Iranian Journal of Pediatric Hematology and Oncology
Volume:9 Issue: 1, Winter 2019

With the increased survival rates following the treatment of childhood cancer, it becomes equally important that the need for evidence based surveillance of long term effects of cancer therapy is addressed. This includes the risk of development of metabolic syndrome features like obesity, altered lipid and sugar profile, which was attempted in the present study. In this cross sectional case study, 50 survivors of childhood cancer aged between 5 – 18 years were recruited. Positive history of obesity, diabetes mellitus, dyslipidemia, and stroke in family were recorded and their anthropometry was noted with calculation of their Body Mass Index (BMI). Fasting lipid profile, blood sugar, and serum insulin levels were tested; the Homeostatic model assessment of Insulin Resistance (HOMA IR) value and the Fasting Glucose to Insulin Ratio(FGIR) were derived as markers of insulin sensitivity. The data were analyzed using SPSS (version 17.0). In these fifty children, the risk factors studied for dyslipidemia and insulin resistance due to chemotherapy were: age at diagnosis, sex, radiation exposure, steroid, and L-asparaginase use during the treatment for cancer. Among the fifty survivors, 7 were found obese, 32 normal, and 11 underweight as per the age specific BMI charts. Their metabolic parameters showed that 12 had raised cholesterol levels, 8 had raised triglyceride levels, and 4 had lowered HDL-C levels. Nine survivors also had raised HOMA-IR levels. However, these metabolic derangements were not found to be statistically significant (p value>0.05) and no correlation was found between the risk factors and obesity, dyslipidemia, or insulin resistance. As against the prior evidence, there was no risk of developing obesity, dyslipidemia, and insulin resistance in survivors of childhood cancers.

Drug induced hemolytic anemia and thrombocytopenia (DIHA and DIT) are common drug adverse effects of antibiotics in patients admitted to hospital. This reaction is important in patients who have a chronic disease especially in pediatrics. In this study, possible hemolytic anemia was investigated before and after the antibiotics administration. A total of 835 children were investigated in this retrospective study. The laboratory tests were performed before and at least one week after antibiotics administration. The red blood cell (RBC), platelet (plt), hematocrit (Hct), and hemoglobin (Hb) were measured. With respect to age, 76.11% of studied patients were under 6 years old. The others were between 6-10 years (mean 5.38 years). The two tailed T tests results on the patients’ information showed a difference between RBC, platelet, hematocrit, and hemoglobin values before and after antibiotics administration to the point where the RBC mean counts before and after administration were 4.53 to 3.82 *1012/L, respectively. These changes for plt, Hb, and Hct were 323.5 to 232.5 *109/L, 13.61 to 11.46 mg/dL, and 40.83 to 34.38 %, respectively. The p-values were 0.000025, 0.000051, 0.000061, and 0.000032 for RBC, platelet, hematocrit, and hemoglobin; respectively. This finding confirmed that antibiotics administration can decrease the platelets and RBC count. The antibiotics used in the children were ceftriaxone (38.2%), clindamycin (23.3%), Clarithromycin (19.6%), and acyclovir (12.1%); respectively. The dose of the ceftriaxone varied from 50 mg/kg to 70 mg/kg in shigelloses and pneumonia, respectively. Additionally, clindamycin, clarithromycin, and acyclovir were prescribed for 10 mg/kg, 5-10, and 10 mg/kg per day; respectively. This study showed that antibiotics administration had adverse effects and should be considered when they are prescribed to children with chronic diseases. The physicians should be awarded about proper dosing to decrease adverse effects.

Reticulocytes are immature red blood cells with RNA, spending the final stages of their maturation in the peripheral blood. The number of reticulocytes in the peripheral blood is the salient evidence of the effectiveness of bone marrow to produce red blood cells. Currently, reticulocyte count is done manually or automatically in clinical laboratories. Difficulties and limitations of these approaches, including imprecision, poor reproducibility and laboriousness are clear. This study used modification in manually staining method to achieve better counting. The reticulocyte counts of 30 samples were obtained from patients referred to Afzalipour hospital in Kerman, Iran. The patients aged between 0-3 years old (median age= 1.4 years). Samples were investigated for reticulocyte count by three different methods, including conventional, modified, and flow cytometry methods. Modified staining method is used in the optimized method which helps operator to countg reticulocyte better than conventional method. Analysis of the results showed a reasonable agreement. Sphericity assumption was checked by Kolmogorov-Smirnov and Mouchley analyses. Repeated measurement analysis was performed using Greenhouse-Geisser correction (F=16.6, df=1.105) according to pairwise comparisons with Bonferroni adjustment. Mean differences for flow cytometry, optimized and common methods were 3.88, 3.59, and 2.46 were significant at the 0.05 level, respectively. The proposed optimized method was simple method, highly reliable, and comparable to flow cytometry. Due to the ease of implementation, this method did not need specialized training or the use of complex devices so it is affordable.

Thalassemia is one of the most common genetic disorders throughout the world. Blood transfusion plays an important role in the treatment of thalassemia but it leads to numerous complications such as iron overload and alloimmunization. This study evaluated the frequency and risk factors associated with alloimmunization in thalassemia major patients living in Markazi province, Iran.

In this descriptive study, 48 thalassemia major patients who underwent blood transfusion at Amirkabir hospital were included. Patients' demographic data were recorded using a questionnaire. In order to perform alloimmunization screening and autoantibody assessment, patients were referred to Tehran Blood Transfusion Organization Laboratory.

The current study was performed on 48 patients with thalassemia major,. The mean age of patients was 12.5 ± 8.3 years. Among patients 26 (54.16%) were male and 22 (45.83%) were female, 13 patients (27.08%) had alloantibodies. Among 48 patients, 19 (39.58%) had undergone splenectomy. The patients' age of the first blood transfusion ranged from 1 month to 14 months and the mean age of the first blood transfusion was 9.5 ± 7.08 months. The blood transfusion intervals in patients were from 21 days to 40 days and the blood volume received at each transfusion session was 10-15 cc/kg of the body weight. In the current study, the data analysis indicated no significant correlation between alloantibodies and RH phenotype (P=0.43), patients' gender (P=0.9), or blood groups (P=0.4); whereas, a significant correlation was found between alloantibodies and splenectomy (P=0.02) as an increase in the prevalence of alloantibodies was reported in splenectomised patients.

No significant difference was found between the patients with and without alloantibodies in terms of the prevalence of Rh phenotype, gender, and blood groups. However, there was a significant difference between the patients with and without alloantibodies in terms of splenectomy.

Thalassemia major (TM) is one of the most common hereditary anemia with multiple endocrinopathies (especially hypogonadism). So, we evaluated the rate of delayed puberty (DP) and its relation with serum ferritin level in patients. This cross-sectional (descriptive-analytical) study was conducted on 100 patients with TM between 14-64 years old, admitted to Amirkola Thalassemia Center, Babol, Iran, in 2016. The pubertal status, (Marshall-Tanner scale), existance of DP, and its different types were evaluated. Mean serum ferritin level was measured and the data were classified to three groups of <1500, 1500-2500, and >2500 ng/ml. Data were analyzed using SPSS (version20). Out of 100 patients, 64 (64%) and 36 (36%) were female and male, respectively. Considering age, 23, 77 patients (%) were under and over 20 years old, respectively. Totally, 69 (69%) of them had DP, of whom 64 (92.8%) ones had secondary (central) hypogonadotropic hypogonadism. Mean serum ferritin level (±SD) was 2707.94±1683.42 ng/ml. In addition, 26, 29, and 45 patients had ferritin level <1500, 1500-2500, and >2500 ng/ml, respectively. Thirty two patients with DP (46.4%) had ferritin level above 2500 ng/ml (p-value= 0.623). The results showed a high frequency of DP in TM patients, requiring careful examination and follow-up in terms of puberty for early diagnosis and proper treatment to improve their quality of life, and prevention of the complications like osteoporosis. We couldn't find any significant relationship between serum ferritin level and hypogonadism, even for cases who received enough iron chelators.

Recurrent blood transfusion is a common treatment in patients with thalassemia. The development of antibodies against red blood cell (RBC) antigens complicates RBC cross-matching, enhances the in vivo destruction of transfused cells, accelerates tissue iron overloading, delays the provision of safe transfusion, and reduces health-related quality of life. In total, 516 thalassemia patients with a mean age of 18.5 years were included in this cross-sectional study in Mashhad University of Medical Sciences, Razavi Khorasan Province, Iran, in cooperation with the Abu Rayhan Special Medical Center and Hormozgan Blood Transfusion Organization between June 2015 and May 2016. The detection and identification of alloantibodies were done using 3 screen cells and 11 panel cells, respectively. To detect autoantibodies, auto-control was performed using polyspecific Coombs (IgG + C3d) standard method. Alloantibodies and autoantibodies were observed in 16 (3.1%) and 21 (4.1%) patients, respectively. Among patients with alloantibodies, 2 patients (12.5%) developed 3 antibodies (Anti-c,E,P1; Anti-c,E,K), 1 patient (6.25%) developed 2 antibodies (Anti-D,C), and 13 patients developed 1 antibody (4 patients Anti-D (25%); 3 Anti-K (18.75%); 2 Anti-E (12.5%); 2 Anti-C (12.5%); 1 Anti-Jka (6.25%); and 1 Anti-Jkb (6.25%)). A statistically significant correlation between patient age (P = 0.031), age of splenectomy (P = 0.006), Rh(D) (P = 0.001), leukoreduction of RBCs (P = 0.043), and type of disease (P = 0.006) with RBC alloimmunization was seen. This study emphasized the need for the determination of RBC minor antigens, especially for Rh, Kell, and Kidd blood group systems, before the first transfusion and transfusion of antigen-matched blood. In addition, transfusion of prestorage leukoreduced packed cells is recommended for these patients.

Pediatric palliative care is a holistic caring approach for children and families that begins with diagnosis of a life-threatening illness and continues until death; it aimed to relieve pain and other symptoms in physical, mental, social and spiritual aspects. In spite of available evidence concerning optimal outcomes of the provision of palliative care, establishment of a palliative care system for children has hardly been feasible so far due to a number of challenges. Therefore, this review study aimed at identifying the challenges of the provision of pediatric palliative care along with the relevant solutions. The identified challenges were classified into two categories including structure-based challenges (i.e. lack of a clear structure in the health system and classification of services, shortage of specialized staff, insufficient home care services, absence of health care tariffs along with insurance coverage of palliative care services) and process-based challenges (i.e. absence of guidelines, lack of educational programs for family, family attitudes and beliefs, communication barriers, and lack of access to opioids). Thereafter, the solutions for each challenge are provided in accordance with the available literature separately. Given the significance of palliative care for children with cancer and in order to improve the quality of life of the children and the families, it appears necessary that policymakers and managers take account of the challenges as well as the feasibility and the implementation of provided solutions.

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm that can be potentially low malignant and mainly affects infants and adolescents. This tumor usually is seen in the skin, soft tissue, and retroperitoneum. This study is a case of KHE in the long bone of the ulna. The subject was a 7-year-old female patient with osteolytic lesion in the forearm without cutaneous lesions. Histologically, the neoplasms comprised of nodules of spindle-to oval-shaped cells that grew in an infiltrative fashion. The results of 2-year follow-up of the patient after enblec resection were desirable and no recurrence was observed. This is the first study to report a case of KHE of the bone in Iran.