Search Results (8)

Objectives: This retrospective study evaluated the short-term effects of removable slow maxillary expansion (SME) on eruption patterns of labially ectopic canines in a Chinese pediatric population, comparing treated patients with untreated controls. Methods: Seventy-six patients (mean age 8.38 ± 0.88 years) underwent SME treatment for 11.04 ± 4.44 months. Canine positions were categorized as labial ectopic (TE: n = 40) or normally positioned (TN: n = 112). The TE group was stratified vertically: superior (TES; n = 15, canines above lateral incisors’ roots or adjacent to unerupted incisors) and inferior (TEI; n = 25, canines adjacent to erupted lateral incisors’ roots). Untreated controls (n = 58; mean age 8.46 ± 0.78 years) included labial ectopic (CE group; n = 32) and normal canines (CN group; n = 84), with CE further divided vertically into CES (n = 24) and CEI (n = 8). Panoramic radiographs at baseline (T0) and follow-up (T1) evaluated sector distribution, midline proximity (3c-ML: canine cusp to midline distance), vertical position (3c-OP: cusp to occlusal plane distance), and angular (3^ML: canine-midline angle). Results: SME significantly improved midline proximity (3c-ML increased) while reducing vertical height (3c-OP decreased) and angulation (3^ML reduced) in the TE group. Notably, TE patients revealed a significantly greater increase in 3c-ML compared to CE. Subgroup analysis showed that TEI canines exhibited significant improvements in all three parameters (3c-OP, 3c-ML, and 3^ML), whereas TES canines displayed minimal changes. The shifts in sector distribution were similar between the treatment and control groups. Conclusions: SME demonstrated short-term efficacy in guiding labially ectopic canines toward more favorable eruption trajectories, particularly when erupted beyond the roots of the lateral incisor. The observed positional improvements underscore SME’s potential to optimize eruption outcomes during early orthodontic intervention. Full article

Background: Children generally face a higher incidence of airway management complications, intubation difficulties, and the risk of failed intubation. Currently, there is sufficient evidence in clinical practice for the use of videolaryngoscopes in pediatric airway management. However, there are a number of standard-blade videolaryngoscopes available for children. In addition, there is no clear recommendation on which videolaryngoscope is superior. The primary objective of this study is to compare the first pass success rate and the Percentage of Glottic Opening (POGO) scores with Cormack–Lehane (CML) scores obtained through direct and indirect laryngoscopy with HugeMed and McGrath Mac videolaryngoscopes in pediatric patients with an unanticipated, difficult airway. Materials and Methods: Following the Ethics Committee approval and written parental consents, a total of 40 elective surgical patients, aged 3 and under, with ASA 1–3 risk classification, and undergoing general anesthesia, were included in the study. After induction of general anesthesia, the first group of patients (Group McGrath, n = 20) was intubated with the McGrath Mac videolaryngoscope, and the second group (Group HugeMed, n = 20) with the HugeMed videolaryngoscope. Before intubation, CML and POGO scores were recorded for both groups using direct and indirect laryngoscopy with videolaryngoscopes. Intubation time, number of attempts, need for cricoid pressure, optimization maneuver requirement, and hemodynamic parameters were recorded for both groups. Results: There was no significant difference between groups in demographic data including age, gender, body mass index, ASA, and hemodynamic parameters. A significant improvement was observed in CML and POGO scores using indirect laryngoscopy (p < 0.001). CML scores obtained with the McGrath Mac were significantly lower than the HugeMed Group (p = 0.0034). The mean POGO value calculated with indirect laryngoscopy was significantly higher in the McGrath Group compared to the HugeMed Group (92.63 ± 6.09 vs. 88.75 ± 4.44, respectively). Conclusions: Videolaryngoscopes improved laryngeal visualization in children under 3 years old. Compared to HugeMed, in indirect laryngoscopy, the McGrath Mac videolaryngoscope was found to be superior, with better CML and POGO scores. However, number of tracheal intubation attempts, success rate, complication risk, and hemodynamic parameters did not show any significant difference between the groups. Clinical trial registration number was NCT06484517. Full article

Pediatric chronic myeloid leukemia (CML) is a very rare malignancy (age-related incidence 0.1/100,000) typically presenting with leucocyte counts >100,000/µL. However, clinical signs of leukostasis are observed at diagnosis in only approximately 10% of all cases and among these, priapism is infrequent. Here, we analyze data from pediatric CML registries on the occurrence of priapism heralding diagnosis of CML in 16/491 (3.2%) boys (median age 13.5 years, range 4–18) with pediatric CML. In the cohort investigated, duration of priapism resulting in a diagnosis of CML was not reported in 5 patients, and in the remaining 11 patients, occurred as stuttering priapism over 3 months (n = 1), over 6 weeks (n = 1), over 1–2 weeks (n = 2), over several days (n = 2), or 24 h (n = 1), while the remaining 4 boys reported continuous erection lasting over 11–12 h. All patients exhibited splenomegaly and massive leukocytosis (median WBC 470,000/µL, range 236,700–899,000). Interventions to treat priapism were unknown in 5 patients, and in the remaining cohort, comprised intravenous fluids ± heparin (n = 2), penile puncture (n = 5) ± injection of sympathomimetics (n = 4) ± intracavernous shunt operation (n = 1) paralleled by leukocyte-reductive measures. Management without penile puncture by leukapheresis or exchange transfusion was performed in 3 boys. In total, 7 out 15 (47%) long-term survivors (median age 20 years, range 19–25) responded to a questionnaire. All had maintained full erectile function; however, 5/7 had presented with stuttering priapism while in the remaining 2 patients priapism had lasted <12 h until intervention. At its extreme, low-flow priapism lasting for longer than 24 h may result in partial or total impotence by erectile dysfunction. This physical disability can exert a large psychological impact on patients’ lives. In a narrative review fashion, we analyzed the literature on priapism in boys with CML which is by categorization stuttering or persisting as mostly painful, ischemic (low-flow) priapism. Details on the pathophysiology are discussed on the background of the different blood rheology of hyperleukocytosis in acute and chronic leukemias. In addition to the data collected, instructive case vignettes demonstrate the diagnostic and treatment approaches and the outcome of boys presenting with priapism. An algorithm for management of priapism in a stepwise fashion is presented. All approaches must be performed in parallel with cytoreductive treatment of leukostasis in CML which comprises leukapheresis and exchange transfusions ± cytotoxic chemotherapy. Full article

Chronic myeloid leukemia (CML) in childhood represents only 3% of newly diagnosed pediatric leukemia. The diagnostic hallmark of CML is the Philadelphia (Ph) chromosome, which derives from the fusion of the ABL1-oncogene located on chromosome 9 to the breakpoint cluster region (BCR) gene on chromosome 22, resulting in a constitutively dysregulated ABL1 tyrosine kinase, either as 210 kDa or 190 kDa. Depending on the localization of the breakpoint site within the major BCR region, the majority of CML patients exhibit transcripts with either the b3a2 or b2a2 junction, or both. Several questions are still open with regard to childhood CML, especially concerning the biologic and clinical features of the disease, and the treatment of choice for pediatric patients with CML. Moreover, over the last few years, several tyrosine kinase inhibitors (TKIs) have been available for children and adolescents with CML, and current clinical practice investigates what the effective and optimal doses of TKIs are in these two categories of patients. The use of TKIs in pediatric patients with CML has also opened up questions on the following items: (1) the long-term effects of these drugs on children; (2) the management of pediatric CML forms resistant or intolerant to TKIs; (3) the monitoring of disease outcomes during treatment; (4) and the right timing to discontinue therapy. Despite the efficacy of TKIs also in the pediatric population, the potential late adverse effects, and the drug resistance, leave open the possibility of allogeneic hematopoietic stem cell transplantation as a treatment option in pediatric CML. Published data and personal experiences regarding these issues will be analyzed and discussed. Full article

Philadelphia chromosome-positive chronic myeloid leukemia (CML) is cytogenetically characterized by the classic translocation t(9;22)(q34;q11), whereas additional non-Philadelphia aberrations (nPhAs) have been studied extensively in adult patients with CML, knowledge on nPhAs in pediatric patients with CML is still sparse. Here, we have determined nPhAs in a cohort of 161 patients younger than 18 years diagnosed with chronic phase CML and consecutively enrolled in the German national CML-PAED-II registry. In 150 cases (93%), an informative cytogenetic analysis had been performed at diagnosis. In total, 21 individuals (13%) showed nPhAs. Of these, 12 (8%) had a variant translocation, 4 (3%) additional chromosomal aberrations (ACAs) and 5 (3%) harbored a complex karyotype. Chromosome 15 was recurrently involved in variant translocations. No significant impact of the cytogenetic subgroup on the time point of cytogenetic response was observed. Patients with a complex karyotype showed an inferior molecular response compared to patients carrying the classic translocation t(9;22)(q34;q11), variant translocations or ACAs. No significant differences in the probability of progression-free survival and overall survival was found between patients with nPhAs and patients with the classic Philadelphia translocation only. Our results highlight the distinct biology of pediatric CML and underline the need for joint international efforts to acquire more data on the disease pathogenesis in this age group. Full article

Children with chronic myeloid leukemia (CML) tend to present with higher white blood counts and larger spleens than adults with CML, suggesting that the biology of pediatric and adult CML may differ. To investigate whether pediatric and adult CML have unique molecular characteristics, we studied the transcriptomic signature of pediatric and adult CML CD34+ cells and healthy pediatric and adult CD34+ control cells. Using high-throughput RNA sequencing, we found 567 genes (207 up- and 360 downregulated) differentially expressed in pediatric CML CD34+ cells compared to pediatric healthy CD34+ cells. Directly comparing pediatric and adult CML CD34+ cells, 398 genes (258 up- and 140 downregulated), including many in the Rho pathway, were differentially expressed in pediatric CML CD34+ cells. Using RT-qPCR to verify differentially expressed genes, VAV2 and ARHGAP27 were significantly upregulated in adult CML CD34+ cells compared to pediatric CML CD34+ cells. NCF1, CYBB, and S100A8 were upregulated in adult CML CD34+ cells but not in pediatric CML CD34+ cells, compared to healthy controls. In contrast, DLC1 was significantly upregulated in pediatric CML CD34+ cells but not in adult CML CD34+ cells, compared to healthy controls. These results demonstrate unique molecular characteristics of pediatric CML, such as dysregulation of the Rho pathway, which may contribute to clinical differences between pediatric and adult patients. Full article

Depending on the analytical tool applied, the hallmarks of chronic myeloid leukemia (CML) are the Philadelphia Chromosome and the resulting mRNA fusion transcript BCR-ABL1. With an incidence of 1 per 1 million of children this malignancy is very rare in the first 20 years of life. This article aims to; (i) define the disease based on the WHO nomenclature, the appropriate ICD 11 code and to unify the terminology, (ii) delineate features of epidemiology, etiology, and pathophysiology that are shared, but also differing between adult and pediatric patients with CML, (iii) give a short summary on the diseases to be considered as a differential diagnosis of pediatric CML, (iv) to describe the morphological, histopathological and immunophenotypical findings of CML in pediatric patients, (v) illustrate rare but classical complications resulting from rheological problems observed at diagnosis, (vi) list essential and desirable diagnostic criteria, which hopefully in the future will help to unify the attempts when approaching this rare pediatric malignancy. Full article

The therapeutic approach to Chronic Myeloid Leukemia (CML) has changed since the advent of the tyrosine kinase inhibitor (TKI) imatinib, which was then followed by the second generation TKIs dasatinib, nilotinib, and, finally, by ponatinib, a third-generation drug. At present, these therapeutic options represent the first-line treatment for adults. Based on clinical experience, imatinb, dasatinib, and nilotinib have been approved for children even though the studies that were concerned with efficacy and safety toward pediatric patients are still awaiting more specific and high-quality data. In this scenario, it is of utmost importance to prospectively validate data extrapolated from adult studies to set a standard therapeutic management for pediatric CML by employing appropriate formulations on the basis of pediatric clinical trials, which allow a careful monitoring of TKI-induced adverse effects especially in growing children exposed to long-term therapy. Full article