Lower Respiratory Infections in Children With Febrile Neutropenic Leukemia: A Case in a Jordanian Hospital.

The study aimed to examine the prevalence of pneumonia in pediatric children diagnosed with leukemia at King Hussein Medical Center, Royal Medical Services, Amman, Jordan. The study was conducted from January 2019 to March 2020. A total of 100 hospitalized leukemia patients with febrile neutropenic episodes were evaluated for the presence of pneumonia. Samples were collected from all patients and tested for microbial growth. Univariate analysis revealed that age (P = .033) and packed cell volume (P = .006) were statistically significant risk factors, associated with the prevalence of pneumonia in leukemia patients with febrile neutropenia episodes. Similarly, as the absolute neutrophil count counts increased with an odds ratio and a 95% confidence interval of 2.386 (0.859-6.625), the odds of pneumonia in febrile neutropenic patients were more prevalent. The study reported the prevalence of pneumonia in immunocompromised febrile neutropenic patients with leukemia, which could lead to the development of evidence-based febrile neutropenic treatment protocol development. It will assure more responsive patient management and treatment.

Epidemiology of human brucellosis in military hospitals in Jordan: A five-year study.

INTRODUCTION: Brucellosis is a zoonotic disease with significant impacts on livestock and human health. It is a severe community health burden in the Middle East with an estimated 2000 times higher prevalence than in North America and Western Europe. To date, there are limited studies on human brucellosis and risk factors for infection in Jordan. Our study aimed to analyze documented brucellosis cases in Jordan and use geographic and socio-demographic data to better understand its prevalence and transmission. METHODOLOGY: This retrospective study examined electronic medical records describing 1,497 cases of febrile illness that were tested for brucellosis at Royal Medical Services hospitals between 2016 and 2020. A total of 465 confirmed brucellosis cases, aged 0 to 80 years were included. Serum samples were screened for anti-Brucella antibodies, and positive samples were additionally tested for antibody titer using the Wright tube agglutination test. RESULTS: Our results showed that 31.1% (456/1497) of the febrile diseases were brucellosis. We found that young adults and working age, northern governorates, rural location of residency, occupations involving regular contact with livestock, and Spring/Summer season were highly significant as risk factors. Seropositivity was highest among dairy factory workers with 64.4%. CONCLUSIONS: This study adds to our understanding of human brucellosis in Jordan and its impact on public health. These data will be useful in the prevention of brucellosis and will inform reliable disease control policies.

Sickle Cell Disease in Jordan: The Experience of a Major Referral Center.

INTRODUCTION: Sickle cell disorders are the most frequently encountered hemoglobin variants in Jordan. Both alpha and beta thalassemias are also prevalent in this population. However, studies on the interaction between these hemoglobin disorders are lacking. AIM: To determine the genotypes responsible for Sickle cell disease in Jordan, by retrospectively reviewing the data from a major referral center in the country's capital. METHODS: A total 29,712 peripheral blood samples referred and investigated for hemoglobinopathies over a 10-year period at Princess Iman Center at Amman, Jordan were retrospectively reviewed. In addition to full blood counts, high performance liquid chromatography, those who were identified with sickle cell hemoglobin were studied using polymerase chain reaction and reverse hybridization to determine the various sickle cell disease genotypes. RESULTS: Out of the (29,712) blood samples, 450 were sickle cell trait, while 216 had sickle cell disease. Of the latter: 120 were found to be cases of Sickle cell anemia (Hb SS), 66 were compound heterozygous for Sickle cell and a beta thalassemia mutation (Sickle/ß-thalassemia), while 30 had concomitant alpha thalassemia (HbSS/alpha thalassemia). The most frequent genotype associated with sickle/ß-thalassemia was HbS/ IVS-110 (G>A), followed by Hb S/IVS-I-6 (T>C), HbS/IVS-II-745 (C>G) and HbS/ IVS-II-1 (G>A). While the most frequent alpha genotype detected in HbSS/α-thalassemia samples was (-α3.7/αα) followed by (-α3.7/-α3.7). Hb SS patients had the severest hematological phenotype compared to those with sickle/ß-thalassemia and sickle/ α-thalassemia. Furthermore, within the sickle/ß-thalassemia subgroup the least severe hematological phenotype was encountered in HbS/IVS-1-6 (T>C), while the most severe in HbS/IVS-II-1 (G>A) genotype. CONCLUSION: The most frequent Sickle cell disease genotype in Jordanians is Sickle cell anemia (HbSS), followed by Sickle/ß-thalassemia and least frequent is HbSS/alpha thalassemia. The concomitant identified thalassemia mutations were consistent with their spectrum among the Jordanian population.

Prevalence of FVIII Inhibitors Among Children with Hemophilia A: Experience at the Jordanian Royal Medical Services.

INTRODUCTION: Replacement therapy is constantly required by Hemophilia A (HA) patients lacking coagulation factor VIII (FVIII). The most serious complication of this treatment is the development of neutralizing antibodies (inhibitors). AIM: The aim of this study is to determine the frequency of FVIII inhibitors among children treated for HA at the Jordanian Royal Medical Services. METHODS: A total of 165 diagnosed HA patients receiving on-demand treatment, were tested for FVIII inhibitors between 2003 and 2018. The age range was 6 months to 16 years. Coagulation and inhibitor screening assays were performed, followed by Bethesda assay for inhibitor-positive samples to quantify FVIII inhibitor titers. RESULTS: Out of the 165 patients, 111 had severe hemophilia with FVIII level < 1%, 26 had moderate hemophilia with FVIII levels of 1-5% and 28 had mild hemophilia with FVIII levels of > 5%. Twenty patients had FVIII inhibitors, of whom 18 had high titers, 2 had low titers. The mean inhibitor level in low (titer) responders was 2.40 ± 0.85 BU, as opposed to 116.25 ±169.25 BU in high (titer) responders. In terms of disease severity, 18 of the 20 patients with FVIII inhibitors had severe HA, whereas two had moderate HA. No inhibitors were encountered in the mild HA group. CONCLUSION: Inhibitors only developed in moderate and severe cases of HA. The severity of the disease and age were the main contributing factors. The association between family history of inhibitors and the incidence of inhibitor formation warrants genetic evaluations to look for relevant mutations.

Hematopoietic Stem Cell Transplantation in Thalassemia Patients: a Jordanian Single Centre Experience.

INTRODUCTION: Beta thalassemia major is the commonest inherited hematological disorder worldwide which needs lifelong sufficient supportive management. Hematopoietic stem Cell transplantation (HSCT) is the only curative treatment available till now. AIM: To evaluate the outcome of children who underwent allogenic hematopoietic stem Cell transplantation as a curative approach for Thalassemia Major, treated at Queen Rania AL- Abdullah children Hospital (QRCH). METHODS: A retrospective review of the medical files was conducted for all children (< 15 years) who had thalassemia major and received HSCT between January, 2010 and January, 2019. The following variables were studied for all patients: age , gender, Pesaro classifications, the count of infused raw bone marrow stem cell (CD34), engraftment time, outcome and complications. RESULTS: A total of 34 children were transplanted for thalassemia major, at an average of 4 cases per year. All underwent allogenic raw bone marrow transplantation from matched related donors. Thirteen patients (38.2%) were males and twenty one (61.2%) were females. The age ranged between 2 and 15 years, with a median age of 6.5 years. According to Pesaro classification, 31 patients were class 2 (91.2%) and 3 patients were class 3 (8.8%) while no single case met the criteria for class 1 Pesaro classification. The median CD34 count was 3.5 million/Kg of recipient weight (range, 1.5*106-7*106 /kg). The median time for neutrophil engraftment was 15.5 days. At a median follow up of 5 years (range 1- 9.5), 33 patients were alive. One patient died before 100 days post transplantation due to grade IV acute gastrointestinal Graft Versus Host Disease (GVHD). Three patients had secondary graft failure (8.8%). Six patients (17.5 %) developed mild grade 1-2 skin GVHD while another patient developed hemorrhagic cystitis due to BK virus and cytomegalovirus (CMV) which reactivated simultaneously, and was successfully managed. CONCLUSION: The outlook for Thalassemia major has dramatically changed after HSCT, with a considerable success in Jordan and results comparable to international data.