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Features of the Wolf-Hirschhorn Syndrome (WHS) from Infant to Young Teenager Cover

Features of the Wolf-Hirschhorn Syndrome (WHS) from Infant to Young Teenager

Open Access
|Jul 2023

Figures & Tables

Figure 1.

Distinctive clinical facial features of the proband: age 11 months (a, b), age 4 years (c, d) age 16 years (e, f).
Distinctive clinical facial features of the proband: age 11 months (a, b), age 4 years (c, d) age 16 years (e, f).

Figure 2.

Clinodactyly of the hands (a) and anomaly of the feet (b)
Clinodactyly of the hands (a) and anomaly of the feet (b)

Figure 3.

High-arched palate (a) and abnormal dermatoglyphics (b)
High-arched palate (a) and abnormal dermatoglyphics (b)

Figure 4.

X-ray examination of hands at 11 months (a) and 16 years (b)
X-ray examination of hands at 11 months (a) and 16 years (b)

Figure 5.

Transposition between the left lateral incisor and canine (a) open bite (b) enamel hypoplasia and dental caries (c) at 16 years
Transposition between the left lateral incisor and canine (a) open bite (b) enamel hypoplasia and dental caries (c) at 16 years

Figure 6.

Orthopantomography at 16 years
Orthopantomography at 16 years

Figure 7.

Profile Teleradiography (a); Ricketts lateral analysis (b) at 16 years
Profile Teleradiography (a); Ricketts lateral analysis (b) at 16 years

Figure 8.

FISH analysis. FISH finding in chromosome 4 with green signal corresponding to centromere region and red signal corresponding to 4p.16.3 region. The green arrow showed the deletion of chromosome 4 and the red arrow indicated normal chromosome: a) the FISH sample shows chromosomes 4 without deletion; b) FISH test shows 4p monoallelic deletion
FISH analysis. FISH finding in chromosome 4 with green signal corresponding to centromere region and red signal corresponding to 4p.16.3 region. The green arrow showed the deletion of chromosome 4 and the red arrow indicated normal chromosome: a) the FISH sample shows chromosomes 4 without deletion; b) FISH test shows 4p monoallelic deletion

Cephalometric analysis based on the profile teleradiography

MEASUREMENTNORMAL VALUEVALUEDIFFERENCEBIAS
Cranial Base Evaluation
Cranial deflection O27.026.1−0.9
Ant. Cranial Length mm64.058.2−5.8
Post. Cranial Length mm43.532.7−10.8
Face Typology
Total Facial Height O60.046.5−13.5•••
Facial Taper O68.072.34.3
Mandible Growth Direction
Facial axis O90.094.84.8
Lower Facial Height O46.041.7−4.3
Mandible Shape
Mandibular arc O27.248.221.0 •••
FMA O21.520.2−1.3
Corpus Axis mm77.459.9−17.5•••
Posterior Facial Height mm64.060.8−3.2
Skeletal Relations
Convexity mm0.00.60.6
A-N-Ba O63.062.1−0.9
Maxillary Depth O90.088.3−1.7
Facial Depth O91.787.6−4.1
Dental Condition
Upper 6 to PTV mm21.019.6−1.4
Lower 6 to PTV mm24.014.5−9.5•••
Interincisal angle O126.0148.622.6 ••
Upper 1 to Lips Embrasure mmN/A8.3N/A ••
Lower 1 Extrusion mm1.2−1.6−2.8 ••
Lower 1 to A-Po mm1.0−2.7−3.7
Lower 1 to A-Po angle O22.020.1−1.9
Esthetic
Li’/E-line mm−3.83.87.6

Retrognathia in Cephalometry Heb_Uni_ Analysis

DescriptorMeasTypeMeanSdPatientGraphComment
Maxilla To Cranium
SNA Deg82.02.083.65− ( * )+
NA TO F.H. Deg90.06.093.4− ( * )+
Mandible To Cranium
FACIAL ANGLEFH-N-PogDeg87.83.693.56− ( * )+Mandible forward to Base

j_bjmg-2023-0006_tab_003

At 11 months:G=5700g, T=65cm PC=42cm
SD: −3.54 for waist
SD: −4.4 for weight
SD: −3.25 for the cranial perimeter (PC)
At 4 years:G=12.3kg, T=91cm PC=47cm
SD: −2.68 for waist
SD: −2.46 for weight
SD: −2.27 for PC
At 16 years:G=47kg, T=148cm PC=51
SD: −2.94 for waist
SD: −1.54 for weight
SD: −2.75 for PC
Language: English
Page range: 75 - 82
Published on: Jul 31, 2023
In partnership with: Paradigm Publishing Services
Publication frequency: 2 issues per year

© 2023 D.E. Popescu, D. Marian, M. Zeleniuc, Ch. Samoila, V. Belengeanu, published by Macedonian Academy of Sciences and Arts
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.