scholarly journals EARLY PREDICTORS OF RENAL DYSFUNCTION IN Β-THALASSEMIA MAJOR AND INTERMEDIA PATIENTS

2004 ◽  
Vol 6 (1) ◽  
pp. e2014057 ◽  
Author(s):  
Azza A.G. Tantawy ◽  
Nagham El Bablawy ◽  
Amira A. M Adly ◽  
Fatma S E Ebeid
Keyword(s):  
Renal Dysfunction ◽  
Serum Ferritin ◽  
Total Protein ◽  
Plasma Osmolality ◽  
Thalassemia Major ◽  
Total Serum ◽  
Protein Z ◽  
Early Predictors ◽  
Group I ◽  
Significant Difference

Background: Better survival of thalassemia patients allowed previously unrecognized renal complications to emerge. Objectives: Assess prevalence and early predictors of renal dysfunction in young β-thalassemia major (β-TM) and intermedia (β-TI) patients. Subjects: 66 β-TM (group I), 26 β-TI (group II) Egyptian patients and 40 healthy controls. Methods: History, examination and investigations that included kidney function tests, serum ferritin, serum bicarbonate, plasma osmolality and urinary total proteins, microalbuminuria (MAU), N-acetyl-β-D-glucosaminidase (NAG), retinol binding protein (RBP), α-1 microglobulin, bicarbonate, osmolality, Creatinine clearance (CrCl), % fractional excretion of bicarbonate (% FE-HCO3). Results: The most common renal abnormality was proteinuria (71%), followed by increased urinary level of RBP (69.4%), NAG (58.1%), α-1 microglobulin (54.8%) and microalbumin (29%) and also decreased urinary osmolality (58.1%). Although serum creatinine and BUN were not statistically different between thalassemia patients and control, CrCl were significantly lowered in thalassemia patients. Total serum protein and albumin was significant lower in splenectomized β-TM, whereas urinary total protein and MAU were significantly increased in all thalassemia patients. NAG, RBP and α-1 microglobulin were negatively correlated with CrCl and positively correlated with serum ferritin and urinary total protein. Z-score analysis for discrimination of patients with renal dysfunction proved superiority of urine total protein and RBP. Comparative statistics of different frequencies revealed significant difference between the urinary total protein and both MAU and % FE-HCO3. Conclusion: Asymptomatic renal dysfunctions are prevalent in young β-TM and β-TI patients that necessitate regular screening and urinary total protein and RBP may be cost-effective for early detection.

Role of Cord Blood Carboxyhemoglobin in Detecting Significant Hyperbilirubinemia in Term Neonates with ABO Alloimmunization

2021 ◽  
Author(s):  
Mahir Tıraş ◽  
Emrah Can ◽  
Şahin Hamilçıkan
Keyword(s):  
Cord Blood ◽  
Total Serum ◽  
Healthy Controls ◽  
Term Neonates ◽  
Group Iii ◽  
Group I ◽  
Significant Difference ◽  
Group Ii ◽  
Positive Direct ◽  
Severe Hyperbilirubinemia

Objective This study aimed to assess whether cord blood carboxyhemoglobin (COHb) levels in jaundiced term neonates with and without a positive direct Coombs test (DCT) and in healthy controls could be used as a predictor of severe hyperbilirubinemia. The percentage of cord blood COHb should be higher among neonates with Coombs-positive ABO hemolytic disease than among those with Coombs-negative ABO incompatibility and higher than that of ABO-compatible control neonates. Study Design This cross-sectional descriptive study of 198 term neonates comprised three subgroups: group I featured 68 DCT-positive ABO-incompatible neonates (ABO + DCT), group II featured 60 DCT-negative ABO-incompatible neonates with hyperbilirubinemia (ABO–DCT), and group III featured 70 healthy controls. COHb was determined by an OSM3 hemoximeter. Results Group I differed from groups II and III for cord blood bilirubin, cord blood hemoglobin, and cord blood hematocrit. Groups I and II had higher mean total serum bilirubin (TSB) levels than group III, while there was no difference in the mean TSB levels between groups I and II. There was no significant difference between the COHb group means for groups I, II, and III (p = 0.98). The area under the receiver operating characteristic curve calculated for group I/group III and group II/group III were found to be 0.62 and 0.54, respectively. Conclusion COHb levels did not prove to be superior to the DCT for predicting the risk of developing severe hyperbilirubinemia in term neonates. Key Points

Is adequate and balanced nutrition during pregnancy more effective than iron and folic acid supplements?

Open Medicine ◽  
2010 ◽  
Vol 5 (2) ◽  
pp. 235-242 ◽  
Author(s):  
Funda Elmacioglu ◽  
Bulent Surucu ◽  
Tayfun Alper ◽  
Aliye Ozenoglu ◽  
Serdal Ugurlu
Keyword(s):  
Folic Acid ◽  
Nutritional Status ◽  
Food Consumption ◽  
Serum Ferritin ◽  
Binding Capacity ◽  
Average Energy ◽  
Group I ◽  
Significant Difference ◽  
Iron And Folic Acid ◽  
Group Ii

AbstractTo provide instruction for pregnant women regarding adequate and balanced nutrition and determine whether iron and folic acid supplementation is essential. The research was an experimental clinical intervention. The study was conducted between March 2004 and May 2005 with 80 pregnant volunteers. The study participants were in their 16th to 24th weeks of pregnancy; all participants were healthy, carried only one fetus, and successfully completed their pregnancy. All participants were instructed about adequate and balanced nutrition. Until the participants gave birth, 40 (Group 1) consumed an iron-rich diet that was equivalent to the inclusion of a supplement containing 100 mg Ferro III plus 0,35 mg folic acid; the other group (Group 2) was also instructed in proper nutrition and was given by a gynecologist 1 tablet (100 mg) Ferro III hydroxide polymaltose complex and iron pharmaceutical with 0,35 mg folic acid (Maltofer Fol). In both groups, before and after the instruction, consumption frequency was noted, and the levels of serum ferritin, serum iron, total iron-binding capacity, folic acid, and vitamin B12 in the blood were determined at monthly intervals. Between the two groups, no statistical difference was found with regard to age, number of pregnancies, weight before pregnancy, body mass index (BMI) before pregnancy, and weight of the newborn (p>0,05). At the end of the study, the hemoglobin, hematocrit, and serum ferritin levels decreased considerably in both groups compared to the initial values (p<0.01). No statistically significant difference in serum ferritin levels could be found between the two groups (p>0,05). The comparison of Group I and Group II in terms of nutritional status (average energy and food consumption) in the pre-instruction and post-instruction periods revealed that intake of total protein, heme protein, dietary fiber, folic acid, carotene, vitamins A, B1, B2, B6, C, and B12, potassium, calcium, phosphorus, iron, and zinc was higher in Group I in the post-instruction period (p=0.000); no statistically significant change in nutritional status during pregnancy was observed in Group II. Conclusion: Medical diet programs with iron sources are examined in association with food consumption. Assessment of hematological results suggests that, during pregnancy, each patient should receive a specific dose, rather than a routine dose, of iron and folic acid.

scholarly journals FATTENING AND MEAT QUALITIES OF GEESE OF OBROSHIN SELECTION WITH GRAY AND WHITE PLUMAGE

2021 ◽  
Vol 22 (1) ◽  
pp. 243-250
Author(s):  
L. V. Ferenc ◽  
M. D. Petriv
Keyword(s):  
Total Protein ◽  
Research Work ◽  
Live Weight ◽  
Total Protein Content ◽  
Mass Selection ◽  
Group I ◽  
Breed Group ◽  
Significant Difference ◽  
Males And Females ◽  
Group Ii

The research work provided a solution to the problem of preserving the gene pool of Obroshyn gray (OS) and Obroshyn white (OB) geese, which will improve and enhance the valuable qualities of the original flock of geese, gain the desired economic characteristics and thus ensure the competitiveness of the industry in modern conditions. The research was conducted in the laboratory of small livestock ISGKR NAAS and on the basis of SE DG "Miklashiv". The main method of breeding is the selection and selection of individuals with high productive qualities in order to obtain the same type of bird that would meet the planned performance parameters. Selection work was carried out by individual mass selection, aimed at consolidating the standard for each breed group of traits. Before the beginning of the breeding period, males and females of both groups were individually assessed by the exterior, typical plumage, live weight. It was found that both Obroshyn gray and Obroshyn white geese were well selected for productive characteristics and showed high fattening and meat qualities. All birds are well adapted to the natural conditions of the western region. Body sex measurements (chest circumference, torso length, keel, and metatarsus) were higher in males of the Obroshyn gray breed group of geese. Males at different ages had higher rates than females. Obroshyn gray geese (group I) outperformed their peers from group II in terms of meat qualities, the weight of uncorked and gutted carcass, and yield of edible parts. The pre-slaughter live weight of carcasses of males of group I (OS) was 4722 g and prevailed over peers of group II (OB) by 2.94%, and the live weight of females was 4149 g and was higher by 4.19%. It was found that the difference in the number of erythrocytes in males and females was insignificant and almost does not change with age, and hemoglobin on the contrary - increases with age. Males in all study periods had higher serum total protein compared to females. The lowest rate was in males and females аt 4 weeks of age, however, there was no statistically significant difference between the groups in the number of erythrocytes, hemoglobin, and total protein content.

Effects of selected herbal supplements in persons with type 2 diabetes

2020 ◽  
Vol 22 (4) ◽  
pp. 254-259
Author(s):  
Gajendra Prasad Rauniyar ◽  
R Sinha ◽  
DR Pandey ◽  
K Chapagain ◽  
R Maskey
Keyword(s):  
Lipid Profile ◽  
Blood Sugar ◽  
Serum Triglyceride ◽  
Multiple Comparisons ◽  
Lipid Lowering ◽  
Total Serum ◽  
Fenugreek Seeds ◽  
Group I ◽  
Significant Difference ◽  
Post Hoc

Diabetes mellitus is a metabolic abnormality leading to an increase in the plasma concentration of glucose and is a major cause of stroke and peripheral circulatory disorders. Momordica charantia (MC), commonly known as bitter gourd/Karela, and Trigonella foenum-graecum (TFG) (fenugreek/Methi) have several medicinal values like anti-diabetic, lipid-lowering property, anti-oxidant activity, anti-inflammatory, and anti-mutagenic activity. This is an open-label, four-parallel-group, prospective interventional clinical trial with a total number of 48 patients enrolled in the study and divided into four equal groups (12 in each group) viz; Group I (allopathic drug), Group II (allopathic drug and Karela (MC), Group III (allopathic drug and Methi (TFG) and Group IV (allopathic drug, methi, and karela). Blood sugar and lipid profile were measured at day 0 and day 90. One way ANOVA test was applied to find the significant difference between the groups and Tukey HSD post hoc test was applied for multiple comparisons among the four groups with probability p-value 0.05%. Multiple comparisons by post-hoc analysis between groups on day 90 showed a significant reduction of fasting blood sugar by 19.0% (p = 0.021), postprandial blood sugar by 35.0% (p= 0.001), total serum cholesterol by 14.0% (p= 0.000), serum triglyceride by 21.0% (p=0.000), and serum LDL cholesterol by 17.0% (p= 0.000)ingroup receiving Karela and fenugreek seeds supplementation. Whereas serum HDL cholesterol on the 90th day was higher in the group by 10.0% (p=0.015) receiving only fenugreek seeds as a supplementation. Fenugreek and karela, when given as a supplement, have a beneficial effect on blood sugar and lipid profile.

Onset of Cardiac Iron Loading in a Large and Homogeneous Cohort of Thalassemia Major Paediatric Patients

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2157-2157
Author(s):  
Alessia Pepe ◽  
Antonella Meloni ◽  
Brunella Favilli ◽  
Marcello Capra ◽  
Domenico Giuseppe D'Ascola ◽  
...  
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Conflicts Of Interest ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Myocardial Iron ◽  
Cardiac Iron ◽  
Paediatric Patients ◽  
Biventricular Function ◽  
Significant Difference

Abstract Abstract 2157 Introduction: Magnetic Resonance Imaging (MRI) by the T2* technique allows highly reproducible and non invasive quantifications of myocardial iron burden and it is the gold standard for quantifying biventricular function parameters. It is important to determine the appropriate age to start MRI screening, because its high cost. Few data are available in the literature and they are contrasting. So the aim of this study was to address this issue in our paediatric patients with thalassemia major (TM). Methods: We studied retrospectively 72 patients (47 males, 4.2–17.9 years old, mean age 13.03 ± 3.70 years), enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) network. Myocardial iron overload was measured by T2* multislice multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images. Results: The global heart T2* value was 29.7 ± 11.2 ms (range 6.2 – 48.0 ms). No significant correlation was found between global heart T2* value and age (see figure). The global heart T2* value did not show significant differences according to the sex (male 30.2 ± 11.0 ms versus female 28.7 ± 11.8 ms, P=0.568). Sixteen patients (22%) showed an abnormal global heart T2* value (<20 ms) and none of them was under 8 years of age. Global heart T2* value was negatively correlated with mean serum ferritin levels. Odds Ratio for high serum ferritin levels (≥ 1500 ng/ml) was 8.4 (1.01–69.37, OR 95%CI) for abnormal global heart T2* values (< 20 ms). The global heart T2* value did not show a significant difference with respect to the chelation therapy (P=0.322). No significant correlations were found between the global heart T2* values and the bi-atrial areas or the LV and RV morphological and functional parameters. Eight patients showed a left ventricular (LV) ejection fraction (EF) < 57% and none of them was under 7 years of age. Two patients showed a right ventricular (RV) EF < 52% and none of them was under 14 years of age. Conclusion: The MRI screening for both cardiac iron overload and function assessment can be started for TM patients at the age of 7 years. At this age not sedation is generally needed. If the availability of cardiac MRI is low, the serum ferritin levels could be used as a discriminating factor. Disclosures: No relevant conflicts of interest to declare.

1-Year Results From A Prospective Randomized Trial Comparing Phlebotomy with Deferasirox for the Treatment of Iron Overload in Pediatric Patients with Thalassemia Major Following Curative Stem Cell Transplantation

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 904-904 ◽  
Author(s):  
Adlette Inati ◽  
Nada Sbeiti ◽  
Evelyne Khoriaty ◽  
M Domenica Cappellini ◽  
Suzanne Koussa ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Iron Overload ◽  
Randomized Trial ◽  
Cell Transplantation ◽  
Serum Ferritin ◽  
Research Funding ◽  
Thalassemia Major ◽  
Prospective Randomized Trial ◽  
Significant Difference

Abstract Abstract 904 Background: As a result of previous transfusions, β-thalassemia major (TM) patients who have undergone curative hematopoietic stem cell transplantation (HSCT) are at increased risk of iron overload. There are, however, limited data on iron removal in such patients with either phlebotomy (PHL) or iron chelation. The aim of this study was to compare the efficacy, safety and convenience of the oral iron chelator deferasirox (DFX; Exjade®) with PHL for the treatment of iron overload in children with TM following HSCT, over a 1-year period. Methods: LB03T is a prospective, randomized trial enrolling children with TM aged 2-<18 yrs who had undergone HSCT. Patients were chelation-naïve, hepatitis B- and C-negative, with confirmed iron overload (serum ferritin ≥500 ng/mL on ≥2 monthly occasions, and liver iron concentration [LIC] >3 mg Fe/g dry weight [dw]). Eligible patients were randomized to PHL (6 mL/kg blood/2 weeks) or DFX (10 mg/kg/day starting dose; 5 mg adjustments up to 20 mg/kg/day were permitted). One of the primary endpoints was change in LIC assessed using magnetic resonance imaging techniques. Changes in serum ferritin levels, hemoglobin (Hb), total iron binding capacity (TIBC), non-transferrin-bound iron (NTBI), adverse events (AEs) and compliance with study treatment (PHL: ratio of performed:planned; DFX: tablet count) were also assessed. Convenience of treatment was evaluated using parents' responses to pre-prepared questions. Results: 27 patients were randomized to DFX or PHL; one patient randomized to PHL refused treatment, hence 12 patients received DFX and 14 received PHL. Mean age was 12.4 yrs and 53.8% were male. Patients were followed up for a mean of 12.0 months. 2 and 5 patients had DFX dose increases to 15 and 20 mg/kg/day, respectively. Mean DFX dose at last visit was 11.0 and 18.1 ng/mL in the LIC≤7 and LIC>7 groups, respectively. Median serum ferritin was significantly reduced from baseline over 12 months with DFX (–497.5 ng/mL, P=0.004 vs baseline) and PHL (–901.8 ng/mL, P<0.0001 vs baseline); there was no significant difference between groups (P=0.425). Mean LIC (for 20 patients with evaluable LIC following 1 yr of treatment) was significantly reduced with DFX (–5.78 mg Fe/g dw, P=0.0005 vs baseline) and PHL (–3.27 mg Fe/g dw, P=0.050 vs baseline); no significant difference between groups (P=0.270). In patients with serum ferritin levels ≥1000 ng/mL at baseline, DFX resulted in a significantly greater decrease in mean LIC than PHL (–8.1 vs –3.5 mg Fe/g dw, P=0.048; Table). For TIBC, the increase with DFX was significantly greater than with PHL (P=0.0003). NTBI decreased significantly from baseline by –1.91 μmol/L (P=0.014; n=9) with DFX (baseline 2.0 μmol/L) and –2.83 μmol/L (P=0.0015; n=11) with PHL (baseline 2.3 μmol/L); there was no significant difference between groups (P=0.362). NTBI and LIC were positively correlated (R=0.565; P=0.0026) at baseline and at last follow-up (R=0.881; P<0.0001). Baseline mean Hb was 12.5 and 12.6 g/dL in the DFX and PHL groups, respectively; levels were maintained with DFX (change –0.17 g/dL; P=0.426) (PHL: change –0.53 g/dL; P=0.033); no significant difference between groups (P=0.279). AEs reported for patients receiving DFX were skin rash [n=1], gastrointestinal upset [n=1], increased liver function tests [n=1]; for patients receiving PHL, difficulty with venous access [n=4] and distress during procedure [n=1] were reported. Compliance was excellent for 11 (91.7%) and 12 (85.7%); good for 1 (8.3%) and 1 (7.1%); and poor with 0 and 1 (7.1%) patients receiving DFX or PHL, respectively. Parents of 13/14 children randomized to PHL desired their children to receive DFX due to pain, risk of anemia and longer/more frequent hospital visits associated with PHL. Parents of 1/14 children were satisfied with PHL due to concerns over possible AEs with DFX. Conclusions: In pediatric post-HSCT patients with TM, both LIC and serum ferritin were reduced with DFX and PHL over 1 year. In patients with higher baseline iron burden, DFX decreased LIC to a greater extent than PHL. TIBC was also significantly increased with DFX compared with PHL. DFX dose adjustments from 10 to 20 mg/kg/day were required to achieve therapeutic goals in some patients, underscoring the need for appropriate and timely dose adjustments. DFX had a clinically manageable safety profile, compliance was high and the majority of parents with children receiving PHL stated a desire to switch to DFX. Disclosures: Inati: Novartis: Honoraria, Research Funding, Speakers Bureau. Cappellini:Novartis: Speakers Bureau. Taher:Novartis: Honoraria, Research Funding.

scholarly journals A Study of Vitamin D and Calcium Level in Beta Thalassemia Major in Duhok Province

2019 ◽  
Vol 7 (11) ◽  
pp. 1924
Author(s):  
Ghorbat Saleh Ali ◽  
Malika Kassem Najeeb ◽  
Amad M. Saleh Jubrail ◽  
Zeliha Selamoğlu
Keyword(s):  
Vitamin D ◽  
Blood Transfusion ◽  
Vitamin D Deficiency ◽  
Serum Calcium ◽  
Colorimetric Method ◽  
Thalassemia Major ◽  
Total Serum ◽  
Control Group ◽  
Significant Difference ◽  
25 Hydroxy Vitamin D

β-thalassemia major is severe hemolytic anemia which requires repeated blood transfusion and for survival, which leading to iron overload. Serious complications due to iron over load are recognized in the thalassemia patients. objectives Vitamin D and calcium are important factors for homeostasis of calcium in this study. This study was conducted for a period of two year including from patients with β- thalassemia major from Duhok Thalassemia Centre between 2014 -2015. Thirty eighth β- thalassemia major patients and thirty eighth persons with age- matched as control group were enrolled in the study. Serum concentration of 25- hydroxy vitamin D and calcium were estimated by enzyme linked fluorescent assay and colorimetric method, respectively. In patients, the level of serum 25- hydroxy vitamin D was significantly lower 11.11±4.36 ng/mL compared with control group (14.03±5.96ng/mL). Vitamin D deficiency is observed about 94.73% in patients. The mean level of serum calcium in patients group is 7.9±0.47mg/dL and 8.98±0.77 mg/dL in control group. There was a statistically significant difference in serum calcium value between patients and control group. 44.73% of patients have hypocalcaemia. This study demonstrated a high prevalence of Vitamin D deficiency and low total serum calcium levels in thalassemia patients with a significantly lower than healthy group. Hypocalcemia and vitamin D deficiency may be results from the negative effect of the repeated blood transfusion and malnourishment.

scholarly journals The Relation between Serum Hepcidin, Ferritin, Hepcidin: Ferritin Ratio, Hydroxyurea and Splenectomy in Children with β-Thalassemia

2019 ◽  
Vol 7 (15) ◽  
pp. 2434-2439
Author(s):  
Nagwa Abdallah Ismail ◽  
Sonia Adolf Habib ◽  
Ahmed A. Talaat ◽  
Naglaa Omar Mostafa ◽  
Eman A. Elghoroury
Keyword(s):  
Serum Ferritin ◽  
Iron Homeostasis ◽  
Peptide Hormone ◽  
Thalassemia Major ◽  
Knowing That ◽  
Significant Difference ◽  
Serum Hepcidin ◽  
Effect Of Splenectomy ◽  
Main Regulator ◽  
Beijing China

BACKGROUND: Hepcidin, a small peptide hormone, is established as the main regulator of iron homeostasis. AIM: To estimate serum hepcidin, ferritin, and hepcidin: ferritin ratio in β-thalassemia patients and to determine the effect of splenectomy and hydroxyurea on serum hepcidin. METHODS: A study was conducted on 30 thalassemia major (βTM), 29 thalassemia intermedia (βTI) and 29 healthy children's controls. Data were collected by patient interviewing where detailed history-taking and thorough clinical examinations were carried out. Serum ferritin and hepcidin were measured by ELISA assay (Bioneovan Co. Ltd Beijing, China). RESULTS: Βeta-thalassemia patients had higher serum ferritin, serum hepcidin and lower Hb and hepcidin: ferritin ratio compared to the controls (p < 0.001, 0.010, 0.001, 0.001) respectively. Β-TM patients had higher mean serum hepcidin and serum ferritin compared to β-TI, with statistically significant difference (P = 0.042, P < 0.001, respectively). Twenty-one patients out of 29 βTI was on hydroxyurea therapy; these patients had significantly lower levels of serum ferritin (P < 0.004) and significantly higher levels of Hb (P < 0.004). Serum ferritin was statistically significantly higher in splenectomized patients P < 0.009. Serum hepcidin level was insignificantly higher in splenectomized patients than non-splenectomized patients (21.6 ± 14.75, 17.76 ± 10.01 ng/mL). Hepcidin showed a significantly positive correlation with hepcidin: ferritin ratio in all studied groups. CONCLUSION: Serum hepcidin was elevated in β-thalassemia children with more evident elevation in βTM patients. Splenectomy played no major role in hepcidin regulation. Knowing that hepcidin in serum has a dynamic and multi-factorial regulation, individual evaluation of serum hepcidin and follow up, e.g. every 6 months could be valuable, and future therapeutic hepcidin agonists could be helpful in management of iron burden in such patient.

scholarly journals Cystatin C an early marker of Glomerular dysfunction in thalassemia major.

2020 ◽  
Vol 27 (02) ◽  
pp. 300-308
Author(s):  
Sumera Saghir ◽  
Adnan Riaz ◽  
Aisha Hasan ◽  
Yasir Ali Bhatti ◽  
Ahmed Ashar Ghuman ◽  
...  
Keyword(s):  
Serum Ferritin ◽  
Cystatin C ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Renal Diseases ◽  
Serum Cystatin C ◽  
Group Iii ◽  
Early Marker ◽  
Group I

Objectives: The objective of the present study is to investigate the role of cystatin C as an early marker of glomerular dysfunction in thalassemia major. Study Design: Cross sectional comparative study. Setting: Department of Biochemistry Post Graduate Medical institute with the Thalassemia Center in Sir Ganga Ram Hospital Lahore. Period: July 2017 and July 2018. Material & Methods: This study examined 90 male children all between the ages of 5-11, using non probability sampling techniques. The children were grouped as 21 healthy male children as control group I and 69 diagnosed male thalassemia major children further subdivided on the basis of serum ferritin level as group II, serum ferritin level <2500ng/ml, group III, serum ferritin level 2500-5000ng/ml and group IV serum ferritin level >5000ng/ml respectively. Individuals with hereditary renal diseases, on steroid therapy, or other co-morbid renal diseases were excluded from the study. Complete blood analysis, serum ferritin, creatinine and cystatin C were measured by Micro lab 300 and solid phase enzyme linked immune sorbent assay (ELISA) respectively. The results were compared by using SPSS version 20. Results: Group I: n=21 healthy children with ferritin between normal range 105.33 ± 30.03, serum creatinine 0.41 ± 0.05, serum cystatin C 0.57 ± 0.14. eGFR-creatinine 133.38±7.63, eGFR-cystatin C 122.9±17.63. Group II: n=20 (β-TM with ferritin <2500ng/ml). Mean ferritin was 1997.5±300.68 ng/ml (P<0.001), creatinine was 0.43± 0.05, serum cystatin C 0.66 ± 0.05 (P<0.05). eGFR-creatinine 121.45±4.89 P<0.05, eGFR-cystatin C 105.15±6.49 P<0.001. Group III: n= 25 (β-TM with ferritin 2500-5000 ng/ml). Mean ferritin level was 3850.0± 718.18 ng /ml (P<0.001), creatinine was 0.5±.07, cystatin C 0.96±0.13 (P<0.001). eGFR-creatinine 103.29±8.26(P<0.001), eGFR-cystatin C 75.75±10.67 (P<0.001). Group IV: n=24 (β-TM with ferritin >5000 ng/ml). Mean ferritin level was 6311.67±1060.61 ng/ml P value (P<0.001), creatinine was 0.57 ± 0.07, cystatin C 1.11 ± 0.09 (P<0.001). eGFR-creatinine 94.42±8.69 (P<0.001), eGFR cystatin C 64.67±4.23(P<0.001). Conclusion: A highly significant positive relation was found between serum ferritin-cystatin C as compared to creatinine among the study groups II, III and IV and significant inverse relationship between cystatin C and eGFR that concluded cystatin C as an early marker of glomerular dysfunction than creatinine in thalassaemia major children.

Efficacy of deferasirox median dose of 30 mg/kg /day in pediatric patients with β- thalassemia major during one year follow- up therapy.

2019 ◽  
Vol 10 (03) ◽  
Author(s):  
Nashwan M Al-Hafidh ◽  
Mozahim S. Younis
Keyword(s):  
Serum Ferritin ◽  
Pediatric Patients ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Mean Value ◽  
Median Dose ◽  
Baseline Serum ◽  
Significant Difference ◽  
One Year

Objective: To assess the efficacy of deferasirox median dose of 30 mg /kg /day in pediatric patients with β- thalassemia major during one year of follow up Patients and methods: This study was conducted at Ibn Al Atheer center of thalassemia, Mosul city, Iraq during the period from 3rd of February 2013 to 2nd of February 2014. Serum ferritin was measured at baseline and at four weekly intervals thereafter among 49 transfusion dependent children with β-thalassemia major, who were treated with median deferasirox dose of 30 mg /kg /day. Results: No statistically significant difference was detected between mean serum ferritin level at baseline (2189.39 ± 85.7) ng/mL and its mean value at four weekly intervals during forty-eight weeks of deferasirox therapy. There was significant (p = 0.027) improvement of serum ferritin at fifty – two weeks reading (1750.6 ± 202.8 ng/mL) compared to baseline reading. Percentage of patients with baseline serum ferritin levels of >2,500 ng/ml was 32.7% (16/49), which increased significantly (p=0.000) to 65% at four weeks of therapy, and ranged between 32.1% - 46.2 % in the remaining readings. Conclusions: There was no significant reduction of serum ferritin during initial forty-eight weeks of deferasirox median dose of 30 mg /kg /day among patient with baseline mean serum ferritin above 2000 ng /ml.

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