Mortality Rate In Pediatric Patients Research Articles

Whole exome sequencing identifies variants in the \(\textit{TNNI3}\) gene in vietnamese patient with restrictive cardiomyopathy - a case report

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease in which the heart wall is rigid leading to diastolic dysfunction caused by abnormal elastic properties of the myocardium and/or intercellular matrix. The prognosis is generally poor, and RCM has a high mortality rate in pediatric patients. There are no curative treatments for RCM, so cardiac transplantation is the only effective treatment. Diagnosis RCM, clinical diagnosis can be challenging because clinical presentations and imaging manifestations of RCM are similar to other cardiomyopathies so it requires other specific diagnoses. Currently, pathogenic mutations in 22 different genes have been identified in patients with RCM. Identifying mutations in these genes helps discriminate RCM from other cardiomyopathies. Besides, next-generation sequencing (including whole genome sequencing, whole exome sequencing,...) has provided an effective tool for simultaneously analyzing mutations in many different genes. In this study, we conducted sequencing of the entire gene coding region (WES) in the patient and identified a compound heterozygote variants (c.289C>G, p.Arg97Gly and c.433C>T, p.Arg145Trp) in the TNNI3 gene. These variants were inherited from the patient's father and mother, who were heterozygous variant carriers. These variants were also identified as the pathogenic variants in the ClinVar database (accession number VCV001331910.2 and VCV000012426.28, respectively) and were the cause of the patient's disease. Our results suggest that WES can be used to definitively diagnose the genetic variants associated with RCM and show that genetic screening is essential for families of RCM patients.

Abstract 237: Effect of Hospital Arrival Time on Mortality Rate in Pediatric Patients With Severe Trauma: A Nationwide Database Study in Japan

Introduction: Previous studies have reported that hospital arrival during nighttime was associated with worse outcomes in critically ill pediatric patients. However, this “nighttime effect” in pediatric patients with severe trauma has not yet been well-investigated. Hypothesis: We hypothesized that hospital arrival during nighttime is associated with higher mortality rates in pediatric patients with severe trauma. Methods: We conducted a nationwide retrospective cohort study in Japan from 2004 to 2019 using the Japan Trauma Data Bank, a nationwide trauma registry. Patients who were aged <18 years and had an Injury Severity Score (ISS) >15 were included. Patients with cardiac arrest on hospital arrival were excluded. Hospital arrival was categorized into daytime (from 8:00 am to 4:59 pm) arrival and nighttime (from 5:00 pm to 7:59 am) arrival. The main outcome was the in-hospital mortality rate, and the secondary outcome was the emergency department (ED) mortality rate. As a main analysis, an inverse probability of treatment weighting (IPTW) analysis was performed using propensity scores to adjust for confounders such as age, sex, type of injury, transport time, vital signs, and ISS. In addition, we conducted 1) a subgroup analysis in patients who underwent operation (OP) or interventional radiology (IR) during hospitalization and 2) sensitivity analysis using a multilevel mixed-effects logistic regression model. Results: In total, 6,574 pediatric patients with severe trauma were included, and the overall in-hospital mortality rate was 6.5%. The IPTW analysis showed that compared with daytime arrivals, nighttime arrivals were associated with significantly higher in-hospital mortality (odds ratio [OR], 1.41; 95% confidence interval [CI], 1.09-1.83; P = 0.010) and ED mortality rates (OR, 2.42; 95% CI, 1.07-5.45; P = 0.034) in the entire cohort but not in the subgroup who underwent OP or IR during hospitalization. The sensitivity analysis consistently supported the results of the main analysis. Conclusions: Hospital arrival during nighttime was associated with a higher in-hospital mortality rate in pediatric patients with severe trauma. Further investigations are needed to elucidate the reasons for this “nighttime effect.”

The Platelet-to-lymphocyte ratio, PELOD-2 score, and mortality rate in pediatric sepsis

Background Sepsis is life-threatening organ dysfunction caused by a regulated immune response to infection. Sepsis remains the most common cause of death in infants and children worldwide. The Pediatric Logistic Organ Dysfunction (PELOD-2) score, one of the most widely used scoring systems in pediatric sepsis patients, has been shown to be accurate in predicting mortality. The platelet-to-lymphocyte ratio (PLR) is a new clinical indicator of inflammation in a variety of diseases including sepsis.
 Objective To investigate the relationship between PLR, PELOD-2 score, and clinical outcomes in pediatric patients with sepsis.
 Methods This retrospective cohort study was conducted in the Pediatric Intensive Care Unit (PICU), Prof R.D. Kandou Hospital, Manado, North Sulawesi, from February to August 2020. Subjects’ PELOD-2 score and PLR were recorded once within the first 24 hours of PICU admission. We analyzed patients’ PELOD-2 score, PLR, and mortality rate, with 95% confidence interval (CI) for each value.
 Results Of 96 children with sepsis admitted to the PICU during the study period, 87 patients (46 boys; 52.9%) met the inclusion criteria. In total, 50 (57.47%) patients were non-survivors. Mean PLR values among survivors [77.54 (SD 50.08)] was significantly lower compared to the values among non-survivors [157.13 (SD 67.38)]; as well as the PELOD-2 score in the survivors group [12 (SD 1.32)] was significantly lower than its value in the non-survivors [14.65 (SD 2.09)]. Spearman’s analysis showed a moderately positive correlation between PLR and PELOD-2 score (r=0.444; 95%CI 0.44 to 1; P<0.01). Biserial point correlation analysis revealed a significant association between PLR and mortality rate (rpb=0.566; P<0.0001),with elevated PLR related to an increased risk of mortality.
 Conclusion There are positive correlations between PLR, PELOD-2 score, and mortality rate in pediatric patients with sepsis. Higher PLR and PELOD-2 score are associated with higher mortality.

Invasive candidiasis in children

The prevalence of invasive candidiasis (IC) in pediatric hospitals is from 4,3 to 15,2 per 10,000 hospitalized, in ICU – from 3,5 to 7 cases per 1,000, with HSCT – 2,9%. The average length of stay of a patient in the hospital before the development of IC varies from 21 to 56 days, in the ICU – more than 15 days. Knowledge of risk factors (ICU stay for ≥15 days, use of antibacterial drugs and parenteral nutrition, active malignant neoplasm, etc.) makes it possible to identify patients with a high (10-46%) risk of developing IC. Candida albicans remains the leading causative agent of IC in children, but infections with non-albicans Candida spp. have increased and an increase in the resistance of IC pathogens to azole antimycotics was noted. The main clinical variant of IC in children is candidemia, other forms include the central nervous system, abdominal organs, eyes, heart, bones and joints, kidneys, skin and subcutaneous tissue involvement, as well as chronic disseminated (hepatolienal) candidiasis. Blood culture, the main method of laboratory diagnostics of IC, is characterized by low sensitivity and requires a long time. Methods of noncultural diagnostics of IC (1,3-β-D-glucan, mannan and antimannan antibodies, T2 Candida etc) in children have not been sufficiently studied. The main drugs for the treatment of IC in children are echinocandins (anidulafungin, etc.), and CVC removal/replacement is necessary. The overall mortality rate in pediatric patients within 30 days after the diagnosis of IC is 37% to 44%.

Chemical pleurodesis and somatostatin in treating spontaneous chylothorax in pediatric patients: a retrospective analysis and review of the literature.

BackgroundChylothorax is a rare disease, defined as an abnormal accumulation of chylous lymphatic effusion in thoracic cavity, with a high mortality rate in pediatric patients. At present, there are few studies on the treatment of pediatric chylothorax, and conservative treatments like somatostatin (SST) and pleurodesis are performed empirically. SST has been used for treating pediatric chylothorax over 20 years, and povidone-iodine chemical pleurodesis (PICP) is adopted in recent years with a high cure rate, but both the effect are still uncertain. The safety and efficacy of SST and PICP in treating pediatric chylothorax was compared in this study.MethodsFrom January 2009 to May 2020, 27 pediatric chylothorax patients who accepted life support, SST and PICP treatment were retrospectively reviewed in this study. Their medical history, clinical manifestations, pleural effusion examinations, treatment methods, effects, and complications were screened. PICP and SST was performed in 19 and 8 children, respectively. Each patient was followed up after discharge from the hospital.ResultsAfter admission, chest X-ray and ultrasound diagnosed pleural effusion in 27 patients (18 males and 9 females), including 13 and 6 cases on the left and right side, and 8 cases on both sides. In the pleural effusion, the mean leukocyte count was (9,826±9,482)×106/L, the mean lymphocyte ratio was (84.82±6.58)%, the mean triglyceride content was 7.11±6.63 mmol/L. In PICP and SST group, the mean length of stay was 38.42±19.42 and 35.13±12.72 d (P=0.664), the mean time of thoracic drainage was 24.58±16.34 and 19.63±8.88 d (P=0.440), the mean time of parenteral nutrition was 27.16±18.29 and 25.25±13.52 d (P=0.793), respectively. PICP was performed for 3.16±2.27 times, and SST was used for 14.75±9.08 d with the dosage of 0.5 to 10 µg/kg/hour. After the treatment, all patients in PICP group had mild chest pain and low-grade fever, and 12 cases were observed with transient decrease of oxygen saturation. Eight patients in SST group had nausea and vomit, and 1 child had diarrhea simultaneously. Both were cured by symptomatic treatment, thoracic drainage and discontinuing using SST. Three children were diagnosed with refractory chylothorax and cured by PICP after SST. Pleural effusion in 27 patients was finally absorbed. All of them achieved a clinical cure successfully, and there was no recurrence during discharge follow-up.ConclusionsPICP has a high success rate and few adverse reactions in treating spontaneous chylothorax in pediatric patients, expected to be a new treatment option for pediatric chylothorax after SST.

Povidone-iodine chemical pleurodesis in treating spontaneous chylothorax in pediatric patients.

Chylothorax is a rare disease with a high mortality rate in pediatric patients like newborns and infants. As one of the minimally invasive treatments, chemical pleural pleurodesis has been gradually used in treating pediatric chylothorax patients in recent years. This study explored the feasibility, safety and effect of povidone-iodine chemical pleurodesis (CP) for spontaneous chylothorax in young pediatric patients. From January 2009 to December 2019 the clinical data of 22 children treated for spontaneous chylothorax at Guangdong Second Provincial General Hospital were retrospectively analyzed. The data included the patients' medical history, clinical manifestations, pleural effusion examinations, treatment methods, and effects and complications. On the basis of conventional conservative treatment and closed thoracic drainage, all the children were treated by CP with povidone-iodine until the pleural effusion was absorbed and the thoracic tube was removed. After discharge from the hospital, each patient was followed up for 1-10 years. Of the 22 children (16 males and 6 females), 3 cases were diagnosed with pleural effusion at prenatal examination, 5 cases had acute respiratory distress at birth, and 6 case had experienced pneumonia repeatedly since birth. All of the children had different degrees of cyanosis and dyspnea. Pleural effusion was diagnosed in all patients by chest X-ray and chest ultrasound after admission (12 cases on the left side, 5 cases on the right side, and 5 cases on both sides). The results of all chyle tests during pleural effusion examination were positive. The mean leukocyte count was (9,278.8±9,504.6)×106 /L, the lymphocyte ratio was (83.9±6.1)%, and the mean triglyceride content was (7.18±6.10) mmol/L. All patients were treated with thoracic drainage, diet control, nutritional support, and povidone-iodine CP, among 6 of them with pneumonia on admission received antibiotics. The mean length of stay (LOS) was (36.0±18.2) days. The patients received thoracic drainage for (23.0±15.6) days and pleurodesis (3.0±2.0) times. No serious side effects occurred after pleurodesis, although some patients experienced mild chest pain and low fever. All the patients successfully achieved a clinical cure. Povidone-iodine CP is a safe, effective, and minimally invasive treatment, with high success rate and few complications in young pediatric patients with spontaneous chylothorax.

Common challenges and coping strategies in pediatric anesthesia

Background Progress in pediatrics, anesthesiology, apparatus for anesthesia, and computerized technology promotes the development of pediatric anesthesia. But pediatric anesthesia currently lags far behind adult anesthesia as the incidence of postoperative complications and mortality rate in pediatric patients is still higher than those in adult patients. Objective To review the recent advancement in pediatric anesthesiology and provide guidance for the practice of pediatric anesthesia. Content Recent radiological imaging and bronchoscopy studiesreveal the location and shape of the narrowest part of children's trachea. This finding provides anatomical guidance for appropriate performance of balloon catheter intubation.The treatment of obtrusive sleep apnea(OSA) in pediatric patients requires tonsillectomy. In these cases, dexamethasone, serotonin receptor blockers and metoclopramide can alleviate postoperative complications. Airway management is more challenging in pediatric anesthesia than in adult anesthesia and requires tranquilizer treatment and real-time monitoring to avoid adverse reactions. Appropriate perioperative anesthesia and analgesia management may effectively control emergence agitation. Neurotoxicity of general anesthetics is another important concern for pediatric anesthesia. Trend Further clinical investigations are required to optimize intervention strategies to prevention adverse outcomes in pediatric anesthesia and to clarify the cause-effect relationship between anesthesia and neurodevelopmental deficits. Key words: Pediatric; Anesthesia; Airway management; Obstructive sleep apnea hyponea; Emergence igitation; Neurotoxicity

Mortality in Children With Early-Detected Congenital Central Hypothyroidism.

Approximately 60% to 80% of patients with congenital central hypothyroidism (CH-C) have multiple pituitary hormone deficiencies (MPHDs), making CH-C a potentially life-threatening disease. Data on mortality in patients with CH-C are lacking. To study the mortality rate in pediatric patients with early-detected and treated CH-C in the Netherlands and to investigate whether causes of death were related to pituitary hormone deficiencies. Overall mortality rate, infant mortality rate (IMR), and under-5 mortality rate were calculated in all children with CH-C detected by neonatal screening between 1 January 1995 and 1 January 2013. Medical charts were reviewed to establish causes of death. A total of 139 children with CH-C were identified, of which 138 could be traced (82 with MPHD, 56 with isolated CH-C). Total observation time was 1414 years with a median follow-up duration of 10.2 years. The overall mortality rate was 10.9% (15/138). IMR and under-5 mortality rate were 65.2/1000 (9/138) and 101.4/1000 (14/138), respectively, compared with an IMR of 4.7/1000 and under-5 mortality of 5.4/1000 live-born children in the Netherlands during the same time period (P < 0.0001). Main causes of death were severe congenital malformations in six patients, asphyxia in two patients, and congenital or early neonatal infection in two patients. Pituitary hormone deficiency was noted as cause of death in only one infant. We report an increased mortality rate in patients with early-detected CH-C that does not seem to be related to endocrine disease. This suggests that mortality due to pituitary insufficiency is low in patients with early-detected and early-treated CH-C.

Early referral is essential for successful pediatric small bowel transplantation: The Canadian experience

Purpose: The mortality rate for pediatric patients on the waiting list for transplantation has a major impact on the overall effectiveness of pediatric small bowel transplantation. This review was undertaken to determine the fate of Canadian children assessed for small bowel transplant and the outcome of those who undergo transplant in the tacrolimus era. Methods: The authors reviewed retrospectively all of the pediatric small bowel patients listed since 1988 through the Canadian Organ Replacement Register and all the children referred to our program in its first year. All children who received a small bowel transplant between January 1993 and December 1999 also were reviewed. Results: The mortality rate for pediatric patients on the small bowel transplant list was 53% after an average of 105 days on the list compared with 212 days for those who underwent transplant. Patients who died while on the list were younger and had signs of advanced liver disease at the time of listing. Thirteen Canadian children have received a small bowel transplant with an overall 1-year patient and graft survival rate of 61% and 53%, respectively. Survivors are all independent from total parenteral nutrition. Conclusion: Many Canadian children miss their opportunity for a successful small bowel transplant because of late referrals and a shortage of donor organs. J Pediatr Surg 36:681-684. Copyright © 2001 by W.B. Saunders Company.