[Predictive value of glycemic variability within 6 hours on the short-term prognosis of patients with septic shock].

OBJECTIVE: To investigate the predictive value of different glycemic variability indexes within 6 hours on the short-term prognosis of septic shock patients. METHODS: A retrospective study was conducted. The 133 patients with septic shock admitted to intensive care unit (ICU) of Nanjing Hospital Affiliated to Nanjing Medical University from December 2014 to December 2019 were enrolled. Patients with septic shock admitted to ICU died during hospitalization were enrolled in the death group and others in the survival group. General data of the patients including gender, age, underlying disease, site of infection, duration of mechanical ventilation, length of ICU stay, whether to use continuous renal replacement therapy (CRRT) and acute physiology and chronic health evaluation II (APACHE II) scores within 24 hours were collected. The blood glucose (GLUadm), mean arterial pressure (MAP), serum creatinine (SCr) and procalcitonin (PCT) were recorded at ICU admission. The patients admitted to ICU received bundle therapy within 6 hours and blood glucose was observed every 2 hours. The blood glucose difference (GLUdif), average blood glucose (GLUave), blood glucose standard deviation (GLUsd) and blood glucose variation coefficient (GLUcv) within 6 hours were calculated. Multivariate Logistic regression analysis was used to analyze the prognostic factors of short-term prognosis of patients with septic shock, and receiver operating characteristic (ROC) curve was plotted to evaluate the diagnostic efficacy of glycemic parameters for short-term prognosis of septic shock patients. RESULTS: A total of 133 patients with septic shock were admitted to ICU, among them 87 patients survived and 46 patients died during the ICU hospitalization. Compared with the survival group, the SCr at ICU admission and APACHE II score within 24 hours were significantly higher in the death group [SCr (µmol/L): 208.5 (143.0, 286.5) vs. 172.0 (91.0, 234.0), APACHE II score: 30.28±6.67 vs. 24.03±5.90, both P < 0.05], the length of ICU stay was shorter [days: 4.00 (2.00, 10.25) vs. 9.00 (4.00, 13.00), P < 0.01]. However, there were no significant differences in the baseline data of gender, age, underlying disease, infection site, CRRT ratio, MAP or PCT at ICU admission between the two groups. Compared with the survival group, the GLUsd and GLUcv within 6 hours in the death group were higher [GLUsd (mmol/L): 2.33 (1.95, 3.14) vs. 2.02 (1.66, 2.52), GLUcv: (31.00±7.06)% vs. (23.31±10.51)%, both P < 0.05]. There were no statistically significant differences in the levels of GLUadm, GLUdif or GLUave within 6 hours between the two groups. Multivariate Logistic regression analysis showed that APACHE II score within 24 hours and GLUsd and GLUcv within 6 hours were independent risk factors of the short-term prognosis of septic shock patients [APACHE II score: odds ratio (OR) = 1.173, 95% confidence interval (95%CI) was 1.095-1.256, P = 0.000; GLUsd: OR = 1.465, 95%CI was 1.038-2.067, P = 0.030; GLUcv: OR = 1.089, 95%CI was 1.043-1.138, P = 0.000]. ROC curve analysis showed that GLUsd and GLUcv within 6 hours both had certain predictive value for the short-term prognosis of septic shock patients, the area under ROC curve (AUC) of GLUcv within 6 hours was higher than that of APACHE II score (0.765 vs. 0.753), and AUC of GLUsd within 6 hours was close to APACHE II score (0.629 vs. 0.753); and the diagnostic value of GLUsd combined with GLUcv within 6 hours was higher than the two respectively (AUC: 0.809 vs. 0.629, 0.765), the sensitivity was 97.8%, and the specificity was 66.7%. CONCLUSIONS: GLUsd combined with GLUcv within 6 hours can be used to estimate the short-term prognosis of septic shock patients.

Two novel mutations in myosin binding protein C slow causing distal arthrogryposis type 2 in two large Han Chinese families may suggest important functional role of immunoglobulin domain C2.

Distal arthrogryposes (DAs) are a group of disorders that mainly involve the distal parts of the limbs and at least ten different DAs have been described to date. DAs are mostly described as autosomal dominant disorders with variable expressivity and incomplete penetrance, but recently autosomal recessive pattern was reported in distal arthrogryposis type 5D. Mutations in the contractile genes are found in about 50% of all DA patients. Of these genes, mutations in the gene encoding myosin binding protein C slow MYBPC1 were recently identified in two families with distal arthrogryposis type 1B. Here, we described two large Chinese families with autosomal dominant distal arthrogryposis type 2(DA2) with incomplete penetrance and variable expressivity. Some unique overextension contractures of the lower limbs and some distinctive facial features were present in our DA2 pedigrees. We performed follow-up DNA sequencing after linkage mapping and first identified two novel MYBPC1 mutations (c.1075G>A [p.E359K] and c.956C>T [p.P319L]) responsible for these Chinese DA2 families of which one introduced by germline mosacism. Each mutation was found to cosegregate with the DA2 phenotype in each family but not in population controls. Both substitutions occur within C2 immunoglobulin domain, which together with C1 and the M motif constitute the binding site for the S2 subfragment of myosin. Our results expand the phenotypic spectrum of MYBPC1-related arthrogryposis multiplex congenita (AMC). We also proposed the possible molecular mechanisms that may underlie the pathogenesis of DA2 myopathy associated with these two substitutions in MYBPC1.