1.Research progress of obesity and multiple sclerosis
Jiaxin MA ; Mingfang ZHU ; Xiaodi HAO ; Lihua YANG ; Yakun ZHANG ; Qi ZHOU ; Yuan XUE ; Jiewen ZHANG ; Yue HUANG
Chinese Journal of Neurology 2024;57(8):922-927
Sedentary bad habits and unhealthy diets in modern lifestyles have led to an upward trend in the incidence of obesity, and a series of diseases related to obesity have also gradually received attention. Multiple sclerosis is a chronic inflammatory disease of the central nervous system, and obesity has a common inflammatory component with most chronic diseases. Therefore, this paper reviews the research progress on the relationship between obesity and multiple sclerosis in order to better understand the role of obesity in the management of multiple sclerosis.
2.Analysis of changes in bacterial drug resistance and treatment of Salmonella typhimurium enteritis in children
Jiewen ZHOU ; Bingnan MA ; Jingyi OU
Tianjin Medical Journal 2024;52(12):1282-1285
Objective To summarize changes in antibiotic resistance of Salmonella typhimurium enteritis in children in our hospital and analyze its treatment.Methods A total of 186 children with Salmonella typhimurium enteritis in the pediatrics department of our hospital were selected.The drug resistance of common antibiotics against Salmonella typhimurium for children was analyzed,and the annual and pre-/post-treatment changes of multi-drug resistant bacteria were also analyzed.Based on the initial treatment of antibiotics and the first drug sensitivity test results,patients were divided into the initial sensitive group(121 cases)and the initial insensitive group(65 cases).The hospital stay and fecal culture negative conversion time were compared between the two groups of children.Results Among the Salmonella typhimurium isolated from 186 children,the resistance rate of Salmonella typhimurium to commonly used cephalosporin antibiotics in pediatrics demonstrated an overall upward tendency.Among them,the resistance rates of ceftriaxone(43.6%)and cefotaxime(35.5%)were relatively high.From 2017 to 2019,the proportion of multi-drug resistant bacteria presented a gradual upward trend,while from 2020 to 2023,the proportion declined.The incidence of multi-drug resistant bacteria exhibited an increasing trend after treatment(P<0.05).After 1 week of treatment,there were no significant differences in the above three types of drug resistance changes.The hospital stay and the time for fecal culture to turn negative were both shorter in the initial sensitive group than those in the initial insensitive group(P<0.05).Conclusion The problem of bacterial resistance in Salmonella typhimurium enteritis in children is severe.Initial empirical treatment with sensitive antibiotics and a reasonable course of anti-infective treatment can help reduce the bacterial drug resistance rate and shorten the hospital stay.
3.Microbiological surveillance result of endoscopes after INTERCEPT Foam Spray: a quasi-experimental pilot study in Singapore
Caihong WANG ; Rong ZHANG ; Ruhui FAN ; Jiewen LOW ; Ruochen DU ; Xueyun MA ; Congcong CAI
Clinical Endoscopy 2024;57(6):821-831
Background/Aims:
This study aimed to assess the impact of INTERCEPT Foam Spray (IFS) application on delayed endoscope reprocessing through microbiological surveillance culture (MSC).
Methods:
A quasi-experimental, matched-comparison pilot study was conducted using gastrointestinal endoscopy. IFS was applied to the endoscopes after precleaning and before reprocessing the next day. An equal number of endoscopes, matched by endoscope type, were subjected to routine reprocessing. The MSC were subjected to high-level disinfection to detect any contamination. Data were analyzed using the chi-square test or Fisher exact test (categorical data) and Student t-test (continuous data).
Results:
In total, 150 MSCs were collected from 42 endoscopes. Positive MSCs were observed in 4.0% (4/75) of the sprayed group and 1.3% (1/75) of the control group (95% confidence interval, 30.34–0.31; p>0.05), all of which were contributed by colonoscopes. Colonoscope were more prone to positive MSC (mean difference in percentage, p<0.05). Mean spraying hours were not associated with detected growth (11.7% vs. 13.6%; 95% confidence interval, 1.43 to –5.27; p>0.05), with environmental and skin flora being the primary contaminants.
Conclusions
IFS may be applied when delayed endoscope processing is necessary, but with caution when applied to colonoscopes. However, further research is warranted to verify the result.
4.Microbiological surveillance result of endoscopes after INTERCEPT Foam Spray: a quasi-experimental pilot study in Singapore
Caihong WANG ; Rong ZHANG ; Ruhui FAN ; Jiewen LOW ; Ruochen DU ; Xueyun MA ; Congcong CAI
Clinical Endoscopy 2024;57(6):821-831
Background/Aims:
This study aimed to assess the impact of INTERCEPT Foam Spray (IFS) application on delayed endoscope reprocessing through microbiological surveillance culture (MSC).
Methods:
A quasi-experimental, matched-comparison pilot study was conducted using gastrointestinal endoscopy. IFS was applied to the endoscopes after precleaning and before reprocessing the next day. An equal number of endoscopes, matched by endoscope type, were subjected to routine reprocessing. The MSC were subjected to high-level disinfection to detect any contamination. Data were analyzed using the chi-square test or Fisher exact test (categorical data) and Student t-test (continuous data).
Results:
In total, 150 MSCs were collected from 42 endoscopes. Positive MSCs were observed in 4.0% (4/75) of the sprayed group and 1.3% (1/75) of the control group (95% confidence interval, 30.34–0.31; p>0.05), all of which were contributed by colonoscopes. Colonoscope were more prone to positive MSC (mean difference in percentage, p<0.05). Mean spraying hours were not associated with detected growth (11.7% vs. 13.6%; 95% confidence interval, 1.43 to –5.27; p>0.05), with environmental and skin flora being the primary contaminants.
Conclusions
IFS may be applied when delayed endoscope processing is necessary, but with caution when applied to colonoscopes. However, further research is warranted to verify the result.
5.Microbiological surveillance result of endoscopes after INTERCEPT Foam Spray: a quasi-experimental pilot study in Singapore
Caihong WANG ; Rong ZHANG ; Ruhui FAN ; Jiewen LOW ; Ruochen DU ; Xueyun MA ; Congcong CAI
Clinical Endoscopy 2024;57(6):821-831
Background/Aims:
This study aimed to assess the impact of INTERCEPT Foam Spray (IFS) application on delayed endoscope reprocessing through microbiological surveillance culture (MSC).
Methods:
A quasi-experimental, matched-comparison pilot study was conducted using gastrointestinal endoscopy. IFS was applied to the endoscopes after precleaning and before reprocessing the next day. An equal number of endoscopes, matched by endoscope type, were subjected to routine reprocessing. The MSC were subjected to high-level disinfection to detect any contamination. Data were analyzed using the chi-square test or Fisher exact test (categorical data) and Student t-test (continuous data).
Results:
In total, 150 MSCs were collected from 42 endoscopes. Positive MSCs were observed in 4.0% (4/75) of the sprayed group and 1.3% (1/75) of the control group (95% confidence interval, 30.34–0.31; p>0.05), all of which were contributed by colonoscopes. Colonoscope were more prone to positive MSC (mean difference in percentage, p<0.05). Mean spraying hours were not associated with detected growth (11.7% vs. 13.6%; 95% confidence interval, 1.43 to –5.27; p>0.05), with environmental and skin flora being the primary contaminants.
Conclusions
IFS may be applied when delayed endoscope processing is necessary, but with caution when applied to colonoscopes. However, further research is warranted to verify the result.
6.Analysis of the clinical, pathological and genetic features of patients with myopathy-type very long chain acyl-coenzyme A dehydrogenase deficiency
Mi PANG ; Jun FU ; Jia SONG ; Gang LI ; Yan LU ; Jiewen ZHANG ; Mingming MA
Chinese Journal of Neurology 2023;56(2):143-150
Objective:To investigate the clinical, pathological and genetic characteristics of myopathy-type very long chain acyl-coenzyme A dehydrogenase deficiency (VLCADD).Methods:The detailed clinical data, muscle biopsy pathology and molecular results of 4 patients with genetically confirmed myopathy-type VLCADD admitted to Henan Provincial People′s Hospital and Xuanwu Hospital, Capital Medical University from June 2014 to November 2019 were retrospectively analyzed.Results:All of the 4 patients were late-onset myopathy-type VLCADD. The onset age ranged from 13 to 16 years, with a mean age of 14.5 years. The age at diagnosis ranged from 21 to 54 years, with a mean age of 42.5 years. The main clinical manifestation was repeated rhabdomyolysis, including myalgia, weakness and dark urine. Obvious somnolence was observerd in 1 patient. Muscle biopsy pathology revealed mild lipid accumulation, without vacuoles. Six ACADVL variations were detected in the 4 patients, including c.1283G>A (p.R428H), c.1532G>A (p.R511Q), c.833_835delAGA (p.K278del), c.1843C>T (p.R615 *), c.1748C>T (p.S583L) and c.1391C>T (p.T464I),among which c.1391C>T (p.T464I) was a novel variation, predicted to be likely pathogenic. Other 5 variations were reported pathogenic variations. Conclusions:Myopathy-type VLCADD is characterized by paroxysmal rhabdomyolysis and can be associated with somnolence. There is no specificity in muscle pathology. There are ACADVL variations, among which c.1391C>T is a novel variation.
7.Muscle MRI features of the lower extremities in patients with limb girdle muscular dystrophy type R1
Jia SONG ; Gang LI ; Mi PANG ; Li GAO ; Jiewen ZHANG ; Mingming MA
Chinese Journal of Neuromedicine 2023;22(4):355-360
Objective:To investigate the muscle MRI features of the lower extremities and correlations between MRI fatty degeneration total scores and other clinical features in limb girdle muscular dystrophy type R1 (LGMDR1) patients.Methods:Clinical data of 8 patients with LGMDR1 diagnosed by genetic examination in Department of Neurology, He'nan Provincial People's Hospital&People's Hospital of Zhengzhou University from May 2016 to November 2021 were retrospectively analyzed. Disease severity was evaluated by Gardner-Medwin and Walton (GM-W) scale. Pathological staining results of the lower limb muscles were observed; the fatty infiltration and edema of the muscles were observed by MRI T1WI and short-tau inversion recovery (STIR) sequences. Lower limb muscles were scored using Mercuri's scale. Spearman rank correlation was used to analyze the correlations of MRI fatty degeneration total scores of the lower extremities with age, age of onset, disease duration, GM-W scale scores and creatinine kinase (CK) level.Results:Of the 8 patients with LGMDR1, 7 had decreased muscle strength in the proximal lower extremity, including 4 with decreased muscle strength in the distal lower extremity at the same time. Muscular dystrophy-like pathological changes of skeletal muscles were noted. All 8 LGMDR1 patients showed different degrees of fatty infiltration in the lower extremities: at the thigh level, the adductor magnus, biceps femoris long head, semimembranes, semitendinosus and adductor longus were the most severely fatty degeneration muscles (mean scores>4), with relatively sparing of the sartorius and rectus femoris; regarding the calves, gastrocnemius medial head was the mostly involved, followed by soleus, with relative sparing of the tibialis posterior and anterior compartment. Edema-like changes (mild) were observed in 7 patients; the muscles that most frequently and relatively severely displayed edema-like changes were the gastrocnemius lateral head and quadriceps. The fatty degeneration total scores of the lower extremities were positively correlated with GM-W scale scores ( r=0.872, P=0.005) and negatively correlated with CK level ( r=-0.929, P=0.001), but not significantly correlated with age, age of onset or disease duration ( r=0.635, P=0.091; r=0.571, P=0.139; r=0.551, P=0.157). Conclusion:The lower limb muscles with severe fatty infiltration are less prone to show edema-like changes; fatty degeneration can be used to evaluate LGMDR1 progress; involvement pattern of muscle MRI of the lower extremities is helpful in diagnosing and differentially diagnosing LGMDR1.
8.Analysis of clinical, imaging and gene mutations in 2 pedigrees with hereditary spastic paraplegia type 7
Gang LI ; Jun FU ; Mi PANG ; Jia SONG ; Mingming MA ; Jiewen ZHANG
Chinese Journal of Neurology 2023;56(11):1254-1262
Objective:To analyze the clinical, imaging and genetic characteristics of 2 pedigrees with hereditary spastic paraplegia type 7 (SPG7).Methods:The clinical data of the probands and related members of 2 families hospitalized in the Department of Neurology of Henan Provincial People′s Hospital from December 2018 to December 2021 were collected. The probands and all family members were subjected to cranial MRI imaging and genetic testing, and the clinical characteristics and genetic variation of SPG7 families were compared with those reported in the literature.Results:Four patients from the 2 families were observed with adult-onset age in this group. The main manifestations were wide-base ataxic gait in 4 cases, and spastic gait in 1 case during follow-up. Pyramidal tract involvement mainly in the lower limbs were found in all cases, and dysarthria in 3 cases. MRI of 3 patients showed varying degrees of cerebellar atrophy. Genetic testing revealed compound heterozygous or homozygous variants of the SPG7 gene in the 4 patients, of which c.2062C>T and c.2176C>T were novel mutations. At present, only 5 SPG7 families have been reported in China. Among the 12 patients in all groups, 12 cases of pyramidal tract involvement, 10 cases of cerebellar ataxia, 7 cases of dysarticulation, 3 cases of cognitive impairment, 11 cases of complex hereditary spastic paraplegia, 1 case of simple hereditary spastic paraplegia, and 9 cases of cerebellar atrophy were reported. Six novel mutations have been reported in 5 families. Conclusions:SPG7 family is rarely reported in China, mainly manifested as pyramidal tract involvement combined with cerebellar ataxia, accompanied by cerebellar atrophy. SPG7 mutation is confirmed by genetic detection, and there are many novel mutations in SPG7 family in China.
9.Clinical and imaging features and prognoses of myelin oligodendrocyte glycoprotein antibody associated disorders
Huiqin LIU ; Hongpei CUI ; Milan ZHANG ; Rui PANG ; Weifeng MA ; Yongshi TENG ; Jiewen ZHANG ; Yue HUANG ; Lipin YUAN ; Wei LI
Chinese Journal of Neuromedicine 2022;21(2):164-171
Objective:To explore the clinical and imaging features and prognoses of myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD).Methods:Thirty-nine MOGAD patients, admitted to our hospital from January 2018 to April 2021, were chosen in our study. The clinical and imaging data and follow-up results of these patients at acute attack period (first-onset or relapse) were collected and their features were analyzed.Results:In these 39 patients with MOGAD, 20 patients (51.3%) had non-reversing course, and 19 patients (48.7%) had relapsing course. The clinical and imaging data of 55 episodes of these 39 patients were collected. In these 55 episodes, optic neuritis was noted in 27 episodes (49.1%), encephalitis was noted in 10 episodes (18.2%), brainstem encephalitis was noted in 8 episodes (14.5%), meningoencephalitis in 2 episodes (14.5%), myelitis in 3 episodes (5.5%), encephalomyelitis in 1 episode (1.8%), optic neuromyelitis in 1 episode(1.8%), optic neuritis+meningoencephalitis in 2 episodes (3.6%), and optic neuritis+encephalitis in 1 episode (1.8%). The positive rate of antinuclear antibody (ANA) was 11.1% (4/36); the cerebrospinal fluid results of 28 samples were collected from 22 patients, and CSF pleocytosis occurred in 67.9% of the samples with value of 54.89±67.70×10 6/L. Twenty-seven brain MRIs of 19 patients at the acute episode were collected; one completely normal MRI was recorded; among the remaining 26 MRIs, 6 were with one single lesion, 5 were with 2 lesions, and 15 were with 3 or more lesions; in terms of distribution, lesions involving brainstem and its adjacent structures were found in 9 MRIs, lesions involving diencephalon and deep gray matter were found in 7 MRIs, supratentorial white matter lesions were found in 13 MRIs, and cortical lesions were found in 13 MRIs. Meningeal enhancement were found in 4 contrast-enhanced brain MRIs (4/20). Long or short segmental myelitis in the spinal MRIs was noted in spinal lesions, involving cervical spinal cord, thoracic spinal cord and conus, and the "H" sign could be seen in the cross section. All patients received steroids therapy at the acute phase and the doses of steroids were tapered down gradually. Thirty-eight patients (97.4%) had good prognosis after 3 months of treatment. Conclusions:MOGAD is a disease entity widely involving the white matter, gray matter and meninges of the central nervous system with various clinical manifestations such as optic neuritis, encephalitis, brainstem encephalitis, meningoencephalitis and myelitis or a combination of the above. Immunotherapy is effective in most patients, but the recurrence rate is high, and some patients require long-term immunotherapy.
10.Clinical and genetic features of 12 families with Pompe disease
Jun FU ; Gang LI ; Mi PANG ; Jia SONG ; Jiewen ZHANG ; Mingming MA
Chinese Journal of Neuromedicine 2022;21(4):379-386
Objective:To investigate the clinical and genetic features of Pompe disease, and analyze the effect of enzyme replacement therapy on it.Methods:A retrospective study was performed. The clinical data and genetic results of 14 patients with Pompe disease from 12 families, admitted to our hospital from January 2017 to June 2021, were collected. Some patients were followed up after therapies.Results:Twelve of the 14 patients were late onset, with onset age ranged from 1.5 to 37.0 years (mean 15.2 years), and the other 2 patients were infantile onset. The predominant manifestations included proximal lower limb weakness, accompanied by easy fatigue and myalgia; 8 patients presented with dyspnea, of which one had dyspnea as initial presentation. Serum creatine kinase ranged from 172 to 1397 IU/L (mean 878 IU/L). Electromyography revealed myogenic pattern in 6 patients and myotonic discharge in 4 patients. Forced vital capacity decreased in 10 patients, and scoliosis was detected in 5 patients; 13 patients had decreased acid-alpha-glucosidase (GAA) activity; muscle pathology indicated vacuolar myopathy in 8 patients. Genetic test revealed 17 variants in GAA gene, among which c.2331G>C, c.1622C>T, c.1585T>C, and c.1837T>C were 4 novel likely pathogenic variants. The c.2238G>C and c.2662G>T were found in 5 and 3 families, respectively. Muscle strength and lung function got improvement in 1 patient who received enzyme replacement therapy and had regular follow-up, while muscle strength and lung function were worsened in those who did not receive enzyme replacement therapy. Conclusions:Pompe disease is characterized by skeletal muscle weakness and pulmonary dysfunction, and may be associated with spinal deformity; creatine kinase is mildly to moderately elevated, and myotonic discharge can be detected. GAA c.2238G>C and c.2662G>T are hotspot mutations in China; the 4 novel variants enrich the GAA mutational spectrum. Enzyme replacement therapy may improve motor and pulmonary function.

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