Table 7. Frequencies of various manifestations of NF1 in clinician-reported studies compared with NF registry.
Data Source | YEAR | ADULTS/ CHILDREN | Self/ Clinician | Single/ Multicenter | SUBJECTS | PN % | OPG | MPNST | FRECK | LISCH | TIBIAL | LD/ ADHD |
---|---|---|---|---|---|---|---|---|---|---|---|---|
CTF Registry | 2015 | A & C | S | M Intl | 4020 | 35 | 19 | 2 | 45 | 7 | 51 | |
Friedman [4] | 1997 | A & C | C | M Intl | 1728 | 23 | NA | 5 | NA | 59 | 2 | |
Huson [20] | 1989 | A & C | C | S Wales | 135 | 32 | 1.5 | 70 | 85 | 4 | 33 | |
McKeever [21] | 2008 | C | C | M N Ireland | 75 | 6.7 | 42 | 6 | 5.3 | 49 | ||
Cnossen [22] | 1998 | C | C | S Rotterdam | 150 | 26.6 | 11.3 | 85.3 | 52 | 2 | ||
Nemethova [23] | 2013 | Collection of NF | C | S Slovakia | 108 | 31 | 85 | 36 | ||||
Overweg-Plandsoen [24] | 1997 | C & A | C | S Netherlands | 196 | 10 | 92 | 93 | ||||
Boulanger [25] | 2005 | C | C | S Montreal | 279 | 24.7 | 14.7 | 1.8 | 59.6 |
PN = Plexiform neurofibroma; OPG = Optic pathway glioma; MPNST = Malignant peripheral nerve sheath tumor; FRECK = skin-fold freckling; LISCH = Lisch nodules; LD/ADHD = Learning disability/Attention Deficit Hyperactivity Disorder.