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Al-Gazali syndrome(ALGAZ)

MedGen UID:
373020
Concept ID:
C1836121
Disease or Syndrome
Synonyms: Al Gazali Al Talabani syndrome; Eye defects arachnodactyly cardiopathy
SNOMED CT: Eye defects, arachnodactyly, cardiopathy syndrome (1208342001); Al Gazali Al Talabani syndrome (1208342001); Al Gazali Lytle syndrome (1208342001)
 
Gene (location): B3GALT6 (1p36.33)
 
Monarch Initiative: MONDO:0012282
OMIM®: 609465
Orphanet: ORPHA2725

Definition

Al-Gazali syndrome (ALGAZ) is characterized by prenatal growth retardation, skeletal anomalies including joint contractures, camptodactyly, and bilateral talipes equinovarus, small mouth, anterior segment eye anomalies, and early lethality (summary by Ben-Mahmoud et al., 2018). [from OMIM]

Clinical features

From HPO
Hydronephrosis
MedGen UID:
42531
Concept ID:
C0020295
Disease or Syndrome
Severe distention of the kidney with dilation of the renal pelvis and calices.
Bilateral talipes equinovarus
MedGen UID:
332956
Concept ID:
C1837835
Congenital Abnormality
Bilateral clubfoot deformity.
Broad distal phalanx of finger
MedGen UID:
342551
Concept ID:
C1850630
Finding
Abnormally wide (broad) distal phalanx of finger.
Bowed humerus
MedGen UID:
395269
Concept ID:
C1859460
Finding
A bending or abnormal curvature of the humerus.
Failure to thrive
MedGen UID:
746019
Concept ID:
C2315100
Disease or Syndrome
Failure to thrive (FTT) refers to a child whose physical growth is substantially below the norm.
Gastrojejunal tube feeding in infancy
MedGen UID:
896852
Concept ID:
C4280681
Finding
Feeding problem necessitating gastrojejunal tube feeding.
Recurrent fractures
MedGen UID:
42094
Concept ID:
C0016655
Injury or Poisoning
The repeated occurrence of bone fractures (implying an abnormally increased tendency for fracture).
Micrognathia
MedGen UID:
44428
Concept ID:
C0025990
Congenital Abnormality
Developmental hypoplasia of the mandible.
Osteopenia
MedGen UID:
18222
Concept ID:
C0029453
Disease or Syndrome
Osteopenia is a term to define bone density that is not normal but also not as low as osteoporosis. By definition from the World Health Organization osteopenia is defined by bone densitometry as a T score -1 to -2.5.
Wrist flexion contracture
MedGen UID:
592338
Concept ID:
C0409345
Acquired Abnormality
A chronic loss of wrist joint motion due to structural changes in muscle, tendons, ligaments, or skin that prevent normal movement of the joints of the wrist.
Proximal radio-ulnar synostosis
MedGen UID:
436690
Concept ID:
C2676443
Finding
An abnormal osseous union (fusion) between the proximal portions of the radius and the ulna.
Recurrent pneumonia
MedGen UID:
195802
Concept ID:
C0694550
Disease or Syndrome
An increased susceptibility to pneumonia as manifested by a history of recurrent episodes of pneumonia.
Midface retrusion
MedGen UID:
339938
Concept ID:
C1853242
Anatomical Abnormality
Posterior positions and/or vertical shortening of the infraorbital and perialar regions, or increased concavity of the face and/or reduced nasolabial angle.
Polyhydramnios
MedGen UID:
6936
Concept ID:
C0020224
Pathologic Function
The presence of excess amniotic fluid in the uterus during pregnancy.
Corneal opacity
MedGen UID:
40485
Concept ID:
C0010038
Finding
A reduction of corneal clarity.
Sclerocornea
MedGen UID:
344000
Concept ID:
C1853235
Disease or Syndrome
A congenital anomaly in which a part or the whole of the cornea acquires the characteristics of sclera, resulting in clouding of the cornea.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAl-Gazali syndrome

Recent clinical studies

Etiology

Leoni C, Tedesco M, Radio FC, Chillemi G, Leone A, Bruselles A, Ciolfi A, Stellacci E, Pantaleoni F, Butera G, Rigante D, Onesimo R, Tartaglia M, Zampino G
Am J Med Genet A 2021 Oct;185(10):3153-3160. Epub 2021 Jun 23 doi: 10.1002/ajmg.a.62399. PMID: 34159694
Salik I, Gupta A, Tara A, Zaidman G, Barst S
Paediatr Anaesth 2020 May;30(5):577-583. Epub 2020 Mar 16 doi: 10.1111/pan.13843. PMID: 32107814

Diagnosis

Leoni C, Tedesco M, Radio FC, Chillemi G, Leone A, Bruselles A, Ciolfi A, Stellacci E, Pantaleoni F, Butera G, Rigante D, Onesimo R, Tartaglia M, Zampino G
Am J Med Genet A 2021 Oct;185(10):3153-3160. Epub 2021 Jun 23 doi: 10.1002/ajmg.a.62399. PMID: 34159694
Sellars EA, Bosanko KA, Lepard T, Garnica A, Schaefer GB
Semin Pediatr Neurol 2014 Jun;21(2):84-7. Epub 2014 Apr 13 doi: 10.1016/j.spen.2014.04.007. PMID: 25149931
Karaer K, Rosti RO, Torun D, Sanal HT, Guran S
Genet Couns 2012;23(4):457-63. PMID: 23431744

Prognosis

Sellars EA, Bosanko KA, Lepard T, Garnica A, Schaefer GB
Semin Pediatr Neurol 2014 Jun;21(2):84-7. Epub 2014 Apr 13 doi: 10.1016/j.spen.2014.04.007. PMID: 25149931

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