RESUMO
Currently, Li is mainly produced through evaporation of Li-rich brines obtained from South American countries such as Bolivia, Chile, and Argentina. The most commonly used process, the lime-soda evaporation, requires a long time and several purification steps, which produces a considerable amount of chemical waste. Various electrochemical methods have been proposed as alternatives, but they use expensive metals such as Ag or Pt, thus rendering these methods economically unacceptable. In this work, we present KNiFe(CN)6 , an abundant and environmentally friendly material, as alternative to these expensive components. The Prussian blue derivate has a higher affinity toward cations (Na(+) or K(+) ) than for Li(+) . Additionally, the use of KNiFe(CN)6 permits the utilization of seawater or brine water as recovery solution, thus reducing the consumption of fresh water, which is typically a scarce element in Li production sites.
Assuntos
Ferrocianetos/química , Lítio/química , Níquel/química , Técnicas Eletroquímicas , Prata/químicaRESUMO
Mitochondrial disorders are human genetic diseases with extremely variable clinical and genetic features. To better define them, we made a genotype-phenotype correlation in a series of 207 affected patients, and we examined most of them with six laboratory examinations (serum CK and basal lactate levels, EMG, cardiac and EEG studies, neuroradiology). We found that, depending on the genetic abnormality, hyperckemia occurs most often with either chronic progressive external ophthalmoplegia (CPEO) and ptosis or with limb weakness. Myopathic EMGs are more common than limb weakness, except in patients with A8344G mutations. Peripheral neuropathy, when present, is always axonal. About 80% of patients with A3243G and A8344G mutations have high basal lactate levels, whereas pure CPEO is never associated with increased lactate levels. Cardiac abnormalities mostly consist of conduction defects. Abnormalities on CT or MRI of the brain are relatively common in A3243G mutations independently of the clinical phenotype. Patients with multiple mtDNA deletions are somehow "protected" against the development of abnormalities with any of the tests. We conclude that, despite the phenotypic heterogeneity of mitochondrial disorders, correlation of clinical features and laboratory findings may give the clinician important clues to the genetic defect, allowing earlier diagnosis and counselling.
Assuntos
DNA Mitocondrial/genética , Deleção de Genes , Doenças Mitocondriais/genética , Doenças Mitocondriais/fisiopatologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Criança , Pré-Escolar , Creatina Quinase/sangue , Eletroencefalografia , Eletromiografia , Feminino , Coração/fisiopatologia , Humanos , Lactente , Ácido Láctico/sangue , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Doenças Mitocondriais/diagnóstico , Músculo Esquelético/patologia , Músculo Esquelético/fisiopatologia , Fenótipo , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
In this study multiple deletions of mitochondrial genome were found in a patient presenting with periodic attacks of paralysis. Morphological studies revealed mitochondrial abnormalities along with typical histopathological features of periodic paralysis. Southern blot and PCR analysis revealed multiple mtDNA deletions. Our patient could be affected by two unrelated diseases, idiopathic periodic paralysis and presymptomatic mitochondrial myopathy. Alternatively, mtDNA alterations and oxidative deficiency might express themselves phenotypically as periodic paralytic attacks, although this correlation has never been reported.
Assuntos
Deficiência de Citocromo-c Oxidase , DNA Mitocondrial/genética , Miopatias Mitocondriais/genética , Proteínas Musculares/deficiência , Paralisias Periódicas Familiares/genética , Deleção de Sequência , Biópsia , Complexo IV da Cadeia de Transporte de Elétrons/genética , Humanos , Hipopotassemia/genética , Masculino , Pessoa de Meia-Idade , Miopatias Mitocondriais/enzimologia , Miopatias Mitocondriais/patologia , Proteínas Musculares/genética , Músculos/enzimologia , Músculos/patologia , Paralisias Periódicas Familiares/enzimologia , Paralisias Periódicas Familiares/patologiaRESUMO
AIM AND METHOD: To verify whether muscle necrosis in critically ill patients could be due to an inflammatory process, we tested muscle biopsies from five intensive care patients with different inflammation-specific immunocytochemical markers (antibodies anti-class I major histocompatibility complex products (class I MHCP or HLA I), membrane attack complex (MAC), T lymphocytes helper-inducer (CD4), cytotoxic (CD8) and pan-B-lymphocytes). RESULTS: In three patients muscle biopsy showed class I MHCP positivity on the surface membrane of several groups of fibres, mainly perifascicular, and scattered microvascular deposits of MAC. In the other two patients muscle biopsy did not show class I MHCP and MAC positivity. CONCLUSION: Our results suggest that inflammation may be a component of muscle damage in some critically ill patients.
Assuntos
Estado Terminal , Inflamação/patologia , Doenças Musculares/patologia , Adulto , Idoso , Biópsia , Feminino , Histocitoquímica , Humanos , Imuno-Histoquímica , Masculino , Microscopia Eletrônica , Pessoa de Meia-Idade , NecroseRESUMO
In situ hybridization combined with immunohistochemical techniques has been applied to study patients affected by mitochondrial myopathies with large mitochondrial (mt)DNA deletions. All patients' muscle biopsies showed ragged red fibers (RRFs) and cytochrome oxidase (COX) deficiency. Two digoxigenin-labeled, polymerase chain reaction (PCR)-amplified DNAs were used as probes. One probe was designed to hybridize only with wild-type mtDNAs, while the other recognized both wild-type and deleted mtDNAs. Concomitant immunocytochemical analysis using antibodies against subunits II, III, (encoded by mtDNA) and IV (encoded by nuclear DNA) of COX was carried out. In our patients deleted mtDNAs are overexpressed in COX-negative RRFs, while wild-type mtDNAs are decreased in the same fibers. Immunohistochemistry studies show that COX IV is overexpressed in RRFs and that COX II and COX III subunits are still present. Deleted mtDNAs are spatially segregated in muscle fibers, where they interfere with the local population of normal mitochondrial genomes, causing a regional deficiency of the mitochondrial respiratory activity.