Pseudohyperkalemia in pediatric patients with newly diagnosed hematological malignancies.

Patients with newly diagnosed hematological malignancies often present with a considerable cellular burden, leading to complications including hyperkalemia. However, pseudohyperkalemia, arising from in vitro cell lysis, can pose challenges in clinical practice. Although pseudohyperkalemia is frequently reported in adult hematological malignancies, its occurrence in pediatric patients is underreported, and its incidence in this demographic remains unclear. We retrospectively reviewed the medical records of pediatric patients who received a new diagnosis of hematological malignancies from 2011 to 2022 at Taichung Veterans General Hospital. Hyperkalemia was defined by a serum or plasma potassium level exceeding 5.5 mEq/L. Pseudohyperkalemia was defined by 1) a potassium decrease of over 1 mEq/L in within 4 h without intervention or 2) the absence of electrocardiography changes indicative of hyperkalemia. Cases with apparent red blood cell hemolysis were excluded. A total of 157 pediatric patients with a new diagnosis of hematological malignancies were included, 14 of whom exhibited hyperkalemia. Among these 14 cases, 7 cases (4.5%) were of pseudohyperkalemia. This rate increased to 21.2% in patients with initial hyperleukocytosis. Pseudohyperkalemia was associated with a higher initial white blood cell count and lower serum sodium level. All episodes of pseudohyperkalemia occurred in the pediatric emergency department, where samples were obtained as plasma, whereas all true hyperkalemia cases were observed in the ordinary ward or intensive care unit, where samples were obtained as serum. Timely recognition of pseudohyperkalemia is crucial to avoiding unnecessary potassium-lowering interventions in pediatric patients with newly diagnosed hematological malignancies.

Isolated partial anomalous origin of a branch pulmonary artery from the descending aorta.

The condition of partial anomalous origin of a branch pulmonary artery from the descending aorta could be found in several diseases and should be carefully differentiated. We report an unusual case of anomalous systemic arterial supply to normal basal segments of the left lower lung and another case of intralobar pulmonary sequestration. These two cases were treated successfully by transarterial embolisation using the Amplatzer Vascular Plug. We also set up a diagnostic algorithm to differentiate these diseases from anomalous systemic arterial supply to the pulmonary region. It is possible to make the correct diagnosis using the step-by-step diagnostic algorithm and careful interpretation of chest computed tomography angiography.

Detecting Patent Foramen Ovale after Cryptogenic Stroke - A Single Center Experience in Taiwan.

Background: Cryptogenic strokes related to a patent foramen ovale (PFO) in young patients have been reported. Percutaneous transcatheter occlusion is considered a safe and effective method for achieving PFO closure. Here, we present our experience of PFO closure in a series of young patients diagnosed with cryptogenic stroke. Methods: This was a retrospective study of 62 patients who were younger than 60 years of age and previously diagnosed with cryptogenic stroke. They underwent cardiac catheterization for the diagnosis of PFO and/or device closure. Bubble and echocardiography studies, the patients' characteristics, complications, and recurrence of stroke were recorded. The Risk of Paradoxical Embolism (RoPE) score index was also calculated. Results: Patients with a bubble study grade greater than grade I were likely to have PFO (odds ratio: 22.2 [95% confidence interval: 1.2-412.3]). The mean RoPE score in the PFO closure group was 7.18. Two patients (3.9%) developed atrial fibrillation during the procedure. Ischemic stroke recurred in two patients (3.3%). Conclusions: Combining a bubble study with transcranial Doppler ultrasound or transthoracic echocardiography may increase the validity of PFO diagnosis. Because of the low recurrence rate of stroke and low complication rate, it is important to identify PFO patients with a bubble study grade greater than grade I. Closure of PFO may be a good strategy for preventing recurrent stroke among such patients.

Ultrasonographic measurements of femoral vessel diameter in neonates weighing less than 2.5 kg.

BACKGROUND: Cannulation in low birth weight (LBW) neonates using larger sheaths could increase the risk of vascular injury. This study investigated the relationship between body weight (BW) and diameter of femoral vessels in LBW neonates and whether BW can be used to predict femoral vessel diameter. METHODS: The cohort included 100 neonates weighing < 2.5 kg (.57-2.42 kg) with a gestational age of 24-39 weeks. Vascular ultrasonography was used to measure diameters of the bilateral femoral arteries (FA) and veins (FV). The cohort was divided into four groups according to weight: group-A, 2-2.49 kg (n = 28); group-B, 1.5-1.99 kg (n = 38); group-C, 1-1.49 kg (n = 21); and group-D, < 1 kg (n = 13); or according to BSA: group-A, BSA > .16 m2 (n = 25); group-B, .13-.16 m2 (n = 40); group-C, .1-.13 m2 (n = 22); and group-D, < .1 m2 (n = 13). RESULTS: The median vessel diameters (mm) in groups A-D according to weight were FA, 1.96, 1.86, 1.78, and 1.53, and FV, 2.30, 2.28, 2.13, and 1.87, respectively. The median vessel diameters (mm) in groups A-D according to BSA were FA, 1.96, 1.86, 1.76, and 1.53, and FV, 2.30, 2.28, 2.05, and 1.87, respectively. There were positive correlations between BW and femoral vessel diameter (correlation coefficient: .56 and .55 between BW and FA and FV, respectively) (p < 0.001), and between BSA and femoral vessel diameter (correlation coefficient: .56 and .55 between BSA and FA and FV, respectively) (p < 0.001). CONCLUSIONS: BW is a predictor of femoral vessel diameter in LBW newborns. This finding may help to avoid using larger sheath in smaller vessels.

Transcatheter closure of post-procedural right ventricular pseudoaneurysms in a neonate with pulmonary atresia.

Post-procedural right ventricular pseudoaneurysm is a rare but life-threatening complication of interventional catheterisation. We describe a 3-day-old newborn who underwent transcatheter intervention for pulmonary atresia with a complication of right ventricular pseudoaneurysms, and transcatheter embolisation of the pseudoaneurysms was performed at 3-week-old. It is the first described case that receives transcatheter closure of right ventricular pseudoaneurysms in a newborn with a favourable outcome.

Urine catecholamines in children with severe Enterovirus A71 infection: comparison with paediatric septic shock.

Purpose: Hypercatecholaminemia-related heart failure has been proposed as the main cause of enterovirus A71-related (EV-A71) early mortality. The purpose of this study was to measure urine catecholamine concentrations in severe EV-A71-infected children. Methods: A total of 35 children, aged 2.5 ± 2.1 years, were divided into three groups. Group I: 15 septic shock patients, group II: 17 EV-A71-stage-2 patients, and group III: 3 EV-A71-stage-4 patients. The laboratory results, cardiac biomarkers and urine catecholamine concentrations were statistically analysed. Results: Group I had the highest C-reactive protein (CRP) levels and group II had the lowest B-type natriuretic peptide (BNP) and its N-terminal prohormone among the groups (p = 0.039, <0.01 and <0.01, respectively). Group III patients had significantly higher urine catecholamine and troponin-I values among the groups. If urine epinephrine (Epi) >134 ug/gCr, norepinephrine (NE) >176 ug/gCr and vanillylmandelic acid (VMA) >11.7 mg/gCr were used as the cutoff points to differentiate groups II and III, the sensitivities and specificity were all 100%. Conclusions: The significantly elevated urine catecholamine concentrations in EV-A71-stage-4 patients support the hypothesis that hypercatecholaminemia-related heart failure is involved in severe EV-A71 infection. Urine catecholamines could be used as reliable biomarkers for differentiation of severe EV-A71 infection with or without heart failure and septic shock.

Mid-term follow-up study of neonatal isolated aberrant right subclavian artery.

BACKGROUND: Aberrant right subclavian artery is the most common congenital aortic arch anomaly. There are a few reports concerning the clinical manifestations and follow-up of this CHD detected by echocardiographic screening. METHODS: A total of 1737 full-term neonates, with a male-to-female ratio of 900:837, received echocardiographic screening. Neonates with or without isolated aberrant right subclavian artery were studied during infancy. RESULTS: Among the 1737 cases, a total of 15 (0.86%) female-predominant neonates, with a male-to-female ratio of 6:9, had isolated aberrant right subclavian artery. They were compared with 20 age- and gender-matched normal neonates. There were no significant differences in maternal age, gestational age, or para gravity between the two groups, except for birth size (birth length- and weight-for-age percentiles, p = 0.006 and 0.045, respectively), which was smaller in the aberrant right subclavian artery group. Although there were no significant differences in developmental history, gastrointestinal, or respiratory symptoms, neonates with aberrant right subclavian artery had a higher incidence of mild developmental delay and feeding difficulty than normal infants (21 versus 0%, p = 0.061; 36 versus 20%, p = 0.264, respectively). The growth rates of body length and weight during infancy were not significantly different between the two groups. CONCLUSIONS: Echocardiography can be applied as a first-line investigation in the diagnostic work-up for aberrant right subclavian artery. Neonates with aberrant right subclavian artery had a smaller size at birth, although growth rates were not significantly different from those of normal infants. This study does not support an active surgical policy for asymptomatic infants with isolated aberrant right subclavian artery. A larger study and longer follow-up of affected infants is recommended.

Smartphone applications (apps) for heart rate measurement in children: comparison with electrocardiography monitor.

Heart rate (HR) measurement is essential for children with abnormal heart beats. The purpose of this study was to determine whether HR measurement by smartphone applications (apps) could be a feasible alternative to an electrocardiography (ECG) monitor. A total of 40 children, median age of 4.3 years, were studied. Using four free smartphone apps, pulse rates were measured at the finger (or toe) and earlobe, and compared with baseline HRs measured by ECG monitors. Significant correlations between measured pulse rates and baseline HRs were found. Both correlation and accuracy rate were higher in the earlobe group than the finger/toe group. When HR was <120 beats per min (bpm), the accuracy rates were not different between the two different measuring sites for each app (median of 65 vs 76%). The accuracy rates in the finger/toe group were significantly lower than those in the earlobe group for all apps when HR was ≥ 120 bpm (27 vs 65%). There were differences among apps in their abilities to measure pulse rates. Taking children's pulse rate from the earlobe would be more accurate, especially for tachycardia. However, we do not recommend that smartphone apps should not be used for routine medical use or used as the sole form of HR measurement because the results of their accuracy are not good enough.

EFFICACY OF INTRAVENOUS IMMUNOGLOBULIN ALONE ON CORONARY ARTERY LESION REDUCTION IN KAWASAKI DISEASE.

Background: Though Aspirin and intravenous immunoglobulin (IVIG) remain the standard treatments for Kawasaki Disease (KD) to minimize coronary artery damage, the duration and dosage of aspirin are inconsistent across hospitals. However, the lack of multi-center randomized trials prevents definitive answers to the impact of high-dose aspirin. Methods: This clinical trial was structured as a prospective, evaluator-blinded, multi-center randomized controlled trial with two parallel arms, aiming to assess the effectiveness of IVIG as a standalone primary therapy of KD in comparison to the combination of IVIG with high-dose aspirin therapy. KD patients were enrolled between September, 2016 and August, 2019. A final cohort of 134 patients were randomly assigned to the standard and test groups with 69 and 65 patients, respectively. The Standard group received IVIG (2 g/kg) along with aspirin (80-100 mg/kg/day) until fever subsided for 48 hours. The test group received IVIG (2 g/kg) alone. Following the initial treatment, both groups received a daily aspirin dose (3-5 mg/kg) for six weeks. The primary outcome measure was the occurrence of coronary artery lesions (CAL) at the 6-8 weeks mark. The secondary outcome is IVIG resistance. Results: The overall rate of CAL in test group decreased from 10.8% at diagnosis to 1.5% and 3.1% at 6 weeks and 6 months, respectively. The CAL rate of standard group declined from 13.0% to 2.9% and 1.4%, with no statistically significant difference (P>0.1) in the frequency of CAL between the two groups. Furthermore, no statistically significant differences were found for treatment (P>0.1) and prevention (P>0.1) effect between the two groups. Conclusions: This marks the first prospective multi-center randomized controlled trial comparing the standard treatment of KD using IVIG plus high-dose aspirin against IVIG alone. Our analysis indicates that addition of high-dose aspirin during initial IVIG treatment is neither statistically significant nor clinically meaningful for CAL reduction. Registration: URL: http://www.clinicaltrials.gov ; identifier: NCT02951234. What is New?: This study represents the first multi-center randomized controlled trial investigating the efficacy of high-dose aspirin or intravenous immunoglobulin (IVIG) during the acute stage of KD. This study assessed the impact of discontinuing high-dose aspirin (80-100 mg/kg/day) on the occurrence of CAL during the acute phase treatment of Kawasaki Disease.No significant differences were observed between high-dose aspirin plus IVIG treatment and IVIG alone treatment in terms of the frequency of abnormal coronary artery abnormalities. Additionally, our analysis revealed no statistically significant differences in either the treatment effect (the number of cases successfully treated) or prevention effect (the prevention of new cases) between these two treatments. What Are the Clinical Implications?: Comparison analysis indicated the non-inferiority between two groups with or without high-dose aspirin.Administering the standard 2 g/kg/day IVIG without high-dose aspirin (80-100 mg/kg/day) during the acute phase therapy for KD does not increase the risk of coronary artery lesions, which are a primary cause of morbidity and mortality in KD patients.Addition of high-dose aspirin during initial IVIG treatment is not statistically significant or clinically meaningful.

The association between Kawasaki disease and allergic diseases, from infancy to school age.

Kawasaki disease (KD) is the most common acquired heart disease among preschool children in most industrialized countries. An atopic trend after KD illness has been observed in epidemiological studies. This is consistent with the findings of elevated IgE levels and increased IL-4 in KD patients. However, studies on the early allergic association among children with KD are still limited. This study aimed to evaluate the association between KD and allergic diseases, from infancy to school age. Allergic diseases included atopic dermatitis, allergic rhinitis (AR), asthma, and urticaria. This matched case-control study used the National Health Insurance Research Database of Taiwan as its data source. Patients born between 1997 and 2004 and with a main diagnosis of KD were retrieved for analysis. A 1:4 matched control group was selected by zip code, gender, and age. The prevalence rates and progression sequence of allergic manifestations were analyzed. During the first 5 years of life, children with KD had higher rates of allergic manifestations. Both groups have similar atopic march. In 2010, at the age of 6-13 years, there were 7072 children with KD and 27,265 children without KD. Children with KD had more AR (odds ratio [OR], 1.30; 95% confidence interval [CI], 1.22-1.38) and asthma (OR, 1.16; 95% CI, 1.05-1.27) than controls. Children with KD have a higher allergic susceptibility recognized from their 1st year of life. The atopic tendency persists until school age. Additional studies are needed to elucidate the underlying determinants of this distinct immune phenotype.

Asymptomatic Right Ventricular Perforation by a Temporary Transvenous Pacing Lead in an Infant.

UNLABELLED: A 3-month-old male infant received transvenous temporary pacemaker for acquired complete atrioventricular block. However, a right ventricle perforation by the pacing lead was found incidentally when the patient was receiving permanent pacemaker implantation nineteen days after temporary pacing. There was no pacemaker dysfunction, active bleeding or hemopericardium. The temporary pacing lead was extracted and the site of perforation was sutured. This case is unique because of the patient's unusual clinical presentation, and we suggested that echocardiography should be performed cautiously to check the pacing catheter tip daily, even if asymptomatic and/or normal function of pacemaker. KEY WORDS: Complete atrioventricular block; Right ventricle perforation; Transvenous pacing lead.

Case report: Transcatheter arterial embolization in a newborn with cervical rapidly involuting congenital hemangioma and Kasabach-Merritt phenomenon.

Congenital hemangiomas (CHs) are rare vascular tumors and do not exhibit progressive postnatal growth. The incidence is less than 3% of all hemangiomas. Most CHs have a favorable prognosis; however, the Kasabach-Merritt phenomenon (KMP) is a rare but life-threatening complication in CHs that requires aggressive treatment. Medical treatments with corticosteroids and interferon have been suggested. Surgical resection can be considered for the treatment of complicated CHs in medically resistant lesions. Vascular embolization could be an alternative method if surgery is not considered feasible. Herein, we report a case of a 9-day-old newborn who underwent arterial embolization for a CH with KMP, combined with sirolimus treatment, and the outcome was favorable. The hemangioma completely regressed by 3 months and rapidly involuting congenital hemangioma (RICH) was diagnosed. Our successful experience with treating RICH associated with KMP revealed that RICH can have potentially serious complications although they usually resolve rapidly after birth without treatment. Surgical resection is considered to be the standard method for the treatment of medically resistant vascular tumors, but it is difficult to perform during the active phase of KMP due to acute bleeding and severe coagulopathy. Arterial embolization is feasible and can be used as an alternative to surgical resection, even in small babies.

Case report: VA-ECMO for fulminant myocarditis in an infant with acute COVID-19.

Fulminant myocarditis in children was rare during the coronavirus disease 2019 pandemic, but it had the potential for high morbidity and mortality. We describe the clinical course of a previously healthy 9-month-old young male infant who rapidly deteriorated into cardiogenic shock due to coronavirus disease 2019-related fulminant myocarditis. He developed severe heart failure and multiple organ dysfunction syndrome that were treated promptly with central venoarterial extracorporeal membrane oxygenation and continuous venovenous hemofiltration. He made a good recovery without significant morbidity.

Case Report: One heart with two lobes: a rare infantile congenital giant left atrial appendage aneurysm.

Left atrial appendage aneurysm (LAAA) is an extremely rare congenital heart abnormality, with varying degrees of symptoms, ranging from asymptomatic to arrhythmia, thromboembolic event or airway obstruction. Most infantile cases were incidentally found by echocardiography. Contrast-enhanced chest tomography can confirm the diagnosis and inform surgical plan. We describe an asymptomatic young female infant who had a unique extreme cardiomegaly on a chest x-ray and received surgical aneurysmectomy. Her heart was restored to a normal cardiac size after the heart surgery.

Novel technique to reduce prolapsed device in atrial septal defect closure.

Objective: Transcatheter closure of atrial septal defect (ASD) has become an alternative treatment to surgical repair. One of the challenges is the prolapse of the left atrial disc during the procedure. Many techniques have been developed to prevent the prolapse but not reduce it. In this study, we present a novel technique, termed push back technique, that help reduce the prolapsed device. Methods: We enrolled 24 patients (8 males, 16 females) between May 2008 and January 2023 who underwent the push back technique during transcatheter closure of ASD in Taichung Veterans General Hospital. We recorded the hemodynamic data, success rate and complications including device embolization/migration, valvular regurgitation, pericardial effusion, and residual shunt. Results: The median age was 6.3 years (1.2-70.5 years) and the median weight was 19.1 kg (7.8-90 kg). Fifteen (62.5%) patients had mild pulmonary hypertension. The median Qp/Qs was 2.54 (1.5-8.8). The median ASD stretched size was 21.2 mm (7.7-35.3 mm). The median device size was 22 mm (8-40 mm). The median fluoroscopy time was 14 min (5-23 min) and median procedure time was 47 min (25-78 min). The push back technique successfully reduced the prolapsed device in 21 (87.5%) patients. There was no complication in all patients. Conclusion: We present a novel push back technique that can successfully reduce the prolapsed device in 87.5% (21/24) patients without complications. It is feasible, safe and effective.

B-type natriuretic peptide in children with atrial or ventricular septal defect: a cardiac catheterization study.

In this study, we investigated the relationship between plasma B-type natriuretic peptide (BNP) levels and hemodynamics from cardiac catheterization in pediatric patients with atrial or ventricular septal defect. A total of 59 patients were studied including 80% of patients had Qp/Qs > 1.5 and 25% of patients had pulmonary hypertension. The mean BNP value and BNP z-score were 10.9 ± 11.2 pg/mL and -0.28 ± 1.7 (-2.85 to 3.29), respectively. There was a statistically significant linear correlation between BNP value and the size of defects (r = 0.303, p = 0.002) and a trend toward to positive correlation between BNP value and Qp/Qs ratio (r = 0.183, p = 0.166) among all patients. To identify patients with a Qp/Qs ratio >1.5, the sensitivity and specificity were 28%, 100% in all patients at a plasma BNP cut-off point of 15 pg/mL. We concluded that a BNP > 15 pg/mL would help identify patients who need further intervention.

Pseudohyperkalemia accompanying actual hyperphosphatemia and hypocalcemia in an adolescent with T-lymphoblastic lymphoma.

Tumor lysis syndrome (TLS) is a life-threatening condition that may occur in patients with lymphoma, leukemia, or cancers with high cellular burdens. Without appropriate treatment, electrolyte imbalances, namely hyperkalemia, hyperphosphatemia, and hypocalcemia, can be fatal in patients with TLS. In pseudohyperkalemia, concurrent hyperphosphatemia and hypocalcemia can render devising a treatment strategy challenging. We report an adolescent with T-lymphoblastic lymphoma who presented with pseudohyperkalemia but actual hyperphosphatemia and hypocalcemia, to highlight the importance of accurate clinical interpretations of laboratory data in patients with TLS.