Analysis of related factors of citrin deficiency complicated with liver failure
10.3969/j.issn.1008-9691.2023.05.012
- VernacularTitle:希特林蛋白缺乏症合并肝衰竭的相关因素分析
- Author:
Yijun LIN
1
;
Yan CHEN
;
Rui ZHANG
;
Hong YE
Author Information
1. 福建省妇幼保健院儿内科,福建医科大学妇儿临床医学院,福建福州 350001;福建省儿童医院(上海儿童医学中心福建医院)消化营养科,福建福州 350014
- Keywords:
Citrin deficiency;
Clinical features;
Infant;
Liver failure;
Related factor
- From:
Chinese Journal of Integrated Traditional and Western Medicine in Intensive and Critical Care
2023;30(5):576-581
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the related factors of citrin deficiency(CD)complicated with liver failure(LF).Methods A retrospective analysis was performed on 91 patients with CD admitted to the department of pediatrics of Fujian Children's Hospital and Fujian Maternity and Child Health Hospital from January 2011 to August 2022,including 28 patients with LF(LF group)and 63 patients without LF(non-LF group).The general information of the patients(gender,age,body weight,feeding method and whether they were co-infected,whether they had light-colored stool,whether they had congenital heart disease,and whether they were accompanied by hepatomegaly)and laboratory indicators of the patients[white blood cell count(WBC),neutrophil count(NEU),hemoglobin(Hb),platelet count(PLT),C-reactive protein(CRP),blood lactic acid(Lac),blood ammonia,blood sodium,blood potassium,alanine aminotransferase(ALT),aspartate aminotransferase(AST),γ-glutamyl transpeptides(γ-GGT),total bilirubin(TBil),direct bilirubin(DBil),albumin,alkaline phosphatase(ALP),bile acid,cholesterol,uric acid,MB isoenzyme of creatine kinase(CK-MB),blood glucose(GLU),prothrombin time(PT),international standard normalized ratio(INR)were collected.The above indicators in two groups were compared,and the indicators with statistically significant differences in univariate analysis were included in Logistic regression analysis to find the correlated factors of CD complicated with LF.The predictive value of each related factor for CD complicated with LF was analyzed by drawing receiver operator characteristic curve(ROC curve).The area under the ROC curve(AUC)was used to evaluate the prediction efficiency of each factor.Results Compared with non-LF group,the patients in LF group had higher proportion of infections(60.7%vs.12.7%),higher TBil[μmol/L:242.50(210.95-278.68)vs.168.20(141.50-232.65)]and DBil[μmol/L:146.40(100.73-173.55)vs.79.40(65.39-99.60)],lower Hb(g/L:79.21±20.39 vs.94.75±12.56),PLT(×109/L:215.61±148.73 vs.377.63±163.00)and GLU(mmol/L:2.79±1.32 vs.4.10±1.36),worse prognosis[mortality:15.0%(3/28)vs.0.0%(0/63)],the differences were statistically significant(all P＜0.05).Logistic regression multi-factor analysis showed infections[odds ratio(OR)=5.160,95%credibility interval(95%CI)was 1.006-26.455,P = 0.049],decreased Hb(OR = 0.930,95%CI was 0.869-0.996,P = 0.037),decreased PLT(OR = 0.992,95%CI was 0.987-0.998,P = 0.009),decreased GLU(OR = 0.355,95%CI was 0.174-0.724,P = 0.004)and increased TBil(OR = 1.016,95%CI was 1.001-1.030,P = 0.034)were the related factors of CD complicated with LF.ROC curve analysis showed that Hb,PLT,GLU,TBil and the combined diagnosis had certain predictive value for CD complicated with LF[AUC and 95%CI were 0.754(0.639-0.868),0.756(0.646-0.867),0.749(0.644-0.854),0.807(0.717-0.898)and 0.944(0.897-0.992),respectively,all P = 0.000],among them,the combined diagnostic efficiency was the highest.When the best cut-off values of Hb,PLT,GLU and TBil were 86.50 g/L,254.50×109/L,3.29 mmol/L and 185.40 μmol/L,the sensitivity of Hb,PLT,GLU and TBiL were 74.6%,81.0%,76.2%and 96.4%,respectively,the specificity were 71.4%,63.3%,64.3%and 65.1%,respectively.Conclusion Infections,decreased Hb,PLT,GLU and increased TBil are the related factors associated with LF in CD,and combined diagnosis can improve the diagnostic efficiency.
