Downregulation of the immunoproteasome subunit PSMB8 attenuates sepsis-associated acute kidney injury through the NF-κB pathway.

Sepsis-associated acute kidney injury (S-AKI) is a prevalent and life-threatening complication in hospitalized and critically ill patients. Recent researches indicates that immunoproteasome, especially proteasome 20S subunit beta 8 (PSMB8), is highly associated with various kidney diseases. This study aims to investigate the potential involvement of PSMB8 in S-AKI and its impact on apoptosis and inflammation.

QSOX1 exerts anti-inflammatory effects in sepsis-induced acute lung injury: Regulation involving EGFR phosphorylation mediated M1 polarization of macrophages.

Sepsis is a systemic inflammatory response caused by an infection, which can easily lead to acute lung injury. Quiescin Q6 sulfhydryl oxidase 1 (QSOX1) is a sulfhydryl oxidase involved in oxidative stress and the inflammatory response. However, there are few reports on the role of QSOX1 in sepsis-induced acute lung injury (SALI).

Relation between the time of the transport team in reaching the bedside of children and the 30-day mortality rate after admission.

Background: China's medical system has not yet issued quality control indicators related to the transfer of critically ill children, and when transport teams receive a transfer request for these children, due to various reasons, the time to arrive at the bedside varies. The aim of this study was to investigate the effect of the time taken by the pediatric intensive care transport team to reach the bedside of children after receiving a transport request on the prognosis of these children.

Methods: Clinical data of 298 critically ill children admitted to Anhui Children's Hospital through long-distance transport from March 2020 to February 2022 were retrospectively analyzed.

A Case of Ulcerative Colitis Induced by Paraneoplastic Syndrome?

Background: Paraneoplastic syndromes often cause endocrine, neurological, cutaneous, and hematologic pathologies, and cases with digestive symptoms as prominent cases are rare.

Case Description: A 1-year-old child admitted to the emergency department with severe abdominal distension was later diagnosed with sacrococcygeal yolk cystoma with ulcerative colitis. After symptomatic management, surgical removal of the tumor, and JEB chemotherapy, the symptoms of ulcerative colitis disappeared completely.

MOGS-CDG: Quantitative analysis of the diagnostic Glc Man tetrasaccharide and clinical spectrum of six new cases.

Congenital disorders of glycosylation (CDG) are a clinically and biochemically heterogeneous subgroup of inherited metabolic disorders. Most CDG with abnormal N-glycosylation can be detected by transferrin screening, however, MOGS-CDG escapes this routine screening. Combined with the clinical heterogeneity of reported cases, diagnosing MOGS-CDG can be challenging.

Ascorbic acid and hydrocortisone synergistically inhibit septic organ injury via improving oxidative stress and inhibiting inflammation.

Background: The current study aimed to investigate the effect of the combination of ascorbic acid (AscA) and hydrocortisone (Hyd) on septic organ injury and its potential mechanism.

Method: Sepsis was induced in mice by a single intraperitoneal injection of lipopolysaccharides.

Results: AscA and Hyd combined showed more effective protection of the injured liver and kidney in septic mice by decreasing alanine aminotransferase, aspartate aminotransferase, serum urea nitrogen, and serum creatinine and ameliorating pathological manifestations than Hyd or AscA alone.

Epidemiology of Cardiopulmonary Arrest and Outcome of Resuscitation in PICU Across China: A Prospective Multicenter Cohort Study.

Objective: To investigate the epidemiology and the effectiveness of resuscitation from cardiopulmonary arrest (CPA) among critically ill children and adolescents during pediatric intensive care unit (PICU) stay across China.

Methods: A prospective multicenter study was conducted in 11 PICUs in tertiary hospitals. Consecutively hospitalized critically ill children, from 29-day old to 18-year old, who had suffered from CPA and required cardiopulmonary resuscitation (CPR) in the PICU were enrolled (December 2017-October 2018).

Assessment of continuous neutrophil CD64 index measurement for diagnosing sepsis and predicting outcome in a Chinese pediatric intensive care unit: a prospective study.

Background: The high affinity immunoglobulin-Fc fragment receptor I CD64 on neutrophils is widely assumed to be a useful biomarker in the early identification of sepsis, and it improves outcomes. We aimed to determine its ability to diagnose sepsis and predict its prognosis with continuous measurements.

Methods: A total of 335 patients admitted to a Chinese PICU were prospectively stratified into two groups according to the presence of sepsis (defined by clinical criteria for sepsis) between 2018 and 2019.

A rare case of Pseudomonas aeruginosa bacteremia in a newborn with 58 perforations in the small intestine.

Background: Community-acquired infections of Pseudomonas aeruginosa (P. aeruginosa) occur very rarely.

Case Presentation: P.

Impact of and risk factors for pediatric acute kidney injury defined by the pROCK criteria in a Chinese PICU population.

Background: The definition of pediatric AKI continues to evolve. We aimed to find a better AKI definition to predict outcomes and identify risk factors for AKI in a Chinese PICU.

Methods: This study consisted of 3338 patients hospitalized in a Chinese PICU between 2016 and 2018.

IDDCA syndrome in a Chinese infant due to GNB5 biallelic mutations.

Herein, we present a Chinese infant with an early-onset intellectual developmental disorder with cardiac arrhythmia syndrome. A 6-month-old boy visited our hospital because of convulsions and paroxysmal cyanosis for 1 day. Mental development analysis showed that the patient had a neurodevelopmental delay.

Fatal congenital tuberculosis owing to late diagnosis of maternal tuberculosis: case report and review of congenital tuberculosis in China.

Congenital tuberculosis (TB) is rare and the prognosis is poor if not detected early. The diagnosis is often delayed owing to non-specific clinical presentation, misdiagnosis and undiagnosed maternal TB during pregnancy. A 12-day-old girl presented with a 5-day history of fever, cough, poor feeding and respiratory distress.

[Risk factors and prognosis of acute kidney injury in children with sepsis in pediatric intensive care unit].

Objective: To investigate the risk factors and prognosis of acute kidney injury (AKI) in children with sepsis in pediatric intensive care unit (PICU).

Methods: A retrospective analysis of clinical data of PICU sepsis children in Anhui Children's Hospital from May 2015 to May 2018 was performed. The children were divided into AKI group and non-AKI group according to whether AKI occurred within 48 hours of PICU [referring to the diagnostic criteria for Kidney Disease: Improving Global Outcomes (KDIGO)].

Compound heterozygous variants in MOGS inducing congenital disorders of glycosylation (CDG) IIb.

This study is to present two Chinese siblings who were diagnosed with congenital disorders of glycosylation (CDG) IIb because of mannosyl-oligosaccharide glucosidase (MOGS) deficiency. The siblings visited our hospital due to "pulmonary infection". Facial dysmorphism including long eyelashes, blepharophimosis, depressed nasal bridge, and high palate was noted.

[Etiology and prognosis of thrombocytopenia in children: a case analysis in 683 children within 10 years of pediatric intensive care unit].

Objective: To analyze the etiology and prognosis of children with thrombocytopenia (TP) in pediatric intensive care unit (PICU).

Methods: The data of children with TP (exclusion of congenital and unknown TP) admitted to PICU of Anhui Provincial Children's Hospital from January 2008 to December 2017 was analyzed retrospectively. According to the age of onset, the children were divided into infantile group (29 days to less than 1 year), early childhood group (1 to less than 3 years), preschool group (3 to less than 6 years), school age group (6 to less than 10 years) and puberty group (more than 10 years).

Whole-exome Sequencing Helps the Diagnosis and Treatment in Children with Neurodevelopmental Delay Accompanied Unexplained Dyspnea.

Neurodevelopmental delay accompanied unexplained dyspnea is a highly lethal disease in clinic. This study is to investigate the performance characteristics of trio whole exome sequencing (Trio-WES) in a pediatric setting by presenting our patient cohort and displaying the diagnostic yield. A total of 31 pediatric patients showing neurodevelopmental delay accompanied unexplained dyspnea were admitted to our hospital and referred for molecular genetic testing using Trio-WES.

[Effects of different sedation regimens on sedation and inflammatory response in critically ill children with multiple trauma].

Objective: To compare the sedation and anti-inflammatory effects of dexmedetomidine and midazolam on critical ill children with multiple trauma.

Methods: A prospective randomized controlled trial was conducted. Sixty-five critical ill children with multiple trauma admitted to pediatric intensive care unit (PICU) of Anhui Province Children's Hospital from January 2014 to September 2016 were enrolled, who were randomly divided into dexmedetomidine group (33 cases) and midazolam group (32 cases).

Novel NFU1 Variants Induced MMDS Behaved as Special Leukodystrophy in Chinese Sufferers.

Multiple mitochondrial dysfunctions syndrome (MMDS) is an autosomal recessive disorder of systemic energy metabolism. This study is to present the diagnosis of two MMDS Chinese sufferers. Physical and auxiliary examination was performed.

[Clinical characteristics and genetic analysis of two cases with Leigh syndrome with acute pulmonary hemorrhage as predominant manifestation].

Objective: To analyze clinical and imaging features and genetic characteristics of Leigh syndrome with emergent pulmonary edema.

Method: The clinical features and imaging data of 2 cases (1 male, 1 female) seen in Anhui Provincial Children's Hospital from 2012 to 2014 were analyzed and summarized. Venous blood samples were sent to Guangzhou Jinyu Medical Examination Center for genetic analysis.

[Report of a case with late-onset ornithine transcarbamylase deficiency with gas chromatography-mass spectrometry and DNA sequencing confirmation and literatures review].

Objective: To discuss the clinical characteristics, diagnosis and treatment management of ornithine transcarbamylase deficiency (OTCD).

Method: Data of the clinical diagnosis and treatment of a case with OTCD were analyzed, and the domestic and international literature was reviewed.

Result: (1) The case was a boy, 8 years old; and the initial symptoms were vomiting and reduced consciousness for a day after eating a lot of eggs as previous similar history.

[Analysis of serious respiratory complications in PICU in children with measles].

Objective: To evaluate the characteristics of the serious complications of 17 cases with measles in ICU.

Method: Seventeen cases with measles with serious complications in ICU in Anhui Provincial Children's Hospital were recruited from May 2012 to May 2013. Clinical characteristics, image finding, and prognosis were analyzed retrospectively.

[Negative pressure pulmonary edema with upper airway obstruction: analysis of 3 patients].

Objective: To investigate the clinical characteristics and treatment of negative pressure pulmonary edema (NPPE) with upper airway obstruction (UAO) in children.

Method: Data of 3 cases with NPPE and UAO in pediatric intensive care unit (PICU) from Mar, 2007 to May, 2013 were analyzed.

Result: (1) Two cases were male and 1 was female with age respectively 6, 16 and 30 months.