Turkish Journal of Pediatrics最新文献

Background: Heavy menstrual bleeding (HMB) is a common condition in adolescents. However, bleeding disorders are known to be one of the causes of HMB in adolescent girls, so they should be considered. Simple methods that can be used in primary health care are needed to determine whether patients have bleeding disorders. The aim of this study was to evaluate the bleeding score of patients admitted with HMB and to determine the diagnostic value of patients who were symptomatic but whose initial hemostatic tests were normal.

Methods: A total of 113 adolescents with HMB and 20 healthy adolescent girls were included in the study. The Pediatric Bleeding Questionnaire (PBQ) and the International Society of Thrombosis Haemostasis-Bleeding Assessment Tool (ISTH-BAT) were used for evaluation.

Results: Overall, approximately 18% (n= 20) of the adolescents in the study were diagnosed with a bleeding disorder. The cut off value for the `clinically significant bleeding score` was found to be 3.5.

Conclusions: The PBQ and ISTH-BAT can help distinguish a significant bleeding history from an otherwise trivial bleeding and can be included in the algorithm for the primary care of adolescents with HMB with suspected bleeding disorders.

Background: Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is caused by dysfunctional C1-INH protein due to mutations in the SERPING1 gene encoding C1-INH. Marfan syndrome is a genetic connective tissue disease that affects the cardiovascular and ocular systems along with the skeletal system. In this case, we present the successful treatment of post-pericardiotomy syndrome unresponsive to classical therapy, which has not been described in the literature. The syndrome developed in a patient with hereditary angioedema (HAE) who underwent open heart surgery due to cardiac involvement in Marfan syndrome.

Case: A nine-year-old male HAE-C1INH patient underwent open heart surgery secondary to cardiac involvement caused by Marfan syndrome. To prevent HAE attacks, 1000 units of C1 inhibitor concentrate therapy were given 2 hours before and 24 hours after the operation. Post-pericardiotomy syndrome was diagnosed on the postoperative second day and ibuprofen 15 mg/kg/day (3 weeks) was started. Since there was no response to classical treatment on the 21st postoperative day, C1 inhibitor concentrate treatment was planned as 1000 units/ dose for 2 days a week considering a prolonged hereditary angioedema attack. In the second week of treatment, complete recovery was achieved for pericardial effusion with a total of 4 doses.

Conclusions: We emphasize that in patients with hereditary angioedema undergoing this treatment, care should be taken in terms of complications that may be associated with the disease even if short-term prophylaxis is given before operations and that longer-term use of C1 inhibitor concentrate has a place in treatment.

Background: The incidence of vaccine hesitancy is increasing in many countries. This study aims to determine parents` attitudes and related factors regarding COVID-19 vaccine acceptance for themselves and their children aged 12-18.

Methods: A cross-sectional survey was conducted on parents between 16th November and 31st December 2021, after COVID-19 vaccines were initiated for children in Türkiye. In the survey, the sociodemographic characteristics of the parents, whether they and their children were vaccinated against COVID-19, and if not, the reasons for this were asked. Multivariate binary logistic regression analysis was used to evaluate the factors affecting parents` refusal to vaccinate their children for COVID-19.

Results: Three hundred and ninety-six mothers and fathers were included in the final analysis. Overall, 41.7% of parents reported vaccine refusal for their children. COVID-19 vaccine refusal was higher in mothers younger than 35 (β = 6.5, p = 0.002, 95% CI: 2.0-23.1), children aged 15 and younger (β = 2.3, p = 0.001, 95% CI: 1.4-3.7). Concerns about the side effects of the COVID-19 vaccine (29.7%) and their children not wanting to be vaccinated (29.0%) were the most common causes of COVID-19 vaccine refusal.

Conclusions: In the present study, the rate of children not vaccinated due to COVID-19 vaccine refusal was relatively high. Parents` concerns about vaccine side effects, as well as their children`s unwillingness to be vaccinated, suggest that both parents and adolescents should be informed about the importance of COVID-19 vaccines.

Background: The purpose of this study was to investigate the validity and reliability of the Turkish versions of the Measure of Processes of Care, MPOC-56 and MPOC-20, in children with disability aged 5-17 years.

Methods: A total of 290 parents of children with disability due to various disorders were evaluated with the MPOC-56 and MPOC-20. Internal consistency was determined with Cronbach`s alpha, and test-retest reliability with the intraclass correlation coefficient (ICC). Confirmatory factor analysis was performed to investigate the factor structure of the Turkish MPOC-56 and -20.

Results: Cronbach`s alpha values for the MPOC-56 and MPOC-20 ranged between 0.84-0.97 and 0.87-0.92, respectively. Test-retest ICC values were 0.96-0.99 for MPOC-56 and 0.94-0.98 for MPOC-20. The correlations of the subscale scores of MPOC- 56 and MPOC-20 were shown to be at very good to excellent levels for reliability. Factor structure for MPOC-20 and MPOC-56 were found to be acceptable.

Conclusions: This study has shown that the Turkish versions of MPOC-56 and MPOC-20 are valid, reliable, and applicable for the evaluation of parents` experiences of processes of care for children with disability aged 5-17 years.

Background: Rapid changes in the size of the pituitary gland occur during the pubertal period. Therefore, measuring and reporting magnetic resonance imaging (MRI) in adolescents with pituitary disorders can cause unease among radiologists. Our aim was to compare the size of the pituitary gland, stalk and other previously described imaging tools in patients with isolated hypogonadotropic hypogonadism (HH) versus adolescents with a normal pituitary gland.

Methods: Forty-one patients (22 female, 19 male, mean age 16.3 ±2.0 years) with HH who underwent MRI prior to starting hormone treatment were enrolled. Age, sex, and genetic mutations were noted. Pituitary height, width on the coronal plane, anteroposterior (AP) diameter on the sagittal plane, stalk thickness, pons ratio (PR), clivus canal angle (CCA) and Klaus index (KI) were measured by two radiologists twice with a one-month interval blinded to each other and patient information. Measurements were compared with the control group, including 83 subjects with normal hypothalamic-pituitary-gonadal axis and normal pituitary gland on MRI. Inter-rater and intra-rater agreements were also evaluated.

Results: No significant differences were found between the two groups regarding height, width or AP diameter (p = 0.437, 0.836, 0.681 respectively). No significant differences were found between the two groups regarding CCA and PR (p = 0.890, 0.412 respectively). The KI of the male patients was significantly higher than that of the female patients and the control group (p < 0.001). The interrater agreement was moderate for pituitary height and width, poor for pituitary AP diameter and stalk thickness, good for PR and KI, and excellent for CCA.

Conclusions: The measurements of the pituitary gland, stalk and posterior fossa structures were similar in adolescents with or without isolated HH. Consequently, pituitary gland, stalk or other posterior fossa measurements are unnecessary when evaluating a normal appearing pituitary gland on MRI.

Background: School readiness (SR) has been adopted by the American Academy of Pediatrics (AAP) as a component of health supervision, but the medical community`s role is unknown. We evaluated the pediatricians` attitudes, practices, and perceived barriers to SR.

Methods: This multicenter, cross-sectional descriptive study was performed among 787 general pediatricians, pediatric residents, subspecialists, and subspecialty fellows. A 41-item survey was administered.

Results: Forty-nine point two percent of the pediatricians defined SR as a multidimensional issue, as outlined by the AAP, whereas 50.8% defined it as the child`s set of skills or passing the SR tests. Three-quarters of pediatricians believed that SR assessment tests are necessary before starting school, and children who do not appear ready should wait a year. To promote SR, the rates of usually fostering at least four of the five `Rs` (reading, rhyming, routines, rewarding, relationships) and integrating developmental surveillance into daily practice were 37.8% and 23.8%, respectively. Only 2.2% of pediatricians usually inquired about eight adverse childhood experiences (ACEs), and 68.9% did not usually ask about any. Usually fostering at least four of the five `Rs` was associated with usually integrating developmental surveillance (p < 0.001), usually inquiring about each ACE (p < 0.001), and being perceived as responsible for promoting SR (p < 0.01). Training on SR during pediatric residency was 2.7%. Time constraints and insufficient knowledge were the most common barriers.

Conclusions: Pediatricians were not familiar with the concept of SR and had some misconceptions. There is a need for additional training regarding pediatricians` roles in promoting SR along with addressing multiple, modifiable barriers within the health system. < strong > Supplementary: < a href="https://www.turkishjournalpediatrics.org/uploads/2573-supplementary.pdf" target=`_blank` > Supplementary Appendix < /a > < /strong >.

Background: While macrovascular thrombosis is common in adult COVID-19 patients, thrombotic microangiopathy as a part of endothelitis might play an important role in severe organ dysfunction. Thrombocytopenia-associated multiple organ failure (TAMOF) is a thrombotic microangiopathy syndrome that is associated with endothelial damage. Herein, we aim to report a pediatric TAMOF case related to SARS-CoV-2 infection which has been scarcely reported to date.

Case: A 7-month-old boy who became severely ill after being infected with SARS-CoV-2 required advanced critical care treatments such as continuous renal replacement therapy, therapeutic plasma exchange, and extracorporeal membrane oxygenation. A heart and lung biopsy obtained during sternotomy showed thrombotic microangiopathy. Despite early plasma exchange, mortality was inevitable because of severe liver failure.

Conclusions: This case report implies that SARS-CoV-2 infection could cause TAMOF in children. To the best of our knowledge, this is the second SARS-CoV-2-induced pediatric TAMOF case. More studies are needed to determine alternative treatments for patients with TAMOF who are resistant to conventional therapies.

Background: Mexiletine, a class IB antiarrhythmic, is a structural analog of lidocaine. Our knowledge of mexiletine overdose is based on lidocaine overdose reports. Only a few cases of mexiletine overdose have been reported, including fatal overdoses. Mexiletine toxicity primarily affects the central nervous, cardiovascular, and gastrointestinal systems.

Case: A 16-year-old female was brought to our hospital by ambulance after taking an unknown dose of mexiletine in a suicide attempt. Ventricular fibrillation developed while in the ambulance; cardiopulmonary resuscitation was started and spontaneous circulation returned within 1 min. The patient had been taking oral mexiletine for 1 month to treat primary erythromelalgia. Her vital signs were normal, but she was unconscious. Following gastric lavage she was transferred to the pediatric intensive care unit. Midazolam and levetiracetam were required due to uncontrolled seizures. During the first hour of hospitalization, severe dyskinesia characterized by abnormal involuntary large hyperkinetic movements in all 4 extremities was observed and successfully treated with 2 doses of intravenous biperiden. The patient was discharged on day 6 of hospitalization.

Conclusions: Mexiletine overdose can be life-threatening. In addition to rapid and effective resuscitation, rapid identification and management of cardiovascular and central nervous system manifestations are key to preventing morbidity and mortality. The presented case had severe dyskinesia that was successfully treated with repeated doses of biperiden. Biperiden did not cause arrhythmia. Based on the presented case, we think biperiden should be considered for the treatment of movement disorders in cases of mexiletine overdose.

Background: Purulent meningitis remains an important cause of mortality and morbidity among children worldwide. An immediate diagnosis of the causative microorganism is critical to significantly improving the outcome of this condition.

Case: In this study, we collected cerebrospinal fluid (CSF) samples from four patients clinically diagnosed with purulent meningitis. Patients with purulent meningitis may present with a variety of clinical symptoms or laboratory results. Infectious microorganisms including Pseudomonas aeruginosa, Staphylococcus aureus, Streptococcus pneumonia, and Haemophilus influenzae were identified in the CSF samples via metagenomic nextgeneration sequencing (mNGS).

Conclusions: mNGS is effective for the immediate detection of pathogens, which can in turn facilitate prompt diagnosis and treatment among individuals with purulent meningitis, especially if conventional CSF results (such as CSF culture and polymerase chain reaction) are negative.

Background: Optimum management of food allergy (FA) includes consideration of co-allergies and multimorbidities and tolerance assessment. Documentation of FA practices may pave the way for better practice.

Methods: Patients aged 3-18 years, with persistent IgE-mediated hen`s egg allergy were reviewed.

Results: A total of 102 children with a median age of 59 months (IQR= 40-84) (72.2% males) were included. All were diagnosed during infancy and the initial symptoms were atopic dermatitis (65.6%), urticaria (18.6%), and anaphylaxis (5.9%). Of the total population, 21 (20.6%) experienced anaphylaxis with hen`s eggs, and 79.4%, 89.2%, and 30.4% had multiple FAs (≥2 food categories), ever atopic dermatitis, and asthma, respectively. The most common co-allergies were tree nuts, cow`s milk, and seeds, respectively. From 52 heated egg yolk and 47 baked egg oral food challenges, 48 (92.3%) and 41 (87.2%) were found as tolerant, respectively. The baked egg nontolerant group had a greater egg white skin prick test diameter [9 mm (IQR: 6-11.5) vs. 6 mm (IQR: 4.5-9); (p=0.009)] and specific IgE [12.6 kU/L (IQR: 4.11-45.4) vs. 6.2 kU/L (IQR: 1.9-12.4) (p=0.009)], respectively. In the multivariate analysis, baked egg tolerance was more likely in those with egg yolk-tolerant subgroup (OR: 6.480, 95% CI: 2.524-16.638; p < 0.001) and heated egg tolerance in those with baked egg tolerance (OR: 6.943, 95% CI: 1.554-31.017; p=0.011).

Conclusions: Persistent hen`s egg allergy is characterized by multiple food allergies and age-related multimorbidities. Baked egg and heated egg yolk tolerance were more likely to be considered in a subgroup with a high expectation for finding a way to eliminate their allergy.