A New Approach Based on Erythrocyte Indices to Exclude Normal Populations from Chromatography Based Thalassemia Screening Programs with Very High Fidelity 2 2 Background: Screening and counselling is the most effective way to prevent the birth of children with thalassemia major. An accurate and relatively less time-consuming protocol is necessary to screen large populations. Separating iron deficiency anaemia from thalassemia trait based on blood cell parameters has been used by hematologists for many years. We aimed to design a new approach to screen the microcytic populations. Methods: Blood cell parameters and chromatography were used to screen the populations traditionally. Validating the result with a five-point decision tree analysis with two equations based on cut-off values of five blood cell parameters was performed. 2984 participants were screened traditionally, of which 289 were found to be beta-thalassemia trait, 63 were Hemoglobin E carriers, 15 were found to be Hemoglobin D (Hb D) Punjab, 4 hereditary persistent fetal hemoglobin (HPFH), and 14 belonged to beta thalassemia traits with HbA2 levels between 3.3% to 3.8% associated with reduced mean corpuscular volume (MCV) and mean corpuscular haemoglobin (MCH) (borderline cases). Results: In the decision tree approach, 51.3% with beta thalassemia trait and 11.65% Hb E carriers were detected perfectly. 27% of participants were detected as non-thalassemia carriers which could be excluded from further chromatographic analysis. Conclusion: During the early stages of the carriers screening program, a large portion of the sample could be excluded, based on segregating the IDA and thalassemia carrier population. Decision tree analysis and equation derived from the regression are essential to from limit of exclusion which implies significant cost reductions. 107 118 2020/11/16 1399/8/26 2021/11/29 1400/9/8 Abhishek Samanta Abhishek Samanta Department of Zoology, Panskura Banamali College, P.O. – Panskura R.S., West Bengal PIN 721152, India abhisheksamanta1234@gmail.com Paban Kumar Chaudhuri Paban Kumar Chaudhuri Thalassaemia Control Unit, School of Tropical Medicine, Kolkata, India stm.tcu@gmail.com Ushnish Das Ushnish Das Statistical Programming and Analysis Division, Tata Consultancy Services, Puna, India ushnish.ushnish@gmail.com Nandan Bhattacharyya Nandan Bhattacharyya Department of Biotechnology, Panskura Banamali College, P.O. – Panskura R.S., West Bengal PIN 721152, India bhattacharyya_nandan@rediffmail.com Erythrocyte indices Thalassemia Screening ## ##

Effect of Probiotics on Salivary Flow Rate and pH in Children with Acute Lymphocytic Leukemia (ALL) During Chemotherapy 2 2 Background: This study was performed to evaluate the effects of probiotics on salivary flow rate and pH in children with Acute Lymphocytic Leukemia (ALL) during chemotherapy. Methods: A randomized clinical trial was conducted on 11 children with ALL on induction and consolidation phases of chemotherapy. At the beginning, clinical examination of the oral mucosa and dental hygiene and interview regarding the presence of dry mouth were performed. Saliva samples were collected in the morning between 09.00-12.00 a.m., before and after 7 and 14 days gargling with probiotics. Each participant was instructed to gargle probiotics for 60 seconds, morning and night, for 14 days. Descriptive analysis was performed to compare salivary flow rate and salivary pH before and after 7 to 14 days gargling, using Paired t-test because the data is normally distributed (P<0.05). Results: 7 (63.6%) out of 11 patients complained of a dry mouth sensation. Before gargling probiotics, salivary flow rate and salivary pH were 0.52 and 6.8, respectively. After 14 days of gargling with probiotics, there was a significant increase in salivary flow rate to 0.64 (P<0.05), while salivary pH changed unsignificantly to 6.99 (P>0.05). Conclusion: Gargling with probiotics in children with ALL during induction and consolidation chemotherapy phase, after 14 days showed a significant increase in salivary flow rate but unsignificant change in salivary pH. 119 124 2020/11/162020/11/14 1399/8/24 2021/11/292021/10/25 1400/8/3 Tri Damayanti Tri Damayanti Postgraduate Program, Department of Pediatric Dentistry, Faculty of Dentistry, Universitas Indonesia, Jakarta 10430, Indonesia yanthi_dhedhe@yahoo.com Margaretha Suharsini Margaretha Suharsini Department of Pediatric Dentistry, Faculty of Dentistry, Universitas Indonesia, Jakarta 10430, Indonesia m.suharsini@ui.ac.id Sarworini Budiardjo Sarworini Budiardjo Department of Pediatric Dentistry, Faculty of Dentistry, Universitas Indonesia, Jakarta 10430, Indonesia sarworini_b@ui.ac.id Probiotics Salivary flow rate Salivary pH ALL Chemotherapy Mucositis ## ##

Thrombophilia, Anticoagulant Therapy, and Pregnancy Outcome in Women with Poor Obstetric History 2 2 Background: The role of anticoagulant medications in preventing placental mediated pregnancy complications in patients with and without thrombophilia has not been investigated well. One underlying cause is associated with adverse effects of anticoagulants in pregnancy including teratogenicity, complexities in dosing and management of anticoagulants during pregnancy and labor. We aimed to assess effects of prophylactic anticoagulant medications in pregnant women with history of the PMPCs who were tested for hereditary thrombophilia. Methods: This retrospective cohort study was done in obstetric clinics of Tehran University of Medical Sciences on medical records of 148 pregnant women with history of poor obstetric outcome due to placental complications. Pregnant women with both positive and negative thrombophilia test results were included in the study. They were divided into two group according to receiving anticoagulants. Results: 148 patients were analyzed over 1.5 years. Among them, 85 women received anticoagulant medications and 63 did not receive these treatments for the next pregnancy. Moreover, 58 out of 148 pregnant women were thrombophilic according to positive tests. Successful pregnancy outcomes were significantly higher in treated groups. The risk of abortion and unsuccessful pregnancy was significantly reduced in the treated groups. The occurrence of intrauterine fetal death (IUFD), intrauterine growth retardation (IUGR), and preeclampsia were not reduced. The use of anticoagulant during pregnancy did not have any adverse effects. The results in thrombophilia group and non-thrombophilia group demonstrated the benefit of anticoagulant therapy in improvement of pregnancy outcomes. Conclusion: Testing for inherited thrombophilia in women who have experienced placental mediated pregnancy complications is not recommended. Anticoagulant therapy can be useful in women without thrombophilia and with poor obstetric history because of placental mediated pregnancy complications. 125 130 2020/11/162020/11/142021/04/1 1400/1/12 2021/11/292021/10/252021/12/26 1400/10/5 Marziyeh Ghalamkari Marziyeh Ghalamkari Iran University of Medical Science, Tehran, Iran dr.ghalam@yahoo.com Gholamreza Toogeh Gholamreza Toogeh Thrombosis Hemostasis Research Center, Tehran University of Medical Science, Tehran. Iran gh_toogeh@yahoo.com Sedigheh Hantoushzadeh Sedigheh Hantoushzadeh Maternal, Fetal, and Prenatal Research Center, Tehran University of Medical Science, Tehran, Iran hantoushzadeh@yahoo.com Habibeh Yekehtaz Habibeh Yekehtaz Rollins School of Public Health, Emory University, United states of America habibeh.yekehtaz@emory.edu Nader Safarian Nader Safarian 2Thrombosis Hemostasis Research Center, Tehran University of Medical Science, Tehran. Iran nsafarian@gmail.com Mohsen Esfandbod Mohsen Esfandbod 2Thrombosis Hemostasis Research Center, Tehran University of Medical Science, Tehran. Iran mesfandbod@yahoo.com Thrombophilia Anticoagulant therapy Placental mediated pregnancy complications Pregnancy outcomes Poor obstetric history ## ##

Iranian Pediatric Hematology and Oncology Fellowship Curriculum: Updated Version 2 2 Background: Educational evaluation is a broad concept that is related to all elements of the educational system. This concept is the result of the interaction of all values that are implemented with different titles and forms inside and outside the educational system to increase the performance of the educational system. The field of pediatric hematology and oncology is one that is constantly evolving due to extensive and numerous researches in various fields. These changes must be in line with changes in the health care delivery system. In this study, among the decision models, the CIPP model which is an evaluation model for curriculum evaluation given by Stufflebeam in 1983 which includes four elements: C- Context, I- Input, P- Process and P- Product., was selected to evaluate the educational curriculum of Iranian pediatric hematology and oncology fellowship. Methods: The present study has two quantitative and qualitative aspects and a quantitative cross-sectional, descriptive-analytical study. This analysis was conducted in 2021 by the strategic group of the Iranian pediatric hematology and oncology association. Its statistical population consisted of members of this association. Most members have a degree in pediatric hematology and oncology. The research was conducted by census method. Data were collected using a researcher-made questionnaire. In general, the training course was examined in 4 areas of education and research, hardware facilities of the training and current environment, and professional abilities other than the content of the course. The efficacy of the evaluation questionnaire of the Pediatric hematology and oncology fellowship course was a combination of open and closed questions based on the “Kirk Patrick evaluation model”. This questionnaire had 20 questions. The internal evaluation based on Cronbach’s alpha was 0.92. The items surveyed in the questionnaire were: learning modern medical principles such as evidence-based medicine and clinical reasoning, learning the principles of medical ethics, study skills, understanding of legal procedures, ability to electronically research and adequacy of educational subjects. Satisfaction of the faculty members, students ‘satisfaction and the need for supplementary courses, the current method of evaluating students’ communication skills, the scientific ability of the eligible faculty in teaching current topics and participation in educating the students were among the other items of the questionnaire. Results: In the internal validity study, Cronbach’s alpha coefficient of 0.92 was obtained for the current situation and 0.96 for the optimal situation. Descriptive statistics (mean and standard deviation) and one-group and independent t-test were used to analyze the data. Findings indicated that there was a significant difference between the current and desired status of free and absentee university exams in the areas of purpose, design, implementation, modification and feedback and the three components of each of these axes. According to the obtained results, changing the current educational curriculum of the subspecialty fellowship in pediatric blood and cancer is necessary and inevitable. Conclusion: Corrective suggestions for writing a new curriculum in accordance with modern sciences and medical needs of the country were extracted and applied in the new curriculum. 131 139 2020/11/162020/11/142021/04/12021/12/31 1400/10/10 2021/11/292021/10/252021/12/262021/12/10 1400/9/19 Babak Abdolkarimi Babak Abdolkarimi Department of Pediatrics, Lorestan University of Medical Sciences, Khoramabad, Iran b.abdolkarimi@yahoo.com Hassan Abolghasemi Hassan Abolghasemi Department of Pediatrics, Baghyatallah University of Medical Sciences, Tehran, Iran h.abolghasemi.ha@gmail.com Mohammad Faranoush Mohammad Faranoush 3Pediatric Growth and Development Research Center, Iran University of Medical Sciences, Tehran, Iran Peyman Eshghi Peyman Eshghi Department of Pediatrics, Shahid Beheshti University of Medical Sciences, Tehran, Iran Shahin Shamsian Shahin Shamsian Department of Pediatrics, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mahdi Shahriari Mahdi Shahriari Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran Bijan Keikhaei Bijan Keikhaei Thalassemia and Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Nader Momtazmanesh Nader Momtazmanesh Department of Pediatrics, Shahid Beheshti University of Medical Sciences, Tehran, Iran Arash Alghasi Arash Alghasi Thalassemia and Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Fatima Malek Fatima Malek Department of Pediatrics, Shahid Beheshti University of Medical Sciences, Tehran, Iran Educational curriculum Pediatric hematology and oncology fellowship Educational program Medical education ## ##

TdT Negative Lymphoblastic Lymphoma: A Case Report 2 2 Acute lymphoblastic leukemia/lymphomas are tumors made of the precursor B or T cells. Precursor B cell type presents as acute leukemia, and most cases of pediatric leukemia are of this type. Malignancies originated from T cells are less common and are often manifested as lymphoma in adolescents. Terminal deoxynucleotidyl tranferase (TdT) is a DNA polymerase that is present in immature pre-B and pre-T cells. TdT enzyme is a sign of cell immaturity and is used to differentiate the dominant types of lymphoblastic lymphoma from mature lymphoma/leukemia cells. TdT is expressed in 90%–95% of lymphoblastic lymphoma cells. Childhood TdT negative lymphoblastic leukemia/lymphoma is very infrequent and its prognostic significance remains challenging. It is suggested that TdT negative lymphoblastic leukemia/lymphoma shows poor response to chemotherapy and has a more disastrous course. Here, we report a case of TdT negative T-cell lymphoblastic lymphoma in a 10-year-old boy who presented with respiratory distress. 140 143 2020/11/162020/11/142021/04/12021/12/312021/02/6 1399/11/18 2021/11/292021/10/252021/12/262021/12/102022/01/2 1400/10/12 Fariba Binesh Fariba Binesh Department of Pathology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran binesh44@yahoo.com Alireza Jenabzadeh Alireza Jenabzadeh Hematology and Oncology Research Center, Shahid Sadoughi University of Medical Sciences and Health Services, Yazd, Iran emamrezamashhad@gmail.com Mohammad Ali Dideban Mohammad Ali Dideban Department of Pathology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran akhond1362@gmail.com Najmeh Beheshti Najmeh Beheshti Department of Pathology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran mojahedmasudah@gmail.com Fatemeh Khaleghi Fatemeh Khaleghi Department of Radiology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran jarya931@gmail.com Seyed Hossein Shahcheraghi Seyed Hossein Shahcheraghi Infectious Diseases Research Center, Shahid Sadoughi Hospital, Shahid Sadoughi University of Medical Sciences, Yazd, Iran shahcheraghih@gmail.com Terminal deoxynucleotidyl tranferase (TdT) Acute lymphoblastic leukemia/lymphomas TdT negative T cell malignancy Children ## ##

Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) Syndrome in an 8-Year-Old Girl with Ganglioneuroma and SARS-COV-2 infection: A Case Report and Literature Review 2 2 Background: ROHHAD syndrome (rapid-onset obesity, hypoventilation, hypothalamic dysfunction, and autonomic dysregulation) is an extremely rare disease in children. This article describes the clinical features, laboratory findings, imaging results, and treatment of a case of ROHHAD syndrome with ganglioneuroma. Case Report: An 8-year-old girl in Covid-19 pandemic was admitted to our emergency department due to dyspnea, cyanosis, and respiratory distress. She had a history of close contact with his father (a case of covid 19) prior to hospital admission. Spiral chest CT scan of the patient revealed bilateral ground-glass opacities and also a right paravertebral mass. She had a history of rapid onset morbid obesity that had started 2 years ago. Furthermore, she had sleep disturbances and hypoventilation, electrolyte imbalance, polyuria and enuresis, irritability, and impulsivity. She was diagnosed as a case of ROHHAD syndrome based on her clinical manifestations. She was also diagnosed as a case of SARS-COV-2 infection. The paravertebral mass was resected which pathology was consistent with ganglioneuroma. Conclusion: Because of the high prevalence of morbidity and mortality in ROHHAD syndrome and accompanying neural crest tumors, early diagnosis of this syndrome should be kept in mind. It is essential to pay attention to the cases with rapid onset obesity associated with hypoventilation to prevent severe complications. 144 147 2020/11/162020/11/142021/04/12021/12/312021/02/62021/07/27 1400/5/5 2021/11/292021/10/252021/12/262021/12/102022/01/22021/09/4 1400/6/13 Ghamartaj Khanbabaei Ghamartaj Khanbabaei Pediatric pulmonologist, Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. khanbabaeegh@yahoo.com Seyed Ahmad Tabatabaei Seyed Ahmad Tabatabaei Pediatric pulmonologist, Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. ahmadtabatabaii@gmail.com Nazanin Farahbakhsh Nazanin Farahbakhsh Pediatric pulmonologist, Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. nazanin26farahbakhsh@yahoo.com Seyedeh Zalfa Modarresi Seyedeh Zalfa Modarresi Pediatric Pulmonology Fellowship, Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. dr.modarres@gmail.com Matin Poorghasem Matin Poorghasem Pediatric Pulmonology Fellowship, Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. matinpourghasem@yahoo.com Ebrahim Tabiei Ebrahim Tabiei Pediatric Pulmonology Fellowship, Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. asndr370780@yahoo.com Abdolhamid Taghizadeh Abdolhamid Taghizadeh Pediatric Pulmonology Fellowship, Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. hamidt1000@gmail.com Morteza Managheb Morteza Managheb Anesthesiologist, Fellowship of cardiac Anesthesia, Erfan Niayesh Hospital. Tehran, Iran mmanagheb@yahoo.com Hosein Behzad Moghadam Hosein Behzad Moghadam Pediatrician and Neonatologist, Neonatal and NICU ward, Erfan Niayesh hospital, Tehran, Iran mhbmoghadam@yahoo.com Kourosh Vahidshahi Kourosh Vahidshahi Associate Professor of Pediatric Cardiology, School of Medicine, Modarres Teaching Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran kvahidshahi@gmail.com Seyed Morteza Tabatabaei Yazdi Seyed Morteza Tabatabaei Yazdi Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran saminalavi@hotmail.com Hossein Taheri Hossein Taheri Student Research Committee, Shahid Beheshti University of Medical Sciences, Tehran, Iran h.taheri98@yahoo.com ROHHAD syndrome Rapid-onset obesity Hypoventilation Ganglioneuroma Covid 19 ## ##

A Pregnant Woman with Factor V Deficiency 2 2 Factor V deficiency and other rare bleeding disorder are scarcity and orphan bleeding disorders. Thus, general physicians may rarely encounter with these disorders in clinical practice. If we do not treat them satisfactorily, the hemorrhagic propensity of these diseases may be fatal. Rarity and distribution of affected patients in all countries in the world is one of problem in their clinical practice. In reality, the distribution of small number and various type of coagulation factor concentrates for rare bleeding disorders in a wide country is troublesome in practice for health provider systems. 148 148 2020/11/162020/11/142021/04/12021/12/312021/02/62021/07/272020/08/4 1399/5/14 2021/11/292021/10/252021/12/262021/12/102022/01/22021/09/42022/01/2 1400/10/12 Hassan Mansouritorghabeh Hassan Mansouritorghabeh Immunology Research Center, Inflammation and Inflammatory Disease Division, Mashhad University of Medical Sciences, Mashhad, Iran mansouritorghabeh@mums.ac.ir Factor V deficiency Recombinant factor V concentrarte ## ##

Hemophilic Pseudotumor of Humerus in an Adolescent with Severe Hemophilia A 2 2 Repeated bleeding into the joints often occurs in the first decade of life in patients with hemophilia. Joint degeneration is progressive, and although early treatment can slow the process, destruction of the joint is unavoidable. Hemophilic pseudotumors (HPT) are developed due to recurrent bleeding from extra-articular bone or soft tissues. 149 150 2020/11/162020/11/142021/04/12021/12/312021/02/62021/07/272020/08/42022/02/13 1400/11/24 2021/11/292021/10/252021/12/262021/12/102022/01/22021/09/42022/01/22021/12/10 1400/9/19 Soha Mohammadi Moghaddam Soha Mohammadi Moghaddam Pediatric Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mitra Khalili Mitra Khalili Pediatric Radiology Department, Shahid Beheshti University of Medical Sciences, Tehran, Iran Behnaz Habibpanah Behnaz Habibpanah Pediatric Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Hemophilic Pseudotumor Severe Hemophilia A ## ##