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  1. Sreetharan SS, Hazim M, Saim L
    Med J Malaysia, 2006 Mar;61(1):103-5.
    PMID: 16708745 MyJurnal
    Fibrous dysplasia is an uncommon benign disorder of unknown etiology. Rarely, it presents isolated in the temporal bone. We present three cases of monostotic fibrous dysplasia that involved the entire temporal bone.
    Matched MeSH terms: Fibrous Dysplasia, Polyostotic/diagnosis*; Fibrous Dysplasia, Polyostotic/physiopathology; Fibrous Dysplasia, Polyostotic/surgery
  2. Abdullah NRA, Jason WLC, Nasruddin AB
    PMID: 28567291 DOI: 10.1530/EDM-17-0029
    Pachydermoperiostosis is a very rare osteoarthrodermopathic disorder whose clinical and radiographic presentations may mimic those of acromegaly. In the evaluation of patients with acromegaloid appearances, pachydermoperiostosis should be considered as a differential diagnosis. In this article, we report a 17-year-old boy who presented with 2-year history of acral enlargement and facial appearance changes associated with joint pain and excessive sweating. He had been investigated extensively for acromegaly, and the final diagnosis was pachydermoperiostosis.

    LEARNING POINTS: There is a broad range of differential diagnosis for acromegaloid features such as acromegaly, pseudoacromegaly with severe insulin resistance, Marfan's syndrome, McCune-Albright and a rare condition called pachydermoperiostosis.Once a patient is suspected to have acromegaly, the first step is biochemical testing to confirm the clinical diagnosis, followed by radiologic testing to determine the cause of the excess growth hormone (GH) secretion. The cause is a somatotroph adenoma of the pituitary in over 95 percent of cases.The first step is measurement of a serum insulin-like growth factor 1 (IGF1). A normal serum IGF1 concentration is strong evidence that the patient does not have acromegaly.If the serum IGF1 concentration is high (or equivocal), serum GH should be measured after oral glucose administration. Inadequate suppression of GH after a glucose load confirms the diagnosis of acromegaly.Once the presence of excess GH secretion is confirmed, the next step is pituitary magnetic resonance imaging (MRI).Atypical presentation warrants revision of the diagnosis. This patient presented with clubbing with no gigantism, which is expected in adolescent acromegalics as the growth spurt and epiphyseal plate closure have not taken place yet.

    Matched MeSH terms: Fibrous Dysplasia, Polyostotic
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