-
| Abnormality of circulating hormone level
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| Episodic peripheral neuropathy
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| Absent or minimally ossified vertebral bodies
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| Supernumerary vertebral ossification centers
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| Medially fused radial epiphyseal plates
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| Thoracic platyspondyly
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| Disc-like vertebral bodies
-
| Hypoplastic sacrum
-
| Lobular glomerulopathy
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| Low-frequency sensorineural hearing impairment
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| hump-shaped mound of bone in central and posterior portions of vertebral endplate
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| Abnormality of ion homeostasis
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| Prominent scrotal raphe
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| Overgrowth of external genitalia
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| Penile hypospadias
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| Underfolded helix
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| Increased phosphoribosylpyrophosphate (PRPP) synthetase
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| External genital hypoplasia
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| Gonadal tissue inappropriate for external genitalia or chromosomal sex
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| Genital ulcers
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| Severe
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| Profound
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| Neonatal short-limb short stature
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| Biilateral vocal cord paralysis
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| Unilateral vocal cord paresis
-
| Bilateral vocal cord paresis
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| Clinical modifier
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| Severity
-
| Mild
-
| Moderate
-
| Borderline
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| Global glomerulosclerosis
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| Crossed fused renal ectopia
-
| Lytic defects of ulnar metaphysis
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| Irregular ulnar metaphysis
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| Sloping ulnar metaphysis
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| Impaired renal uric acid clearance
-
| Wide ulnar metaphysis
-
| Spurred ulnar metaphysis
-
| Decreased carpal angles of wrist
-
| Narrow joint spaces of wrist
-
| Wide epiphyses of the upper limbs
-
| Nonprogressive muscular atrophy
-
| mesomelic/rhizomelic limb shortening
-
| Absent rod-and cone-mediated responses on ERG
-
| Flattened moderately deformed vertebrae
-
| Bilateral elbow dislocations
-
| Contractures of the joints of the lower limbs
-
| Redundant skin
-
| Symphalangism of the proximal phalanx of the 4th toe with the 4th metatarsal
-
| Triangular epiphysis of the proximal phalanx of the 4th finger
-
| Stippling of the epiphysis of the proximal phalanx of the 4th finger
-
| Deviation of the 4th finger
-
| Aplasia/Hypoplasia of the 4th finger
-
| Contracture of the distal interphalangeal joint of the 4th finger
-
| Joint contracture of the 4th finger
-
| Contracture of the metacarpophalangeal joint of the 4th finger
-
| Contracture of the proximal interphalangeal joint of the 4th finger
-
| Radial deviation of the 4th finger
-
| Ulnar deviation of the 4th finger
-
| Abnormality of the malleus
-
| Partial duplication of the phalanx of hand
-
| Mask-like facies
-
| Chin dimple
-
| Inappropriate laughter
-
| Paroxysmal bursts of laughter
-
| Abnormality of the temporomandibular joint
-
| Asymmetry of the maxilla
-
| Aplasia/Hypoplasia of the premaxilla
-
| Aplasia of the premaxilla
-
| Abnormality of the forehead
-
| Abnormality of facial adipose tissue
-
| Loss of facial adipose tissue
-
| Full cheeks
-
| Low anterior hairline
-
| Doll-like facies
-
| Low frustration tolerance
-
| Facial hypotonia
-
| Abdominal symptom
-
| Esophageal carcinoma
-
| Carotid artery stenosis
-
| Duplication of phalanx of hand
-
| Leg muscle stiffness
-
| Prolonged brainstem auditory evoked potentials
-
| Lethal skeletal dysplasia
-
| Flattened knee epiphyses
-
| Arterial stenosis
-
| Second metatarsal posteriorly placed
-
| Absent brainstem auditory responses
-
| Hydronephrosis
-
| Renal salt wasting
-
| Renal tubular dysfunction
-
| Pelvic kidney
-
| Unilateral renal agenesis
-
| Nephritis
-
| Nephrocalcinosis
-
| Renal potassium wasting
-
| Clinodactyly of hallux
-
| Finger clinodactyly
-
| Chromsome breakage
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