BACKGROUND: Neurofilaments are the major cytoskeletal components of neurons, and cell injury leads to their release into the surrounding area. The aim of this study was to compare the cerebrospinal fluid (CSF) and serum (S) concentrations of neurofilament light chains (NFLs) and phosphorylated neurofilament heavy chains (pNFHs). METHODS: Neurofilament concentrations were measured in CSF and S samples from 172 patients using three enzyme-linked immunosorbent assays. Excel, Stata version 13, MedCal version 17.9.7., and NCSS 2007 software were used for the statistical analysis. RESULTS: There was a statistically significant correlation between the concentrations of CSF NFL and CSF pNFH (rs = 0.748; n = 89; P < 0.001), but Passing-Bablok regression showed systematic deviation between the values obtained using the two assays. This indicates that the assays were not interchangeable. CSF pNFH and S pNFH concentrations showed low correlation. The kappa statistic showed moderate conformity between CSF pNFH and CSF NFL concentrations (κ = 0.556). CONCLUSIONS: The CSF NFL and CSF pNFH assays gave clinically consistent results that reflected the degree of axonal damage, independent of any particular neurological diagnosis. The S pNFH assays had a lower predictive value due to the low correlation coefficient and the kappa index of the CSF pNFH method.
- MeSH
- analýza rozptylu MeSH
- intermediární filamenta metabolismus MeSH
- lidé středního věku MeSH
- lidé MeSH
- nemoci nervového systému krev diagnóza metabolismus MeSH
- neurofilamentové proteiny krev mozkomíšní mok metabolismus MeSH
- regresní analýza MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- srovnávací studie MeSH
The purpose of this work was to investigate the associations of serum cholesterol and apolipoproteins with measures of blood-brain barrier (BBB) permeability and CNS inflammation following the first clinical demyelinating event. This study included 154 patients [67% female; age, 29.5 ± 8.2 years (mean ± SD)] enrolled in a multi-center study of interferon β1-a treatment following the first demyelinating event. Blood and cerebrospinal fluid (CSF) were obtained at screening prior to treatment. A comprehensive serum lipid profile and multiple surrogate markers of BBB breakdown and CNS immune activity were obtained. Higher levels of serum HDL cholesterol (HDL-C) and ApoA-I were associated with lower CSF total protein level, CSF albumin level, albumin quotient, and CSF IgG level (all P ≤ 0.001 for HDL-C and all P < 0.01 for ApoA-I). HDL-C was also associated with CSF CD80+ (P < 0.001) and with CSF CD80+CD19+ (P = 0.007) cell frequencies. Higher serum HDL is associated with lower levels of BBB injury and decreased CD80+ and CD80+CD19+ cell extravasation into the CSF. HDL may potentially inhibit the initiation and/or maintenance of pathogenic BBB injury following the first demyelinating event.
- MeSH
- apolipoproteiny krev MeSH
- biologické markery krev MeSH
- demyelinizační nemoci MeSH
- dospělí MeSH
- HDL-cholesterol krev MeSH
- hematoencefalická bariéra účinky léků metabolismus patologie MeSH
- interferon beta terapeutické užití MeSH
- lidé MeSH
- longitudinální studie MeSH
- nemoci nervového systému krev MeSH
- roztroušená skleróza krev farmakoterapie patologie MeSH
- zánět krev MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- pozorovací studie MeSH
- práce podpořená grantem MeSH
BACKGROUND & AIMS: Wilson disease (WD) is an inherited disorder of copper disposition caused by an ATP7B transporter gene mutation, leading to copper accumulation in predisposed tissues. In addition to a genetic predisposition, other factors are likely to contribute to its clinical manifestation. The aim of the study was to assess whether oxidative stress affects the phenotypic manifestation of WD. METHODS: In 56 patients with WD (29 men; 26 with the hepatic form, 22 with the neurologic form, and eight asymptomatic; mean age 38.5 ± 12 years), total serum antioxidant capacity (TAC) and inflammatory parameters (hs-CRP, IL-1β, IL-2, IL-6, IL-10, and TNF-α) were analyzed and related to the clinical manifestation, and mutations of the ATP7B gene. The control group for the TAC and inflammatory parameters consisted of 50 age- and gender-matched healthy individuals. RESULTS: WD patients had a significantly lower TAC (p < 0.00001), lower IL-10 levels (p = 0.039), as well as both higher IL-1β (p = 0.019) and IL-6 (p = 0.005) levels compared to the control subjects. TNF-α, hs-CRP, and IL-2 did not differ from the controls. Patients with the neurological form of WD had a significantly lower TAC than those with the hepatic form (p < 0.001). In addition, the lower TAC was associated with the severity of the neurological symptoms (p = 0.02). No relationship between the inflammatory parameters and clinical symptoms was found. CONCLUSIONS: Data from our study suggest that the increased oxidative stress contributes significantly to the clinical manifestation of WD; as a lower TAC is associated with the neurological symptoms in WD patients.
- MeSH
- adenosintrifosfatasy genetika MeSH
- antioxidancia metabolismus MeSH
- dospělí MeSH
- genetická predispozice k nemoci MeSH
- hepatolentikulární degenerace krev MeSH
- interleukin-10 metabolismus MeSH
- interleukin-1beta metabolismus MeSH
- interleukin-6 metabolismus MeSH
- lidé středního věku MeSH
- lidé MeSH
- měď metabolismus MeSH
- mutace MeSH
- nemoci nervového systému krev MeSH
- oxidační stres MeSH
- proteiny přenášející kationty genetika MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
Autoantibodies against different axonal cytoskeletal proteins [the light (NFL) and medium (NFM) subunit of neurofilament and tubulin (TUB)] in serum and cerebrospinal fluid may be generated in response to the release of cytoskeleton from damaged neurons. We studied the relationships among these autoantibodies. Paired cerebrospinal fluid (CSF) and serum samples were obtained from 47 multiple sclerosis (MS) patients, 14 patients with neurodegenerative diseases, 21 patients with various neurological diseases and 16 normal control subjects. Levels of antibodies against NFL, NFM and TUB were related to each other in CSF in all groups, whereas close association of anti-cytoskeletal antibodies in serum was found in the MS group only. A concordant spectrum of anti-cytoskeletal antibodies is present in serum of MS patients, unlike in other neurological patients. The synergy between the spectrum of anti-cytoskeletal antibodies in serum and CSF might be one of the immunological features typical for the MS patients.
- MeSH
- axony metabolismus MeSH
- cytoskeletální proteiny imunologie krev MeSH
- dospělí MeSH
- financování organizované MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- nemoci nervového systému imunologie krev MeSH
- neurodegenerativní nemoci imunologie krev MeSH
- protilátky imunologie MeSH
- roztroušená skleróza imunologie krev MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
Terapeutické hemaferézy jsou léčebné výkony, jejich cílem je redukce obsahu patogenní substance nebo buněčných elementů v cirkulaci pacienta, přičemž odebrané složky se nahrazují roztoky krystaloidů, koloidů, plazmou nebo buněčnými elementy od zdravého dárce. Léčebné techniky jsou rozděleny do skupin deplečních metod, které jsou zastoupeny leukocytaferézami, lymfocytaferézami, erytrocytaferézami a deplecí trombocytů. Z výměnných výkonů jsme se zabývali problematikou výměnné plazmaferézy a výměnné erytrocytaferézy. Dále uvádíme zkušenosti s přípravou koncentrátu lymfocytů (DLI) pro navození graft-versus leukemia efektu (GVL), s metodou extrakorporální fotochemoterapie (ECP), s problematikou pediatrických hemaferéz a se separacemi hemopoetických progenitorových buněk z periferní krve (PBPC), které jsme prováděli ve standardním a ve velkoobjemovém režimu (LVL). LVL separace umožnily přípravu vyššího počtu CD 34+ buněk ve srovnání se standardními výkony. Vyšší dávky progenitorových buněk jsme získali jak u pacientů s dobrým efektem mobilizace, tak u nedostatečné mobilizovaných nemocných. U pacientů s dobrým efektem mobilizace postačilo provedení jedné LVL separace k přípravě dávky CD 34+ buněk vyšší než 5xl0 /kg. LVL výkony můžeme doporučit ve všech situacích, kdy je pacient nebo dárce schopen je tolerovat. Zvláště významnou úlohu představují tyto výkony u pacientů s nedostatečným efektem mobilizace, u nichž jsou jedinou možností jak připravit alespoň minimální dávku progenitorových buněk pro transplantaci a pro ovlivnění průběhu základního onemocnění.
Therapeutic haemaphereses are therapeutic procedures the objective of which is to reduce the contents of a pathogenic substance or cellular elements in the patient's circulation whereby the removed constituents are replaced by solutions of crystalloids, colloids, plasma or cellular elements from a healthy donor. The therapeutic techniques are divided into groups of depletion methods which are represented by leukaphereses, lymphocytaphereses, erythrocytaphereses and thrombocyte depletion. As to exchange procedures, the authors were concerned with the problem of exchange plasmapheresis and exchange erythrocytapheresis. They present also experience with the preparation of a lymphocyte concentrate (DLI) to induce the graft-versus leukaemia effect (GVL), the method of extracorporeal photochemotherapy (ECP), the problem of paediatric haemaphereses and separations of haemopoietic progenitor cells from the peripheral blood stream (PBPC) which were implemented by a standard and large volume (LVL) regime. LVL preparations made it possible to prepare a larger number of CD34+ cells as compared with standard operations. Higher amounts of progenitor cells were obtained from patients with a good effect of mobilization as well as in inadequately mobilized patients. In patients with a good effect^of mobilization one LVL separation was sufficient to prepare a CD34+ dose of cells of more than 5x10 /kg. LVL procedures can be recommended under all circumstances when the patient or donor can tolerate them. A particularly important part is played by these procedures in patients with an inadequate effect of mobilization where they are the only possibility how to prepare at least a minimal amount of progenitor cells for transplantation and for influencing the course of the basic disease.
- MeSH
- krevní nemoci krev terapie MeSH
- lidé MeSH
- metabolické nemoci krev terapie MeSH
- náhražky plazmy MeSH
- nemoci nervového systému krev terapie MeSH
- převod jednotlivých krevních složek MeSH
- separace krevních složek metody normy přístrojové vybavení MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
- MeSH
- beta-2-mikroglobulin krev MeSH
- lidé MeSH
- mozkomíšní mok chemie MeSH
- nemoci nervového systému krev MeSH
- Check Tag
- lidé MeSH
- MeSH
- apolipoprotein A-I krev MeSH
- imunoglobuliny krev MeSH
- lidé MeSH
- makrofágy MeSH
- mozkomíšní mok cytologie chemie MeSH
- nemoci nervového systému krev MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- srovnávací studie MeSH
