The Turkish journal of pediatrics最新文献

Background: Intensive multimodal treatment can improve survival in patients with high-risk neuroblastoma, and consolidative radiation therapy has contributed to local control. We examined the clinical outcomes of patients who underwent consolidative radiation therapy at our institution.

Methods: We retrospectively reviewed the records of patients with high-risk neuroblastoma who underwent consolidative radiation therapy from March 2001 to March 2021 at Asan Medical Center. Patients underwent multimodal treatment including high-dose chemotherapy, surgery, stem cell transplantation, and maintenance therapy. Radiation (median, 21.0 Gy; range, 14-36) was administered to the primary site and surrounding lymph nodes.

Results: This study included 37 patients, and the median age at diagnosis was 2.8 years (range, 1.3-10.0). Four patients exhibited local failure, and 5-year free-from locoregional failure rate was 88.7%, with a median followup period of 5.7 years. The 5-year disease-free survival (DFS) and overall survival (OS) rates were 59.1% and 83.6%, respectively. Univariate analysis revealed that patients with neuron-specific enolase levels > 100 ng/mL had significantly worse DFS and OS (P = 0.036, 0.048), and patients with no residual disease before radiation therapy showed superior OS (P = 0.029). Furthermore, patients with 11q deletion or 17q gain exhibited poor DFS and OS, respectively (P = 0.021, 0.011). Six patients experienced grade 1 acute toxicity. Late toxicity was confirmed in children with long-term survival, predominantly hypothyroidism and hypogonadism, typically < grade 3, possibly attributed to combination treatment. Four patients experienced late toxicity ≥ grade 3 with chronic kidney disease, growth hormone abnormality, ileus, premature epiphyseal closure, and secondary tumor, and recovered by hospitalization or surgical treatment.

Conclusions: In patients with high-risk neuroblastoma, consolidative radiotherapy to the primary tumor site resulted in excellent local control and a tolerable safety profile.

Background: We assessed the relationship between sepsis occurrence and the serum levels of angiopoietin (Ang-1, Ang-2), vascular endothelial growth factor (VEGF) and soluble fms-like tyrosine kinase-1 (sFlt-1) in pediatric patients with cancer-related febrile neutropenia.

Methods: Fifty-two children with malignant tumors who experienced 86 episodes of febrile neutropenia (FN) were examined between June 2016 and June 2018. Each FN episode was considered a separate event and the total number of FNs were recorded (86 FN episodes = FN group). The control group consisted of 21 healthy children. Ang-1, Ang-2, VEGF-A and sFlt-1 were measured at the baseline and 48th hour of each FN episode -alongside routine characterization of inflammation (C-reactive protein; white blood cell and absolute neutrophil count).

Results: Among the episodes, 29 (34.5%) developed sepsis while 57 were classified as non-complicated FN. The baseline values of patients and controls were significantly different for Ang-1, Ang-2, VEGF and sFlt-1 values (all, p < 0.05). In the subgroup with sepsis, Ang-2 values were higher than in the subgroup without sepsis (p = 0.017). In predicting sepsis, Ang-2 had 60.7% sensitivity and 66.7% specificity at the 74.6 cut-off value (AUC: 0.662 [95%CI: 0.541 - 0.783], p = 0.022), Ang-2 / Ang-1 ratio had 65.5% sensitivity and 60.0% specificity at the 0.405 cut-off value (AUC: 0.633 [95%CI: 0.513 - 0.753], p = 0.046).

Conclusions: Our results reveal that Ang-2 and Ang-2/Ang-1 were higher in the sepsis group and Ang-2 might be a biomarker to indicate the risk of sepsis in patients with FN and/or cancer.

Background: When mother`s own milk (MOM) is unavailable or insufficient, donor milk (DM) from a human milk bank serves as an alternative feeding option. Our study sought to investigate and compare the outcomes and complications of very low birth weight (VLBW) preterm infants who receive MOM versus DM.

Methods: In this retrospective cohort study conducted between 2018 and 2022, we compared 70 VLBW preterm infants exclusively fed with DM to 70 randomly selected counterparts fed with MOM. Both groups began enteral feeding within 72 hours of birth. Various clinical outcomes were investigated during a three-month follow-up. The clinical outcomes were compared via independent t-tests, Mann-Whitney U, and Fisher`s exact test.

Results: The mean gestational age of the infants who were included was 29.6 ± 1.6 weeks, 84 (60%) were males, and the average birth weight was 1217 ± 151 grams. Both groups had similar baseline characteristics. The results of the study demonstrated no statistically significant differences between the groups in terms of hospital length of stay (37±16.3 days in MOM vs 40.3±16.9 days in DM group, P= 0.17), growth rate (13±4 gram/day in MOM vs 13±4 gram/day in DM group, P=0.51), growth velocity (9.8±3.0g/kg/d in MOM vs 9.5±3.2 g/kg/d in DM group), infants with in-hospital vomiting (51 cases in MOM vs 59 cases in DM group, P=0.15),vomiting frequency (1.3±1.1 times in MOM vs 1.5±1.0 times in DM group), incidence of retinopathy of prematurity (ROP) (4 cases in MOM vs 5 cases in DM group, P > 0.999) and incidence of bronchopulmonary dysplasia (BPD) (7 cases in MOM vs 6 cases in DM group, P > 0.999).

Conclusion: Our study findings indicate that the utilization of DM didn`t have a substantial negative impact on infants` outcomes nor any complications in comparison with MOM.

Background: At the onset of the pandemic, we conducted a study on adolescents with eating disorders (EDs) and found no deterioration in ED symptoms. The objective of this subsequent study was to conduct a follow-up evaluation of the same cohort and investigate the consequences of the prolonged pandemic.

Methods: This longitudinal study was conducted one year after the first study between May 2021 and June 2021 with 37 adolescents aged 12-18 years (pre-existing EDs). The reassessment included an evaluation of sociodemographic and clinical characteristics, the impact of pandemic-related restrictions on ED behaviors, well-being, and quality of life. All the participants underwent a re-administration of the ED examination questionnaire (EDE-Q), Beck Depression Inventory, the State Anxiety Inventory for Children, and the Maudsley Obsessive Compulsive Inventory.

Results: No significant difference was observed in the EDE-Q scores or the ED examination questionnaire scores between the initial (T1) and subsequent (T2) study. The ED-related quality of life was seen to have slightly improved in the later stage. While depression (T1: 18, T2: 15, p=0.883) and obsession scores (T1: 11, T2: 14, p: 0.536) showed no disparity between the studies, anxiety scores (T1: 38, T2: 43, p:0.011) exhibited a significant increase.

Conclusions: Consistent with the early phase, no exacerbation of ED symptoms in adolescents was observed during the later stages of the pandemic. Close clinical monitoring during the pandemic might have been protective against the deteriorating effects of the pandemic. During social isolation, it is important to monitor adolescents with EDs continously for depression and anxiety.

Background: Parents of a child with neurological problems such as seizures and epilepsy experience significant mental distress. Little is known about the mental state of parents in such a stressful situation. This study aims to determine the prevalence of self-reported depression, anxiety, sleep disorders, and quality of life in parents of children with epilepsy and first unprovoked seizure.

Methods: This cross-sectional study was conducted among the parents of children diagnosed with first unprovoked seizure and epilepsy admitted to the Pediatric Neurology Department, Outpatient Unit of İnönü University Medical Faculty Hospital. Participants filled out a questionnaire investigating demographic variables, Beck Anxiety Inventory (BAI), Beck Depression Inventory (BDI), Pittsburgh Sleep Quality Index (PSQI), and 36- Item Short-Form Health Survey (SF-36).

Results: 113 parents participated in the study. Depression was found in 7%, anxiety in 14%, and sleep quality disorder in 33.3% of parents of children diagnosed with epilepsy on the basis of moderate or higher severity, while depression was found in 8.9%, anxiety in 14.3%, and sleep disorder in 21.4% of parents of children diagnosed with first unprovoked seizure. There was no statistically significant difference between the groups. Mothers were at higher risk for loss of physical function and social functionality. There was a positive correlation between BAI, BDI, and PSQI scores. Quality of life sub-dimension measured by SF-36 was associated with different levels of depression, anxiety, and sleep quality.

Conclusion: Addressing parental psychiatric problems by professionals involved in the treatment of children with a history of seizures may have the potential to provide further support for the family and the care of patients.

Background: This study aimed to examine early clinical and laboratory findings in infants born to mothers who had organ transplants and received immunosuppressive treatment.

Methods: Between 2016 and 2023, the study examined infants of mothers who underwent organ transplantation and were receiving immunosuppressive treatment, and followed at the Department of Neonatology at Akdeniz University. Demographic, clinical, and laboratory characteristics of mothers and infants were recorded. On the first day of life, complete blood count values were examined, as well as potassium levels on the first, third, and seventh days, and creatinine levels on the third and seventh days. The tacrolimus blood level was calculated by taking the average of the tacrolimus blood values of the mother measured during the pregnancy. The infants were evaluated for any potential morbidities caused by intrauterine immunosuppressive drug exposure.

Results: The study included 21 mothers (some with multiple pregnancies) and 27 infants. According to the findings of this study, 74% of these infants were born premature, 67% had low birth weight, and all were delivered via cesarean section. Prematurity was associated with the morbidities found in the infants. In the early period, lymphopenia was detected in 37%, neutropenia in 25.9%, thrombocytopenia in 11.1%, hyperkalemia in 18.5%, and creatinine elevation in 7.4%, all of which returned to normal within a few days. There was no significant relationship between maternal tacrolimus blood levels and infant potassium and creatinine levels.

Conclusion: Apart from an increased risk of prematurity, low birth weight, and cesarean delivery, no effects were observed in these infants during the early period. However, long-term follow-up is necessary to monitor for any potential morbidities.

Background: Overdose with calcium-channel blockers (CCBs) still maintain their importance with a high lethality rate after exposure. We report the intravenous lipid emulsion therapy (ILE) therapy in our CCB overdose patients.

Methods: We retrospectively analyzed the records of 6 patients with CCB intoxication from Batman Training and Research Hospital PICU between March 2021 and September 2022. Patients aged 0-18 years who received ILE treatment for CCB poisoning were included.

Results: All six patients ingested CCB with the intention of committing suicide and were followed up in the pediatric intensive care unit (PICU). All patients received ILE therapy due to hemodynamic instability despite intravenous fluid boluses, calcium, glucagon, insulin-dextrose, and vasoactive agents. Vasoactive-Inotropic Score (VIS) decreased after ILE treatment. All patients were transferred from the PICU after recovery.

Conclusions: ILE therapy should be kept in mind as a salvage therapy in hemodynamically unstable CCB poisoning cases that do not respond to initial and advanced options.

Background: Campotodactyly-artrhropathy-coxa vara-pericarditis (CACP) syndrome is a very rare autosomal recessive genetic disorder. It is characterized by flexion contracture of the fifth finger (camptodactyly); noninflammatory arthropathy; decreased angle between the shaft and the head of the femur (coxa vara) and pericarditis. Its association with mitral stenosis has not yet been reported. Hereby we report this unique association with CACP syndrome.

Case: An eleven-year-old girl presented with non-productive cough, dyspnea, and orthopnea. She was diagnosed CACP syndrome at the age of seven and a biallelic frameshift mutation in the PRG4 gene was determined. The physical examination revealed pectus excavatum, camptodactyly, genu valgum, tachypnea and orthopnea. The functional capacity was NYHA III-IV. She had 2/6 soft pansystolic murmur at 4th left intercostal space and a rumbling diastolic murmur at apex. Echocardiography revealed an enlarged left atrium, severe stenotic mitral valve with a mean diastolic transmitral gradient of 22.5 mmHg, mild mitral regurgitation and mild apical pericardial effusion. The patient had mitral comissurotomy and partial pericardiectomy operation. Her post-operative transmitral gradient decreased to 6.9 mmHg and the pulmonary pressure was 30 mmHg. Her functional capacity increased to NYHA I-II.

Conclusions: The main defect is the proteoglycan 4 protein which acts like a lubricant in articular and visceral surfaces. Therefore, the leading clinical feature is arthropathy. Cardiac involvement other than clinically mild pericarditis is not usually expected. Three types of proteoglycans (decorin, biglycan, and versican) are present in the mitral valve. This could be the reason of mitral valve involvement in rare cases as like ours. It is important that these patients undergo echocardiographic examination regularly.

Background: Nomophobia (NoMP) is the fear of being unable to interact with others via mobile phones and is a current topic in adolescents` mental health. The purpose of this study was to investigate the association between NoMP in adolescents and their mothers` level of NoMP.

Methods: The levels of depression, anxiety, attention deficit/hyperactivity-impulsivity symptoms, NoMP, and perceived parental emotional availability were examined.

Results: One hundred fifty-five adolescents (60% girls) were included in the study with their mothers. Ninetythree (60%) adolescents (67.7% girls) and 64 (41.3%) mothers were classified as nomophobic. There was a positive correlation between the NoMP levels of adolescents and their mothers. Nomophobic girls perceived less paternal support. All psychopathologic symptoms were higher in the nomophobic mothers. There was no difference between nomophobic and non-nomophobic adolescents in terms of maternal psychopathologies.

Conclusions: We suggest that parental effects should be investigated during the assessment of NoMP, especially in adolescent girls. The phone usage habits of mothers and their relationship with their adolescent children were closely associated with adolescent NoMP.