U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Hypochondrogenesis

MedGen UID:
107448
Concept ID:
C0542428
Congenital Abnormality
Synonym: HYPOCHONDROGENESIS
SNOMED CT: Hypochondrogenesis (205483007)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Monarch Initiative: MONDO:0019669
OMIM®: 120140; 200610
Orphanet: ORPHA93297

Definition

Affected infants have short arms and legs, a small chest with short ribs, and underdeveloped lungs. Bones in the skull develop normally, but the bones of the spine (vertebrae) and pelvis do not harden (ossify) properly. The face appears flat and oval-shaped, with widely spaced eyes, a small chin, and, in some cases, an opening in the roof of the mouth called a cleft palate. Individuals with hypochondrogenesis have an enlarged abdomen and may have a condition called hydrops fetalis in which excess fluid builds up in the body before birth.

As a result of these serious health problems, some affected fetuses do not survive to term. Infants born with hypochondrogenesis usually die at birth or shortly thereafter from respiratory failure. Babies who live past the newborn period are usually reclassified as having spondyloepiphyseal dysplasia congenita, a related but milder disorder that similarly affects bone development.

Hypochondrogenesis is a rare, severe disorder of bone growth. This condition is characterized by a small body, short limbs, and abnormal bone formation (ossification) in the spine and pelvis. [from MedlinePlus Genetics]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHypochondrogenesis
Follow this link to review classifications for Hypochondrogenesis in Orphanet.

Professional guidelines

PubMed

Offiah AC, Vockley J, Munns CF, Murotsuki J
Pediatr Radiol 2019 Jan;49(1):3-22. Epub 2018 Oct 3 doi: 10.1007/s00247-018-4239-0. PMID: 30284005Free PMC Article
Schramm T, Gloning KP, Minderer S, Daumer-Haas C, Hörtnagel K, Nerlich A, Tutschek B
Ultrasound Obstet Gynecol 2009 Aug;34(2):160-70. doi: 10.1002/uog.6359. PMID: 19548204
Krakow D, Williams J 3rd, Poehl M, Rimoin DL, Platt LD
Ultrasound Obstet Gynecol 2003 May;21(5):467-72. doi: 10.1002/uog.111. PMID: 12768559

Recent clinical studies

Etiology

Handa A, Grigelioniene G, Nishimura G
Radiographics 2021 Jan-Feb;41(1):192-209. Epub 2020 Nov 13 doi: 10.1148/rg.2021200075. PMID: 33186059
Schramm T, Gloning KP, Minderer S, Daumer-Haas C, Hörtnagel K, Nerlich A, Tutschek B
Ultrasound Obstet Gynecol 2009 Aug;34(2):160-70. doi: 10.1002/uog.6359. PMID: 19548204
Potocki L, Abuelo DN, Oyer CE
Am J Med Genet 1995 Nov 20;59(3):295-9. doi: 10.1002/ajmg.1320590306. PMID: 8599352
Tiller GE, Polumbo PA, Weis MA, Bogaert R, Lachman RS, Cohn DH, Rimoin DL, Eyre DR
Nat Genet 1995 Sep;11(1):87-9. doi: 10.1038/ng0995-87. PMID: 7550321
Maroteaux P, Stanescu V, Stanescu R
Eur J Pediatr 1983 Oct;141(1):14-22. doi: 10.1007/BF00445662. PMID: 6641761

Diagnosis

Ježová M, Pavlovská D, Grochová I, Michenková A, Vlašín P
Cesk Patol 2023 Summer;59(2):68-79. PMID: 37468326
Offiah AC, Vockley J, Munns CF, Murotsuki J
Pediatr Radiol 2019 Jan;49(1):3-22. Epub 2018 Oct 3 doi: 10.1007/s00247-018-4239-0. PMID: 30284005Free PMC Article
Barat-Houari M, Sarrabay G, Gatinois V, Fabre A, Dumont B, Genevieve D, Touitou I
Hum Mutat 2016 Jan;37(1):7-15. Epub 2015 Oct 21 doi: 10.1002/humu.22915. PMID: 26443184
Potocki L, Abuelo DN, Oyer CE
Am J Med Genet 1995 Nov 20;59(3):295-9. doi: 10.1002/ajmg.1320590306. PMID: 8599352
Stanescu V, Stanescu R, Maroteaux P
J Bone Joint Surg Am 1984 Jul;66(6):817-36. doi: 10.2106/00004623-198466060-00002. PMID: 6376516

Prognosis

Mortier GR, Weis M, Nuytinck L, King LM, Wilkin DJ, De Paepe A, Lachman RS, Rimoin DL, Eyre DR, Cohn DH
J Med Genet 2000 Apr;37(4):263-71. doi: 10.1136/jmg.37.4.263. PMID: 10745044Free PMC Article
Potocki L, Abuelo DN, Oyer CE
Am J Med Genet 1995 Nov 20;59(3):295-9. doi: 10.1002/ajmg.1320590306. PMID: 8599352
Stanescu V, Stanescu R, Maroteaux P
J Bone Joint Surg Am 1984 Jul;66(6):817-36. doi: 10.2106/00004623-198466060-00002. PMID: 6376516

Clinical prediction guides

Mortier GR, Weis M, Nuytinck L, King LM, Wilkin DJ, De Paepe A, Lachman RS, Rimoin DL, Eyre DR, Cohn DH
J Med Genet 2000 Apr;37(4):263-71. doi: 10.1136/jmg.37.4.263. PMID: 10745044Free PMC Article
Tiller GE, Polumbo PA, Weis MA, Bogaert R, Lachman RS, Cohn DH, Rimoin DL, Eyre DR
Nat Genet 1995 Sep;11(1):87-9. doi: 10.1038/ng0995-87. PMID: 7550321
Freisinger P, Ala-Kokko L, LeGuellec D, Franc S, Bouvier R, Ritvaniemi P, Prockop DJ, Bonaventure J
J Biol Chem 1994 May 6;269(18):13663-9. PMID: 8175802
Le Guellec D, Mallein-Gerin F, Treilleux I, Bonaventure J, Peysson P, Herbage D
Histochem J 1994 Sep;26(9):695-704. doi: 10.1007/BF00158202. PMID: 7843983
Maroteaux P, Stanescu V, Stanescu R
Eur J Pediatr 1983 Oct;141(1):14-22. doi: 10.1007/BF00445662. PMID: 6641761

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...