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Spot Diagnosis - Growth retardation with Hypertension

Discussion in 'Spot Diagnosis' started by neo_star, Jan 25, 2013.

  1. neo_star

    neo_star Moderator

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    03.jpg

    Yes, this child has Cushingoid features, but that is not the answer i am looking for.

    Hint: Bilateral adrenalectomy can prove disastrous, in-spite of supplementing with hormones.
     

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  2. Chriss

    Chriss Well-Known Member

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    High doses of glucocorticoids such as betamethasone or dexamethasone exerts negative feedback on the pituitary and decreases the ACTH levels. As a result, the child develops Cushing's Syndrome. This form of CS is called Iatrogenic Cushing's Syndrome.

    Hope this is what you were looking for!

    :)
     

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  3. neo_star

    neo_star Moderator

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    Another Hint: Bilateral Adrenalectomy will result in Nelson syndrome.

    Generally, if on Rx with steroids and with the level of awareness that we have these days of it's adverse effects...steroids are lowered or terminated in leiu of steroid sparing agents b4 it gets to this stage.

    This problem generally passes off as ordinary obesity, till the parents think that the obesity is out of proportion to the child's intake. Sometimes, headache is the chief complaint...but if the child is a girl then it's ignored as pseudo intra-cranial hypertension sec to obesity.
     

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  4. neo_star

    neo_star Moderator

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    Answer: Cushing Disease due to an ACTH secreting microadenoma (most common scenario) causing Bilateral Adrenal Hyperplasia.

    This is the 2nd most common cause of Cushing syndrome ( medication being first ) in the pediatric age group after age 7...in infancy and the first few yrs of life it is adrenal carcinoma. Although rare it should be among our differentials when investidating obesity of unknown cause and again underscores the importance of checking the vitals in the pediatric age group.

    Mx ( courtesy - Oxford textbook of medicine )


    The treatment of Cushing's disease has been improved by trans-sphenoidal surgery conducted by an experienced surgeon. Before the selective removal of a pituitary microadenoma the treatment of choice was bilateral adrenalectomy. This had an appreciable mortality even in the best centres (about 4 per cent) as well as morbidity. The main risk was the subsequent development of Nelson's syndrome (postadrenalectomy hyperpigmentation with locally aggressive pituitary tumour) (Fig. 12 and Plate 1). To avoid this, pituitary irradiation was often carried out following bilateral adrenalectomy. These patients required lifelong replacement therapy with hydrocortisone and fludrocortisone. Nowadays bilateral adrenalectomy is reserved for the occasional patient with Cushing's disease in whom no pituitary tumour can be found, or when pituitary surgery has failed, or where the condition has recurred.


    After selective removal of a microadenoma, the surrounding corticotrophs are normally suppressed. In these cases plasma cortisol concentrations are also suppressed postoperatively and glucocorticoid replacement therapy is required. Using the dexamethasone regime described above after removal of an adrenal adenoma, there is usually (but not invariably) gradual recovery of the hypothalamic-pituitary-adrenal axis. A non-suppressed plasma cortisol postoperatively suggests that the patient is not cured, even though cortisol secretion may have fallen to normal or subnormal values. Close follow-up of such individuals is required.

    In the past, pituitary irradiation was often used in the treatment of Cushing's disease. However, the improvements in pituitary surgery have resulted in far fewer patients being so treated. In children pituitary irradiation appears to be effective. Radiotherapy is not recommended as a primary treatment but is reserved for patients not responding to pituitary microsurgery or when bilateral adrenalectomy has been performed, or in those with established Nelson's syndrome.

    Mx gone wrong.JPG
     

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