Clinical Features and Morbidities of Hb H Disease in Taiwan

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4836-4836
Author(s):  
Meng Yao Lu ◽  
Ming Chung Kuo ◽  
Shih Chung Wang ◽  
Shih Hsiang Chen ◽  
Bor Sheng Ko ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Features ◽  
Serum Ferritin ◽  
Thromboembolic Event ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Globin Gene ◽  
Iron Concentration ◽  
Hb H Disease ◽  
The Mean

Abstract Introduction Patients with non-transfusion-dependent thalassemia experience a wide array of clinical complications despite their independence from frequent, regular red blood cell transfusions. They have the higher incidence of osteoporosis, extramedullary hematopoeisis (EMH), hypogonadism, cholelithiasis, thromboembolic disease, pulmonary hypertension, silent cerebral ischemia, and leg ulcers. Thalassemia is highly prevalent in Taiwan and Hb H disease is predominant. But limited data are available about clinical features and morbidities. Here, we studied clinical features and morbidities in Taiwanese patients with Hb H disease. Methods & Results We collected 90 patients with Hb H disease in three hospitals since 2014 Nov till 2016 July. Male to female were 43/59. The mean age was 33.1 years ( from 0.5 to 92.3 years). Two cases died of pulmonary hypertension and old age at 31 years old and 87 years old. Alfa-globin gene genotype studies were done in 44 cases. The (- -(SEA)) type of α(0)-thalassemia mutation was detected in all patients. Twenty- four (57.1%) cases were deletional (α(3.7)/ α(4.2)/unknown 19/4/1) and 20 (42.9%) were nondeletional (CS/RS 18/2) type. The mean of Hemoglobin (Hb) and serum ferritin level were 8.7 g/dL and 730 ng/mL. We also revealed the positive correlation between age and serum ferritin level. The liver iron concentration (LIC) were 6.694 mg Fe/g dw (n=35). The Hb, ferritin and LIC level were not different between deletional and non- deletional groups. They received the transfusion management : 1 with regular transfusion ≦ 6 weeks interval, 5 with irregular transfusion ≧ 6 weeks interval, 27 with occasional transfusion and 57 without transfusion. Fifteen cases received splenectomy. There were significantly higher prevalence for transfusion frequency and splenectomy in non-deletional group. The prevalence of morbidities were 16/79 for cholelithiasis, 12/90 for thromboembolic event, 4/90 for heart failure symptoms ( 2 for pulmonary hypertension), 5/90 for arrhythmia, 3/90 for bone fracture, 5/20 for osteoporosis and 0 for renal stone. There were non-significantly higher prevalence for morbidities in non-deletional group. Discussion & Conclusion The study provides the clinical features and the prevalence of morbidities in Hb H disease in Taiwan. Surprisingly, the prevalence of thromboembolic event and pulmonary hypertension are overlooked in our routine Hb H disease care. We need to schedule close and careful clinical follow up of Hb H patients as they get older, they get some morbidities or they are non-deletional genotype. Disclosures No relevant conflicts of interest to declare.

scholarly journals The Effect of Chronic Viral Hepatitis on Serum Ferritin Level in Thalassemia Patients

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4819-4819
Author(s):  
Natthapat Rujeerapaiboon ◽  
Adisak Tantiworawit ◽  
Pokpong Piriyakhuntorn ◽  
Thanawat Rattanathammethee ◽  
Sasinee Hantrakool ◽  
...  
Keyword(s):  
Viral Load ◽  
Iron Overload ◽  
Viral Hepatitis ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Conflicts Of Interest ◽  
Ferritin Level ◽  
Iron Concentration ◽  
Chronic Viral Hepatitis ◽  
The Mean

Background: Serum ferritin is widely used as a marker of iron overload in thalassemia patients. However, the ferritin level is affected by active infections or inflammation. The association between viral hepatitis and serum ferritin level in thalassemia patients is still unclear. This study aimed to determine the effect of chronic viral hepatitis on serum ferritin level in thalassemia patients. Methods: This was a cross-sectional study in thalassemia patients aged ≥15 years-old at Chiang Mai University hospital. We expected that thalassemic patients in our clinic have a mean serum ferritin of 767 ng/mL with a standard deviation of 210 ng/mL. As a result, we have to enroll a total of 28 patients to demonstrate 30% difference of mean serum ferritin when the power was set at 80% with alpha level of 0.05. Information on chronic viral hepatitis, mean serum ferritin and liver iron concentration (LIC) as measured by T2* MRI were collected. Chronic viral hepatitis status was confirmed by either HBV DNA or HCV RNA testing. Patients were categorized to hepatitis and non-hepatitis group. Serum ferritin levels were compared between two groups. LIC measurement was used as a gold standard for iron overload. Subgroup analysis was performed according to iron overload and transfusion requirement status. Categorical and continuous variables were compared using the Chi-squared test and T-test, respectively. The correlation between viral loads and mean serum ferritin levels was analyzed by Pearson's correlation. Result: Of 32 thalassemia patients (25 non-transfusion dependent [NTDT] and 7 transfusion dependents [TDT]), 13 patients had chronic viral hepatitis (7 with hepatitis B and 6 with hepatitis C infections). The LIC between hepatitis and non-hepatitis groups were not significantly different (7.28 [SD 4.7] vs 9.08 [SD 5.2] mg Fe/g, p=0.19). In the higher LIC group (≥ 5 mg Fe/g), the mean serum ferritin level was higher in the hepatitis group than non-hepatitis group (1,776 [SD 488] vs 967 [SD 860] ng/mL, p=0.03). For the lower LIC group (<5 mg Fe/g), the mean ferritin levels were not significantly different between the hepatitis and non-hepatitis groups (646 [SD 224] vs 459 [SD 205] ng/mL, p=0.22). The correlation between the viral load and mean ferritin level in NTDT group showed a significant linear correlation with R=0.7 (p=0.04). Conclusions: We observe a higher serum ferritin level among thalassemia patients who concurrently have chronic viral hepatitis. Chronic viral hepatitis is a possible cause of a falsely high ferritin level in these patient population. Furthermore, the viral load is positively correlated with serum ferritin level. Disclosures No relevant conflicts of interest to declare.

scholarly journals Noninvasive assessment of iron overload by magnetic resonance imaging

2020 ◽  
Vol 19 (3) ◽  
pp. 158-163
Author(s):  
E. E. Nazarova ◽  
D. A. Kupriyanov ◽  
G. A. Novichkova ◽  
G. V. Tereshchenko
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Iron Concentration ◽  
The Body ◽  
Iron Accumulation ◽  
Resonance Imaging

The assessment of iron accumulation in the body is important for the diagnosis of iron overload syndrome or planning and monitoring of the chelation therapy. Excessive iron accumulation in the organs leads to their toxic damage and dysfunction. Until recently iron estimation was performed either directly by liver iron concentration and/or indirectly by measuring of serum ferritin level. However, noninvasive iron assessment by Magnetic resonance imaging (MRI) is more accurate method unlike liver biopsy or serum ferritin level test. In this article, we demonstrate the outlines of non-invasive diagnostics of iron accumulation by MRI and its specifications.

scholarly journals Red meat and egg intake and serum ferritin concentrations in Colombian children: results of a population survey, ENSIN-2015

2020 ◽  
Vol 9 ◽  
Author(s):  
Oscar F. Herran ◽  
Jhael N. Bermúdez ◽  
María Del Pilar Zea
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Red Meat ◽  
Cross Sectional ◽  
Egg Consumption ◽  
Dietary Consumption ◽  
The Mean ◽  
The Relationship ◽  
Meat And Eggs

Abstract The present study aimed to (a) establish the frequency of consumption of red meat and eggs; (b) determine serum ferritin levels (μg/l); and (c) establish the relationship between serum ferritin and the consumption of red meat and eggs. In Colombia during 2014–2018, an analytical study was conducted in 13 243 Colombian children between the ages of 5 and 17 years, based on cross-sectional data compiled by ENSIN-2015 (Encuesta Nacional de la Situación Nutricional en Colombia-2015) on serum ferritin levels and dietary consumption based on a questionnaire of the frequency of consumption. Using simple and multiple linear regression, with the serum ferritin level as the dependent variable and the frequency of consumption as the main explanatory variable, the crude and adjusted partial regression coefficients (β) between serum ferritin levels and consumption were calculated. The frequency of habitual consumption of red meat was 0⋅49 (95 % CI 0⋅47, 0⋅51) times/d. The frequency of habitual egg consumption was 0⋅76 (95 % CI 0⋅74, 0⋅78) times per d. The mean serum ferritin level in men was 41⋅9 (95 % CI 40⋅6, 43⋅1) μg/l and in women, 35⋅7 (95 % CI 34⋅3, 37⋅7) μg/l (P < 0⋅0001). The adjusted β between the consumption of red meat and eggs and serum ferritin levels were β = 3⋅0 (95 % CI 1⋅2, 4⋅7) and β = 2⋅5 (95 % CI 1⋅0, 3⋅9) for red meat and eggs, respectively. In conclusion, red meat and eggs are determinants of serum ferritin levels in Colombia and, therefore, could be considered public policy options to reduce anaemia and Fe deficiency.

Different Adverse Event Profiles and Significant Hematological Improvement Are Noted When Deferasirox Was Used in Adult Patients with Chronic Transfusion-Related Iron Overload in Taiwan: Results from a Prospective Observational Clinical Trial

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4554-4554
Author(s):  
Bor-Sheng Ko ◽  
Ming-Chih Chang ◽  
Tzeon-Jye Chiou ◽  
Te-Kau Chang ◽  
Yeu-Chin Chen ◽  
...  
Keyword(s):  
Adverse Events ◽  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Platelet Response ◽  
Chronic Anemia ◽  
Erythroid Response ◽  
The Mean ◽  
Grade 3

Abstract Background Iron overloading is a common problem for adult with myelodysplastic syndrome (MDS), aplastic anemia (AA) or other chronic anemia. Deferasirox (DFX) has been proven as an effective therapy to chelating iron in these patients. Anyway, the safety of DFX is still a concern, and the information of safety profiles and efficacy are less understood in Taiwan. This study is planned primarily to collect long-term safety data of DFX treatment in iron-overloaded MDS, AA and other chronic anemia patients in Taiwan. Study Design This is an observational, single-arm, and multi-center study. Low-risk MDS or AA patients with transfusion-related iron overload, or patients with other anemia and serum ferritin more than 2000 ug/ml, were enrolled within the 18-month enrolling period if DFX is planned to be prescribed. Exposure of iron chelating agents other than DFX before trial initiation was allowed. The initial dose and subsequent adjustment of DFX were up to investigator¡¦s preference. All patients were followed for 3 years for adverse events (AEs) and disease outcomes. Results From 2009 to 2011, 79 patients were enrolled in this study, including 38 MDS, 23 AA and 18 other chronic anemias. Forty-seven cases (59.5%) were male, with mean age 64.3¡Ó17.8 y/o. Fifty-six (70.9%) subjects failed to complete the 3-year study period, but only 8 (10.1%) of the subjects withdrew DFX due to drug-related AEs. The mean DFX exposure dose during study was 17.7¡Ó4.02 mg/Kg/day. In contrast with those reported in literature, the most frequently reported drug-related AEs were rash (16, 20.3%), diarrhea (11, 14.0%), hypercreatinemia (8, 10.1%), pruritus (7, 8.9%), and so on (as in Table 1). When classified by organ systems, skin disorders were the frequently reported one (26, 32.9%), and followed by GI disorders (n=24, 30.4%). Grade 3-4 drug-related adverse events were rare (n=4, 5.1%). For all subjects, DFX could effectively decrease serum ferritin level from baseline (-985+/-2090 ng/ml (p=0.0154 vs. baseline) and -1710+/-2290 ng/ml (p=0.0424 vs. baseline) at 1 yr and 3 yr, respectively) (as in Figure 1). Notably, after DFX usage, 23 patients (32.4%) developed erythroid response according to IWG 2006 criteria; the mean hemoglobin could increase from 7.77+/-1.63 gm/dl (baseline) to 8.25+/-2.60 gm/dl (at 36 month, p=0.6172 vs. baseline), when the average transfusion amount was decreased from 2.3+/-1.4 units (baseline) to 1.6¡Ó0.5 units (at 36 months, p=0.0406 vs. baseline). (as in Figure 2). Ten patients (10/46, 21.7%) had platelet response. For the 38 MDS patients, DFX also could significantly lower serum ferritin level (-590+/-2490 ng/ml (p=0.4095 vs. baseline) and -1310+/-362 ng/ml (p=0.0013 vs. baseline) at 1 yr and 3 yr, respectively) but seemed to have a less extent than that in overall population. Similarly, 10 patients (21.7%) developed erythroid response after DFX use. The mean hemoglobin increment (from 7.67+/-1.67 gm/dl (baseline) to 8.55+/-3.45 gm/dl (at 36 month, p=0.6012 vs. baseline)) and the decrease of average transfusion amount (from 2.1+/-1.2 units (baseline) to 1.6+/-0.6 units (at 36 months, p=0.2943 vs. baseline) were not significant, probably due to low case number (as in Figure 2). Four (4/19, 21.1%) patients experienced platelet response. Conclusion This study showed that the profiles of AEs regarding DFX use for adult anemic patients with transfusion-related iron overload in Taiwan were significantly different from those reported in Western countries. The AE-related discontinuation rate was also relatively low. An expected efficacy to lowering serum ferritin by DFX, and a significantly degree of hematological improvement was noted, too. Table 1. Drug-related adverse events, for all events with incidences > 5% and all grade 3-4 events: Table 1. Drug-related adverse events, for all events with incidences > 5% and all grade 3-4 events: Figure 1. Figure 1. Figure 2. Figure 2. Disclosures Chang: Novartis: Honoraria.

scholarly journals Correlation between serum ferritin level, cardiac and hepatic T2-star MRI in patients with β-thalassemia major in Al-Diwaniya thalassemia center

2018 ◽  
Vol 9 (SPL1) ◽  
Author(s):  
Alaa Mutter Jabur Al-Shibany ◽  
AalanHadi AL-Zamili
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Iron Level ◽  
The Mean ◽  
T2 Mri

Patients with transfusion dependent thalassemia major is often associated with iron overload. Proper use of iron chelators to treat iron overload requires an accurate measurement of iron levels. Magnetic resonance T2-star (T2* MRI) is the preferred method to measure iron level in the liver andthe heart. The goal of our study was to see if there is an association exists between serum ferritin level and T2* MRI results in patients with beta thalassemia major.This study was done in Al-Diwaniya Thalassemia center,Maternity and children teaching hospital,Iraq. During the period from 1st of January to 31st of October. Fifty eight patients with a diagnosis of beta thalassemia major were enrolled in the study. They were older than five years old,transfusion dependent and on chelation therapy. Hepatic and Myocardial T2*MRI and the mean serum ferritin levels were measured during the study period for all patients.There is a significant correlation was observed between serum ferritin level and cardiac T2*MRI (p=0.018 ). also a significant correlation was observed between serum ferritin and hepatic T2*MRI (p=0.02). Neither cardiac T2* MRI nor hepatic T2* MRI show any correlation with the mean age.our study also showa positive correlation between the patients withcardiac T2* MRI and the development of diabetes mellitus in contrast to hepatic T2* MRI in which there is no any correlation. Hypothyroidism was observedno correlation with either cardiac or hepatic T2* MRI.Our results showed a positiveassociation between hepatic, cardiac T2*MRI and serum ferritin levels.

The role of iron chelation activity of wheat grass juice in patients with myelodysplastic syndrome

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 7012-7012 ◽  
Author(s):  
S. Mukhopadhyay ◽  
J. Basak ◽  
M. Kar ◽  
S. Mandal ◽  
A. Mukhopadhyay
Keyword(s):  
Myelodysplastic Syndrome ◽  
Serum Ferritin ◽  
Crude Extract ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Performance Status ◽  
Iron Chelation ◽  
Iron Chelator ◽  
The Mean ◽  
Age Range

7012 Background: A pilot study with wheat grass juice in major thalassaemia patients was done by a group of clinicians in IPGMR, Chandigarh, India. We performed a study of 200 patients of intermediate thalassaemia with wheat grass juice and found 80% patients becoming transfusion independent. During the study in majority of the patients, serum ferritin level was significantly less as compared to pretreatment values. The aim of our study was to see the effect of wheat grass juice in reducing Ferritin level in myelodysplastic syndrome and also do the biochemical analysis of the wheat grass juice. Methods: During period from January 2003 to December 2007 we selected 20 patients of transfusion dependent myelodysplastic syndrome in the oncology department of Netaji Subhash Chandra Bose Cancer Research Institute. The age range of the patients was 42 years to 72 years (median 55 years). The fresh leaves of 5–7-day-old wheat grasss including stems were made fresh juice and had given 30 mL of juice daily to all 20 patients for continuous 6 months. Wheat grass juice was analyzed by column chromatography and found to be rich in oxalic and malic acid which might have some role in dietary absorption of iron from intestine. Beside that the wheat grass juice was found to contain two unique active ingredients with iron chelating property which was performed by deoxyribose degradation assay. We compared aqueous soluble extract of 5–7-day-old plant and dose-dependent study showed a significant iron chelating activity of crude extract in comparison to known standard iron chelator desferroxamine (DFO). The active compounds of crude extract of wheat grass may chelate catalytic iron in iron overload disorders when taking systematic dose. Result: The mean serum Ferritin level of the patients was 2,250 (range 650–4,800) before wheat grass treatment. The mean reduced to 950 (range 68–1680) (p < 0.0001). The performance status was improved from 60% to 80% (Karnofsky) after wheat grass treatment. The mean interval between transfusions was found increased. Conclusions: Wheat grass juice is an effective iron chelator and its use in reducing serum ferritin should be encouraged in myelodysplastic syndrome and other diseases where repeated blood transfusion is required. No significant financial relationships to disclose.

Oral Chelator Deferiprone in Adult with Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4615-4615
Author(s):  
Ruben Nzouakou ◽  
Anoosha Habibi ◽  
Ketty Lee ◽  
Alain Luciani ◽  
Jean-François Deux ◽  
...  
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Cardiac Iron ◽  
Median Level ◽  
Cardiac Iron Overload ◽  
The Mean

Abstract Abstract 4615 INTRODUCTION Long-term blood transfusion is essential for patients with sickle cell disease (SCD) in case of cerebral vasculopathy, organ dysfunction, leg ulcer, failure or intolerance of hydroxyurea treatment. Secondary iron overload is a factor of morbidity and mortality by organ damage. In practice, three chelators are available: deferoxamine (DFO) which is administrated by subcutaneous infusion and therefore source of poor compliance in SCD. Deferasirox (DFX), the new oral chelator is the first line therapy since 2007. Deferiprone (DFP) is an option when DFX or DFO are contraindicated or inadequate. However, DFP has no approval for SCD. The purpose of this study is to describe the characteristics of SCD patients treated by DFP. METHODOLOGY The patients included in this study arise from the group of the patients with long-term blood transfusion by manual exchange in Henri Mondor's SCD center, and in whom iron overload is treated by DFP. The monitoring of iron overload is obtained by regular serum ferritin level, combined liver and heart MRI. Only one iron measure by MRI is available for each patient throughout the study. RESULTS Nine patients (8 SS and 1 Sβ0thal) are included: 5 men and 4 women. The mean age is 44.2 years (22 to 64 years). The median duration of chronic transfusion is 10 years (4 to 27 years). The average dose of DFP is 68 mg / kg / day (50 to 93 mg / kg / day). The median follow-up under DFP is 30 months (7 to 60 months). The median level of serum ferritin before the initiation of DFP is 5830 μg / l (1800 to 9300 μg / l); and the median level of serum ferritin at the end point is 7940 μg / l (4540 to 11300 μg / l). MRI shows an important hepatic iron overload (up to 320 μmol) in all patients and one cardiac iron overload (T2* = 12 ms). Three patients stopped DFP and switch to deferasirox (DFX) as soon as DFX was available. For the other patients, the reason of prescribing DFP instead of DFX was renal failure in 5 patients and DFX related GI symptoms in one patient. No agranulocytosis is observed. The weekly then monthly monitoring of blood count is insured for all patients. No cytolysis by drug's toxicity is observed, except for one patient with liver transplant and who has an active HCV infection. DISCUSSION Serum ferritin level is the easiest marker of iron overload follow-up, but is subject of important variations due to inflammation, hemolysis, and cytolysis. Indeed, MRI is the only one reliable measure. The evidence of cardiac iron overload is proved in one patient, and confirms the importance of this measure on SCD patients. This motivates the edition of guidelines concerning the prevention and monitoring of iron overload among these patients. The dosage of DFP remains reasonable compared to the mean dosage use in other pathologies. This dosage depends of the degree of iron overload and the individual tolerance. Data are not sufficient at these days to evaluate the efficacy of DFP to reduce or to stabilize the level of iron overload. However, we observe globally a good clinical and biological tolerance, even in patients who have organ transplant and therefore have several concomitant treatments. CONCLUSION DFP in patients with SCD is globally well-tolerated, but its efficiency is not proved yet. Approval of DFP for SCD is needed. As life expectancy improves in SCD, more patients will require long-term transfusion and thus iron chelation therapy. Cardiac Iron overload is possible in patients with SCD. So, it would be systematically looked after. Disclosures: No relevant conflicts of interest to declare.

scholarly journals JADENU® SUBSTITUTING EXJADE® IN IRON OVERLOADED Β- THALASSEMIA MAJOR (BTM) PATIENTS: A PRELIMINARY REPORT OF THE EFFECTS ON THE TOLERABILITY, SERUM FERRITIN LEVEL, LIVER IRON CONCENTRATION AND BIOCHEMICAL PROFILES

2018 ◽  
Vol 10 ◽  
pp. e2018064 ◽  
Author(s):  
Vincenzo De Sanctis
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Iron Concentration ◽  
Liver Iron Concentration ◽  
Liver Iron ◽  
Transfusion Therapy ◽  
Biochemical Profiles

Abstract. Introduction: Due to the chronic nature of chelation therapy and the adverse consequences of iron overload, patient adherence to therapy is an important issue. Jadenu ® is a new oral formulation of deferasirox (Exjade ®) tablets for oral suspension. While Exjade®  is a dispersible tablet that must be mixed in liquid and taken on an empty stomach, Jadenu ® can be taken in a single step, with or without a light meal, simplifying administration for the treatment of  patients with chronic iron overload. This may significantly improve the compliance to treatment of patients withβ-thalasemia major (BMT). The aim of this study was to evalute the drug tolerability and the effects of chelation therapy on serum ferritin concentration, liver iron concentration (LIC) and biochemical profiles in patients with BMT and iron overload. Patients and Methods: Twelve selected adult patients BMT (mean age: 29 years; range:15-34 years) were enrolled in the study. All patients were on monthly regular packed cell transfusion therapy to keep their pre-transfusional hemoglobin (Hb) level not less than 9 g/dL. They were on Exjade ® therapy (30 mg/kg per day) for 2 years or more before starting Jadenu ® therapy (14-28 mg/kg/day). The reason for  shifting from Deferasirox ® to Jadenu ® therapy was lack of tolerability,  since most of the patients described Deferasirox ® as not palatable. Lab investigations included montly urine analysis and measurement of their serum concentrations of creatinine, fasting blood glucose (FBG), serum ferritin, alkaline phosphatase (ALP), alanine transferase (ALT), aspartate transferase (AST) and albumin concentrations. LIC was measured using FerriScan ®. Thyroid function, vitamin D and serum parathormone, before and one year  after starting  Jadenu ® therapy, were also assessed. Results: Apart from some minor gastrointestinal complaints reported in 3 BMT patients that did not require discontinuation of therapy, other side effects were not registered during the treatment.  Subjectively, patients reported an improvement in the palatability of Jadenu® compared to Exjade ® therapy in 8 out of 12 BMT patients.  A non-significant decrease in LIC and  serum ferritin levels was observed after 1 year of  treatment with Jadenu ® . A positive significant correlation was found between serum ferritin level and LIC measured by FerriScan ® method. LIC and serum ferritin level correlated significantly with ALT level (r = 0.31 and 0.45 respectively, p < 0.05). No significant correlation was detected between LIC and other biochemical or hormonal parameters. Conclusion: Our study shows that short-term treatment with Jadenu ® is safe but is associated with  a non-significant decrease in LIC and serum ferritin levels. Therefore, there is an urgent need for adequately-powered and high-quality trials to assess the clinical efficacy and  the long-term outcomes of new deferasirox formulation.

scholarly journals Importance of Serum Ferritin Level for Early Diagnosis and Differentiation in Patients with Kawasaki Disease with Macrophage Activation Syndrome

Children ◽  
2021 ◽  
Vol 8 (4) ◽  
pp. 269
Author(s):  
Da Eun Roh ◽  
Jung Eun Kwon ◽  
Hee Joung Choi ◽  
Yeo Hyang Kim
Keyword(s):  
Kawasaki Disease ◽  
Clinical Features ◽  
Serum Ferritin ◽  
Screening Test ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Early Screening ◽  
Activation Syndrome

We aimed to evaluate the utility of the serum ferritin level as an early screening test of Kawasaki disease with macrophage activation syndrome (KD-MAS). We analyzed the serum ferritin levels on the first day of admission and the clinical progress of patients diagnosed with complete or incomplete KD. Of the 158 patients, 5 were diagnosed with KD-MAS. Conjunctival injection was significantly more frequent in KD group (p = 0.035), although there were no significant differences in other clinical features. On the first day of admission, the serum ferritin level in the KD-MAS group was >500 ng/mL, which was higher than that in the KD group (p = 0.001). In the KD-MAS group, total bilirubin, triglyceride, and lactate dehydrogenase (LDH) were significantly higher, and erythrocyte sedimentation rate (ESR), total protein, albumin, and fibrinogen were significantly lower than the KD group (p < 0.05). Four patients were diagnosed with MAS within 7 days after admission, and 4 (80%) patients with KD-MAS survived. In conclusion, carrying out an early ferritin screening test is important in patients with principal clinical features that may suspect KD. We propose to include ferritin level in the primary laboratory test to differentiate between KD with and without MAS early.

scholarly journals Mean ferritin level comparison in patients with coronary heart disease and control subjects.

2019 ◽  
Vol 26 (12) ◽  
pp. 2048-2053
Author(s):  
Sindhu Rehman ◽  
Mona Aziz ◽  
Sabeen Fatima ◽  
Sobia Ashraf ◽  
Ambreen Hameed ◽  
...  
Keyword(s):  
Coronary Heart Disease ◽  
Heart Disease ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Binding Capacity ◽  
Ferritin Level ◽  
Total Iron ◽  
The Mean ◽  
Iron Binding Capacity ◽  
And Control

To determine and compare Mean ferritin level in stable diagnosed coronary heart disease and healthy control groups. Study Design: Analytic (case control study). Setting: Department of Hematology, Sheikhs Zayed Hospital, Lahore. Period: Six months (10th October, 2015 to 11th April, 2016). Material & Methods: Serum iron, total iron binding capacity (TIBC), transferrin saturation (TSAT) and serum ferritin level were measured in 75 cases with coronary heart disease(CHD) and 75 controls (health individuals). All collected information was entered into SPSS version 20. Quantitative variables like age and serum ferritin were presented in the form of Mean ± SD for both cases and control. Qualitative variables like gender was presented in the form of frequency and percentages. T-test was used to compare the mean serum ferritin level between cases and controls. p≤ 0.05 was considered statistically significant. Results: Out of 75 patients, 62(82.7%) were males and 13 (17.3%) females .Serum ferritin levels were substantially raised in patient with CHD versus control subjects {1119±272 ng/l versus 82±139 ng/l (p<.001)}. Total iron binding capacity (TIBC) was lower in patient with CHD {274±102µl versus 355±95 µmol/l (p<.000)} compared to healthy subjects. Conclusion: The mean ferritin level in both groups showed statistically significant difference of serum ferritin level in patient with chronic heart disease and control group and serum ferritin level was considerably higher in patients.

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