scholarly journals Assessment of serum visfatin level in beta thalassemia patients

QJM ◽  
2020 ◽  
Vol 113 (Supplement_1) ◽  
Author(s):  
S E Abdelwahab ◽  
D M M Habashy ◽  
M A Shams ◽  
A H M Amin

Abstract Background Thalassemias are monogenetic hematologic disorders which are caused by faulty synthesis of one or more of the Hb chains which leads to imbalance in globin chains resulting in hemolysis and impaired erythropoiesis and chronic inflammatory condition. Visfatin is a pro-inflammatory adipocytokine which is mostly expressed in visceral adipose tissue and its testing may help in assessment of severity of the disease. Aim of the work This study aims at measuring serum visfatin level in Beta thalassemia patients and its possible correlation to disease severity (major andintermedia). Patients and methods Forty-one patients diagnosed as β-thalassemia (major, intermedia) and twenty age and sex-matched healthy individuals as the control group were tested for serum visfatin levels by enzyme-linked immunosorbent assay. Results Serum visfatin was higher in theß-thalassemia major(ß-TM) group than in the control group (P<0.001). Serum visfatinand serum ferritin were higher inß-TM group than in ß-thalassemia intermediagroup (ß-TI)(p < 0.001).Serum visfatin was found positively correlated with platelet count (P< 0.001) in the ß-TM group.As per ROC curve analysis, measured serum visfatin level was found to discriminate ß-TM group from β-TI group and control group. Conclusion There is an association between serum visfatin and the degree of severity of β thalassemia disease. Recommendations Testing of the relationship between serum visfatin level and other vascular inflammatory markers in β-thalassemia disease (asadiponectin, resistin, intracellular adhesion molecule (ICAM)) to clarify the exact mechanism of the inflammatory process in the disease and its complications to alter the course of the disease.Also to study serum visfatin in complicated β-thalassemia patients to establish its possible role in cardiac, vascular, endocrine or any other complications and comparing it with non-complicated patients.

Author(s):  
Yusuf Abisowo Oshodi ◽  
Kabiru Afolarin Rabiu ◽  
Agbara Joy Onyinyechi ◽  
Akinlusi Fatimat Motunrayo ◽  
Kuye Olufunmilayo Taiwo ◽  
...  

Background: Adipocytokines have been recently implicated in the pathogenesis of preeclampsia. Visfatin is one of such adipokines. Objective: To determine the association between serum visfatin levels and preeclampsia. Methods: A prospective, case-control study was carried out in 160 pregnant women consisting of 80 pre-eclamptics and 80 normotensive controls, matched for age and parity during the third trimester. Maternal serum visfatin levels were determined in both groups using a visfatin (Human) enzyme- linked immunosorbent assay. Serum Visfatin levels were compared between the groups and correlated to the blood pressure, proteinuria, fetal birth weight and Apgar scores. Results: The mean serum visfatin level was significantly higher (10.3±6.9 ng/ml) in preeclampsia than (7.4±4.4 ng/ml) in the control group (p=0.001). The mean serum visfatin level was higher in severe pre-eclamptics (10.8±8.9 ng/ml) compared to (9.6±5.8ng/ml) in mild preeclamptics and this was statistically significant (p=0.021). Visfatin levels showed a negative and non-significant correlation with both systolic (r= -0.011 and p=0.924), diastolic blood pressure (r= -0.012, p=0.913) and body mass index (r= -0.142, p=0.209) in both study and control groups. Mean birth weight was significantly lower in the preeclampsia (2.8±0.25 kg) compared to the control group (3.2±0.31 kg) P=0.000. the mean birth weight was lower in severe preeclampsia (2.7±0.25 kg compared to 2.9±0.39 in mild preeclamsia. There was no significant correlation between the visfatin levels and Apgar score at 5minutes and birth weights in both groups (P=>0.05). Conclusion: This study showed a significant increase in the level of visfatin in preeclampsia compared to their normo-tensive controls. However, this increased level was not consistent with the severity of the disease.


Author(s):  
Hasan Smesam ◽  
Hasan Qazmooz ◽  
Sareh Arjmand ◽  
Hussein Kadhem Al-Hakeim ◽  
Seyed Omid Ranaei‐Siadat,

Beta thalassemia major (β-TM) disorder characterized by the lack, or severe reduction in the production of hemoglobin β-globin chains. The standard protocol for the management of β-TM is blood transfusion and iron chelation therapy to reduce the iron overload state. The present study aimed to investigate the relationships between two iron regulatory hormones, hepcidin (HEPC) and erythroferrone (ERFE) levels and iron status parameters (ISPs) in Iraqi patients with β-TM. ISPs and hormones were measured in sixty patients and compared with thirty healthy controls. The results indicated significant changes in different iron status parameters, while ferritin (FRT) with the ~11 fold increase showed the most change. Significant reduction in HEPC and increase in ERFE levels were detected in patients as compared to the control group, while no direct correlation was identified with the other measured ISPs. Receiver operating characteristic (ROC) analysis showed that the z-score of the composite of ERFE+FRT has a full diagnostic ability for β-TM. In conclusion, our finding indicated the correlation between different ISPs, FRT as the leading predictor of iron overload and tow main iron regulatory hormones.


Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 100
Author(s):  
Asmaa A. Mahmoud ◽  
Doaa M. Elian ◽  
Nahla MS. Abd El Hady ◽  
Heba M. Abdallah ◽  
Shimaa Abdelsattar ◽  
...  

Background: A good survival rate among patients with beta thalassemia major (beta-TM) has led to the appearance of an unrecognized renal disease. Therefore, we aimed to assess the role of serum cystatin-C as a promising marker for the detection of renal glomerular dysfunction and N-acetyl beta-D-glucosaminidase (NAG) and kidney injury molecule 1 (KIM-1) as potential markers for the detection of renal tubular injury in beta-TM children. Methods: This case-control study was implemented on 100 beta-TM children receiving regular blood transfusions and undergoing iron chelation therapy and 100 healthy children as a control group. Detailed histories of complete physical and clinical examinations were recorded. All subjected children underwent blood and urinary investigations. Results: There was a significant increase in serum cystatin-C (p < 0.001) and a significant decrease in eGFR in patients with beta-TM compared with controls (p = 0.01). There was a significant increase in urinary NAG, KIM-1, UNAG/Cr, and UKIM-1/Cr (p < 0.001) among thalassemic children, with a significant positive correlation between serum cystatin-C, NAG and KIM-1 as regards serum ferritin, creatinine, and urea among thalassemic patients. A negative correlation between serum cystatin-C and urinary markers with eGFR was noted. Conclusion: Serum cystatin-C is a good marker for detection of glomerular dysfunction. NAG and KIM-1 may have a predictive role in the detection of kidney injury in beta-TM children.


2021 ◽  
Vol 9 (B) ◽  
pp. 149-153
Author(s):  
Khalaf Hussein Hasan ◽  
Hasan Abdulla Aswad ◽  
Aspazija Sofijanova

BACKGROUND: Zinc is one of the most important minerals incorporated in the enzymes of the human body. Zinc may be deficient in patients with the β-thalassemia major with possible adverse outcomes. AIM: The purpose of this study was to assess the serum zinc status in β-thalassemia major patients in Duhok city. PATIENTS, MATERIAL, AND METHODS: In this case–control study, 70 children with β-thalassemia major (2–12 years) of both genders were enrolled and were matched with 70 apparently healthy children for age and sex. A venous blood sample was obtained from each child for the measurement of serum zinc and serum ferritin levels at Jin Center in Duhok City between January 1 and June 30, 2017. RESULTS: The mean serum zinc in the thalassemia patients (74.79 [±25.14] μg/dl) was significantly lower compared to the control group (93.61 [±15.12] μg/dl), (p = 0.0001). The serum zinc was not significantly different in thalassemia patients in terms of age, disease onset, gender, height, weight, body mass index, amount of blood transfusion, and type of chelation. There was a statistically significant correlation between serum zinc levels with a serum ferritin level of patients. CONCLUSION: The study showed that thalassemia patients have significantly lower serum levels of zinc with no relation to medical factors.


2021 ◽  
Vol 9 (2) ◽  
Author(s):  
Uni Gamayani ◽  
Titin Junaidi ◽  
Nushrotul Lailiyya ◽  
Nur Suryawan ◽  
Nanan Sekarwana

Vitamin B9 (folic acid) and B12 (cobalamin) are essential vitamins that play roles in the process of hematopoiesis and maintaining the function of peripheral nerves. Therefore, these deficiencies may create a risk for peripheral neuropathy in beta-thalassemia major patients. The purpose of this study is to determine the relationship between vitamin B9 level, vitamin B12 level, and peripheral neuropathy in beta-thalassemia major children. It was an observational analytical study with a case-control design has been conducted at Dr. Hasan Sadikin General Hospital Bandung, Indonesia, in May–July 2019. There were 47 beta-thalassemia major children with peripheral neuropathy (case) and 41 healthy children (control). All subjects completed a general demographic questionnaire, underwent neurological examination, and were tested for vitamin B9 and B12 serum levels. Data were then analyzed using the unpaired t test to compare the vitamin levels between both groups and Spearman’s rank correlation test to investigate the correlation between vitamin levels and the number of affected nerves in the case group. Comparison of folic acid levels in the case group (21.52±6.22 ng/mL) and the control group (23.81±7.51 ng/mL) showed no significant difference (p=0.19). In contrast, cobalamin in the case group (288.57±168.61 ng/mL) and the control group (385.95±197.48 ng/mL) showed a significant difference (p=0.01). In addition, there was a moderate correlation (p=0.004, r=0.41) between folic acid level and the number of motoric nerves affected in the case group. In conclusion, cobalamin level correlates with peripheral neuropathy in beta-thalassemia major patients, and folic acid level correlates with the number of affected nerves, especially motoric nerves. HUBUNGAN ANTARA VITAMIN B9 (ASAM FOLAT), VITAMIN B12 (KOBALAMIN), DAN NEUROPATI PERIFER PADA ANAK DENGAN TALASEMIA BETA MAYORVitamin B9 (asam folat) dan B12 (kobalamin) merupakan vitamin esensial yang berperan dalam proses hematopoiesis dan menjaga fungsi saraf tepi. Defisiensi vitamin ini dapat menimbulkan risiko neuropati perifer pada pasien talasemia beta mayor. Tujuan penelitian ini mengetahui hubungan antara kadar vitamin B9, vitamin B12, dan neuropati perifer pada anak talasemia beta mayor. Metode penelitian ini adalah analitik observasional dengan rancangan studi kasus kontrol yang dilakukan di RSUP Dr. Hasan Sadikin Bandung, Indonesia pada Mei–Juli 2019. Terdapat 47 anak talasemia beta mayor dengan neuropati perifer (kelompok kasus) dan 41 anak sehat (kelompok kontrol). Seluruh subjek penelitian mengisi kuesioner demografi umum, menjalani pemeriksaan fisis neurologis, serta dilakukan tes kadar vitamin B9 dan B12 serum. Uji t test tidak berpasangan digunakan untuk membandingkan kadar vitamin pada kedua kelompok dan uji korelasi Spearman untuk membandingkan kadar kedua vitamin tersebut dengan jumlah saraf yang terkena pada kelompok kasus. Perbandingan kadar asam folat kelompok kasus (21,52±6,22 ng/mL) dan kelompok kontrol (23,81±7,51 ng/mL) menunjukkan perbedaan yang tidak bermakna (p=0,19), sedangkan perbandingan kadar kobalamin kelompok kasus (288,57±168,61 ng/mL) dan kelompok kontrol (385,95±197,48 ng/mL) menunjukkan perbedaan yang bermakna (p=0,01). Selain itu, terdapat korelasi sedang (p=0,004; r=0,41) antara kadar asam folat dam jumlah saraf motorik yang terkena pada kelompok kasus. Kesimpulan, kadar kobalamin berhubungan dengan neuropati perifer pada penderita talasemia beta mayor dan kadar asam folat berhubungan dengan jumlah saraf yang terkena, terutama saraf motorik.


2016 ◽  
Vol 64 (3) ◽  
pp. 771-781 ◽  
Author(s):  
Galila M Mokhtar ◽  
Wafaa E Ibrahim ◽  
Nancy S Elbarbary ◽  
Randa M Matter ◽  
Ahmed S Ibrahim ◽  
...  

The aim of this study is to assess the correlation between cardiac and hepatic T2* MRI findings with the endocrine and exocrine pancreatic functions in known patients with β-thalassaemia major (β-TM). A total of 50 adolescent patients with β-TM and 44 healthy controls were investigated via: serum amylase, lipase, triglyceride index, oral glucose tolerance test and T2* MRI, to assess iron content in the heart and liver. Diabetes was found in 20%, and 40% of patients had impaired fasting glucose (IFG). Cardiac T2* was less than 10 ms in 22% indicating heavy load with iron in cardiac tissues. There was a significant decrease in median serum amylase (63.5 vs 87.5 IU/L, p=0.003) and lipase (63 vs 90 IU/L, p=0.017) among patients in comparison with the control group. Patients with β-TM and diabetes had lower serum amylase (32 vs 68 IU/L), lipase (28 vs 79 IU/L), cardiac and hepatic T2* MRI (7 vs 25.5 ms; 3 vs 6 ms, p<0.001 for all) than those without diabetes. Similar results were found among patients with IFG when compared with others (p<0.001 for all). Cardiac and hepatic T2* were inversely correlated to triglyceride index (r=−0.376, p=0.014 and r=−0.475, p=0.001, respectively) and positively correlated to amylase (r=0.791 and r=0.790) and lipase (r=0.784 and r=0.783; p<0.001 for all). The endocrine and exocrine pancreatic functions might become an equivalent predictor to cardiac and hepatic iron overload, especially in countries where MRI is not available or where it is expensive. The early occurrence of these abnormalities warrants more intensive chelation therapy.


Blood ◽  
1984 ◽  
Vol 63 (4) ◽  
pp. 803-806
Author(s):  
A Correra ◽  
JH Graziano ◽  
C Seaman ◽  
S Piomelli

The relationships among hemoglobin concentration (Hb), red cell 2,3- diphosphoglycerate (2,3-DPG), and p50 were studied in 20 chronically hypertransfused patients with thalassemia major. In the nontransfused control group, which included normal individuals as well as patients with sickle cell disease or iron deficiency anemia, the Hb correlated inversely with both 2,3-DPG concentration and p50, as is well established. In contrast, however, prior to transfusion, at the nadir of Hb, patients with thalassemia major had inappropriately low 2,3-DPG concentrations and p50s. These findings occurred in all patients, regardless of whether they had received packed, leukocyte-poor, or frozen-thawed red cells. The hypothesis that the time of blood storage was a factor was excluded by repeatedly transfusing one patient with packed red cells administered within 4 hr of collection in CPDA-1. A second hypothesis, that red cell function might be impaired by the iron- overloaded thalassemic environment, was excluded by studying a newly diagnosed, newly transfused patient with aplastic anemia. In both cases, the same inability to appropriately increase 2,3-DPG and p50 as the Hb fell during the intertransfusion interval was noticed. These data suggest that red cells of chronically transfused patients are unable to adapt to the decline in Hb that occurs during the intertransfusion interval.


Author(s):  
MURAT DOĞAN ◽  
MEHMET KOSE ◽  
Mehmet ÖZTÜRK ◽  
melih hangul ◽  
HÜMEYRA ASLANER

Objective: Acute bronchiolitis is one of the most common causes of hospitalization for children younger than 1 year. Although the prognosis for these children is generally good, the condition involves a risk of mortality. Here, we evaluate the immature platelet fraction (IPF) as a biomarker for the severity of acute bronchiolitis. Material and Methods: In total, 179 children who had been diagnosed with acute bronchiolitis were classified into three groups: mild, moderate, and severe bronchiolitis, and 80 healthy children were included as a control group. The diagnostic capacity of the IPF, mean platelet volume (MPV), platelet distribution width (PDW), white blood cell count (WBC), and platelet count (PLT) values to predict bronchiolitis was evaluated using receiver operating characteristic (ROC) curves and their respective areas under the curves (AUCs) calculated with 95% confidence intervals. Results: Patients with acute bronchiolitis had a larger IPF than their healthy counterparts (p < 0.001). Additionally, a positive correlation was observed between the clinical severity of the disease and the IPF. The ROC curve analysis indicated that the IPF cut-off point for predicting acute bronchiolitis was >3.2%, with a sensitivity of 84% and specificity of 97%. Our results clearly demonstrate that the AUCs for IPF, MPV, PDW, WBC, and PLT were statistically significant for the bronchiolitis versus the control group. The AUC was greatest for the IPF. Conclusion: The IPF is a new marker for diagnosing and evaluating the clinical severity of acute bronchiolitis.


Blood ◽  
1995 ◽  
Vol 86 (10) ◽  
pp. 3945-3950 ◽  
Author(s):  
J Yuan ◽  
A Bunyaratvej ◽  
S Fucharoen ◽  
C Fung ◽  
E Shinar ◽  
...  

The thalassemias are a heterogeneous group of disorders characterized by accumulation either of unmatched alpha or beta globin chains. These in turn cause the intramedullary and peripheral hemolysis that leads to varying anemia. A partial explanation for the hemolysis came our of our studies on material properties that showed that beta-thalassemia (beta- thal) intermedia ghosts were very rigid but unstable. A clue to this instability came from the observation that the spectrin/band 3 ratio was low in red blood cells (RBCs) of splenectomized beta-thal intermedia patients. The possible explanations for the apparent decrease in spectrin content included deficient or defective spectrin synthesis in thalassemic erythroid precursors or globin chain-induced membrane changes that lead to spectrin dissociation from the membrane during ghost preparation. To explore the latter alternative, samples from different thalassemic variants were obtained, ie, beta-thal intermedia, HbE/beta-thal, HbH (alpha-thal-1/alpha-thal-2), HbH/Constant Spring (CS), and homozygous HbCS/CS. We searched for the presence of spectrin in the first lysate of the standard ghost preparation. Normal individuals and patients with autoimmune hemolytic anemia, sickle cell anemia, and anemia due to chemotherapy served as controls. Using gradient sodium dodecyl sulfate-polyacrylamide gel electrophoresis analysis, no spectrin was detected in identical aliquots of the supernatants of normals and these control samples. Varying amounts of spectrin were detected in the first lysate supernatants of almost all thalassemic patients. The identification of spectrin was confirmed by Western blotting using an affinity-purified, monospecific, rabbit polyclonal antispectrin antibody. Relative amounts of spectrin detected were as follows in decreasing order: splenectomized beta-thal intermedia including HbE/beta-thal; HbCS/CS; nonsplenectomized beta-thal intermedia, HbH/CS; and, lastly, HbH. These findings were generally confirmed when we used an enzyme-linked immunosorbent assay technique to measure spectrin in the first lysate. Subsequent analyses showed that small amounts of actin and band 4.1 also appeared in lysates of thalassemic RBCs. Therefore, the three major membrane skeletal proteins are, to a varying degree, unstably attached in severe thalassemia. From these studies we could postulate that membrane association of abnormal or partially oxidized alpha- globin chains has a more deleterious effect on the membrane skeleton than do beta-globin chains.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Chuchu Gao ◽  
Zongtai Feng ◽  
Lixia Wang ◽  
Xingxing Zhao ◽  
Kai Fu ◽  
...  

Abstract Background Late-onset sepsis (LOS) is a systemic inflammatory response syndrome in neonates, and the molecular mechanism of LOS is incompletely characterized. The purpose of this study was to explore the potential value of receptor interacting protein 3 (RIP3) in LOS. Methods 63 neonates with LOS supported by positive culture and 79 neonates without sepsis were enrolled in this study from September 2019 to March 2021. Plasma RIP3 was detected by enzyme-linked immunosorbent assay (ELISA) and assessed along with the whole blood hypersensitive C-reactive protein (hs-CRP) level and platelet count (PLT). Differences in RIP3, hs-CRP and PLT between the two groups were compared. Changes in the three indicators in sepsis were also observed after treatment. The diagnostic value of indicators for LOS was evaluated by receiver operating characteristic (ROC) curve analysis. Results In the sepsis group, RIP3 and hs-CRP levels were significantly higher than those in the control group (RIP3, p < 0.0001; hs-CRP, p < 0.0001), and PLT was significantly lower than that in the control group (p < 0.0001). After treatment, RIP3 and hs-CRP levels among septic survivors were significantly decreased (p < 0.0001) and PLT significantly improved (p = 0.0216). With RIP3 > 15,845.19 pg/mL, hs-CRP > 5.00 mg/L, and PLT < 204.00 × 109/L as the positive criteria, the sensitivity values of the three indicators in the diagnosis of LOS were 69.8%, 60.3%, 60.3%, respectively, and the specificity values were 92.4%, 96.2%, 79.8%, respectively. The combination of RIP3, hs-CRP and PLT had a sensitivity of 77.8% and specificity of 97.5%. Conclusions RIP3 may contribute to the early diagnosis of LOS and monitoring of treatment effect. The combined detection of RIP3, hs-CRP and PLT may be more effective than individual detection in the diagnosis of LOS.


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