The frequency of synovitis and calcium pyrophosphate deposition with ultrasound is increased in transfusion-dependent beta-thalassaemia patients: effect of iron accumulation?

OBJECTIVES: To determine the frequency of synovitis and calcium pyrophosphate deposition (CPDD) with ultrasound (US) in the wrists of transfusion dependant (TD) beta-thalassaemia patients and to investigate the associated factors with these pathologies. METHODS: Eighty-seven beta-thalassaemia patients (46 thalassaemia major and 41 thalassaemia minor patients) were grouped into two as TD and transfusion non-dependent (TND)-thalassaemia patients. Under bilateral wrist US the presence of synovial hypertrophy (SH), power Doppler signal (PD) combined synovitis (SH+PD), tenosynovitis, and triangular fibrocartilage complex (TFC)-cartilage calcification (CC) were examined. SH, PD, and combined synovitis in the US were classified as Grade-0 (no), Grade-1 (minimal), Grade-2 (moderate), and Grade-3 (severe). RESULTS: The incidence of moderate/severe SH, PD, and combined synovitis with US was 34.8%, 17.4%, and 34.8% in TD-thalassaemia patients, respectively, but none in TND patients (p<0.001, p=0.006, p<0.001). The frequency of TFC-CC with US was 32.6% in TD and 2.4% in TND-thalassaemia patients (p<0.001). Ferritin level was positively correlated with SH (r=0.414, p<0.001), PD (r=0.279, p=0.009) and combined synovitis scores (r=0.402, p<0.001). Ferritin level (OR:1.001, CI:1.000-1.002) and the presence of TFC-CC (OR:25.048, CI:5.187-120.951) were determined as to be associated with moderate/severe combined synovitis. CONCLUSIONS: The presence of synovitis and TFC-CC with the US is common in patients with beta-thalassaemia who have had recurrent blood transfusions. Iron overload in beta-thalassaemia patients may cause CPDD and synovial inflammation.

Mean Platelet Volume and Mean Platelet Volume/Platelet Count Ratio Are Predictors of Late-Onset Sepsis in Preterm Infants: A Case-Control Study.

Objective: Our aim was to assess mean platelet volume (MPV) and mean platelet volume to platelet count ratio (MPR) in the setting of late-onset sepsis (LOS) and their association with the type of bacteria causing LOS. Study design: The MPV and MPR levels were obtained at the onset of LOS and then assessed in intra/inter group analyses in preterm infants. Results: Overall, 136 preterm infants were enrolled. The MPV and MPR levels were higher during a LOS event (P < 0.001). A MPV cutoff of >9.2 was related with a sensitivity of 63% and a specificity of 73% for predicting LOS (P < 0.001). A MPR cutoff of >0.15 was related with a sensitivity of 88% and a specificity of 63% for predicting gram negative LOS (P < 0.001). Conclusion: Elevated MPV values and MPR ratios may be helpful in assessing LOS.

Red Cell Distribution Width to Platelet Count Ratio Reference Intervals in Premature Infants Beyond the First Week of Life.

Objective: Red cell distribution width (RDW) is a parameter of complete blood count (CBC). The RDW to platelet count ratio (RPR) is a new index that has been shown to reflect the severity of inflammation. We aim to determine the reference interval (RI) of RPR for premature newborns. Study design: The medical records of preterm infants who were followed up between January 2016 and December 2018 were reviewed. CBC levels were measured in 144 infants at <72 hours of age. Results: CBCs of infants (gestational age from 28 to 35weeks) had a RI of 0.038-0.126 for the RPR. The RI for RPR in infants with a gestational age of 32-35weeks was 0.042-0.129; and the RI for infants at 28-31weeks was 0.022-0.121. Conclusion: Establishment of RI for RPR in premature infants will allow clinical correlation of RPR alterations in this population.

Evaluation of thiol/disulfide hemostasis and serum Ischemia modified albumin as oxidative stress biomarkers in systemic lupus erythematosus patients: Relationship with major organ involvement and disease activity.

OBJECTIVES: We aimed to compare thiol/disulfide hemostasis and serum ischemia-modified albumin (IMA) levels, which are indicators of oxidative stress (OS), in patients with systemic lupus erythematosus (SLE), with the healthy control (HC) group and to evaluate the relationship of these parameters with disease activity and major organ involvement. MATERIAL-METHODS: Eighty-four SLE patients and 96 HCs were included in this study. The disease activity of SLE patients was calculated using The Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K). Patients with SLEDAI-2K ≤ 5 were classified as low disease activity (LDA) and those with SLEDAI-2K > 6 as high disease activity (HDA). Thiol/disulfide hemostasis was evaluated using a new automated method and natural thiol (NT), total thiol (TT), disulfide (SS) levels, SS/NT, SS/TT, NT/TT ratios, and serum IMA levels were recorded. RESULTS: NT and TT levels were significantly lower (490.11 ± 123.61 vs 536.96 ± 86.05, p = 0.003) (532.56 ± 125.80 vs 565.72 ± 89.82, p = 0.046), SS level (21.22 ± 11.75 vs 13.37 ± 9.31, p < 0.001) was higher, and SS/TT (4.64 ± 2.93 vs 2.52 ± 1.82, p < 0.001) and SS/NT (4.12 ± 2.33 vs 2.35 ± 1.59, p < 0.001) ratios were significantly higher in SLE patients compared to HCs. IMA values were not different between the two groups (p = 0.920). NT (449.84 ± 136.98 vs 520.32 ± 104.11, p = 0.012) and TT levels (492.01 ± 138.45±562.97 ± 107.09, p = 0.013) were significantly lower and serum IMA levels (0.802 ± 0.089 vs 0.764 ± 0.040, p = 0.023) were significantly higher in SLE patients with HDA than in LDA patients. There was a weak negative correlation between NT (r = -0.284, p=0.009) (r = -0.291, p = 0.007) and TT levels (r = -0.281, p = 0.010) (r = -0.289, p = 0.008) and a weak positive correlation between IMA levels (r = 0.279, p = 0.011) (r = 0.263, p = 0.016) and SLEDAI-2K, and major organ involvement. CONCLUSION: It is thought that thiol/disulfide hemostasis and IMA levels may be used as ideal biomarkers of OS in SLE patients and may reflect the disease activity and major organ involvement.

Useful Platelet Indices for the Diagnosis and Follow-Up of Patent Ductus Arteriosus.

OBJECTIVE: The aim of this study was to assess the utility of early postnatal platelet indices in the prediction of hemodynamically significant patent ductus arteriosus (hsPDA) and its response to pharmacological treatment in preterm infants. STUDY DESIGN: The medical records of 971 infants with gestational age < 30 weeks and birth weight < 1,500 g were analyzed retrospectively. Infants with hsPDA comprised the study group and those without hsPDA comprised the control group. Complete blood count results were recorded, and red cell distribution width-to-platelet ratio (RPR) and platelet mass were calculated. RESULTS: A total of 481 infants, 169 in the hsPDA group and 312 in the control group, were included. In terms of platelet indices, the hsPDA group showed significantly lower mean platelet volume (MPV) and platelet mass, whereas RPR was significantly higher (p < 0.05, respectively). Multiple logistic regression analysis showed that RDS (relative ratio [RR]: 2.39; 95% confidence interval [CI]: 1.45-3.93; p < 0.001), MPV < 7.85 (RR: 3.71; 95% CI: 2.29-6.01; p < 0.001), and RPR > 0.070 (RR: 5.33; 95% CI: 3.28-8.65; p < 0.001) were independent risk factors for hsPDA. CONCLUSION: Low MPV and platelet mass and high RPR in the first hours of life are risk factors for hsPDA and hsPDA refractive to pharmacological treatment with ibuprofen in preterm infants.

Increased vitamin B12 levels in children with zinc deficiency.

Additional nutritional deficiencies may accompany zinc deficiency. We determined the vitamin B12 levels in patients with zinc (Zn) deficiency. This retrospective study enrolled 256 patients age 6 months to 16 years (176 females and 80 males) in whom Zn levels in hair samples were measured concurrently with serum vitamin B12 and ferritin levels. For all patients, we retrospectively assessed the hair Zn levels, serum ferritin and vitamin B12 levels, red blood cell count, hemoglobin, hematocrit, mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration, and red cell distribution levels. Data were analyzed to determine whether there was a significant difference between any of these parameters and the presence of vitamin B12 deficiency between patients with and without Zn deficiency. In all 118 patients had Zn levels < 100 µg/g and 138 patients had levels > 100 µg/g. No significant differences were observed in ferritin levels, red blood cell parameters, or presence of iron deficiency or anemia between the two groups (p > 0.05). The median vitamin B12 level was 323 (range 238-440) pg/mL in the Zn-deficient group and 276 (range 208-382) pg/mL in those with normal Zn levels. Vitamin B12 levels were significantly higher in the Zn-deficient group (p = 0.02). A significant negative correlation was detected between vitamin B12 levels and Zn levels (r = -0.17, p = 0.004). Vitamin B12 levels are higher in patients with Zn deficiency; thus, Zn may have a negative effect on vitamin B12 levels.

Evaluation of Oxidative Stress by Dynamic Thiol/Disulfide Homeostasis and Ischemia-Modified Albumin Levels in Children with ß-Thalassemia Major.

OBJECTIVE: ß-thalassemia major (ß-TM) is a hemoglobinopathy characterized by reduced or absent ß-globin production. A balance remains between the production of free radicals and suppression of increased levels of reactive oxygen species by the antioxidant system. This study aimed to examine thiol/disulfide homeostasis (TDH) and serum ischemia-modified albumin (IMA) levels to evaluate the oxidant/antioxidant balance in healthy children and persons with ß-TM receiving and not receiving chelation therapy. METHODS: This prospective study was carried out from January to June 2021 among 46 individuals with ß-TM and 35 healthy controls. A spectrophotometric method was used to analyze TDH and IMA concentrations. RESULTS: We found that, compared to controls, native thiol (NT) (P = .048) and total thiol (TT) (P = .027) values were lower in the patient group, whereas disulfide (P < .001), disulfide/native thiol (D/NT) (P = .004), disulfide/total thiol (D/TT) (P = .005), native thiol/total thiol (NT/TT) (P = .004) and IMA (P = .045) values were higher. NT and TT levels were significantly lower in the chelation- group compared to the chelation+ and control groups (P = .002, P = .001). D/NT, D/TT, and NT/TT levels were higher in the chelation+ group than the control group (P = .007), and IMA levels were significantly higher in the chelation+ and chelation- groups compared to the control group (P = .002). The receiver operating characteristic analysis demonstrated that IMA levels were significantly higher in the children with ß-TM not taking regular chelation therapy. CONCLUSION: Thiol/disulfide homeostasis was observed to be weakened in children with ß-TM in our study. Our findings show that when children with ß-TM do not receive regular chelation therapy, their oxidant imbalance worsens.

Optical coherence tomography angiography findings in young ß-thalassemia patients.

PURPOSE: The purpose was to evaluate retinal vascular parameters by optical coherence tomography angiography in ß-thalassemia major patients. METHODS: Thirty-three patients with ß-thalassemia major (study group) and 29 healthy children (control group) were enrolled in the study. All subjects underwent a complete ocular examination. The mean foveal avascular zone, non-flow area, foveal avascular zone perimeter, acircularity index of foveal avascular zone, foveal density, the superficial capillary plexus, and deep capillary plexus were scanned using 6 × 6 mm optical coherence tomography angiography scans centered on the macula. Superficial capillary plexus and deep capillary plexus were also scanned centered on the optic disk. We collected data on histories of patients, and hemoglobin and ferritin were also studied from both groups. RESULTS: The mean age was 13.85 ± 4.69 years (range: 4-21 years) in ß-thalassemia major group and 12.59 ± 3.66 years (range: 6-18 years) in the control group. The mean foveal avascular zone value was 0.265 ± 0.11 mm2 in the study group and 0.296 ± 0.12 mm2 in the control group. The mean non-flow area value was 0.468 ± 0.12 mm2 in the study group and 0.479 ± 0.14 mm2 in the control group (p > 0.05). Differences in the mean values for foveal density and acircularity index were statistically significant between the study group and control group (p < 0.05, p = 0.026, and p = 0.026, respectively). Superficial capillary plexus and deep capillary plexus were not a significant difference between the study and control groups in 6 × 6 mm scans on macula and 4.5 × 4.5 mm scans on optic disk area (p > 0.05). Acircularity index was negatively correlated (r = -0.292, p = 0.026), and foveal density was positively correlated with hemoglobin (r = 0.292, p = 0.026). CONCLUSION: By using optical coherence tomography angiography, we detected foveal microvascular changes in young ß-thalassemia patients before significant ocular anomalies development.