Disease relevance of Dwarfism, Pituitary

• Eighteen patients with pituitary dwarfism were treated for 1 7/12 to 6 years with human growth hormone (hGH) at a dose of 0.19--0.62 unit (U) per kg of body weight per week [1].

High impact information on Dwarfism, Pituitary

• Mutations that cause pituitary dwarfism in the mouse reside in the gene encoding the transcription factor growth hormone factor 1 (GHF1 or pit1) [2].
• Hereditary pituitary dwarfism in mice affects skeletal and cardiac myosin isozyme transitions differently [3].
• The results provide an explanation for the previously observed lack of epinephrine sensitivity of the cyclase of lymphoid cells of immunodeficient pituitary dwarf mice and suggest that the depressed lymphoid cell population of the dwarf is the result of an inability to recover from stress-related lymphoid involution [4].
• In conclusion, although somatic gene therapy for GH delivery is beneficial for pituitary dwarfism, it may have adverse metabolic consequences in those with normal hypothalamic-pituitary functions, and the female mice were more severely affected than males [5].
• Acromegalic sera produced curves parallel to the hGH standard and pituitary dwarf serum had no 125I-hGH displacing activity [6].

Chemical compound and disease context of Dwarfism, Pituitary

• Low setting of feedback regulation of TSH secretion by thyroxine in pituitary dwarfism with TSH-releasing hormone deficiency [7].
• Four patients with idiopathic pituitary dwarfism were shown to have growth hormone (GH), adrenocorticotropin (ACTH), and luteinizing hormone (LH) deficiencies [7].
• In hypophysectomized or pituitary dwarf mice the reduced androgen stimulation of GUS can be partially restored with GH treatment [8].
• Assessment of prolactin release in pituitary dwarfism with the sulpiride test [9].
• Urinary excretion of proline and hydroxyproline was studied in five groups of subjects, viz. controls, pituitary dwarfs, familial dwarfs, patients with fibrodysplasia ossificans progressive and patients with generalized scleroderma [10].

Biological context of Dwarfism, Pituitary

• Immunological competence in Snell-Bagg pituitary dwarf mice: response to the contact-sensitizing agent oxazolone [11].

Anatomical context of Dwarfism, Pituitary

• A case of severe pituitary dwarfism associated with prolactin and thyroid stimulating hormone deficiencies [12].
• These findings suggest that one of the causes of idiopathic pituitary dwarfism is insufficient GHRH release from the hypothalamus [13].

Gene context of Dwarfism, Pituitary

• Occasional expression of GH and PRL in the Ames dwarf pituitary may result from the "partial loss of function" nature of the Ames Prop-1 mutation [14].
• The fasting plasma GHRH level in 22 patients with idiopathic pituitary dwarfism was 6.3 +/- 2.3 ng/l (mean +/- SD), which was significantly lower than that in normal children (9.8 +/- 2.8 ng/l, N = 21), and eight of them had undetectable concentrations (less than 4.0 ng/l) [13].
• Pituitary dwarfism in the R271W Pit-1 gene mutation [15].
• Furthermore, CRF and GRF-stimulation tests are useful in elucidating the cause of anterior pituitary failure and GRF may be of benefit for the treatment of pituitary dwarfism [16].
• Isolated growth hormone (GH) deficiency (IGHD) is detected in 1/10 of pituitary dwarfism, but there are only a few reports on IGHD as an autosomal-dominant trait [17].

Analytical, diagnostic and therapeutic context of Dwarfism, Pituitary

• With the aid of a recently developed commercially available radioimmunoassay for Somatomedin-C (Sm-C) we measured the Sm-C levels in 38 controls, 24 untreated acromegalics, 45 inactive acromegalics and five pituitary dwarfs [18].
• Measurement of zinc, copper, manganese, and iron concentrations in hair of pituitary dwarfism patients using flameless atomic absorption spectrophotometry [19].
• Little or no response of plasma GHRH to oral administration of L-dopa was observed in 7 of 10 pituitary dwarfs, and 3 of the 7 patients showed a response of plasma GH to iv administration of GHRH (1 microgram/kg) [13].

References