Objective To discuss the value of diagnosing gastrointestinal dysfunction in.critically ill children.Methods Seventy-six critically ill patients were reviewed,48 cases combined with gastrointestinal dysfunction.Plasma D-lactate,intestinal fatty acid binding protein(IFABP)of serum and urine were performed in all patients,detected the sensitivity and specificity,ealculated the area under the ROC curve (AUC).Results Plasma D-Iactate level≥9.63 mg/L had a sensitivity of 85.2%,specificity of 70.9%,and AUC of 0.822 for diagnosing gastrointestinal dysfunction.Serum IFABP level≥0.129μg/L had a sensitivity of 65.2%,specificity of 63.8%,and AUC of 0.744 for diagnosing gastrointestinal dysfunction.Urine IFABP ≥0.330μg/Lhad a sensitivity of 78.3%,specificity of 72.3%,and AUC of 0.820 for diagnosing gastrointestinal dysfunction.There was no significant difference in AUC between plasma and urine IFABP(P＞0.05),there was no significant difference in AUC between serum D-lactate and serum IFABP(P＞0.05).Conclusions Plasma D-Lactgte,serum and urine WABP may be useful markers as warning gastrointestinal dysfunction of critically ill children.Plasma D-lactate and urine IFABP level may be more sensitively,but they need to be further studied.

Objective To explore the significance of motor unit number estimation (MUNE) by using multiple point stimulating technique to evaluate patients with Hirayama disease (HD).Methods Multiple point stimulating technique was used to estimate the motor unit number of abductor pollicis brevis and abductor digiti minimi in 35 normal subjects [14-33 years old,mean (20.9 ±4.0) years old,33 men and 2 women]without nerve and muscle disease and 69 patients definitely diagnosed as HD [16-35 years old,mean (21.46 ±6.61 ) years old,67 men and 2 women].The differences between the two groups were examined by Fisher's exact test and t test.Results There were 42 patients with atrophy and 27 patients with normal clinical manifestation of left hand.For right hand there were 54 patients with atrophy and 15 normal.For controls,the MUNE value of left abductor pollicis brevis was 226.97 ± 30.59,while that of right side was 22g.31 ± 25.35.The MUNE value of left abductor digiti minimi was 237.43 ± 30.78,while that of right side was 240.20 ± 37.73.For HD patients,the MUNE of left abductor pollicis brevis and abductor digiti minimi was 145.66 ± 126.10 ( t =5.07,P ＜ 0.01 ) and 102.20 ± 112.67 ( t =9.31,P ＜ 0.01 )respectively,while those of right hand was 149.72 ± 117.80 ( t =5.31,P ＜ 0.01 ) and 64.23 ± 69.27 ( t =16.76,P ＜0.01 ) respectively.MUNE of left abductor digiti minimi in 17 patients that was below 200 among 27 patients with normal clinical manifestation (x2 =9.57,P =0.002).MUNE of right abductor digiti minimi in 12 patients that was below 200 among 15 patients with normal clinical manifestation ( x2 =4.64,P =0.03).Conclusions The differences of MUNE values by multiple point stimulating technique between the normal subjects and the HD patients is significant,which suggests this method is very useful to evaluate HD in the early state.

Objective To investigate the clinical and radiological features of medullary infarction (MI), and to compare the clinical characteristics of lateral medullary infarction (LMI) and medial medullary infarction (MMI).Methods Patients diagnosed as acute MI who were treated from January 2009 to December 2014 in Department of Neurology, Peking University Third Hospital, were retrospectively enrolled in the study and data were analyzed including risk factors, clinical features, laboratory findings, radiological characteristics, etiology and outcomes.Results A total of 62 cases of MI were enrolled with 48 cases of LMI and 14 cases of MMI , including 2 cases of bilateral MMI.The mean onset age of LMI and MMI was 60.2 ± 12.3 and 56.9 ± 14.2, respectively.The frequently affected location of LMI was the middle and upper part of medulla [40 cases (83.3％)].The common symptoms and signs of LMI were dizzy (38 cases ,79.2％), sensory disturbance (33 cases ,68.8％), dysarthria (32 cases ,66.7％), dysphagia (30 cases ,62.5％), diminished pharyngeal reflex (30 cases, 62.5％), Homer's sign (29 cases, 60.4％), ataxia (26 cases, 54.2％) and nausea or vomiting (25 cases, 52.1％).The frequently affected location of MMI was the upper part of medulla (13 cases, 92.9％).The common symptoms and signs of MMI were motor dysfunction (12 cases, 85.7％), sensory disturbances (11 cases, 78.6％), dizzy (10 cases, 71.4％) and dysarthria (10 cases, 71.4％).Infarctions caused by atherosclerosis were found in 35 cases of LMI (72.9％) and 12 cases of MMI (85.7％).Five cases (10.4％) of LMI died in hospital, while 1 case (7.1％) of MMI died in hospital.No lesion was found in 16 cases (25.8％) by MRI-DWI within the first 24 hours of onset.Conclusions Our study showes that the mean onset age of LMI is older than that of MMI.The lesion of LMI is frequently located in the upper and middle medulla, whereas the lesion of MMI is mostly in the upper medulla.The prognosis of LMI is worse than that of MMI.Atherosclerosis of the vertebral arteries is the predominant vascular pathology in MI.

Adult ; China ; Female ; Humans ; Ichthyosis Vulgaris ; diagnosis ; genetics ; Male ; Middle Aged ; Pedigree ; Young Adult

Objective:To establish an experimental model for intracellular antibacteria and endotoxin neutralization in vitro to detect the antibacterial and endotoxin neutralization activity of the muBPI_(25) protein.Methods: RAW264.7 cells were transfected with pcDNA3.1(+)muBPI_(36-259), and then were infected with intracellular bacterial of either G ~+/G~-to establish the experimental model of intracellalar antibacteria.The RAW264.7 cells were co-transfected with the pSecTag2B-muBPI_(36-259) and dual-luciferase reporter gene plasmids for establishment of the experimental model of endotoxin neutralization.Results:The experimental model of intracellular antibacteria confirmed that the muBPI_(25) protein could inhibit/kill Salmonella typhi.The experimental model of endotoxin neutralization indicated that the muBPI_(25) protein could neutralize endotoxin.Conelusion: We firstly demonstrate that murine BPI N-terminal functional fragment(muBPI_(25) protein)can inhibit/kill Salmonella typhi,and can neutralize, its lysating product, endotoxin.

The clinical and imaging data in 6 patients with corticobasal syndrome were retrospectively analyzed. Six patients presented asymmetric clinical symptoms, including 5 with cognitive impairment, 6 with emotional disorders, 2 with cortical sensory deficit, 5 with lalopathy, and 4 with apraxia. All patients developed limb dystonia and limb or trunk stiffness, 4 with tumble, 4 with bradykinesia, and 2 with tremor. Brain magnetic resonance imaging (MRI) showed that 4 patients had unilateral cerebral atrophy and 2 had mild atrophy of bilateral hippocampus. Localized low glucose metabolism in the unilateral cerebral lobe was seen in four patients by positron emission computed tomography (PET) examination, suggesting that PET is helpful for the diagnosis of corticobasal syndrome.

To explore clinical characteristics,electrophysiological findings and treatment response in diabetic patients with chronic inflammatory demyelinating polyradiculoneuropathy (DM-CIDP). Thirty-six CIDP patients were identified, 10 were DM-CIDP and 26 were non-DM-CIDP. Clinical medical records, electrophysiological data and treatment were retrospectively reviewed.DM-CIDP patients were significantly older than non-DM-CIDP patients [(56.7 ± 10.0) years old vs. (40.4 ± 16.9) years old, P=0.001]. However, clinical characteristics and abnormalities of electrophysiological tests in both groups were comparable. DM-CIDP subjects responded to corticosteroids or intravenous immunoglobulin, thus with better prognosis.

Objective:To explore the relationship between seizure cluster of temporal lobe epilepsy with hippocampal sclerosis (TLE-HS) and cortisol (COR) rhythm, and understand its mechanism from the perspective of neuroendocrine.Methods:Fifty-seven patients with unilateral TLE-HS were recruited from the Qinghai Provincial People′s Hospital from May 1st 2012 to December 31st 2020. According to the history of seizure clusters one month before admission, 27 patients were enrolled in seizure clusters group (SC group), 30 patients were included in without seizures cluster group (NSC group). The clinical characteristics were systematically analyzed and compared between the SC and NSC groups. Plasma COR levels were measured at 8:00, 16:00 and 24:00 (COR8, COR16 and COR0) on the same day, and bilateral magnetic resonance spectroscopy (MRS) diagnosis was performed in two groups. Independent sample t test, chi-square test, repeated analysis of variance, covariance analysis, and multivariate Logistic regression were used for statistical analysis. Results:Time effect, grouping effect and the interaction effect of the time and grouping in the level of COR were statistically significant. Covariance analysis excluded age as an influential factor, COR16, COR0 and the slope of COR8-16 in the SC group [(126.22±19.98) μg/L, (51.63±21.43) μg/L, -7.78±4.54] were higher than the NSC group [(97.70±18.55) μg/L, (31.90±10.73) μg/L, -12.40±4.16], and the difference was statistically significant ( F=5.587, 4.320, 4.013, all P<0.05). The slope of COR0-8 in the SC group (17.11±6.32) was lower than that in the NSC group (20.62±6.54), and the difference was statistically significant ( F=-2.065, P<0.05). There was no significant difference in lateralization of hippocampal sclerosis between the two groups, and there was no significant difference in the ratio of N-acetyl aspartic acid(NAA)/[choline(Cho)+creatinine(Cr)] in the unilateral hippocampal sclerosis zone of the two groups, but the NAA/(Cho±Cr) ratio of the contralateral hippocampus in the SC group (0.71±0.03) was lower than that in the NSC group (0.76±0.06),and the difference was statistically significant ( t=4.999, P=0.029). Multivariate Logistic regression analysis showed that COR16 ( OR=1.328, 95% CI 1.073-1.642, P=0.009), COR8-16 ( OR=3.657, 95% CI 1.404-9.525, P=0.008) were independent risk factors of seizure clusters in TLE-HS. Conclusion:COR rhythm disturbance may be the neuroendocrine basis of seizure clusters in patients with TLE-HS.