Assessmrent of iron overload in thalassaemic patients by magnetic resonance imaging: a pilot study.

The patients of thalassaemia major need repeated blood transfusion which leads to excess iron deposition in various organs like liver, heart, pituitary etc. This iron accumulation causes various complications and ultimately organs' failure. There is no non-invasive, standard and reliable method to know the status of iron overload in various organs of the body. This paper attempts to use magnetic resonance imaging to know the liver iron overload in 8 thalassaemic patients as a pilot study. Eight children suffering with thalassaemia and 3 controls who were the normal siblings of the patient group underwent magnetic resonance imaging of the abdomen using spin-echo T, weighted sequence. Blood serum ferritin levels in the patients' group were also determined on the same day of magnetic resonance imaging examination. It was observed that the ratio of magnetic resonance imaging signal intensity (in spin-echo T1 weighted image) in paraspinous muscle to liver was significantly different in normal control (0.65) compared to that in thalassaemia patients (2.1 to 11.4 depending upon extent of iron deposition). The magnetic resonance signal intensity ratio correlated with the blood serum ferritin level of patients (p = 0.01) which is generally taken as indirect measure of body iron burden. Spin-echo sequence is the simplest imaging sequence and it increases the chance of its routine use. The study concludes that magnetic resonance imaging has good potential to quantify the liver iron deposition non-invasively and may denote the efficacy of iron-chelation therapy which is used to reduce the body iron burden in these patients.

Spinal cord compression due to intraspinal extramedullary hematopoiesis in thalassemia intermedia

Extramedullary hematopoiesis is a common phenomenon in thalassemia. During the disease there are very rare occasions when compensatory hematopoietic tissue is located in the intraspinal epidural space, causing spinal cord compression. This complication requires urgent neurosurgical consideration and decision for further treatment. We present a case of thoracic spinal cord compression secondary to extramedullary hematopoiesis in thalassemia intermedia, treated with irradiation therapy. The therapeutic options are discussed, and the need for more explicit therapeutic directions is highlighted

Peripheral neuropathy in thalasemia

Patients with thalassemia may complain of numbness and weakness of the lower extremities. The aim of the study was to document whether these patients suffer from a polyneuropathy and to determine any contributing factors for the development of neuropathy. We examined 30 patients with thalassemia major and intermedia, clinically and electrophysiologically. We corre_lated these findings with demographics, blood status, and treatment and compared electrophysiologic data with 30 age- and sex-matched normal subjects or historical controls. We found that 78% of thalassemic patients suffered from a mild sensory polyneuropathy. The neuropathy seemed to be worse in the intermedia type. Thalassemic patients who received blood transfusions and deferoxamine had better nerve function than those who did not, irrespective of the dose of deferoxamine. The neuropathy was worse for older patients, irrespective of sex. The hemoglobin level, and the fact that some patients underwent splenectomy, did not affect the status of the patients' nerves. Patients with thalassemia may suffer from a sensory polyneuropathy especially as they grow older and if they are not opti_mally treated

Thalassemia syndromes in Saudi Arabia meta-analysis of local studies

The geographical distribution of Alpha and Beta-Thalassemias differ markedly. Alpha-Thalassemia being particularly prevalent in Southeast Asia and Beta-Thalassemia in the Mediterranean basin. Thalassemia syndromes are common in Saudi Arabia: the Beta-Thalassemia genes occur with variable frequency in different regions of Saudi Arabia and both B+ and Bo thalassemia have been reported. Alpha-Thalassemia is also highly prevalent here and the interaction with the sickle cell gene is commonly observed. Over the last few years, a great deal of information regarding the clinical, molecular and management of these disorders has accumulated in the literature. In this paper we summarize some of the recent studies on the subject along with our experience and our attempt to highlight a number of questions still awaiting answers

Changes in early and late diastolic filling pattern in patients with B-thalassemia major before and after captopril therapy

Diastolic left ventricular function has been assessed by echo- Doppler examination in 23 patients [mean age = 11.6 years] with B- thalassemia major. The results were compared with those obtained from 10 normal age-matched control individuals. Parameters showed statistically significant difference between patients and controls were peak flow velocity in late diastole [A], ratio of peak flow velocity in early to late diastole [E/A], isovolumic relaxation time [IVRT], left atrial [LA] and aortic root [Ao] dimensions, left ventricular posterior wall thickness in diastole [LPWdiast], interventricular septal thickness [IVS], left ventricular end diastolic [LVEDD] and end systolic [LVESD] dimensions. Captopril [Capoten], an angiotensin converting enzyme inhibitor, was given to these patients in the therapeutic dose [0.5-1 mg/kg body weight/24 hours]. A follow up study was done for 15 patients after 10-30 days [intermediate study]. The effect of captopril appeared in the form of decrease of A wave with an increase of E/A ratio, which probably suggest improved myocardial diastolic function. Furthermore, left ventricular end diastolic dimension displayed statistically significant decrease

Hypertension perioperative splenectomy in thalassemic children.

We retrospectively studied the intraoperative hemodynamic changes in 100 thalassemic children undergoing general anesthesia for elective splenectomy. They were divided into 2 groups owing to the postoperative hemodynamic response. Eighty-four patients who had an unremarkable postoperative course were group 1, while 16 patients who developed immediate postoperative hypertension were group 2. There were no differences between the groups regarding age, body weight, sex, anesthetic time and operation time, except for the preoperative hematocrits of group 1 patients were slightly but significantly higher. Both groups similarly showed significant increase of the systolic as well as diastolic blood pressure throughout the anesthetic course. The heart rate was significantly increased at the beginning but declined to normal toward the end of the operation. Although none of the patients died postoperatively, all of group 2 patients needed aggressive treatment with diuretic and antihypertensive drugs. Despite the proper management, 3 of 16 patients developed convulsion and one of them had persistent neurological deficit. Since intraoperative and postoperative hypertension which commonly occur in these patients may lead to more serious neurological complications, the authors suggest that careful hemodynamic monitoring must be considered for all thalassemic children undergoing general anesthesia for splenectomy.

An echo-cardiographic (M-mode & 2D) analysis of thalassaemia major.

25 cases of thalassaemia major were studied by 2D and M-mode echocardiography. A significantly increased (p less than 0.001) mean value (100.8 +/- 27.37 msec, range 80 to 140 msec) of A2-E (early relaxation period) interval on M-mode was observed in thalassemia in comparison to mean level (82.6 +/- 5.7, range 60 to 100 msec) of control population. No significant differences were noted in FS % (fractional shortening) and EF% (ejection fraction) when compared to corresponding normal values respectively. Mean serum iron concentration (142.2 +/- 29.1 micrograms/dl, range 102 to 192 micrograms/dl) was significantly higher in thalassaemia as compared to normal population (mean 106.3 +/- 11.4 micrograms/dl, range 75 to 120 micrograms/dl). There was also a direct correlation between serum iron concentration and A2-E interval. 11 patients (44%) showed abnormal A2-E interval but only 3 patients (12%) showed abnormal percentage of FS and EF. It is therefore concluded that A2-E interval will help to detect early left ventricular dysfunction much before overt and irreversible heart failure becomes manifest and which will also help to optimise transfusion and chelation therapy.

Metaplasia mielóide em gânglios mediastinais em portador de anemia de Cooley: diagnóstico através de punçäo mediastinal / Myeloid metaplasia in mediastinal ganglia in patients with Cooley's anemia: diagnosis by mediastinal puncture

Apresenta-se um caso de metaplasia mielóide em gânglios linfáticos mediastinais em paciente portador de anemia de Cooley, chamando a atençäo por tratar-se de evento raro, que é diagnóstico diferencial das adenomegalias mediastinais. Ressaltam, também, o valor da punçäo mediastinal e do exame citológico no diagnóstico desta patologia