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Hao-Fountain syndrome(HAFOUS)

MedGen UID:
1719035
Concept ID:
C5393908
Disease or Syndrome
Synonym: HAFOUS
 
Related gene: USP7
 
Monarch Initiative: MONDO:0014805
OMIM®: 602519; 616863
Orphanet: ORPHA500055

Definition

Hao-Fountain syndrome (HAFOUS) is a neurodevelopmental disorder characterized by global developmental delay, variably impaired intellectual development with significant speech delay, behavioral abnormalities, such as autism, and mild dysmorphic facies. Additional features are variable, but may include hypotonia, feeding problems, delayed walking with unsteady gait, hypogonadism in males, and ocular anomalies, such as strabismus. Some patients develop seizures and some have mild white matter abnormalities on brain imaging (summary by Fountain et al., 2019). [from OMIM]

Recent clinical studies

Diagnosis

van der Laan L, Karimi K, Rooney K, Lauffer P, McConkey H, Caro P, Relator R, Levy MA, Bhai P, Mignot C, Keren B, Briuglia S, Sobering AK, Li D, Vissers LELM, Dingemans AJM, Valenzuela I, Verberne EA, Misra-Isrie M, Zwijnenburg PJG, Waisfisz Q, Alders M, Sailer S, Schaaf CP, Mannens MMAM, Sadikovic B, van Haelst MM, Henneman P
Genet Med 2024 Mar;26(3):101050. Epub 2023 Dec 18 doi: 10.1016/j.gim.2023.101050. PMID: 38126281
Priolo M, Mancini C, Pizzi S, Chiriatti L, Radio FC, Cordeddu V, Pintomalli L, Mammì C, Dallapiccola B, Tartaglia M
Genes (Basel) 2022 May 16;13(5) doi: 10.3390/genes13050889. PMID: 35627274Free PMC Article

Clinical prediction guides

Wimmer MC, Brennenstuhl H, Hirsch S, Dötsch L, Unser S, Caro P, Schaaf CP
Clin Genet 2024 May;105(5):499-509. Epub 2024 Jan 14 doi: 10.1111/cge.14480. PMID: 38221796

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