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.

Remission and long-term remission of pediatric-onset systemic lupus erythematosus.

Objectives: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical presentations and prognoses. Remission can be achieved with or without glucocorticoid (GC) use, and several recent studies have suggested that long-term remission can be achieved in a small portion of patients. Nevertheless, few studies have investigated remission or long-term remission in the pediatric-onset SLE subgroup. This study analyzed the characteristics and factors associated with long-term remission and GC use in pediatric-onset SLE. Methods: We enrolled 226 patients aged <18 years who received a diagnosis of SLE between January 2006 and December 2016. Three remission condition groups were defined: (A) complete remission, (B) clinical remission off GCs, and (C) clinical remission on GCs. Long-term remission was defined as remission for more than 5 years. We analyzed the treatment durations before remission, durations of remission, and risk factors for non-remission with persistent GC use. Results: During follow-up, 8 patients (3.5%) achieved complete remission, 35 patients (15.5%) achieved clinical remission off GCs, and 93 patients (41.2%) achieved clinical remission on GCs. In groups A, B, and C, 12.5%, 68.6%, and 65.6% of patients, respectively, remained in remission for >1 year. Conclusion: This study assessed remission of pediatric-onset SLE. Up to 60.2% of patients had clinical remission after treatment, and 19% of patients achieved remission off GCs. Long-term remission is rarer in pediatric-onset SLE than in adult-onset SLE.

Automatic Screening of Pediatric Renal Ultrasound Abnormalities: Deep Learning and Transfer Learning Approach.

BACKGROUND: In recent years, the progress and generalization surrounding portable ultrasonic probes has made ultrasound (US) a useful tool for physicians when making a diagnosis. With the advent of machine learning and deep learning, the development of a computer-aided diagnostic system for screening renal US abnormalities can assist general practitioners in the early detection of pediatric kidney diseases. OBJECTIVE: In this paper, we sought to evaluate the diagnostic performance of deep learning techniques to classify kidney images as normal and abnormal. METHODS: We chose 330 normal and 1269 abnormal pediatric renal US images for establishing a model for artificial intelligence. The abnormal images involved stones, cysts, hyperechogenicity, space-occupying lesions, and hydronephrosis. We performed preprocessing of the original images for subsequent deep learning. We redefined the final connecting layers for classification of the extracted features as abnormal or normal from the ResNet-50 pretrained model. The performances of the model were tested by a validation data set using area under the receiver operating characteristic curve, accuracy, specificity, and sensitivity. RESULTS: The deep learning model, 94 MB parameters in size, based on ResNet-50, was built for classifying normal and abnormal images. The accuracy, (%)/area under curve, of the validated images of stone, cyst, hyperechogenicity, space-occupying lesions, and hydronephrosis were 93.2/0.973, 91.6/0.940, 89.9/0.940, 91.3/0.934, and 94.1/0.996, respectively. The accuracy of normal image classification in the validation data set was 90.1%. Overall accuracy of (%)/area under curve was 92.9/0.959.. CONCLUSIONS: We established a useful, computer-aided model for automatic classification of pediatric renal US images in terms of normal and abnormal categories.

The correlation between VitD3 levels and the disease activity of childhood-onset systemic lupus erythematosus.

BACKGROUND: There is growing evidence linking low levels of vitamin D3 to an increased risk of many autoimmune diseases. Compared to the general population, hypovitaminosis D is more prevalent among children with systemic lupus erythematosus (SLE), which can be associated with sun exposure avoidance, long-term corticosteroid treatment, and renal disease. Therefore, we launched this study to assess the correlation between 25 (OH) D3 (VitD3) levels and the disease activity of children with SLE (cSLE) in Taiwan. METHODS: From September to December 2018, we recruited 31 cSLE patients from the Pediatric Out-patient Department of Taichung Veterans General Hospital. Their basic data, including SLE disease index 2000 (SLEDAI-2K) score, laboratory values, prescribed drugs and VitD3 levels were collected and analyzed statistically. RESULTS: The mean serum VitD3 concentration was 19.7 ± 7.9 ng/mL and SLEDAI-2K 6.2 ± 5.0. Those patients (N = 16) with an SLEDAI-2K≦4 had higher VitD3 levels when compared to those (N = 15) with an SLEDAI-2K>4 (22.9 ± 7.7 vs 16.3 ± 6.7 points, p = 0.020). Five patients not taking systemic corticosteroids (SCS) had significantly higher VitD3 levels and lower SLEDAI-2K than those who took SCS (N = 26). Additionally, we found VitD3 levels to be negatively correlated to SLEDAI-2K (rs = -0.55, p = 0.001) and daily SCS dosages (rs = -0.49, p = 0.005). CONCLUSION: This study shows that VitD3 deficiency is common in patients with cSLE. It was also noted that serum VitD3 levels negatively correlate to SLEDAI-2K, which can be partially explained by less usage of SCS.

Frasier Syndrome: A Rare Cause of Refractory Steroid-Resistant Nephrotic Syndrome.

Frasier syndrome is a rare disease that affects the kidneys and genitalia. Patients who have Frasier syndrome develop nephrotic syndrome (NS) featuring focal segmental glomerulosclerosis (FSGS) that is resistant to steroid treatment in early childhood. Male patients can have female external genitalia (pseudo-hermaphroditism) at birth and develop gonado-blastoma in their adolescence. Frasier syndrome is caused by mutations in the splice donor site at intron 9 of the Wilms' tumor WT1 gene; these mutations result in an imbalanced ratio of WT1 protein isoforms and affect the development of the urogenital tract, podocyte function, and tumor suppression. Here, we report on a patient with long-term refractory NS who developed a malignant mixed germ cell tumor arising in a gonado-blastoma of the ovary 8 years after the onset of proteinuria.

Allergic children with extremely high total IgE but no allergen identified in the initial screening panel.

BACKGROUND: High serum IgE level in atopic children usually implies a highly sensitized condition. However, there is a subgroup of atopic children for whom a specific allergen cannot be identified. In this study, we analyzed follow-up data from these children. METHODS: From March 2014 to July 2017, we recruited 14 atopic children with serum total IgE level higher than 500 Ku/L, but with no specific allergen identified by repeated MAST tests initially. Follow-up studies of specific IgE were conducted by the OPTIGEN MAST Allergy test and ImmunoCAP assays (Thermo Fisher Scientific/Phadia), while total IgE and specific IgG were measured by ImmunoCAP. RESULTS: The patients were aged from 2 to 17 y/o. The follow-up MAST tests showed significantly positive results in 10 patients. There were no significant differences in any of the clinical characteristics between the MAST-positive and MAST-negative groups. In the MAST-negative group, five allergen-specific IgE antibodies, including those for cockroach, Euroglyphus maynei, Blomia tropicalis, shrimp, and crab, were strongly predictive of negative ImmunoCAP results, according to ROC (Receiver operating characteristic curve) analysis of the AUC (Area under the Curve of ROC) (0.70-0.95), with significance set at p < 0.05. CONCLUSION: In two thirds of atopic children with a high serum IgE whose specific allergen had yet to be identified, it was possible to identify the specific MAST allergen(s) after an average follow-up of 33.2 months. For patients who still had negative results in follow-up MAST, mite DP, DF, and DM may be suitable choices for further allergen identification by ImmunoCAP.

Systemic lupus erythematosus and thyroid disease - Experience in a single medical center in Taiwan.

BACKGROUND: To investigate the association of systemic lupus erythematosus (SLE) with thyroid diseases in a medical center in central Taiwan. METHODS: This is a retrospective cohort of 2796 SLE patients in a tertiary referral medical center from 2000 to 2013. We screened SLE by catastrophic illness registration from national insurance bureau; and thyroid diseases by ICD 9 codes, then confirmed by thyroid function test, auto-antibody, medical and/or surgical intervention. We compared the rate of hyperthyroidism, hypothyroidism and autoimmune thyroid disease (AITD) in SLE patients and the 11,184 match controls. We calculated the rate of these thyroid diseases and positive antibodies to thyroglobulin (ATGAb), thyroid peroxidase (TPOAb) in SLE patients grouped by the presence of overlap syndrome and anti-dsDNA antibody. We also compared the association of thyroid diseases to severe SLE conditions, including renal, central nervous system (CNS) involvement, and thrombocytopenia. RESULTS: Compared to the matched controls, the cumulative incidence of thyroid disease, including hyperthyroidism, hypothyroidism and AITD, were all higher in SLE patients (p < 0.0001). The average age of SLE patients with thyroid diseases patients were older than those without thyroid diseases (p = 0.002). Those had euthyroid AITD were younger than other patients with thyroid diseases (p = 0.02). Up to 30.3% SLE patients had overlap syndrome and had higher relative risk of thyroid diseases than those without overlap syndrome, in terms of hypothyroidism and AITD, but not hyperthyroidism. SLE patients with thyroid diseases also carry higher risk for severe complications such as renal involvement (p = 0.024) central nervous system involvement (p < 0.0001). CONCLUSION: SLE patients had significantly higher rate of hyperthyroidism, hypothyroidism, and AITD than the matched control. Among lupus patients, the risks of thyroid diseases are even higher in the presence of overlap syndrome. SLE patients with thyroid diseases had higher risk of renal and CNS involvement.

Low-molecular-weight heparin and unfractionated heparin decrease Th-1, 2, and 17 expressions.

BACKGROUND: We evaluated the effects of T helper cell differentiation in a mite-allergic animal model treated with inhaled heparins of different molecular weight. METHOD: BALB/c mice were divided into four groups: 1. Control, 2. Mite intratracheal (mIT), 3. Inhaled heparin (hIN), 4. Inhaled low-molecular-weight heparin (lmwhIN). Groups 2, 3, and 4 were sensitized twice with Der p allergen subcutaneously on day 1 and day 8. Der p allergen was administered intratracheally on day 15. Groups 3 and 4 were treated with heparin or low-molecular-weight (lmw) heparin intranasally from day 1 to 22. Splenocytes from sacrificed mice stimulated with 16 µg/ml of Der p were cultured for 72 hours. Supernatants of splenocyte were collected to analyze the effect of Interleukin (IL)17-A/F, Interferon(IFN)-γ, IL-4, IL-13, and IL-10. Serum was also collected for Der P-specific IgE level on day 23. Total RNA was extracted from spleen tissue for mRNA expression. Gene expression of Foxp3, IL-10 IFN-γ, GATA3, IL-5, and RORγt were analyzed. RESULTS: Both hIN and lmwhIN groups had lower serum IgE level than that of the mIT group (both p<0.0001). Both hIN and lmwhIN groups showed significantly decreased transcripts of GATA-3, IFN-γ, IL-5, and RORγt mRNA in their spleen. Regarding the supernatant of splenocyte culture stimulated with Der p, compared with the mIT group, there were significant decreases in IL-17A/F, IFN-γ, IL-4, IL-13, and IL-10 secretion in inhaled hIN and lmwhIN groups. CONCLUSIONS: From this balb/c mice study, the analyses of mRNA and cytokines revealed that both intranasal heparin and lmw heparin treatment decreased the expression of Th1, Th2, and Th17 in spleen. The underlying mechanism(s) warrant further studies.

Asthma exacerbation in children: a practical review.

Asthma is the most common chronic lower respiratory tract disease in childhood throughout the world. Despite advances in asthma management, acute exacerbations continue to be a major problem in patients and they result in a considerable burden on direct/indirect health care providers. A severe exacerbation occurring within 1 year is an independent risk factor. Respiratory tract viruses have emerged as the most frequent triggers of exacerbations in children. It is becoming increasingly clear that interactions may exist between viruses and other triggers, increasing the likelihood of an exacerbation. In this study, we provide an overview of current knowledge about asthma exacerbations, including its definition, impact on health care providers, and associated factors. Prevention management in intermittent asthma as well as intermittent wheeze in pre-school children and those with persistent asthma are discussed. Our review findings support the importance of controlling persistent asthma, as indicated in current guidelines. In addition, we found that early episodic intervention appeared to be crucial in preventing severe attacks and future exacerbations. Besides the use of medication, timely education after an exacerbation along with a comprehensive plan in follow up is also vitally important.

Sodium sulfite aggravated allergic sensitization and airway inflammation in mite allergen sensitized BALB/c mice.

Sulfur dioxide is a typical air pollutant. Sulfite, which is formed at the bronchial mucosa from inhaled sulfur dioxide, might play a role in the exacerbation of asthma. In this study, we investigated the effects of sodium sulfite and its interaction with a house dust mite (Dermatophagoides pteronyssinus, Der p) on allergic sensitization and airway inflammation. BALB/c mice were divided into four groups: control (n = 10), mite intranasal (mIN, n = 12), sodium sulfite intranasal (sIN, n = 12) and mIN + sIN (n = 12). In non-control groups, the mice were sensitized on day 8 and day 15 with mite allergen subcutaneously. Mite allergen was then administrated intranasally from day 15 to day 22 in mIN and mIN+sIN groups. Sodium sulfite was administrated in sIN and mIN + sIN groups intranasally from day 1 to day 22. Plasma Der p-specific IgE, IgG2a, lung histopathology and cytokine levels (IL-5 and IFN-γ) were analyzed. In comparison between mIN (or sIN) and mIN + sIN group, Der p-specific IgE levels were significantly higher in mIN + sIN group (p < 0.01). Besides, Der p-specific IgG2a level was significantly lower in mIN + sIN group than mIN (or sIN) group (p < 0.01). The peribronchiolar, alveolar and total inflammatory scores were increased in the mIN + sIN group comparing with the control group (p < 0.05, p < 0.01, p < 0.01, respectively). Lung supernatant in mIN + sIN group has higher IL-5/IFN-γ ratio than control, mIN or sIN group (all p < 0.05). Our study concluded sodium sulfite may enhance allergic sensitization as well as airway inflammation in mite allergen sensitized BALB/c mice.

Prevalence of attention deficit/hyperactivity disorder in pediatric allergic rhinitis: a nationwide population-based study.

Allergic rhinitis (AR) is the most common chronic condition in pediatric populations. Characteristic symptoms in AR may bother daily activities and disturb sleep, leading to daytime inattention, irritability, and hyperactivity, which are also components of attention deficit/hyperactivity disorder (ADHD). Conflicting data exist in the literature regarding the relationship between ADHD and AR. The aim of this nationwide population-based study was to examine the prevalence and risk of ADHD among AR patients in a pediatric group. Data from a total of 226,550 pediatric patients <18 years old were collected from Taiwan's National Health Insurance Research Database from January 1 to December 31, 2005 and analyzed. We calculated the prevalence of allergic diseases based on various demographic variables, as well as in ADHD patients. We also used multivariable logistic regression to analyze the risk factors of ADHD. In 2005, the period prevalence rates of atopy and ADHD in patients <18 years of age were 15.35 and 0.6%, respectively. Pediatric patients with AR had a substantially increased rate of ADHD (p < 0.001) in terms of period prevalence and odds ratio. This significance existed across various demographic groups regardless of age, gender, area, or degree of urbanization. Neither comorbidity of atopic dermatitis nor bronchial asthma carried high risk for ADHD in AR patients. The present study revealed an increased rate of ADHD among AR patients. Therefore, evaluation of ADHD is advised for treatment of AR children.