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J Laryngol Otol. 2002 Sep;116(9):736-9.
Lipoid proteinosis of the larynx.
Oz F, Kalekoglu N, Karakullukcu B, Ozturk O, Oz B.
Department of Otolaryngology, Head and Neck Surgery, Istanbul University Cerrahpasa Medical Faculty, Turkey.
Lipoid proteinosis is a rare disease that presents with hyaline deposits in many tissues. It involves predominantly the skin and upper aerodigestive tract, presenting with small yellowish papules and hoarseness. It may involve the central nervous system and cause intracerebral calcifications. Laryngeal lesions may resemble singer's nodule or chronic laryngitis. The pathogenesis of the disease is not clear although several studies suggest a defective collagen production and/or lysosomal storage disease. In this article two cases with skin and larynx involvement are reported.
online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12437814&dopt=Abstract
Folia Biol (Krakow). 2000;48(1-2):37-42.
Myotomal myogenesis in Triturus vulgaris L. (Urodela) with special reference to the role of mesenchymal cells.
Daczewska M, Kielbowna L.
Laboratory of Evolutionary and Developmental Biology of Vertebrates, University of Wroclaw, Poland. daczeiol.uni.wroc.pl
Two stages can be distinguished in the differentiation of myotomal muscle fibres in Triturus vulgaris. In the first stage only synchronously differentiating myotomal cells are engaged; in the second stage mesenchymal cells also take part in the process. Myotomal cells (primary myoblasts) fuse to form 2-3 nucleate myotubes. Only in the caudal part of the embryo mononucleate myotubes persist. The mononucleate myotubes, like polynucleate ones, occupy the whole length of the myotome. The differentiation of myotubes is accompanied by vitellolysis. At further development stages mesenchymal cells enter the intermyotomal fissure, after which they migrate to the myotomes, between the myotubes. The cells that remain in the intermyotomal fissures retain their fibroblastic potential (they synthesise collagen). Their daughter cells adjoining the myotubes acquire myogenic abilities. Their myoblastic potential is evidenced by their ability to fuse with the myotube. Fusion of secondary myoblasts (of mesenchymal origin) with the myotube results in further growth of the myotubes. In T. vulgaris myotomal myotubes and muscle fibres developing from them are of myotomal-mesenchymal origin.
online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11080917&dopt=Abstract
Pituitary. 1998 Apr;1(1):59-67.
Growth hormone deficiency in elderly patients with hypothalamo-pituitary tumors.
Colao A, Cerbone G, Pivonello R, Klain M, Aimaretti G, Faggiano A, Di Somma C, Salvatore M, Lombardi G.
Department of Molecular and Clinical Endocrinology, Federico II University, Naples, Italy.
In 18 patients with hypothalamo-pituitary diseases aged over 60 years and in 18 sex, age- and BMI-matched healthy subjects, the results of plasma IGF-I and IGF-BP3 levels and the GH response to GHRH + arginine test (GHRH + ATT) were correlated to the results of body composition, serum osteocalcin (OC) and urinary cross-linked N-telopeptides of type I collagen (Ntx) and the bone mineral density (BMD). In 10 patients and 10 controls, the GH response to ITT was also evaluated. The GH response to GHRH + ATT and ITT was markedly reduced in patients compared to controls (3.1 +/- 0.7 vs. 23.2 +/- 2.3 micrograms/L, P < 0.001 and 1.1 +/- 0.3 vs. 6.4 +/- 0.8 micrograms/L, P < 0.001), so all patients were classified as GHD, though no significant difference was found in plasma IGF-I and IGF-BP3 levels between the two groups. Body composition analysis revealed a significant increase of fat mass (37.4 +/- 2.2 vs. 28.0 +/- 1.0%, P < 0.001), a significant decrease of lean mass (62.6 +/- 2.2 vs. 72.0 +/- 1.0%, P < 0.001) and total body water (45.7 +/- 1.5 vs. 52.5 +/- 1.1%, P < 0.001) in patients compared to controls. Serum OC levels were lower (1.9 +/- 0.1 vs. 4.6 +/- 0.4 micrograms/L, P < 0.001) in patients than in controls, whereas urinary Ntx levels were similar. BMD values in lumbar spine (0.81 +/- 0.02 vs. 0.90 +/- 0.02 g/cm2, P < 0.001) and femoral neck (0.70 +/- 0.02 vs. 0.82 +/- 0.02 g/cm2, P < 0.001) were significantly lower in patients than in controls. A significant inverse correlation was found between GHD duration and lumbar spine (r = -0.73, P&1t; 0.001) or femoral neck (r = -0.81, P&1t; 0.001) BMD values and a significant direct correlation was found between GH peak after GHRH + ATT and lumbar BMD (r = 0.69, p = 0.001) in GHD patients. In conclusion, GHD in patients over 60 yrs aged with a characteristic history of hypothalamus-pituitary pathology is distinct from the physiological decline in GH secretion associated with aging.
online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11081184&dopt=Abstract
Nippon Ganka Gakkai Zasshi. 2000 Oct;104(10):706-10.
[Collagen fibrils in nanophthalmic sclerae]
[Article in Japanese]
Fukuchi T, Abe H, Sawaguchi S.
Department of Ophthalmology, Niigata University School of Medicine, Japan.
PURPOSE: To examine collagen fibrils in 3 nanophthalmos sclerae and to compare them with normal control sclerae morphometrically. MATERIALS AND METHODS: Three cases of nanophthalmos associated with uveal effusion were studied. When sclerectomy was performed, scleral specimens were collected and fixed with 3% glutaraldehyde/2.5% paraformaldehyde. After epon-embedding and ultrathin sectioning, they were examined by transmission electron microscopy. Collagen fibrils from both nanophthalmos and normal control sclerae were compared in diameters and numbers per micron 2 areas. RESULTS: All scleral tissues from the three cases were associated with irregularly woven and unclear collagen bundles. Several abnormal findings, such as twisting or fraying, were also detected in a few collagen fibrils. The diameter and density of normal-appearing collagen fibrils that occupied most areas of nanophthalmos sclerae were the same as those from normal control sclerae morphometrically. CONCLUSIONS: Although nanophthalmos sclerae even with uveal effusion showed thick irregular collagen bundles and a few abnormal collagen fibrils, most collagen fibrils appeared the same as normal controls.
online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11081305&dopt=Abstract
Bone Marrow Transplant. 2000 Oct;26(8):881-6.
Predictive markers for hepatic veno-occlusive disease after hematopoietic stem cell transplantation in adults: a prospective single center study.
Tanikawa S, Mori S, Ohhashi K, Akiyama H, Sasaki T, Kaku H, Hiruma K, Matsunaga T, Morita T, Sakamaki H.
Bone Marrow Transplantation Team, Tokyo Metropolitan Komagome Hospital, Japan.
Hepatic veno-occlusive disease (VOD) is a major complication after hematopoietic stem cell transplantation (HSCT). Aetiological determinants, diagnosis and treatment remain unclear. Changes in coagulation-fibrinolysis parameters and N-terminal propeptide for type III procollagen (P-III-P) have been studied in patients with or without VOD after HSCT. We prospectively measured protein C activity, tissue plasminogen activator (t-PA), antithrombin III (AT-III), plasminogen activity (PLG), thrombin-antithrombin III (TAT), alpha2-plasmin inhibitor (alpha2-PI),fibrinogen (Fbg) and P-III-P in 44 consecutive adult patients undergoing allogeneic HSCT. Each parameter was determined before conditioning, on day 0 of HSCT and weekly for 5 weeks. Five of the 44 patients developed VOD at a median post HSCT of day 3 (range, day 3 to 12). On repeated analysis of variance (ANOVA), there were significant differences between patients with and without VOD in P-III-P (P < 0.0001), protein C (P < 0.0001), t-PA (P < 0.0001), PLG (P < 0.0001), AT-III(P < 0.0001), Fbg (P < 0.0001), alpha2-PI (P = 0.0002). Levels of P-III-P were significantly higher in patients with VOD than without VOD, before preparative chemotherapy (P < 0.005) and on days 0 and 7 (P < 0.001). On day 0, levels of t-PA were significantly higher in patients with VOD than without VOD (P < 0.05). On day 7, levels of protein C were significantly lower in patients with VOD than without VOD (P < 0.01). On day 0, there were trends of differences (P = 0.0515) between patients with and without VOD in the levels of protein C. These results suggest P-III-P, t-PA and protein C are predictive markers for VOD after HSCT in adults. Moreover, the serum P-III-P level before start of conditioning might indicate patients at risk for developing VOD.
online pharmacy ref. source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11081389&dopt=Abstract
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