Percutaneous Tracheostomy Via Grigg's Technique in Children: Does Age and Size Matter?

OBJECTIVES: Percutaneous tracheostomy is rarely performed in children, especially in infants. In the present study, we aimed to evaluate the complications and outcomes of PT via the Griggs technique according to the age and size of pediatric patients. METHODS: This study included 110 PICU patients who underwent PT using the Griggs technique between 2012 and 2020. The patients were divided into six groups according to their age, demographic data, primary disease, mean duration of intubation before PT, mean duration of PICU and hospitalization after PT, complications, and decannulation outcomes were compared between these groups. RESULTS: The mean age and mean weight of the patients were 43.6 ± 58.9 months (1 month-207 months) and 14.6 ± 14.9 kg (2.6-65 kg), respectively. Mean intubation times before the procedures were 64.6 ± 40 days and 38.6 ± 37.9. Thirty-seven (33.6%) infants were under 6 months of age(Group 1). There were no intraoperative complications. Tracheostomy site stenosis was significantly greater in Group 1 than in the other age groups (p = 0.032). Granuloma formation and dermatitis incidence were similar in all age groups. CONCLUSION: PT is a safe and feasible procedure even in small infants. The accidental decannulation risk is lower than standard tracheostomy. Interacting with rigid bronchoscopy guidance is essential to perform a safer procedure. The first tracheostomy change after PT in small infants under 6 months of age, the possibility of tracheostomy site (stoma) stenosis should be considered. LEVEL OF EVIDENCE: Level III Laryngoscope, 2024.

Extracorporeal Membrane Oxygenation after Pediatric Cardiac Surgery: A Single-Center Experience.

Extracorporeal membrane oxygenation (ECMO) is a life-saving treatment option providing cardiopulmonary support when standard therapies prove insufficient for reversible diseases. The mean objective of this study was to evaluate our center's experience with ECMO following pediatric cardiac surgery. This retrospective study was conducted in our pediatric intensive care unit (PICU) between November 2014 and March 2021 and included patients who received ECMO following cardiac surgery. Over the 7-year period, 324 patients underwent cardiac surgery, of which 24 (7.4%) required ECMO support. Among them, 13 (54.2%) were female, with a median age of 16.0 (2.0- 208) months and a median weight of 7.0 (3.5-70) kg. The mean vasoactive inotrope score (VIS) was 53.9 ± 44.5. Atrioventricular septal defect repair was the most common surgical procedure (n = 8/24, 41.6%). The primary indication for ECMO was low cardiac output syndrome (LCOS) in 14 (58.3%) patients. The median duration of ECMO support was 6.0 (1.0-46.0) days. Nonsurvivors had significantly higher Pediatric Risk Score of Mortality (PRISM) III scores (P = .014) and VIS scores during the pre-ECMO period (P = .004). Early or late neurological complications developed in 12 (50%) patients, with significant differences in lactate levels and pH levels preECMO between those with and without neurological complications (P = .01, P = .02, respectively). We successfully decannulated 16 (66.6%) patients, with a final survival rate of 12 (50%). ECMO plays a crucial role in providing pre- and post-cardiac surgery support for children. LCOS remains the main indication, and high PRISM III and VIS scores are valuable predictors of outcomes.

Comparison of radiologic findings between SARS-CoV-2 and other respiratory tract viruses in critically ill children during the COVID-19 pandemic.

Background/aim: This study was planned because the radiological distinction of COVID-19 and respiratory viral panel (RVP)-positive cases is necessary to prioritize intensive care needs and ensure non-COVID-19 cases are not overlooked. With that purpose, the objective of this study was to compare radiologic findings between SARS-CoV-2 and other respiratory airway viruses in critically ill children with suspected COVID-19 disease. Materials and methods: This study was conducted as a multicenter, retrospective, observational, and cohort study in 24 pediatric intensive care units between March 1 and May 31, 2020. SARS-CoV-2- or RVP polymerase chain reaction (PCR)-positive patients' chest X-ray and thoracic computed tomography (CT) findings were evaluated blindly by pediatric radiologists. Results: We enrolled 225 patients in the study, 81 of whom tested positive for Coronovirus disease-19 (COVID-19) caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). The median age of all patients was 24 (7-96) months, while it was 96 (17-156) months for COVID-19-positive patients and 17 (6-48) months for positive for other RVP factor (p < 0.001). Chest X-rays were more frequently evaluated as normal in patients with SARS-CoV-2 positive results (p = 0.020). Unilateral segmental or lobar consolidation was observed more frequently on chest X-rays in rhinovirus cases than in other groups (p = 0.038). CT imaging findings of bilateral peribronchial thickening and/or peribronchial opacity were more frequently observed in RVP-positive patients (p = 0.046). Conclusion: Chest X-ray and CT findings in COVID-19 patients are not specific and can be seen in other respiratory virus infections.

Use of Intravenous Lipid Emulsion Therapy and Insulin in a Case of Tarka Intoxication.

INTRODUCTION: Tarka (trandolapril/verapamil hydrohloride extended-release) is a fixed-dose combination antihypertensive drug formed from verapamil hydrochloride and trandolapril. Toxicologic manifestations of Tarka overdose are altered mental status, bradycardia, hypotension, atrioventricular block (first-degree), hyperglycemia, metabolic acidosis, and shock. CASE PRESENTATION: We report a case of Tarka toxicity in a 2-year-old girl who presented with altered mental status, cardiogenic shock, hypotension, bradycardia, severe metabolic acidosis, hyperglycemia, and first-degree atrioventricular block. We started fluid resuscitation, epinephrine, norepinephrine, and insulin. Because of the patient's hyperlactatemia and hypotension despite standard therapies, we initiated intravenous lipid emulsion (ILE) therapy, after which her condition improved promptly. DISCUSSION: Tarka overdose may be life-threatening as it can cause cardiogenic shock. In our patient, the regression of lactate elevation in a short time with ILE therapy and the improvement of her general condition highlight the importance of ILE. CONCLUSIONS: ILE is an alternative treatment method for acute lipophilic drug intoxications, such as Tarka.

Very-early-onset Inflammatory Bowel Disease in an Infant with a Partial RIPK1 Deletion.

The monogenic causes of very-early-onset inflammatory bowel disease (VEO-IBD) have been defined by genetic studies, which were usually related to primary immunodeficiencies. Receptor-interacting serine/threonine-protein kinase-1 (RIPK1) protein is an important signalling molecule in inflammation and cell death pathways. Its deficiency may lead to various clinical features linked to immunodeficiency and/or inflammation, including IBD. Here, we discuss an infant with malnutrition, VEO-IBD, recurrent infections and polyathritis who has a homozygous partial deletion in RIPK1 gene.

Correlation between early computed tomography findings and neurological outcome in pediatric traumatic brain injury patients.

Traumatic brain injury (TBI) is a leading cause of morbidity and mortality in children. Head computed tomography (CT) is frequently utilized for evaluating trauma-related characteristics, selecting treatment options, and monitoring complications in the early stages. This study assessed the relationship between cranial CT findings and early and late neurological outcomes in pediatric TBI patients admitted to the pediatric intensive care unit (PICU). The study included children aged 1 month to 18 years who were admitted to the PICU due to TBI between 2014 and 2020. Sociodemographic data, clinical characteristics, and cranial CT findings were analyzed. Patients were categorized based on their Glasgow Coma Scale (GCS) score. Of the 129 patients, 83 (64%) were male, and 46 (36%) were female, with a mean age of 6.8 years. Falls (n = 51, 39.5%) and in-vehicle traffic accidents (n = 35, 27.1%) were the most common trauma types observed. Normal brain imaging findings were found in 62.7% of the patients, while 37.3% exhibited intracranial pathology. Hemorrhage was the most frequent CT finding. Severe TBI (n = 26, p = 0.032) and mortality (n = 9, p = 0.017) were more prevalent in traffic accidents. The overall mortality rate in the study population was 10.1%. In children with TBI, cranial CT imaging serves as an essential initial method for patients with neurological manifestations. Particularly, a GCS score of ≤ 8, multiple hemorrhages, diffuse cerebral edema, and intraventricular bleeding are associated with sequelae and mortality.

Successful Treatment with Ascorbic Acid in a Case of Methemoglobinemia Due to Glucose-6-Phosphate Dehydrogenase Deficiency.

Cite this article as: Karaçoban G, Gurbanov A, Özen H, Gün E, Çakmakli HF, Kendirli T. Successful treatment with ascorbic acid in a case of methemoglobinemia due to glucose-6-phosphate dehydrogenase deficiency. Turk Arch Pediatr. 2024;59(1):112-113.

GIMAP5 deficiency reveals a mammalian ceramide-driven longevity assurance pathway.

Preserving cells in a functional, non-senescent state is a major goal for extending human healthspans. Model organisms reveal that longevity and senescence are genetically controlled, but how genes control longevity in different mammalian tissues is unknown. Here, we report a new human genetic disease that causes cell senescence, liver and immune dysfunction, and early mortality that results from deficiency of GIMAP5, an evolutionarily conserved GTPase selectively expressed in lymphocytes and endothelial cells. We show that GIMAP5 restricts the pathological accumulation of long-chain ceramides (CERs), thereby regulating longevity. GIMAP5 controls CER abundance by interacting with protein kinase CK2 (CK2), attenuating its ability to activate CER synthases. Inhibition of CK2 and CER synthase rescues GIMAP5-deficient T cells by preventing CER overaccumulation and cell deterioration. Thus, GIMAP5 controls longevity assurance pathways crucial for immune function and healthspan in mammals.

Cardiovascular manifestations and cardiac magnetic resonance follow-up of multisystem inflammatory syndrome in children (MIS-C).

OBJECTIVE: This study aimed to evaluate the cardiovascular manifestations and surveillance of multisystem inflammatory syndrome in children (MIS-C) and to determine the correlation of echocardiographic findings with cardiac magnetic resonance imaging findings. METHODS: Forty-four children diagnosed as MIS-C with cardiac involvement were enrolled in this observational descriptive study. The diagnosis of MIS-C was made according to the criteria of Centers for Disease Control and Prevention. Clinical findings, laboratory parameters, and electrocardiographic and echocardiographic findings at the time of diagnosis and during follow-up were evaluated. Cardiac magnetic resonance was performed on 28 (64%) cases. The 1-year follow-up imaging was performed in all cases with abnormal initial cardiac magnetic resonance findings. RESULTS: Forty-four patients (56.8% male) with a mean age of 8.5 ± 4.8 years were enrolled in this study. There was a significant positive correlation between high-sensitivity cardiac troponin T (mean: 162 ± 444.4 pg/ml) and N-terminal pro b-type natriuretic peptide (mean: 10,054 ± 11,604 pg/ml) (p < 0.01). Number of cases with an electrocardiographic and echocardiographic abnormality was 34 (77%) and 31 (70%), respectively. Twelve cases (45%) had left ventricular systolic dysfunction and 14 (32%) cases had pericardial effusion on admission. Three cases (11%) had cardiac magnetic resonance findings that may be attributed to the presence of myocardial inflammation, and pericardial effusion was present in seven (25%) cases. Follow-up cardiac magnetic resonances of all cases were normal. Cardiac abnormalities were completely resolved in all except two cases. CONCLUSIONS: Myocardial involvement can be seen during acute disease, but MIS-C generally does not lead to prominent damage during a year of surveillance. Cardiac magnetic resonance is a valuable tool to evaluate the degree of myocardial involvement in cases with MIS-C.

The impact of fragmented QRS on clinical findings and outcomes in children with dilated cardiomyopathy with or without left ventricular non-compaction.

OBJECTIVE: The aim of this study is to investigate the frequency of fragmented QRS and its associations with clinical findings and prognosis in children diagnosed with dilated cardiomyopathy with or without left ventricular non-compaction. METHODS: This retrospective study was conducted between 2010 and 2020. Patients with dilated cardiomyopathy were classified into two groups according to the presence of left ventricular non-compaction: Dilated cardiomyopathy with left ventricular non-compaction and dilated cardiomyopathy without left ventricular non-compaction. Patients were also divided into two groups according to the presence of fragmented QRS (fragmented QRS group and non-fragmented QRS group). RESULTS: Twenty-three of 44 patients (52.3%) were male. Among left ventricular non-compaction patients, the fragmented QRS group had more complex ventricular arrhythmias (p = 0.003). Patients with fragmented QRS had a significantly higher rate of major adverse cardiac events and/or cardiac death in both cardiomyopathy groups (p = 0.003 and p = 0.005). However, the rate of major adverse cardiac events and/or cardiac death was similar between dilated cardiomyopathy patients with and without left ventricular non-compaction. Multivariate logistic regression analysis showed that the presence of fragmented QRS strongly predicts major adverse cardiac events and/or cardiac death (odds ratio, 31.186; 95% confidence interval, 2.347-414.307). Although the survival rates between cardiomyopathy groups were similar, patients with fragmented QRS had a markedly lower survival rate during the follow-up period, as mean of 15 months (p = 0.001). CONCLUSION: Our study showed that the presence of fragmented QRS may be an important ECG sign predicting an major adverse cardiac event and/or cardiac death in patients with dilated cardiomyopathy. We believe that recognising fragmented QRS could be valuable in forecasting patient prognosis and identifying high-risk patients who require additional support.

Evaluation of 601 children with multisystem inflammatory syndrome (Turk MISC study).

PURPOSE: Due to its link with the 2019 coronavirus, the multisystem inflammatory syndrome in children (MISC) has garnered considerable international interest. The aim of this study, in which MISC patients were evaluated multicenter, and the data of the third period of the Turk-MISC study group, to compare the clinical and laboratory characteristics and outcomes of MISC patients who did and did not require admission to an intensive care unit (ICU). METHODS: This retrospective multicenter observational study was carried out between June 11, 2021, and January 01, 2022. The demographics, complaints, laboratory results, system involvements, and outcomes of the patients were documented. RESULTS: A total of 601 patients were enrolled; 157 patients (26.1%) required hospitalization in the intensive care unit (ICU). Median age was 8 years (interquartile range (IQR) 4.5-11.3 years. The proportion of Kawasaki disease-like features in the ICU group was significantly higher than in the non-ICU group (56.1% vs. 43.2% p = 0.006). The ICU group had considerably lower counts of both lymphocytes and platelets (lymphocyte count 900 vs. 1280 cells × µL, platelet count 153 vs. 212 cells × 103/ µL, all for p< 0.001). C-reactive protein, procalcitonin, and ferritin levels were significantly higher in the ICU group (CRP 164 vs. 129 mg/L, procalcitonin 9.2 vs. 2.2 µg/L, ferritin 644 vs. 334 µg/L, all for p< 0.001). Being between ages 5-12 and older than 12 increased the likelihood of hospitalization in the ICU by four [95% confidence intervals (CI)1.971-8.627] and six times (95% CI 2.575-14.654), respectively, compared to being between the ages 0-5. A one-unit increase in log D-dimer (µg/L) and log troponin (ng/L) was also demonstrated to increase the need for intensive care by 1.8 (95% CI 1.079-3.233) and 1.4 times (95% CI 1.133-1.789), respectively.     Conclusion: By comparing this study to our other studies, we found that the median age of MISC patients has been rising. Patients requiring an ICU stay had considerably higher levels of procalcitonin, CRP, and ferritin but significantly lower levels of lymphocyte and thrombocyte. In particular, high levels of procalcitonin in the serum might serve as a valuable laboratory marker for anticipating the need for intensive care. WHAT IS KNOWN: • Lymphopenia and thrombocytopenia were an independent predictor factors in patients with MISC who needed to stay in intensive care unit. • The possibility of the need to stay in the intensive care unit in patients with MISC who had Kawasaki disease-like findings was controversial compared with those who did not. WHAT IS NEW: • A one-unit increase log D dimer and log troponin was demonstrated to require for intensive care unit by 1.8 and 1.4 times, respectively. • Serum procalcitonin levels had the best performance to predict stay in the intensive care unit stay.

Venovenous Versus Venoarterial Extracorporeal Membrane Oxygenation: Pediatric Acute Respiratory Distress Syndrome.

OBJECTIVE: This study aimed to compare the efficacy, complication, and mortality of patients who were supported by venovenous (VV) extracorporeal membrane oxygenation (ECMO) and venoarterial (VA) ECMO for pediatric acute respiratory distress syndrome (PARDS). MATERIALS AND METHODS: This study is a single-center, retrospective cohort study between 2014 and 2022. We evaluated to indication of ECMO support, ECMO type, patients' demographic features, complications, and children's outcomes supported by ECMO for PARDS. RESULTS: Twenty-two patients with PARDS, 12 (54%) with VV, and 10 (46%) with VA ECMO were selected. The median number of days to be intubated before ECMO cannulation was 5 (0-16) days. The distribution of intubated days before the patients underwent ECMO was as follows: 0-1 days, 7 (31.8%) patients; 2-3 days, 2 (9.1%) patients; 4-7 days, 7 (31.8%) patients; 8-14 days, 5 (22.8%) patients; >14 days, 1 (4.5%) patient. The median ECMO cannulation day after admission to the pediatric intensive care unit was 3 (range, 1-9) days in the VV ECMO patient group, whereas it was 8 (range, 0-19) days in the VA ECMO group (P = .02). Considering hospital survival, 4 (45%) patients who underwent double-lumen VV ECMO, 1 (33%) patient who underwent VV ECMO, and 3 (30%) patients who supported by VAECMO survived. There was no difference between the groups in terms of hospital discharge rates. CONCLUSION: The highest survival rate was found in the VV ECMO patient group established with double-lumen cannulas, similar to the literature. There was no difference in mortality between the groups whose intubation time before ECMO was 14 days or less.

Evaluation of nutritional status in pediatric intensive care unit patients: the results of a multicenter, prospective study in Turkey.

Introduction: Malnutrition is defined as a pathological condition arising from deficient or imbalanced intake of nutritional elements. Factors such as increasing metabolic demands during the disease course in the hospitalized patients and inadequate calorie intake increase the risk of malnutrition. The aim of the present study is to evaluate nutritional status of patients admitted to pediatric intensive care units (PICU) in Turkey, examine the effect of nutrition on the treatment process and draw attention to the need for regulating nutritional support of patients while continuing existing therapies. Material and Method: In this prospective multicenter study, the data was collected over a period of one month from PICUs participating in the PICU Nutrition Study Group in Turkey. Anthropometric data of the patients, calorie intake, 90-day mortality, need for mechanical ventilation, length of hospital stay and length of stay in intensive care unit were recorded and the relationship between these parameters was examined. Results: Of the 614 patients included in the study, malnutrition was detected in 45.4% of the patients. Enteral feeding was initiated in 40.6% (n = 249) of the patients at day one upon admission to the intensive care unit. In the first 48 h, 86.82% (n = 533) of the patients achieved the target calorie intake, and 81.65% (n = 307) of the 376 patients remaining in the intensive care unit achieved the target calorie intake at the end of one week. The risk of mortality decreased with increasing upper mid-arm circumference and triceps skin fold thickness Z-score (OR = 0.871/0.894; p = 0.027/0.024). The risk of mortality was 2.723 times higher in patients who did not achieve the target calorie intake at first 48 h (p = 0.006) and the risk was 3.829 times higher in patients who did not achieve the target calorie intake at the end of one week (p = 0.001). The risk of mortality decreased with increasing triceps skin fold thickness Z-score (OR = 0.894; p = 0.024). Conclusion: Timely and appropriate nutritional support in critically ill patients favorably affects the clinical course. The results of the present study suggest that mortality rate is higher in patients who fail to achieve the target calorie intake at first 48 h and day seven of admission to the intensive care unit. The risk of mortality decreases with increasing triceps skin fold thickness Z-score.

Evaluation of the efficacy and associated complications of regional citrate anticoagulation in neonates: experience from a fourth level neonatal intensive care unit.

Continuous kidney replacement therapy (CKRT) use has increased in recent years, but anticoagulation is a challenge for neonates. Regional citrate anticoagulation (RCA) is rarely preferred in neonates because of citrate accumulation (CA) and metabolic complications. We aimed to demonstrate the efficacy and safety of RCA in neonates. We retrospectively analyzed the medical records of 11 neonates treated with RCA-CKRT between 2018 and 2023. The initial dose of RCA was 2.1-3 mmol/l, and then, its dose was increased according to the level of ionized calcium (iCa+2) in the circuit and patients. The total/iCa+2 ratio after-treatment > 2.5 was indicated as CA. We evaluated to citrate dose, CA, circuit lifespan, and dialysis effectivity. The median gestational age was 39 (36.4-41.5) weeks, the median body weight (BW) was 3200 (2400-4000) grams, and the mean postnatal age was 4 (2-24) days. The most common indication for CKRT was hyperammonemia (73%). All neonates had metabolic acidosis and hypocalcemia during CKRT. Other common metabolic complications were hypophosphatemia (90%), hypokalemia (81%), and hypomagnesemia (63%). High dialysate rates with a median of 5765 ml/h/1.73 m2 allowed for a rapid decrease in ammonia levels to normal. Four patients (36.3%) had CA, and seven (63.7%) did not (non-citrate accumulation, NCA). Mean BW, median postnatal age, biochemical parameters, coagulation tests, and ammonia levels were similar between the CA and NCA groups. Low pH, low HCO3, high lactate, and SNAPPE-II scores could be associated with a higher T/iCa ratio. CONCLUSION:  RCA was an efficient and safe anticoagulation for neonates requiring CKRT. Metabolic complications may occur, but they could be managed with adequate supplementation. WHAT IS KNOWN: • Continuous kidney replacement therapy (CKRT) has become popular in recent years due to its successful treatment of fluid overload, electrolyte imbalance, metabolic acidosis, multi-organ failure, and hyperleucinemia/hyperammonemia associated with inborn errors of metabolism. • The need for anticoagulation is the major difficulty in neonatal CKRT. In adult and pediatric patients, regional citrate anticoagulation has been shown to be effective. WHAT IS NEW: • RCA is an effective and safe anticoagulation method for neonates who require CKRT. • Electrolyte imbalances and metabolic acidosis could be managed with adequate supplementation and appropriate treatment parameters such as citrate dose, blood flow rate, and dialysate flow rate.