Numerous problematic disorders such as vitamin D (Vit-D) deficiency subsequent to large iron loading can be developed in patients with β-thalassemia. The study aimed to estimate Vit-D insufficiency and its risk factors in patients with β-thalassemia. In this multicenter and observational study, all β-thalassemia patients, who referred to 14 hospital-based thalassemia divisions or clinics in Mazandaran province, Iran were included in the study. The data belong to December 2015 until December 2019. The study population was made of transfusion dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT) patients. Serum levels of 25-OHD3 have been measured by high performance liquid chromatography (HPLC) method as ng/mL. Demographic and clinical information along with some biological tests, as well as the results of T2*-weighted magnetic resonance imaging were analyzed. Of 1959 registered patients, 487 (24.9%) patients had Vit-D-related data. The prevalence of Vit-D insufficiency (< 30 ng/mL) was 41.9, 95% CI 37.5–46.3. The adjusted risks of moderate to severe liver siderosis and raised AST (aspartate aminotransferase) for Vit-D insufficiency (< 30 ng/mL) were 2.31, 95% CI 1.38–3.89 and 2.62, 95% CI 1.43–4.79, respectively. The receiver operating characteristic (ROC) curve analysis showed that the predictive accuracy of ferritin for Vit-D insufficiency status was 0.61, 95% CI 0.54–0.68 with a cutoff point of 1,078 ng/mL (P = 0.03, sensitivity 67%, specificity 49%, positive predictive value [PPV] 47% and negative predictive value [NPV] 68%). In spite of the national programs for treating Vit-D deficiency and our previous efforts for giving supplements to all patients, Vit-D insufficiency/deficiency is still common in our patients. Also, moderate to severe liver siderosis and raised AST were the independent risk factors for the Vit-D insufficiency.
One of the most common nutritional deficiencies worldwide is iron deficiency. Fatigue, pallor, vertigo, dyspnea, cold intolerance, lethargy, palpitation, headache, and the pallor of the mucous membranes or nail beds are the most frequent symptoms and signs of iron deficiency. Thrombocytosis is commonly observed in iron deficiency anemia; it seems that erythropoietin plays the main role in this respect. Furthermore, thrombocytopenia and even leukopenia have been reported in iron deficiency; however, pancytopenia is a very rare condition. In this report, we presented two unusual cases of pancytopenia due to severe iron deficiency that improved after treatment with oral iron supplements. Iron deficiency anemia, if sufficiently severe, may be associated with reduced platelet and leukocyte counts. Accordingly, this condition should be considered as a differential diagnosis in all patients with pancytopenia.
Introduction: Germ cell tumors account for 3.5 % of pediatric tumors and yolk sac tumors are the most common pure malignant germ cell tumors in children. Testis and ovary are the most common sites of involvement in children older than 2 years. Case Presentation: A 20-month-old boy with a complaint of limping was described in this case report. Abdominal ultrasonography showed a 52 × 50 mm solid abdominal mass. Abdominal and pelvic computed tomography scans showed a left paraspinal heterogeneous mass. The tumor was extended into the spinal column with spinal cord compression. Four months later, at the end of chemotherapy, the tumor was completely resolved. Conclusions: Spinal cord compression is a rare presentation of yolk sac tumor (YST) and although this tumor is an uncommon malignancy, it can be cured completely.
Ferritin is frequently used to screen some dire consequences of iron overload in β-thalassemia patients. The study aimed to define the best cutoff point of ferritin to screen for cardiac and liver hemosiderosis in these cases. This was a registry-based study on β-thalassemia patients living throughout Mazandaran province, Iran (n = 1959). In this diagnostic research, the index test was ferritin levels measured by a chemiluminescent immunoassay. As a reference test, T2*-weighted magnetic resonance imaging (T2*-weighted MRI) was applied to determine cardiac and liver hemosiderosis. A cutoff point of 2027 ng/mL for ferritin showed a sensitivity of 50%, specificity 77.4%, PPV 42.1%, and NPV 82.5% for cardiac hemosiderosis (area under curve [AUC] 0.66, 95% CI 0.60–0.71, adjusted odds ratio [OR] 2.05, 95% CI 1.05–4.01). At an optimum cutoff point of 1090 ng/mL, sensitivity 66.7%, specificity 68%, PPV 82.9%, and NPV 46.8% for liver hemosiderosis were estimated (AUC 0.68, 95% CI 0.63–0.73, adjusted OR 3.93, 95% CI 2.02–7.64. The likelihood of cardiac hemosiderosis serum ferritin levels below 2027 ng/mL is 17.5%. Moreover, 82.9% of β-thalassemia patients with serum ferritin levels above 1090 ng/mL may suffer from liver hemosiderosis, regardless of the grades.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the causes of severe hyperbilirubinemia, prolonged jaundice, and bilirubin-induced encephalopathy in neonates. In a randomized controlled trial, we evaluated the effect of oral ursodeoxycholic acid (UDCA) on indirect hyperbilirubinemia in G6PD-deficient neonates requiring phototherapy. Intervention group I (N = 45; received phototherapy and 10 mg/kg/day UDCA), Intervention group II (N = 40; received phototherapy and 20 mg/kg/day UDCA), and a control group (N = 49; received phototherapy and placebo). Levels of total serum bilirubin (TSB) in all 3 groups decreased significantly over time ( P = .001) but the level of TSB at different hours after admission and the duration of phototherapy did not differ significantly between the 3 groups. After discharge, the 2 intervention groups had a significantly lower rate of readmission than the control group ( P = .001). No significant difference was observed between the 10 and 20 mg/kg/day groups. Further evaluation is recommended, especially in terms of the pharmacokinetics of UDCA in neonates. Trial registration number: IRCT20091201002801N4, prospectively registered on 2019-06-1
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