Hereditary spherocytosis in an 18-month-old boy with pancytopenia: A case report

Hereditary spherocytosis (HS) is a familial hemolytic disorder with marked heterogeneity of clinical features, ranging from an asymptomatic condition to a fulminant hemolytic anemia. Although a positive family history of spherocytosis increases the risk for this disorder, it may be sporadic in some cases. In severe cases the disorder may be detected in early childhood, but in mild cases it may go unnoticed until later in adulthood. The case was an 18-month-old boy from Sanandaj, Iran with 3 days decreased activity movement, poor feeding, pallor and urine discoloration since 3 days ego following an episode of fever. He was a case of anemia who was managed conservatively on nutritional supplements. Blood film showed 80% spherocytes, reticulocyte was 0.5%, increased osmotic fragility test and a negative direct Coombs

Examination of the psychometric properties of the persian version of the COVID-19-impact on Quality of Life Scale

Introduction As a result of high transmission and mortality rates, the Covid-19 pandemic has led to a worldwide health crisis, isolation, and widespread fear, therefore negatively influencing people's quality of life (QOL). The goal of the present study was to examine the psychometric properties (validity and reliability) of the Persian version of the COVID-19-Impact on Quality of Life (COV19-QoL) scale. Methods After translating the scale using the forward-backward method, face and content validly was qualitatively assessed. Then the scale was distributed to 488 individuals from the general population via online platforms. Construct validity was assessed using exploratory (EFA) and confirmatory (CFA) factor analysis. In addition, internal consistency was examined using Cronbach's alpha coefficient and McDonald's omega, relative stability was assessed using interclass correlation coefficient (ICC), and absolute stability was calculated through examination of standard error of measurement. Results The EFA revealed one factor that explained 55.96% of the total variance of the scale. Internal consistencies of 0.823 and 0.882 were found using Cronbach's alpha coefficient and McDonald's omega, respectively. In addition, an ICC of 0.837 (with a two-week interval) was found. Covid-19 had a greater impact on the QOL of healthy participants than that of those with underling conditions (p = 0.004), and also on the QOL of single participants than that of married ones (p = 0.032). Conclusion The Persian version of the COV19-QoL is a valid and reliable instrument that can be used to examine the impact of Covid-19 on QOL

Correlation between Heart and Liver Iron Levels Measured by MRI T2* and Serum Ferritin in Patients with β-thalassemia Major

Background Transfusion-induced iron overload leads to many complications in patients with β-thalassemia major. This study aimed to compare the level of iron stored in the liver and heart measured by MRI T2 * with ferritin levels in these patients. Materials and Methods This study was done on 52 patients with β-thalassemia major aged 7 to 29 years. Serum ferritin level was checked and heart and liver MRI T2* operation was performed, then the liver and heart iron level measured by MRI T2 * was compared with serum ferritin levels. Results 150-180 cc/kg packed cells were received on average. The mean ± SD serum ferritin level was 2644.8±1988.3 ng/mL. The mean ± SD relaxation times in liver and heart MRI T2* were 4.39±5.8 and 26.59±10.62 millisecond, respectively. A negative significant correlation was found between serum ferritin levels and liver MRI T2* (P0.05). Conclusion Since there was no correlation between serum ferritin levels and cardiac iron levels, accurate assessment of cardiac iron load using MRI T2* is necessary in patients with β-thalassemia major. Serum ferritin level is reliable for assessing liver iron levels

Prevalence of celiac disease in patients with type 1 diabetes: a systematic review and meta-analysis

Background. Celiac disease (CD) is more prevalent among type 1 diabetes (T1D) patients compared to the general population and can be accompanied by hypoglycemia episodes in T1D patients. Studies worldwide have reported CD prevalence with large variability. This study aimed to estimate the pooled prevalence of CD in T1D patients.   Methods. PubMed, Web of Science, Science Direct, and Scopus were searched without time limitation using the keywords: Celiac Disease, Gluten Enteropathy, Gluten- Sensitive Enteropathy, Wheat Hypersensitivity, Tissue Transglutaminase Antibody, Endomysial Antibody Disease, Diabetes Mellitus, and IDDM. Random-effects inverse variance-weighted model, subgroup analysis, and meta-regression were implemented. Heterogeneity was examined using Cochran’s Q test and I2 statistics.   Results. A pooled analysis of 55 articles with total sample size 71,853 revealed that the CD prevalence in patients with T1D was 5.08% (95% CI: 4.44%, 5.73%) with large heterogeneity (I2 = 84%). To account for publication bias, using trim-and-fill method, the pooled prevalence was 4.0% (95% CI: 3.38%, 4.73%). The prevalence of CD was higher in Asia (6.53%, 95% CI: 4.89%, 8.16%) compared to USA (4.89%, 95% CI: 3.85%, 5.93%) and Europe (4.76%, 95% CI: 3.78%, 5.74%). In addition, studies conducted after 2008 reported pooled prevalence (6.37%, 95% CI: 5.25%, 7.49%) significantly higher than those conducted before 2008 (4.14%, 95% CI: 3.19%, 5.09%). Studies with quality score 10 had significantly higher prevalence (7.0%, 95% CI: 5.04%, 8.96%) compared with quality score 7 (3.66%, 95% CI: 2.61%, 4.70%).   Conclusion. CD is highly prevalent in T1D patients. Studies from Asia, those published after the year 2008, and studies with quality score 10 had higher pooled CD prevalence. Therefore, early screening for CD in T1D patients is important to prevent complications of CD