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 | ||
+ | *Deficiency of growth hormone | ||
+ | *Proportionate dwarfism | ||
+ | *Growth plates remain open for up to 4 years | ||
+ | *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.
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.