scholarly journals Pulmonary dysfunction in children with beta thalassemia major in relation with iron overload - a cross sectional hospital based study

2015 ◽  
Vol 6 (5) ◽  
pp. 47-50 ◽  
Author(s):  
A Boddu ◽  
A Kumble ◽  
S Mahalingam ◽  
BS Baliga ◽  
B Achappa
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
P Value ◽  
Pulmonary Dysfunction ◽  
Cross Sectional ◽  
Transfusion Therapy ◽  
Respiratory Dysfunction ◽  
Respiratory Impairment

Background: Previously many patients with thalassemia major died from severe anemia during first decade, but with modern transfusion therapy many now survive beyond childhood. Because of such therapy, however excessive iron deposition occurs in lungs and causing respiratory dysfunction. Respiratory impairment occurs because of defective chelation and repeated transfusions. Objectives: To a) study pattern of respiratory impairment using spirometry, b) estimate iron overload by measuring serum ferritin levels, c) correlate a&b. Methodology:Thalassemia children >7year, on regular blood transfusion were included in the study after getting institutional ethical clearance .Standardised pulmonary function test was done using spirometry. Iron overload was assessed using serum ferritin levels. Severity of pulmonary dysfunction was correlated with serum ferritin levels. Results:Total of 42 children were included 62% were males and 38% were females (with median age 12yrs). By spirometry 95% had restrictive pattern of respiratory dysfunction. Mean ferritin value was 4152. Out of them10 (23.8%) mild, 25(59%) moderate and 5(12%) severe dysfunction based on FEV1 and FVC. The mean ferritin values in severe respiratory dysfunction is 6275 which is significantly higher when compared to moderate (4249) and mild (3066) pattern of respiratory dysfunction. None of the children had evidence of CCF. Significant correlation (p value=0.003) was found between severity of pulmonary dysfunction with ferritin values and also with weight of the child (p value=0.007). No other significant correlation found between severity pattern and transfusion index, age or height. Conclusion:Restrictive pattern is most common pulmonary dysfunction seen in chronic iron overloaded thalassemia major children. Regular blood transfusions with adequate chelation decrease incidence of pulmonary dysfunction. Screening of all thalassemia children using spirometry is need of the hour. DOI: http://dx.doi.org/10.3126/ajms.v6i5.11782Asian Journal of Medical Sciences Vol.6(5) 2015 47-50

Pulmonary Dysfunction in Beta Thalassemia.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3851-3851
Author(s):  
Amit Kandhari ◽  
Anupam Sachdeva ◽  
Neeraj Jain ◽  
Subash C. Arya ◽  
Virender K. Khanna ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Serum Ferritin ◽  
Pulmonary Function Tests ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
P Value ◽  
Age Group ◽  
Pulmonary Dysfunction ◽  
Function Tests ◽  
Abnormal Results

Abstract Thalassemia is one of the commonest hereditary hemolytic anemia. Survival of patients with thalassemia major has greatly improved during recent years, with the introduction of moderate -transfusion programs and chelation therapy with subcutaneous Desferal (deferrioxamine) and/or Kelfer (Deferiprone). The repeated transfusions in thalassemics lead to deposition of iron in different tissues, leading to damage and dysfunction of various systems. Iron deposition is also reported to exist in the lungs at autopsy. Although lung impairment in thalassemic patients has been reported in the 1980’s, yet it is one of the most under evaluated and functionally not well-characterized complication. Since there have been contradictory results reported in literature ranging from restrictive spirometric pattern to an obstructive one, and there is total paucity of data from India, this study was designed to find out the pulmonary function tests of the beta thalassemic patients attending our hospital. A total of 30 cases of beta thalassemia major who were attending the thalassemia clinic at Sir Ganga Ram Hospital were taken up for the study after a written informed consent. These patients were on moderate -transfusion regimen, maintaining a hemoglobin concentration of more than 9 gm %. Patients with asthma and cardiac disease were excluded. Pulmonary function tests done included Forced vital capacity (FCV), Forced expiratory volume in one second (FEV1), FEV1/FVC percent (FEV1 %) and peak expiratory flow rate (PEFR). Results were interpreted as obstructive, restrictive and normal pulmonary Function test (PFT). The data was analyzed using software SPSS version 10. We included three age groups in our study, with total 30 patients in all. Age group 6–10 yr had 9 patients (mean Serum ferritin 2301 ng/ml), 11–15 yr had 11 children (mean Serum ferritin 3315) and >15 yr had 11 children (mean Serum ferritin 5147 ng/ml) in the study. Mean age was 14.46 with standard deviation of 6.75 (mean ferritin was 3682 ng/ml). Overall out of 30 patients 13 had normal PFT and 17 had abnormal PFT (1 had obstructive PFT and 16 had restrictive PFT). In the age group of 6–10 years 7 had normal and 2 had abnormal PFT and in age group 11–15 yr 3 had normal and 6 had abnormal PFT and in age group >15 yr 3 had normal and 8 had abnormal PFT. Of 17 patients on chelation with Desferal alone 11 had normal and 6 had abnormal PFT. 6 patients were on Kelfer alone of which 1 had normal and 5 had abnormal PFT. Of 7 patients who were on both Desferal and Kelfer, 1 had normal and 6 had abnormal PFT. All patients on Kelfer or Kelfer + Desferal aged >10 years except one which may explain reason for increased number of abnormal results in this group. Critical age above which abnormal results were higher was 11.5 years. Mean Ferritin in patients with normal PFT was 2456 ng/ml and 4621 ng/ml for patients with abnormal PFT. (p value 0.037) We found a negative Pearson correlation between FVC (% of predicted) with increasing ferritin (p value 0.038). There was no difference detected between pre and post transfusion PFT results by using paired t test (p value 0.973). In conclusion the major pulmonary dysfunction in our study group is that of restrictive type. The abnormal PFT were found in older age group. FVC was negatively co-related with increasing ferritin level. The number of abnormal results was higher when the serum ferritin was > 4000 ng/ml and blood transfusion had no effect on the PFT.

scholarly journals Pulmonary function tests in children with beta-thalassemia major

2020 ◽  
Vol 35 ◽  
pp. 52-56
Author(s):  
Jayaraj Harsoor ◽  
Vinod H. Ratageri ◽  
C. Shilpa ◽  
Shivanand Illalu ◽  
Prakash Wari
Keyword(s):  
Pulmonary Function ◽  
Serum Ferritin ◽  
Respiratory Symptoms ◽  
Pulmonary Function Tests ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Pulmonary Dysfunction ◽  
Cross Sectional ◽  
Beta Thalassemia Major ◽  
Function Tests

Objectives: The objective of the study was to study the pattern of lung functions in thalassemia major children and correlation of pulmonary function tests (PFTs) with serum ferritin. Materials and Methods: A hospital-based cross-sectional descriptive study done from January 2017 to December 2017. Inclusion criteria: Children with confirmed diagnosis of beta-thalassemia major in the age group of 5–15 years were included in the study. Exclusion criteria: Already diagnosed cases of pulmonary dysfunctions, CHD and RHD were excluded from the study. All enrolled children underwent a detailed clinical history, physical examination and blood sample were sent for Hb and serum ferritin before blood transfusion (BT). PFT was done within 24 h of BT using spirometer (Helios-401). Statistical analysis was done using SPSS (Version22). Results: Forty-five children enrolled in the study and majority of them were <10 years (37 children) with M:F ratio 1.6:1. The pulmonary dysfunction was present in 35 (77.8%), but none of them had respiratory symptoms. The pulmonary dysfunction observed was restrictive 31 (88.5%), obstructive 2 (5.7%), and combined 2 (5.7%). A reduced forced vital capacity (FVC) % in 33 (73.3%), a reduced forced expiratory volume in the 1st second (FEV1%) in 25 (55.5%), a normal FEV1/FVC in 41 (91.2%), and a reduced FEF 25–75% in 23 (51.1%) children were observed. Risk factors such as, age, height, and duration of chelation (>5 years) were significantly associated with pulmonary dysfunction (P < 0.05). There was no correlation between serum ferritin levels and PFT. However, PFT values were found to be decreased in patients with a high serum ferritin (>2500 ng/ml), but these differences were statistically not significant. Conclusion: Abnormal patterns of lung function were common (restrictive type, predominant), even though none of these children had any respiratory symptoms.

scholarly journals Liver Enzymes in Children with beta-Thalassemia Major: Correlation with Iron Overload and Viral Hepatitis

2015 ◽  
Vol 3 (2) ◽  
pp. 287-292 ◽  
Author(s):  
Khaled M. Salama ◽  
Ola M. Ibrahim ◽  
Ahmed M. Kaddah ◽  
Samia Boseila ◽  
Leila Abu Ismail ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Iron Overload ◽  
Viral Hepatitis ◽  
Serum Ferritin ◽  
Liver Enzymes ◽  
Transferrin Saturation ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Elevated Liver Enzymes ◽  
Hcv Antibody

BACKGROUND: Beta Thalassemia is the most common chronic hemolytic anemia in Egypt (85.1%) with an estimated carrier rate of 9-10.2%. Injury to the liver, whether acute or chronic, eventually results in an increase in serum concentrations of Alanine transaminase (ALT) and Aspartate transaminase (AST).AIM: Evaluating the potentiating effect of iron overload & viral hepatitis infection on the liver enzymes.PATIENTS AND METHODS: Eighty (80) thalassemia major patients were studied with respect to liver enzymes, ferritin, transferrin saturation, HBsAg, anti-HCV antibody and HCV-PCR for anti-HCV positive patients.RESULTS: Fifty % of the patients were anti-HCV positive and 55% of them were HCV-PCR positive. Patients with elevated ALT and AST levels had significantly higher mean serum ferritin than those with normal levels. Anti-HCV positive patients had higher mean serum ferritin, serum ALT, AST and GGT levels and higher age and duration of blood transfusion than the negative group. HCV-PCR positive patients had higher mean serum ferritin and serum ALT and also higher age and duration of blood transfusion than the negative group.CONCLUSION: Iron overload is a main leading cause of elevated liver enzymes, and presence of HCV infection is significantly related to the increased iron overload.

scholarly journals Thyroid Function in Chronically Transfused Children with Beta Thalassemia Major: A Cross-Sectional Hospital Based Study

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Suraj Haridas Upadya ◽  
M. S. Rukmini ◽  
Sowmya Sundararajan ◽  
B. Shantharam Baliga ◽  
Nutan Kamath
Keyword(s):  
Thyroid Function ◽  
Serum Ferritin ◽  
Subclinical Hypothyroidism ◽  
Thyroid Dysfunction ◽  
Genetic Disorder ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
P Value ◽  
Blood Transfusions ◽  
Beta Thalassemia Major

Background. Thalassemia is the most common genetic disorder worldwide. Use of iron chelators has improved survival but endocrine complications have become more frequent. The frequency of hypothyroidism in Beta Thalassemia Major (BTM) children ranges from 6 to 30 %. Thyroid dysfunction mainly occurs by gland infiltration, chronic tissue hypoxia, free radical injury, and organ siderosis. Objectives. (a) To evaluate the thyroid function status in chronically transfused children with BTM, in the first and second decade of life and (b) to study the influence of factors like duration and amount of blood transfusions, serum ferritin level, and iron chelation therapy on thyroid function. Methodology. BTM children, 3 years old and above, on regular blood transfusions with serum ferritin > 1500 mcg/l were included in the study. Thyroid function and ferritin assessment was done using ELISA kits. Autoimmune thyroiditis was ruled out by antithyroid peroxidase and antithyroglobulin antibody testing. Results. A study population of 83 children consisted of 49 boys (59%) and 34 girls (41%). 4.8% of the children had evidence of subclinical hypothyroidism. Among them two belonged to the first decade and the other two to the second decade of life. Mean TSH, FT4, and ferritin values among children with thyroid dysfunction were 6.38 ± 0.83 mIU/ml, 1.08 ± 0.45 ng/dl, and 3983.0±1698.30 ng/ml, respectively. The severity of thyroid dysfunction was statistically significantly associated with higher serum TSH values in children in the second decade of life with a p value = 0.001. No other significant correlation was found between oral chelation, amount and duration of blood transfusion, or serum ferritin levels. Conclusion. Subclinical hypothyroidism was the thyroid dysfunction observed in our study. Regular blood transfusions with adequate chelation may decrease incidence of thyroid dysfunction.

scholarly journals Correlation between Pancreatic MRI T2* and Iron Overload in Adult Transfusion Dependent Beta Thalassemia Patients with Growth Retardation: A Single Centre Study in Indonesia

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4907-4907
Author(s):  
Faizal Drissa Hasibuan ◽  
Tubagus Djumhana Atmakusuma
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Growth Retardation ◽  
Transferrin Saturation ◽  
Endocrine Function ◽  
Beta Thalassemia ◽  
Endocrine Disorders ◽  
Cross Sectional ◽  
Excess Iron ◽  
Iron Load

Abstract Correlation Between Pancreatic MRI T2* And Iron Overload in Adult Transfusion Dependent Beta Thalassemia Patients With Growth Retardation : A Single Centre Study in Indonesia Faizal Drissa Hasibuan , MD 1,2 , Tb. Djumhana Atmakusuma , MD, PhD 3, 4 1Department of Internal Medicine, 2Faculty of Medicine Yarsi University Jakarta, Indonesia, 3Medical Hematology - Oncology Division of Internal Medicine Department Cipto Mangunkusumo Hospital, 4Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia Correspondence: [email protected] phone +6281533197733 The prevalence of thalassemia in Indonesia is one of the highest in the world. It is estimated that the prevalence of beta thalassemia carrier is around 3-10%. In 2016, thalassemia center in Cipto Mangunkusumo Hospital (RSCM) Jakarta recorded 9031 patients suffering from major thalassemia in Indonesia. 441 are adult thalassemia patients (age 18 and above) recorded in Kiara thalassemia and hematology-oncology clinics in RSCM. Based on a survey from TIF, endocrine aspect of the thalassemia patients is often ignored by the clinicians. Growth retarded patients are commonly found in the thalassemia clinic in RSCM. Publication regarding the pancreas and its correlation with iron overload in adult beta TDT patients is currently not available in Indonesia. Therefore, the aim of this study was to describe iron overload condition based on the pancreatic MRI T2* and its correlation with beta TDT adult patients who suffer from growth retardation. A cross sectional study was conducted to determine the prevalence of endocrine disorders in adult TDT beta patients, followed by looking for correlation of excess iron load with endocrine function in adult TDT beta patients with growth retardation in adult Thalassemia clinic RSCM Jakarta on December 2017. Patients with HBsAg or Anti HCV positive were excluded. Excess iron is defined as Transferin Saturation (ST) greater than 50% regardless of serum ferritin or serum ferritin (FS) levels greater than 1000 ng/mL regardless of ST or both.The growth retardation is defined as the standing height of the research subject which is lower than the Mid Parental Height (MPH) value of both parents. Pancreatic MRI T2* used magneto avanto Siemens 1,5T with CMR software. We found from 58 patients who followed the study, 32 patients underwent the pancreatic MRI T2* examination with 13 female (40,6%) and 19 male (59,4%). Patients with homozygous beta thalassemia are 16 people (50%) and beta/HbE thalassemia 16 people (50%). The Proportion of low pancreatic MRI T2* values was found to be 87.5%, with moderate hemosiderosis in 13 patients (40.63%) and severe hemosiderosis not found (Table 2). The age range of the study subjects was relatively young with a median age of 21 years. Although the median body weight of study subjects was 42 kg, the median BMI still included in normal range. Excess iron content in this study was assessed with serum ferritin, obtained median 4982.5 ng/mL and transferin saturation with a median of 100%, indicates the subject of research are in a state of excess iron load. This is due to the possibility of inflammation, inadequate use of chelation, hemolysis in thalassemia, hypertransfusion to achieve the target of 12 g/dL for women and 13 g/dL for men. There was no significant correlation between serum ferritin and pancreatic MRI T2* value, nor did a significant correlation between transferrin saturation with pancreatic MRI T2* value (Table 3). In this study, there was a high proportion of subjects with low pancreatic MRI T2* value of 28 subjects (87,5%), divided into 15 mild hemosiderosis (46,87%), 13 moderate hemosiderosis (40,62%) and none of severe hemosiderosis. Our study is the first study which look for the correlation of the excess iron load (serum ferritin and transferrin saturation) with endocrine function in adult TDT beta patients with retardation of growth in Indonesia. This research has limitations. First, it was a cross sectional study so it is not known exactly the beginning of endocrine disorders in the subject . The second limitation, analysis of iron chelation therapy did not do in this study. Finally, we concluded that there was no correlation between pancreatic MRI T2* and iron overload based on serum ferritin and transferrin saturation. Further longitudinal studies in adult TDT patients with thalassemia who have not and have retarded growth were needed. Disclosures No relevant conflicts of interest to declare.

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.

Correlation of Serum Ferritin Levels and Liver Iron Concentration Determined by R2* MRI in Patients with Thalassemia Intermedia.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3818-3818
Author(s):  
Ali Taher ◽  
F. El Rassi ◽  
H. Ismaeel ◽  
S. Koussa ◽  
A. Inati
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Care Center ◽  
Iron Concentration ◽  
Iron Chelation ◽  
Thalassemia Intermedia ◽  
Liver Iron Concentration ◽  
Liver Iron ◽  
Cross Sectional ◽  
Transfusion Therapy

Abstract Background: Unlike patients with thalassemia major (TM), those with thalassemia intermedia (TI) do not require regular blood transfusion therapy but remain susceptible to iron overload due to increased intestinal iron uptake triggered by ineffective erythropoiesis. TI patients can accumulate 1–3.5 g of excess iron per year, and effective monitoring of iron burden is an important element of patient management. Assessment of serum ferritin (SF) levels is a convenient and widely used method, and a correlation between SF and liver iron concentration (LIC) has been demonstrated in patients with TM. SF levels may, however, be a poor indicator of LIC in patients with TI and the limited data available on the SF:LIC correlation prove equivocal; in fact, reports suggest a discrepancy between LIC and SF in patients with TI. This is the largest study to use R2* MRI to evaluate the SF:LIC correlation in patients with TI. Methods: This was a cross-sectional study of randomly selected, infrequently/non-transfused TI patients treated at a chronic care center in Hazmieh, Lebanon. Patient charts were reviewed and a medical history was compiled. Blood samples were taken for SF assessment, and LIC was determined by R2* MRI. Results: Data from 74 TI patients were included in this analysis (33 male, 41 female; mean age 26.5 ± 11.5 years). Of this group, 59 (79.7%) patients were splenectomized, 20 were transfusion-naive, 45 had received several transfusions in their lifetime but none in the past year, and 9 patients were regularly transfused 2–4 times per year. Overall mean SF values were 1023 ± 780 ng/mL (range 15–4140); mean LIC levels were 9.0 ± 7.4 mg Fe/g dry weight [dw] (range 0.5–32.1). In contrast to previous findings, a significant positive correlation between mean LIC and SF values was seen in the whole group (R=0.64; P&lt;0.001), and in a subset of splenectomized patients (R=0.62; P&lt;0.001). In comparison with data obtained from a randomly selected group of patients with TM treated at the center, SF levels in TI were seen to be significantly lower, while the mean LIC values were similar in both groups of TI and TM. For a given LIC, SF values were lower in patients with TI than those with TM (Figure). Conclusions: Evaluation of iron levels shows that many patients with TI have SF and LIC levels above the recommended threshold levels, indicating a risk of significant morbidity/mortality. Similar to TM, a significant correlation between SF and LIC was observed in patients with TI; however, the relationship between SF and LIC was different between TI and TM (for the same LIC, the SF values in TI were lower than those in TM). Therefore, use of the current threshold for iron overload based on SF values in TM will lead to significant underestimation of the severity of iron overload in patients with TI. This may result in delayed chelation therapy, and expose patients to morbidity and mortality risks associated with iron overload. Disease-specific management approaches are therefore required in patients with TI. This includes either regular assessments of LIC, ideally by non-invasive R2* MRI, or lowering the SF threshold for initiating iron chelation in patients with TI. Figure Figure

Pubertal status and its relation with serum ferritin level in thalassemia major patients

2019 ◽  
Author(s):  
Morteza Alijanpour Aghamaleki MD ◽  
Ahmad Tamaddoni MD ◽  
Hassan Mahmoodi Nesheli MD ◽  
Mahmoud Hajiahmadi PhD ◽  
Motahareh Amouzadeh Samakoush MD ◽  
...  
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Hypogonadotropic Hypogonadism ◽  
Thalassemia Major ◽  
Careful Examination ◽  
Delayed Puberty ◽  
P Value ◽  
Cross Sectional ◽  
Pubertal Status

Background: Thalassemia major (TM) is one of the most common hereditary anemia with multiple endocrinopathies (especially hypogonadism). So, we evaluated the rate of delayed puberty (DP) and its relation with serum ferritin level in patients. Materials and Methods: This cross-sectional (descriptive-analytical) study was conducted on 100 patients with TM between 14-64 years old, admitted to Amirkola Thalassemia Center, Babol, Iran, in 2016. The pubertal status, (Marshall-Tanner scale), existance of DP, and its different types were evaluated. Mean serum ferritin level was measured and the data were classified to three groups of <1500, 1500-2500, and >2500 ng/ml. Data were analyzed using SPSS (version20). Results: Out of 100 patients, 64 (64%) and 36 (36%) were female and male, respectively. Considering age, 23, 77 patients (%) were under and over 20 years old, respectively. Totally, 69 (69%) of them had DP, of whom 64 (92.8%) ones had secondary (central) hypogonadotropic hypogonadism. Mean serum ferritin level (±SD) was 2707.94±1683.42 ng/ml. In addition, 26, 29, and 45 patients had ferritin level <1500, 1500-2500, and >2500 ng/ml, respectively. Thirty two patients with DP (46.4%) had ferritin level above 2500 ng/ml (p-value= 0.623). Conclusion: The results showed a high frequency of DP in TM patients, requiring careful examination and follow-up in terms of puberty for early diagnosis and proper treatment to improve their quality of life, and prevention of the complications like osteoporosis. We couldn't find any significant relationship between serum ferritin level and hypogonadism, even for cases who received enough iron chelators. Keywords: Delayed Puberty, Ferritin, Hypergonadotropic Hypogonadism, Hypogonadotropic Hypogonadism, Thalassemia Major    

scholarly journals Reproductive Hormone in Transfusion dependent βeta thalassemia major patients.

2019 ◽  
Vol 26 (12) ◽  
pp. 2179-2183
Author(s):  
Sana Fatima Baloch ◽  
Ikram Din Ujjan ◽  
Sadia Shahmeer ◽  
Nazia Hafeez ◽  
Kiran Amir ◽  
...  
Keyword(s):  
Luteinizing Hormone ◽  
Thalassemia Major ◽  
Cross Sectional Study ◽  
Beta Thalassemia ◽  
P Value ◽  
Transfusion Rate ◽  
Cross Sectional ◽  
Endocrine Profile ◽  
History Of ◽  
Study Setting

Objective: To evaluate endocrine profile in transfusion dependent beta-thalassemia major patients. Study Design: Descriptive cross sectional study. Setting: Department of Pathology Liaquat University of Medical and Health Sciences, Jamshoro /Hyderabad and Thalassemia Centre Hyderabad. Period: 6 months from January 2018 to June 2018. Material & Methods: Cases already diagnosed with β- thalassemia major with recurrent blood transfusions (>20 transfusions or transfusion period over 2 years) both genders were included. All the patients went through routine laboratory checkups including Testosterone hormone, Luteinizing Hormone, and Follicle Stimulating Hormone. Data was recorded in preplanned proforma. Results: Overall 114 subjects were studied and their mean age was found to be 12.38+5.71 years. Females remained in majority as 56.1%. Mean of follicle stimulate hormone (FSH) and luteinizing hormone (LH) level were 4.60+4.88 and 5.39+8.39 respectively. Most of the patients, 35.1% had >300 of transfusion rate. Ferritin levels were significantly correlated with patients with history of transfusion rate >300; p-value 0.003. Testosterone level’s mean was considerably high among patients presented with transfusion rate of 201 to 300 and >300 transfusions; p-value 0.006. Likewise, mean of FSH and LH level was as well significantly high among the female subjects with history of transfusion rate of 201 to 300 and >300 transfusions; P-Values 0.019 and 0.026 respectively. Conclusion: Frequent hypogonadism was noted among thalassemia patients. Very low level average of FSH, LH and testosterone was seen.

scholarly journals Estimation of iron overloads using oral exfoliative cytology in beta-thalassemia major patients

CytoJournal ◽  
2016 ◽  
Vol 13 ◽  
pp. 6 ◽  
Author(s):  
Swati Leekha ◽  
Amit Kumar Nayar ◽  
Preeti Bakshi ◽  
Aman Sharma ◽  
Swati Parhar ◽  
...  
Keyword(s):  
Iron Overload ◽  
Prussian Blue ◽  
Serum Ferritin ◽  
Thalassemia Major ◽  
The Body ◽  
Transport Cost ◽  
Beta Thalassemia ◽  
Safe Procedure ◽  
Beta Thalassemia Major ◽  
Mucosal Cells

Background: Iron overload is a medical condition that occurs when too much of the mineral iron builds up inside the body and produces a toxic reaction. Thalassemia is a genetic disorder of hemoglobin synthesis, which requires regular blood transfusion therapy, and the lack of specific excretory pathways for iron in humans leads to iron overload in the body tissues. It is a major cause of morbidity and mortality in these patients. The estimation of iron levels in exfoliated buccal mucosal cells may provide a simple, noninvasive, and a safe procedure for estimating the iron overload by using the Perls’ Prussian blue stain. Methods: Smears were obtained from buccal mucosa of 40 randomly selected beta-thalassemia major patients and 40 healthy subjects as controls. Smears were stained with Perls’ Prussian blue method. Blood samples were taken for estimation of serum ferritin levels. Images of smears were analyzed using the software image J software version 1.47v and correlated with serum ferritin. Results: Perls’ positivity was observed in 87.5% of thalassemic patients with a positive correlation to serum ferritin levels. Conclusion: The use of exfoliative buccal mucosal cells for the evaluation of iron overloads in the body provides us with a diagnostic medium that is noninvasive, easy to collect, store, and transport, cost effective, and above all reliable.

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