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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of bone and mineral metabolism 7 (1989), S. 42-48 
    ISSN: 1435-5604
    Keywords: Male hypogonadal osteoporosis ; Slipped capital femoral epiphysis ; Testosterone ; GH ; Somatomedin C
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two males with bone abnormalities associated with hypogonadotropic hypogonadism are reported. Case 1, 28 years old male, developed growth disturbance at the age of eight years, after suffering from tuberculous meningitis. No secondary sex characteristics appeared and fractures occurred at five times. Case 2, 29 years old male, also suffered from growth disturbance from around the age of 6 years, without appearance of secondary sex characteristics even after puberty. Bone X-ray studies and bone biopsy revealed marked osteoporosis in Case 1, while in Case 2, slipped capital femoral epiphysis was also noted with mild osteoporosis. In these two cases, osteoporosis is associated with eunuchoidism, in agreement of the concept of so-called “male hypogonadal osteoporosis”. Both patients showed insufficient secretion of somatomedin C, testosterone and growth hormone (GH) with insulin tolerance test and arginine tolerance test. The insufficient secretion of LH and FSH with LH-RH tolerance test was also revealed in both cases. The decrease of GH and somatomedin C was quite pronounced in Case 1, whereas the fall of testosterone was more conspicuous in Case 2. The imbalance between these hormone deficiencies might lead to different expression of bone abnormalities.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-2307
    Keywords: Membranous lipodystrophy (Nasu-Hakola disease) ; Membranocystic lesion ; Lectin histochemistry ; α-D-galactose ; MPA-HPR colloidal gold
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary This paper reports three cases of membranous lipodystrophy (Nasu-Hakola disease) in two families and studies the carbohydrate components of membranocystic lesions in all three cases, using twelve kinds of lectins labelled by horseradish peroxidase (HRP).Maclura pomifera agglutinin (MPA), which specifically binds α-D-galactose residues, strongly stained typical membranocystic lesions, whereas the other lectins did not. However,Helix pomatia agglutinin (HPA), which specifically binds to N-acetyl-D-galactosamine (GalNAc), stained the membranes of degenerated adipose cells. These were thought to appear during the initial or early stage of the membranocystic lesions. This suggests that a change of carbohydrate residues occurs during the formation of the membranocystic lesions. We also investigated the lectin binding sites at the ultrastructural level using MPA-HRP colloidal gold (CG) conjugate. In the well developed membrane, CG particles were arranged regularly along the minute tubular structures. On the other hand, there were a few irregularly spaced CG particles on the thinner membranes and also on the membranes of the degenerating adipose cells. No CG particles labelled the cell membranes of normal adipose cells. The presence of α-D-galactose residues in the membranocystic lesions is demonstrated for the first time at the electron microscopic level.
    Type of Medium: Electronic Resource
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