| PART I BONE METABOLISM IN IRON OVERLOAD GERBILSObjective:To investigate the changes of bone tissue in and changes of bone metabolism indexes in iron overload gerbils.Methods:20 female Mongolia gerbils were randomly divided into 10 group and NC group.10 group were induced by intraperitoneal injection of iron dextran. NC group were intraperitoneal injected the same volume of normal saline. Animals were sacrificed after 18 weeks, detected ferritin of venous blood. Bone tissue were stained with HE, Prussian blue staining, immunohistochemistry staining. Observed bone tissue and the expression of ALP, OC and RANKL detected by immunohistochemistry of bone tissue.Results:The expression of ALP, OC, RANKL in 10 group was more than NS group (P<0.05).Conclusions:1.There was obvious iron deposition on iron overload gerbils’bone.2.Iron overload can cause both bone resorption and bone formation enhanced.PART II CHANGES OF BONE METABOLISM IN PATIENTS WITH β THALASSEMIA MAJOR IN GUANGXI AND ITS RELATIONSHIP WITH IRON OVERLOADObjective:1. To investigating the prevalence and severity of iron overload in a young Guangxi population with beta thalassemia major.2.To investigate the changes of bone metabolic markers in beta thalassaemia major patients and the relationship between bone metabolic markers and iron overload.Methods:We studied 250 patients (196 males and 114 females) with beta thalassemia major from January 2010 to December 2012 in blood Department of hematology of Guangxi hospitals. The median age was 8(3-25) years. Meanwhile, we selected 97 healthy children (61 males and 15 females)for control group. The median age was 10(3-15) years. Through the review of blood transfusion and chelation questionnaire survey to collect messages of age, sex, first blood transfusion age, amount of blood transfusion. Measurement of height, weight, calculation of BMI. All subjects for detection of PTH, ALP, OC, serum Ca, serum P, testosterone (male) and estrogen (female). Accept cardiac magnetic resonance and 25(OH)D examination were respectively 165 and 192 patients.Results:Out of 250 β thalassemia major patients, there were 246 patients (41.98%) had iron overload. The youngest patients with myocardial iron overload were 6 years old. (2)The level of Serum Ca,25(OH)D of 0 thalassemia major patients were significantly higher than the control, the level of serum P, PTH, ALP and OC were significantly lower (P<0.05). (3)63 (25.2%) patients had hypocalcemia (serum Ca<2.2mmol/L).131 (52.4%) patients had hyperphosphatemia (serum P>1.8mmol/L).14 (5.6%) patients had hypophosphatemia (serum P<1.45mmol/L). Patients had hyperphosphatemia were significantly younger than patients had hypophosphatemia (8.60±4.04vsl 1.57±4.57,P<0.05).26 (10.4%) patients’PTH above the upper normal limit,14 (5.6%) patients’PTH below the lower normal limit. PTH was positively correlated with the age in<10 years old patients (R=0.174, P< 0.05). No significant correlation between PTH and age in> 10 years old patients (R=-0.127, P>0.05). (4)PTH was positively correlated with Myocardial iron-cardiovascular magnetic resonance T2* values in<10 years old patients (R=-0.212, P<0.05). (5)The estradiol and testosterone levels were significantly decreased, but had no relationship with iron overload.Conclusions:1. P thalassemia major patients in GuangXi had severe iron overload.2. The level of serum P, PTH, ALP and OC of P thalassemia major patients in GuangXi were significantly lower, while the level of Serum Ca,25(OH)D of β thalassemia major patients were significantly higher.3. The level of serum P, Serum Ca,25(OH)D, PTH, ALP and OC of β thalassemia major patients in GuangXi had no direct relationship with iron overload.PART III PREVALENCE OF BONE DISEASE IN β THALASSEMIA MAJOR IN GUANGXI AND ITS INFLUENCE FACTORSObjective:This study was aimed at determining the frequency of bone disease, contrast the factors causing osteopenia with other countries’study, to explore the causes of bone mass changes, put forward more suitable treatment and prevention programs to Chinese patients.Methods:We studied 81 patients with β-thalassemia major (male 48, female 33, aged 4-20 years, median age 10 years) and 101 healthy age-matched controls (male 61, female 40, aged 3-20 years, median age 10 years). All patients were recorded in detail the history, physical examination, measurement of height, weight, calculation of BMI, and accepted bone density in lumbar spine and femur were measured by dual energy X-ray absorptiometry. Patients with β-thalassemia major had the additional investigations:1):serum ferritin, T2* cardiac magnetic resonance; 2) parathyroid hormone (PTH); 3 bone formation index:alkaline phosphatase (ALP), osteocalcin (OC); 4) serum calcium, serum phosphorus; 5) testosterone in males, female estradiol in female.Results:Thalassemia patient height, weight is generally lower than the normal level. 34 patients (41.98%)had short stature,25 patients (30.86%) with underweight, 74 patients(91.36%) of low body mass index(BMI<18.5). Out of 81 patients,8 patients (9.76%) with osteoporosis,23 patients (28.4%) with osteopenia, respectively. Osteoporosis occurred in a 5 years old patient, osteopenia occurred in a 4 years old patient This study found no correlation between BMD,Z-score and ferritin,cardiac T2* neither(P> 0.05).The relationship of thalassemia patients between BMD and age was positively correlation(P<0.05), Z-score was negative linear relation with age(P<0.05),but bone disease prevalcence had no differences between age groups(P> 0.05).Patients with osteopenia had significantly lower serum cacium,while higher ALP and PTH compared to patients with normal BMD. (P<0.05) gonadal hormone was positively related with BMD and Z-score(P<0.05).Conclusions:Thalassemia major patients should be early screen bone mineral density, recommendations from the age of 4 regularly. Patients should be note the electrolyte change, appropriate to add calcium or vitamin D, supplement of hormone, in order to improve the life quality in adult and future life, prevention of pain and disability. |
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