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

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

2. 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

3. Abnormalities of GH – Causes/ lesionsGH 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

4. GH abnormalities – Typesexcess 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

5. Deficiency of GH – the typesHereditary or acquired • The manifestations of GHdeficiency 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

6. Deficiency of GH in childrenDwarfism • Deficiency of GH in childrenproduces 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

7. Dwarfism – Special features 1Appropriate proportion of body parts • All the physical body parts like size of hands and feetdevelop 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

8. 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

9. Dwarfism 0r growth retardation in children

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

11. 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

12. GH excess in adults – Acromegalyexcess 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

13. 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

14. Acromegaly –↑ in bone thickness and soft tissues

15. Acromegaly - Wide sole of foot

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

17. GH excess in Children- Giantismwhen 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

18. Gigantism 7 feet 8 inches Pituitary gland tumor

19. Difference B/W Acromegaly and Giantism