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Pituitary dwarfism

image of Pituitary dwarfism
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Abstract

This chapter focuses on congenital growth hormone (GH) deficiency. chapter begins with an overview of normal pituitary gland development and adenohypophysis formation. The chapter also discusses the regulation and effects of growth hormone secretion, including the role of insulin-like growth factor-1. It describes the pathophysiology of congenital GH deficiency, including the genetic mutations that can cause the condition. Clinical features, differential diagnosis, and diagnostic tests for pituitary dwarfism are also discussed. The chapter concludes with discussions of treatment options, prognosis and the availability of genetic testing for pituitary dwarfism.

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Figures

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12.1 Schematic representation of canine and feline pituitary gland development.
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12.2 Simplified model of the differentiation of anterior lobe cell lineages. Each type of endocrine cell is labelled with the hormone(s) it synthesizes. Steps in precursor cell differentiation and some of the involved transcription factors are indicated. ACTH = adrenocorticotropic hormone; DAX1 = dosage-sensitive sex reversal-adrenal hypoplasia congenita critical region on the X chromosome 1; FSH = follicle-stimulating hormone; GH = growth hormone; LH = luteinizing hormone; Lhx3/4 = LIM-domain transcription factors 3 and 4; LIF = leukaemia inhibiting factor; α-MSH = alpha-melanocyte-stimulating hormone; Neuro D1 = neurogenic differentiation factor D1; Pit-1 = pituitary transcription factor 1; POMC = pro-opiomelanocortin; PRL = prolactin; Prop-1 = prophet of Pit-1; Ptx = pituitary homeobox; SF1 = steroidogenic factor 1; Tpit = T-box pituitary transcription factor; TSH = thyroid-stimulating hormone.
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12.3 The pituitary secretion of growth hormone (GH) is under inhibitory (somatostatin) and stimulatory (GH-releasing hormone, GHRH) hypothalamic control and is also modulated by negative feedback control by insulin-like growth factor-1 (IGF-1) and GH itself. + = stimulation; – = inhibition. (Redrawn after )
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12.4 Circulating concentrations of growth hormone, prolactin, thyroid-stimulating hormone (TSH), luteinizing hormone (LH) and adrenocorticotropic hormone (ACTH) before and after the combined injection of four hypothalamic releasing hormones (corticotropin-, growth hormone-, TSH- and gonadotropin-releasing hormones) in eight German Shepherd Dogs () with pituitary dwarfism (mean + standard error). The mean values for eight healthy Beagles are given for comparison (); the shaded areas represent the standard error.
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12.5 A 6-month-old German Shepherd Dog bitch with growth retardation, retention of secondary hairs (puppy coat) and lack of primary hairs due to pituitary dwarfism.
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12.6 German Shepherd Dog dwarf with the characteristic pointed muzzle typical of affected animals.
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12.7 Contrast-enhanced computed tomography image of the pituitary area of a German Shepherd Dog dwarf at 6 months of age with a pituitary gland of normal size (height 3.6 mm; width 4.3 mm). A radiolucent area with a diameter of 1.5 mm is visible in the pituitary gland, suggestive of a cyst (arrowed).
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