Berardinelli Seip Congenital Lipodystrophy Syndrome: 10 Year Follow-up

Lipodystrophy syndromes are extremely rare disorders ofdeficient body fat associated with potentially serious metaboliccomplications. Here, we describe a 10-year-old girl withgenetically proven Berardinelli Seip congenital generalizedlipodystrophy type 2, diagnosed at 10 months of age. Shedeveloped comorbidities like proteinuria, hypertension, diabetesmellitus, and liver fibrosis.

Icaritin delays CCl4-induced hepatic cirrhosis in rats by protecting hepatocytes from oxidative injury / 第二军医大学学报

Objective To investigate the role of icaritin in delaying the progression of liver cirrhotic process in rats and the related mechanism. Methods For invitro study, primary rat hepatocytes were obtained by perfusing the liver of male Wistar rats; the cultured cells we reexposed to the fresh medium containing CCl4, and then treated with various concentrations of icaritin. The activities of alanine transaninase(ALT) and glutamic-oxaloacetic transaminase(AST) inculture medium were measured with an automatic biochemical analyzer. The intracellular contents of malondialdehyde (MDA) and superoxide dismutase(SOD) were determined by spectro-photometry; the apoptotic cells were detected by the TUNEL method. Forin vivostudy, CCl4-induced experimental rat hepatic fibrosis model was established and was treated with icaritin. Serum levels of ALT, AST, albumin(ALB), andglobulin(GLB) were measured; the pathological changes and collagen-expression in livers were observed by HE staining and immunohistochemistry, respectively. Results CCl4 significantly increased the activities of ALT, AST, and the contents of MDA in culture media of hepatocytes, and significantly decreased the SOD activity. More apoptotic cells were observed in CCl4 group than that in icaritin group. The ALT, AST activities in culture supernatant and the intracellular MDA contents in icaritin group were significantly lower than those in both model group and drug carrier group, while intracellular SOD activity was much higher than that in other two groups(P<0.05). Icaritin also reduced the apoptotic ratios of hepatocytes induced by CCl4 compared with model group(P<0.05 or 0.01). In the in vivo experiment, icaritin treatment significantly reduced serum levels of ALT and AST compared with model group(P<0.05) and greatly improved CCl4-induced liver histopathological injuries and collagen I deposition in the liver tissues. Conclusion Icaritin treatment can attenuate CCl4-induced cirrhosis in rats, atleast in part, by protecting the hepatocytes from peroxidation product.

Evaluation of apri test as a liver fibrosis marker / Avaliação do modelo APRI como marcador de fibrose hepática

Objective: to evaluate the performance of APRI test as an indirect marker of liver fibrosis in patients of FSCMPA with chronic viral hepatitis. Methods: laboratorial and histopathological (META VIR) data of 102 patients were collected with the aim of comparing biopsy reports to test results, considering: FO- F 1 as absent/ insignificant fibrosis and F2-F4 as presence of expressive fibrosis; FO-F3 and F4 as absence and presence ofcirrhosis, respectively. The evaluation was based on the use of simultaneous cut-off points suggested by Wai et ar (2003) and also the development of single cut-off points, through ROC curves,for this sample. Results: the specificities found through the original cut-off points were 96% and 87% for significant fibrosis and cirrhosis, respectively. A superior perforinance was detected for the diagnosis of expressive fibrosis (PPV 90%) and for the exclusion ofliver cirrhosis (NPV 95%). The only advantages accomplished with the use ofthe single cutoffpoints definedfor this study were the classification of all patients and an increase insensibility, which do not justify their applicability. Conclusion: APRI test is able to confirm the existence of significant fibrosis and exclude cirrhosis, what makes it available, as an altemative, in clinical practice.

Objetivo: avaliar o desempenho do teste APRI como marcador indireto de fibrose hepática em pacientes portadores de hepatites virais crônicas da FSCMPA. Método: foram avaliados dados laboratoriais e histopatológicos (META VIR) de 102 pacientes afim de comparar os resultados da biópsia com o cálculo do teste. Considerou-se: F0-F1 e F2-F4 como fibrose ausente/inexpressiva e presença de fibrose expressiva, respectivamente; F0-F3 e F4 como ausência e presença de cirrose, respectivamente. Utilizou-se os pontos de corte simultâneos sugeridos por Wai et al. (2003) e desenvolveu-se pontos de corte únicos, através de curvas ROC, para esta amostra. Resultados: as especificidades encontradas com os cortes originais foram, parafibrose significativa e cirrose, 96% e 87%, respectivamente. Evidenciou-se melhor desempenho ao confirmar diagnóstico de fibrose expressiva (VPP 90%) e em excluir cirrose hepática (VPN 95%). As únicas vantagens obtidas com os pontos definidos neste estudo foram a classificação de 100% dos pacientes e aumento da sensibilidade, o que não justifica sua aplicabilidade. Conclusão: o teste APRI é capaz de afirmar a existência de fibrose importante e de excluir o diagnóstico de cirrose, o que viabiliza seu uso, como alternativa, na prática clínica.