The Turkish journal of pediatrics最新文献

Background: Takayasu arteritis (TA) is an uncommon chronic inflammatory and autoimmune disease primarily affecting large vessels, particularly the aorta and its branches. Skin manifestations have been documented in association with TA. Pyoderma gangrenosum (PG) is a chronic neutrophilic dermatosis characterized by destructive, necrotizing, and painful ulcers, predominantly found on the lower extremities. The coexistence of PG and TA is extremely rare, with most reported cases involving adult patients. Interestingly, the association between PG and TA appears to be more common in Japan compared to North American and European populations. Childhood TA (c-TA) accompanied by PG is exceptionally rare, with only 10 cases reported in the literature thus far.

Case report: We present the case of a 7-month-old patient initially diagnosed with PG. Despite aggressive immunosuppressive therapy, the patient`s high acute phase reactants remained elevated. Although the abdominal ultrasound was normal, advanced imaging was performed due to severe abdominal pain. Contrastenhanced computerized tomography angiography of the aorta and its branches revealed extensive vascular involvement consistent with TA.

Conclusion: In this report, we highlight an infantile case of PG that was subsequently diagnosed as infantile TA. Recognizing the rare association between PG and TA is important. Thorough evaluation and prompt diagnosis of TA in infants with PG can guide further investigations and prevent vascular complications.

Background: The aim of the study was to evaluate the approaches of pediatric rheumatologists and pediatric hematologists to patients with similar musculoskeletal (MSK) complaints and to highlight the differences that general pediatricians should consider when referring patients to these specialties.

Methods: This is a cross-sectional study involving the patients who applied to pediatric rheumatology centers with MSK complaints and were diagnosed with malignancy, as well as patients who were followed up in pediatric hematology centers with a malignancy diagnosis, and had MSK complaints at the time of admission.

Results: A total of 142 patients were enrolled in the study. Of these patients, 83 (58.4%) applied to pediatric rheumatology centers, and 59 (41.6%) applied to pediatric hematology centers. Acute lymphoblastic leukemia (ALL) was the most common diagnosis among the patients who applied to both centers, with 80 cases (56.3%). The median age of diagnosis was 87 (interquartile range, IQR: 48-140) months. The most common preliminary diagnosis in pediatric rheumatology centers was juvenile idiopathic arthritis (JIA), with 37 cases (44.5%). MSK involvement was mainly seen as arthralgia, and bone pain. While arthralgia (92.7%) was the most common complaint in rheumatology centers, bone pain (88.1%) was more common in hematology centers. The most frequently involved joints were the knee (62.9%), ankle (25.9%), hip (25%), and wrist (14%). The most common laboratory abnormalities were high lactate dehydrogenase (LDH), high C-reactive protein (CRP), anemia, and high erythrocyte sedimentation rate (ESR). Thrombocytopenia, neutropenia, and high LDH were statistically significantly more frequent in patients admitted to hematology centers than in patients admitted to rheumatology centers (p < 0.001, p=0.014, p=0.028, respectively). Patients who applied to rheumatology clinics were found to have statistically significantly higher CRP levels (p=0.032).

Conclusions: Malignancies may present with only MSK system complaints in childhood. Therefore, malignancies should be included in the differential diagnosis of patients presenting with MSK complaints.

Background: Adolescent pregnant women have significant risk factors in terms of preterm birth, low birth weight, gestational and neonatal complications, and neonatal and infant deaths. In many countries, living as a refugee differs from living as a local citizen regarding education level, access to health services, and lifestyle. We aimed to compare the obstetric, perinatal, and neonatal outcomes of Turkish and refugee adolescent pregnant women admitted to a tertiary maternity center.

Methods: The study was planned as a retrospective cross-sectional. We included adolescent pregnant women who delivered between February 2018 and August 2023. Adolescent pregnant women were divided into two groups, the Turkish group and the Syrian refugee group, and compared with each other.

Results: One thousand and fifty-one Turkish and 742 refugee adolescent pregnant women were included in the study. Adolescent pregnancy rates are higher in refugees than in the Turkish group (p < 0.001). We found that maternal age (p < 0.001), preeclampsia rates (p=0.029), gestational age at delivery (p < 0.001), and cesarean delivery rates (p=0.02) were lower in refugee adolescent pregnant women. Furthermore, we found that the anemia rates (p < 0.001) and low birth weight newborn rates (p = 0.011) were higher in refugee adolescent pregnant women.

Conclusions: Enhancing the outcomes of adolescent pregnancies among refugees necessitates a heightened focus on education regarding sexual reproduction, increased prenatal follow-ups, and enhanced training in family planning.

Background: Isolated mitral valve aneurysm is rarely reported in children. In most cases it is associated with an underlying disease such as infective endocarditis. MVA can lead to severe complications that needs surgical intervention.

Case: In this report, we present a 9-year old asymptomatic male patient with anterior mitral valve aneurysm and rhythm disturbance diagnosed incidentally during pre-operative evaluation.

Conclusions: Being rare in children, isolated MVA should be kept in mind in the differential diagnosis of mass lesions seen on the atrial side of the mitral valve. A 24-hour electrocardiogram may define subtle rhythm disturbances in these patients.

Background: Intravascular fasciitis (IF) is a benign, reactive, myofibroblastic proliferation that originates from the superficial or deep fascia of small / medium-sized arteries and veins.

Case report: An 8-year-old male patient was admitted to a health center with the complaint of swelling in the inguinal region. Lower extremity venous Doppler ultrasonography showed deep vein thrombosis (DVT) of the femoral vein and anticoagulation with low-molecular weight heparin (LMWH) was initiated. The patient was referred to our center for follow-up. The D-dimer level was detected within normal limits. Doppler ultrasonography was repeated and showed an intraluminal expanding mass lesion with increasing vascularity, without distinct borders and LMWH was discontinued. This lesion at the sapheno-femoral junction was excised surgically and the histopathological examination revealed intravascular fasciitis.

Conclusion: Clinicians should be aware that the clinical findings of IF may mimic sarcoma and thrombosis.

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.