Difference between revisions of "Congenital Panhypopituitarism"

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(Created page with "Also known as ''Pituitary Dwarfism'', Congenital Panhypopituitarism is defined by failure of differentiation of oesophageal ectoderm from which the pituitary is derived. The pit...")
 
 
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*Rare in animals, reported in German Shepherd Dogs
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*Deficiency of growth hormone
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*Proportionate dwarfism
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*Growth plates remain open for up to 4 years
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*Disorganised proliferating chondrocytes
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Also known as ''Pituitary Dwarfism'', Congenital Panhypopituitarism is defined by failure of differentiation of oesophageal ectoderm from which the pituitary is derived.  The pituitary is replaced by a cystic dilatation of ''Rathke's Cleft'' which replaces normal tissue with multilocular cysts- lined with non-secretory cuboidal epithelium.   
 
Also known as ''Pituitary Dwarfism'', Congenital Panhypopituitarism is defined by failure of differentiation of oesophageal ectoderm from which the pituitary is derived.  The pituitary is replaced by a cystic dilatation of ''Rathke's Cleft'' which replaces normal tissue with multilocular cysts- lined with non-secretory cuboidal epithelium.   
 
[[Image:Pituitary dwarfism.jpg|right|thumb|125px|<small><center>'''Cystic Rathke's Pouch'''. Courtesy of A. Jefferies</center></small>]]
 
[[Image:Pituitary dwarfism.jpg|right|thumb|125px|<small><center>'''Cystic Rathke's Pouch'''. Courtesy of A. Jefferies</center></small>]]
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[[Category:Pituitary Gland - Pathology]]
 
[[Category:Pituitary Gland - Pathology]]
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[[Category:Bones - Metabolic Pathology]]

Latest revision as of 18:43, 27 February 2011

  • Rare in animals, reported in German Shepherd Dogs
  • Deficiency of growth hormone
  • Proportionate dwarfism
  • Growth plates remain open for up to 4 years
  • Disorganised proliferating chondrocytes


Also known as Pituitary Dwarfism, Congenital Panhypopituitarism is defined by failure of differentiation of oesophageal ectoderm from which the pituitary is derived. The pituitary is replaced by a cystic dilatation of Rathke's Cleft which replaces normal tissue with multilocular cysts- lined with non-secretory cuboidal epithelium.

Cystic Rathke's Pouch. Courtesy of A. Jefferies

Incidence:

  • Seen in German Shepherd Dog. Inherited as a simple autosomal recessive trait in affected dogs.
  • Congenital hypoplasia of the pituitary in the cat has also been reported.

Clinical signs:

Variable with degree of involvement of the pituitary. Most obvious signs are due to the lack of GH during the growing phase of the puppy's life:

  • Pups appear normal at birth as the placenta supplies the foetus' need for GH.
  • Retarded growth and proportionate dwarfism.
  • Delayed growth plate closure; long bone physes normally close at 7-12 months but those of a pituitary dwarf will remain open until 3-4 years.
  • Delayed dental eruption.
  • Prognathism; undershot lower jaw as GH needed for mandible length.
  • Pups are quieter, more nervous and perhaps slower to learn.
  • Retention of puppy coat; wooly. Adult coat does not develop and a bilaterally symmetric, non-pruritic alopecia occurs over areas of friction E.g. neck and trunk.
  • Hyperpigmentation with comedomes.
  • Reproductive signs E.g. failure to cycle, testicular atrophy.

May also see signs of

  • Secondary hypothyroidism
  • Secondary hypoadrenocorticism

Diagnosis:

  • Presumptive diagnosis based on signalment and clinical signs.
  • Radiography to detect growth plate closure.
  • GH stimulation test; Use alpha-2 agonist E.g. Xylazine which inhibit somatostatin and so induce GH production and release. Normal dogs will have 2-4X increased GH after 20 mins while a pituitary dwarf will show little change in GH upon stimulation.
  • Somatomedin Assay; Also called insulin-like growth factors. GH mediates its effects by stimulating the production of IGFs which can be more easily measured. Low levels will be found in pituitary dwarfs.
  • May also need to consider TSH and ACTH stimulation tests.

Treatment:

Need to treat early or will remain dwarfs.

  • Sub-cut bovine/human GH can induce the growth of an adult haircoat.
    Nb. GH is diabetogenic so overdose will induce Diabetes Mellitus.
  • Progestagens may induce GH production from the mammary gland.
  • May need to replace thyroid hormones and/or glucocorticoids.