ABSTRACT
OBJECTIVES: multi-system inflammatory syndrome in children (MIS-C) is an immune-mediated process that develops after infections like SARS-CoV-2. The authors aimed to reveal the mucocutaneous findings of patients diagnosed with MIS-C at presentation and evaluate the frequency of these mucocutaneous findings and their possible relationship with the severity of the disease. METHODS: A prospective study was conducted of 43 children admitted to a tertiary hospitals between January 2021 and January 2022 who met Centers for Disease Control and Prevention criteria for MIS-C. RESULTS: 43 children (25 [58.1%] male); median age, 7.5 years [range 0.5â15 years]) met the criteria for MIS-C. The most common symptom was cutaneous rash 81.4%, followed by gastrointestinal symptoms 67.4%, oral mucosal changes 65.1%, and conjunctival hyperemia 58.1%. The most common mucosal finding was fissured lips at 27.9%, diffuse hyperemia of the oral mucosa at 18.6%, and strawberry tongue at 13.9%. Urticaria (48.8%) was the most common type of cutaneous rash in the present study's patients. The most common rash initiation sites were the trunk (32.6%) and the palmoplantar region (20.9%). The presence or absence of mucocutaneous findings was not significantly associated with disease severity. STUDY LIMITATIONS: The number of patients in the this study was small. CONCLUSIONS: The present study's prospective analysis detected mucocutaneous symptoms in almost 9 out of 10 patients in children diagnosed with MIS-C. Due to the prospective character of the present research, the authors think that the characteristic features of cutaneous and mucosal lesions the authors obtained will contribute to the literature on the diagnosis and prognosis of MIS-C.
Subject(s)
COVID-19 , Connective Tissue Diseases , Hyperemia , Child , Humans , Male , Infant , Child, Preschool , Adolescent , Female , COVID-19/complications , COVID-19/epidemiology , SARS-CoV-2 , Prospective Studies , Pandemics , Systemic Inflammatory Response Syndrome/epidemiologyABSTRACT
Kinesins are canonical molecular motors but can also function as modulators of intracellular signaling. KIF26A, an unconventional kinesin that lacks motor activity, inhibits growth-factor-receptor-bound protein 2 (GRB2)- and focal adhesion kinase (FAK)-dependent signal transduction, but its functions in the brain have not been characterized. We report a patient cohort with biallelic loss-of-function variants in KIF26A, exhibiting a spectrum of congenital brain malformations. In the developing brain, KIF26A is preferentially expressed during early- and mid-gestation in excitatory neurons. Combining mice and human iPSC-derived organoid models, we discovered that loss of KIF26A causes excitatory neuron-specific defects in radial migration, localization, dendritic and axonal growth, and apoptosis, offering a convincing explanation of the disease etiology in patients. Single-cell RNA sequencing in KIF26A knockout organoids revealed transcriptional changes in MAPK, MYC, and E2F pathways. Our findings illustrate the pathogenesis of KIF26A loss-of-function variants and identify the surprising versatility of this non-motor kinesin.
Subject(s)
Kinesins , Neurons , Humans , Animals , Mice , Kinesins/genetics , Neurons/metabolism , Focal Adhesion Protein-Tyrosine Kinases/metabolism , Apoptosis , Brain/metabolismABSTRACT
Introduction: There have been some significant changes regarding healthcare utilization during the COVID-19 pandemic. Majority of the reports about the impact of the COVID-19 pandemic on diabetes care are from the first wave of the pandemic. We aim to evaluate the potential effects of the COVID-19 pandemic on the severity of diabetic ketoacidosis (DKA) and new onset Type 1 diabetes presenting with DKA, and also evaluate children with DKA and acute COVID-19 infection. Methods: This is a retrospective multi-center study among 997 children and adolescents with type 1 diabetes who were admitted with DKA to 27 pediatric intensive care units in Turkey between the first year of pandemic and pre-pandemic year. Results: The percentage of children with new-onset Type 1 diabetes presenting with DKA was higher during the COVID-19 pandemic (p < 0.0001). The incidence of severe DKA was also higher during the COVID-19 pandemic (p < 0.0001) and also higher among children with new onset Type 1 diabetes (p < 0.0001). HbA1c levels, duration of insulin infusion, and length of PICU stay were significantly higher/longer during the pandemic period. Eleven patients tested positive for SARS-CoV-2, eight were positive for new onset Type 1 diabetes, and nine tested positive for severe DKA at admission. Discussion: The frequency of new onset of Type 1 diabetes and severe cases among children with DKA during the first year of the COVID-19 pandemic. Furthermore, the cause of the increased severe presentation might be related to restrictions related to the pandemic; however, need to evaluate the potential effects of SARS-CoV-2 on the increased percentage of new onset Type 1 diabetes.
Subject(s)
Heart Septal Defects, Ventricular , Respiratory System Abnormalities , Scimitar Syndrome , Bronchi/abnormalities , Bronchi/diagnostic imaging , Child , Heart Septal Defects, Ventricular/diagnostic imaging , Humans , Lung , Scimitar Syndrome/diagnostic imaging , Trachea/abnormalities , Trachea/diagnostic imagingABSTRACT
Objective: Diabetic ketoacidosis (DKA) - a potentially preventable complication of type 1 diabetes mellitus (T1D) - is one of the most common chronic childhood diseases, and is associated with a significant risk of morbidity and mortality. The limited use of healthcare services due to fear of Coronavirus disease-2019 (COVID-19) transmission during the pandemic has raised concerns of delays in T1D diagnosis, among other diseases. This study investigated the presenting characteristics of newly diagnosed T1D patients assessed in a single clinic during the pandemic and compares them with the pre-pandemic period. Methods: For the purpose of this study, the first year of the pandemic is referred to as the "pandemic period", and the previous three years as the "pre-pandemic period". Patient files were reviewed retrospectively, the demographic and clinical characteristics and laboratory findings of the patients were recorded, and the findings from both periods were compared. Results: The number of patients diagnosed with T1D in the pandemic period was 44, and in the pre-pandemic period 39 in 2017, 22 in 2018 and 18 in 2019. The two groups had similar age, sex, pubertal stage and anthropometric characteristics (p>0.05). Regarding the type of presentation, the frequency of DKA was significantly higher in the pandemic period (68.2%) than in the pre-pandemic period (40.5%) (p=0.006), and this difference was also observed in the comparison by years (p=0.016). The duration of symptoms (16.5±10.7 vs. 23.5±17.6 days) and the length of hospital stay (10±3.9 vs. 15.2±5.5 days) were significantly shorter in the pandemic period (p=0.032, and p<0.001, respectively). There was no difference in the frequency of severe DKA between the pandemic (46.7%) and the pre-pandemic (37.5%) periods (p>0.05). However, pH (7.17±0.16 vs. 7.26±0.14) and bicarbonate (12.8±6.3 vs. 16.6±6.3) levels were significantly lower in the pandemic period (p<0.005). Additional signs of infection on admission were less frequent in the pandemic period (9.1%) than in the pre-pandemic period (27.8%) (p=0.027). The groups did not differ in terms of hemoglobin A1c, C-peptide, concurrent thyroid autoantibodies and tissue transglutaminase antibodies (p>0.05). The rate of anti-glutamic acid decarboxylase positivity was higher in the pandemic period (73.8% vs. 39.2%) (p=0.001) while the frequency of other diabetes-associated autoantibodies was similar between the groups (p>0.05). The polymerase chain reaction test for COVID-19 was negative in six patients with a history of contact. Conclusion: There was an increased frequency and severity of DKA in children with newly diagnosed T1D in the pandemic period, and these findings justify concerns related to the diagnosis of other diseases during the pandemic. Studies to raise awareness of diabetes symptoms during the pandemic should be continued regularly to reach all segments of society. Our study provides an additional contribution to the literature in its coverage of the one-year period during the pandemic and its comparison with the previous three years.
Subject(s)
COVID-19 , Diabetes Mellitus, Type 1 , Diabetic Ketoacidosis , Autoantibodies , COVID-19/diagnosis , COVID-19/epidemiology , Child , Diabetes Mellitus, Type 1/complications , Diabetes Mellitus, Type 1/diagnosis , Diabetes Mellitus, Type 1/epidemiology , Diabetic Ketoacidosis/complications , Humans , Pandemics , Retrospective StudiesABSTRACT
Sudden cardiac arrest (SCA) is the sudden cessation of regular cardiac activity so that the victim becomes unresponsive, with no signs of circulation and no normal breathing. Asystole, ventricular tachycardia (VT), ventricular fibrillation (VF), and pulseless electrical activity are the underlying rhythm disturbances in the pediatric age group. If appropriate interventions (cardiopulmonary resuscitation-CPR and/or defibrillation or cardioversion) are not performed rapidly, this condition progresses to sudden death. There have not been many reported cases of the approach and treatment of cardiac arrhythmias after SCA. Herein, we would like to report a case of a 15-year-old female patient with dilated cardiomyopathy (DCM) who was admitted to our clinic a year ago, and while her left ventricular systolic functions were improved, SCA suddenly occurred. Since the SCA event occurred in another city, intravenous treatment of amiodarone was done immediately and was switch to continuous infusion dose of amiodarone until the patient arrived at our institution's pediatric intensive care unit (PICU) 3 hours later. During the patient's 20-day PICU hospitalization, she developed pulseless VT and VF from time to time. The patient's pulseless VT and VF attacks were brought under control by the use of a defibrillator and added antiarrhythmic drugs (amiodarone, flecainide, esmolol, and propafenone). Intriguingly, therapy-resistance bigeminy with premature ventricular contractions (PVCs) continued despite all these treatments. The patient did not have adequate blood pressure measured by invasive arterial blood pressure monitoring while having bigeminy PVCs. The intermittent bigeminy PVCs ameliorated rapidly after intermittent boluses of lidocaine. In the end, multiple antiarrhythmic therapies and intermittent bolus lidocaine doses were enough to bring her cardiac arrhythmias after SCA under control. This case illustrates that malign PVC's should be taken very seriously, since they may predispose to the development of VT or VF. Also, this case highlights the importance of close vigilance of arterial pressure tracings of patients with bigeminy PVCs which develop after SCA and should not be accepted as normal.
ABSTRACT
Herein, we aimed to present a child with extremely severe hypertriglyceridemia (ESHTG) secondary to diabetic ketoacidosis concomitant with type IX glycogen storage disease (GSD). Extremely severe hypertriglyceridemia (10 700 mg/dL) was detected through the apparent lipemic appearance of the sampled blood in a 17-year-old male patient with severe diabetic ketoacidosis. In spite of insulin infusion, the patient's clinical condition deteriorated to acute pancreatitis. Single sessions of therapeutic plasma exchange (TPE) along with insulin treatment have successfully intercepted the progression of the state of acute pancreatitis. The patient was also diagnosed with type IX GSD on the basis of the genetic analyses performed for the potential underlying metabolic diseases. In conclusion, underlying metabolic diseases, such as glycogen storage disease, should be investigated in patients with diabetic ketoacidosis accompanied by severe hypertriglyceridemia. If ESHTG does not relieve despite insulin infusion, and/or acute pancreatitis occurs as a complication, TPE should be kept in mind.
Subject(s)
Diabetes Complications/complications , Diabetic Ketoacidosis/etiology , Diabetic Ketoacidosis/therapy , Glycogen Storage Disease/complications , Glycogen Storage Disease/therapy , Hypertriglyceridemia/therapy , Plasma Exchange/methods , Adolescent , Diabetic Ketoacidosis/physiopathology , Glycogen Storage Disease/pathology , Humans , MaleABSTRACT
Mitochondrial encephalomyopathy, lactic acidosis, and recurrent stroke-like episodes (MELAS) syndrome is a rare but one of the most common maternally inherited multisystem disorder. Although patients with MELAS present a variable clinical profile, strokelike lesions have been detected in 90% of cases, with stroke being the first presenting symptom in 25% of cases. However, cases of local brain edema requiring decompressive craniectomy has not been reported. A 12-year-old male patient was admitted to our pediatric intensive care unit with altered mental status, seizures, and vision loss. The patient was stuporous and presented neck stiffness. Complete blood count, serum electrolytes, biochemistry (including lactate level), acute phase reactants, and repeated blood gas analysis were unremarkable. Brain magnetic resonance imaging (MRI) revealed an edematous stroke-like lesion in the right occipital lobe accompanied by brain swelling. Intravenous ceftriaxone, acyclovir, intravenous immunoglobulin (IVIG), and pulse steroid therapy were started for possible diagnosis of viral/bacterial/autoimmune encephalitis; levetiracetam, phenytoin, and an infusion of sodium thiopental were started for refractory status epilepticus; and a 3% NaCl infusion was started for local brain edema. The results of serum autoimmune encephalitis panel were negative. Further investigations for rheumatic, vascular, and metabolic disorders were unremarkable. Despite these supportive treatments, the patient was clinically decompensated due to brain swelling that progressed to the left midline shift, and he underwent decompressive craniectomy. Histologic examination of brain biopsy specimen revealed non-specific encephalitis findings. A pathogenic variant of the MT-TL1 gene (m.3243A > T), responsible for MELAS, was detected. The patient?s condition dramatically improved after specific treatment for MELAS. If the diagnosis and treatment are delayed, MELAS syndrome can cause serious brain edema, which may ultimately require decompressive craniectomy.
Subject(s)
Brain Edema , Decompressive Craniectomy , MELAS Syndrome , Stroke , Brain/diagnostic imaging , Brain/surgery , Brain Edema/diagnostic imaging , Brain Edema/etiology , Child , Humans , MELAS Syndrome/complications , MELAS Syndrome/diagnostic imaging , Magnetic Resonance Imaging , MaleABSTRACT
BACKGROUND: Juvenile dermatomyositis associated interstitial lung disease, rarely seen in pediatric age groups, has adverse effects on survival. Anti-melanoma differentiation associated gene 5, one of the identified autoantibodies in juvenile dermatomyositis, preferentially affects the lung tissue and may cause rapidly progressive interstitial lung disease. It is a major cause of mortality in juvenile dermatomyositis. In this case report, we present a pediatric patient diagnosed with juvenile dermatomyositis without anti-melanoma differentiation associated gene 5 antibody positivity. CASE: A six-year-old male patient admitted to the Pediatric Intensive Care Unit with symptoms of respiratory failure, 1.5 months after the diagnosis of juvenile dermatomyositis. Thorax computed tomography examination revealed pneumomediastinum, a trace of left-sided pneumothorax, atelectasis on the left posterior lung region, ground-glass opacity, minimal subpleural patchy consolidation, and subcutaneous emphysema especially on the sides of the chest wall. Broad-spectrum antibiotics were started. His nasal swab sample was positive in terms of influenza B; therefore, oseltamivir was added to the treatment. Autoimmune myositis antibodies panel was examined but all of them including anti-melanoma differentiation associated gene 5 antibody resulted as negative. There was no notable reduction in lung infiltrations with the patient`s current treatment regimen. On the 12 < sup > th < /sup > day of Pediatric Intensive Care Unit admission, thorax computed tomography scan revealed progressed radiological lung findings compatible with rapidly progressive interstitial lung disease secondary to juvenile dermatomyositis. Despite intensive medical and extracorporeal treatments such as pulse steroid, intravenous immunoglobulin, methotrexate, cyclophosphamide, rituximab, therapeutic plasma exchange and, extracorporeal membrane oxygenation, the patient died on the 35 < sup > th < /sup > day. CONCLUSIONS: Juvenile dermatomyositis patients should be carefully monitored for the development of interstitial lung disease. Rapidly progressive interstitial lung disease with a high mortality may develop shortly after diagnosis, even if the anti-melanoma differentiation associated gene 5 antibody is negative.
Subject(s)
Dermatomyositis , Lung Diseases, Interstitial , Autoantibodies , Child , Dermatomyositis/diagnosis , Humans , Interferon-Induced Helicase, IFIH1 , Lung , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/etiology , MaleABSTRACT
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.
Subject(s)
Orthomyxoviridae , Respiratory Distress Syndrome , Aged , Child , Humans , Infant , Respiration, Artificial , Respiratory Distress Syndrome/etiology , Respiratory Distress Syndrome/therapy , Retrospective StudiesABSTRACT
Neurodevelopmental disorders (NDDs) are clinically and genetically heterogenous; many such disorders are secondary to perturbation in brain development and/or function. The prevalence of NDDs is > 3%, resulting in significant sociocultural and economic challenges to society. With recent advances in family-based genomics, rare-variant analyses, and further exploration of the Clan Genomics hypothesis, there has been a logarithmic explosion in neurogenetic "disease-associated genes" molecular etiology and biology of NDDs; however, the majority of NDDs remain molecularly undiagnosed. We applied genome-wide screening technologies, including exome sequencing (ES) and whole-genome sequencing (WGS), to identify the molecular etiology of 234 newly enrolled subjects and 20 previously unsolved Turkish NDD families. In 176 of the 234 studied families (75.2%), a plausible and genetically parsimonious molecular etiology was identified. Out of 176 solved families, deleterious variants were identified in 218 distinct genes, further documenting the enormous genetic heterogeneity and diverse perturbations in human biology underlying NDDs. We propose 86 candidate disease-trait-associated genes for an NDD phenotype. Importantly, on the basis of objective and internally established variant prioritization criteria, we identified 51 families (51/176 = 28.9%) with multilocus pathogenic variation (MPV), mostly driven by runs of homozygosity (ROHs) - reflecting genomic segments/haplotypes that are identical-by-descent. Furthermore, with the use of additional bioinformatic tools and expansion of ES to additional family members, we established a molecular diagnosis in 5 out of 20 families (25%) who remained undiagnosed in our previously studied NDD cohort emanating from Turkey.
Subject(s)
Genomics/methods , Mutation , Neurodevelopmental Disorders/epidemiology , Phenotype , Adolescent , Adult , Child , Child, Preschool , Cohort Studies , Female , Humans , Infant , Infant, Newborn , Male , Middle Aged , Neurodevelopmental Disorders/genetics , Neurodevelopmental Disorders/pathology , Pedigree , Prevalence , Turkey/epidemiology , Exome Sequencing , Young AdultABSTRACT
BACKGROUND: Research into new markers has been intensified for early diagnosis, prognosis, and differentiation of SIRS, sepsis, and septic shock in recent years. This study aimed to investigate the role of soluble triggering receptor expressed in myeloid cells-1 (sTREM-1) and interleukin (IL)-6 in distinguishing between systemic inflammatory response syndrome (SIRS), sepsis, and septic shock in pediatric intensive care unit (PICU) patients. METHODS: Between June 2014 and July 2015, 90 consecutive patients who were treated in the PICU were included in this prospective observational study. Patients were divided into four groups: control (n = 23), SIRS (n = 22), sepsis (n = 23), and septic shock (n = 22). All patients were evaluated for white blood cell (WBC), serum C-reactive protein (CRP), procalcitonin (PCT), IL-6, and sTREM-1 levels at 0, 24, and 72 h of admission. The prognostic evaluations were made using the Pediatric Risk of Mortality III (PRISM III) and Pediatric Logistic Organ Dysfunction (PELOD) scores. Patients were evaluated in terms of age, gender, prognosis, pathogen growth in culture, PRISM III and PELOD score, WBC, CRP, PCT, IL-6, and sTREM-1 levels and a comparison was made between groups. RESULTS: There was no significant difference between all groups in terms of the 0-, 24-, and 72-h sTREM-1 values (p = 0.761, p = 0.360, and p = 0.822, respectively). CRP and PCT values did not differ between the septic shock, sepsis, and SIRS groups at 0, 24, and 72 h. In the septic shock group, the 0-h IL-6 value was significantly higher than that of the SIRS group (p = 0.025). The 24-h IL-6 value in the septic shock group was significantly higher than the values of the sepsis and SIRS groups (p = 0.048 and p = 0.043, respectively). No significant difference was detected between the septic shock, sepsis, and SIRS groups in terms of IL-6 values at 72 h. CONCLUSION: sTREM-1 is not useful for the diagnosis of infection and for distinguishing between sepsis, septic shock, and SIRS since it does not offer a clear diagnostic value for PICU patients, unlike other reliable markers such as WBC, CRP, and PCT. Elevated IL-6 levels may indicate septic shock in PICU patients. More research on sTREM-1 is needed in this setting.
Subject(s)
Sepsis/diagnosis , Systemic Inflammatory Response Syndrome/diagnosis , Triggering Receptor Expressed on Myeloid Cells-1/analysis , Adolescent , Chi-Square Distribution , Child , Child, Preschool , Diagnosis, Differential , Female , Humans , Infant , Infant, Newborn , Intensive Care Units, Pediatric/organization & administration , Intensive Care Units, Pediatric/statistics & numerical data , Male , Prospective Studies , Statistics, NonparametricABSTRACT
Cardiovascular involvement is uncommon in pediatric patients with hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC-HUS). In this case report we presented a case of 17-month-old toddler who had a sporadic type of STEC-HUS complicated by acute myocarditis. The patient was successfully treated by a single dose of eculizumab after six doses of therapeutic plasma exchange (TPE) were inefficient to prevent the cardiac complication. Hepatotoxicity was observed after a single dose of eculizumab. Hepatic and cholestatic enzyme levels slowly returned to normal within 6 months. To the best of our knowledge, this is the first case of myocarditis/cardiomyopathy treated with eculizumab in STEC-HUS. This case illustrates the need for vigilance regarding myocardial involvement and eculizumab-induced hepatotoxicity in STEC-HUS.
ABSTRACT
BACKGROUND: Coronavirus disease 2019 (COVID-19) usually leads to a mild infectious disease course in children, while serious complications may occur in conjunction with both acute infection and neurological symptoms, which have been predominantly reported in adults. The neurological complications in these patients vary based on patient age and underlying comorbidities. Data on clinical features, particularly neurological features, and prognostic factors in children and adolescents are limited. This study provides a concise overview of neurological complications in pediatric COVID-19 cases. MATERIALS AND METHODS: The retrospective study reviewed medical records of all patients who were admitted to our hospital and were diagnosed with COVID-19 by real-time reverse-transcription polymerase-chain-reaction (RT-PCR) assay between 11 March 2020 and 30 January 2021. Patients with a positive PCR result were categorized into two groups: outpatient departments patients and inpatient departments (IPD). RESULTS: Of the 2530 children who underwent RT-PCR during the study period, 382 (8.6%) were confirmed as COVID-19 positive, comprising 188 (49.2%) girls and 194 (50.8%) boys with a mean age of 7.14±5.84 (range, 0-17) years. Neurological complications that required hospitalization were present in 34 (8.9%) patients, including seizure (52.9%), headache (38.2%), dizziness (11.1%) and meningoencephalitis (5.8%). CONCLUSION: The results indicated that neurological manifestations are not rare in children suffering from COVID-19. Seizures, headaches, dizziness, anosmia, ageusia and meningoencephalitis are major neurological manifestations during acute COVID-19 disease. Although seizures were the most common cause of hospitalization in IPD patients, the frequency of meningoencephalitis was quite high. Seizures were observed as febrile seizures for children under 6 years of age and afebrile seizures for those over 6 years of age. Febrile seizure accounted for half of all seizure children.
Subject(s)
COVID-19 , Adolescent , Adult , Child , Child, Preschool , Female , Headache/epidemiology , Headache/etiology , Humans , Infant , Infant, Newborn , Male , Retrospective Studies , SARS-CoV-2 , Seizures/epidemiology , Seizures/etiologyABSTRACT
BACKGROUND: Fulminant myocarditis (FM) is a potentially lethal condition in children due to rapid progressive hemodynamic instability and cardiogenic shock. Patients with FM might show different clinical manifestations on emergency department admission. CASE: Herein, we describe the case of a 12-year-old girl who was admitted to our institution's emergency department due to complaints of abdominal pain and incessant vomiting. However, we detected an early onset of atrial fibrillation (AF) accompanied by FM. The patient's condition of AF and severe hemodynamic disorder was successfully treated in our institution's pediatric intensive care unit. CONCLUSION: To the best of our knowledge, this is the first report of the co-occurrence of FM and AF successfully treated in childhood. This case report will serve as a guide for the treatment of cases with FM accompanied by AF.
ABSTRACT
Although Crimean-Congo hemorrhagic fever (CCHF) is mild and self-limited in children, some patients may develop excessive bleeding, massive liver necrosis, and multiple organ failure associated with secondary hemophagocytic lymphohistiocytosis (HLH) induced by cytokine storm. Treatment of CCHF is mainly symptomatic and supportive. The efficacy of ribavirin, which is the only antiviral drug in the treatment of CCHF, remains controversial. Although therapeutic plasma exchange (TPE) has been shown to beneficial in small case series with primary and secondary HLH, there is no pediatric patient with HLH secondary to CCHF treated with TPE in the literature. In this report, we describe the first pediatric patient who was successfully recovered from HLH secondary to CCHF with ribavirin, intravenous immunoglobulin, and TPE.
Subject(s)
Hemorrhagic Fever, Crimean/complications , Immunoglobulins, Intravenous/administration & dosage , Lymphohistiocytosis, Hemophagocytic/therapy , Plasma Exchange/methods , Ribavirin/administration & dosage , Adolescent , Humans , MaleABSTRACT
Streptococcus anginosus can be frequently isolated from brain abscesses, but is a rare cause of the liver, lung, and deep tissue abscesses. In this report, we present a patient with subdural empyema, brain abscess, and superior sagittal cerebral venous thrombosis as complications of rhinosinusitis whose purulent empyema sample yielded S. anginosus. A 13-year-old female patient was referred to our pediatric intensive care unit with altered mental status, aphasia, and behavioral change. On a brain computed tomography scan, subdural empyema extending from the left frontal sinus to the frontal interhemispheric area and left hemispheric dura was detected. Intravenous ceftriaxone, vancomycin, and metronidazole treatments were started. Subdural empyema was surgically drained. Postoperative brain magnetic resonance venography imaging showed superior sagittal sinus thrombosis. Cultures obtained from purulent empyema sample revealed S. anginosus. On the third day of hospitalization, a brain computed tomography scan showed brain edema, especially in the left hemisphere and significantly increased subdural empyema that had been previously drained. She was reoperated and decompressive craniectomy was performed. On the fifth day, partial epileptic seizures occurred. Brain magnetic resonance imaging showed a brain abscess on the interhemispheric area. The magnetic resonance imaging findings of abscess formation improved on 30th day and she was discharged on the 45th day after the completion of antibiotic therapy.
ABSTRACT
Developing an effective and safe vaccine against Covid-19 will facilitate return to normal. Due to hesitation toward the vaccine, it is crucial to explore the acceptability of the COVID-19 vaccine to the public and healthcare workers. In this cross-sectional survey, we invited 2251 pediatricians and 506 (22%) of them responded survey and 424 (84%) gave either nasopharyngeal swap or antibody assay for COVID-19 and 71 (14%) of them got diagnosis of COVID-19. If the effective and safe COVID-19 vaccine was launched on market, 420 (83%) of pediatrician accepted to get vaccine shot, 422 (83%) of them recommended vaccination to their family members, 380 (75%) of them accepted to vaccine their children and 445 (85%) of them offered vaccination to their pediatric patients. Among the participated pediatricians 304 (60%) of them thought COVID-19 vaccine should be mandatory. We found that there are high COVID-19 vaccine willingness rates for pediatricians for themselves, their own children, family members and their pediatric patients. We also found that being a pediatric subspecialist, believing in achieving an effective vaccine, willingness to participate in the phase 1-2 clinical vaccine trial, willingness to get an influenza shot this season, believing a vaccine and vaccine passport should be mandatory were significant factors in accepting the vaccine. It is important to share all information about COVID-19 vaccines, especially effectiveness and safety, with the public in a clear communication and transparency. The opposite will contribute to vaccine hesitancy and anti-vaccine movement.