Cíl: Cílem této práce bylo zjistit distribuci a rozsah péče o děti s podezřením na získaný demyelinizační syndrom (zahrnující akutní diseminovanou encefalomyelitidu, klinicky izolovaný syndrom, roztroušenou sklerózu a okruh neuromyelitis optica) a identifikovat oblasti ke zkvalitnění péče v ČR. Soubor a metodika: Elektronické dotazníkové šetření na všech lůžkových pracovištích dětské neurologie (n = 7) a pediatrie (n = 22) na úrovni krajů a fakultních nemocnic. Odpovědi jsme získali ze všech oslovených nemocnic. Výsledky: Na péči se podílí všech sedm pracovišť dětské neurologie a deset pediatrií. Všichni disponují potřebnými diagnostickými možnostmi, alw terapeutické možnosti se liší. Identifikovali jsme konkrétní rozdíly v diagnostickém přístupu – v indikaci a interpretaci vyšetření a ve využití aktuálních diagnostických kritérií. V šesti krajích je péče primárně směřována na lůžka dětské neurologie, v ostatních osmi krajích na lůžka pediatrická s konziliárním dětským neurologem. Složitější případy jsou překládány na dětské neurologie, kde je diagnostický přístup komplexnější a terapeutické možnosti rozsáhlejší. Závěr: Péče o děti se získaným demyelinizačním syndromem je v ČR na vysoké úrovni, ale není standardizována. Nejlépe zajištěná onemocnění jsou RS a okruh neuromyelitis optica, pro která fungují centra vysoce specializované péče. Vzhledem k distribuci péče musí specializovaná edukace cílit i na pediatry a konziliární dětské neurology.
Aim: The aim of this study was to investigate the distribution and extent of healthcare provided to children with a suspected acquired demyelinating syndrome (including acute disseminated encephalomyelitis, clinically isolated syndrome, multiple sclerosis and neuromyelitis optica spectrum disorder) and to identify areas for care improvement in the Czech Republic. Patients and methodology: Electronic questionnaire survey at all inpatient departments of pediatric neurology (N = 7) and pediatric departments (N = 22) at the regional and university hospital level. Responses were obtained from all contacted hospitals. Results: All inpatient departments of pediatric neurology and 10 pediatric departments are involved in the care of patients. All have the necessary diagnostic methods available, but therapeutic options differ. We identified specific differences in the diagnostic approach -in the indication and interpretation of examinations and in the use of current diagnostic criteria. In six regions, care is provided primarily by departments of pediatric neurology. In the remaining eight regions, patients are admitted to pediatric departments with an available pediatric neurology consultant. More complex cases are transferred to pediatric neurology departments, where the diagnostic approach is more comprehensive and the therapeutic options are more extensive. Conclusion: In the Czech Republic, healthcare for children with an acquired demyelinating syndrome is at a high level, but it is not standardized. The most appropriate standard of care is provided in specialized care centers for multiple sclerosis and neuromyelitis optica spectrum disorders. Given the distribution of care, specialized education must also include pediatricians and pediatric neurology consultants.
- MeSH
- demyelinizační nemoci * terapie MeSH
- dítě MeSH
- lidé MeSH
- management nemoci MeSH
- management péče o pacienta * organizace a řízení MeSH
- neuromyelitis optica terapie MeSH
- průzkumy a dotazníky MeSH
- roztroušená skleróza terapie MeSH
- terciární prevence organizace a řízení MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- práce podpořená grantem MeSH
BACKGROUND: Inflammatory activity in children with paediatric onset multiple sclerosis (POMS) is higher than that in adults with MS. Chemokine/cytokine profiling in children may provide new insights into the disease pathogenesis and clinical course. The levels of chemokines/cytokines and their roles in POMS remain largely unknown. OBJECTIVE: To identify the possible utility of chemokines/cytokines in children with POMS, we analysed their levels at the time of disease diagnosis and in the context of subsequent clinical relapse. METHODS: CC and CXC motif ligand chemokines (CCL2, CXCL8, CXCL10, and CXCL13), interleukin (IL)-4, IL-17A, interferon gamma and B cell-activating factor in the blood and cerebrospinal fluid (CSF) of 34 POMS patients and 20 age-related controls were measured using Luminex multiplex bead and enzyme-linked immunosorbent assay techniques. Nonparametric tests were used for statistical analyses. RESULTS: The CSF levels of CXCL8 (p = 0.002), CXCL10 (p = 0.001), and CXCL13 (p<0.0001) were higher in POMS than in controls; CXCL10 and CXCL13 correlated with pleocytosis and oligoclonal bands. A subsequent clinical relapse occurred in 17/34 of the children; the median time from the diagnosis of POMS was 6 months (range, 2-64 months). The follow-up period of patients who did not experience a clinical relapse was significantly longer than the time to first relapse (p = 0.003). The initial CCL2 level was lower in relapsing than in non-relapsing patients (p = 0.063) and correlated negatively with the CSF/serum albumin ratio and positively with the time to relapse (p<0.04). CONCLUSIONS: Elevated CSF levels of CXL10 and CXCL13 in children with POMS at the time of disease diagnosis reflect inflammatory activity and suggest the involvement of adaptive immunity; elevated CXCL8 levels further indicate the involvement of innate immunity. An initial low CSF level of CCL2 may be associated with an unfavourable early MS course.
