Background: Thalassemia is the most common inherited anemia in worldwide. Heart failure is the most common cause of mortality and morbidity in patients with major and intermedia β-thalassemia. This study aimed to evaluate the effect of oral administration of L-arginine on the improvement of systolic Pulmonary Artery Pressure (PAP) and cardiac function in patients with major and intermedia β-thalassemia. Methods: This randomized clinical trial was done on 88 patients with β-thalassemia admitted to Ali Asghar Hospital, Tehran, Iran between 2020 and 2021. Echocardiography was performed for all the patients before the intervention. Afterwards, the patients were randomly divided into two groups of placebo and L-arginine. The patients underwent echocardiography after eight weeks and were compared with respect to the results. Results: The mean blood transfusion interval was 20.21 d in the placebo group and 17.14 d in the L-arginine group (P=0.082). The results revealed no significant difference between the two groups regarding the mean levels of Hemoglobin (Hb) and ferritin, frequency of splenectomy. However, the mean PAP significantly decreased from 32.88 to 26.02 in the L-arginine group (P=0.009), but did not change in the placebo group. Nonetheless, no significant change was observed in the mean Ejection Fraction (EF) before and after L-arginine administration. Conclusion: L-arginine administration prevented the increase of PAP and was effective in preventing cardiovascular disorders including increased systolic PAP in patients with major and intermedia B-thalassemia. However, the results have to be confirmed in further studies with larger sample sizes.

Background: Patients with beta-thalassemia (BT) are more prone to infections than others, especially when they have some risk factors. Some reports showed the lower severity of Coronavirus disease 2019 (COVID-19) infection in this population. Objectives: In this study, we aimed to determine the serological status of COVID-19 in BT patients. Methods: This cross-sectional study was conducted in September 2020-February 2021 at Ali-Asghar Children's Hospital, Iran. All major and intermedia β-thalassemia patients dependent on blood transfusion were enrolled in this study. Demographic information, including age, gender, thalassemia type, history of splenectomy, and blood type, were recorded in a checklist. Moreover, the patients were asked about their exposure to COVID-19, and the related symptoms, such as cough, fever, headache, and anosmia, in the last six months. The enrolled cases were examined for anti-COVID-19 IgM and IgG to determine the serological status against this virus. Results: The positive IgG and IgM tests against COVID-19 were in 35.7% (25/70) and 20% (14/70) of cases, respectively, while 42.9% (30/70) were either IgM- or IgG-positive. Among the serologically positive cases, six had a history of at least one symptom suspicious of COVID-19. These six included one fever, two coughs, one anosmia, one dyspnea, and one cough and anosmia. Only one of the patients was hospitalized (1%) with fever and dyspnea. No Intensive Care Unit admission or mortality was reported. Conclusions: It seems that COVID-19 is more likely to be mild or asymptomatic in patients with BT.

Factor VII deficiency is a rare congenital coagulopathy disorder. In most cases, this disorder is diagnosed in childhood. Common symptoms of congenital factor VII deficiency are different and consist of cutaneous, mucosal hemorrhage, gastrointestinal bleeding, and joint bleeding. CNS hemorrhage is a fatal and severe complication of congenital factor VII deficiency. The incidence of gross hematuria is a rare symptom of factor VII deficiency. Isolated presentation of hematuria is rarer and usually is accompanied by bleeding in other sites. The patient reported here is a 6-month-old girl who was diagnosed with congenital Factor VII deficiency following episodes of isolated gross hematuria. We decided to report this case to demonstrate if there is no other organic cause in the investigation of a child with recurrent hematuria, we should also consider a coagulation factors deficiency. Since isolated hematuria is a rare symptom in the coagulation factors deficiency, the coagulation tests may be of less interest.

Background and Objective: Beta-thalassemia major is an important health problem in Iran. A high rate of psychiatric disorders has been reported in patients with thalassemia in different surveys and may be one of the reasons that cause problems in the quality of their sleep. Although sleep quality is important for the health and quality of life (QOL) of these patients, a limited study in Iran on sleep quality and its relation to the mental health status of these patients has been conducted so far. Materials and Methods: A total of 101 patients with thalassemia were assessed. Data were collected via a demographic information checklist, the Pittsburgh Sleep Quality Index (PSQI), and the 28-item General Health Questionnaire (GHQ-28). Results: Participants in this study had an average age of 25.90 ± 5.63 years and 71 (70.30%) of them were women. Seventy-seven patients (76.2%) suffered from poor sleep quality while the mean and standard deviation (SD) of the patients' total general health score (31.66 ± 11.35) was good at the general health level. Among the demographic factors, there was a significant relationship between job and sleep quality. Sleep quality, subjective sleep quality, sleep delay, sleep duration, sleep disorders, use of sleeping pills, and daily functional disorders had a positive and significant relationship with general health. Conclusion: Thalassemia reduces the quality of sleep. There is a positive and significant association between sleep quality and general health, meaning that by improving sleep quality, patients have a greater chance of improving their general health.