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Systemic Evaluation (systemic + evaluation)

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Medical Sciences	100%

Selected Abstracts

Nonalcoholic fatty liver disease and nonalcoholic steatohepatitis: Selected practical issues in their evaluation and management,

HEPATOLOGY, Issue 1 2009
Raj Vuppalanchi
Nonalcoholic fatty liver disease (NAFLD) is among the most common causes of chronic liver disease in the western world. It is now recognized that these patients have myriad of important co-morbidities (e.g., diabetes, hypothyroidism and metabolic syndrome). The workup of patients with suspected NAFLD should consist of excluding competing etiologies and systemic evaluation of metabolic comorbidities. NAFLD is histologically categorized into steatosis and steatohepatitis, two states with fairly dichotomous natural history. While significant progress has been made in terms of noninvasively predicting advanced fibrosis, insufficient progress has been made in predicting steatohepatitis. Currently, liver biopsy remains the gold standard for the histological stratification of NAFLD. While sustained weight loss can be effective to treat NASH, it is often difficult to achieve. Foregut bariatric surgery can be quite effective in improving hepatic histology in selected patients without liver failure or significant portal hypertension. Thiazolidinediones have shown promise and the results from the ongoing, large multicenter study should become available soon. Large multicenter studies of CB, receptor anatagonists are also underway but their results will not be available for several years. Several recent studies have highlighted that cardiovascular disease is the single most important cause of morbidity and mortality in this patient population. Conclusion: Health care providers should not only focus on liver disease but also concentrate on aggressively modifying and treating their cardiovascular risk factors. (HEPATOLOGY 2009;49:306-317.) [source]

Gingival involvement of oral lichen planus in a series of 700 patients

JOURNAL OF CLINICAL PERIODONTOLOGY, Issue 10 2005
Michele D. Mignogna
Abstract Background: Oral lichen planus (OLP) is one of the most common oral mucosa disorders. OLP gingival involvement is very frequently observed, and it is characterized by wide variations in clinical appearance and symptoms, leading, in many cases, to misdiagnosis or undiagnosis. This can be potentially harmful since OLP patients require appropriate management in oral and periodontal care, together with an adequate systemic evaluation. Objective: In this paper, we have analysed the prevalence and clinical aspects of gingival lesions in our series of 700 patients affected by OLP. Furthermore, we have discussed the possible periodontal implications on the basis of the available literature. Patients and Methods: Data from 700 patients affected by OLP, clinically and histologically assessed, have been studied; the location and morphology of lesions, the symptoms and the progression of the disease have been considered, with particular attention given to gingival involvement. Results: Gingival lesions have been diagnosed in 48% of cases, usually associated with diffuse oral involvement. Only 7.4% of patients had OLP lesions confined to the gingiva. The morphology of lesions included all the forms originally described for OLP (reticular, papular, plaque, atrophic, erosive and bullous). The symptoms, if present, varied from mild discomfort to severe oral pain, with the general trend increasing from the keratotic to the erosive forms. The gingiva was involved in four out of 21 of our oral cancer cases, which developed from pre-existing OLP lesions. Conclusion: OLP is a very proteiform disorder; considering the high frequency of gingival involvement and its influence on oral health, it is our opinion that periodontologists should be involved in OLP management and should become familiar with its clinical aspects and related themes. [source]

Hunter's syndrome and buphthalmos in a girl: an unusual ophthalmic association

ACTA OPHTHALMOLOGICA, Issue 2009
S SETHI
Purpose To report an unusual ophthalmic presentation of a case of Hunter's syndrome/MPS II. Methods A sixteen-year-old girl presented to us with total loss of vision and forward protrusion OU since early childhood. Detailed examination, including slit lamp biomicroscopy, Intra ocular pressure (IOP) and fundoscopy was carried out. Thorough systemic evaluation including Computed Tomography (CT), metabolic and genetic analysis was undertaken in collaboration with internists. Results Characteristic facies, detection of glycosaminoglycan (GAG) variants in urine (chondroitin sulfate B and heparin sulfate) and iduronate-2-sulphatase activity in fibroblasts/leucocytes confirmed the diagnosis of MPS II. Child had severe photophobia but with no perception of light OU. OU buphthalmos with Haab's striae was noted, making a clear view of the fundus difficult. IOP OU was elevated, and 90D slit lamp biomicroscopy revealed a total glaucomatous optic atrophy in both eyes. On CT there was thickening and edema of preseptal and periorbital soft tissue with marked thinning of the optic nerves with prominent perineural CSF sleeves, indicative of marked optic atrophy. Conclusion Glaucoma is a known association of Hurler's, Scheie's and Maroteaux-Lamy syndromes but not Hunter's. In fact, there is only one report of suspected angle closure glaucoma in MPS II. Buphthalmos is not a likely presentation as the sclera in these patients is known to be thickened due to deposition of GAG. To the best of our knowledge, this is the first case report of buphthalmos in association with MPS II. The importance of a meticulous examination in this subset of patients cannot be overemphasised. An appropriate and timely intervention may result in a better quality of life for them. [source]

Decreased bone density and treatment in patients with autosomal recessive cutis laxa

ACTA PAEDIATRICA, Issue 3 2009
C Noordam
Abstract Aim: Due to the occasional association pathological fractures and osteoporosis we evaluated four patients with cutis laxa syndrome for skeletal anomalies. Patient/Methods: We prospectively evaluated four patients, a male and a female child and a brother-sister sib pair, with dysmorphic features, growth delay, joint anomalies, psychomotor retardation and congenital cutis laxa. The clinical features and the family history were suggestive for autosomal recessive cutis laxa syndrome type II, partially overlapping with geroderma ostedysplastica. Skeletal survey, sequential bone density measurements, endocrine and metabolic investigations were performed including N- and O-linked glycosylation analysis. ATP6V0A2 and FBLN5 mutations were ruled out in all patients. Results: All children were diagnosed with significantly decreased bone density, especially in the lumbar spine, including spontaneous vertebral and rib fractures in three children. Following 24 months of bisphosphonate treatment a total restitution of bone density was observed in three cases and no relapse was detected in the 2-year follow-up period. A spontaneous improvement was found in one female during puberty. Conclusion: Bone disease might occur early in the course in autosomal recessive cutis laxa syndrome. We report on a significant clinical improvement and stabilization in our patients following bisphosphonate therapy. We suggest early, systemic evaluation and follow up of bone density in all children presenting with inherited cutis laxa. [source]