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Tel Hashomer camptodactyly syndrome

MedGen UID:
347860
Concept ID:
C1859356
Disease or Syndrome
Synonym: Camptodactyly with muscular hypoplasia, skeletal dysplasia, and abnormal palmar creases
SNOMED CT: Tel Hashomer camptodactyly syndrome (719946008)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
Monarch Initiative: MONDO:0008901
OMIM®: 211960
Orphanet: ORPHA3292

Definition

A rare syndrome with characteristics of camptodactyly, muscle hypoplasia and weakness, skeletal anomalies, facial dysmorphism and abnormal dermatoglyphics. Dysmorphic features include facial asymmetry, hypertelorism, broad nasal bridge, long philtrum and a small mouth. Winging scapulae, scoliosis, syndactyly and clinodactyly are commonly observed. The affected patients usually have normal mental development. The molecular basis of the syndrome has not yet been elucidated. [from SNOMEDCT_US]

Clinical features

From HPO
Clubfoot
MedGen UID:
3130
Concept ID:
C0009081
Congenital Abnormality
Clubfoot is a congenital limb deformity defined as fixation of the foot in cavus, adductus, varus, and equinus (i.e., inclined inwards, axially rotated outwards, and pointing downwards) with concomitant soft tissue abnormalities (Cardy et al., 2007). Clubfoot may occur in isolation or as part of a syndrome (e.g., diastrophic dysplasia, 222600). Clubfoot has been reported with deficiency of long bones and mirror-image polydactyly (Gurnett et al., 2008; Klopocki et al., 2012).
Small thenar eminence
MedGen UID:
335432
Concept ID:
C1846474
Finding
Underdevelopment of the thenar eminence with reduced palmar soft tissue mass surrounding the base of the thumb.
Small hypothenar eminence
MedGen UID:
396124
Concept ID:
C1861395
Finding
Reduced muscle mass on the ulnar side of the palm, that is, reduction in size of the hypothenar eminence.
Dermatoglyphic ridges abnormal
MedGen UID:
870698
Concept ID:
C4025152
Anatomical Abnormality
Mitral valve prolapse
MedGen UID:
7671
Concept ID:
C0026267
Disease or Syndrome
One or both of the leaflets (cusps) of the mitral valve bulges back into the left atrium upon contraction of the left ventricle.
Spina bifida
MedGen UID:
38283
Concept ID:
C0080178
Congenital Abnormality
Incomplete closure of the embryonic neural tube, whereby some vertebral arches remain unfused and open. The mildest form is spina bifida occulta, followed by meningocele and meningomyelocele.
Inguinal hernia
MedGen UID:
6817
Concept ID:
C0019294
Finding
Protrusion of the contents of the abdominal cavity through the inguinal canal.
Joint contracture of the hand
MedGen UID:
56382
Concept ID:
C0158113
Finding
Contractures of one ore more joints of the hands meaning chronic loss of joint motion due to structural changes in non-bony tissue.
Skeletal dysplasia
MedGen UID:
98053
Concept ID:
C0410528
Disease or Syndrome
A general term describing features characterized by abnormal development of bones and connective tissues.
EMG abnormality
MedGen UID:
99199
Concept ID:
C0476403
Finding
Abnormal results of investigations using electromyography (EMG).
Camptodactyly
MedGen UID:
195780
Concept ID:
C0685409
Congenital Abnormality
The distal interphalangeal joint and/or the proximal interphalangeal joint of the fingers or toes cannot be extended to 180 degrees by either active or passive extension.
Elevated circulating creatine kinase concentration
MedGen UID:
69128
Concept ID:
C0241005
Finding
An elevation of the level of the enzyme creatine kinase (also known as creatine phosphokinase (CK; EC 2.7.3.2) in the blood. CK levels can be elevated in a number of clinical disorders such as myocardial infarction, rhabdomyolysis, and muscular dystrophy.
Long philtrum
MedGen UID:
351278
Concept ID:
C1865014
Finding
Distance between nasal base and midline upper lip vermilion border more than 2 SD above the mean. Alternatively, an apparently increased distance between nasal base and midline upper lip vermilion border.
Abnormal dermatoglyphics
MedGen UID:
609464
Concept ID:
C0432333
Congenital Abnormality
An abnormality of dermatoglyphs (fingerprints), which are present on fingers, palms, toes, and soles.
Hypertelorism
MedGen UID:
9373
Concept ID:
C0020534
Finding
Although hypertelorism means an excessive distance between any paired organs (e.g., the nipples), the use of the word has come to be confined to ocular hypertelorism. Hypertelorism occurs as an isolated feature and is also a feature of many syndromes, e.g., Opitz G syndrome (see 300000), Greig cephalopolysyndactyly (175700), and Noonan syndrome (163950) (summary by Cohen et al., 1995).

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTel Hashomer camptodactyly syndrome
Follow this link to review classifications for Tel Hashomer camptodactyly syndrome in Orphanet.

Recent clinical studies

Etiology

Melegh B, Hollódy K, Aszmann M, Méhes K
Am J Med Genet A 2005 Jun 15;135(3):320-3. doi: 10.1002/ajmg.a.30736. PMID: 15887272

Diagnosis

Kessel I, German A, Peleg A; Regeneron Genetics Center, Gonzaga-Jauregui C, Paperna T, Ekhilevitch N, Kurolap A, Baris Feldman H, Sagi-Dain L
Am J Med Genet A 2021 Oct;185(10):3161-3166. Epub 2021 Jun 19 doi: 10.1002/ajmg.a.62401. PMID: 34145742
Wijerathne BT, Meier RJ, Agampodi SB
J Med Case Rep 2016 Sep 20;10(1):258. doi: 10.1186/s13256-016-1048-7. PMID: 27650795Free PMC Article
Shah K, Sreekanth R, Thomas B, Danda S
West Indian Med J 2013 Jan;62(1):81-3. PMID: 24171333
Smolkin T, Blazer S, Gershoni-Baruch R, Makhoul IR
Clin Dysmorphol 2011 Oct;20(4):214-216. doi: 10.1097/MCD.0b013e32834a044f. PMID: 21814136
Melegh B, Hollódy K, Aszmann M, Méhes K
Am J Med Genet A 2005 Jun 15;135(3):320-3. doi: 10.1002/ajmg.a.30736. PMID: 15887272

Prognosis

Melegh B, Hollódy K, Aszmann M, Méhes K
Am J Med Genet A 2005 Jun 15;135(3):320-3. doi: 10.1002/ajmg.a.30736. PMID: 15887272

Clinical prediction guides

Wijerathne BT, Meier RJ, Agampodi SB
J Med Case Rep 2016 Sep 20;10(1):258. doi: 10.1186/s13256-016-1048-7. PMID: 27650795Free PMC Article
Gollop T, Dal Colletto GM, Ferraretto I, Grimaldi A
Prog Clin Biol Res 1982;104:269-77. PMID: 7163271

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