Correlation between echocardiographic estimation of right atrial pressure and invasive measurement of central venous pressure in postoperative pediatric patients with congenital heart disease: a prospective observational study.

BACKGROUND: Right atrial pressure plays a critical role as a hemodynamic parameter in diagnosing pulmonary hypertension and other cardiac diseases, as well as guiding the treatment and prognosis of various cardiac disorders. If there is no obstruction between the inferior or superior vena cava (SVC) as central veins and the right atrium, the pressures in these veins could be considered equal to the right atrial pressure. This study aimed to examine the correlation between echocardiographic methods for estimating right atrial pressure and invasive measurements of central venous pressure (CVPi) in infants and children with congenital heart disease during the 48 h after cardiac surgery and to establish regression equations for echocardiographic estimation of central venous pressure (CVPe). RESULTS: We prospectively enrolled 43 infants and children, ranging in age from 6 months to 16 years, including 20 males and 23 females. We found a significant correlation between CVPi and the ratio of the maximal diameter of IVC to the maximal diameter of the descending aorta ratio (IVCmax/DAOmax) (r = 0.529, P < 0.001), SVCS/D velocity ratio (SVCS/D) (r = 0.462, P = 0.006), right atrial vertical diameter (RAVD) (r = 0.409, P = 0.01), area (r = 0.384, P = 0.014), and tricuspid valve A wave acceleration rate (TVAAR) (r = 0.315, P = 0.048). Multiple regression analysis yielded an equation for estimating central venous pressure using four parameters related to the IVC, SVC, tricuspid valve, and right atrium. The equation is as follows: estimated CVP = 4.36 + (2.35 × IVCmax/DAOmax) + (1.06 × SVCS/D) + (0.059 × RAVD) + (0.001 × TVAAR). This equation is strongly correlated with CVPi (Pearson r = 0.698, P = 0.002). CONCLUSIONS: The estimation of central venous pressure through a multi-parametric equation that included the ratio of the maximal diameter of the inferior vena cava to the maximal diameter of the descending aorta, the ratio of S to D velocity of the superior vena cava, the vertical diameter of the right atrium, and the acceleration rate of the A wave of the tricuspid valve demonstrated a robust correlation with invasively measured central venous pressure. To assess the accuracy of predicted pressures by this equation, further investigations are required to apply this innovative multi-parametric formula to a prospective population of pediatric patients with congenital heart disease.

Prevalence, risk factors, and outcomes of acute kidney injury in a pediatric cardiac intensive care unit: A cross-sectional study.

Background and Aims: Acute kidney injury (AKI) is a common complication in pediatric cardiac intensive care unit (CICU). This study aims to identify the prevalence, risk factors, and outcomes of AKI in pediatrics admitted to a CICU unit of a tertiary hospital. Methods: We retrospectively gathered the data of 253 randomly selected patients admitted to the CICU unit from March 2018 to March 2022. Data were collected from EHRs. We used the Kidney Disease Improving Global Outcomes (KDIGO) criteria for identifying AKI in patients. Results: Overall, AKI prevalence was 22.9% in our population. In the multivariable analysis, vancomycin intake (odds ratio [OR]: 2.109, 95% confidence interval [CI]: 1.15-3.84), angiography (OR: 4.38, 95% CI: 1.28-14.93), and mechanical ventilation (OR: 2.08, 95% CI: 1.02-4.23) were independent risk factors of AKI development and patients with AKI had a higher in-hospital mortality rate (OR: 5.81, 95% CI: 2.55-13.19), higher need for cardiopulmonary resuscitation (OR: 3.08, 95% CI: 1.17-8.09), and longer ICU length of stay (OR: 6.49, 95% CI: 3.31-9.67). Furthermore, furosemide administration was associated with lower risk of developing AKI (OR: 0.52, 95% CI: 0.27-0.97). Conclusion: AKI is common and is associated with worse outcomes in patients with congenital heart disease. Our results emphasize the importance of early identification and monitoring of AKI in the pediatric CICU setting.

The characteristics of Guillain-Barre syndrome in children in pre-COVID-19 and during the COVID-19 pandemic: A cross-sectional study.

Background and Aims: In the pathophysiology of Guillain-Barre syndrome (GBS), inflammation and immunity are believed to play a key role. The neutrophil-lymphocyte ratio (NLR), monocyte-lymphocyte ratio (MLR), and platelet-lymphocyte ratio (PLR) have been recently identified as potential markers of inflammation or immunity. This study aimed to investigate whether NLR, MLR, and PLR are associated with GBS characteristics in children. We also assessed the impact of the COVID-19 pandemic on the characteristics of GBS in Iran. Methods: In this retrospective cross-sectional study, we reviewed the records of all 150 children diagnosed with GBS in the Children's Medical Center hospital affiliated with Tehran University of Medical Sciences (TUMS) from March 2017 until March 2022. The TUMS research ethics committee approved the study (Ethics code: IR.TUMS.CHMC.REC.1399.125). Patients' data including gender, age, clinical symptoms, laboratory findings, and electrodiagnostic study results were collected and analyzed. Results: This study involved 150 children, comprising 93 boys and 57 girls, with an average age of 7.53 ± 3.75 years. The analysis demonstrated that the number of hospitalization days increased with an increase in NLR (p = 0.025). Moreover, patients with abnormal electrodiagnostic study patterns had a higher risk of intensive care unit (ICU) admission (p: 0.027), although according to binary logistic regression, respiratory failure at admission time was the only significant factor increasing the risk of ICU admission (p = 0.035). The study also found that the pandemic has resulted in a shift from acute inflammatory demyelinating polyneuropathy to acute motor axonal neuropathy as the most common EMG-NCV pattern in our patients (p < 0.001). Conclusion: We found that higher NLR was associated with a longer hospitalization duration and could potentially distinguish between severe and mild cases of GBS. We have also shown that the COVID-19 pandemic has changed our patients' most frequent electromyography and nerve conduction velocity (EMG-NCV) patterns.

Giant Coronary Aneurysms with Multiple Large Resistant Thromboses in an 8-Month-Old Boy with IVIg-Resistant Kawasaki Disease: A Case Report.

Kawasaki disease is an acute self-limiting systemic vasculitis in childhood, resulting in arterial swelling or inflammation and eventually leading to cardiovascular problems, such as coronary artery aneurysms. Based on previous studies, serum sodium ≤133 mmol/L, albumin ≤3.2 g/dL, alanine transaminase ≥80 U/L, and neutrophil percentage ≥80% at diagnosis are risk factors for intravenous immunoglobulin (IVIg). However, the prevalence of resistance to Ig among children with Kawasaki disease varies among different countries due to diversity in evaluation, treatment, and diagnosis. Approximately, 10% to 20% of patients have IVIg-resistant Kawasaki disease. As the probability of coronary artery damage associated with IVIg-resistant Kawasaki disease is higher than that with IVIg-sensitive Kawasaki disease, the early detection and appropriate treatment of IVIg-resistant Kawasaki disease can decrease the probability of damage to coronary arteries and hospital lengths of stay and cost. Kawasaki disease in early infancy is uncommon, and sometimes it occurs with thrombosis and peripheral gangrene. A positive genetic background may play a role in susceptibility to thrombosis. We herein describe a patient suffering from an IVIg-resistant Kawasaki disease with severe coronary artery thrombosis and positive genetic mutation. Medical treatment resolved the thrombosis, but the coronary arteries remained dilated.

COLQ-related congenital myasthenic syndrome: An integrative view.

Congenital myasthenic syndromes are inherited disorders caused by mutation in components of the neuromuscular junction and manifest early in life. Mutations in COLQ gene result in congenital myasthenic syndrome. Here, we present the analysis of data from 209 patients from 195 unrelated families highlighting genotype-phenotype correlation. In addition, we describe a COLQ homozygous variant a new patient and discuss it utilizing the Phyre2 and I-TASSER programs. Clinical, molecular genetics, imaging (MRI), and electrodiagnostic (EEG, EMG/NCS) evaluations were performed. Our data showed 89 pathogenic/likely pathogenic variants including 35 missenses, 21 indels, 14 nonsense, 14 splicing, and 5 large deletions variants. Eight common variants were responsible for 48.46% of those. Weakness in proximal muscles, hypotonia, and generalized weakness were detected in all individuals tested. Apart from the weakness, extensive clinical heterogeneity was noted among patients with COLQ-related patients based on their genotypes-those with variants affecting the splice site exhibited more severe clinical features while those with missense variants displayed milder phenotypes, suggesting the role of differential splice variants in multiple functions within the muscle. Analyses and descriptions of these COLQ variants may be helpful in clinical trial readiness and potential development of novel therapies in the setting of established structure-function relationships.

Incidence of neurological complications following pediatric heart surgery and its association with neutrophil-to-lymphocyte ratio.

Background and Aims: Due to increased rate of open-heart surgeries in children, postsurgical mortality and morbidities have increasingly gained attention. Neurological complications are some of the most important postsurgical events. However, the number of studies regarding postsurgical neurological complications seems to be inadequate. We aimed to study the incidence of neurological complications following cardiac surgery in the pediatric cardiac intensive care unit (ICU) of the children's medical center. Methods: This cross-sectional study was conducted from March to September 2019. We included all of the children who underwent cardiac surgery and were admitted to ICU at CMC. We collected their demographic data, lab test results (white blood cell count, absolute neutrophile and lymphocyte counts) and calculated their Risk Adjustment for Congenital Heart Surgery (RACHS) score. We then documented neurological adverse events and investigated the associations between those events and the patients' data. Results: Of the 267 studied patients, 14 developed neurological complications (5.2%); seven developed chorea (2.6%), four developed seizures (1.5%), and two developed both seizure and chorea (0.7%). One case developed subarachnoid hemorrhage (SAH). We observed that age (p = 0.000), weight (p = 0.000), and RACHS score (p = 0.006) were associated with the development of neurological complications. Additionally, we observed that "neutrophil to lymphocyte ratio" was not associated with the risk of postsurgical neurological complications. Conclusion: Younger age, lower weight, and higher RACHS score were associated with neurological complications after operations. Given the importance of postsurgical neurological complications, further investigations should be carried out to cover this issue and discover preventive strategies for such morbidities.

Catastrophic Brain Aspergillosis after Liver Transplantation.

Background. Liver transplantation has many complications. Because of receiving immunosuppressive regimens, infectious complications in these patients may have fatal results. Aspergillosis in solid organ recipients is one of the most common fungal infections that usually occur 1 month after transplantation. Aspergillus infection mainly involves the lungs. Although the central nervous system may get involved due to hematogenous spreading from lungs, isolated central nervous system involvement is rarely reported. Case Presentation. The patient was an 8-year-old boy, with a case of Wilson disease, who underwent liver transplantation due to acute fulminant hepatic failure. Four days after the surgery, he was affected by fever, agitation, loss of consciousness, hemiparesis, and focal seizure. Brain MRI showed abscess formation, whereas chest X-ray was normal. Intravenous antibiotics were initiated but the patient's condition was not improving; therefore, surgical drainage of the abscess was performed. The pathological investigation was compatible with aspergillosis. Antifungal therapy with voriconazole was administrated. His symptoms were resolved but unfortunately, brain lesions caused persistent vegetative state. Discussion. Aspergillus is a ubiquitous organism that mainly occurs in immunocompromised patients. Aspergillosis could be prevented by environmental modification such as installing high-efficiency particulate air filters. Chemoprevention with triazoles, echinocandins, and polyenes is also effective. Voriconazole is the drug of choice for aspergillosis treatment. Although voriconazole is a highly effective antifungal drug, cerebral aspergillosis is often fatal.

Potential Prognostic Markers in the Heart Rate Variability Features for Early Diagnosis of Sepsis in the Pediatric Intensive Care Unit using Convolutional Neural Network Classifiers.

Blood infection due to different circumstances could immediately develop to an extreme body reaction that leads to a serious life-threatening condition, called Sepsis. Currently, therapeutic protocols through timely antibiotic resuscitation strategies play an important role to fight against the adverse conditions and improve survival. Therefore, timing, and more specifically early diagnosis of the illness, is crucially important for an effective treatment. Studies have indicated that vital signals such as heart rate variability (HRV) could provide potential prognostic biological markers that can help with early detection of sepsis before it is clinically diagnosed through its actual symptoms. Therefore, this study employs neonatal and pediatric electrocardiogram (ECG) to extract 52 hourly sets of linear and non-linear features from the HRV, starting from 24 hours prior to the clinical diagnosis of sepsis in patients with positive blood cultures (n=14). Similar sets of features were also obtained from a non-sepsis control group to create an evaluation benchmark (n=14).In particular, this study initially demonstrates how the variations within the 24 hours values of specific HRV feature-sets could effectively reveal prognostic information about the evolution of sepsis, prior to the actual clinical diagnosis. Moreover, this study demonstrates that differences in the values of a particular set of features at 22 hours before the actual clinical diagnosis/symptoms can be reliably used to train a convolutional neural network for automatic classification between the individuals in the sepsis and non-sepsis groups with 88.89±7.86% accuracy.

Potential Prognostic Markers in the Heart Rate Variability Features for Early Diagnosis of Sepsis in the Pediatric Intensive Care Unit using Convolutional Neural Network Classifiers.

Blood infection due to different circumstances could immediately develop to an extreme body reaction that leads to a serious life-threatening condition, called Sepsis. Currently, therapeutic protocols through timely antibiotic resuscitation strategies play an important role to fight against the adverse conditions and improve survival. Therefore, timing, and more specifically early diagnosis of the illness, is crucially important for an effective treatment. Studies have indicated that vital signals such as heart rate variability (HRV) could provide potential prognostic biological markers that can help with early detection of sepsis before it is clinically diagnosed through its actual symptoms. Therefore, this study employs neonatal and pediatric electrocardiogram (ECG) to extract 52 hourly sets of linear and non-linear features from the HRV, starting from 24 hours prior to the clinical diagnosis of sepsis in patients with positive blood cultures (n=14). Similar sets of features were also obtained from a non-sepsis control group to create an evaluation benchmark (n=14).In particular, this study initially demonstrates how the variations within the 24 hours values of specific HRV featuresets could effectively reveal prognostic information about the evolution of sepsis, prior to the actual clinical diagnosis. Moreover, this study demonstrates that differences in the values of a particular set of features at 22 hours before the actual clinical diagnosis/symptoms can be reliably used to train a convolutional neural network for automatic classification between the individuals in the sepsis and non-sepsis groups with 88.89±7.86% accuracy.Clinical relevance- Results suggest potential early diagnosis of sepsis through real-time automatic classification of HRV features as prognostic indicators in clinical ECG recordings.

Antibiotic Susceptibility of Bacterial Agents Causing Meningitis in Children Older than 1 Month.

The frequency and antibiotic susceptibility of bacterial meningitis in children older than one month at Children's Medical Center during 2012-2017 were evaluated in this study. The CSF samples were cultured, and antibiotic sensitivity tests were performed. The samples were cultured on chocolate agar, blood agar (for gram positive) and Mkanky (for gram negative). The antimicrobial susceptibility of the isolates was determined using the disc diffusion method. In total, 72 samples were positive for bacterial infection where Staphylococcus epidermidis (20.8%) was seen most and Enterobacter (1.4%) and Pseudomonas aeruginosa (1.4%) was seen least. Most of these patients were under 1 year of the age and overall frequency of positive cultures of CSF in men (58.3%) was greater than women (41.7%). Bacterial meningitis has relatively diverse etiological factors that include; time of infection, geographical location and age. Most commonly seen bacteria were Staphylococcus aureus with S.epidermidis whereas, Escherichia coli and Pseudomonas aeruginosa was seen least. This result can be substantial in hospitalized patients, because these bacteria are also the result of nosocomial infections.

Identifying Optimal Features from Heart Rate Variability for Early Detection of Sepsis in Pediatric Intensive Care.

Sepsis as bacterial infection is the most common and costly causes of mortality in critically ill patients. The early diagnosis of sepsis is significantly important for effective treatment. In this study, over a period of two years, the electrocardiogram of nearly 500 pediatric and neonate patients with heart diseases were collected in 24 hours before diagnosis. The collected data of 22 patients were studied including 11 sepsis patients with positive blood cultures and 11 non-sepsis patients. After extracting the HRV (Heart Rate Variability) signal, 28 linear and nonlinear features according to previous research were extracted. By using the relative entropy method as a feature selection technique, the extracted features were evaluated for their ability to discriminate the data in sepsis and non-sepsis groups, and the best features were entered into the classification process. Using the four classification models of SVM, LDA, KNN and Decision Tree, the accuracy of 86.36% was obtained with Decision Tree for discrimination of sepsis patients from other patients.

Brain on FIRES: Super Refractory Seizure in a 7 yr Old Boy.

We present a 7 yr old boy afflicted with super-refractory seizure that responded poorly to antiepileptic drugs and sustained a long course of hospitalization and complications of high doses of medications as well as longstanding stay in hospital. The differential diagnoses were, fever-induced refractory epileptic encephalopathy (FIRES), and infectious and autoimmune encephalitis. However, work-ups had not revealed any evidence of any specific diagnosis, so we assumed that he was afflicted by viral infectious encephalitis as he had, fever, vomiting, and prodromal symptoms of infectious (most probably viral) disease prior to onset of the seizure attacks.

Nicolau Syndrome due to Penicillin Injection: A Report of 3 Cases without Long-Term Complication.

Nicolau syndrome (NS) or livedo-like dermatitis is a rare complication of injection of various medications such as penicillin. The pathophysiology of this events is not clear, but some hypotheses are suggested, such as sympathetic nerve stimulation, embolic occlusion, inflammation, or mechanical injury. In this paper we report 3 cases of NS following benzathine penicillin. Clinical symptoms improved in 2 cases during 2-month follow-up, but one of them had a residual necrosis in the distal phalanges of the toes.

Caustic Agent Ingestion by a 1.5-Year-Old Boy.

We present a case of caustic ingestion by a 1.5-year-old boy. The caustic agent was drain opener which is a strong alkaline substance. Children in Iran and many other countries are still exposed to not "child proof" (child resistant packaging) toxic substance containers. Ingestion of caustic agents may lead to necrosis, perforation, and strictures. Substances that are ingested more frequently are liquid alkali material which causes severe, deep liquefaction necrosis. Common signs and symptoms of caustic agents are vomiting, drooling, refusal to drink, oral burns, stridor, hematemesis, dyspnea, dysphagia and abdominal pain. Even if no oropharyngeal lesion is seen, a significant esophageal injury which can lead to perforation and stricture cannot be ruled out. If abdominal pain or rigidity, substernal, chest or back pain exists, visceral perforation should be considered. The first thing to be checked is airway assessment. A lot of patients should be admitted to intensive care unit, and endoscopic evaluation, surgical intervention, long-term hospitalization, and worsening quality of life or among the complications. Preventive measures especially at the country level and approving proper legislation for obligating the related industries to produce child proof containers for house hold toxic products are the urgent measures to be followed by all of us.

A 15-Month-Old Boy With Respiratory Distress and Parapharyngeal Abscess: A Case Report.

Parapharyngeal abscess is a life-threatening disease. Upper respiratory tract infection is the main cause in children. We present a 15-month-old boy admitted to the emergency ward with the chief complaint of difficulty in breathing caused by parapharyngealabscess. His condition deteriorated gradually, and he transferred to the operation theater quickly for abscess drainage and because of the difficulty in orotracheal intubation; a tracheostomy was performed. His respiratory condition deteriorated 2 days after PICU admission, and the medical team noticed an unexplainable respiratory distress. A chest x ray obtained and showed a right side pneumothorax and subcutaneous emphysema around theneck area. The case presented here, had not been diagnosed at the first examination; however, there were enough clinical clues (such as respiratory distress, drooling, torticollis, bulging of theneck, previous viral respiratory infection, possible pharyngeal trauma). The story of this case reminds us the importance of the precise physical exam and history taking which could be life-saving.

Be careful of lies: a six-year-old boy with respiratory distress and decreased level of consciousness.

We reports a six-year-old child with autistic disorder and methadone intoxication. Developmental disorders may mislead the caretakers and doctors to interpret the clinical manifestation and behavioral changes of these patients. Methadone indigestion can be fatal in children even in a tiny amount and cardiac monitoring should be performed at least for the first 24 hours. The pervasive prescription of methadone for detoxification programs and its extensive availability at homes of the addicted parents should point parents, doctors and media to pay more attention to provide safe-home environment for children. 

A neonate with indurate dermal papules and nodules and pneumonia: a case report.

We presents an infant with several indurated plaques and nodules scattered on her body. She was brought to the hospital because of fever, runny nose and cough from one month ago. During the examination and investigation the plaques and nodules grabbed the attention of the clinicians and the skin biopsy and other lab works revealed the diagnosis of congenital leukemia.

Sudden impolite behavior in a polite girl: a case report.

In this case we present a previously healthy child with sudden behavioral change and acute confusional state (ACS). ACS is a neurologic emergency. The clinical manifestations of ACS are; confused affect, limited verbal response, disturbance in performing orders, some automatism, stable vital signs and absence of tonic-clonic movements and it has a wide range of differential diagnosis which should be assessed by detailed medical history, medical examination and laboratory tests. The only abnormal test found in this patient, was cerebrospinal fluid abnormality, consistent with viral encephalitis.