In recent years the association between severe pregnancy complications and fetal fatty acid oxidation (FAO) disorders has been reported. However, there are few descriptions of a maternal FAO disorder leading to these complications. We describe acute liver failure associated with an undiagnosed maternal medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. The previously healthy proband presented at the 39th week with an itchy rash, palmar erythema and trace proteinuria; she was admitted onto a maternity ward. Acute fatty liver was suspected from the blood tests and a Caesarean section was performed, delivering a healthy boy. Cord blood samples were taken at delivery as part of an ongoing research project. The analysis of the cord blood sample showed a high concentration of octanoylcarnitine of 2.3 micromol/L (reference <0.1), suggesting a possible fatty acid oxidation disorder. However, subsequent acylcarnitine analyses of the baby's blood showed a normal pattern. The proband was further evaluated by urine organic acids and acylcarnitine profile. Elevated concentrations of hexanoylglycine in urine and octanoylcarnitine in blood spots were found, consistent with a diagnosis of MCAD deficiency. Mutation analyses confirmed that she was homozygous for c.985A>G (K329E). Even though these pregnancy complications are rare and it is not possible to affirm that the proband's acute liver failure was secondary to an undiagnosed MCAD deficiency, it seems likely.
-Background -Although gastric cancer has been decreasing in incidence in many countries, it is still the second most common cause of cancer deaths worldwide. Its prognosis is poor and depends, among other factors, on early diagnosis as well as on surgeon expertise. Aim -To compare the outcomes of gastric cancer patients treated at a university hospital by a general surgical team and later on by a gastric cancer surgical team. Methods -Gastric cancer patients were separated into two groups according to whether they were treated by a general surgical team (group 1, n = 136; 1984 to 1993) or by gastric cancer team (group 2, n = 149; 1994 to 2003). Clinical and pathologic features and survival rates were assessed. Results -During a 20-year period, a decreased number of patients underwent surgical resection in the second period (94% vs 86%), a greater number of upper gastrointestinal endoscopies were performed resulting in an increased number of tumors diagnosed as stage I (5% vs 22%). Also, D2 gastrectomies were more frequently performed instead of D0 gastrectomies and negative surgical margins were adequate. Mortality decreased from 9% to 6% in group 1 and 2, respectively and adjuvant therapy has been considered. Conclusion -Surgical specialized units for gastric cancer are necessary if better results are to be expected since this approach definitely provides better patient care.
INTRODUÇÃO: Embora decrescendo nos países do chamado primeiro mundo, o adenocarcinoma gástrico mantém-se como terceiro tumor mais frequente no sexo masculino mundialmente. Sua mortalidade é muito elevada, fruto do diagnóstico tardio em lesões muito avançadas, o que frequentemente torna paliativo seu tratamento, motivos pelos quais se justificam estudos no sentido de melhorar estes resultados. MÉTODO: Revisão da literatura através do portal de periódicos da CAPES indicados por busca no sites da Bireme e PubMed. Além disso, foram consultados os sumários do 8º Congresso Internacional de Câncer Gástrico em 2009. Foi apresentada uma sugestão de algoritmo de atendimento destes pacientes. CONCLUSÕES: O surgimento de novas drogas anticancer, mais efetivas, está propiciando novas alternativas para a ressecção gástrica como tratamento paliativo. Novos protocolos estão surgindo mostrando boas perspectivas para melhorar os resultados desta doença.
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