Glomerular and Tubular Functions in Children and Adults with Transfusion-Dependent Thalassemia

This study aimed at assessing renal functions in patients with transfusion-dependent thalassemia (TDT). Fifty patients and 30 controls were enrolled in this prospective study. Serum levels of electrolytes and albumin were measured by a spectrophotometer. Serum levels of cystatin-C and urinary levels of β2-microglobulin were measured by nephelometric method. Thirty-eight patients were receiving deferasirox and 8 were on deferiprone. Serum electrolytes and albumin levels of the patients were found to be within normal ranges. Urinary β2-microglobulin and serum cystatin-C levels were significantly higher in patients than controls. They did not significantly differ between the subgroup of patients on deferiprone and the control group, whereas they were found to be higher in patients using deferasirox compared to controls. Urinary β2-microglobulin levels significantly increased in patients who were receiving high-dose deferasirox compared to those who were receiving a daily dose of 15-20 mg/kg or controls. Subclinical renal injury may be present in TDT patients

THE TOTAL ANTIOXIDANT CAPACITY MAY NOT BE RELATED TO BILIRUBIN AND URIC ACID LEVEL IN PATIENTS WITH BETA THALASSEMIA

Objective: Iron burden resulting from ineffective erythropoiesisand multiple transfusions can cause oxidative stress in patientswith ß-thalassemia. Here we aimed to examine the total antioxidant and oxidant capacity (TAC and TOC) along with its relationto endogenous antioxidants (bilirubin and uric acid) in patientswith ß-thalassemia.Material and Method: Forty-five patients with transfusion-dependent (TDT) (n=30) and non-transfusion-dependent (NTDT)(n=15) ß-thalassemia and 20 healthy subjects were enrolled inthe study. Analyses were done using Total Antioxidant Status(TAS) and Total Oxidant Status (TOS) kits.Results: The TAC level of the patients was significantly increasedcompared to healthy subjects (2.75 vs. 2.10 mmol/L; p=0.01).The total bilirubin level was significantly elevated in NTDT patients compared to TDT patients (5.7±3.3 vs.1.9±1.4; p<0.001).No significant relationship between endogenous antioxidantsand total antioxidant capacity of patients was detected (p=0.20)Conclusion: The total antioxidant capacity of patients withß-thalassemia might not be directly related to endogenous anti-oxidative status

THE TOTAL ANTIOXIDANT CAPACITY MAY NOT BE RELATED TO BILIRUBIN AND URIC ACID LEVEL IN PATIENTS WITH BETA THALASSEMIA

Objective: Iron burden resulting from ineffective erythropoiesis and multiple transfusions can cause oxidative stress in patients with beta-thalassemia. Here we aimed to examine the total antioxidant and oxidant capacity (TAC and TOC) along with its relation to endogenous antioxidants (bilirubin and uric acid) in patients with beta-thalassemia

Sustained hyperferritinemia in a child with macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis - perforinopathy: case based review

Systemic juvenile idiopathic arthritis is a subtype of juvenile idiopathic arthritis and characterized by arthritis and many systemic features like fever, rash, hepatosplenomegaly, lymphadenopathy and serositis. Macrophage activation syndrome is the most dreadful complication of systemic juvenile idiopathic arthritis and can cause mortality and morbidity if not recognized and treated early and aggressively

Mortality risk factors among critically ill children with MIS-C in PICUs: a multicenter study

Background: This study evaluated of clinical characteristics, outcomes, and mortality risk factors of a severe multisystem inflammatory syndrome in children admitted to a the pediatric intensive care unit. Methods: A retrospective multicenter cohort study was conducted between March 2020 and April 2021 at 41 PICUs in Turkey. The study population comprised 322 children diagnosed with multisystem inflammatory syndrome. Results: The organ systems most commonly involved were the cardiovascular and hematological systems. Intravenous immunoglobulin was used in 294 (91.3%) patients and corticosteroids in 266 (82.6%). Seventy-five (23.3%) children received therapeutic plasma exchange treatment. Patients with a longer duration of the PICU stay had more frequent respiratory, hematological, or renal involvement, and also had higher D-dimer, CK-MB, and procalcitonin levels. A total of 16 patients died, with mortality higher in patients with renal, respiratory, or neurological involvement, with severe cardiac impairment or shock. The non-surviving group also had higher leukocyte counts, lactate and ferritin levels, and a need for mechanical ventilation. Conclusions: In cases of MIS-C, high levels of D-dimer and CK-MB are associated with a longer duration of PICU stay. Non-survival correlates with elevated leukocyte counts and lactate and ferritin levels. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality. Impact: MIS-C is a life-threatening condition.Patients need to be followed up in the intensive care unit.Early detection of factors associated with mortality can improve outcomes.Determining the factors associated with mortality and length of stay will help clinicians in patient management.High D-dimer and CK-MB levels were associated with longer PICU stay, and higher leukocyte counts, ferritin and lactate levels, and mechanical ventilation were associated with mortality in MIS-C patients.We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality