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Neurodevelopmental disorder with hypotonia, brain anomalies, distinctive facies, and absent language(NEDHAFA)

MedGen UID:
1854654
Concept ID:
C5935628
Disease or Syndrome
Synonym: ReNU SYNDROME
 
Monarch Initiative: MONDO:0971172
OMIM®: 620851

Definition

ReNU syndrome (RENU), also known as neurodevelopmental disorder with hypotonia, brain anomalies, distinctive facies, and absent language (NEDHAFA), is characterized by hypotonia, global developmental delay, severely impaired intellectual development with poor or absent speech, delayed walking or inability to walk, feeding difficulties with poor overall growth, seizures (in most), dysmorphic facial features, and brain anomalies, including ventriculomegaly, thin corpus callosum, and progressive white matter loss (Greene et al., 2024; Schot et al., 2024; Chen et al., 2024). [from OMIM]

Recent clinical studies

Diagnosis

Brockschmidt A, Todt U, Ryu S, Hoischen A, Landwehr C, Birnbaum S, Frenck W, Radlwimmer B, Lichter P, Engels H, Driever W, Kubisch C, Weber RG
Hum Mol Genet 2007 Jun 15;16(12):1488-94. Epub 2007 May 3 doi: 10.1093/hmg/ddm099. PMID: 17478476

Clinical prediction guides

Battaglia A, Novelli A, Bernardini L, Igliozzi R, Parrini B
Am J Med Genet A 2009 Jun;149A(6):1200-4. doi: 10.1002/ajmg.a.32847. PMID: 19449418

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