Thyroid Status in Children with Transfusion Dependent Hb-E ß-Thalassaemia.

Despite improved haematological care, multi-endocrine dysfunction is a common complication in thalassemia. Iron overload is thought to be the most likely mechanism in thyroid dysfunction in these patients. Moreover, chronic tissue hypoxia might havedirect toxic effect on thyroid gland resulting in hypothyroidism. This study was designed to evaluate the thyroid status of children with Hb-E ß-thalassemia. This cross sectional analytic study was conducted among thepatients with Hb-E ß-thalassemia attending both in-patient & out-patient department of Paediatrics, Dhaka Medical College & Hospital, Dhaka, Bangladesh from April 2012 to March 2013. The children who attended inpatient and outpatient department of Paediatrics for some other illness were screened out for thalassaemia and were recruited as comparison group. Thyroid function tests (TSH & FT4 level) were performed in both thalassaemic patients and comparison group. Serum ferritin level was also measured for assessing iron status of thalassaemic patients and their pre-transfusion Hb levels were recorded in the pre-formed data collection form. Of the 50 thalassaemia patients, 13(26%) had subclinical hypothyroidism. This proportion of subclinical hypothyroidism was significantly high, compared to that (2.5%) of non thalassaemia comparison group. Among the subclinically hypothyroid thalassaemia subjects, 8 were males and 5 were females and their mean age was 102.38±33.29 months. The mean serum ferritin levels in hypothyroid and euthyroid thalassaemia cases were 2387.87±1642.85ng/ml and 1822.95±1345.33ng/ml respectively (normal level upto 300ng/ml). This difference was not statistically significant (p=0.279); but the pre-transfusion Hb level wasfound significantly different (p=0.02) among the two groups. It was 5.57±0.98g/dl in hypothyroid & 6.37±0.09g/dl in euthyroid thalassaemic cases. Subclinical hypothyroidism was quite high among the children with transfusion dependent Hb-E ß-thalassaemia. Their hypothyroid status had no significant correlation with their serum ferritin level but had significant correlation with low haemoglobin status.

Intracranial sewing needles: Review of 13 cases.

Three new cases of intracranial sewing needles are reported and are reviewed with 10 other published cases. These needles must have been introduced in infancy before the closure of fontanelles. The intention, at least in most cases, has been infanticide. Except in one case which the mother reported and two cases found at necropsy, the others were diagnosed by skull radiography. Headache and epilepsy were the main symptoms and they often appeared when the patient had reached adulthood. Removal of the needles lessened the symptoms.

Intramedullary pearly tumour.

The presentation treatment and postoperative progress of two patients with pearly intraspinal epidermoids is described. In one of the patients the tumour recurred after the first removal, necessitating a second operation. Despite serious disturbances of gait both patients improved following surgery. The literature in respect of these tumours is reviewed.

Hydatid disease of the spine.

In 42 cases of hydatid diseases of the spine the preoperative diagnosis was as follows: hydatid disease--19, benign tumour--8, Tuberculosis--6, malignant disease--4, herniated disk--3, syringomyelia--1, infectious spondylitis--1. The site of the hydatid lesion in these patients was as follows: cervical--5, thoracic (T6-T12)--26, lumbosacral--11. Seven of these cases had previously been operated on for a hydatid cyst in other organs. We had eight recurrences of which five did not respond completely to surgical intervention.

Bilateral stenosis of the internal carotid artery, associated with aneurysm of the basilar trunk.

1) A case of bilateral stenosis of the internal carotid artery with aneurysm of basilar trunk is reported. 2) Via femoral angiography confirmed the diagnosis which is strongly recommended in diagnosing such cases. 3) No surgical treatment is considered. 4) No similar case has been reported in literature.