ABSTRACT
The 2030 Immunization Agenda of the World Health Organization （WHO） states that everyone in the world should fully benefit from vaccines to achieve good health and well-being. With the ever-changing disease spectrum and the improvement of residents' health literacy， relying solely on vaccines included in the National Immunization Program （NIP） is insufficient to meet the current requirements for disease prevention and control. Non-NIP vaccines play an important role in meeting people's diverse needs. Vaccine hesitancy is a global issue and an important factor affecting vaccine uptake. By reviewing relevant studies on vaccine hesitancy in recent years， this paper summarized different vaccination situations， current situation of vaccine hesitancy， measuring tools of vaccine hesitancy， and major influencing factors. It aims to provide references for the development of scientific and effective vaccine education strategies， which can increase public knowledge and understanding of vaccines， enhance healthcare professional's willingness and behavior in recommending vaccines， improve public vaccine literacy， and reduce vaccine hesitancy. At the same time， the supervision and guidance of media discourse should be strengthened to enhance the protective role of non-NIP vaccines in immunization barriers.
ABSTRACT
Spinal and bulbar muscular atrophy (SBMA) is a rare X-linked motor neuron disease with significant phenotypic viability. Here, we present a genetically identified SBMA family without bulbar paralysis or androgen insensitivity. All four male patients presented with progressive lower motor neuron paralysis in all limbs, with distal extremities more dominant. None of them had bulbar palsy or androgen insensitivity. A consistently mild elevated blood creatine phosphokinase (CPK) levels were detected in all patients and the EMG showed a chronic neurogenic damage. Muscle biopsy of propositus indicated a typical neurogenic amyotrophy. Genetic testing for SMA of mutation in SMN1 was negative, while for SBMA of androgen receptor showed the increased CAG repeat in exon 1, suggesting that although bulbar symptoms and androgen insensitivity are characteristic symptoms of SBMA, they are not obligatory for the diagnosis. In adult males with a chronic motor neuron syndrome without upper motor neuron signs, even in absence of the classical features of androgen insensitivity or bulbar findings, genetic testing for SBMA should be strongly considered.
Subject(s)
Adult , Humans , Male , Bulbo-Spinal Atrophy, X-Linked , Diagnosis , Genetics , Creatine Kinase , Blood , Genetic Testing , Motor Neurons , Pathology , Muscular Atrophy , Mutation , Genetics , Paralysis , Diagnosis , Pedigree , Receptors, Androgen , GeneticsABSTRACT
Objective To analyze the clinical and pathological features of limb-girdle muscular dystrophy2B(LGMD2B).Methods The clinical and pathological features of 7 patients with LGMD2B were analyzed retrospectively.Results Seven patients had a slow onset,and progressive proximal muscle weakness,muscle atrophy,progressive,and incresed serum creatine phosphokinase; muscle biopsy showed different degree of muscle fiber degeneration,necrosis; stromal and inflammatory cell infiltration in muscle fiber; monoclonal antibody immunohistoehemical staining:showed expression of Dysferlin protein was not found in muscle cell membrane,Dystrophin,Sarcoglycans protein expression was normal.Monoclonal antibody immunohistochemical staining the proteins were expressed in normal cell membrane.Conclusions LGMD2B is a slow onset,progressive proximal muscle weakness,muscle atrophy.Histochemical staining on the basis of further immunohistochemical staining,to detect the membrane protein and Dysferlin protein expression,that is a necessary means to diagnose LGMD2B and inflammatory myopathies.
ABSTRACT
@#Objective To investigate the clinical, electrophysiological and magnetic resonance imaging (MRI) characteristics of Hirayama disease. Methods 12 cases with Hirayama Disease were analyzed retrospectively. Results and Conclusion All the patients were male, onset at adolescence, with muscle weakness and atrophy on one or two upper limbs, but without sensory disorder or pyramidal sign. The EMG showed neurogenic abnormalities in the areas dominated by C7-T1 nerve in all the patients. Cervical cord MRI found slight atrophy of lower cervical cord in 3 cases in routine position，compressed and displacement forward in flexion position in 12 cases，with posterior epidural space widening，lunular or striped high signal and voids of vessel in some patients. Enhancement scanning in 4 cases showed lunular sign enhanced. Biceps muscles biopsies were performed in 3 cases, the pathology Results appear neuropathic change in 1 cases, another 2 cases were normal.