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X-linked myotubular myopathy-abnormal genitalia syndrome

MedGen UID:
335354
Concept ID:
C1846169
Disease or Syndrome
Synonym: Myotubular myopathy with abnormal genital development
SNOMED CT: Xq28 contiguous gene deletion syndrome (1255278004); X-linked myotubular myopathy, abnormal genitalia syndrome (1255278004)
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:0010271
OMIM®: 300219
Orphanet: ORPHA456328

Definition

A rare chromosomal anomaly, partial deletion of the long arm of chromosome X, with characteristics of a combination of clinical manifestations of X-linked myotubular myopathy and a 46,XY disorder of sex development. Patients present with a severe form of congenital myopathy and abnormal male genitalia. [from SNOMEDCT_US]

Clinical features

From HPO
Bifid scrotum
MedGen UID:
90968
Concept ID:
C0341787
Congenital Abnormality
Midline indentation or cleft of the scrotum.
Bilateral cryptorchidism
MedGen UID:
96568
Concept ID:
C0431663
Congenital Abnormality
Absence of both testes from the scrotum owing to failure of the testis or testes to descend through the inguinal canal to the scrotum.
Unilateral cryptorchidism
MedGen UID:
98467
Concept ID:
C0431664
Congenital Abnormality
Absence of a testis from the scrotum on one side owing to failure of the testis or testes to descend through the inguinal canal to the scrotum.
Glandular hypospadias
MedGen UID:
452551
Concept ID:
C0452168
Congenital Abnormality
Hypospadias
MedGen UID:
163083
Concept ID:
C0848558
Congenital Abnormality
Abnormal position of urethral meatus on the ventral penile shaft (underside) characterized by displacement of the urethral meatus from the tip of the glans penis to the ventral surface of the penis, scrotum, or perineum.
Penile hypospadias
MedGen UID:
305577
Concept ID:
C1691215
Congenital Abnormality
Location of the urethral opening on the inferior aspect of the penis.
Ambiguous genitalia, male
MedGen UID:
867446
Concept ID:
C4021823
Finding
Ambiguous genitalia in an individual with XY genetic gender.
Micropenis
MedGen UID:
1633603
Concept ID:
C4551492
Congenital Abnormality
Abnormally small penis. At birth, the normal penis is about 3 cm (stretched length from pubic tubercle to tip of penis) with micropenis less than 2.0-2.5 cm.
Feeding difficulties
MedGen UID:
65429
Concept ID:
C0232466
Finding
Impaired ability to eat related to problems gathering food and getting ready to suck, chew, or swallow it.
Hyporeflexia
MedGen UID:
195967
Concept ID:
C0700078
Finding
Reduction of neurologic reflexes such as the knee-jerk reaction.
Hypotonia
MedGen UID:
10133
Concept ID:
C0026827
Finding
Hypotonia is an abnormally low muscle tone (the amount of tension or resistance to movement in a muscle). Even when relaxed, muscles have a continuous and passive partial contraction which provides some resistance to passive stretching. Hypotonia thus manifests as diminished resistance to passive stretching. Hypotonia is not the same as muscle weakness, although the two conditions can co-exist.
Myopathy
MedGen UID:
10135
Concept ID:
C0026848
Disease or Syndrome
A disorder of muscle unrelated to impairment of innervation or neuromuscular junction.
Retrognathia
MedGen UID:
19766
Concept ID:
C0035353
Congenital Abnormality
An abnormality in which the mandible is mislocalised posteriorly.
Thin ribs
MedGen UID:
98095
Concept ID:
C0426818
Finding
Ribs with a reduced diameter.
Centrally nucleated skeletal muscle fibers
MedGen UID:
330782
Concept ID:
C1842170
Finding
An abnormality in which the nuclei of sarcomeres take on an abnormally central localization (or in which this feature is found in an increased proportion of muscle cells).
Joint hypermobility
MedGen UID:
336793
Concept ID:
C1844820
Finding
The capability that a joint (or a group of joints) has to move, passively and/or actively, beyond normal limits along physiological axes.
Generalized hypotonia
MedGen UID:
346841
Concept ID:
C1858120
Finding
Generalized muscular hypotonia (abnormally low muscle tone).
Atelectasis
MedGen UID:
13946
Concept ID:
C0004144
Pathologic Function
Collapse of part of a lung associated with absence of inflation (air) of that part.
Respiratory distress
MedGen UID:
96907
Concept ID:
C0476273
Sign or Symptom
Respiratory distress is objectively observable as the physical or emotional consequences from the experience of dyspnea. The physical presentation of respiratory distress is generally referred to as labored breathing, while the sensation of respiratory distress is called shortness of breath or dyspnea.
High palate
MedGen UID:
66814
Concept ID:
C0240635
Congenital Abnormality
Height of the palate more than 2 SD above the mean (objective) or palatal height at the level of the first permanent molar more than twice the height of the teeth (subjective).
Polyhydramnios
MedGen UID:
6936
Concept ID:
C0020224
Pathologic Function
The presence of excess amniotic fluid in the uterus during pregnancy.
Decreased fetal movement
MedGen UID:
68618
Concept ID:
C0235659
Finding
An abnormal reduction in quantity or strength of fetal movements.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVX-linked myotubular myopathy-abnormal genitalia syndrome

Recent clinical studies

Etiology

Verlinsky Y, Rechitsky S, Verlinsky O, Kenigsberg D, Moshella J, Ivakhnenko V, Masciangelo C, Strom C, Kuliev A
Reprod Biomed Online 2002 Jan-Feb;4(1):38-42. doi: 10.1016/s1472-6483(10)61913-x. PMID: 12470351

Diagnosis

Verlinsky Y, Rechitsky S, Verlinsky O, Kenigsberg D, Moshella J, Ivakhnenko V, Masciangelo C, Strom C, Kuliev A
Reprod Biomed Online 2002 Jan-Feb;4(1):38-42. doi: 10.1016/s1472-6483(10)61913-x. PMID: 12470351

Prognosis

Verlinsky Y, Rechitsky S, Verlinsky O, Kenigsberg D, Moshella J, Ivakhnenko V, Masciangelo C, Strom C, Kuliev A
Reprod Biomed Online 2002 Jan-Feb;4(1):38-42. doi: 10.1016/s1472-6483(10)61913-x. PMID: 12470351

Clinical prediction guides

Verlinsky Y, Rechitsky S, Verlinsky O, Kenigsberg D, Moshella J, Ivakhnenko V, Masciangelo C, Strom C, Kuliev A
Reprod Biomed Online 2002 Jan-Feb;4(1):38-42. doi: 10.1016/s1472-6483(10)61913-x. PMID: 12470351

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