POC1A

Clinical Characteristics

The clinical features of SOFT syndrome include:

Growth problems

  • Growth retardation
  • Short stature
  • Low birth weight 
  • Abnormally large head in childhood 
  • Abnormally small head in adulthood 
  • Abnormally long head (rare)

Facial differences (variable)

  • Long face 
  • Triangular face 
  • Prominent forehead 
  • Large jaw 
  • Pointed chin 
  • Small ears 
  • Abnormally increased distance between the eyes
  • Deep-set eyes
  • Prominent nose 
  • Variable dental anomalies

Genitourinary problems (some patients)
Female

  • Underdevelopment or absence of clitoris

Male

  • Low sperm concentration in semen

Skeletal problems

  • Short neck of the femur (thigh bone)
  • Bone age delay 
  • Delayed bone formation in the vertebral bodies
  • Underdevelopment of the pelvis and sacrum 
  • Short long bones e.g. femur, humerus (arm bone between the shoulder and elbow)
  • Thick long bones 
  • Small hands 
  • Shortening of the fingers 
  • Underdevelopment of the middle bone in the fifth finger, causing a wedge-shaped finger 
  • Delay in bone formation
  • Short toes 

Hair and nail changes

  • Underdevelopment of the nails 
  • Sparse hair (e.g. axillary hair, pubic hair, facial hair) after puberty 
  • Baldness (some patients)

Neurologic problems (rare)

  • Psychomotor delay
  • Low muscle tone

Other problems (variable; rare or found in some patients)

  • Unusual, high-pitched voice 
  • Waddling gait 
  • Type 2 diabetes mellitus (rare) 
  • Intellectual disability (rare)
  • Deafness (rare)
  • Severe insulin resistance and dyslipidemia (rare)
  • Severe narrowing of foramen magnum (the hole in the base of the skull through which the spinal cord passes) (rare)
  • Narrowing of the airway (rare)
  • Underdeveloped breasts (rare) 
  • Gynecomastia (enlargement of breast tissue in males)