U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Radioulnar synostosis with amegakaryocytic thrombocytopenia 2(RUSAT2)

MedGen UID:
901732
Concept ID:
C4225221
Disease or Syndrome
Synonyms: RADIOULNAR SYNOSTOSIS AND AMEGAKARYOCYTIC THROMBOCYTOPENIA 2; RUSAT2
 
Gene (location): MECOM (3q26.2)
 
Monarch Initiative: MONDO:0014758
OMIM®: 616738

Definition

Radioulnar synostosis with amegakaryocytic thrombocytopenia (RUSAT) is characterized by thrombocytopenia that progresses to pancytopenia, in association with congenital proximal fusion of the radius and ulna that results in extremely limited pronation and supination of the forearm (summary by Niihori et al., 2015). For a discussion of genetic heterogeneity of radioulnar synostosis with amegakaryocytic thrombocytopenia, see RUSAT1 (605432). [from OMIM]

Clinical features

From HPO
Hydrocele testis
MedGen UID:
318568
Concept ID:
C1720771
Congenital Abnormality
Accumulation of clear fluid in the between the layers of membrane (tunica vaginalis) surrounding the testis.
Overlapping fingers
MedGen UID:
252954
Concept ID:
C1446712
Finding
A finger resting on the dorsal surface of an adjacent digit when the hand is at rest.
Limited pronation/supination of forearm
MedGen UID:
348328
Concept ID:
C1861331
Finding
A limitation of the ability to place the forearm in a position such that the palm faces anteriorly (supination) and to place the forearm in a position such that the palm faces posteriorly (pronation).
Short middle phalanx of the 4th finger
MedGen UID:
867139
Concept ID:
C4021497
Anatomical Abnormality
Hypoplastic/small middle phalanx of the 4th finger, also known as the ring finger.
Hepatosplenomegaly
MedGen UID:
9225
Concept ID:
C0019214
Sign or Symptom
Simultaneous enlargement of the liver and spleen.
Sensorineural hearing loss disorder
MedGen UID:
9164
Concept ID:
C0018784
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
Intellectual disability, mild
MedGen UID:
10044
Concept ID:
C0026106
Mental or Behavioral Dysfunction
Mild intellectual disability is defined as an intelligence quotient (IQ) in the range of 50-69.
Anemia
MedGen UID:
1526
Concept ID:
C0002871
Disease or Syndrome
A reduction in erythrocytes volume or hemoglobin concentration.
Thrombocytopenia
MedGen UID:
52737
Concept ID:
C0040034
Disease or Syndrome
A reduction in the number of circulating thrombocytes.
Congenital thrombocytopenia
MedGen UID:
124422
Concept ID:
C0272278
Disease or Syndrome
Thrombocytopenia with congenital onset.
Radioulnar synostosis
MedGen UID:
57861
Concept ID:
C0158761
Congenital Abnormality
An abnormal osseous union (fusion) between the radius and the ulna.
Neutropenia
MedGen UID:
163121
Concept ID:
C0853697
Finding
An abnormally low number of neutrophils in the peripheral blood.
Cleft palate
MedGen UID:
756015
Concept ID:
C2981150
Congenital Abnormality
Cleft palate is a developmental defect of the palate resulting from a failure of fusion of the palatine processes and manifesting as a separation of the roof of the mouth (soft and hard palate).
Hydrops fetalis
MedGen UID:
6947
Concept ID:
C0020305
Disease or Syndrome
The abnormal accumulation of fluid in two or more fetal compartments, including ascites, pleural effusion, pericardial effusion, and skin edema.

Professional guidelines

PubMed

Geddis AE
Int J Lab Hematol 2013 Feb;35(1):14-25. Epub 2012 Jul 30 doi: 10.1111/j.1751-553X.2012.01454.x. PMID: 22846067

Recent clinical studies

Diagnosis

Huang D, Jiang M, Zhu Y, Li D, Lu X, Gao J
BMC Pediatr 2024 Jan 20;24(1):62. doi: 10.1186/s12887-024-04552-1. PMID: 38245683Free PMC Article
Wall E, Forsyth J, Kinning E, Marton T
Prenat Diagn 2023 Jun;43(6):717-720. Epub 2023 May 17 doi: 10.1002/pd.6353. PMID: 37160698
Geddis AE
Int J Lab Hematol 2013 Feb;35(1):14-25. Epub 2012 Jul 30 doi: 10.1111/j.1751-553X.2012.01454.x. PMID: 22846067

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...