湘雅儿科精品课件-Rickets of VitD Deficiency

脑积水 Hydrocephalus VitD抵抗性佝偻病 Vit D resitant rickets
先天性甲状腺功能低下 congental hypothyroidism
骨软骨营养不良 Achondroplasia
脑积水
Hydrocephalus
治疗 Treatment
目的 控制活动期，防止畸形和复发
increased decalcification of old bone Serum Ca remain normal or slightly lowered
Ca of old bone can’t be mobilized Serum Ca decreased
Ca P product decreased
预防 Prevention
多晒太阳
补充VitD制剂，预防剂量为 400~800IU/d 提倡母乳喂养，及时添加辅食 积极治疗原发病 孕妇多户外活动，注意营养
Pathogenesis
Vit D deficiency
Disturbance of calcification of osteoid tissue + decalcification of skeleton Diminished absorption of Ca and P from intestine
Originates of VitD
食物中摄取麦角固醇（次要） 皮肤中7-脱氢胆固醇（主要） 7- dehydrocholesterol 紫外线照射 ultraviolet plant ergosterol
VitD2
内源性VitD3
1，25（OH）2D3的作用
促进肾小管对 钙磷的重吸收 促进小肠粘膜 对钙磷的吸收
A fall of serum Ca
Proliferation of osteoid tissue
Compensatory function of parathyroid
Increased
Insufficient
Excretion of urine P increased Rachitic metaphases and soften shaft A fall of serum P
促进旧骨脱钙，增加 细胞外液钙磷的浓度
Metabolism of VitD
Ingested from food
Human skin contains 7dehydrocholesterol
(ultraviolet rairradiation)
Vit D3
Liver(25-hydroxylase) 25-(OH) -D3（ 25-dihydroxycholecalciferol） kidney (1-hydroxylase)
Rachitic Tetany
Pathogenesis
Rickets
临床表现 Clinical manifestations

初期 The early stage 年龄 3 months 症状 神经精神症状 （neuro-psychic symptoms） 体征 枕秃 （ the loss of hair in the occipital position） 生化改变 Ca normal ,P normal or , Ca P , AKP X线 No obvious skeletal changes
normal
X线
诊断 Diagnosis
病史 症状：尤其注意早期症状
骨骼改变：注意年龄不同而出现的骨骼
畸形有所不同
实验室检查
判断
结合年龄、季节做出综合
鉴别诊断 Differential diagnosis
先天性甲低
congental hypothyroidism
骨软骨营养不良Achondroplasia
漏斗胸 Funnel breast
肋骨串珠 Rachitic rosary
赫氏沟 Harrison grooves
猫背 鸡胸 脊柱弯曲 Spinal column Cat back
Chicken breast
手镯 bracelets of hands O型腿 bowleg X型腿 knock knees
第五章第五节（2）
Rickets of VitD Deficiency
维生素D缺乏性佝偻病
中南大学儿科学教研室
刘晓艳
Purpose
Be familiar with etiology and pathogenesis of rickits and tetany of Vitamin D deficiency. To master their clinical manifestations, diagnosis ,treatment and prevention . To understand their differential diagnosis.
Definition
Rickets
is a very common nutritional deficient disease in young children especially under two years old of age. VitD deficiency cause rickets, a metabolic disorder of calcium and phosphorus with a failure to mineralize osteoid tissue of growing bone resulting in bony deformities.
枕秃
the loss of hair in the occipital position
Clinical manifestations
激期
The advanced stage 症状和体征 生化改变 Ca ↓, P ↓↓ Ca P <30, AKP ↑ X线
方颅 Square head
1,25-(OH) 2-D3 (1, 25-dihydroxycholecalciferol)
Regulation of metabolism of Vit D3
Vit D
(-)
Liver
25-(OH) D3 Hypo serum P
(+) (-) (-)
Hyper serum P
Kidney
(-)
关于VitD制剂的应用 ●对活动期的患儿，VitD剂量20004000UI/d， 视临床症状和X-线改善情况于 1个月改为预防量 ● 对有并发症者或无法口服者可肌注VitD3 一次，量为20-30万IU，3个月后改为口服预 防量。 钙剂 葡萄糖酸钙1-3g/d
Clinical manifestations
恢复期
Recovered stage of rickets
症状 These become subsided 体征 生化 Ca and P return to normal
Ca P returns to normal AKP may be return slowly to normal
X线 it returns to normal after 2 to 3 weeks
Clinical manifestations
后遗症期
Sequela stage of rickets 年龄 more than 2 years old 症状 without any clinical symptoms 体征 in severe case the deformities of bones may remain 生化 all the serum level of Ca, P and AKP are
PTH
(+)
Hypo serum Ca
Hyper serum Ca
1,25- (OH)2 D3
(+)
(+)
(-)
Calcitonin
病因 Etiology
日光照射不足
Lack of sunshine 摄入不足 Underfeeding 生长过快 Rapid growth 疾病影响 Influence of diseases 药物影响 Influence of medicine