[A rare cause of AA amyloidosis: Hereditary epidermolysis bullosa]. / Une cause rare de l'amylose AA : les épidermolyses bulleuses héréditaires.

INTRODUCTION: Recessive dystrophic epidermolysis bullosa is a rare genetic condition characterized by fragile skin and mucous membrane, caused by mutations in the COL7A1 gene. AA amyloidosis is a rare complication of these genodermatosis. OBSERVATIONS: Two patients with recessive dystrophic epidermolysis bullosa, generalized severe in the first case and generalized intermediate in the second case, developed at the age of 38 and 28, respectively, nephrotic syndrome. The diagnosis of secondary renal amyloidosis was confirmed by renal biopsy in the first case and by minor salivary gland biopsy in the second case. Death occurred 2 months after diagnosis in both cases. CONCLUSION: Renal involvement is quite common in AA amyloidosis in patients with recessive dystrophic epidermolysis bullosa. Nephrotic syndrome and rapid decline in renal function renal are characteristic features. The prognosis is poor due to underlying conditions and the lack of an etiological treatment.

Chondroitin/dermatan sulfate purified from corb (Sciaena umbra) skin and bone: In vivo assessment of anticoagulant activity.

The present work deals with the extraction and purification of chondroitin sulfate/dermatan sulfate from skin (CSG) and bone (CBG) of corb (Sciaena umbra). Electrophoresis of these polymers in barium acetate buffer on cellulose acetate revealed two fractions similar to dermatan sulfate and chondroitin sulfate. The in vivo anticoagulant activity of both chondroitin sulfate/dermatan sulfate (CS/DS) were evaluated, at 25 and 75 mg kg-1 of body weight (b.w), using activated partial thromboplastin time (aPTT), prothrombine time (TT) and thrombin time (PT) tests. Results showed that aPTT of CSG and CBG at 75 mg kg-1 of b.w were prolonged by 1.59 and 1.48-fold respectively, compared with the control. Further, toxicity studies on liver performed by the catalytic activity of transaminases in plasma, oxidative stress markers and hepatic morphological changes demonstrated that CSG and CBG at both doses are not toxics. In summary, the higher activity and lower toxicity of both CS/DS, especially at 25 mg kg-1 of b.w, recommended these compounds as a better drug candidate.

Acute oral toxicity study on Wistar rats fed microalgal protein hydrolysates from Bellerochea malleus.

Protein hydrolysates and bioactive peptides from various protein sources have demonstrated their effectiveness for the prevention of illness and the improvement of symptoms from several diseases. In particular, the use of microalgae to generate bioactive peptides has received a growing interest because of their potential to be cultivated on non-arable land and high nutritional value. However, scant research is available on the toxicity of peptide-based preparations. The present study aims to evaluate the toxicity of microalgal protein hydrolysates (MPH) from one marine species of microalgae (Bellerochea malleus) to determine the feasibility of their use for functional food applications. Results showed that the oral administration of MPH at three doses (D1, 100 mg kg-1 BW; D2, 400 mg kg-1 BW; and D3, 2000 mg kg-1 BW) to male Wistar rats did not induce any adverse effects or mortality up to13 days of treatment. Data analysis of relative organ weights and biochemical and hematological parameters did not show any significant differences between control and treated groups at the three doses investigated. Data from histopathological observations did not reveal any signs of major toxicity at the doses D1 and D2. However, mild signs of inflammation and necrosis were observed in the kidney of rats fed MPH at D3. All together, these results reveal the overall safety of MPH and provide new evidence for advocating their use for functional food or nutraceutical applications.

Polysaccharide from Pimpinella anisum seeds: Structural characterization, anti-inflammatory and laser burn wound healing in mice.

The polysaccharide preparation from Pimpinella anisum seeds (PAP) was isolated and characterized to evaluate its laser burn wound-healing and anti-inflammatory activities in mice. The structure characterization of PAP by Infra-red spectrometry (IR), Nuclear magnetic resonance (NMR), Gas chromatogram-Mass spectrometer (GC-MS) and colorimetric methods revealed an optimum yield of 8.84%, a high quantity of carbohydrate (64.75%) and low levels of lipids, protein and sulfate. Galactose (33.47%), ß-d-Glucose (26.71%) and α-d-Mannose (18.21%) were the major monosaccharides components presenting in PAP, and a smaller amounts of ß-d-Galactose, d-Fructose, α-d-Glucose, α-l-Galactose and arabinose were detected. PAP showed noticeable antioxidant and antibacterial properties. The anti-inflammatory activity of PAP in the carrageenan-induced paw edema model in mice, demonstrated by reduced edema and cellular infiltration, and oxidative stress markers in muscle tissue. A beneficial wound healing effect was also revealed. The topical application of PAP based gel on laser burn lesions accelerates wound contraction, the re-epithelization and remodeling phases after seven days of treatment. The results demonstrated that PAP is a novel promising source of natural wound healing and anti-inflammatory drugs. The high content and varied PAP monosaccharides seem to be responsible for the observed biological activities.

Combination of amphotericin B and caspofungin in the treatment of mucormycosis.

We report a case of invasive mucormycosis in 52 year-old woman. CT-scan and magnetic resonance imaging found a partial right sinus thrombosis associated with homolateral ethmoidal and maxillary sinusitis with submucosal inflammation. Histopathological examination of excised tissue was positive for mucormycosis. Our patient was treated by surgical debridement and a combination of amphotericin B and caspofungin, with a good outcome.

Sulfated polysaccharide isolated from Globularia alypum L.: Structural characterization, in vivo and in vitro anticoagulant activity, and toxicological profile.

A sulfated polysaccharide from Globularia alypum L. (GASP) was extracted with a yield of 14.2%. GASP is composed mostly of sulfate and total sugars (13.29% and 71.56%, respectively) with small amount of proteins and lipids. The chemical and structural characterization was studied by Infra-Red spectroscopic and gas chromatography-mass spectrometry (GC-MS). GASP composed of eight carbohydrates where galactose, glucose, and mannose are the major compounds (33.47%, 26.71% and 18.21%, respectively). The in vitro and in vivo anticoagulant activities in rats were tested using the standard coagulation assays activated partial thromboplastin time (aPTT), prothrombine time (TT) and thrombin time (PT) tests. Both doses of GASP (200 and 500 mg/kg b.w) displayed a significant in vitro (1.22 and 1.33-fold, 1.17 and 1.27-fold, and 1.21 and 1.26-fold, respectively) and in vivo (1.47 and 2.52-fold; 1.20 and 1.43-fold; 1.21 and 1.40-fold, respectively) compared with the control. Toxicity studies on liver performed by the catalytic activity of transaminases in plasma, oxidative stress markers and hepatic morphological changes indicated that GASP at both doses are not toxics. The important pharmacological and toxicological profile of GASP revealed that this compound may be used as a novel and effective drug.

Combined use of Vitamins E and C improve nephrotoxicity induced by colistin in rats.

This study was performed to investigate the protective effect of combined use of Vitamins E and C on colistin-induced tubular damage in rat. Animals were treated with sterile saline, colistin methanesulfonate (CMS), CMS + Vitamin E + Vitamin C, and Vitamin E + vitamin C, respectively, for seven days. Thereafter, animals were sacrificed and the urine N-acetyl-b-D-glucosaminidase (NAG) and gamma-glutamyl transferase (GGT) levels, plasma level of creatinine (Cr), vitamin E and vitamin C, and renal tissue levels of malondialdehyde (MDA), superoxide dismutase (SOD), catalase (CAT), and glutathione peroxidase (GPx), as well as renal histology were performed. CMS induced acute tubular necrosis, increased the NAG, GGT, and MDA levels, and reduced the Vitamin E, Vitamin C, SOD, CAT, and GPx activities. Co-treatment with vitamins E and C restored all biochemical parameters cited above and improved the histopathological damage. Tubular damage induced by colistin is at least partly due to oxidative stress. Nephroprotective effect of Vitamins E and C is partially mediated through its antioxidant properties, and the higher protection by the combination of these vitamins is related to its synergistic effects.

Effect of vitamin E on reversibility of renal function following discontinuation of colistin in rats: Histological and biochemical investigations.

This study was carried out to evaluate spontaneous renal regeneration after stopping colistin methanesulfonate (CMS), which induces tubular damage, and the curative effect of Vitamin E (vit E) in rats. Animals were given the following: sterile saline (n = 6), 300,000 IU/kg/ day of CMS (n = 24), or 450,000 IU/kg/day of CMS (n = 24) for seven days. Each CMS group was subdivided into four subgroups (n = 6) and sacrificed as follows: (i) 12 h after stopping CMS, (ii) two weeks after stopping CMS, (iii) two weeks after stopping treatment with vit E, and (iv) two weeks after stopping treatment with olive oil. Subsequently, plasma creatinine (pCr), urine N-acetyl-b-D-glucosaminidase (NAG), renal tissue level of malondialdehyde (MDA), superoxide dismutase (SOD), glutathione reductase (GSH), and renal histology were tested. CMS-induced tubular damage increased the NAG and MDA levels and decreased the SOD and GSH activities. After two weeks of stopping CMS, there was no significant renal recovery. However, treatment with vit E improved tubular regeneration and reduced the biochemical impairments. Two weeks might not be long enough for significant spontaneous renal regeneration. Improvement of renal parameters by vit E could be explained by the reduction of oxidative stress damage.

Ewing's sarcoma of the cranial vault: a case report.

Ewing's sarcoma is a malignant tumor that mainly affects young patients. It represents 10% of primary malignant tumors of the bone and 3% of malignant tumors of the child. Cranial localization is extremely rare representing less than 1% of all the localizations. We report a case of a 10-year-old girl who presented with an intracranial hypertension syndrome with left parietal mass of progressive installation. The X-ray skull showed a lytic lesion with irregular margins involving the left parietal bone. Brain magnetic resonance imaging revealed extensive parietal bone destruction involving both the inner and outer tables. The girl was operated in emergency. Histological examination concluded to Ewing's Sarcoma. The resection was incomplete (R1). The girl received induction's chemotherapy. The cerebral scanner evaluation showed no abnormalities. Then, she received consolidation's chemotherapy with concomitant local radiation therapy. Currently, the girl is in complete remission with a seven-month decline.