Congenital dystrophic epidermolysis bullosa (DEB) in Sprague Dawley rats: a case series

2016 ◽  
Vol 27 (2) ◽  
pp. 122-e34 ◽  
Author(s):  
Kristin B. Eden ◽  
Ashley Peterson ◽  
Harold R. Payne ◽  
Wayne V. Corapi ◽  
Joanne Mansell ◽  
...  
Author(s):  
Delphine Darbord ◽  
Geoffroy Hickman ◽  
Nathalie Pironon ◽  
Claire Barbieux ◽  
Mathilde Bonnet‐des‐Claustres ◽  
...  

2019 ◽  
Vol 13 (1) ◽  
pp. 3-7
Author(s):  
Inne Arline Diana ◽  
Srie Prihianti Gondokaryono ◽  
Reiva Farah Dwiyana ◽  
July Iriani Rahardja ◽  
Yuri Yogya ◽  
...  

Background: Epidermolysis bullosa, a group of inheritable blistering diseases with considerable clinical and genetic diversity, is divided into distinct subtypes depending on the level of tissue separation in the dermal–epidermal basement membrane zone. The dystrophic form of epidermolysis bullosa (DEB) is characterized by tense blisters and erosions which heals with extensive scarring. The fact that DEB can be inherited in either autosomal dominant (DDEB) or autosomal recessive (RDEB) pattern adds to its clinical diversity. The cause of marked clinical diversity in mild to severe DDEB is still unidentified. Main Observation: We report an intrafamilial diversity of clinical severity in dominant dystrophic epidermolysis bullosa (DDEB) cases within three generations. Conclusion: We emphasize the variety of clinical severity in DDEB cases within three generations which might be caused by unknown gene modifiers and environmental factors.


2020 ◽  
pp. 1-2
Author(s):  
S. Madhulika ◽  
Sreekala. R ◽  
Uday Kiran Raja ◽  
A.Vijaya Mohan Rao

Epidermolysis bullosa (EB) refers to a group of inherited disorders that involve the formation of blisters following trivial trauma. Here we report a case series of Dystrophic Epidermolysis bullosa(DEB) at our hospital presented with various clinical features. History ,clinical spectrum and histopathological examination gives appropriate diagnosis for this disorder. Treatment remains challange. A multidisciplinary approach is needed for the effective management of EB. Good nursing care, and gene therapy could possibly significantly alleviate the suffering of the patients in the future.


2021 ◽  
Vol 141 (10) ◽  
pp. S179
Author(s):  
D. Darbord ◽  
G. Hickman ◽  
N. Pironon ◽  
C. Barbieux ◽  
M. Bonnet-des-Claustres ◽  
...  

2020 ◽  
Vol 40 (6) ◽  
pp. 561-565
Author(s):  
Sebastián Véliz ◽  
Hinrich Huber ◽  
María Joao Yubero ◽  
Ignacia Fuentes ◽  
Fatimah Alsayer ◽  
...  

Author(s):  
D. J. McComb ◽  
J. Beri ◽  
F. Zak ◽  
K. Kovacs

Investigation of the spontaneous pituitary adenomas in rat have been limited mainly to light microscopic study. Furth et al. (1973) described them as chromophobic, secreting prolactin. Kovacs et al. (1977) in an ul trastructural investigation of adenomas of old female Long-Evans rats, found that they were composed of prolactin cells. Berkvens et al. (1980) using immunocytochemistry at the light microscopic level, demonstrated that some spontaneous tumors of old Wistar rats could contain GH, TSH or ACTH as well as PRL.


Author(s):  
F. G. Zaki ◽  
E. Detzi ◽  
C. H. Keysser

This study represents the first in a series of investigations carried out to elucidate the mechanism(s) of early hepatocellular damage induced by drugs and other related compounds. During screening tests of CNS-active compounds in rats, it has been found that daily oral administration of one of these compounds at a dose level of 40 mg. per kg. of body weight induced diffuse massive hepatic necrosis within 7 weeks in Charles River Sprague Dawley rats of both sexes. Partial hepatectomy enhanced the development of this peculiar type of necrosis (3 weeks instead of 7) while treatment with phenobarbital prior to the administration of the drug delayed the appearance of necrosis but did not reduce its severity.Electron microscopic studies revealed that early development of this liver injury (2 days after the administration of the drug) appeared in the form of small dark osmiophilic vesicles located around the bile canaliculi of all hepatocytes (Fig. 1). These structures differed from the regular microbodies or the pericanalicular multivesicular bodies. They first appeared regularly rounded with electron dense matrix bound with a single membrane. After one week on the drug, these vesicles appeared vacuolated and resembled autophagosomes which soon developed whorls of concentric lamellae or cisterns characteristic of lysosomes (Fig. 2). These lysosomes were found, later on, scattered all over the hepatocytes.


Author(s):  
D. J. McComb ◽  
J. Beri ◽  
F. Zak ◽  
K. Kovacs

Gonadotroph cell adenomas of the pituitary are infrequent in human patients and are not invariably associated with altered gonadal function. To date, no animal model of this tumor type exists. Herein, we describe spontaneous gonadotroph cell adenomas in old male and female Sprague-Dawley rats by histology, immunocytology and electron microscopy.The material consisted of the pituitaries of 27 male and 38 female Sprague Dawley rats, all 26 months of age or older, removed at routine autopsy. Sections of formal in-fixed, paraffin-embedded tissue were stained with hematoxylin-phloxine-saffron (HPS), the PAS method and the Gordon-Sweet technique for the demonstration of reticulin fibers. For immunostaining, sections were exposed to anti-rat β-LH, anti-ratβ-TSH, anti-rat PRL, anti-rat GH and anti-rat ACTH 1-39. For electron microscopy, tissue was fixed in 2.5% glutaraldehyde, postfixed in 1% OsO4 and embedded in epoxy-resin. Tissue fixed in 10% formalin, embedded in epoxy resin without osmification, was used for immunoelectron microscopy.


Author(s):  
Russell N. A. Cecil ◽  
H. Clarke Anderson

Unfixed proximal tibial epiphyseal growth plates were studied by freeze-etch to confirm the presence of extracellular calcifying matrix vesicles and to determine the substructure of matrix vesicle membranes as compared to plasma and other membranes of intact chondrocytes. Growth plates from 6-10 week old Sprague-Dawley rats were cut into 1x3 mm blocks whose long dimension was oriented either perpendicular or parallel to the long axis of the tibia. Some blocks were fixed at pH 7. 0 in 0. 2M cacodylate - buffered 2. 5% glutaraldehyde for 1 hour at 4ÅC. The blocks were immersed in 30% glycerol solution at 4ÅC for 1 hour, frozen in liquid nitrogen, and then fractured, etched for 2 minutes, and coated with platinum, carbon and 0. 2% Formvar solution. The replicas were cleaned with chromic acid, floated onto Formvar coated grids, and examined with a Phillips EM 300 electron microscope.Fixed and unfixed specimens appeared similar in ultrastructure. Chondrocytes, matrix, and matrix vesicles were identified. In specimens fractured parallel to the long axis of the tibia, the reserve, proliferative, hypertrophic, and calcifying zones could be discerned as described by light and electron microscopy.


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