Iranian Journal of Pediatric Hematology & Oncology

A study of pulmonary infectious and non-infectious complications in a pediatric hematopoietic stem cell transplantation (HSCT) center in Iran

Seyed Ahmad Tabatabaei — 2024-10-01

Background: Pulmonary complications are important enough to notice in hematopoietic stem cell transplantation (HSCT). The patients undergoing HSCT might have infectious and non-infectious problems associated with morbidity and mortality. The pulmonary complications of HSCT are well-recognized in adults; however, studies on children are limited, especially in Iran. This study was done to evaluate the infectious and non-infectious pulmonary complications and the corresponding factors in children who underwent HSCT.

Materials and Methods: This retrospective cohort study included the patients who underwent HSCT in Mofid Children’s Hospital in Tehran, Iran, during the years 2015 -2021. Overall, 144 medical files were evaluated, out of which 128 had undergone HSCT. The extracted data were about underlying diseases, age at transplant, sex, type of HSCT, donor type, cell source, conditioning regimen, graft versus host disease (GVHD) prophylaxis, infectious and non-infectious pulmonary complications, and associated factors. The data analysis was done by the SPSS software version 26. Chi-square, Fisher exact test and regression were also used for the analysis.

Results: Infectious and non-infectious pulmonary complications were reported in 26 people (20.3%) and 11 people (8.6%), respectively. Positive coronavirus disease 2019 (COVID-19) PCR was detected only in two patients. Infectious complications were significantly lower in patients with neuroblastoma compared to other underlying diseases (2.7% vs. 27.5%, P = 0.002). These complications were significantly more frequent among those with other HSCT complications compared to those without such complications (34.6% vs. 16.7%, P=0.042). Non-infectious pulmonary complications were significantly higher in boys (13.5%) than in girls (1.9%) (P = 0.024).

Conclusion: Due to the high rate of pulmonary infections in bone marrow transplant patients, clear differential diagnosis and diagnostic work are essential.

Beta-Thalassemia Haplotypes in Southwest of Iran

Bijan Keikhaei Dehdezi — 2024-10-01

Background: Thalassemia is a widespread disease affecting people across various ethnicities and regions. In comparison to previous studies conducted in different regions of Iran, such as those in Lorestan and Sistan-Baluchestan, this study highlights unique mutation patterns prevalent in the southwestern population, emphasizing the genetic heterogeneity in this region. The identification of common mutations of beta-thalassemia in various ethnic groups within the nation is regarded as a practical solution for thalassemia prevention and prenatal diagnosis.

Materials and Methods: In this retrospective observational study, the medical records of 545 patients with various types of beta-thalassemia (silent, minor, intermediate, and major), referred to the center at Baqaei 2 hospital over a 14-year period (2008–2022), were examined. The age range of patients spanned from a 2-month-old fetus to a 34-year-old individual. Their mutations and thalassemia types were determined and confirmed using molecular methods, including PCR-ARMS (polymerase chain reaction-amplification refractory mutation system) and sequencing. The results were analyzed using SPSS software.

Results: The study examined 545 patients and identified 81 types of mutations. The most frequent mutations observed were CD36-37(-T)/N, IVSII-1/N, and IVS1-110(G>A). The study also noted population heterogeneity, reflected in the wide range of mutations found in the region. Among the patients, 6 had the silent form of beta-thalassemia, 488 had the minor form (464 patients and 24 fetuses), 9 had the intermediate form (8 patients and 1 fetus), and 42 had the major form (26 fetuses and 16 adults).

Conclusion: The identification of prevalent beta-thalassemia mutations facilitates disease control and prevention programs and is crucial for the identification of various beta-thalassemia gene mutations. This should be re-evaluated periodically. Observing a wide range of beta-thalassemia genotypes in the southwestern region of Iran suggests gene flow; thus, identifying these genotypes is instrumental in preventing and controlling the disease.

Impact of Vitamin D Supplementation on Pain Management in Sickle Cell Disease Patients

Ehsan Rezaeinejad — 2024-10-01

Background: Vitamin D deficiency is prevalent among patients with sickle cell disease, regardless of their age or the season. Since there has been no extensive research on the impact of vitamin D on pain in sickle cell disease, this study aimed to evaluate how vitamin D supplementation influences pain management in these patients.

Materials and Methods: This retrospective study was conducted on children (12.3±4.45 years old) with definitive diagnosis of sickle cell. The study was done in the Department of Oncology of Shahid Sadoughi Hospital, Yazd, Iran, over the years 2019-2021. Thirty patients (14 boys and 16 girls) with vitamin D insufficiency and deficiency entered the study and 50000 IU vitamin D was given weekly for 16 weeks. The measurement of vitamin 25(OH) D3 before and after intervention was done by ELISA method (Monobind kit, USA). Pain intensity assessment was also done. Other relevant data were extracted from the patients’ medical records.

Results: In this study, amomg 30 patients, 46.6% of them had vitamin D deficiency. A significant reduction in pain intensity was observed in patients following the intervention (p<0.05).

Conclusion: Based on the findings of this study, vitamin D was effective in reducing pain intensity in these patients. Therefore, vitamin D can be used in the pain management of these patients.

Comparison of the Volumetric Parameters of Platelets in Diabetic Children with and without Diabetic Ketoacidosis: A Case-Control Study in the North of Iran

Fatemeh Mirdoosti — 2024-10-01

Background: This study aimed to determine the volumetric parameters of platelets including MPV (Mean Platelet Volume) and PDW (Platelet Distribution Width) in diabetic children with and without diabetic ketoacidosis (DKA) and to evaluate their relationship with the severity of DKA.

Materials and Methods: A case-control study was conducted on 112 children with type 1 diabetes mellitus (T1DM), with and without DKA, who referred to Amirkola Children's Hospital, Iran, in 2016-2021. Of them, 43 were boys, and 69 were girls. The diagnosis of diabetes was based on clinical symptoms and diagnostic criteria, and DKA was diagnosed through the analysis of arterial blood gas. The patients’ MPV and PDW were also evaluated, and the P values less than 0.05 were considered significant.

Results: Out of 112 children with diabetes (with the mean age of 8.71 ± 3.22 years), 56 persons had DKA. In terms of DKA severity, 17 patients (30.4%) had mild, 29 (51.8%) had moderate, and 10 (17.8%) had severe DKA. The children with DKA had the mean blood glucose of 490.98±46.11mg/dL, MPV of 10.02± 1.19fl), and PDW of 13.07± 1.18flwere significantly higher (P<0.001). It was found that, an increase in the severity of DKA would raise the mean MPV (P = 0.006) and PDW(P<0.001) significantly.

Conclusion: Based on the results of this study, as the severity of DKA increases in diabetic children, the mean levels of MPV and PDW increase significantly. Therefore, it is suggested that MPV and PDW be used to predict the severity of DKA in diabetic children.

Evaluating the Role of Silymarin in Coordinating the Relationship between Inflammation and Thrombosis by Affecting Endothelial Cells

Roya Sharifi — 2024-10-01

Background: A widespread crosstalk between inflammation and coagulation has been shown in numerous studies. This suggests that coagulation can trigger an inflammatory response which ultimately leads to coagulation activation. Previous research has shown that polyphenols can affect blood pressure and endothelial dysfunction, resulting in reduced risk of cardiovascular diseases. This study aimed to investigate whether Silymarin, a ﬂavonolignans, could play a role in the interaction between inflammation and coagulation by influencing endothelial cells.

Materials and Methods: In this experimental study, human umbilical vein endothelial cells (HUVECs) were seeded with and without various concentrations of silymarin. In vivo, treatment with silymarin was also carried out. Coagulative and fibrinolytic factors, including Von Willebrand factor (VWF) and Factor VIII (FVIII), tissue plasminogen activator-1 (TPA-1), and inflammatory factors, including interleukin 8 (IL-8) and tumor necrosis factor-alpha (TNF-α), were evaluated by flow cytometry, Real-Time Polymerase Chain Reaction (qPCR), Enzyme-Linked Immunosorbent Assay (ELISA) and Immunocytochemistry (ICC).

Results: Silymarin increased the gene expression, release, and storage of VWF while diminishing the gene expression, release, and storage of TPA-1 (P ˂ 0.05). The activity of FVIII was dramatically increased, and IL-8 and TNF-α levels were augmented. The in vivo study also indicated an elevated plasma level of VWF and IL-8 by silymarin administration.

Conclusion: The results showed that, although silymarin reduces inflammatory factors, it can affect coagulation factors by increasing the levels of VWF and FVIII activity and inhibiting TPA-1 production, thereby making thrombosis probable. Consequently, it is advisable to prescribe this medication with caution for individuals who are susceptible to thrombotic events.

The Evaluation of Ocular Toxicity of Chemotherapy Drugs

Seyed Mojtaba Sohrevardi — 2024-10-01

Cancer continues to pose a substantial global health burden and remains one of the leading causes of mortality worldwide. Encouragingly, survival rates have consistently improved, largely due to advancements in diagnosis and treatment. The development of anticancer drugs, including cytotoxic chemotherapy, hormonal agents, and targeted therapies, has significantly enhanced the efficacy of cancer treatments.

Chemotherapy-induced ocular toxicity encompasses a wide range of disorders, influenced by the eye's unique anatomical and physiological characteristics. The mechanisms of these drugs can lead to systemic and ocular side effects, including cytotoxicity, inflammation, and neurotoxicity. While ocular side effects from targeted therapies are less common, they can be severe, disabling, and potentially irreversible. In some cases, immediate discontinuation of the drug may be necessary to prevent vision-threatening complications.

Understanding these ocular side effects is crucial for early recognition and intervention by ophthalmologists and oncologists to prevent blindness. Additionally, anticipating treatment-related toxicities enables pharmacists to develop strategies that minimize or mitigate these side effects.

This review focuses on the ocular toxicity associated with the most significant anticancer chemotherapeutic agents.

Locally Advanced Childhood Nasopharyngeal Cancer: A Case Series

Julius Oentario — 2024-10-01

Nasopharyngeal carcinoma is a rare malignant tumor in childhood. To date, no consensus has been achieved concerning the recommendations for the management of childhood Nasopharyngeal carcinoma. Chemotherapy and radiotherapy are the main modalities in the management of nasopharyngeal carcinoma (NPC). Five cases (with an age range between 10 and 16 years), including one patient with stage III NPC and four patients with stage IV NPC, are reported in this study. All patients received four courses of induction chemotherapy followed by concurrent chemoradiation with cumulative irradiation, with a dose of 70 Gy in 33-37 fractions. Patients were treated with intensity modulated radiation therapy with simultaneous integrated boost technique using helical Tomotherapy. After two-year’s follow-up, four patients were achieved complete response, one patient showed residual disease in lymph nodes in the neck and bone metastases one year after the completion of treatment. Treatment side effects were well tolerated, there was no acute toxicity grade 3 or 4, and late toxicity consisted of chronic sinusitis, grade 1 xerostomia, and grade 1 skin fibrosis.

A Compound Heterozygous of Hb E and Beta Thalassemia Independent of Transfusion: A Rare Case Report

Anju — 2024-10-01

Hemoglobinopathy is now a worldwide problem. Mainly described in Southeast Asian countries. A compound heterozygous form of hemoglobin (Hb) E & b thalassemia was reported to be 50% of all severe forms of thalassemia. Clinical presentation of HbE/ b thalassemia is reported to be moderate to severe anemia. The children usually present at the age range of 7 months to 6.9 years. The clinical severity is affected by genetic factors, including mutation in a chain (b thalassemia), alpha-chain (a thalassemia), amount of adult Hb, and co-inheritance of another hemoglobinopathy. Here, we present a rare case of compound heterozygous of HbE/ b thalassemia), who survived without transfusion and complication until eight years of age. Peripheral blood smears and complete blood count showed microcytic hypochromic anemia. High-performance chromatography showed a compound heterozygous of HbE/ b thalassemia.