Nephrogenic fibrosing dermopathy (NFD) is a rare and recently recognized sclerosing skin disorder of unknown etiology. Reported cases have occurred in patients with chronic renal failure, with or without renal replacement therapy. All previous cases have been reported in older adult patients. We describe two pediatric patients who recently developed this condition and review the existing literature for NFD. Our patients included an 8-year-old boy on peritoneal dialysis with no prior renal transplant and a 19-year-old boy on hemodialysis with a history of previous failed renal transplants. We speculate that the recent emergence of this condition and occurrence in patients with chronic renal failure suggest an association with some newer pharmacological agent that has recently come into wide use. Since both our patients also had previously experienced large vessel thrombosis, hypercoagulable states may also be implicated.
Background: Reduced blood coagulability seems to protect against inflammatory bowel disease; pilot studies using heparin in patients with inflammatory bowel disease have reported positive results. Aim: To evaluate the effects of heparin treatment on microangiographic and on inflammatory parameters in experimental colitis, induced by trinitrobenzene sulphonic acid (TNBS)‐ethanol. Methods: Four groups of rats: (i) controls (saline enema), TNBS‐induced colitis with (ii) sham treatment (saline, s.c.), (iii) dexamethasone (0.25 mg/kg/day s.c.) and (iv) heparin (500 U/kg t.d.s., s.c.). Microangiography was performed 2 and 4 days after colitis induction. Partial thromboplastin time, colonic wet weight, macroscopic damage score and mucosal myeloperoxidase (MPO) activity were determined at day 4. Results: TNBS‐induced colitis caused a reduction in visible bowel wall vessels, which was prevented by heparin (P < 0.05) but not by steroids. The macroscopic damage scores and colon wet weights were similar in all colitis groups. Compared to untreated colitis the MPO activity in heparin‐treated animals was of borderline significance. Conclusions: Heparin treatment improved microangiographic features and reduced inflammation to a certain degree. Steroids delayed development of colon hypoperfusion, but were ineffective on MPO activity. It remains to be determined if the observed effects are due to the antithrombotic activity of heparin or to an anti‐inflammatory action.
A 56-year-old Caucasian female with end-stage liver disease secondary to ␣1-antitrypsin deficiency and end-stage renal disease secondary to idiopathic membranous nephropathy presented with a one-week history of increased bilateral lower extremity edema and anasarca. On admission, the patient complained of raised hardened areas especially prominent on the upper medial thighs that were painful, warm, and nonpruritic. She denied any constitutional symptoms and denied using any new medications. On exam, the patient was found to be afebrile with stable oxygen saturation. The abdomen was distended with evidence of hepatomegaly. Bilateral lower extremity edema to the groin were present, and hyperpigmented plaques ( Fig. 1) (tender, indurated, and warm) were noted on the upper medial aspects of her thighs, but not involving the groin. There was no visible face or upper extremity involvement.Laboratory tests did not reveal leukocytosis or eosinophilia. ESR was 40 and ANA test was negative. Serum protein electrophoresis was negative for paraproteinemia. Doppler ultrasound did not reveal deep venous thromboses. The patient received a short course of antibiotics without any clinical improvement. A skin punch biopsy revealed increased dermal fibroblast proliferation, thick collagen bundles with surrounding clefts, elastic fibers, and increased mucin deposition consistent with NFD ( Fig. 2 and 3). The patient declined any steroid treatment and was subsequently discharged.Eventually the patient underwent liver transplant and received oral prednisone as part of the immunosuppression regimen, which has led to some improvement of the cutaneous lesions. Currently she has extensive skin contraction involving her calves and shins with decreased range of motion, and she has become largely wheelchair bound. Nephrogenic Fibrosing Dermopathy (NFD) is a recently described derma- From the
Lignin is a fascinating aromatic biopolymer with high valorization potentiality. Besides its extensive value in the biorefinery context, as a renewable source of aromatics lignin is currently under evaluation for its huge potential in biomedical applications. Besides the specific antioxidant and antimicrobial activities of lignin, that depend on its source and isolation procedure, remarkable progress has been made, over the last five years, in the isolation, functionalization and modification of lignin and lignin-derived compounds to use as carriers for biologically active substances. The aim of this review is to summarize the current state of the art in the field of lignin-based carrier systems, highlighting the most important results. Furthermore, the possibilities and constraints related to the physico–chemical properties of the lignin source will be reviewed herein as well as the modifications and processing required to make lignin suitable for the loading and release of active compounds.
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