Radiol 2015 Oct;44(10):1461-6. Epub 2015 Jun 21 doi: 10.1007/s00256-015-2191-8. PMID: 26093538
Lee JI, Suh DH, Byun JS, Bae JH, Hong JY, Park JH, Park JW
J Hand Surg Am 2012 May;37(5):975-81. doi: 10.1016/j.jhsa.2012.02.038. PMID: 22541154
Shill HA, Adler CH, Sabbagh MN, Connor DJ, Caviness JN, Hentz JG, Beach TG
Neurology 2008 Apr 15;70(16 Pt 2):1452-5. doi: 10.1212/01.wnl.0000310425.76205.02. PMID: 18413570
Haworth JM, Watt I, Park WM, Roylance J
Br J Radiol 1981 Nov;54(647):932-8. doi: 10.1259/0007-1285-54-647-932. PMID: 6946854

Diagnosis

Vekszler G, Granner M, Nebot Valenzuela E, Winter E, Dockner M, Weber GW, Pretterklieber M, Teschler-Nicola M, Pietschmann P
Wien Klin Wochenschr 2022 Jun;134(11-12):449-457. Epub 2022 Mar 20 doi: 10.1007/s00508-022-02017-y. PMID: 35307770Free PMC Article
You X, Sun X, Yang C, Fang Y
Medicine (Baltimore) 2017 Dec;96(49):e9058. doi: 10.1097/MD.0000000000009058. PMID: 29245313Free PMC Article
Miller SF
Pediatr Radiol 2014 Jan;44(1):56-63. Epub 2013 Aug 17 doi: 10.1007/s00247-013-2770-6. PMID: 23955368
Haworth JM, Watt I, Park WM, Roylance J
Br J Radiol 1981 Nov;54(647):932-8. doi: 10.1259/0007-1285-54-647-932. PMID: 6946854
Taylor PM
J Foot Surg 1976 Summer;15(2):55-8. PMID: 1030721

Therapy

Wizniter M, Packer RJ, Rorke LB, Meadows AT
J Neurooncol 1987;4(4):353-60. doi: 10.1007/BF00195606. PMID: 3471868
Söreide O, Lillestöl J, Alho A, Hvidsten K
Acta Orthop Scand 1982 Apr;53(2):265-72. doi: 10.3109/17453678208992214. PMID: 7136575

Prognosis

Marina V, Milena R, Vesna P, Slađana P, Vera A
Skeletal Radiol 2015 Jun;44(6):863-8. Epub 2014 Nov 30 doi: 10.1007/s00256-014-2067-3. PMID: 25433719
Akansu B, Atık E, Altintaş S, Kalaci A, Canda S
Turk Patoloji Derg 2012;28(2):172-4. doi: 10.5146/tjpath.2012.01119. PMID: 22627638
Devaney K, Vinh TN, Sweet DE
Clin Orthop Relat Res 1994 Mar;(300):233-40. PMID: 8131341
Crosby EB, Linscheid RL, Dobyns JH
J Hand Surg Am 1978 May;3(3):223-34. doi: 10.1016/s0363-5023(78)80086-0. PMID: 659818
Taylor PM
J Foot Surg 1976 Summer;15(2):55-8. PMID: 1030721

Clinical prediction guides

Vekszler G, Granner M, Nebot Valenzuela E, Winter E, Dockner M, Weber GW, Pretterklieber M, Teschler-Nicola M, Pietschmann P
Wien Klin Wochenschr 2022 Jun;134(11-12):449-457. Epub 2022 Mar 20 doi: 10.1007/s00508-022-02017-y. PMID: 35307770Free PMC Article
Akansu B, Atık E, Altintaş S, Kalaci A, Canda S
Turk Patoloji Derg 2012;28(2):172-4. doi: 10.5146/tjpath.2012.01119. PMID: 22627638
Lee JI, Suh DH, Byun JS, Bae JH, Hong JY, Park JH, Park JW
J Hand Surg Am 2012 May;37(5):975-81. doi: 10.1016/j.jhsa.2012.02.038. PMID: 22541154
Ratech H, Greco MA, Gallo G, Rimoin DL, Kamino H, Hirschhorn R
Am J Pathol 1985 Jul;120(1):157-69. PMID: 4014441Free PMC Article
Crosby EB, Linscheid RL, Dobyns JH
J Hand Surg Am 1978 May;3(3):223-34. doi: 10.1016/s0363-5023(78)80086-0. PMID: 659818

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