Sickle retinopathy was correlated with TAMV in MCAs but not in OAs. A significant difference was found between initial and follow-up TAMVs in the MCAs, after 1 year of regular HU and transfusion therapy, in those with conditional velocities.
Background
Decreased bone mineral density (BMD) is a significant morbidity in haemophilia. Vitamin D is important for the bone health of people with haemophilia. Regular factor VIII prophylaxis can prevent bleeding and arthropathy.
Aim
To determine the 25(OH) vitamin D level in severe haemophilia A patients and correlate it to their Hemophilia Joint Health Score (HJHS) and dual‐energy X‐ray absorptiometry (DEXA). We also compared the 25(OH) vitamin D and DEXA in haemophilia A and healthy children and in haemophilia A children on prophylaxis versus on‐demand therapy.
Methods
Fifty severe haemophilia A patients were compared to 50 age‐matched healthy boys. Patients were recruited from the Pediatric Hematology Clinic, Ain Shams University from May 2017 to April 2018. Full medical history was taken with emphasis on frequency of bleeding episodes, duration and amplitude of pain assessed by the pain score. Weight, height, body mass index and HJHS were assessed. 25(OH) vit‐D3, calcium, phosphorus and alkaline phosphatase were measured. BMD was assessed using Lunar DEXA, paediatric software.
Results
People with haemophilia had significantly lower 25(OH) vit‐D3 (P < .001) and DEXA z‐score (P < .001) than controls. Seventy per cent of patients were on factor VIII prophylaxis twice weekly (15U/kg/dose). Significant difference was found regarding DEXA z‐score (P = .012), 25(OH) vit‐D3 (P = .033) and HJHS (P = .022) among patients on prophylaxis and on‐demand therapy.
Conclusion
Severe haemophilia A patients showed significantly lower 25(OH) vit‐D3 and DEXA than controls. Hence, vitamin D deficiency should be tested in all people with haemophilia for early diagnosis and treatment. Low‐dose prophylaxis in severe haemophilia preserves BMD and increases vitamin D. Further studies are required to evaluate the effect of different prophylaxis protocols on BMD and haemophilic arthropathy.
In diabetes, angiogenesis is disturbed, contributing to proliferative retinopathy, nephropathy and neuropathy. Kallistatin, a serine proteinase inhibitor, has anti-angiogenic effects. We assessed serum kallistatin in children and adolescents with type 1 diabetes as a potential marker for microvascular complications and its relation to carotid intima media thickness (CIMT). Sixty patients with type 1 diabetes were divided into two groups according to the presence of microvascular complications and compared with 30 healthy controls. High-sensitivity C-reactive protein (hs-CRP), HbA1c, urinary albumin creatinine ratio (UACR), kallistatin levels and CIMT were assessed. Kallistatin levels were significantly higher in patients with microvascular complications (9.9 ± 2.38 ng/mL) and those without complications (5.0 ± 1.5 ng/mL) than in healthy controls (1.39 ± 0.55 ng/mL; p<0.001). Kallistatin was increased in patients with microalbuminuria compared with the normoalbuminuric group (p<0.001). Positive correlations were found between kallistatin and disease duration, fasting blood glucose, HbA1c, triglycerides, total cholesterol, hs-CRP, UACR and CIMT (p<0.05). A kallistatin cut-off value at 6.1 ng/mL could differentiate patients with and without microvascular complications, with a sensitivity of 96.87% and specificity of 93.75%. Increased kallistatin levels in type 1 diabetes and its relation with CIMT may reflect vascular dysfunction and suggest a link between micro-and macro-angiopathy.
Orosomucoid is an acute-phase serum protein that is upregulated in urine samples of patients with diabetic nephropathy. We assessed serum and urinary orosomucoid levels in children and adolescents with type 1 diabetes and their relation to microvascular complications and carotid intima-media thickness (CIMT). Sixty patients with type 1 diabetes were divided into 2 groups according to the presence of microvascular complications and compared with 60 healthy controls. High-sensitivity C-reactive protein (hs-CRP), hemoglobin A1c (HbA1c), urinary albumin-creatinine ratio (UACR), serum and urinary orosomucoid, and CIMT were assessed. Both serum and urinary orosomucoid levels were significantly increased in patients with and without microvascular complications compared with controls, and the highest levels were in patients with complications (P < .001). Serum and urinary orosomucoid were higher in patients with microalbuminuria than normoalbuminuric group (P < .001). The cutoff value of urinary orosomucoid at 2825 ng/mL could differentiate patients with and without microvascular complications. Serum and urinary orosomucoid were positively correlated. Multiple regression analysis showed that HbA1c, UACR, hs-CRP, and CIMT were independently related to orosomucoid. We suggest that orosomucoid is a significant independent factor for diabetic microvascular complications and can be considered as an early marker of renal injury. High orosomucoid levels in type 1 diabetes reflect endothelial dysfunction and subclinical atherosclerosis.
T1DM subjects had significantly poorer performance than controls on all subtests of the BVRT, on all subscales of the WISC (verbal, performance and total IQ) and on most subtests of WCST (p < 0.05). T1DM subjects with good glycemic control performed significantly better than subjects with poor glycemic control on all subtests of the BVRT and on all subscales of the WISC (p < 0.05), but there was no difference on the WCST. T1DM subjects differed from controls on memory, intelligence, and executive functions. They also differed according to good or poor glycemic control (except on the WCST). Cognitive performance significantly correlated with a number of demographic and clinical variables.
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