Retrospective analysis of children diagnosed with Kawasaki disease.

BACKGROUND: The aim of our study was to evaluate the long-term impacts of Kawasaki disease on our patients regarding coronary involvement demographic characteristics, treatment regimens, and clinical course. METHODS: Our study included 104 patients diagnosed and hospitalized with Kawasaki disease in our center, from January 2004 to January 2019. In our study, patients were divided into three groups according to coronary artery involvement. Patients in group 1 had no echocardiographic findings, while the ones in group 2 had coronary artery dilatation and ones in group 3 had coronary artery aneurysm (CAA). RESULTS: Among 104 patients, the median age was 9.15 (3.0-22.0) years, and 61 of the patients were male while 43 of the patients were female. With a wide range of 1.50-16.50 years of follow-up time, the median diagnosis age of our patients was 31 months (3.0-164.0). Fever duration (median day 10 (5-21), p = 0.025) was statistically significantly higher in group 3. Blood C-reactive protein (CRP) levels, white blood cell (WBC) counts, and neutrophil counts were significantly higher in group 3. There was a statistically significant difference between patients in group 3 and group 2 in which the lowest strain deformation values were in the patients of group 3. In contrast to group 1, the time for initiation of IVIG therapy is significantly prolonged both in group 2 (median: 9.5 days, p = 0.028) and group 3 (median: 10 days, p = 0.036). DISCUSSION: In our study, serum CRP levels, WBC count, and neutrophil count were higher in patients with coronary artery abnormalities, in agreement with the previous studies. In the light of our results, we consider that the most important determining factor for the development of coronary artery aneurysm is the time of intravenous immunoglobulin (IVIG) administration.

Why has plasma exchange failed in TRACK syndrome? Lessons from a new variant of the atypical hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening form of thrombotic microangiopathy, associated with high mortality and morbidity. Most cases present with hemolytic anemia, thrombocytopenia, and renal insufficiency. However, it can have unusual multiple end-organ injuries including extrarenal organ and system involvements such as neurologic, cardiac, gastrointestinal, and respiratory systems. We describe a 4-year-old girl who developed aHUS due to the TSEN2 mutation and had cardiac involvement. She did not benefit from plasma exchange, as stated in previous cases. It should be kept in mind that therapeutic plasma exchange may not be beneficial in some cases of aHUS, especially due to genetic mutations.

Does the severity of diabetic ketoacidosis in children with type 1 diabetes change during the COVID-19 pandemic? A single-center experience from a pediatric intensive care unit.

OBJECTIVE: During the COVID-19 pandemic, health-care services for diseases other than COVID-19 were interrupted, and patient referrals to health institutions were postponed due to their fear of being infected with COVID-19. Under this situation, we conducted this study to evaluate the clinical and laboratory findings of COVID-19 in patients with Type 1 Diabetes Mellitus (T1DM) hospitalized in our pediatric intensive care unit (PICU) with the diagnosis of diabetic ketoacidosis (DKA) during the pandemic period, and the impact of the pandemic on these findings. METHODS: We retrospectively evaluated 55 children aged from 1 month to 18 years old, diagnosed with DKA, and followed up at Istanbul Sehit Prof. Dr. Ilhan Varank Sancaktepe Training and Research Hospital PICU between April 2020 and December 2021. RESULTS: A total of 55 patients with DKA as a complication of T1DM were admitted to the PICU during the COVID-19 pandemic. While there was no significant difference in pH and HCO3 values between those with newly diagnosed T1DM and those with previously-diagnosed T1DM, the HbA1c ratio of newly diagnosed DMs was significantly higher. Of the 55 patients, 4 were COVID-19 PCR positive, and two patients had COVID-19 antibody positivity. When COVID-19 positive patients were compared with negative patients, no significant difference was found between the hospital stay, glucose, HbA1c, lactate, pH, and HCO3 values. CONCLUSION: Higher HbA1c levels of newly diagnosed patients presenting with DKA may be associated with delayed admission to the health institutions due to COVID-19 and the length of insulin-free periods compared to pre-diagnosed patients with T1DM. In conclusion, our results, emphasize the importance of physician's and family's awareness of the symptoms of diabetes in terms of early diagnosis and prevention of DKA during public health measures due to COVID-19.

Efficacy of extracorporeal membrane oxygenation in pediatric COVID-19 and MIS-C cases: A single-center experience.

Background: This study aimed to evaluate the need and the indication of extracorporeal membrane oxygenation (ECMO) in patients diagnosed with coronavirus disease 2019 (COVID-19) and multisystem inflammatory syndrome in children (MIS-C) followed up in the pediatric intensive care unit by the demographic, clinical, and laboratory data and treatment response. Methods: A total of 79 patients (43 males, 36 females; median age: 138 months; range, 6 to 210 months) with COVID-19 and MIS-C followed up between September 2020 - September 2021 were included in this retrospective study. Demographic and clinical data were retrospectively collected from patient files, and clinical data, laboratory findings, chest X-rays, and echocardiography results of six patients (1 male and 5 female, median age: 159 months, range, 13 to 210 months) who needed ECMO due to poor response to medical treatment were recorded before and after the ECMO therapy. Results: Extracorporeal membrane oxygenation therapy was performed on one patient with a positive COVID-19 polymerase chain reaction test and five patients with MIS-C in our unit. Five patients were supported with venoarterial (v-a) ECMO, and one patient was supported with venovenous ECMO. Median hospitalization time was 29 (range, 24 to 50) days, median Pediatric Risk of Mortality score was 19.5 (range, 11 to 36), and median length of mechanical ventilation was 23.5 (range, 10 to 45) days. The median vasoactive inotropic score was 55.5 (range, 18 to 110) before ECMO, while the median vasoactive inotropic score was 11 (range, 0 to 34) after ECMO. Four patients were successfully weaned off ECMO, and one of these patients was lost due to brain death 15 days after the weaning. One patient infected with the delta variant of COVID-19, which remained positive during the clinical course, and one patient diagnosed with MIS-C was lost despite the v-a ECMO support. Three of the patients were discharged. Thrombosis developed in the superficial femoral artery of one patient on the cannulated side during v-a ECMO. No death due to complications of ECMO was recorded. Conclusion: In our study, although the majority of our patients followed up with the diagnosis of COVID-19 and MIS-C showed a mild or moderate clinical course, it was observed that a severe clinical course could develop in a small number of patients and that ECMO treatment may be needed in these patients. In agreement with the ECMO studies with different indications in the literature, we conclude that ECMO therapy may markedly contribute to the prognosis in COVID-19 and MIS-C patients when the initiation and termination timing of therapy is correct.

Successful treatment with therapeutic plasmapheresis of a pediatric patient with Guillain-Barré syndrome associated with neurobrucellosis.

Brucellosis is a multisystemic disease that can present with multiple signs and symptoms. Rarely, brucellosis can manifest as neurobrucellosis, with central or peripheral nervous system involvement. Guillain-Barré syndrome (GBS) is a post-infectious autoimmune disease that progresses rapidly, causing ascending muscle weakness, and is accompanied by areflexia/hyporeflexia. Regarding GBS etiology, it is thought to be an autoimmune disease, triggered by a previous bacterial or viral infection. There are a few Brucella-associated GBS case reports in the literature and in our opinion, only one of them is a pediatric patient. Herein we reported a case of GBS associated with neurobrucellosis, who was successfully treated with therapeutic plasmapheresis (TP) due to poor response to IVIG treatment.

Can charcoal hemoperfusion treatment be an option for pediatric MIS-C patients? A single-center experience in a tertiary pediatric intensive care unit.

INTRODUCTION: Multisystem inflammatory syndrome in children (MIS-C) is a hyper-inflammatory disorder that develops following SARS-CoV-2 infection and has clinical signs that overlap with Kawasaki disease. Immunomodulatory treatments can be used in these patients. One of the alternative treatments reported in the literature is hemoperfusion therapy. In this study, we aim to evaluate our experience of charcoal hemoperfusion therapy in children admitted and followed up with a diagnosis of MIS-C at our Pediatric Intensive Care Unit (PICU). MATERIAL AND METHODS: We performed a retrospective evaluation of children diagnosed with MIS-C and children treated with charcoal hemoperfusion who are admitted to our PICU. RESULTS: Among 49 MIS-C patients, hemoperfusion therapy was performed on 14 patients. Duration of hospitalization, duration of invasive/non-invasive ventilation, VIS, OFI, PRISM 3 scores, and mortality rates were significantly higher in the charcoal hemoperfusion group before treatment. In patients who did not respond to conventional therapies, we observed a statistically significant decrease in the need for inotrope and invasive mechanical ventilation support and statistically significant improvements in clinical indicators after hemoperfusion therapy. DISCUSSION: In our study, we observed a significant clinical and laboratory improvement by charcoal hemoperfusion in our MIS-C patients who had a severe clinical course and multiple organ failure. CONCLUSION: In our opinion, this study is the first report regarding the use of charcoal hemoperfusion therapy in MIS-C patients, and the choice of charcoal hemoperfusion as an initial or rescue therapy is needed to be investigated in large patient groups both in children and adults who are diagnosed with COVID-19 and MIS-C.

Can plasma exchange therapy be an option for the treatment of SARS-CoV-2 Related Splenial Lesion Syndrome: Two cases from the pediatric intensive care unit.

BACKGROUND: Reversible splenial lesion syndrome (RESLES) is characterized by a temporary lesion in the splenium of the corpus callosum. RESLES is one of the most common causes of Mild encephalitis/encephalopathy reversible splenial lesion (MERS) and a rare clinical syndrome for the pediatric population. In a limited number of pediatric case reports, association with SARS-COV-2 in was reported. We aimed to increase the awareness of neurological involvement and treatment options of RESLES in children diagnosed with MIS-C. CASE PRESENTATION: We report two cases with a diagnosis of multisystem inflammatory syndrome-children who developed RESLES during the disease course. Fever, blurred vision, ataxia and encephalopathy were the main central nervous system symptoms. In our first case, we observed a rapid recovery in clinical symptoms and complete resolution of the splenial lesion in with intravenous immunoglobulin (IVIG) and methylprednisolone treatment. However, our second case did not respond to IVIG and methylprednisolone treatment. We performed therapeutic plasma exchange therapy and observed a successful recovery both in brain magnetic resonance imaging and echocardiographic findings. CONCLUSION: Although IVIG and methylprednisolone are the first choice treatment methods in MIS-C cases progressing with RESLES, therapeutic plasma exchange may be an option for the treatment of unresponsive cases.

Treatment of maple syrup urine disease with high flow hemodialysis in a neonate.

Aygün F, Kiykim E, Aktuglu-Zeybek Ç, Zubarioglu T, Cam H. Treatment of maple syrup urine disease with high flow hemodialysis in a neonate. Turk J Pediatr 2019; 61: 107-110. Continious renal replacement therapy (CRRT) is a well recognizied treatment of choice in acute renal failure, however CRRT became a preferred treatment of metabolic emergencies with high leucine and ammonia levels like Maple syrup urine disease (MSUD). MSUD is a rare metabolic disorder caused by deficiency in the activity of the branched-chain a-ketoacid dehydrogenase complex. The toxic accumulation of branched chain amino acids during acute metabolic decompensation is associated with the appearance of permanent neurological symptoms. Four patients were admitted to our pediatric intensive care department with complains of poor feeding, vomitting, irratibility and coma. Physical examination of the neonates were similar having stupor, hypotonia and depressed newborn reflexes. The leucine levels were between 930-4400 µmol/L. The diagnosis of MSUD was confirmed in all four. They were treated successfully with high flow CRRT having the rates were between 4120 ml/h/1.73m2 and 9830 ml/h/1.73m2. Early treatment is essential to prevent neurotoxicity and death. CRRT is a choice of treatment in metabolic crisis of MSUD. Herein, we report the successful treatment of acute metabolic decompensation of MSUD with CRRT in 4 neonates.

Evaluation of Continuous Renal Replacement Therapy and Therapeutic Plasma Exchange, in Severe Sepsis or Septic Shock in Critically Ill Children.

Background and objective: Severe sepsis and septic shock are life-threatening organ dysfunctions and causes of death in critically ill patients. The therapeutic goal of the management of sepsis is restoring balance to the immune system and fluid balance. Continuous renal replacement therapy (CRRT) is recommended in septic patients, and it may improve outcomes in patients with severe sepsis or septic shock. Therapeutic plasma exchange (TPE) is another extracorporeal procedure that can improve organ function by decreasing inflammatory and anti-fibrinolytic mediators and correcting haemostasis by replenishing anticoagulant proteins. However, research about sepsis and CRRT and TPE in children has been insufficient and incomplete. Therefore, we investigated the reliability and efficacy of extracorporeal therapies in paediatric patients with severe sepsis or septic shock. Materials and methods: We performed a multicentre retrospective study using data from all patients aged <18 years who were admitted to two paediatric intensive care units. Demographic data and reason for hospitalization were recorded. In addition, vital signs, haemogram parameters, and biochemistry results were recorded at 0 h and after 24 h of CRRT. Patients were compared according to whether they underwent CRRT or TPE; mortality between the two treatment groups was also compared. Results: Between January 2014 and April 2019, 168 septic patients were enrolled in the present study. Of them, 47 (27.9%) patients underwent CRRT and 24 underwent TPE. In patients with severe sepsis, the requirement for CRRT was statistically associated with mortality (p < 0.001). In contrast, the requirement for TPE was not associated with mortality (p = 0.124). Conclusion: Our findings revealed that the requirement for CRRT in patients with severe sepsis is predictive of increased mortality. CRRT and TPE can be useful techniques in critically ill children with severe sepsis. However, our results did not show a decrease of mortality with CRRT and TPE.

Infections with Carbapenem-Resistant Gram-Negative Bacteria are a Serious Problem Among Critically Ill Children: A Single-Centre Retrospective Study.

Children in paediatric intensive care units (PICUs) are vulnerable to infections because invasive devices are frequently used during their admission. We aimed to determine the prevalence, associated factors, and prognosis of infections in our PICU. This retrospective study evaluated culture results from 477 paediatric patients who were treated in the PICU between January 2014 and March 2019. Ninety patients (18.9%) had bacterial infections, with gram-negative bacteria being the predominant infectious agents. Culture-positive patients were younger than culture-negative patients, and age was related to mortality and various clinical factors. Culture-positive bacterial infections in the PICU were associated with increased use of invasive mechanical ventilation (odds ratio(OR); 2.254), red blood cell (RBC) transfusions (OR:2.624), and inotropic drugs (OR:2.262). Carbapenem resistance was found in approximately one-third of gram-negative bacteria, and was most common in tracheal aspirate specimens and cases involving Klebsiella spp. Total parenteral nutrition was a significant risk factor (OR:5.870). Positive blood culture results were associated with poorer patient survival than other culture results. These findings indicate that infections, especially those involving carbapenem-resistant bacteria, are an important issue when treating critically ill children.

Continuous Renal Replacement Therapy with High Flow Rate Can Effectively, Safely, and Quickly Reduce Plasma Ammonia and Leucine Levels in Children.

Introduction: Peritoneal dialysis and continuous renal replacement therapy (CRRT) are the most frequently used treatment modalities for acute kidney injury. CRRT is currently being used for the treatment of several non-renal indications, such as congenital metabolic diseases. CRRT can efficiently remove toxic metabolites and reverse the neurological symptoms quickly. However, there is not enough data for CRRT in children with metabolic diseases. Therefore, we aimed a retrospective study to describe the use of CRRT in metabolic diseases and its associated efficacy, complications, and outcomes. Materials and Methods: We performed a retrospective analysis of the records of all patients admitted in the pediatric intensive care unit (PICU) for CRRT treatment. Results: Between December 2014 and November 2018, 97 patients were eligible for the present study. The age distribution was between 2 days and 17 years, with a mean of 3.77 ± 4.71 years. There were 13 (36.1%) newborn with metabolic diseases. The patients were divided into two groups: CRRT for metabolic diseases and others. There was a significant relationship between the groups, including age (p ≤ 0.001), weight (p = 0.028), blood flow rate (p ≤ 0.001); dialysate rate (p ≤ 0.001), and replacement rate (p ≤ 0.001). The leucine reduction rate was 3.88 ± 3.65 (% per hour). The ammonia reduction rate was 4.94 ± 5.05 in the urea cycle disorder group and 5.02 ± 4.54 in the organic acidemia group. The overall survival rate was 88.9% in metabolic diseases with CRRT. Conclusion: In particularly hemodynamically unstable patients, CRRT can effectively and quickly reduce plasma ammonia and leucine.

Can Nebulised Colistin Therapy Improve Outcomes in Critically Ill Children with Multi-Drug Resistant Gram-Negative Bacterial Pneumonia?

In the past decade, multidrug-resistant (MDR) gram-negative bacteria have become a major problem, especially for patients in intensive care units. Recently, colistin became the last resort therapy for MDR gram-negative bacteria infections. However, nebulised colistin use was limited to adult patients. Thus, we investigated the efficacy and safety of nebulised colistin treatment against MDR microorganisms in the paediatric intensive care unit (PICU). Data of all patients admitted for various critical illnesses (January 2016 to January 2019) were reviewed. Differences between groups (with and without a history of nebulised colistin) were compared. Of 330 patients, 23 (6.97%) used nebulised colistin. Significant relationships were found between nebulised colistin usage and several prognostic factors (inotropic drug use (p = 0.009), non-invasive mechanical ventilation (p ≤ 0.001), duration in PICU (p ≤ 0.001), and C-reactive protein level (p = 0.003)). The most common microorganism in tracheal aspirate and sputum cultures was Pseudomonas aeruginosa (13 patients). The most common underlying diagnosis was cystic fibrosis, noted in 6 patients. No serious nephrotoxicity and neurotoxicity occurred. This study showed that colistin can be safely used directly in the airway of critically ill children. However, nebulised colistin use did not have a positive effect on mortality and prognosis.

Gamma Glutamyl Transferase and Uric Acid Levels Can Be Associated with the Prognosis of Patients in the Pediatric Intensive Care Unit.

Introduction: Gamma glutamyl transferase (GGT) and uric acid (UA) are reported to be predictive markers in various disorders. It has been reported that these biomarkers can be used to indicate increased risk of mortality in critically ill patients. Herein, we aimed to evaluate the effects of the initial serum GGT and UA levels on the outcomes of patients in the pediatric intensive care unit (PICU) and to investigate if these biomarkers can be used to predict pediatric mortality. Materials and Methods: The relationship between the initial GGT and UA levels and invasive mechanical ventilation (IMV) and noninvasive mechanical ventilation (NIV) support, inotropic drug need, acute renal kidney injury (AKI), continuous renal replacement therapy (CRRT), presence of sepsis, mortality, and hospitalization period were investigated retrospectively. Results: In all, 236 patients (117 males and 119 females) were included in the study. The age distribution of the patients was 1⁻12 years. There was a statistically significant relationship between GGT levels in the first biochemical analysis performed during admission and inotropic drug use, AKI, duration of hospitalization in intensive care unit, and sepsis. There was a statistically significant relationship between initial UA levels and inotropic drug use, AKI, CCRT, and sepsis. Conclusion: We suggest that initial GGT and UA levels during admission could be used to predict the outcomes of patients in PICU.

The impact of continuous renal replacement therapy for metabolic disorders in infants.

BACKGROUND: While Continuous Renal Replacement Therapy (CRRT) is a well established treatment modality for patients with acute kidney insufficiency (AKI), it is now also being used for the management of various illnesses such as acute metabolic disorders presenting with hyperammonemia and elevated leucine levels. Herein, we aimed to describe our experience with CRRT in treatment of acute decompensation of 14 patients with a diagnosis of metabolic disorder who has been admitted to our pediatric intensive care unit (PICU) in the last year. METHODS: Patients who have had life threatening acute metabolic crisis due to various metabolic disorders and were treated with continuous renal replacement therapy (CRRT) were evaluated retrospectively. RESULTS: Between November 2014 and December 2015, 14 patients were found to have received CRRT for various metabolic disorders in the PICU. Ten patients had hyperammonemia and four patients had elevated leucine levels. Nine patients were male and five were female. The age interval was between 2 days and 18 months, with a mean of 5.5 ± 7.4 months. The weight distribution was between 2.5 and 18 kg, with a mean of 7.3 ± 5.6 kg. Eleven patients received continuous veno-venous hemodiafiltration (CVVHDF), and 3 patients with MSUD received continuous veno-venous hemodialysis (CVVHD). All patients have received high throughput hemodialysis and hemofiltration. The dialyzate rate was set to be minimum 4042 ml/h/1.73 m2, and maximum 12,900 ml/h/1.73 m2. Hemofiltration was performed with a replacement rate of 40-76 ml/kg/h. The average CRRT duration was 16.6 ± 15.6 h. CONCLUSIONS: We suggest that CRRT is an efficient method that can be used in hyperammonemia and elevated leucine levels which are metabolic emergencies.

Lung abscess from Staphylococcus aureus after varicella infection in a 3-month-old infant.

Varicella is a common, highly contagious viral infection of childhood. Varicella is a usually benign and self-limited disease, but it can be complicated by severe bacterial infections, especially in immunocompromised hosts. In this study, we describe a previously healthy 3-months-old infant who was admitted with high fever, cough, and respiratory distress, who had a history of varicella infection three weeks before, with exposure from her adolescent, unvaccinated sister. A lung abscess caused by Staphylococcus aureus complicating the varicella infection was discovered. The patient was aggressively treated with drainage of the abscess and intravenous antibiotics and had a good recovery.

Successful Treatment of Bacillus cereus Bacteremia in a Patient with Propionic Acidemia.

Bacillus cereus can cause serious, life-threatening, systemic infections in immunocompromised patients. The ability of microorganism to form biofilm on biomedical devices can be responsible for catheter-related bloodstream infections. Other manifestations of severe disease are meningitis, endocarditis, osteomyelitis, and surgical and traumatic wound infections. The most common feature in true bacteremia caused by Bacillus is the presence of an intravascular catheter. Herein, we report a case of catheter-related bacteremia caused by B. cereus in a patient with propionic acidemia.

Cardiovascular side effects related with use of synthetic cannabinoids "bonzai" : two case reports.

Information about the effects of synthetic cannabinoids "bonzai" on the cardiovascular system is limited. In this article, two patients in whom different cardiological side effects were observed following use of synthetic cannabinoids 'bonzai' were presented. Our first patient who was a 16-year old boy presented to pediatric emergency department with severe chest pain which had started one hour before. On electrocardiographic examination, ST segment elevations greater than 3 mm were observed in DII, DIII, AVF and V5-V6 derivations which caused to suspicious myocardial infarction. Cardiac catheterization was performed and coronary angiographic findings were found to be normal. When the patient was questioned again, it was learned that he used synthetic cannabinoid 'bonzai'. The second patient who was aged 18 years and addicted to synthetic cannabinoid 'bonzai' was brought to our emergency department by his friends because of clouding of consciousness. Dopamine treatment was started in the patient whose cardiac apical beat reduced to 40/min and who was found to develop hypertension, because bradycardia worsened. However, the blood pressure increased rapidly and dopamine treatment was stopped. Heart rate and blood pressure monitoring was continued. Use of synthetic cannabinoids 'bonzai' should be kept in mind in patients who present to emergency departments with chest pain and complaints related with the cardiovascular system.