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1、urinary system,上海交通大学医学院附属新华医院 儿内科教研室 卫敏江,urinary system,Urinary system includes the kidney、ureter、bladder and urethra . The kidney is an important excretory organ , also , it is an essential adjuster and endocrine organ to maintain a precise balance of internal environment .,anatomical character ne

2、onate larger,inmature,small glomerulus ,short tubule infant Ureter is much longer and incurvater, tube muscles and elastic fibers are dysontogenesis . Ureter close to the bladder is straighter and shorter, it is poor to prevent urine reverse flow. female infant has a shorter urethra ,but male infant

3、 often has capistration,physiologic character glomerular filtration commences in the metanephric kidney at about 9 to 12 weeks of gestation, the full complement of nephrons is present at 36 weeks of gestation . low reserve capacity ,inmature regulatory mechanism,easy to dysfunction 。 the renal funct

4、ion(calculated by body weight or body surface area) reaches a value comparable to adult at 1 to 2 years of age.,glomerular filtration rate neonate the first week of birth : glomerular filtration 2 year-old: adult level pathology extra moistureand solutecan not excretion in time edema,the reabsorptio

5、n and the excretory function of renal tubule glomerulotubular balance neonate has already possess glomerulotubular balance ,but inmature。 amino acid/glucose term infant : normal reabsorption capacity premature infant: renal tubule function deficiency, glucose threshold glucosuria (reabsorpt glucose

6、),natrium neonate aldosterone natrium positive balance glomerular filtration rate natrium load natriume excretion edema infant of low-birth weight natriume duction if natriume intake (3mmol/kg/d)hyponatremia shock or convulsion,potassium because Na-K-ATP enzyme system of renal tubules epithelium is

7、inmature, neonate potassium excretionin 10 ayshyperkalemia tendency。,acid-base balance kidneyexcret H、reabsorb HCO3 acid-base balance at 2 weeks after birth the urine PH get the adult level at 2 years old the capability of ammonia excretion close to adult level renal threshold of HCO3 in neonate is

8、low。,urine concentration and dilution,urine dilution ability of newbron or infants close to adult glomerular filtration ratebody protein anabolism activelyurea excretioncan not form enough osmotic pressure gradient in kidney medulla urine concentration ,renal endosecretory function renin prostagland

9、in kallikrein erythropoietin 1,25-dihydroxycholecalciferol,urinate character,neonate urination in 24Hr (93%) urination in 48Hr(99%) pathology oliguria 400ml/d anuria 50ml/ /d daily urinary volume (ml)= 400+100(age1),urine character,yellow, clarity,specific gravity of urine close to adult after 1 yea

10、r,24Hr Urine protein 100mg/m2 (150mg/d)、RBC 、 WBC、 urinary cylinder 、 Addiss count、 pH,examination of kidney disease,EXAMNATION OF THE KIDNEY DISEASE,EVALUATION OF THE KEDNEY AND URINARY TRACT blood-vascular system renal angiography nephrogram,nephrogram,renal insufficiency,EXAMNATION OF THE KIDNEY

11、DISEASE,EVALUATION OF THE KEDNEY AND URINARY TRACT Renal glomeruli proteinuria urinary sediment blood urea nitrogen (BUN) creatinine (Cr) blood2MG creatinine clearance (Ccr) = Kheight (cm) Scr (mg/dl) ( K: 1岁infant of low-birth weight =0.33; 1岁mature infant = 0.45;212岁 = 0.55 ) Cystatin C ( Cys- C )

12、, proteinuria quantitative examination 150mg/d tubular proteinuria 1g/24Hr,small molecule proteinuria nonselective proteinuria 、selective proteinuria urine IgG/serum IgG selective proteinuria index = urine alb/serum alb index0.1 selective, 0.2 nonselective,EXAMNATION OF THE KIDNEY DISEASE,EXAMNATION

13、 OF THE KIDNEY DISEASE,EVALUATION OF THE KEDNEY AND URINARY TRACT proximal renal tubule urine2MG urine trace protein series urine enzyme examination N-aceto- -glucosaminidase (NAG),EXAMNATION OF THE KIDNEY DISEASE EVALUATION OF THE KEDNEY AND URINARY TRACT medullary loop and distal tubule urinometry

14、 urine osmotic pressure free water scavenger Mos test,EXAMNATION OF THE KIDNEY DISEASE EVALUATION OF THE KEDNEY AND URINARY TRACT disreabsorpion intravenous pyelography (IVP) retrograde cystopyelography nephrogram,EXAMNATION OF THE KIDNEY DISEASE EVALUATION OF THE KEDNEY AND URINARY TRACT endocrine

15、in kidney: out of kidney:target organ out of kidney : degradation,imaging examination,vesicoureteral reflux,renal puncture biopsy,负压吸引针,负压吸引针,TRU-CORE活检枪,TRU-CORE活检枪,glomerular diseases typing,clinical typing 1. primary glomerular diseases 1).glomerulonephritis acute glomerulonephritis,glomerular di

16、seases typing,clinical typing 1. primary glomerular diseases 1). glomerulonephritis rapidly progressive glomerulonephritis,glomerular diseases typing,clinical typing 1. primary glomerular diseases 1). glomerulonephritis persistent glomerulonephritis,glomerular diseases typing,clinical typing 1. prim

17、ary glomerular diseases 1). glomerulonephritis chronic glomerulonephritis,glomerular diseases typing clinical typing 1. primary glomerular diseases 2 ). nephrotic syndrome simple nephropathy nephrosonephritis,glomerular diseases typing clinical typing 2.secondary glomerular diseases lupus nephritis

18、purpura nephritis HBV-associated glomerulonephritis renal toxicitic drugs,etc,系膜细胞中度增生,glomerular diseases typing clinical typing 3. hereditary glomerular diseases 1) congenital nephrotic diseases 2) hereditary grogressive nephritis ( Alport syndrome ) 3) familial recurrent hematuria (thin basement

19、membrane nephropathy ) namal basement membrane 32036m,Pathological changes of glomerular nephritis, minimal change nephropathy(MCV) mesangial proliferative glomerulonephritis(MsPGN) membranate glomerulonephritis(MN) membranoproliferative glomerulonephritis(MPGN) focal glomerular sclerosis (FSGS) end

20、ocapillary proliferative glomerulonephritis(ECPGN) extracapillary proliferative glomerulonephritis crescentic glomerulonephritis,Pathological changes of glomerular nephritis, minimal change nephropathy(MCV) mesangial proliferative glomerulonephritis(MsPGN) membranate glomerulonephritis(MN) membranop

21、roliferative glomerulonephritis(MPGN) focal glomerular sclerosis (FSGS) endocapillary proliferative glomerulonephritis(ECPGN) extracapillary proliferative glomerulonephritis crescentic glomerulonephritis,Pathological changes of glomerular nephritis, minimal change nephropathy(MCV) mesangial prolifer

22、ative glomerulonephritis(MsPGN) membranate glomerulonephritis(MN) membranoproliferative glomerulonephritis(MPGN) focal glomerular sclerosis (FSGS) endocapillary proliferative glomerulonephritis(ECPGN) extracapillary proliferative glomerulonephritis crescentic glomerulonephritis,Pathological changes

23、of glomerular nephritis, minimal change nephropathy(MCV) mesangial proliferative glomerulonephritis(MsPGN) membranate glomerulonephritis(MN) membranoproliferative glomerulonephritis(MPGN) focal glomerular sclerosis (FSGS) endocapillary proliferative glomerulonephritis(ECPGN) extracapillary prolifera

24、tive glomerulonephritis crescentic glomerulonephritis,Pathological changes of glomerular nephritis, minimal change nephropathy(MCV) mesangial proliferative glomerulonephritis(MsPGN) membranate glomerulonephritis(MN) membranoproliferative glomerulonephritis(MPGN) focal glomerular sclerosis (FSGS) end

25、ocapillary proliferative glomerulonephritis(ECPGN) extracapillary proliferative glomerulonephritis crescentic glomerulonephritis,Pathological changes of glomerular nephritis, minimal change nephropathy(MCV) mesangial proliferative glomerulonephritis(MsPGN) membranate glomerulonephritis(MN) membranop

26、roliferative glomerulonephritis(MPGN) focal glomerular sclerosis (FSGS) endocapillary proliferative glomerulonephritis(ECPGN) extracapillary proliferative glomerulonephritis crescentic glomerulonephritis,Pathological changes of glomerular nephritis, minimal change nephropathy(MCV) mesangial prolifer

27、ative glomerulonephritis(MsPGN) membranate glomerulonephritis(MN) membranoproliferative glomerulonephritis(MPGN) focal glomerular sclerosis (FSGS) endocapillary proliferative glomerulonephritis(ECPGN) extracapillary proliferative glomerulonephritis crescentic glomerulonephritis,problem,clinical clas

28、sification of Renal glomerular disease evaluation of renal function why the neonate easy to be dehydrate or edema when its in diarrhea or overdose fluid infusion,urinary system,上海交通大学医学院附属新华医院 儿内科教研室 卫敏江,Acute glomerulonephritis,definition,diffuse glomerular apyogenous inflammation caused by postinf

29、ectious immunoreaction. clinical manifestation:acute disease ,haematuria, edema, hypertension,Pathogen,Bacteriastreptococcus (hemolytic streptococcus is the most popular ),staphylococci , pneumococcus, meningococcus Virusmumps virus 、coxsackie B4 virus 、echo-9 virus 、influenza virus, etc other mycop

30、lasma pneumoniae , plasmodium, etc Acute poststreptococcal glomerulonephritis( APSGN),pathogeny,pathogenic strain (antigen) body produce antibody antigen-antibody complex (dissolubility) aggradate at GBM activatcomplement immune inflammatory response,I mmune inflammatory response diffuse glomerular

31、inflammation endothelial cells proliferation middle molecules protein : molecular weight 79.9Mr(albumin); macromolecule protein : molecular weight 10Mr(IgG15Mr), selective albuminuria/nonselective albuminuria serum albumin (30g/L)。 cholesterin (5.7mmol/L) serum complement determine laboratory examin

32、ation of systemic disease laboratory examination of hypercoagulative state&thrombosis biopsy,simple NS nephritis NS,Age 16 7 pathological change MCD MsPGN 、MPGN 、FSGS symptom four chief symptoms four chief symptoms hypertensionhematuria Albuminuria selective nonselective Complemen normal some reduce

33、 Renal function normal some reduce Urine FDP negative positive Reaction to good ,easy to relapse poor glucocorticoid course of the disease relieve in 48weeks delayed easy to relapse , 12year,complication,1. infection URI, skin , urinary tract , peritonitis, etc. 2. hypovolemic shock 3. electrolyte d

34、isturbance hyponatremia , low potassium phosphate , hypocalcemia, etc. 4. thrombogenesis 5. acute renal failure 6. renal tubules malfunction 7. delayed growth,treatment,general treatment rest , diet, sodium 12g/day,intake VitD,calcium,protein 1.52g/kg/day infection prevention vaccination 、the infect

35、ion problem during the glucocorticoid Detumescence&diuresis pay attention to acid-base balance&electrolyte disturbance,Glucocorticoid treatment,mechanism: decrease immunoreaction,improve capillary permeability ,reduce urine protein eduction. Decrease ADH&ALD,inncrease GFR, diuresis short range almos

36、t desuetude intermediate range 1.52mg/Kg/d, tid-qid , continue dose till 2 weeks after urine protein is change to negative(need 4 weeks to basically completed ) The two-thirds dose of the total dose of 2 days , qod ,4weeks ,then reduce 510mg per 2-4weeks (need 6 months to basically completed ) long

37、range same as the intermediate range, then reduce 2.55mg per 24weeks (need 9 months to basically completed ) Long term low dose glucocorticoid (0.4mg/Kg/qod), would not lead to side effect. Enough dose at first, decrease dose slowly, maintenance dose should be long.,immunosuppressor,indication a. Fr

38、equent relapse: relapse& reiteration 1year3 times、6 months twice。 b. Glucocorticoid dependence :sensitive to glucocorticoid, can relieve,but relapse or reiteration in 2 weeks if dose reduction/drug withdrawal ,repeat 23 times 。 c. Glucocorticoid Resistant : regular glucocorticoid treatment 8weeks ,urine protein + +,immunosuppressor,Cyclophosphamide per os : 2.53mg/Kg/d,accumulated dose 200mg/Kg,course 3 months in vein stosstherapy : 812mg/Kg/time,accumulated dose 150mg/Kg, per 2 weeks ,gradually extend ntermittence according

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