U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Links from Gene

Microcephaly 29, primary, autosomal recessive(MCPH29)

MedGen UID:
1823993
Concept ID:
C5774220
Disease or Syndrome
Synonym: MCPH29
 
Gene (location): PDCD6IP (3p22.3)
 
Monarch Initiative: MONDO:0031060
OMIM®: 620047

Definition

Autosomal recessive primary microcephaly-29 (MCPH29) is characterized by small head circumference apparent at birth and associated with global developmental delay, impaired intellectual development, speech delay, and behavioral abnormalities. Affected individuals also have poor overall growth with short stature, mild dysmorphic facial features, and seizures (Khan et al., 2020). For a discussion of genetic heterogeneity of primary microcephaly, see MCPH1 (251200). [from OMIM]

Clinical features

From HPO
Hyperechogenic kidneys
MedGen UID:
477530
Concept ID:
C3275899
Finding
An increase in amplitude of waves returned in ultrasonography of the kidney, which is generally displayed as increased brightness of the signal.
Reduced renal corticomedullary differentiation
MedGen UID:
813461
Concept ID:
C3807131
Finding
Reduced differentiation between renal cortex and medulla on diagnostic imaging.
Cerebellar ataxia
MedGen UID:
849
Concept ID:
C0007758
Disease or Syndrome
Cerebellar ataxia refers to ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- or overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly).
Seizure
MedGen UID:
20693
Concept ID:
C0036572
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
Emotional lability
MedGen UID:
39319
Concept ID:
C0085633
Mental or Behavioral Dysfunction
Unstable emotional experiences and frequent mood changes; emotions that are easily aroused, intense, and/or disproportionate to events and circumstances.
Hyperactivity
MedGen UID:
98406
Concept ID:
C0424295
Finding
Hyperactivity is a condition characterized by constant and unusually high levels of activity, even in situations where it is deemed inappropriate.
Global developmental delay
MedGen UID:
107838
Concept ID:
C0557874
Finding
A delay in the achievement of motor or mental milestones in the domains of development of a child, including motor skills, speech and language, cognitive skills, and social and emotional skills. This term should only be used to describe children younger than five years of age.
Simplified gyral pattern
MedGen UID:
413664
Concept ID:
C2749675
Finding
An abnormality of the cerebral cortex with fewer gyri but with normal cortical thickness. This pattern is usually often associated with congenital microcephaly.
Enlarged cerebellum
MedGen UID:
479654
Concept ID:
C3278024
Finding
An abnormally increased size of the cerebellum compared to other brain structures.
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Intellectual disability, previously referred to as mental retardation, is characterized by subnormal intellectual functioning that occurs during the developmental period. It is defined by an IQ score below 70.
Primary microcephaly
MedGen UID:
383046
Concept ID:
C2677180
Finding
Head circumference below 2 standard deviations below the mean for age and gender at birth.
High forehead
MedGen UID:
65991
Concept ID:
C0239676
Finding
An abnormally increased height of the forehead.
Pointed chin
MedGen UID:
336193
Concept ID:
C1844505
Finding
A marked tapering of the lower face to the chin.
Thick eyebrow
MedGen UID:
377914
Concept ID:
C1853487
Finding
Increased density/number and/or increased diameter of eyebrow hairs.
Hypoplastic philtrum
MedGen UID:
341641
Concept ID:
C1856886
Finding
Underdevelopment of the philtrum.
Strabismus
MedGen UID:
21337
Concept ID:
C0038379
Disease or Syndrome
A misalignment of the eyes so that the visual axes deviate from bifoveal fixation. The classification of strabismus may be based on a number of features including the relative position of the eyes, whether the deviation is latent or manifest, intermittent or constant, concomitant or otherwise and according to the age of onset and the relevance of any associated refractive error.
Deeply set eye
MedGen UID:
473112
Concept ID:
C0423224
Finding
An eye that is more deeply recessed into the plane of the face than is typical.

Recent clinical studies

Etiology

Kelly MN, Tuli SY, Tuli SS, Stern MA, Giordano BP
J Pediatr Health Care 2015 Jan-Feb;29(1):97-103. Epub 2014 Jun 20 doi: 10.1016/j.pedhc.2014.04.006. PMID: 24954735

Diagnosis

Kelly MN, Tuli SY, Tuli SS, Stern MA, Giordano BP
J Pediatr Health Care 2015 Jan-Feb;29(1):97-103. Epub 2014 Jun 20 doi: 10.1016/j.pedhc.2014.04.006. PMID: 24954735
Tan CA, del Gaudio D, Dempsey MA, Arndt K, Botes S, Reeder A, Das S
Clin Genet 2014 Apr;85(4):353-8. Epub 2013 May 13 doi: 10.1111/cge.12172. PMID: 23611254

Therapy

Kelly MN, Tuli SY, Tuli SS, Stern MA, Giordano BP
J Pediatr Health Care 2015 Jan-Feb;29(1):97-103. Epub 2014 Jun 20 doi: 10.1016/j.pedhc.2014.04.006. PMID: 24954735

Prognosis

del Campo M, Hall BD, Aeby A, Nassogne MC, Verloes A, Roche C, Gonzalez C, Sanchez H, Garcia-Alix A, Cabanas F, Escudero RM, Hernandez R, Quero J
Am J Med Genet 1999 Aug 27;85(5):479-85. doi: 10.1002/(sici)1096-8628(19990827)85:5<479::aid-ajmg9>3.3.co;2-4. PMID: 10405446

Clinical prediction guides

Tan CA, del Gaudio D, Dempsey MA, Arndt K, Botes S, Reeder A, Das S
Clin Genet 2014 Apr;85(4):353-8. Epub 2013 May 13 doi: 10.1111/cge.12172. PMID: 23611254

Supplemental Content

Table of contents

    Clinical resources

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...