[Clinical application of plasma exchange combined with continuous veno-venous hemofiltration dialysis in children with refractory Kawasaki disease shock syndrome]. / 血浆置换联合连续性静脉-é™è„‰è¡€æ¶²æ»¤è¿‡é€æžæ²»ç–—åœ¨å„¿ç«¥å·å´Žç— ä¼‘å ‹ç»¼åˆå¾ä¸­çš„ä¸´åºŠåº”ç”¨.

OBJECTIVES: To study the role of plasma exchange combined with continuous blood purification in the treatment of refractory Kawasaki disease shock syndrome (KDSS). METHODS: A total of 35 children with KDSS who were hospitalized in the Department of Pediatric Intensive Care Unit, Hunan Children's Hospital, from January 2019 to August 2022 were included as subjects. According to whether plasma exchange combined with continuous veno-venous hemofiltration dialysis was performed, they were divided into a purification group with 12 patients and a conventional group with 23 patients. The two groups were compared in terms of clinical data, laboratory markers, and prognosis. RESULTS: Compared with the conventional group, the purification group had significantly shorter time to recovery from shock and length of hospital stay in the pediatric intensive care unit, as well as a significantly lower number of organs involved during the course of the disease (P<0.05). After treatment, the purification group had significant reductions in the levels of interleukin-6, tumor necrosis factor-α, heparin-binding protein, and brain natriuretic peptide (P<0.05), while the conventional group had significant increases in these indices after treatment (P<0.05). After treatment, the children in the purification group tended to have reductions in stroke volume variation, thoracic fluid content, and systemic vascular resistance and an increase in cardiac output over the time of treatment. CONCLUSIONS: Plasma exchange combined with continuous veno-venous hemofiltration dialysis for the treatment of KDSS can alleviate inflammation, maintain fluid balance inside and outside blood vessels, and shorten the course of disease, the duration of shock and the length of hospital stay in the pediatric intensive care unit.

[Application of transport ventilator in the inter-hospital transport of critically ill children]. / è½¬è¿å‘¼å¸æœºåœ¨å±é‡æ‚£å„¿é™¢é™ è½¬è¿ä¸­çš„åº”ç”¨ç ”ç©¶.

OBJECTIVES: To study the application value of transport ventilator in the inter-hospital transport of critically ill children. METHODS: The critically ill children in Hunan Children's Hospital who were transported with or without a transport ventilator were included as the observation group (from January 2019 to January 2020; n=122) and the control group (from January 2018 to January 2019; n=120), respectively. The two groups were compared in terms of general data, the changes in heart rate, respiratory rate, and blood oxygen saturation during transport, the incidence rates of adverse events, and outcomes. RESULTS: There were no significant differences between the two groups in sex, age, oxygenation index, pediatric critical illness score, course of disease, primary disease, heart rate, respiratory rate, and transcutaneous oxygen saturation before transport (P>0.05). During transport, there were no significant differences between the two groups in the changes in heart rate, respiratory rate, and transcutaneous oxygen saturation (P>0.05). The incidence rates of tracheal catheter detachment, indwelling needle detachment, and sudden cardiac arrest in the observation group were lower than those in the control group during transport, but the difference was not statistically significant (P>0.05). Compared with the control group, the observation group had significantly shorter duration of mechanical ventilation and length of stay in the pediatric intensive care unit and significantly higher transport success rate and cure/improvement rate (P<0.05). CONCLUSIONS: The application of transport ventilator in the inter-hospital transport can improve the success rate of inter-hospital transport and the prognosis in critically ill children, and therefore, it holds promise for clinical application in the inter-hospital transport of critically ill children.

A variant of RAG1 gene identified in severe combined immunodeficiency: a case report.

BACKGROUND: The recombination-activating gene 1 (RAG1) protein is essential for the V (variable)-D (diversity)-J (joining) recombination process. Mutations in RAG1 have been reported to be associated with several types of immune disorders. Typical clinical features driven by RAG1 variants include persistent infections, severe lymphopenia, and decreased immunoglobulin levels . CASE PRESENTATION: In this study, a 2-month-24-days-old infant with recurrent fever was admitted to our hospital with multiple infections and absence of T and B lymphocytes. The infant was diagnosed with severe combined immunodeficiency (SCID). A homozygous variation c.2147G>A (NM_000448.2: exonme2: c.2147G>A (p.Arg716Gln)) was identified in the RAG1 gene using whole-exome sequencing and Sanger sequencing. The predicted 3D structure of variant RAG1 indicated altered protein stability. Additionally, decreased expression of variant RAG1 gene was detected at both the mRNA and protein levels. CONCLUSIONS: Our study identified a novel homozygous variant in RAG1 gene that causes SCID. This finding expands the variant spectrum of RAG1 in SCID and provides further evidence for the clinical diagnosis of SCID.

Comparison of different modalities of continuous renal replacement therapy with regional sodium citrate anticoagulation in paediatric patients.

BACKGROUND: To evaluate the efficacy and safety of continuous renal replacement therapy (CRRT) modalities with regional sodium citrate anticoagulation (RCA) in children. METHODS: This retrospective study was conducted at the paediatric intensive care unit of Hunan Children's Hospital in China. Medical records of paediatric patients hospitalised for RCA-CRRT between April 2017 and March 2021 were reviewed. Patients received continuous venovenous haemodialysis, continuous venovenous haemofiltration (CVVH), or continuous venovenous haemodiafiltration (CVVHDF). RESULTS: Patients on continuous venovenous haemodialysis (n = 2) were excluded because of their small sample size. The remaining participants were divided into CVVH and CVVHDF groups; 80 patients received CRRT, with 40 and 62 sessions in the CVVH and CVVHDF groups, respectively. The filtre lifespan was longer in the CVVHDF group than in the CVVH group (median value [interquartile range]; 47 [15] hours vs. 35 [17.5] hours; p = 0.029). Compared with the CVVHDF group, the hazard ratio for filtre lifespan in the CVVH group was 3.023 (95% confidence interval 1.820-5.023, p < 0.001). There were no significant differences in ionised calcium levels of the circuits between the two groups at different time points (p < 0.05). Metabolic alkalosis, hyperlactataemia, hypocalcaemia, and hypercalcaemia occurred in both groups, with metabolic alkalosis being the most common complication. No patients in either group experienced sodium citrate accumulation or hypernatraemia. Inter-group differences in the incidence of these complications were not statistically significant (p > 0.05). CONCLUSIONS: Our results suggest that CVVHDF is a better option for RCA-CRRT than CVVH.

[Application of double plasma molecular adsorption system in children with acute liver failure].

OBJECTIVE: To study the efficacy and safety of double plasma molecular absorption system (DPMAS) in the treatment of pediatric acute liver failure (PALF). METHODS: A prospective analysis was performed on the medical data of children with PALF who were hospitalized in the Intensive Care Unit (ICU), Hunan Children's Hospital, from March 2018 to June 2020. The children were randomly divided into two groups:plasma exchange group (PE group) and DPMAS group (n=18 each). The two groups were compared in terms of clinical indices after treatment, laboratory markers before and after treatment, and adverse events after treatment. RESULTS: Compared with the PE group, the DPMAS group had a significantly lower number of times of artificial liver support therapy and a significantly shorter duration of ICU stay (P < 0.05), while there was no significant difference in the 12-week survival rate between the two groups (P > 0.05). There was no significant difference in laboratory markers between the two groups before treatment (P > 0.05). After treatment, both groups had reductions in the levels of total bilirubin, interleukin-6, and tumor necrosis factor-α, and the DPMAS group had significantly greater reductions than the PE group (P < 0.05). Both groups had a significant reduction in alanine aminotransferase (P < 0.05), while there was no significant difference between the two groups (P > 0.05). The PE group had a significant increase in albumin, while the DPMAS group had a significant reduction in albumin (P < 0.05). The PE group had a significant reduction in prothrombin time, while the DPMAS group had a significant increase in prothrombin time (P < 0.05). There was no significant difference between the two groups in the rebound rate of total bilirubin and the overall incidence rate of adverse events after treatment (P > 0.05). CONCLUSIONS: DPMAS is safe and effective in the treatment of PALF and can thus be used as an alternative to artificial liver support therapy.

[Clinical application of blood purification in treatment of severe adenovirus pneumonia].

OBJECTIVE: To study the role of blood purification in the treatment of severe adenovirus pneumonia. METHODS: A total of 57 children with severe adenovirus pneumonia who underwent mechanical ventilation from February to June, 2019, were enrolled. According to whether blood purification was performed, they were divided into a purification group with 22 children and a conventional group with 35 children. Related clinical indices were collected, including duration of fever, duration of mechanical ventilation, length of stay in the intensive care unit (ICU), and mortality rate. The purification group was analyzed in terms of interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) before blood purification and at 48 hours after blood purification, as well as stroke volume variation (SVV), thoracic fluid content (TFC), arterial partial pressure of oxygen/fraction of inhaled oxygen (P/F) value, and partial pressure of carbon dioxide (PCO2) before blood purification and at 6, 12, 24, and 48 hours after blood purification. RESULTS: Compared with the conventional group, the purification group had significantly shorter duration of fever, duration of mechanical ventilation, and length of stay in the ICU (P<0.05), and there was no significant difference in the mortality rate between the two groups (P>0.05). The purification group had significant reductions in IL-6 and TNF-α after blood purification, (P<0.05) and significant reductions in SVV and TFC at 12, 24, and 48 hours after blood purification (P<0.01), as well as a significant increase in P/F value and a significant reduction in PCO2 at 6, 12, 24, and 48 hours after blood purification (P<0.01). CONCLUSIONS: Blood purification as an auxiliary therapy can effectively improve the clinical symptoms of children with severe adenovirus pneumonia, and is thus an option for the treatment of severe adenovirus pneumonia in children.

[Clinical features of severe type 7 adenovirus pneumonia: an analysis of 45 cases].

OBJECTIVE: To study the clinical features of severe type 7 adenovirus pneumonia in children. METHODS: A retrospective analysis was performed for the clinical data of children who were diagnosed with severe type 7 adenovirus pneumonia from February to June, 2019. RESULTS: Among the 45 children, the male/female ratio was 3:2 and the median age was 14 months. All children had repeated fever, cough, and pulmonary moist rales, and the mean duration of fever was 14±4 days. The median time from fever to dyspnea was 8 days, and the time from fever to mechanical ventilation was 11.6±2.5 d. There was no significant increase in white blood cell count, with neutrophils as the main type. There were slight reductions in hemoglobin and albumin, while platelet and fibrinogen remained normal. There were increases in aspartate aminotransferase, lactate dehydrogenase, procalcitonin, and C-reaction protein. The detection rate of mixed pathogens was 84%. Effusion in both lungs was the major change on chest imaging (64%). Bronchoscopic manifestations were endobronchitis, tracheomalacia, and plastic bronchitis. The incidence rate of respiratory complications was 100%, and extrapulmonary complications mainly involved the circulatory system (47%), digestive system (36%), and nervous system (31%). Among the 45 children, 16 were administered with 400 mg/kg intravenous immunoglobulin (IVIG) daily for 5 days, with a mean duration of fever of 16±5 days, and 29 were administered with 1 g/kg IVIG daily for 2 days, with a mean duration of fever of 13±4 days; there was a significant difference in the mean duration of fever between the two groups (P=0.046). The overall mortality rate was 11%. CONCLUSIONS: Severe type 7 adenovirus pneumonia in children has severe conditions, with a high incidence rate of complications and a high mortality rate, so it should be diagnosed and treated as early as possible.

[Value of pancreatic stone protein/regenerating protein in severity evaluation and prognosis prediction for children with sepsis].

OBJECTIVE: To evaluate the value of pancreatic stone protein/regenerating protein (PSP/reg) in severity evaluation and prognosis prediction for children with sepsis. METHODS: In this prospective case-control study, 159 children with sepsis (106 cases in the sepsis group; 53 cases in the severe sepsis group, including 12 cases of septic shock) and 20 children without sepsis (control group) were enrolled. ELISA was applied to measure plasma PSP/reg levels on days 1, 3, and 7 of admission to the PICU. The Spearman rank correlation test was applied to assess the correlations between plasma PSP/reg level and serum procalcitonin (PCT), CRP, WBC count, and pediatric critical illness score (PCIS). The area under the receiver operating characteristic curve (AUC) was used to assess the value of each index in determining severity and predicting prognosis for children with sepsis. RESULTS: On day 1 of admission to the PICU, plasma PSP/reg levels in the sepsis and severe sepsis groups were significantly higher than in the control group (P<0.05), and the severe sepsis group had a significantly higher plasma PSP/reg level than the sepsis group (P<0.05). On day 1 of admission to the PICU, the survival group (n=132) had a significantly lower plasma PSP/reg level than the non-survival group (n=27) (P<0.05). On day 1 of admission to the PICU, plasma PSP/reg level in children with sepsis was positively correlated with WBC count and serum PCT level (rs=0.212 and 0.548, respectively; both P<0.05), and negatively correlated with PCIS score (rs=-0.373; P<0.05). The AUCs of plasma PSP/reg level and serum PCT for determination of severe sepsis, septic shock, and death were higher than 0.7 (P<0.05). CONCLUSIONS: PSP/reg is closely related to infection, and has a certain clinical value in risk stratification of sepsis and prognosis evaluation.

[Clinical study of Pentraxin 3 in diagnosing the severity and cardiovascular function of the children with sepsis].

OBJECTIVE: To study the value of Pentraxin 3 (PTX3) in diagnosing the severity and cardiovascular function of the critically ill children. Method A total of 178 patients who were older than 28 days, with acute infection of respiratory or neurological system, excluding chronic or special disease, and admitted to the pediatric intensive care unit (PICU) of Hunan Children's Hospital from October 1, 2013 to April 30, 2014 were enrolled, including 102 male cases and 76 female cases. The ages ranged from 1 month to 13 years and 1 month, 78 of them were less than 1 year old ; 58 cases were between 1 to 3 years old; 42 cases were above 3 years old; 101 cases were diagnosed as respiratory system diseases, 77 cases had nervous system diseases. PTX3 was detected with enzyme-linked immunosorbent assay (ELISA) within 1 d after enrollment, at 3 days and 7 days, meanwhile, troponin, myocardial enzyme, brain-type natriuretic peptide (BNP), C-reactive protein (CRP), plasma calcitonin (PCT) and WBC etc. Were measured. According to the plasma PTX3 value which were measured within 24 h after enrollment the patients were divided into three groups: mildly elevated group (< 44 µg/L) 41 cases; moderately elevated group (44 - < 132 µg/L) in 66 cases; severely elevated group 71 cases (132 µg/L or higher). Those 178 patients were divided into 3 groups according to the degree of infection: non-sepsis group (78 cases), sepsis group (70 cases), severe sepsis group (30 cases), and in each group, those with heart failure were respectively 19 cases, 28 cases, 17 cases. Analysis of the plasma PTX3 expression changes in different clinical manifestations, different condition, different degrees of organ damages and prognosis for the patient. The continuous variables were analyzed with t-test, F-test, H-test, the categorical variables were analyzed with Chi-square test, and the correlation analysis was performed to calculate Pearson coefficients. RESULT: The PTX3 value measured within 24 h after enrollment increased with the degree of infection (50. 4(35. 2,70. 4) µg/L; 175. 8 (99. 6, 309. 9) µg/L;419. 9 (168. 3, 468. 6) µg/L; H = 88. 345, P = 0. 000). PTX3 level gradually declined, while in severe sepsis group decreased slowly (P <0. 05); the area under the ROC curve of Plasma PTX3 was larger than that of other inflammatory markers such as CRP and PCT, white blood cells and neutrophils in the diagnosis of sepsis; while the former three are PTX3, PCT and CRP (the sensitivity and specificity respectively were 0. 77, 0. 68; 0. 66, 0. 6; 0. 47, 0. 55); the PTX3 value of the severely elevated group was significantly higher than those of the mildly and moderately elevated groups (P <0. 05). The proportion of having 3 or more organs failure increased as the PTX3 rising among the groups of mildly elevated group, moderately elevated group and severely elevated group (1(2. 4%), 4(6. 1%), 14(19. 7%) χ2 =16. 16,P = 0. 000); and in each group, the proportion of having good and poor prognosis for these three groups were different (33 (80.5%) and 8 (19. 5%), 35 (53%) and 31 (47%), 28 (39.4%) and 43 (60.6%), χ = 17. 663, P = 0. 000). The K-M curve for these three groups had statistically significant difference (χ2 = 7. 086, P = 0. 029). Those with heart failure had higher PTX3 value than those in non-heart failure at the same degree of infection. PTX3 value increased with myocardial enzyme (troponin, creatine kinase isoenzyme, BNP) levels. In the diagnosis of heart failure, the area under the ROC curve were respectively PTX3 0. 824; BNP 0. 772; CM-KB 0. 643; CNTIO. 671, the sensitivity and specificity were PTX3 0. 8, 0. 58; CK-MB 0. 56,0. 79; CTNI 0. 60,0. 69; BNP 0. 73, 0. 58. In terms of predicting the prognosis of sepsis with heart failure complications, the PTX3 value's area under ROC curve was larger than that of BNP (respectively 0. 844, 0. 472). CONCLUSION: The PTX3 is an objective biochemical marker in diagnosis of sepsis; it is helpful in assessment of severity and prognosis of sepsis; it also has a certain clinical value in the assessment of sepsis cardiovascular function damage.