A simple index for initiating transfusion treatment in thalassaemia intermedia

Sbyrakis, S; Karagiorga-Lagana, Markissia; Voskaki, I; Efthimiou, H; Karaklis, A

doi:10.1111/j.1365-2141.1987.tb06172.x

Cited by 7 publications

(3 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…1). His cephalofacial deformity was scored as grade 2, applying the Logothetis scoring system (Logothetis et al, 1971;Sbyrakis et al, 1987), with evident mongoloid appearance and mild maxillary hypertrophy. The abdomen was soft with moderate splenomegaly at 10 cm below the costal margin and mild hepatomegaly.…”

Section: Methodsmentioning

confidence: 99%

Otological manifestations of thalassaemia intermedia: evidence of temporal bone involvement and report of a unique cholesteatoma-like lesion

et al. 1992

View full text Add to dashboard Cite

Thalassaemia intermedia should be considered in any chronically anaemic patient presenting fromthe Middle East with hearing impairment. We report here three Saudi siblings with thalassaemia intermedia and features of severe bone marrow expansion, particularly invading the temporal bone. They were seen first for their otological problems before they had access to proper haematological evaluation. One member was admitted for surgical exploration of a cholesteatoma, which was then found to be marrow expansion of the temporal bone. Screening of the family revealed twomore anaemic siblings with thalassaemia intermedia. Audiological examination of all the family members showed that only the two affected members had a high frequency sen-sori-neural hearing loss.Bone marrow expansion into the temporal bone is a rare feature of thalassaemia intermedia. Cholesteatoma-like lesion has not been previously described. It has to be considered in all cases of symptomatic thalassaemia intermedia manifesting with cavitation and lytic lesions in the mastoid system. The likelihood that sensorineu-ral hearing loss may complicate the thalassaemias israised and the possible mechanism for such involvement discussed. The proper management for different otological manifestations of the thalassaemias is suggested. These cases would suggest amore extensive involvement of the temporal bone in the thalassaemias than has been previously recognized. Further large scale studies are required to illuminate the subject.

show abstract

Section: Methodsmentioning

confidence: 99%

Otological manifestations of thalassaemia intermedia: evidence of temporal bone involvement and report of a unique cholesteatoma-like lesion

et al. 1992

View full text Add to dashboard Cite

show abstract

“…A higher oxygenation could also be correlated with an increase in hematocrit and blood volume. The brain dysfunction that occurs in the thalassemia patients can be prevented by chelation therapy and blood trans fusions [12,13,17], The improvement of the brain tissue oxygenation after transfusion may be reflected by the alpha rhythm attenuation detected presently. Searching for neurophysiological monitoring possibilities of the brain function in this case, as in other systematic dis eases influencing the brain function, qEEG analysis may be of great help especially when supported by other examinations (such as the neuropsychological testings).…”

Section: Discussionmentioning

confidence: 99%

The Effect of Blood Transfusion on Alpha EEG Activity in Thalassemic Patients

1991

View full text Add to dashboard Cite

EEG signal analysis could be very helpful in the detection of signs of adverse reaction to the brain by a systemic disease. We performed an EEG spectral analysis in 12 young patients with thalassemia before and after their regular blood transfusion, and in 10 volunteer students. Our aim was to test if the EEG analysis could detect signs of brain dysfunction due to the cerebral hypoxia as a result of the anemia. We analyzed the EEG signals on line using a dedicated computer system estimating the power of the delta, theta, alpha, sigma and beta bands of the EEG. After transfusion, the power spectral density of the alpha band showed a significant enhancement in most areas of the brain, in the group of the thalassemia patients as compared to the normals. These differences correlated with the levels of hemoglobin of the patients, and possibly reflect the degree of oxygenation of the brain. Since the visual interpretation of the EEG is not efficient for estimating the brain dysfunction in the anemic state in thalassemia, the signal analysis of the EEG may provide a more sensitive alternative to study the effects of hypoxia on brain function.

show abstract

“…Metatarsal and metacarpal bones are the first to expand so measurement of the metacarpal bones has been considered a good indicator for initiation of transfusion therapy. 143 Other skeletal deformities include shortening of long bones due to early epiphyseal fusion and overgrowth of the maxilla causing dental malocclusion. The marrow expansion can also lead to pathological fractures, early bone thinning and osteoporosis 144,145 while ineffective drainage of the sinuses and middle ear from skull bone overgrowth can cause chronic sinus and ear infections.…”

Section: Thalassaemia Majormentioning

confidence: 99%