發(fā)布時間：2018-05-09 08:12

  本文選題：重型β-地中海貧血 + 骨質(zhì)疏松　； 參考：《廣西醫(yī)科大學(xué)》2015年碩士論文

【摘要】：第一部分 鐵過載沙鼠的骨代謝目的：探討鐵過載沙鼠骨組織的改變及骨代謝指標(biāo)的變化。方法：20只雌性蒙古沙鼠隨機(jī)分配為鐵過載(iron overload,10)組和生理鹽水(normal saline,NC)組。10組采用腹腔注射右旋糖酐鐵200mg/kg/周法建立鐵過載沙鼠模型。NC組沙鼠腹腔注射等量生理鹽水。18周后處死沙鼠取靜脈血檢測鐵蛋白,取骨組織行HE染色、普魯士藍(lán)染色、免疫組織化學(xué)染色觀察各組沙鼠骨組織及堿性磷酸酶(alkaline phosphatase,ALP)、骨鈣素(osteacalcin,OC)及核因子K B受體活化因子配體(receptor activator of nuclear factor kappa B ligand, RANKL)的表達(dá)。結(jié)果：鐵過載組ALP、OC、RANKL的表達(dá)均高于生理鹽水組,差異有統(tǒng)計學(xué)意義(P0.05)。結(jié)論：1.鐵過載沙鼠骨骼有明顯的鐵沉積。2.鐵過載可使骨吸收和骨形成作用都增強(qiáng)。第二部分 骨代謝指標(biāo)在廣西重型β地中海貧血患者中的變化及其與鐵過載的關(guān)系目的：1.調(diào)查廣西β重型地貧患者鐵過載發(fā)病率及嚴(yán)重程度。2.探討骨代謝指標(biāo)在廣西重型地中海貧血患者中的變化及其與鐵過載的關(guān)系。方法：2010年1月至2012年12月共收集廣西多家醫(yī)院血液內(nèi)科250例重型β-地中海貧血患者(男160,女90,3~25歲,中位年齡8歲)和97例健康對照組(男61,女15,3~15歲,中位年齡10歲)。通過回顧性輸血及排鐵問卷調(diào)查收集患者年齡、性別、起始輸血時間、輸血量等情況,測量身高,體重,計算BMI。所有受試者檢測甲狀旁腺激素(parathyroid hormone, PTH), ALP, OC,血清鈣,血清磷,睪酮(男性),雌二醇(女性)等生化指標(biāo)。接受心鐵和25(OH)D檢查的患者分別為165人和192人。結(jié)果：(1)250例重型地貧患者中有246例(98.4%)患者存在鐵過載,最小出現(xiàn)心鐵沉積的患者為6歲。(2)與正常對照組比較,地貧患者血鈣、25(OH)D明顯下降,血磷、ALP、OC、PTH明顯升高(P0.05)。(3)63(25.2%)例患者發(fā)現(xiàn)有低血鈣癥(血鈣2.2mmol/L)。131(52.4%)例患者發(fā)現(xiàn)有高磷血癥(血磷1.8mmol/L)。14(5.6%)例患者有低磷血癥(P1.45mmol/L),高磷血癥的患者明顯較低磷血癥的患者年輕(8.60±4.04vs11.57±4.57,P0.05)。26(10.4%)例患者PTH高于正常上限,14(5.6%)例患者PTH低于正常下限�！�10歲的患者PTH與年齡呈正相關(guān)(R=0.174, P0.05),10歲的患者PTH與年齡則無明顯相關(guān)性(R=-0.127,P0.05)。(4)≤10歲的患者PTH與心鐵值呈負(fù)相關(guān)(R=-0.212,P0.05)。(5)地貧患者的雌二醇、睪酮水平均明顯降低,但與鐵過載未見明顯相關(guān)性。結(jié)論：1.廣西重型地貧患者存在嚴(yán)重的鐵過載。2.廣西重型地貧患者血鈣、25(OH)D明顯降低,ALP、OC、PTH、血磷明顯升高。3.未發(fā)現(xiàn)廣西重型地貧患者血鈣、25(OH)D、ALP、OC、PTH、血磷與鐵過載存在直接相關(guān)性。第三部分 廣西重型β地中海貧血患者骨疾病發(fā)病情況及其影響因素目的：通過調(diào)查廣西重型β地中海貧血患者的骨疾病發(fā)生率,并對引起骨量下降的因素與其他各國研究進(jìn)行對比,提出更適合廣西患者的治療和預(yù)防方案。方法：我們研究了81例重型β-地中海貧血患者(男48,女33,4-20歲,中位年齡10歲)及101例健康年齡匹配的對照組(男61,女40,3-20歲,中位年齡10歲),詳細(xì)記錄病史,測量身高、體重,計算BMI。所有受試者均接受雙能X線骨密度儀測定腰椎和股骨近端的骨密度(bone mass density,BMD),計算Z值(z-score=(x-mean)/SD,其中x為測量的BMD,mean為同年齡同性別正常組平均骨密度)。地貧患者還另外抽血進(jìn)行下列檢測：A)血清鐵蛋白,心臟鐵；B)甲狀旁腺激素(PTH);C)骨形成指標(biāo)：堿性磷酸酶(ALP),骨鈣素(0C)；D)血清鈣,血清磷；E)男性睪酮,女性雌二醇等檢查。結(jié)果：34(41.98%)例患者有身材矮小,25(30.86%)例患者低體重,74(91.36%)例患者低體重指數(shù)(BMI18.5)。地貧患者骨疾病發(fā)病相當(dāng)普遍。在81例患者中,8(9.76%)例患者骨質(zhì)疏松,23(28.4%)例患者骨量減少。地貧患者骨質(zhì)疏松最早發(fā)生在5歲,骨量減少最早發(fā)生在4歲。未發(fā)現(xiàn)鐵蛋白、T2*值與骨密度及Z值有直接相關(guān)性(P0.05)。證實了患者BMD與年齡呈正性相關(guān),Z值與年齡呈負(fù)相關(guān)(P0.05),但不同年齡分組骨疾病的發(fā)病率并未見明顯差異(P0.05)。相對于正常骨量患者,骨質(zhì)疏松患者血鈣明顯降低,ALP、PTH明顯升高(P0.05)。睪酮、雌激素與骨量或Z值存在正相關(guān)性(P0.05)。結(jié)論：重型地貧患者建議從4歲開始早期規(guī)律檢測骨密度,注意電解質(zhì)的變化,早期補充鈣劑或維生素D,補充性激素,提高成年后生存質(zhì)量,防治骨質(zhì)疏松造成的痛苦及殘疾。
[Abstract]:The first part of the iron overload gerbil bone metabolism Objective: To explore the changes of bone tissue and the changes of bone metabolism index in iron overload gerbils. Methods: 20 female Mongolia gerbil were randomly assigned to iron overload (iron overload, 10) and normal saline (normal saline, NC) group.10 group by abdominal cavity injection of dextran 200mg/kg/ week method to establish iron overload The gerbil group.NC group of gerbil was injected with the same amount of normal saline for.18 weeks to detect the ferritin, the bone tissue was stained with HE, Prussian blue staining, and immunohistochemical staining was used to observe the bone tissue and alkaline phosphatase (alkaline phosphatase, ALP), osteocalcin (osteacalcin, OC) and the activation cause of K B receptor of nuclear factor in each group. The expression of the sub ligand (receptor activator of nuclear factor kappa B ligand, RANKL). Results: the expression of ALP, OC, RANKL were higher than that of the normal saline group, and the difference was statistically significant. Conclusion: the iron overload of 1. iron overload gerbils has an obvious iron deposition. The iron overload can enhance the bone absorption and bone formation. The second part of the iron overload can enhance the bone absorption and bone formation. Changes in bone metabolism and the relationship with iron overload in patients with severe beta thalassemia in Guangxi: 1. investigate the incidence and severity of iron overload in patients with severe poverty in Guangxi beta.2. to explore the changes in bone metabolism index in patients with severe thalassemia in Guangxi and the relationship with iron overload. Methods: from January 2010 to 2012 1 In February, 250 patients with severe beta thalassemia (160 men, 90,3~25 years old, middle age 8 years old) and 97 healthy control groups were collected in the Department of Hematology in Guangxi, and 97 healthy controls (male 61, female 15,3~15 years old and 10 years old). The age, sex, starting time of blood transfusion, blood transfusion and so on were collected and measured by a retrospective blood transfusion and iron drainage questionnaire survey. Weight, all BMI. subjects were calculated to detect parathyroid hormone (parathyroid hormone, PTH), ALP, OC, serum calcium, serum phosphorus, testosterone (male), estradiol (female) and other biochemical indexes. The patients receiving heart iron and 25 (OH) D examination were 165 and 192. Results: (1) 250 cases of severe poor patients (98.4%) had iron overload. The patients with minimal cardiac iron deposition were 6 years old. (2) compared with the normal control group, blood calcium, 25 (OH) D decreased significantly, blood phosphorus, ALP, OC, PTH significantly increased (P0.05). (3) 63 (25.2%) patients found hypocalcemia (blood calcium 2.2mmol/L).131 (52.4%) patients found to have hyperphosphatemia (blood phosphorus 1.8mmol/L).14 (5.6%) patients with hypophosphoremia (P1) (P1) .45mmol/L), patients with hyperphosphatemia were significantly younger (8.60 + 4.04vs11.57 + 4.57, P0.05).26 (10.4%).26 (10.4%) patients higher than normal upper limit, 14 (5.6%) patients with PTH lower than normal lower limit. PTH in patients younger than 10 years was positively correlated with age (R=0.174, P0.05), 10 year old patients had no significant correlation with age (R=-0.127, P0.05). (4) there was a negative correlation between PTH and the value of heart iron (R=-0.212, P0.05). (5) the levels of estradiol and testosterone in the poor patients were significantly decreased, but no significant correlation was found between iron overload and iron overload. Conclusion: 1. severe iron overload in Guangxi severe poor patients with severe iron overload in Guangxi severe poor patients with blood calcium, 25 (OH) D, ALP, OC, PTH, blood phosphorus obviously rise High.3. was not found in Guangxi severe poor patients with blood calcium, 25 (OH) D, ALP, OC, PTH, and there was a direct correlation between blood phosphorus and iron overload. Third the incidence of bone disease and its influencing factors in patients with severe beta thalassemia in Guangxi were investigated by investigating the incidence of bone disease in patients with severe beta thalassemia in Guangxi and the decrease of bone mass. The factors were compared with other countries to propose a more suitable treatment and prevention scheme for Guangxi patients. Methods: We studied 81 patients with severe beta thalassemia (male 48, female 33,4-20 years, middle age 10 years old) and 101 healthy age matched control groups (male 61, female 40,3-20 years, middle age 10 years old), and recorded the history of the disease in detail and measured the body. High, weight, and calculated BMI. all subjects received the dual energy X-ray bone densitometer to determine the bone mineral density (bone mass density, BMD) of the lumbar and proximal femur (BMD), and calculated the Z value (z-score= (x-mean) /SD, X was BMD, mean was the average bone density in the same age group of same sex). White, heart iron; B) parathyroid hormone (PTH); C) bone formation indicators: alkaline phosphatase (ALP), osteocalcin (0C); D) serum calcium, serum phosphorus; E) male testosterone, female estradiol and other tests. Results: 34 (41.98%) patients had short stature, 25 (30.86%) patients with low weight, 74 (91.36%) patients with low body mass index (BMI18.5). Bone disease of the poor patients. Among 81 patients, 8 (9.76%) patients had osteoporosis and 23 (28.4%) patients had reduced bone mass. The first occurrence of osteoporosis in the poor patients was 5 years old, and the earliest osteopenia occurred at 4 years. No ferritin was found. The T2* value had a direct correlation with bone density and Z value (P0.05). It was confirmed that the patient BMD was positively related to age, Z value and Age was negatively correlated (P0.05), but there was no significant difference in the incidence of bone disease in different age groups (P0.05). Compared with normal bone mass, blood calcium decreased significantly in osteoporotic patients, ALP, PTH increased significantly (P0.05). Testosterone, estrogen and bone mass or Z were positively correlated (P0.05). Conclusion: severe poor patients are recommended to begin early from 4 years of age. Regular detection of bone density, attention to the changes in electrolytes, early supplement of calcium or vitamin D, supplemental sex hormones, improve the quality of life after adult, the prevention and treatment of osteoporosis caused by pain and disability.

【學(xué)位授予單位】：廣西醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2015
【分類號】：R556.61;R580

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