Case Report of a Successful Pediatric Central Venovenous Extracorporeal Life Support via Right Atrium-Pulmonary Artery Cannulation for Severe Chest Trauma and Hemorrhagic Shock.

Hypoxemic arrests due to severe traumatic pulmonary injury may not respond to usual medical support. Extracorporeal life support (ECLS) can be life-saving; adequate flows are needed in this setting along with a careful choice of anticoagulation strategies to minimize bleeding. A 44 month old child, who presented with severe blunt chest trauma after being run over by a truck was resuscitated with active compressions and code medications seven times before arrival to the intensive care unit. Failure to adequately oxygenate led to an unconventional approach with cannulations of the right atrium (RA) and pulmonary artery (PA) via sternotomy. Anticoagulation regimen started after bleeding had slowed down with active transfusion of blood products and consisted of low-dose heparin and alprostadil infusions for the initial 48 hours. Heparin was adjusted per institutional guidelines thereafter. Physiologically veno-venous central approach enabled fast stabilization due to full oxygenation without recirculation. Additionally, the dual anticoagulation regimen was safe for circuit maintenance. The described method can be considered in small children with similar risks and conditions.

Influenza Virus Associated Pediatric Acute Respiratory Distress Syndrome: Clinical Characteristics and Outcomes.

BACKGROUND AND OBJECTIVE: The aim of this multicenter retrospective study was to determine the clinical characteristics, treatment approaches and the course of pediatric acute respiratory distress syndrome (PARDS) which developed associated with the influenza virus in the 2019-20 season. METHODS: Patients included 1 month to 18 years who were diagnosed with PARDS associated with the influenza virus in the 2019-20 season. RESULTS: Sixty-seven patients were included in the study. The mean age of the patients was 64.16 ± 6.53 months, with 60% of the group <5 years. Influenza A was determined in 54 (80.5%) patients and Influenza B in 13 (19.5%). The majority of patients (73.1%) had a comorbidity. Fifty-eight (86.6%) patients were applied with invasive mechanical ventilation, Pediatric Acute Lung Injury Consensus Conference classification was mild in 5 (8.6%), moderate in 22 (37.9%) and severe in 31 (52.5%) patients. Ventilation was applied in the prone position to 40.3% of the patients, and in nonconventional modes to 24.1%. A total of 22 (33%) patients died, of which 4 had been previously healthy. Of the surviving 45 patients, 38 were discharged without support and 7 patients with a new morbidity. CONCLUSION: Both Influenza A and Influenza B cause severe PARDS with similar characteristics and at high rates. Influenza-related PARDS cause 33% mortality and 15.5% morbidity among the study group. Healthy children, especially those aged younger than 5 years, are also at risk.

A Rare Cause of Hypophosphatemia: Raine Syndrome Changing Clinical Features with Age.

Raine Syndrome (RS) is caused by biallelic loss-of-function mutations in FAM20C gene and characterized by hypophosphatemia, typical facial and skeletal features. Subperiosteal bone formation and generalized osteosclerosis are the most common radiological findings. Here we present a new case with RS. A 9-month-old male patient on a home-type ventilator was referred for hypophosphatemia. He was born with a weight of 3800 g to non-consanguineous parents. Prenatal ultrasound had demonstrated nasal bone agenesis. A large anterior fontanel, frontal bossing, exophthalmos, hypoplastic nose, high arched palate, low set ears, triangular mouth, and corneal opacification were detected on physical examination. Serial skeletal X-rays revealed diffuse osteosclerosis at birth which was gradually decreased by the age of 5 months with subperiosteal undermineralized bone formation and medullary space of long bone could be distinguishable with bone-within-a-bone appearance. At 9 months of age, hand X-ray revealed cupping of the ulna with loose radial bone margin with minimal fraying and osteopenia. Cranial computed tomography scan showed bilateral periventricular calcification and hydrocephalus in progress. The clinical, laboratory, and radiological examinations were consistent with RS. Molecular analyses revealed a compound heterozygous mutation in FAM20C gene (a known pathogenic mutation, c.1645C > T, p.Arg549Trp; and a novel c.863 + 5 G > C variant). The patient died due to respiratory failure at 17 months of age. This case allowed us to demonstrate natural progression of skeletal features in RS. Furthermore, we have described a novel FAM20C variant causing RS. Previous literature on RS is also reviewed.

Pediatricians' Attitude in Management of Acute Bronchiolitis: Did Guidelines Overcome Practices?

Background: Acute bronchiolitis is one of the most common diseases of early childhood. There are many recent changes in the treatment of acute bronchiolitis. The aim of this study is to evaluate treatment approaches to acute bronchiolitis among clinicians and to observe compliance with clinical guidelines. Materials and Methods: Our study was designed as a multicenter cross-sectional descriptive study. A cohort of pediatric residents, fellows, and attendants were surveyed with a questionnaire including general and occupational characteristics of pediatricians and treatment choices in acute bronchiolitis. Results: A total of 713 questionnaires were collected. Most commonly applied treatment among pediatricians was inhaled salbutamol, followed by intravenous hydration, hypertonic saline, and inhaled steroid. Most commonly preferred treatment in the management of mild bronchiolitis was oral hydration and inhaled salbutamol in severe bronchiolitis. Conclusion: Although recent guidelines for the treatment of acute bronchiolitis does not support the use of many different therapies, pediatricians still tend to use them, especially bronchodilators, corticosteroids, and antibiotics.

Influenza A (H1N1)-associated severe complications; hemolytic uremic syndrome, myocarditis, acute necrotizing encephalopathy.

Influenza is a generally self-limited infection agent that only rarely causes severe complications. To increase awareness about its serious complications, we report three cases of influenza A (H1N1) infection complicated with hemolytic uremic syndrome, myocarditis and acute necrotizing encephalopathy. In all three cases, nasopharyngeal samples confirmed influenza A (H1N1) infection by antigen test and multiplex PCR detection. The first case, a 3-year-old girl, had respiratory distress, anemia, thrombocytopenia and renal failure at admission, and was diagnosed with hemolytic uremic syndrome. Supportive treatment and oseltamivir did not prevent the development of chronic renal failure. The second case, a 5-year-old girl admitted with lethargia and flu-like symtoms and was diagnosed with myocarditis and cardiogenic shock. Oseltamivir and supportive treatment including extra-corporeal membrane oxygenation (ECMO) failed. She died on the 3rd day of admission. The third case, a 21-month-old boy, presented with decreased level of consciousness and was diagnosed with acute necrotizing encephalopathy with the aid of cranial magnetic resonance imagining (MRI). He was discharged without any neurological sequelae three weeks after admission. It should be kept in mind that influenza virus does not always cause a self-limited flu. Multidisciplinary management, early diagnosis and antiviral treatment are critical for the disease and to prevent its life-threatening complications.

The diagnostic value of soluble urokinase plasminogen activator receptor (suPAR) compared to C-reactive protein (CRP) and procalcitonin (PCT) in children with systemic inflammatory response syndrome (SIRS).

BACKGROUND: The aim of the present study was to determine the diagnostic and prognostic values of suPAR and to compare them to CRP and PCT in pediatric patients with systemic inflammatory response syndrome (SIRS). MATERIAL-METHODS: A prospective case-control study was performed.The study was performed in a tertiary university hospital which has a 649-bed capacity. Patients included 27 children with SIRS and 27 control subjects without any infection or immunosuppressive condition. Blood samples were obtained on the day of admission and on the 4-7th days of the hospital stay. RESULTS: The median (min-max) serum levels of suPAR obtained on the first day of the admission were 10.06 (2.7-57.46) and 2.22 (1.08-5.13) ng/Ml for the SIRS group and control group, respectively. The median serum levels of suPAR in the SIRS group was significantly higher than that in the control group (p < 0.05). The serum suPAR levels was significantly higher in nonsurvivors than in survivors in SIRS group (p < 0.05). In the SIRS group, the area under the receiver operating characteristics curve (AUCROC) for suPAR revealed an optimum cut-off value, sensitivity, specificity, NPV and PPV of 0.978, 3.8 ng/mL, 96%, 96%, 96%, and 96%, respectively. CONCLUSIONS: We conclude that suPAR does have diagnostic value in children with SIRS. Additionally, persistent high serum suPAR level predicts mortality in SIRS in children.

Healthcare-Associated Infections in Pediatric Intensive Care Units in Turkey: a National Point-Prevalence Survey.

Health care-associated infections (HCAIs) cause considerable morbidity and mortality in pediatric intensive care units (PICUs). The objective of this point prevalence study was to assess the burden of HCAIs in PICUs in Turkey. Fifty PICUs participated in this study. Data regarding demographics, microbiological findings, therapeutic interventions, and outcomes were collected for all PICU inpatients. A total of 327 patients participated in the study: 122 (37%) experienced 1 or more HCAI. The most frequently reported site of infection was lower respiratory tract (n=77, 63%). The most frequently isolated pathogens were Pseudomonas aeruginosa, Acinetobacter species, and Candida species. Two hundred and forty-seven patients (75%) were receiving antimicrobial therapy at the time of the survey, and the most frequently administered antimicrobials were third generation cephalosporins. Hospital type, male, PICU stay >7 days, and mechanical ventilation were found to be independent risk factors for HCAIs. At the 4-week follow up, 43 (13%) patients had died, 28 (65%) of whom died of HCAIs. Endotracheal intubation, urinary catheter, male, and HCAIs were independent risk factors for mortality. This national, multicenter study documented a high prevalence of HCAIs in Turkey. In light of the 'primum non nocere' principle, the prevention of these infections should be a priority of public health policy.

Use of pumpless extracorporeal lung assist as rescue therapy in adolescent with cystic fibrosis.

There is abundant evidence that artificial ventilation can aggravate pre-existing lung disease, which may contribute to morbidity and mortality. This is especially true for patients with air leakages. This case report describes the use of a pumpless extracorporeal lung assist as a rescue therapy to provide time to heal during the mechanical ventilation of a 16-year-old with cystic fibrosis who could not be managed via conventional means.

Fenestrated angiocatheter for extensive subcutaneous emphysema.

A minimally invasive technique using fenestrated angiocatheters has been described in adult literature for the treatment of extensive subcutaneous emphysema. Here we report a 30 month old infant who developed extensive subcutaneous emphysema and pneumomediastinum, which was relieved by application of microdrainage catheters.

Trial of garlic as an adjunct therapy for multidrug resistant Pseudomonas aeruginosa pneumonia in a critically ill infant.

We present a case of a critically ill infant with severe multidrug-resistant Pseudomonas aeruginosa pneumonia and bacteremia who was treated with garlic and antibiotics after in vitro demonstration of a change in the resistance pattern via the disc diffusion method on garlic-treated Mueller Hinton agar. To our knowledge, this is the first report of therapeutic garlic use in a critically ill infant in the literature.

A pilot pharmacokinetic study of tricyclic antidepressant ovine Fab for TCA poisoning in children.

CONTEXT: A pilot study of tricyclic antidepressant (TCA)-specific antibody fragments (TCA Fab) in TCA-intoxicated adults showed a marked increase in serum total TCA concentrations following TCA Fab infusion with no worsening signs of TCA toxicity. TCA Fab pharmacokinetics (PK) was not described in this adult study. The objective of this study was to evaluate the PK of TCA Fab in children with TCA poisoning. METHODS: This was an open-label, single-center, dose escalation pilot trial of three patients. Inclusion criteria were documented TCA ingestion with at least one serious complication (QRS prolongation, dysrhythmia, hypotension, seizure, or coma). Patients were assigned to either a low-dose intravenous TCA Fab regimen (15, 30, and 60 mg/kg) or a high-dose regimen (30, 60, and 120 mg/kg) as needed to reverse TCA toxicity. Following the administration of TCA Fab, samples of blood and urine were obtained for PK evaluations. The outcomes of interest were serum and urine TCA concentrations (free and total), serum and urine Fab concentrations, improvement or worsening of TCA toxicity, and adverse effects. RESULTS: Three study patients were 11, 11, and 14 years of age. Two patients received 15 mg/kg of TCA Fab and one patient received a total of 90 mg/kg of TCA Fab (30 + 60 mg/kg). Serum-bound TCA increased significantly following TCA Fab administration with concomitant enhanced urinary elimination. Serum-free TCA concentrations were minimal to undetectable. Fab data were available for two patients. The serum TCA Fab area under the curve was 306.12 mg/L/h for the 15 mg/kg dose and 2,198.10 mg/L/h for the 90 mg/kg dose of TCA Fab. Maximum Fab concentrations correlated with maximum bound TCA in serum. The volume of distribution (V(D)) of TCA Fab was 0.2-0.3 L/kg. The clearance was 0.036-0.05 L/kg/h and the elimination half-life was 4 h. No adverse effects were observed. CONCLUSION: The limited PK data from this study are consistent with binding of TCA to TCA Fab and redistribution of TCA from the tissue to serum with subsequent enhanced urinary excretion of TCA. No toxic effects were observed with increased total TCA concentrations and no adverse effects were observed following TCA Fab administration. The small number of patients in this pilot study does not allow for safety or efficacy conclusions.