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Clinician orientation sheet

NM_018489.3:c.4039_4043del

Concise orientation only. Dates, normal findings, uncertainty, source boundaries, and longitudinal context remain in the complete profile.

Molecular result and profile context

Report transcript + cDNA
NM_018489.3:c.4039_4043del
Report protein consequence
p.Lys1347Glufs*7
Zygosity
Heterozygous
Molecular consequence
Frameshift with premature termination
Laboratory classification
Pathogenic
Classification source
Original report reviewed
Inheritance
Not established
Relevant additional finding
Not reported in this public profile
Normalized MANE/map notation
NM_018489.3:c.4039_4043del · NP_060959.2:p.Lys1347Glufs*7
Variant type
Frameshift
Sex
Female
Age group
Ages 6–10
Protein position
First altered residue · aa 1,347

Approved public summary

A girl in the 6–10 age group whose family describes major gains in communication, social connection, play, attention, and school participation alongside sustained therapy and support. Her history includes a neuropsychological profile described as compatible with intellectual disability and ADHD, predominantly inattentive presentation; intense distress when plans change or limits are set; polysomnography-confirmed sleep apnea dominated by obstructive events; ongoing ENT reassessment of her adenoid and tonsils; a left periventricular nodular heterotopia documented on brain MRI; diffuse epileptiform activity on EEG without any convulsive seizures witnessed by her family; feeding difficulty; and a fine finger tremor that has become barely noticeable with occupational therapy.