The Clinical and Haematological Findings in Children Inheriting Two Types of Thalassaemia: High-A2 Type β-Thalassaemia, and High-F Type or δβ-Thalassaemia

S ummary . Eleven children who are double heterozygotes for β- and δβ-thalassaemia are described. Of their parents one was always heterozygous for β-(A₂) thalassaemia (increased Hb A₂), and the other for the high F variant or δβ-thalassaemia (increased Hb F). The clinical syndrome resulting from the combination of β- and δβ-thalassaemia shows some heterogeneity, but in general is of intermediate severity. Red cell abnormalities were considerable, Hb F was very high (mean 70.3 ± 12.6%), Hb A₂ was low or normal (mean 2.36 ± 1.52%), and Hb A was absent in five patients. Hb F was nearly homogeneously distributed in the red cells of most patients. These findings are explained as the outcome of a mutation which suppresses δ- and β -chain synthesis which is associated with a genetically determined increased production of γ- chains.     

Psychometric Properties of the Greek Version of FCV-19S

As COVID-19 delivered a devastating blow around Europe, unprecedented mental health issues have arisen. This study outlines the Greek translation and validation of the Fear of COVID-19 Scale (FCV-19S) in the general population. A sample of 3029 Greek-speaking individuals completed the FCV-19S, as part of a large online survey reporting on the aftermath of the coronavirus on the psychological health of Greeks. A total of 2970 participants completed all items without missing values, and their responses were included in the statistical analysis. The confirmatory factor analysis suggested an acceptable model fit. The internal consistency measured by Cronbach’s alpha = .87 for the whole scale. All hypothesized correlations were as expected, confirming the construct validity of the scale. The results of the analyses suggest that the psychometric properties of the Greek FCV-19S are sufficient.

     

Cleft lip and palate treatment of 530 children over a decade in a single centre

Objective

We sought to evaluate the process of care and the outcomes of cleft lip and palate operations carried by a multidisciplinary team at a centre of craniofacial anomalies with a high patients’ volume.

Methods

A retrospective review of all cleft lips and/or palates cases treated in the centre from 1995 to 2007 was performed. Direct and long term complication rates, clinical, audiologic, speech intelligibility and dental arch assessments were analyzed.

Results

A total of 530 children have been operated this period in the centre (64 isolated cleft lip closures). A detailed presentation of the outcomes is performed in relation to the various types of cleft lip and palates. The majority of parents (70%) reported very good or excellent results 2-5 years after the lip closure with the Millard technique, although those with bilateral clefts were significantly less satisfied (P < 0.002). Forty-two percent of children with cleft palate and otitis media with effusion were self-improved 2-8 months after palate reconstruction and 83.3% of children treated with the two flaps palatoplasty technique had a rather high or very high intelligibility score. Muscles’ retropositioning had a significant effect on intelligibility (P = 0.04).

Conclusions

Children with cleft lips and palates have a variety of conditions and functional limitations even after the surgical correction of their problem that need to be evaluated and treated by several specialists. The treatment protocol utilized by the multidisciplinary team of our centre is efficient with a relative low percentage of complications and unfavorable results.