Objective: To investigate the effectiveness and safety of regional citrate-anticoagulated (RCA) plasma exchange (PE) and whether citrate-related metabolic disorders can be improved by sequential RCA continuous renal replacement therapy (CRRT). Methods: This retrospective, single-center observational study included 79 critically ill children requiring PE followed by CRRT (June 2018 to June 2021) at the Pediatric Intensive Care Unit of Hunan Children’s Hospital, China. Patients were divided into the RCA-PE ( n = 30) and systemic heparin anticoagulation (SHA-PE) ( n = 49) groups. Filter level comparison post-PE assessed RCA-PE efficacy, and metabolic changes occurring pre- and post-PE and CRRT were used to evaluate the effect of CRRT on RCA-based anticoagulation safety. Results: The RCA-PE group had a better overall filter performance than the SHA-PE group. Two hours after PE, pH and HCO₃ − levels increased more significantly for the RCA-PE than the SHA-PE group. The RCA-PE incidence of metabolic alkalosis was 48.3%, higher by 4.2% ( p < 0.001) compared to the SHA-PE group. In the RCA-PE group, pH and HCO₃ − decreased significantly 4 h after CRRT; the metabolic alkalosis caused by RCA-PE decreased to 13.8% ( p = 0.005). No significant difference in pH, HCO₃ − , and metabolic alkalosis incidence was observed between the two groups 4 h after CRRT. Conclusions: The overall filtration performance of RCA-PE is superior to that of SHA-PE followed by CRRT. The metabolic complications associated with RCA-PE are mainly metabolic alkalosis that can be improved by using CRRT after RCA-PE and this is a better alternative for anticoagulation during PE in critically ill children.

BackgroundThe 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 presentationIn 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.ConclusionsOur 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.

PurposeWe aimed to explore the relationship between serum phosphate concentration and 90-day mortality in critically ill children receiving continuous renal replacement therapy (CRRT).MethodsData from the medical records of children aged <13 years who received CRRT at the Pediatric Intensive Care Unit of Hunan Children's Hospital, China from January 2015 to June 2020 were retrospectively collected. Children were grouped into four categories according to the baseline phosphate concentration before CRRT and mean serum phosphate concentration during CRRT: <0.81 mmol/L (hypophosphatemia), 0.81–1.19 mmol/L, 1.2–2.4 mmol/L (normal phosphate concentration), and >2.4 mmol/L (hyperphosphatemia), with the normal phosphate group serving as the comparator group. The correlation of the serum phosphate concentration before and during CRRT with the 90-day mortality after CRRT initiation was analyzed using logistic regression.ResultsA total of 177 children were included in our study. The mean serum phosphate concentration before CRRT was 1.46 mmol/L (quartiles: 1.04, 2.20). The 90-day mortality rate was increased in children with a serum phosphate concentration >2.4 mmol/L before CRRT (adjusted odds ratio [aOR] 3.74, 95% confidence interval [CI] 1.42–9.86, P = 0.008). The mean serum phosphate concentration during CRRT was 1.2 mmol/L (quartiles: 0.91, 1.49). The 90-day mortality rate was increased in children with a mean serum phosphate concentration >2.4 mmol/L during CRRT (aOR 7.34, 95% CI 1.59–33.88, P = 0.011).ConclusionHyperphosphatemia before and during CRRT predicts a higher 90-day mortality rate.

OBJECTIVES:To study the indication for therapeutic plasma exchange (TPE) and related complications in children admitted to the pediatric intensive care unit. METHODS:A retrospective analysis was performed on the medical records of the children who received TPE in the Pediatric Intensive Care Unit, Hunan Children's Hospital, from March 2015 to March 2021. The indication for TPE and related complications were analyzed and compared with the American Society for Apheresis (ASFA) indication categories. RESULTS:A total of 405 TPE treatment sessions were performed for 196 children, among whom 76 children (38.8%) also received continuous renal replacement therapy and 147 children (75.0%) survived. The children with neurological diseases had the highest survival rate of 93.1% (27/29). The top three indications for TPE were hematologic diseases (61/196, 31.1%), sepsis with multiple organ dysfunction (41/196, 20.9%), and liver diseases (36/196, 18.4%). The children with hematologic diseases received the highest number of 129 TPE treatment sessions. The subjects with ASFA category Ⅲ indications accounted for the highest proportion of 76.5% (150/196), followed by those with ASFA category Ⅰ indications (11.2%, 22/196), ASFA category Ⅱ indications (7.1%, 14/196), and unknown category (5.1%, 10/196), and no ASFA category Ⅳ indications were observed. The incidence rate of TPE complications was 12.3% (50/405), and the most common complications were pipeline coagulation (4.2%, 17/405) and hypotension (3.7%, 15/405). No serious adverse events were observed. CONCLUSIONS:TPE can be safely used for the treatment of critically ill children with indications in an experienced pediatric intensive care unit.

OBJECTIVES To investigate the efficacy and application value of plasma exchange as an adjuvant therapy in children with hemophagocytic syndrome (HPS). METHODS A prospective randomized controlled trial was designed. Forty children with severe HPS were enrolled, who were treated in the pediatric intensive care unit (PICU) of Hunan Children's Hospital from October 2018 to October 2020. The children were randomly divided into a plasma exchange group and a conventional treatment group using a random number table, with 20 children in each group. The children in the conventional treatment group received etiological treatment and conventional symptomatic supportive treatment, and those in the plasma exchange group received plasma exchange in addition to the treatment in the conventional treatment group. The two groups were compared in terms of general information, clinical symptoms and signs before and after treatment, main laboratory markers, treatment outcome, and prognosis. RESULTS Before treatment, there were no significant differences between the two groups in gender, age, course of the disease before admission, etiological composition, pediatric critical illness score, involvement of organ or system functions, and laboratory markers (P>0.05). After 7 days of treatment, both groups had remission and improvement in clinical symptoms and signs. After treatment, the plasma exchange group had significantly lower levels of C-reactive protein, procalcitonin, and serum protein levels than the conventional treatment group (P<0.05). The plasma exchange group also had significantly lower levels of alanine aminotransferase and total bilirubin than the conventional treatment group (P<0.05). The length of stay in the PICU in the plasma exchange group was significantly shorter than that in the conventional treatment group (P<0.05). The plasma exchange group had a significantly higher treatment response rate than the conventional treatment group (P<0.05). There were no significant differences between the two groups in the total length of hospital stay and 3-month mortality rate (P>0.05). CONCLUSIONS Plasma exchange as an adjuvant therapy is effective for children with severe HPS. It can improve clinical symptoms and signs and some laboratory markers and shorten the length of stay in the PICU, and therefore, it may become an optional adjuvant therapy for children with severe HPS.

OBJECTIVES:To investigate the risk factors for acute kidney injury (AKI) in children with cardiac arrest (CA) and the influencing factors for prognosis.METHODS:A retrospective analysis was performed on the medical records of the children who developed CA in the pediatric intensive care unit (PICU) of Hunan Children's Hospital from June 2016 to June 2021. According to the presence or absence of AKI within 48 hours after return of spontaneous circulation (ROSC) for CA, the children were divided into two groups: AKI (n=50) and non-AKI (n=113). According to their prognosis on day 7 after ROSC, the AKI group was further divided into a survival group (n=21) and a death group (n=29). The multivariate logistic regression analysis was used to investigate the risk factors for early AKI in the children with CA and the influencing factors for prognosis.RESULTS:The incidence rate of AKI after CA was 30.7% (50/163). The AKI group had a 7-day mortality rate of 58.0% (29/50) and a 28-day mortality rate of 78.0% (39/50), and the non-AKI group had a 7-day mortality rate of 31.9% (36/113) and a 28-day mortality rate of 58.4% (66/113). The multivariate logistic regression analysis showed that long duration of cardiopulmonary resuscitation (OR=1.164, 95%CI: 1.088-1.246, P<0.001), low baseline albumin (OR=0.879, 95%CI: 0.806-0.958, P=0.003), and adrenaline administration before CA (OR=2.791, 95%CI: 1.119-6.961, P=0.028) were closely associated with the development of AKI after CA, and that low baseline pediatric critical illness score (OR=0.761, 95%CI: 0.612-0.945, P=0.014), adrenaline administration before CA (OR=7.018, 95%CI: 1.196-41.188, P=0.031), and mechanical ventilation before CA (OR=7.875, 95%CI: 1.358-45.672, P=0.021) were closely associated with the death of the children with AKI after CA.CONCLUSIONS:Albumin should be closely monitored for children with ROSC after CA, especially for those with long duration of cardiopulmonary resuscitation, low baseline pediatric critical illness score, adrenaline administration before CA, and mechanical ventilation before CA, and such children should be identified and intervened as early as possible to reduce the incidence of AKI and the mortality rate.

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.

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.

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.