Effect of cardiopulmonary bypass on thiol/disulfide homeostasis in congenital heart surgery.

Background: This study aims to investigate whether thiol/disulfide homeostasis parameters measurements could be used as a new biomarker to predict the pre- and post-cardiopulmonary bypass oxidative status of pediatric patients undergoing congenital heart surgery. Methods: A total of 40 children with congenital heart disease (17 males, 23 females; mean age: 39.6±40.0 months; range, 2 to 216 months) who underwent open-heart surgery were included. The control group consisted of 40 age- and sex-matched healthy children (18 males, 22 females; mean age: 42.8±46.6 months; range, 12 to 156 months). The patients with congenital heart disease were divided into two groups as cyanotic patients (n=18) and acyanotic patients (n=22). Thiol/disulfide parameters were compared among the cyanotic, acyanotic congenital heart disease patients, and control group preoperatively (pre-CPB). The effects of cardiopulmonary bypass on thiol/disulfide parameters, pre-CBP, immediately after cardiopulmonary bypass (post-CPB0), and 24 h after cardiopulmonary bypass (post-CPB24) were investigated. Results: The mean native and total thiol levels in the cyanotic patients were significantly lower than those in the acyanotic patients and control group (p<0.0001). The cyanotic group exhibited higher disulfide levels than the acyanotic group (p<0.01). The mean native thiol and total thiol levels significantly decreased in the post-CPB0 (p<0.0001). The mean disulfide levels significantly increased in the post-CPB0 than the pre-CPB values (p<0.001). Post-CPB24 native and total thiol levels were elevated compared to post-CPB0 (p<0.0001). The mean disulfide levels significantly increased in the post-CPB24 period than the post-CPB0 values (p<0.001). The survivor patients responded better to oxidative stress than non-survivor patients. Conclusion: Thiol/disulfide measurement is a promising biomarker in determining the pre- and post-cardiopulmonary bypass oxidative status of pediatric patients undergoing congenital heart surgery. The interpretation of thiol/disulfide levels, pre- and postoperatively, may be used in predicting mortality and outcomes of these patients earlier.

Clinical characteristics and outcomes of continuous renal replacement therapy performed on younger children weighing up to 10 kg.

BACKGROUND: This study aimed to investigate the clinical features, modality, complications, and effecting factors on the survival of children weighing up to 10 kg who received continuous renal replacement therapy (CRRT). METHODS: This study was a retrospective observational study conducted in five pediatric intensive care units in tertiary hospitals in Turkey between January 2015 and December 2019. RESULTS: One hundred and forty-one children who underwent CRRT were enrolled in the study. The median age was 6 (range, 2-12)months, and 74 (52.5%) were male. The median weight of the patients was 6 (range, 4-8.35) kg and 52 (36.9%) weighed less than 5 kg. The most common indication for CRRT was fluid overload in 75 (53.2%) patients, and sepsis together with multiorgan failure in 62 (44%). The overall mortality was 48.2%. DISCUSSION: Despite its complexity, CRRT in children weighing less than 10 kg is a beneficial, lifesaving extracorporeal treatment modality.

Detrimental Results of COVID-19 Fear to Child Health.

The outbreak of coronavirus disease 2019 (COVID-19) and its consequences have led to fear and anxiety among individuals worldwide. The risk of coronavirus transmission frightens people more than any other health problem they face. Parents have concerns about being infected with COVID-19 and delay accessing hospitals even in an emergency which can be very detrimental to child health. Here, in this article, we would like to present eight patients delayed in admission to the hospital to draw attention to the harmful consequences of COVID-19 fear in the community. Although anxiety and fear are encouraging to take necessary precautions, exaggeration of these emotions may cause greater health problems.

Longitudinally extensive transverse myelitis in childhood: Clinical features, treatment approaches, and long-term neurological outcomes.

AIM: Longitudinally extensive transvers myelitis (LETM) is a rare and disabling condition in childhood. The aim of the present study was to share experiences from our center regarding the treatment features and clinical and radiologic course in our LETM patients in light of the literature data. MATERIAL AND METHOD: The study was designed as cross-sectional and included children who followed for LETM at our pediatric neurology clinic between 2010 and 2019. ATM was diagnosed according to the diagnostic criteria report from the Transverse Myelitis Consortium Working Group. LETM was defined as the presence of spinal cord lesions spanning a length of 3 or more consecutive vertebral segments. The patients' medical records were examined in terms of demographic characteristics, presenting symptoms, history of infection prior to and during LETM, prodromal history, neurological examination, laboratory and radiological findings, clinical course, and treatment. The Barthel Index was used to assess the physical independence in activities of daily living of patients with LETM who were followed for at least one year. RESULTS: A total of 15 (8 girl) patients were included in the study. The patients were between 1 and 17 years of age. Presenting symptoms included inability to walk in 12 patients, incontinence in 9 patients, low back pain in 4 patients, abdominal pain in 2 patients, and inability to use the arms in 2 patients. In Barthel Index assessment of physical independence in activities of daily living, 8 patients were evaluated as completely independent, 3 patients as moderately dependent, and 2 patients as slightly dependent. When the 4 patients with motor area impairment and moderate dependency according to the Barthel Index were examined, it was noted that all of them had been admitted 4 days after the onset of symptoms and that 2 (13.3%) had cervicothoracic involvement and 2 (13.3%) had involvement of the entire cord. CONCLUSION: Shorter delay from symptom onset to initiation of immunomodulatory therapy as well as effective rehabilitation resulted in favorable outcomes, with the most noticeable improvement in the areas of motor function and incontinence.

Effect of coronavirus pandemic on organ donation and transplantation in Turkey.

BACKGROUND: To the Editor, In February 2020, the Word Health Organization (WHO) announced the name for a disease caused by the SARS-CoV virus: coronavirus disease 2019 (COVID-19) 1. On March 11, 2020, the first case was officially confirmed in Turkey2, and the WHO declared COVID-19 a pandemic. DISCUSSION: The authors have no conflicts of interest to declare. The authors received no financial support for the research, authorship, and/or publication of this article.

The Burden of Burnout Syndrome in Pediatric Intensive Care Unit and Pediatric Emergency Department: A Multicenter Evaluation.

OBJECTIVE: The objective of this study was to detect variables associated with burnout syndrome (BS) in pediatric intensive care units (PICUs) and pediatric emergency medicine departments (PEDs) in high-volume centers from different parts of Turkey. METHODS: An observational, cross-sectional multicenter study was performed. The Maslach Burnout Inventory scale was administered to all of health care providers working in PICUs and PEDs. In this study, health care providers were defined as physicians, nurses, and other staff (secretaries, cleaning and patient care staff) working in PICU and PEDs. RESULTS: A total of 570 participants completed the survey. The major finding of this study was that 76.1% (n = 434) of PICU and PED health care professionals had BS. The most prominent subscale of BS was emotional exhaustion (62.5%). The rate of BS was higher among health care providers working in PEDs compared with PICUs (79.1% vs 73.7%, P = 0.04). The frequency of BS according to emotional exhaustion and depersonalization subscales was higher in health care providers of PEDs. The rate of BS was also significantly higher in younger employees, females, those working 51 or more hours totally in a week, those having a low monthly salary, those single or divorced, those without children, those with no childcare at home, those not owning a home, those not doing regular exercise and not having regular breakfast, those with total employment time of less than 1 year, and those not having a car or not having a hobby. In PEDs, when the daily evaluated number of patients was equal to or more than 44 (sensitivity, 88%; specificity, 66%), it predicted the occurrence of BS. In PICUs, when the number of patients cared for by 1 nurse was equal to or more than 3, it predicted the occurrence of BS (sensitivity, 78%; specificity, 62%). CONCLUSIONS: By creating early intervention programs to prevent BS, shortages of health care professionals can be avoided and the costs of health care expenditures related to infections can be decreased.

An intronic variant in BRAT1 creates a cryptic splice site, causing epileptic encephalopathy without prominent rigidity.

BRAT1-related neurodevelopmental disorders are characterized by heterogeneous phenotypes with varying levels of clinical severity. Since the discovery of BRAT1 variants as the molecular etiology of lethal neonatal rigidity and multifocal seizure syndrome (RMFSL, OMIM 614498), these variants have also been identified in patients with milder clinical forms including neurodevelopmental disorder with cerebellar atrophy and with or without seizures (NEDCAS, OMIM 618056), epilepsy of infancy with migrating focal seizures (EIMFS), and congenital ataxia (CA). This study aims to examine the consequences and pathogenicity of a novel homozygous splice site variant in BRAT1 in a patient presenting with migrating focal seizures since birth without prominent rigidity. The patient was born from a consanguineous marriage and has had seizures since the neonatal period. He presented with dysmorphic features, pontocerebellar hypoplasia, and migrating focal seizures. Despite supportive treatment, his symptoms rapidly progressed to intractable myoclonic seizures, bouts of apnea and bradycardia, and arrest of head growth, with no acquisition of developmental milestones. Clinical exome sequencing yielded a novel homozygous splice variant in BRAT1. Genetic analysis based on reverse transcription of the patient's RNA followed by PCR amplifications performed on synthesized cDNA and Sanger sequencing was undertaken, and the functional effect of a BRAT1 variant on splicing machinery was demonstrated for the first time. The severe clinical presentation of migrating focal seizures and pontocerebellar hypoplasia in the absence of rigidity further expands the genotypic and phenotypic spectrum of BRAT1-related neurodevelopmental disorders.

Comprehensive Analysis of Severe Viral Infections of Respiratory Tract admitted to PICUs during the Winter Season in Turkey.

OBJECTIVES: To analyze the course of seasonal viral infections of respiratory tract in patients hospitalized in pediatric intensive care units (PICU) of 16 centers in Turkey. MATERIALS AND METHODS: It is a retrospective, observational, and multicenter study conducted in 16 tertiary PICUs in Turkey includes a total of 302 children with viral cause in the nasal swab which required PICU admission with no interventions. RESULTS: Median age of patients was 12 months. Respiratory syncytial virus (RSV) was more common in patients over one year of age whereas influenza, human Bocavirus in patients above a year of age was more common (p <0.05). Clinical presentations influencing mortality were neurologic symptoms, tachycardia, hypoxia, hypotension, elevated lactate, and acidosis. The critical pH value related with mortality was ≤7.10, and critical PCO2 ≥60 mm Hg. CONCLUSION: Our findings demonstrate that patients with neurological symptoms, tachycardia, hypoxia, hypotension, acidosis, impaired liver, and renal function at the time of admission exhibit more severe mortal progressions. Presence of acidosis and multiorgan failure was found to be predictor for mortality. Knowledge of clinical presentation and age-related variations among seasonal viruses may give a clue about severe course and prognosis. By presenting the analyzed data of 302 PICU admissions, current study reveals severity of viral respiratory tract infections and release tips for handling them. HOW TO CITE THIS ARTICLE: Kockuzu E, Bayrakci B, Kesici S, Citak A, Karapinar K, Emeksiz S, et al. Comprehensive Analysis of Severe Viral Infections of Respiratory Tract admitted to PICUs During the Winter Season in Turkey. Indian J Crit Care Med 2019;23(6):263-269.

Brain Death and Organ Donation in Paediatric Intensive Care Unit.

OBJECTIVE: The purpose of the present study was to retrospectively analyse the brain death (BD) cases that were specified within the last 8 years in the paediatric intensive care unit of our hospital. METHODS: Archive files and computer records of 23 paediatric cases were analysed. Data on age, gender, conditions that caused BD, paediatric risk of mortality (PRISM III) scores, time between suspicion of BD and issuing of BD report, confirmatory tests used, complications that occurred following the diagnosis of BD and time to cardiac arrest development after diagnosis of BD were recorded. RESULTS: The average age of the patients was 6.8±5.5 years. The most frequent cause of BD was intracranial haemorrhage (30.4%). The mean time to diagnosis after BD suspicion was 5.9±6.2 days. Electroencephalography was performed in 61% of the patients in addition to the apnoea test. Radiological imaging methods were used in 39% of the patients (n=9). Of the cases, 34.7% developed hypothermia, and 4.3% developed diabetes insipidus (DI). Among them, 43.4% had both DI and hypothermia. The mean PRISM score was calculated as 22±9.2. The donation rate of the families was 17%. The mean time to cardiac arrest development after diagnosis of BD was 6.9±7.4 days in non-donor cases where medical support had been reduced. CONCLUSION: Any patient with a neurologically poor prognosis in the intensive care unit should be considered to develop BD and diagnosed with BD without delay. The donation rate will increase if family interviews are done by an experienced and educated coordinator.

Effects of Hemodialysis on Thiol-Disulphide Homeostasis in Critically Ill Pediatric Patients with Acute Kidney Injury.

AIM: To evaluate thiol/disulphide homeostasis as a new indicator of oxidative stress in AKI patients and to determine the effect of HD on antioxidant balance and oxidative stress through plasma thiols. METHODS: This study was performed in patients aged between 12 months and 18 years prospectively who underwent hemodialysis due to AKI and were followed up for a year in a 22-bed tertiary pediatric intensive care unit. 20 patients and 39 controls were included. RESULTS: No difference was present between the groups in terms of age and gender. Median values of plasma native thiol, total thiol, and percent thiol were significantly lower in AKI group both before and after dialysis when compared to control group. The median dynamic disulphide values were significantly lower in the AKI group of predialysis compared to the controls. When pre- and postdialysis values were compared, disulphide values were statistically higher after dialysis. When pre- and postdialysis native thiol, dynamic disulphide, total thiol, and percent thiol median values were compared, postdialysis values were significantly higher than the predialysis values. There was a positive correlation between albumin, total thiol, and native thiol values before dialysis in the patient group. CONCLUSION: AKI patients have low levels of thiol species showing the presence of oxidative stress and hemodialysis has a positive effect on thiol/disulphide balance. This new method may be an inexpensive and simple tool suitable for clinical studies and can be used in routine screening as a useful indicator to show oxidative stress.

Reversible Dilated Cardiomyopathy Due to Combination of Vitamin D-Deficient Rickets and Primary Hypomagnesemia in an 11-Month-Old Infant.

Vitamin D-deficient rickets is still an important and common health problem in developing countries. Since calcium is an essential ion for cardiac muscle contraction, calcium deficiency caused by rickets can cause secondary dilated cardiomyopathy. This situation can be exacerbated by coexisting hypomagnesemia. Here, we report a case of dilated cardiomyopathy due to hypocalcemia induced by vitamin D-deficient rickets and accompanying primary hypomagnesemia in an infant whose cardiomyopathy was successfully treated by replacement of calcium, vitamin D, and magnesium. In addition to genetic causes, viral infections, and idiopathic factors, metabolic abnormalities are important etiologic factors in pathogenesis of dilated cardiomyopathy and since they are treatable, prompt diagnosis of these disorders is crucial.

Transarterial and Transhepatic Endovascular Intervention to Alleviate Portal Hypertension Secondary to Arterioportal Fistula in a Trisomy 21 Infant.

Individuals with trisomy 21 have an 80% risk reduction of vascular anomalies compared with general population. However, an association of trisomy 21 and portal vascular and arteriovenous anomalies has been defined in the literature. The primary hemodynamic abnormality in portal hypertension is increased resistance to portal blood flow. In various case reports in adults and pediatric age group patients, transarterial coil embolization of hepatoportal fistula was described. One of the authors of this article has previously reported successful treatment of congenital arterioportal fistula (APF) with percutaneous transhepatic liquid embolization in a patient who previously had transarterial coil embolization. To date, eight patients with trisomy 21 (Down syndrome) and congenital portosystemic shunts were reported of which four were treated with embolization. Here, we describe a 3-month-old infant with trisomy 21 and intrahepatic APF associated with extrahepatic portal hypertension and massive ascites. In the current report, a rare case of a patient with a diagnosis of trisomy 21 is discussed who was attempted to be treated with transarterial coil embolization and percutaneous transhepatic liquid embolization of the congenital APF in a single session.