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Objective: Intracranial hemangiopericytomas (HPC) are rare tumors. Radiotherapy (RT) is frequently performed after surgery, depending on tumor size, location, and the type of resection. Moreover, RT is preferred as an effective treatment for local recurrence and metastasis. With this multicenter study, we aimed to investigate the effectiveness of postoperative RT in intracranial HPC patients using modern RT techniques., Materials and Methods: Patients aged 16 years and older who underwent RT for histologically confirmed intracranial HPC were evaluated retrospectively. Forty-four patients from 17 institutions were included. Demographic characteristics of the patients, pathological findings, and prognostic factors were documented. The Kaplan-Meier method was used for local control (LC), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS). The interval for survival analyses was calculated according to the end date of RT. Univariate and multivariate analysis methods were used for factors associated with survival and recurrence., Results: Median age was 42 years (16-71) and 70% of the patients were male. The most common initial symptoms were pain (47.7%) and vision problems (15.9%). A supratentorial location was observed in 79.5% of patients. The median maximum tumor dimension was 4.7 (1.6-14) cm. Gross total (GTR) and subtotal resection (STR) were performed in 43.2% and 47.7% of patients, respectively. Adjuvant RT commenced a median of 6 (2-16) weeks after surgery. Postoperative RT was administered using conventionally fractionated intensity-modulated radiotherapy (IMRT) or stereotactic radiosurgery (SRS). A total median dose of 60 (38-66) Gy in a median of 30 (19-33) fractions was used for patients treated with IMRT and a total median dose of 24 (12-25) Gy in a median of 3 (1-5) fractions was used for patients treated with SRS. Local recurrence occurred in 9 patients and locoregional recurrence in 2 patients at a median of 48 months (range 26-143 months) after RT. Reoperation and reirradiation were applied to 5 patients, reirradiation to 4 patients, and reoperation to 2 patients as salvage treatments. Reirradiation was administered at a median dose of 35 (13.5-54) Gy using a median of 5 (1-30) fractions. At a median follow-up of 63 (6-262) months, 5‑year LC was 68.7%, DMFS 87.2%, PFS 60.8%, and OS 95.7%. The presence of residual macroscopic tumor before RT was associated with lower LC (p = 0.01) and shorter PFS (p = 0.04). In the presence of residual tumor before RT, 5‑year LC decreased from 92.9% to 46.7%, while 5‑year PFS decreased from 81.1% to 43.5% compared to patients with GTR. The presence of postoperative tumor was associated with a lower LC rate in Cox regression analyzes (p = 0.02). The hazard ratio was 6.2 (1.2-30). However, the effect of residual disease before RT on OS was not statistically significant., Conclusion: Adjuvant radiotherapy is performed in the majority of patients with HPC, especially in cases where GTR cannot be performed. In our study, postoperative macroscopic residual tumor was found to be the only factor affecting LC and PFS in patients undergoing adjuvant RT, but its effect on OS was not shown. This may be due to the effectiveness of reoperation and/or reirradiation in the presence of recurrence after RT., Competing Interests: Declarations. Conflict of interest: N. Kaydıhan, G. Yazıcı, P. Erpolat, S. Kamer, B. Erdemci, E. Canyılmaz, B.M. Atasoy, D. Aslan, E.D. Soykut, E. Özyar, F. Demircioğlu, F.Ö. Dinçbaş, M.K. Bolukbas, R. Aksu, S.T. Dinçer, Y. Bölükbaşı, and Y. Güney declare that they have no competing interests. Ethical standards: Ethics approval: This study was performed in line with the principles of the declaration of Helsinki. Approval was granted by the ethics committee of the University of Yüksek İhtisas (reference number 2023/03/56). Consent to participate: Informed consent was obtained before the radiotherapy treatment from all individual participants included in the study. Consent to publish: Our article does not contain data belonging to any person in any way., (© 2024. Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: In this study, we aimed to investigate the bone turnover marker levels according to bisphosphonate usage and radiotherapy (RT) in cancer patients with metastases in osteolytic pattern., Patients and Methods: A total of 52 patients (13 males, 39 females; median age: 52 years; range, 37 to 78 years) treated with RT for osteolytic bone metastases between April 2005 and April 2006 were retrospectively analyzed. Bone-specific alkaline phosphatase (BAP), amino-terminal cross-linked telopeptide of type I collagen (NTX-I), amino-terminal propeptide of type I procollagen (PINP), osteocalcin (OC), deoxypyridinoline (DPD), pyridinoline (PYD), alkaline phosphatase (ALP), creatinine, calcium (Ca), phosphate (P), magnesium (Mg), and 24-h urine Ca levels were measured in blood and urine before the initiation of RT, six weeks and six months after RT., Results: A decrease in BAP, PINP, and creatinine levels was observed after RT (Week 6 p=0.006, Month 6 p=0.008). Sixteen patients who already used bisphosphonate before RT were excluded from statistical calculation. The remaining 36 patients who were treated with bisphosphonate after the first blood test were evaluated separately. In this group of patients, BAP, PINP, NTX, creatinine, and Ca levels significantly increased at six weeks after RT. The PINP and creatinine values significantly decreased at six months after RT. The variation between two different RT arms was assessed with repeated measures variance analysis. There was a statistically significant difference for NTX, OC, and creatinine levels between the first and second measurements., Conclusion: Radiotherapy is an effective method in the treatment of osteolytic bone metastases. Bone turnover markers can provide an objective evaluation on RT response and parallel to imaging modalities criteria for evaluation. Bisphosphonates may alter the levels of these indicators. However, in this study, there were no statistically significant differences between the levels of markers for two different RT schedules.

Sigmoid sinus thrombosis is a rare complication of otitis media and is known to be unilateral. In this report, we present a case of bilateral sigmoid sinus thrombosis secondary to bilateral otitis. To the best of our knowledge, there are no bilateral-simultaneous cases reported in literature to date. Here, the diagnosis of and treatment for the disease as well as predisposing factors are discussed., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors.

Purpose: The aim of the present study was to evaluate retinal and choroidal thicknesses in children with familial Mediterranean fever (FMF)., Methods: Thirty patients with FMF and 28 healthy controls were included in the study. The thicknesses of the retina and choroid of each subject's right eye were measured at the fovea and horizontal nasal and temporal quadrants at 500-µm intervals to 1500 µm from the foveal center using spectral-domain optic coherence tomography., Results: Retinal and choroidal thicknesses at the fovea did not differ between groups (p = 0.32 and p = 0.39, respectively). Horizontal nasal and temporal retinal and choroidal thickness measurements at 500-µm intervals to a distance of 1500 µm from the foveal center were also similar between the groups (all p > 0.05)., Conclusions: The retinal and choroidal thicknesses of children with FMF do not differ from those of age- and sex-matched healthy controls.

Unlabelled: Hepcidin is a regulator of iron balance that is increased in obesity. It reduces the absorption of iron, reduces the transfer of iron from macrophages to the plasma and/or prevents mobilisation of stored iron. Obese patients with non-alcoholic fatty liver disease (NAFLD) demonstrate adipokine and cytokine release promoting inflammatory response. We aimed to analyse the hepcidin levels and iron metabolism in obese children with and without NAFLD and non-obese healthy controls. The study population consisted of 110 children aged 7-18 years in three groups: 50 obese patients without NAFLD, 30 obese patients with NAFLD, and 30 non-obese healthy controls. Serum hepcidin, ferritin, and iron levels, iron-binding capacity, lipid profile, and liver function tests were measured, and hepatic ultrasonography was performed in all participants. Obese patients' white blood cell counts, total cholesterol, triglyceride levels, and homeostatic model assessment of insulin resistance (HOMA-IR) were significantly higher than those of the control group. Iron-binding capacity was significantly higher in obese patients without NAFLD compared with obese patients with NAFLD (p = 0.002). Hepcidin levels were not significantly different between obese patients and the control group. However, hepcidin levels in obese patients with NAFLD were significantly higher than those in obese patients without NAFLD (p < 0.001)., Conclusions: Hepcidin levels were significantly higher in obese children with NAFLD than those without NAFLD. Obese children with NAFLD should receive attention regarding iron metabolism disorders. Serum hepcidin could be a marker of iron metabolism status and NAFLD in these groups of patients.

Aims: This study aims to assess the correlation of mean platelet volume (MPV) and common carotid artery (CCA) thickness in a population of obese adolescents., Methods: Sixty-eight patients and 23 controls were enrolled. Anthropometric measurements, triglyceride, total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase, hemoglobin, white blood cell count, platelet count, MPV and insulin resistance by homeostasis model of assessment of insulin resistance were investigated. Furthermore, CCA thickness was measured by high-resolution ultrasound., Results: MPV and the left CCA thickness were significantly higher in obese adolescents than the healthy controls. The association between MPV and left CCA was checked by linear regression analysis. MPV explained 19% of the variation in left CCA (P < 0.001). At multiple regression analysis, MPV maintained a positive association with the left CCA thickness (P = 0.002) independently of fatty liver grade, relative weight, total cholesterol and homeostasis model of assessment of insulin resistance., Conclusion: MPV is significantly correlated with CCA thickness in obese adolescents., (© 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Fibromuscular dysplasia is a rare, idiopathic and nonatheromatous disease. It is rarely encountered as a cause of stroke in children. We report a nine-year-old girl with stroke in whom extensive fibromuscular dysplasia of intracranial vessels was established. She also had familial combined hyperlipidemia as an additional risk factor. This case suggests that additional risk factors like hyperlipidemia in cases with fibromuscular dystrophy may facilitate the occurrence of stroke at early ages.

The spatial distribution, degree of pollution and major sources of PCBs were evaluated in surficial sediments within the heavily urbanized and industrialized İzmit Bay and its main freshwater inputs. ΣPCB concentrations range from 2.90 to 85.4ngg(-1) in marine sediments and from ND to 47.7ngg(-1) in freshwater sediments. Results suggest that high concentrations of ΣPCBs were localized around a chlor-alkali plant and an industry that handles bulk liquid, dry and drummed chemicals, and petroleum products in the Bay. Using a chemical mass balance receptor model (CMB), major sources of PCBs in the region were investigated. The CMB model identified Aroclor 1254 and 1260 to be the major PCB sources in marine sediments and the less chlorinated Aroclor 1248 and 1242 as the major PCB sources in freshwater sediments. The potential sources for the PCBs were briefly discussed in terms of their use in various industrial applications., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

A 33 weeks' gestation, a baby with rhesus hemolytic disease (RHD), who had received intrauterine transfusions twice, developed cholestatic hepatic disease and late hyporegenerative anemia. Her serum ferritin and bilirubin levels increased to 8842 ng/ml and 17.9 mg/dl, respectively. Liver biopsy showed cholestasis and severe iron overload. Treatment with recombinant erythropoietin (rHuEPO) decreased the transfusion need, and intravenous deferoxamine resulted in a marked decreased in serum ferritin levels and normalization of liver function. In patients who have undergone intrauterine transfusions due to RHD, hyperferritinemia and late hyporegenerative anemia should be kept in mind. Chelation therapy in cases with symptomatic hyperferritinemia and rHuEPO treatment in cases with severe hyporegenerative anemia should be considered.

The aim of this study is to evaluate the carotid artery intima media thickness and serum lipids in pediatric patients with epilepsy treated with valproic acid. The study included 44 pediatric epileptic and 40 healthy children. Intima media thickness of left common carotid artery and fasting lipid profile (total cholesterol, triglycerides, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol) were assessed. Although we did not observe any differences regarding serum lipid profiles, intima media thickness of common carotid artery was significantly higher in epileptic patients treated with valproic acid. We suggest that this increase in intima media thickness of common carotid artery may be due to epilepsy and/or valproic acid treatment.

The authors investigated demographics, clinical and laboratory features, treatment responses, and outcomes of 93 children (median age 5 years) admitted for idiopathic thrombocytopenic purpura (ITP). The therapy responses of high-dose methylprednisolone (HDMP) (n = 77) and intravenous immunoglobulin (IVIG) (n = 10) treatments were similar. None of the patients with hemorrhage died. Fifteen patients (16.1%) had progressed into chronic ITP. Seven infants had a probable relationship with vaccination; none of these infants progressed into chronic ITP. In conclusion, the overall prognosis in childhood ITP is good. The therapy responses of HDMP and IVIG treatments are similar. Also, ITP cases who havw vaccination history have a benign course.

Infection around the shoulder girdle is an infrequent and difficult diagnosis requiring a high index of suspicion and early evaluation by the physician. An infection within the area of the subscapularis muscle and the chest wall is extremely rare. To the best of our knowledge, only 4 cases of subscapular abscess have been reported. In this article, we present a 7-year-old boy with a subscapular abscess after blunt trauma.

Rhabdomyolysis with myoglobinuria is an uncommon complication of bacterial sepsis. The authors describe three pediatric acute lymphoblastic leukemia patients who developed rhabdomyolysis during a neutropenic sepsis episode due to Escherichia coli. All of the patients needed hemodynamic supportive treatment because of septic shock. Broad-spectrum antibiotics, alkalinization, and intravenous fluid therapy was given. One patient with renal insufficiency died, despite aggressive treatment. Muscle pain and dark urine color should alert physicians to the possibility of rhabdomyolysis in immunocompromised patients with sepsis. Early and appropriate treatment is critical in these patients to prevent renal failure and shock, and for a better outcome.

Objective: To test, via prospective case-control study, whether measurements of carotid artery intima-media thickness (cIMT) reflect the susceptibility to atherosclerosis in obese children with nonalcoholic fatty liver disease., Patients and Methods: In all, 80 obese asymptomatic normotensive adolescents (body mass index [BMI], 29.12 +/- 5.13; 8.5-17.5 years) and 30 age- and sex-matched nonobese healthy children (BMI 18.55 +/- 2.24). Children were analysed in 4 groups: Controls formed group 1, 26 children with obesity without hepatosteatosis formed group 2, 32 children with grade 1 hepatosteatosis formed group 3, and 22 children with grade 2 or 3 hepatosteatosis formed group 4. The key parameters were BMI, liver function tests, lipid profiles, glucose, hepatic ultrasonography, and IMT of common carotid artery (CCA), left carotid bulbus (CB), and left internal carotid artery (ICA)., Results: Comparisons of IMT measurements of left CCA, left CB, and left ICA between controls and obesity group revealed statistically significant differences (each segment P < 0.0001). There was an increase in the mean of IMT of each segment with the increase in hepatosteatosis grade (each segment P < 0.0001). The relation between the mean of IMT of left CCA, left CB, and left ICA with BMI (P = 0.001, r = 0.318), alanine aminotransferase (P = 0.007, r = 0.256), and hepatosteatosis (P < 0.0001, r = 0.470) was significant. Multiple regression analysis between left CCA, left CB, and left ICA, and age, sex, relative weight, alanine aminotransferase, total cholesterol, obesity, and hepatosteatosis grade was significant for only hepatosteatosis grade., Conclusions: The cIMT is significantly higher, increasing susceptibility to atherosclerosis, in obese children with hepatosteatosis than in healthy children or obese children without hepatosteatosis.

Summary: We evaluated the frequency, etiologic factors, outcome, and the comorbid conditions affecting the morbidity and mortality of pulmonary complications in acute childhood leukemia. Sixty-six (40.4%) out of 163 patients developed 79 pulmonary complications. Infectious etiology was the leading cause (92.4%). The most identified infectious agents were Gram (-) bacteria, followed by fungi. Acute respiratory distress syndrome, leukostasis, lymphomatoid granulomatosis, pulmonary edema, and pneumothorax were among the noninfectious causes. The pulmonary complications in the induction and consolidation phase of leukemia therapy were more severe and the mortality rate was higher. Tachypnea, shock, oxygen and mechanical ventilation requirement, disseminated intravascular coagulation, involvement of other organs or systems, cytopenias, requirement of modification in antimicrobial drugs were found to be related with increased mortality risk. The mortality rate of pulmonary complications was 8.9%. Pulmonary infections in the maintenance phase of the therapy were frequently treated with oral antibiotics, and they were generally rapidly taken under control. In conclusion, pulmonary complications are frequent in children with acute leukemia, and early diagnosis and appropriate management are important to avoid mortality owing to pulmonary complications, especially in neutropenic patients receiving induction or consolidation phase of chemotherapy.

Chemotherapy-induced acral erythema or palmoplantar erythrodysesthesia syndrome is a well-defined reaction to some of the chemotherapeutic agents such as methotrexate, cytarabine, doxorubicin, fluorouracil, and bleomycin. This reaction is characterized by symmetrical, well-demarcated, painful erythema of the palms and soles, which may progress to desquamation. The authors present a case of acral erythema in a young patient with acute monoblastic leukemia to emphasize this high-dose chemotherapy-induced side effect, which is rarely seen in children and is usually self-limited.

Additional chromosomal abnormalities are found in 5-20% of patients during chronic phase of chronic myeloid leukemia and in 60-80% preceding or accompanying blast crisis. These abnormalities are important in disease progression and, because they may occur before hematological and clinical symptoms, can be taken as a prognostic indicator. An adolescent with chronic myeloid leukemia initially presented with extreme thrombocytosis, increased megakaryopoiesis with dysmorphic features, and focal myelofibrosis in bone marrow examinations and then developed isolated myelosarcoma 1 year after onset, with t(9;22)(q34;q11.2), +8, +14, +21, and der(1)(p36).

The authors overviewed 239 febrile neutropenia (FN) episodes in 82 pediatric leukemia cases treated with BFM treatment protocols. FN was observed mostly during consolidation therapy. Mucositis was the most identified focus; gram-negative microorganisms were the most identified pathogens. Five patients developed invasive fungal infections. Fever resolved after mean 5.3 days and mean antibiotic administration time was 12.7 days. Addition of G-CSF to antimicrobial therapy shortened the duration of neutropenia, but it did not affect duration of fever resolution and antibiotic administration. The duration of neutropenia, fever resolution, and antibiotic administration was significantly longer in children with acute myeloid leukemia. The authors conclude that children with acute leukemia have severe prolonged neutropenia and are in high risk. In these patients, prediction of the risk of bacteremia based on clinical and laboratory features is important for immediate empiric broad-spectrum antimicrobial therapy and for higher survival rate.

We aimed to retrospectively evaluate the skin and soft tissue complications secondary to procedures in acute leukemia patients with and without catheters. Eighty-seven acute leukemia patients (75 acute lymphoblastic leukemia, 12 acute myeloid leukemia ) were included. There were 30 patients with 37 catheter use (6 port, 31 Hickman catheter) and 57 patients without catheter. In patients with catheters, skin and soft tissue complications were seen in 20 (66%) children. The most frequent complication was cellulitis (55%). In the patients without catheter, skin and soft tissue complications were seen in 37 (65%) patients. Cellulitis (37.8%) and extravasation (37.8%) were the most frequent causes. When the frequency of skin and soft tissue complications in patients with and without catheters were compared with each other, there was statistically no significant difference (P=0.792). The duration of chemotherapy was significantly longer in patients who developed skin and soft tissue complications with or without catheters when compared with the duration of the therapy in patients without any skin and soft tissue complications (259.2+/-36.3 and 218.3+/-58.3 d, respectively; P<0.0001). In pediatric leukemia patients, with or without catheters, skin and soft tissue complications are common and these complications may prolong the duration of chemotherapy.

Idiopathic thrombocytopenic purpura usually presents with minor bleeding such as petechia and purpura. Rarely, life-threatening events as intracranial and intra-abdominal bleedings can be seen. We would like to present a rare case diagnosed as idiopathic thrombocytopenic purpura, presenting with abdominal pain and paleness. In this 17-year-old female patient, extensive abdominal sensitivity was revealed on physical examination and massive intra-abdominal hemorrhage secondary to distended ovarian follicle rupture was seen on ultrasonography and abdominal computed tomography. The case was treated successfully with intravenous immunoglobin, thrombocyte suspension, and pulse methylprednisolone.

Background: A newborn with cholestatic hepatic disease and hemophagocytic lymphohistiocytosis due to rhesus hemolytic disease (RHD) is reported., Observation: A 34 weeks' gestation baby with RHD, who had received multiple intrauterine transfusions (IUT), developed cholestatic hepatic disease and secondary hemophagocytic lymphohistiocytosis (HLH). Her serum ferritin level increased to 5,527 ng/mL, and liver biopsy showed severe iron overload. Treatment with intravenous desferrioxamine resulted in a marked decrease in serum ferritin levels and normalization of liver function, Conclusion: We suggest that patients who have undergone IUT be evaluated for hyperferritinemia. If hyperferritinemia is noted, chelation therapy should be considered. As another rare finding, HLH can complicate the course of RHD.

Myelodysplastic syndromes (MDS) are rare in children, representing 3% or less of all hematopoietic malignancies. Cytogenetic abnormalities, such as -7/7q-, +8, and +21 have been reported in 55-80% of children with MDS. Cytogenetic studies have an important impact on diagnosis, treatment selection, and monitoring therapeutic protocols when combined with morphologic data. We report on a pediatric case of MDS with the presence of the rare clonal abnormality del(13)(q14q22) which underwent a malignant transformation to leukemia and ran a very poor clinical course.

This study was performed to determine the absolute PMNL count as well as phagocytic functions in adolescents who had undergone CPB; a radioactive method was used for the first time in this field. Although CPB causes a decrease in the absolute PMNL count when the subject is off bypass, this value exceeded the baseline level within three days. A day prior to surgery PMNL phagocytosis was found to be normal and was unaffected within the first minutes on bypass. Whereas, PMNL phagocytosis decreased significantly off bypass. However, the decrease was transient and returned to normal within three days. Another interesting finding obtained as a result of this study was that in the cases in whom the clinical infection picture was seen, the patient had a longer duration on bypass and a higher inhibition of leukocyte phagocytosis. The correlation between the duration on bypass and inhibition of leukocyte phagocytosis could be shown as a trend. Thus it may be concluded that the longer the on bypass period the higher the inhibition of leukocytic phagocytosis and the higher the infection rate in the postoperative period.