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Osteogenesis imperfecta, type 23(OI23)

MedGen UID:
1846121
Concept ID:
C5882757
Disease or Syndrome
Synonym: Osteogenesis imperfecta, type XXIII
 
Gene (location): PHLDB1 (11q23.3)
 
Monarch Initiative: MONDO:0957988
OMIM®: 620639

Definition

Osteogenesis imperfecta type XXIII (OI23) is a mild recessive form of OI, characterized by osteopenia with or without recurrent fractures, platyspondyly, short and bowed long bones, and widened metaphyses. Metaphyseal and vertebral changes regress after early childhood; osteopenia persists, but responds well to bisphosphonate (Tuysuz et al., 2023). [from OMIM]

Clinical features

From HPO
Genu valgum
MedGen UID:
154364
Concept ID:
C0576093
Anatomical Abnormality
The legs angle inward, such that the knees are close together and the ankles far apart.
Shallow acetabular fossae
MedGen UID:
344384
Concept ID:
C1854910
Finding
Broad femoral head
MedGen UID:
870185
Concept ID:
C4024619
Finding
Increased width of the femoral head.
Short stature
MedGen UID:
87607
Concept ID:
C0349588
Finding
A height below that which is expected according to age and gender norms. Although there is no universally accepted definition of short stature, many refer to "short stature" as height more than 2 standard deviations below the mean for age and gender (or below the 3rd percentile for age and gender dependent norms).
Truncal obesity
MedGen UID:
1637490
Concept ID:
C4551560
Finding
Obesity located preferentially in the trunk of the body as opposed to the extremities.
Torticollis
MedGen UID:
11859
Concept ID:
C0040485
Sign or Symptom
Torticollis is a twisted neck as a result of shortening of sternocleidomastoid muscle. This short and fibrotic muscle pulls the head laterally and rotates the chin and face to the opposite end. Facial asymmetry may be a manifestation (summary by Engin et al., 1997).
Recurrent fractures
MedGen UID:
42094
Concept ID:
C0016655
Injury or Poisoning
The repeated occurrence of bone fractures (implying an abnormally increased tendency for fracture).
Hip dislocation
MedGen UID:
42455
Concept ID:
C0019554
Injury or Poisoning
Displacement of the femur from its normal location in the hip joint.
Osteopenia
MedGen UID:
18222
Concept ID:
C0029453
Disease or Syndrome
Osteopenia is a term to define bone density that is not normal but also not as low as osteoporosis. By definition from the World Health Organization osteopenia is defined by bone densitometry as a T score -1 to -2.5.
Severe platyspondyly
MedGen UID:
338014
Concept ID:
C1850293
Finding
Reduced bone mineral density
MedGen UID:
393152
Concept ID:
C2674432
Finding
A reduction of bone mineral density, that is, of the amount of matter per cubic centimeter of bones.
Bowing of the legs
MedGen UID:
1807399
Concept ID:
C5574706
Finding
A bending or abnormal curvature affecting a long bone of the leg.
Insulin resistance
MedGen UID:
43904
Concept ID:
C0021655
Pathologic Function
Increased resistance towards insulin, that is, diminished effectiveness of insulin in reducing blood glucose levels.
Acanthosis nigricans
MedGen UID:
54
Concept ID:
C0000889
Disease or Syndrome
A dermatosis characterized by thickened, hyperpigmented plaques, typically on the intertriginous surfaces and neck.
Blue sclerae
MedGen UID:
154236
Concept ID:
C0542514
Finding
An abnormal bluish coloration of the sclera.

Professional guidelines

PubMed

MacCarrick G, Aradhya S, Bailey M, Chu D, Hunt A, Izzo E, Krakow D, Mackenzie W, Poll S, Raggio C, Shediac R, White KK, McLaughlin HM, Seratti G
Am J Med Genet A 2024 Sep;194(9):e63646. Epub 2024 May 3 doi: 10.1002/ajmg.a.63646. PMID: 38702915
Montpetit K, Palomo T, Glorieux FH, Fassier F, Rauch F
Arch Phys Med Rehabil 2015 Oct;96(10):1834-9. Epub 2015 Jul 2 doi: 10.1016/j.apmr.2015.06.006. PMID: 26140741
Lindahl K, Åström E, Rubin CJ, Grigelioniene G, Malmgren B, Ljunggren Ö, Kindmark A
Eur J Hum Genet 2015 Aug;23(8):1042-50. Epub 2015 May 6 doi: 10.1038/ejhg.2015.81. PMID: 25944380Free PMC Article

Recent clinical studies

Etiology

Chen P, Zhou Y, Tan Z, Lin Y, Lin DL, Wu J, Li Z, Shek HT, Wu J, Hu Y, Zhu F, Chan D, Cheung KM, To MK
Orphanet J Rare Dis 2023 Sep 20;18(1):295. doi: 10.1186/s13023-023-02906-z. PMID: 37730650Free PMC Article
Harsevoort AGJ, Gooijer K, van Dijk FS, van der Grijn DAFM, Franken AAM, Dommisse AMV, Janus GJM
BMC Musculoskelet Disord 2020 Jan 3;21(1):6. doi: 10.1186/s12891-019-3000-7. PMID: 31900144Free PMC Article
Folkestad L
Dan Med J 2018 Apr;65(4) PMID: 29619932
Calder AD
Endocr Dev 2015;28:56-71. Epub 2015 Jun 12 doi: 10.1159/000380992. PMID: 26138835
Greeley CS, Donaruma-Kwoh M, Vettimattam M, Lobo C, Williard C, Mazur L
J Pediatr Orthop 2013 Jan;33(1):32-6. doi: 10.1097/BPO.0b013e318279c55d. PMID: 23232376Free PMC Article

Diagnosis

Harsevoort AGJ, Gooijer K, van Dijk FS, van der Grijn DAFM, Franken AAM, Dommisse AMV, Janus GJM
BMC Musculoskelet Disord 2020 Jan 3;21(1):6. doi: 10.1186/s12891-019-3000-7. PMID: 31900144Free PMC Article
Folkestad L
Dan Med J 2018 Apr;65(4) PMID: 29619932
Calder AD
Endocr Dev 2015;28:56-71. Epub 2015 Jun 12 doi: 10.1159/000380992. PMID: 26138835
Tedeschi E, Antoniazzi F, Venturi G, Zamboni G, Tatò L
Pediatr Endocrinol Rev 2006 Sep;4(1):40-6. PMID: 17021582
Cooper KL
Curr Probl Diagn Radiol 1994 Mar-Apr;23(2):29-68. doi: 10.1016/0363-0188(94)90006-x. PMID: 8174391

Therapy

Pantoja LLQ, Lustosa M, Yamaguti PM, Rosa LS, Leite AF, Figueiredo PTS, Castro LC, Acevedo AC
Calcif Tissue Int 2022 Mar;110(3):303-312. Epub 2021 Oct 22 doi: 10.1007/s00223-021-00915-3. PMID: 34677656
Kashii M, Kanayama S, Kitaoka T, Makino T, Kaito T, Iwasaki M, Kubota T, Yamamoto T, Ozono K, Yoshikawa H
J Bone Miner Metab 2019 May;37(3):545-553. Epub 2018 Sep 5 doi: 10.1007/s00774-018-0952-x. PMID: 30187275
Folkestad L
Dan Med J 2018 Apr;65(4) PMID: 29619932
Trejo P, Fassier F, Glorieux FH, Rauch F
J Bone Miner Res 2017 May;32(5):1034-1039. Epub 2017 Feb 28 doi: 10.1002/jbmr.3071. PMID: 28019684
Paschalis EP, Gamsjaeger S, Fratzl-Zelman N, Roschger P, Masic A, Brozek W, Hassler N, Glorieux FH, Rauch F, Klaushofer K, Fratzl P
J Bone Miner Res 2016 May;31(5):1050-9. Epub 2016 Feb 11 doi: 10.1002/jbmr.2780. PMID: 26748579

Prognosis

Harsevoort AGJ, Gooijer K, van Dijk FS, van der Grijn DAFM, Franken AAM, Dommisse AMV, Janus GJM
BMC Musculoskelet Disord 2020 Jan 3;21(1):6. doi: 10.1186/s12891-019-3000-7. PMID: 31900144Free PMC Article
Yimgang DP, Brizola E, Shapiro JR
J Matern Fetal Neonatal Med 2016 Dec;29(23):3889-93. Epub 2016 Mar 7 doi: 10.3109/14767058.2016.1151870. PMID: 26857929
Lindahl K, Åström E, Rubin CJ, Grigelioniene G, Malmgren B, Ljunggren Ö, Kindmark A
Eur J Hum Genet 2015 Aug;23(8):1042-50. Epub 2015 May 6 doi: 10.1038/ejhg.2015.81. PMID: 25944380Free PMC Article
Cheung MS, Glorieux FH, Rauch F
J Bone Miner Res 2007 Aug;22(8):1181-6. doi: 10.1359/jbmr.070418. PMID: 17451374
Tedeschi E, Antoniazzi F, Venturi G, Zamboni G, Tatò L
Pediatr Endocrinol Rev 2006 Sep;4(1):40-6. PMID: 17021582

Clinical prediction guides

Madhuri V, Selina A, Loganathan L, Kumar A, Kumar V, Raymond R, Ramesh S, Vincy N, Joel G, James D, Kandagaddala M, B A
Ann Hum Genet 2021 Jan;85(1):37-46. Epub 2020 Aug 7 doi: 10.1111/ahg.12403. PMID: 32770541
Harsevoort AGJ, Gooijer K, van Dijk FS, van der Grijn DAFM, Franken AAM, Dommisse AMV, Janus GJM
BMC Musculoskelet Disord 2020 Jan 3;21(1):6. doi: 10.1186/s12891-019-3000-7. PMID: 31900144Free PMC Article
Rolvien T, Stürznickel J, Schmidt FN, Butscheidt S, Schmidt T, Busse B, Mundlos S, Schinke T, Kornak U, Amling M, Oheim R
Calcif Tissue Int 2018 Nov;103(5):512-521. Epub 2018 Jun 26 doi: 10.1007/s00223-018-0447-8. PMID: 29946973
Folkestad L
Dan Med J 2018 Apr;65(4) PMID: 29619932
Lindahl K, Åström E, Rubin CJ, Grigelioniene G, Malmgren B, Ljunggren Ö, Kindmark A
Eur J Hum Genet 2015 Aug;23(8):1042-50. Epub 2015 May 6 doi: 10.1038/ejhg.2015.81. PMID: 25944380Free PMC Article

Recent systematic reviews

Prado HV, Soares ECB, Carneiro NCR, Vilar ICO, Abreu LG, Borges-Oliveira AC
J Appl Oral Sci 2023;31:e20230040. Epub 2023 Sep 4 doi: 10.1590/1678-7757-2023-0040. PMID: 37672427Free PMC Article

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