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Background:
We have previously demonstrated that therapy with orally administered L-glutamine improves nicotinamide adenosine dinucleotide (NAD) redox potential of sickle red blood cells (RBC). On further analysis of L-glutamine therapy for sickle cell anemia patients, the effect of L-glutamine on adhesion of sickle RBC to human umbilical vein endothelial cells (HUVEC) was examined.
Methods:
The first part of the experiment was conducted with the blood samples of the 5 adult sickle cell anemia patients who had been on L-glutamine therapy for at least 4 weeks on a dosage of 30 grams per day compared to those of patient control group. In the second part of the experiment 6 patients with sickle cell anemia were studied longitudinally. Five of these patients were treated with oral L-glutamine 30 grams daily and one was observed without treatment as the control. t-test and paired t-test were used for determination of statistical significance in cross-sectional and longitudinal studies respectively.
Results:
In the first study, the mean adhesion to endothelial cells with the autologous plasma incubated cells were 0.97 ± 0.45 for the treated group and 1.91 ± 0.53 for the nontreated group (p < 0.02). Similarly with lipopolysaccharide (LPS) incubated cells the mean adhesion to endothelial cells were 1.39 ± 0.33 for the treated group and 2.80 ± 0.47 for the untreated group (p < 0.001). With the longitudinal experiment, mean decrease in the adhesion to endothelial cells was 1.13 ± 0.21 (p < 0.001) for the 5 treated patients whereas the control patient had slight increase in the adhesion to endothelial cells.
Conclusion:
In these studies, oral L-glutamine administration consistently resulted in improvement of sickle RBC adhesion to HUVEC. These data suggest positive physiological effects of L-glutamine in sickle cell disease.

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Background:
In 1983, Graham Hughes described a condition of Antiphospholipid Syndrome in which there was a danger of thrombosis. The condition is readily detectable by blood tests and, once diagnosed; the risk of further thrombosis can be significantly reduced by anticoagulation treatments. Affected groups of patients can be distinguished by a specific blood test – the detection of antiphospholipid antibody (Ref-1). Patients with Hughes syndrome have hypercoaguable state with a markedly increased risk of both arterial and venous thrombosis and there is temporal persistence of antibody positivity.Case presentationA 44-year-old woman was admitted under the acute surgical "take" with left sided abdominal pain radiating to her back. She had a history of borderline thyrotoxicosis in the early 1990s. She was on etonogestrel-releasing implants for contraception and there was no history of previous deep venous thrombosis. She was very tender, locally, over the left side of the abdomen. Investigations showed haemoglobin of 13.2 g/dl, white cell count of 19.9 10*9/L, and platelets 214 10*9/L with neutrophilia. Amylase and renal function tests were found to be normal. Liver function tests were deranged with Gamma GT 244 u/l (twice normal). An abdominal Ultrasound Scan suggested a possible splenic infarction, which was confirmed by a CT scan of her abdomen. Tests were carried out to investigate the possibility of a post thrombotic state. Coagulation risk factors for thrombosis were within the normal limits; Protein S 67 %(60–140), Protein C 103 % (72–146), Antithrombin 3 110 %(80–120) and Activated P C Resistance was 1.9(2.0–4.3). The Hams test was negative but the Anticardiolipin antibody test was positive. IgM level was 52 (normal is up to 10) and IgG was 18.8 (normal is up to 10). She also had border line APC Sensitivity 1.9 (2 to 4.3). Kaolin time 49 sec (70–120) Ktmix 64 sec (70–120), thyroid function test revealed TSH 0.32 mu/L, fT4 20.2 pmol/L (10–25). Subsequent determination of Anticardiolipin antibody was negative. Her symptoms were settled with the use of simple analgesia and she was discharged home with long-term anticoagulation medication. The INR target for long-term anticoagulation was aimed at >3.
Conclusion:
This case presented to us as an acute abdominal pain. Subsequent investigations revealed the presence of splenic infarction. Coagulation risk factors for thrombosis proved negative. Haematological investigations revealed the presence of anticardiolipin antibodies at the first instance but subsequent determinations were negative. Hence, it mimicked Hughes syndrome initially but the criteria for temporal persistence of anticardiolipin antibody was not fulfilled. Unusual surgical presentation of a thrombotic abnormality as abdominal pain due to splenic infarction.

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Background:
Children with haemophilia have lower levels of fitness and strength than their healthy peers. We present the protocol of a study designed to determine whether an exercise intervention improves quality of life, aerobic fitness and strength in children with haemophilia.Methods/DesignThe study will be a randomised, assessor-blinded, controlled trial of exercise treatment. Seventy children aged between 6 and 18 years with haemophilia or von Willebrand disease will be recruited from two paediatric haemophilia clinics in NSW. Each participant will be allocated to an exercise group or a control group using a concealed allocation procedure. The control group will receive usual medical care while the intervention group will receive usual medical care plus an exercise program for 12 weeks. Outcomes (VO2peak, knee extensor strength and quality of life) will be measured at baseline and on completion of the exercise program by a blinded assessor. The primary analysis will be conducted on an intention to treat basis. The effects of the exercise intervention on each of the three primary outcomes will be estimated from between-group differences in the mean outcome adjusted for baseline scores.DiscussionThis study will be the first randomised controlled trial to examine the effects of a structured exercise program on fitness and quality of life in children with haemophilia.

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Background:
In the context of sickle cell anemia, peripheral blood indexes provide key information that is also potentially influenced by age. Therefore, it is necessary to understand the extent and nature of interactions between sickle cell anemia and age, especially in situations where there is a high prevalence of sickle cell anemia.
Methods:
In a cross-sectional study of 374 subjects with varying hemoglobin S (HbS) status, we characterized the interaction between age and sickle hemoglobin using principal components analysis.
Results:
Factor analysis in subjects with hemoglobin AA identified three orthogonal factors – normal erythropoiesis, presence of thalassemia and the aggregability potential of the blood. These three factors were differentially associated with hemoglobin status. Age influenced the association of factors #2 and #3 with hemoglobin status.
Conclusion:
Our findings suggest that the interaction between age and hemoglobin status needs to be considered in both clinical and public health settings.

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