Haemoglobin Constant Spring (Hb CS) mutation and single gene deletions are common underlying genetic abnormalities for alpha thalassaemias. Co-inheritance of deletional and non-deletional alpha (α) thalassaemias may result in various thalassaemia syndromes. Concomitant co-inheritance with beta (β) and delta (δ) gene abnormalities would result in improved clinical phenotype. We report here a 33-year-old male patient who was admitted with dengue haemorrhagic fever, with a background history of Grave's disease, incidentally noted to have mild hypochromic microcytic red cell indices. Physical examination revealed no thalassaemic features or hepatosplenomegaly. His full blood picture showed hypochromic microcytic red cells with normal haemoglobin (Hb) level. Quantitation of Hb using high performance liquid chromatography (HPLC) and capillary electrophoresis (CE) revealed raised Hb F, normal Hb A 2 and Hb A levels. There was also small peak of Hb CS noted in CE. H inclusions was negative. Kleihauer test was positive with heterocellular distribution of Hb F among the red cells. DNA analysis for α globin gene mutations showed a single -α -3.7 deletion and Hb CS mutation. These findings were suggestive of compound heterozygosity of Hb CS and a single -α -3.7 deletion with a concomitant heterozygous δβ thalassaemia. Co-inheritance of Hb CS and a single -α -3.7 deletion is expected to result at the very least in a clinical phenotype similar to that of two alpha genes deletion. However we demonstrate here a phenotypic modification of α thalassemia presumptively as a result of co-inheritance with δβ chain abnormality as suggested by the high Hb F level.

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Extraction of gelatin using traditional acid-base pretreatment method has several limitations such as time consuming and causes serious water pollution. Chemical treatment often being used as an alternative process to overcome the weaknesses of the conventional method. However, excessive chemical elements would damage the structure of the gelatin due to its high sensitivity to the acid content. High Pressure Processing (HPP) is a novel and environmental friendly method that has been suggested to assist gelatin extraction. Pressurization during pretreatment could reduce the extraction time and amount of acid used. It also has a potential in enhancing the properties of the gelatin extract and increasing the gelatin yield. In this research, One-Factor-at-Time (OFAT) and optimization study were done to determine the optimum parameters for extraction of gelatin assisted by HPP from red tilapia skin. Four parameters; applied pressure, pressure holding time, ratio of acid to skin and extraction time have been selected for the OFAT design and concentration of the gelatin extract and percentage of yield gelatin were evaluated. From OFAT, optimum technical parameters for response surface optimization design were 250 MPa pressure, 7.5 ml of acid to 1 g of skin and 12 hours extraction time. Pressure holding time was fixed for 10 min. FCCCD has been used for optimization study. Results from the data shows that the optimum conditions for gelatin extraction from red tilapia skin were 250 MPa for pressure, 10 min of pressure holding time, 7.5 ml of acid for 1 g of skin and 12 hours of extraction time while the maximum concentration and yield were 19.51 mg/ml and 32.04% (320.4 mg/g), respectively. These findings proved that HPP could increase the concentration and the yield of the gelatin while reducing the chemical waste and shortening the extraction process. � All Rights Reserved.