八年制讲课佝偻病

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,Rickets of Vitamin D Deficiency Tetany of Vitamin D Deficiency,Chen Qiuli,Attending Doctor,Pediatric department,The first affiliated hospital,of Sun Yat-sen University,Rickets,& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,& Tetany of VitD Deficiency,Rickets,-failure in mineralization of,growing bone or osteoid tissue (children),the end of long bone, shaft,Osteomalacia,-mature bone (adults),Rickets of Vitamin D deficiency,-,chronic systemic nutrition-deficient disease,(2y),& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,& Tetany of VitD Deficiency,VitDVitamin D,important in mineralization of newly,formed osteoid,PTHparathormone，甲状旁腺素,CTcalcitonine,降钙素,Factors associated with Ca ,P metabolism and bone development,Physiological function of VitD,antirachitic function,facilitation,intestinal,absorption of Ca , P(CaBP),reabsorption of Ca &,P,in the,kidneys,direct effect on mineral metabolism of,bone,(deposition and reabsorption),nonantirachitic function,cell proliferation, differenciation, immunity,Metabolism and regulation of VitD,7-dehydrocholesterol irradiated ergosterol,Provitamin,(skin) (plant),photochemically activation,diet cholecalciferol calciferol,(VitD,3,) (VitD,2,),25-hydroxylase in liver,25(OH)D,3,1-hydroxylase in kidney 24- hydroxylase,1,25-(OH),2,D,3,24,25-(OH),2,D,3,active,inactive,PTH,Ca , P,(),(),Ca ,CT,& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,& Tetany of VitD Deficiency,Etiology,Inadequate gain,of VitD from mother,Inadequate direct exposure,to ultraviolet rays in sunlight (296-310 nm),Rapid growth,low-birth weight infants,Inadequate intake,of VitD,The diets of infants may contain only small amounts of VitD,Predisposing factors to VitD deficiency,disorders interfere with absorption and,utilization of Vit D or Ca or P,celiac disease , steatorrhea, disorders of gastrointestine,biliary duct,pancrea, liver or kidney,drugs administration,* anticonvulsant therapy- (phenytoins, phenobarbital),interfere with the metabolism of VitD,* Glucocorticoids-antagonistic to VitD in Ca transport,& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,& Tetany of VitD Deficiency,normal osteogenesis,: cartilagenous,intramembraneous,cartilage cells normal cycle-( Fig ),zone of resting cartilage,zone of proliferating cartilage,zone of calcifying cartilage,zone of ossication: zone of preparatory,calcification(ZPC,临时钙化带),Pathology,Fig.1 Levels of growth plate,zone of resting cartilage,zone of proliferating cartilage,zone of calcifying cartilage,zone of preparatory calcification,In Ricket cartilage cells fail to complete their normal,cycle of proliferation and degeneration;,subsequent failure of capillary penetration,occurs in a patchy manner(Fig),enlarged costochondral,junction,*Non-calcified, hypertrophied growth plate in rickets (R), compared to a normal growth plate (L).,Pathology,Disturbance of sclerotic calcification,osteomalacia 骨质软化,osteoid deposition(骨样组织堆积),& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,& Tetany of VitD Deficiency,Pathogenesis,can be conceptualized to be,the bodys attempt to maintain,normal serum calcium levels,VitD deficiency,Ca, P absorption from the intestine,serum Ca level,parathyroid gland,P reabsorption,PTH,PTH,in the kidney,mobilization of,Ca ,P from the bone serum Ca,serum P serum Ca,tetany,CaPin ECF,failure in mineralization of osteoid tissue,and cartilaginous matrix,rickets,Pathogenesis,& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,& Tetany of VitD Deficiency,Clinical manifestations,Incidental age,3 m-2 yr,osseous changes in growing bone,relaxation of muscles and ligaments,nonspecific neuropsychatic symptoms,Severe case systemic,Clinical manifestations,Stages,Early stage,Progressing stage,Convalescent stage恢复期,Sequelae stage后遗症期,Early stage,(1),Symtoms,2 yr,Severe rachitic child leave osseous deformities, bowleg or knock-knee,pigeon chest, funnel-like chest,kyphosis, scoliosis,head deformity, etc.,Biochemical change and Roentgenographic examination normal,Sequelae stage,& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,& Tetany of VitD Deficiency,based on,a history of inadequate gain of VitD,Clinical observation,Confirmed by,biochemical test 25(OH)D(1160,g/ml),serum Ca, P,serum AKP (500/L),roentgenograghic examination,Diagnosis,& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,&,Tetany of VitD Deficiency, Other rickets,familiar hypophosphatemia (家族性低磷血症,or X-linked hypophosphatemia(XLH), VitD-dependent rickets,or Ca+ deficient form of rickets, Renal tubular acidosis,rickets, metabolic acidosis, polyuria, alkaluria, Renal Osteodystrophy, Hepatic diseases associated with rickets,Differential diagnosis,& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,& Tetany of VitD Deficiency,pregnant and lactating mothers,adequate sunlight; VitD 800U/d;,diet rich in Ca, P, VitD,Infant,exposure to ultraviolet light,daily VitD and Ca supplementation,Term infant VitD 400 U/d(2w2yr),premature infants VitD 800 U/d(2w3m),400 U/d(3m2yr),*summer !,Prevention,& Rickets of Vitamin D Deficiency,Introduction,Physiological function and regulation of VitD,Etiology,Pathology,Pathogenesis,Clinical manifestations,Diagnosis,Differential diagnosis,Prevention,Treatment,&Tetany of VitD Deficiency,To control the progressive stage,prevent osseous deformity,N,atural and artificial light,Nutrition,VitD analogue administration,Prevent and treat complication,Treatment,VitD analogue administration,Orally,VitD 2,0004,000 IU/d 24 W,1,25(OH),2,D,3,0.5 2 g/d 24 W,then VitD 400 IU/d (10ug/d),Single dose im,VitD,3,200,000-300,000 IU,2-3m later VitD 400 IU/d,reevaluation q1m,Treatment,& Rickets of Vitamin D Deficiency,& Tetany of VitD Deficiency,Introduction,Chemical Pathology,Prediposing factor,Clinical manifestations,Diagnosis & Differential diagnosis,Treatment,R,achitic tetany,Incidental age 6m(,4m-3yr),Form of plasma Ca,810%,40%,4550%,IONIZED,active form PH、serum P,BOUND,protein,COMPLEXED,& Rickets of Vitamin D Deficiency,& Tetany of VitD Deficiency,Introduction,Chemical Pathology,Prediposing factor,Clinical manifestations,Diagnosis & Differential diagnosis,Treatment,VitD deficiency,Ca, P absorption from the intestine,serum Ca level,parathyroid gland,P reabsorption,PTH,PTH,in the kidney,mobilization of,Ca ,P from the bone serum Ca,serum P serum Ca,tetany,CaPin ECF,failure in mineralization of osteoid tissue,and cartilaginous matrix,rickets,Pathogenesis,serum Ca,2+,0.751mmol/L,serum Ca1.751.88,mmol/L,the loss of the inhibitory control that,Ca exert on the neuromuscular junctions,muscular irritability,Chemical Pathology,& Rickets of Vitamin D Deficiency,& Tetany of VitD Deficiency,Introduction,Chemical Pathology,Prediposing factor,Clinical manifestations,Diagnosis & Differential diagnosis,Treatment,VitD,deficiency,accompany with dull parathyroid response,Relative Ca deficiency,*enough exposure to sunlight (spring, summer),or,VitD,administration,*concomitant inadequate Ca,absortion,from the intestine,Pathological condition,fever, infection, neonatal asphyxia, high P level diet,Prediposing factor,& Rickets of Vitamin D Deficiency,& Tetany of VitD Deficiency,Introduction,Chemical Pathology,Prediposing factor,Clinical manifestations,Diagnosis & Differential diagnosis,Treatment,Clinical manifestations,tetany and concurrent rickets,Manifest Tetany,(serum Ca,2+,0.75mmol/L,serum Ca1.75,mmol/L),latent tetany,(0.75 serum Ca,2+, 1mmol/L,1.75 serum Ca 1.88,mmol/L),Manifest Tetany,Convulsions,brief but recurrent, afebrile,conscious between seizures,Laryngospasm,inspiratory obstruction,high-pitched inspiratory crow apnea,Carpopedal spasm,(Fig ),Clinical manifestations,Fig. Carpopedal spasm,The wrist are flexed,the finger extended,the thumbs adducted over the palms,the feet extended and adducted.,latent tetany,symptoms are not evident, but can be elicited,by,ischemia,or mechanical,or electrical,Chvostek sign, Peroneal sign, Trousseau sign,stimulation of,motor nerves,Clinical manifestations,Latent tetany,Chvostek,sign,facial spasm,tapping anterior to external auditory,meatus,7th(facial) cranial nerve,Contraction of the,orbicularis,oris,occurs with,a twitch of the,upperlip,or entire mouth,Peroneal,sign pedal spasm,tapping the head of fibula,peroneal,nerve,dorsiflexion,and abduction of the foot,Latent tetany,Trousseau sign,carpospasm,A blood pressure cuff on the arm is inflated,above the systolic blood pressure for 5 min,Ischemia of motor nerves by reducing the blood supply,Carpospasm,& Rickets of Vitamin D Deficiency,& Tetany of VitD Deficiency,Introduction,Chemical Pathology,Prediposing factor,Clinical manifestations,Diagnosis &,Differential diagnosis,Treatment,bases on,Clinical manifestations,manifest tetany, latent tetany,Biochemical changes,serum Ca,2+,0.751mmol/L,serum Ca10 min cardiac arrest！,not im or s.c. Local necrosis!,Treatment for rickets,Treatment,Thank you!,