Sjögren-Larsson syndrome in two brothers: a case report

Sjögren-Larsson syndrome is a rare autosomal recessive disorder that was originally recognized in the coexistence of congenital ichthyosis, spastic diplegia or quadriplegia and mental retardation. We recently saw two cases with characteristic features of this rare syndrome. Two brothers aged 21 and 25 years presented with triad of congenital ichthyosis, mental retardation and spastic diplegia. Magnetic resonance imaging showed demyelinating disease in one of these cases. Electrodiagnostic studies were normal in all cases

Relieving Pain in Oral Lesions of Pemphigus Vulgaris Using the Non-ablative, Non-thermal, CO2 Laser Therapy (NTCLT): Preliminary Results of a Novel Approach

Introduction: Pemphigus vulgaris (PV) is a chronic, serious autoimmune mucocutaneous bullous disease. Oral lesions in PV may be extremely painful. This pain may adversely affect the patients’ oral intake and quality of life. This before-after clinical trial was designed to assess the pain relieving effects of single session of non-ablative, non-thermal CO2 laser therapy (NTCLT) in oral lesions of PV.Methods: Fifty painful oral lesions of fourteen patients with PV were illuminated by CO2 laser (power: 1 W, scanning the lesions with rapid circular motion of the handpiece) passing through a thick layer of transparent gel with high water content. The pain severity of the oral lesions was reported by the patients up to the fourth postoperative day. They were also asked to continue their existing systemic treatment during the course of this study as a precondition for the participation.Results: The severity of contact and non-stimulate (non-contact) pain declined immediately and significantly after NTCLT (P < 0.001). The pain relieving effect was sustained during the four successive days of follow-up. The procedure was pain free and no kind of analgesics was required. Following NTCLT, there were no visible thermal complications such as destruction, ablation or irritation of the oral lesions.Conclusion: The results of the trial proposed that single session of NTCLT could immediately and significantly relieve pain in oral lesions of PV, without any visible thermal complications

Cognitive impairment and malnutrition, predictors of all-cause mortality in hospitalized elderly subjects with cardiovascular disease

International audienceBackground. - In the elderly, cognitive impairment is associated with loss of independence and may be predictive of mortality. Aims. - Our aim was to determine if cognitive impairment correlated to poor prognosis in an elderly (>70 years) hospitalized population with cardiovascular diseases. Our other goal was to explore other factors that might influence mortality risk. Better understanding of these factors should help practitioners select tools to assess these patients and prevent the occurrence of adverse outcomes. Methods. - During 4 years of follow-up, medical events and all-cause mortality were reported in 331 patients aged above 70 years, as well as clinical and biological variables and Mini Mental State Examination scores. Results. - Patients with cognitive impairment were older and had a lower body mass index than patients without cognitive impairment (P=0.023). When all factors were forced into the Cox model, cognitive impairment remained an independent predictor of mortality (P < 0.001). High plasma glucose, low body mass index and low plasma albumin were associated with overall mortality, independent of cognitive impairment. Conclusion. - In elderly inpatients aged above 70 years with cardiovascular diseases, cognitive impairment and malnutrition are associated, and both are predictors of all-cause mortality. Early nutrition programmes may help to delay mortality, as well as screening the impairment of neuropsychological functioning using the total Mini Mental State Examination score. (C) 2013 Elsevier Masson SAS. All rights reserved

Bulge Cells of Rat Hair Follicles: Isolation, Cultivation, Morphological and Biological Features

Objective: Transplants of multipotent stem cells have been shown to have a neuroprotectiveeffect after central nervous system injury. The bulge region of the hair follicle hasbeen reported as a putative source of hair follicle stem cells (HFSC) for many years;however, few studies have documented the properties of bulge derived cells in vitro untilnow. This study was conducted to isolate and culture bulge cells from rat hair folliclesand to determine the morphological and biological features of the cultured cells.Materials and Methods: The bulge region of the rat whisker was isolated and culturedin Dulbecco's modified eagle medium: nutrient mixture F-12 (DMEM/F12) supplementedwith epidermal growth factor (EGF), cholera toxin. Dissociated bulge stemcells were differentiated on coated substrates together with NT-3. The morphologicaland biological features of cultured bulge cells were observed by light microscopy andimmunocytochemistry methods.Results: Our results showed that newly proliferated cells could be observed on the 4thday after explantation. The expression of a neural progenitor marker, nestin, was seenbefore differentiation of the bulge cells. The differentiated cells expressed βIII-Tubulinand RIP, which are the markers of neural and glial lineages.Conclusion: The results indicated that the bulge cells cultured from the rat hair folliclehad the characteristics of stem cells and could differentiate into neural and glial lineages