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Objective To explore the clinical characteristics of Pierre Robin syndrome and the experience of its diagnosis and therapy, and provide the accurate diagnosis and treatment of Pierre Robin syndrome .Methods Seventeen cases of Pierre Robin syn-drome from September 2008 to August 2013 were enrolled in this study .The clinical data were analyzed retrospectively to explore the clinical characteristics and treatment of Pierre Robin syndrome .Results Seventeen cases of Pierre Robin syndrome had typical clini-cal features, including micrognathia, cleft palate or high palatine arches, glossoptosis, and feeding difficulties.The babies were given corresponding treatment according to their clinical grading. Among those babies, three cases died , including one dying of severe pneu-monia in hospital, and the other two dying of severe pneumonia and malnutrition after giving up treatment .Among 14 survivors, 12 ba-bies were followed up , including 5 babies who achieved optimal growth and development when they were one year old , and the other 7 babies who had feeding difficulties in varying degrees after discharge , and lagged behind normal children in the growth and develop-ment.Conclusions Early diagnosis, accurate classification, and individualized treatment plan for Pierre Robin syndrome might im-prove the prognosis of children with this type of disease .
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Objective To observe the serum levels of high mobility group box-1 protein (HMGB1)in patients with acute cholangitis (AC) and to investigate contributions of HMGB1 in AC.Methods Serum HMGB1 concentrations were determined by an enzyme-linked immunosorbent assay in 30 patients with AC of severe type (ACST) and 42 patients with mild acute cholangitis at the time of admission (within 72 h after the onset).A total of 50 healthy subjects were recruited as the control group.Fluorescent quantitative PCR (FQPCR) was used to detect the HMGB1 mRNA expression and the relationship between serum HMGB1 levels and clinical factors was analyzed.Results The serum HMGB1 levels in healthy control group,mild group and ACST group were (1.82 ± 0.64) μg/L,(10.46 ± 3.75) μg/L,(18.89 ± 6.86) μg/L,respectively.The mean value of serum HMGB1 level in mild group was significantly higher than that in control group,while significantly lower than that in ACST group (P ＜ 0.05).Compared to the control group,the HMGB1 mRNA level in patients of AC increased significantly and the level of ACST group was higher than that of mild group.The serum HMGB1 levels of patients with positive bile or/and blood cultures were higher than that of negative.After emergency endoscopic nasal biliary drainage,the serum HMGB1 levels of patients significantly decreased compared to preoperational (P ＜ 0.05).The HMGB1 levels were significantly positively correlated with white cell counts,C-reactive protein (CRP),total serum bilirubin,direct bilirubin and alkaline phosphatase (ALP).By logistic regression analysis,serum HMGB1 levels had correlation with severity of disease.Conclusion Serum HMGB1 levels significantly increased in patients with AC and the serum concentrations of ACST group were higher than those of mild group.Serum HMGB1 level has a correlation with sepsis.ENBD could lower its serum levels.Serum HMGB1 has predictive value to severity of disease.
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Objective To investigate the role of HMGB1 in the pathogenesis of organ injury of acute necrotizing pancreatitis (ANP).Methods Male ICR mice were randomly allocated into control group,ANP group and HMGB1 monoclonal antibody group.ANP model was induced by intraperitoneal injection of 20％ L-arginine.Mice in HMGB1 monoclonal antibody group were given intraperitoneal injection of 200 μg of HMGB1 monoclonal antibody immediately after the induction of the ANP model.All the mice were sacrificed at 12,24,and 48 h after ANP induction.Serum level of amylase and liver,renal function were determined,level of serum HMGB1 was measured by using enzyme-linked immunosorbent assay,and then the pathologic changes of pancreas and liver were routinely observed and scored.The HMGB1 mRNA levels in the liver and pancreas were studied by real time fluorescence quantitative PCR.Results The serum levels of HMGB1 at 12 h in control group,ANP group and HMGB 1 monoclonal antibody group were (9.09 ± 1.03),(25.04 ± 4.30),(16.84 ± 4.27) μg/L; and pathological scores of pancreatic tissue were (1.50 ± 0.55),(4.33 ± 0.52),(3.03 ± 0.32) points ; and HMGB1 mRNA expressions in pancreas were 0.48 ± 0.18,7.53 ± 2.71,3.26 ±2.33 ; HMGB1 mRNA expressions in liver were-1.23 ± 0.37,0.15 ± 0.65,－ 1.27 ± 0.72.The corresponding values in ANP group were significantly higher than those in control group (P ＜ 0.05).While the corresponding values in HMGB1 monoclonal antibody group were significantly lower than those in ANP group (P ＜0.05).There was a positive linear relationship between serum HMGB1 level and pancreatic pathological scores 24 h after ANP induction (r =0.768,P ＜ 0.05).In addition,the serum levels of AMY,AST,ALT,LDH,BUN,Cr showed a similar trend as that of serum level of HMGB1,and the serum level of HMGB1 was positively associated with serum levels of Cr,BUN and ALT (r =0.824,0.719,0.590,P＜0.05).Conclusions HMGB1 may be a key factor of inflammatory response and organ dysfunction of ANP in mice,and extrinsic supply of its monoclonal antibody may decrease the injuries of pancreas and other organs during ANP.
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Objective To explore the diagnostic effect of serum level of cystatin C (CysC) on the renal function after neonatal asphyxia by detection of serum level of CysC, blood urea nitrogen (BUN) and serum creatinine (SCr) and calculation of glomerular filtration rate (GFR) in neonatal asphyxia. Methods The clinical data of 86 neonates with asphyxia (46 cases in mild asphyxia group,40 cases in severe asphyxia group) and 30 neonates without asphyxia (control group) were collected and the serum level of CysC, BUN and SCr were detected at 24 h to 72 h after birth. Results Serum levels of CysC, BUN and SCr were (1.97 ±0.33) mg/L, (4.97 ±2.15) mmol/L, (90.41 ±24.32) μmol/L in mild asphyxia group, (2.65 ±0.41) mg/L, (10.88 ± 3.31) mmol/L, (125.82 ± 45.44) μ mol/L in severe asphyxia group and (1.24 ± 0.35)mg/L, (4.25 ± 2.04) mmol/L, (58.41 ± 19.22) μmol/L in control group, respectively. The differences were significant among three groups and those values in mild and severe asphyxia groups were higher than those in control group. The sensitivity of CysC level to evaluate renal function in mild asphyxia group was better than BUN and SCr level (P＜ 0.05). In neonata] asphyxia, the serum level of CysC had negative correlation with GFR (P ＜ 0.01). Conclusions Serum level of CysC can be adopted to evaluate the renal function after neonatal asphyxia, which is better than BUN and SCr. With a higer level of CysC, the renal function injury may be worse.
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Child A.The concentration of CGRP varied positively with the diameter of portal vein(r=0.60,P