Frequency of Neurological Manifestations in β-Thalassaemic Patients in Zagazig University Hospitals (original) (raw)
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Journal of the Dow University of Health Sciences, 2020
On the other hand, imaging devices like magnetic resonance imaging (MRI) are employed to evaluate the iron chelation therapeutic effects based on signal intensity on T1 and T2 weighted sequences, as lower 8 signal intensity suggests iron overload. Such intervention expedites the assessment of iron levels in the body before the onset of dysfunction of organs and 10,11 hemosiderosis could set in. Many studies showed that beta thalassemia affects the neurological conditions of those individuals who suffer 12,15 from this disease. However, these neurological anomalies have not been fully compiled or discussed yet. In this article, we will discuss these neurological impairments caused by beta-thalassemia and their possible curative therapy to alleviate some of these conditions. EXTRAMEDULLARY HEMATOPOIESIS Extramedullary hematopoiesis (EMH) is a homeostatic phenomenon that arises among chronic anemic patients in which hematopoietic foci are formed other than bone marrow to compensate the circulatory 16-18 needs. In 1954, Gatto and their colleagues reported
Polyneuropathy and myopathy in beta-thalassemia major patients
Annals of Hematology, 2018
The thalassemias are the most common single gene disorder in the world. Nowadays, the average life expectancy of patients in developed countries has increased significantly, while, there was an increase of complications. We aimed to investigate peripheral neuropathy and myopathy in this patient group using a neurophysiological study. We performed nerve conduction studies and electromyography of upper and lower extremities on 36 beta-thalassemia major (β-thal) patients. The electrophysiological findings were correlated with demographic data and laboratory parameters of the disease. Patients with β-thal present polyneuropathy or myopathy at (50%). Polyneuropathy was detected in (38.9%) and myopathy in (27.8%), while polyneuropathy and myopathy were present at (16.7%) with an overlap of the diseases in 1/3 of the patients. There was not a statistically significant correlation of polyneuropathy and myopathy with age, sex, splenectomy, nor with respect to laboratory parameters, hemoglobin, and ferritin. However, there was a statistically significant correlation of polyneuropathy and myopathy with iron overload, as recorded by the magnetic resonance imaging (MRI) of the heart and the liver. Our findings suggest that iron overload plays a key role in the pathogenesis of polyneuropathy and myopathy in β-thal patients, and performing heart and liver MRI for the prediction of such lesions in an annual basis is warranted.
Neurological Manifestations of Thalassemias and Hemoglobinopathies
Neurological manifestations of hemoglobinopathies may range from mild disabling symptoms such as chronic pain to conditions that may incapacitate life such as paraplegia or stroke. The range of manifestations varies not only with the underlying condition but also with the time course of the disease. The pathophysiological mechanisms of such varied manifestations are not yet fully understood. In this review, we discuss the current understanding and management of neurological manifestations in patients with various hemoglobinopathies, touching upon the diseases most prevalent in India.
Neurophysiologic and intellectual evaluation of beta-thalassemia patients
Brain & Development, 2006
In order to detect involvement of the central and peripheral nervous system in beta-thalassemic patients, 32 children and young adults (mean age 14.5±6.4 years) participated in a systematic neurophysiologic and intellectual prospective study. All patients were in a regular transfusion program, receiving subcutaneous desferrioxamine chelation and maintaining a mean serum ferritin level of 2,101.56±986.32 ng/ml. Study patients underwent neurophysiologic evaluation consisting of brainstem auditory, visual and somatosensory evoked potential examination (BAEP, VEP, SEP) as well as motor and sensory nerve conduction velocity studies (MCV, SCV). Additionally, the verbal, performance and total IQ were assessed in patients under 16 years of age using the Weschler Intelligence Scale for Children (WISC-III). The incidence of abnormal BAEP, VEP, SEP and NCVs was 0, 3.12, 3.12 and 18.75%, respectively, findings comparative to or better than previously reported. On the contrary, the prevalence of abnormal total IQ score was considerably high (36.4%), not correlating, however, to any of the parameters assessed (age, sex, ferritin level, BAEP, VEP, SEP, NCV). Factors associated with chronic illness, rather than the disease per se, could play a potential role in the development of cognitive dysfunction in beta-thalassemia patients.
β -Thalassemia: A Current Overview
International Journal of Health Sciences and Research, 2016
Beta-thalassemia is a group of hereditary blood disorders characterized by reduced or absent beta globin chain synthesis, resulting in chronic hemolytic anemia and ineffective erythropoiesis. Individuals with β-thalassemia have widely variable clinical manifestations, extending from nearly asymptomatic to severe anemia requiring lifelong regular blood transfusions and complicated by multiple organ damage. Chronic transfusion inevitably leads to iron overload which necessitates iron chelation therapy. Current overview about the disease clinical picture, method of diagnosis, complications and principles of management is going to be discussed in brief.
Beta-Thalassemia Presenting as an Acute Neurological Complication: A Case Report
2011
Beta-thalassemia is an inherited defect in the rate of synthesis of one or more beta-globin chains of hemoglobin resulting in anemia, hemolysis and ineffective erythropoeisis. Extramedullary hematopoietic tumors may develop in extreme cases of dyserythropoiesis. Acute neurological complications in patients with beta-thalassemia have been reported in association with cerebral ischemia, spinal cord fractures and compression from extramedullary hematopoietic tumors. However extramedullary hematopeietic tumors in the setting of acute blood loss leading to neurological complications have never been reported. We report this case of a 27-year-old woman with beta-thalassemia who presented with acute neurological defi cit during immediate postoperative period of cesarean section associated with signifi cant drop in hemoglobin. The Magnetic Resonance Imaging of the thoracic and lumbosacral spine revealed compressive intra-spinal and para-spinal extra-medullary hematopoietic tissue. Patient's symptoms improved dramatically with conservative management.
Polyneuropathy Associated with Severe Iron Overload and Oxidative Stress in β-Thalassemia Patients
Indian Journal of Hematology and Blood Transfusion, 2018
To investigate the frequency of peripheral neuropathy in patients with b-thalassemia, and to assess its relation to iron overload and oxidative stress. Sixty bthalassemia patients with mean age of 19 ± 4.9 years were recruited. Serum ferritin was quantitatively assessed by enzyme-linked immunoassay and biomarkers of oxidative stress were estimated calorimetrically. Electrophysiological studies using NEMUS 2, Galileu Software were carried out. The patients were separated into two groups: those with abnormal nerve conduction studies (NCS) {Group A; N = 38} and those with normal NCS {Group B; N = 22}. Thirty-eight (63.3%) patients had axonal motor neuropathy as evidenced by abnormal NCS (group A), they showed higher mean serum ferritin (p \ 0.01), higher mean malondialdehyde (MDA) (p \ 0.01), and lower mean nitrous oxide, total antioxidant capacity, paraoxonase-1 (PON1) (p \ 0.01) compared to group B. Bivariate analysis of NCS data demonstrated that abnormal NCS were more frequent in splenectomized patients (p = 0.002), and poorly-chelated patients with serum ferritin C 2000 ng/ml (p = 0.001). Significant variables associated with abnormal motor NCS were entered in stepwise regression analysis and only elevated serum ferritin (p = 0.01) was independently associated with abnormal motor NCS (p = 0.02; 95% CI 1.433-51.791). None of the studied patients had sensory neuropathy or myopathy. Peripheral motor neuropathy may occur in b-thalassemia patients at a high frequency, regardless of their age and gender. Severe iron overload may contribute to the pathogenesis of neuropathy. Other factors including chelation therapy, splenectomy, and oxidative stress might have an enhancing effect that couldn't be proved in this study.
The Professional Medical Journal
Thalassemia is a single gene inherited blood diseases. Its causes abnormalitiesin in various human organ including oral and maxillofacial regions. A case report of 25 yearsold, β thalassemia patient from Karachi Pakistan. It is evident from this case report that if bloodtransfusion start regularly from early age and iron overload is controlled by proper drugs, thenintensity of manifestation in oral and maxillofacial regions becomes less and thus increasinglife span probability.
The professional medical journal, 2018
… Background: Β-thalassemia is a common single genetic disorder in Pakistan with about 8% gene frequency and roughly 10 million carriers. Growth impairment leading to short stature in thalassemic patients is an important cause of morbidity. Objectives: To determine the frequency of short stature in children with muti-transfused β-Thalassemia major. Study Design: Descriptive cross sectional study. Place and Duration of Study: Pediatric Department, Allied Hospital, Faisalabad from December 2015 to May 2016. Patient & Methods: Ninety multi-transfused β-thalassemia major patients diagnosed by hemoglobin electrophoresis between 06 to 10 years of age of either gender were included. Patients with β-thalassemia major with a concomitant chronic illness like congenital heart disease, tuberculosis, celiac disease and immunodeficiency and those with familial short stature as determined by history and mid-parental height were excluded. Results: Out of 90 cases, 56.67% (n=51) were between 6-8 years of age while 43.33% (n=39) between 9-10 years of age, mean + SD was 7.85+1.50 years, 51.11%(n=46) male and 48.89%(n=44) were females. Frequency of short stature in children with β-thalassemia major receiving multiple transfusion was 41.11% (n=37) while 58.89% (n=53) had normal stature. Conclusion: The frequency of short stature is high among β-thalassemic multi-transfused children. It is recommended that every patient with β-Thalassemia major, should be sort out for short stature. However, surveillance of growth and development in these patients is important.