Abnormalities of Growth (GH) Lecture NO: 2nd MBBS Dr Muhammad Ramzan

GH abnormalities – the definition It refers to the conditions when level of GH is found to be higher or lower than normal body requirements GH is important for children to grow or adults to have a comfortable life

Abnormalities of GH – Causes/ lesions GH production and receptor binding GH deficiency and excess has a very important role of this hormone in normal physiology Such disorders can result from lesions either in the : Hypothalamus or the pituitary affecting production or 1 Target cells for Receptor binding 2

GH abnormalities – Types excess and deficiency GH may be secreted in excess or there can be deficiency of GH with different clinical manifestations GH abnormalities can occur both in children and adults Excess of GH: Giantism in children and Acromegaly in adults Deficiency of GH: Dwarfism in children – Rare in adults

Deficiency of GH – the types Hereditary or acquired The manifestations of GH deficiency depends upon: The age of the of onset of the disorder or 1 The cause whether it is heritable or acquired disease GH deficiency results Dwarfism in children GH deficiency is rare in adults

Deficiency of GH in children Dwarfism Deficiency of GH in children produces a condition called Dwarfism GH is secreted less than the body requirements Dwarfism is commonly associated with Panhypopituitrism It is a total failure of Pituitary in children

Dwarfism – Special features 1 Appropriate proportion of body parts All the physical body parts like size of hands and feet develop in appropriate proportion to each other but 1 The rate of development is greatly reduced, ≥ 4 feet 2 The affected child appears to be younger than his actual age A child at the age of 10 years may have body development of a child aged 4 -5 – years 3

Dwarfism – special features 2 A dwarf individual may not pass through puberty as he never secretes sufficient quantity of Gonadotropins GH in these children may be sufficient but there will be deficiency of IGF-1 – an active metabolite of GH 1

Dwarfism 0r growth retardation in children

GH deficiency in Adults – is rare It results in reduction of muscle mass, visceral fat deposition and osteoporosis Leads to abnormal lipid profile

GH excess – Types. 2. (age) In children and adults GH is secreted more than body requirements The effects of GH excess depend on the age of onset, both in children and adults – 2 conditions Giantism or Gigantism : This condition occurs in children when epiphysis are not Fused Acromegaly: when epiphysis are fused in adults

GH excess in adults – Acromegaly excess of GH/IGF-1 Acromegaly results from excessive secretion of growth hormone in adults with fused epiphysis It is usually the result of benign pituitary adenoma in middle aged individuals Excessive secretion of GH leads to excessive generation of IGF-1 - the mediator for the effects of GH

Acromegaly – No ↑in height ↑in soft tissue /viscera There is no ↑ in height but soft tissue proliferation. There is Enlargement of bones of hands and feet with swelling Over growth of the Skull, lower jaw and bulging of forehead Prominent nose, thick lips and tongue ↑ in the size of the viscera like Liver and heart

Acromegaly –↑ in bone thickness and soft tissues

Acromegaly - Wide sole of foot

Acromegaly – Large skull, lower jaw, prominent nose and bulging forehead

GH excess in Children- Giantism when epiphysis are not fused Giantism in children and adolescents occurs when epiphysis are not fused Chronic GH secretion leads to linear growth of long bones usually to 7- 9 feet Hyper secretion of GH is commonly associated with hypogonadism and cause of non fusion of epiphysis

Gigantism 7 feet 8 inches Pituitary gland tumor

Difference B/W Acromegaly and Giantism