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腎病症候群
腎臟內科
學習目標
PGY UGY
知識
1.腎病症候群相關的影像檢查
2.腎切片檢查的照護
3.腎病症候群病人的處理程序
4.透析病人的照護
5.透析相關併發症的防治
知識
1.腎病症候群的定義
2.腎病症候群的臨床表現
3.腎病症候群的病理生理機制
4.腎病症候群的診斷流程
5.尿毒症的病理生理機制
6.透析的基本原理
7.腎病症候群相關的實驗室檢查判讀,包括BUN/Cr、Ca、P、電解質、動脈血氣體分析、尿液分析、尿鈉、血清及尿之滲透壓、計算鈉離子之fraction excretion
8. 照會做腎超音波之適應症及判讀
技能
1.腎病症候群相關的病史詢問
2.腎病症候群相關的身體檢查
Progression of glomerular diseases
GN -> tubular obstruction, interstitial changes, changes in vascular resistance ->
Renal fibrosis
irreversible renal failure
Glomerular disease are immune-mediated
glomerulonephritis (GN)
Confusion points about classification of glomerular diseases
Nomenclature:--Etiology
--Pathology
--Clinical presentation
Classification of glomerular diseases
Javaid et al, Kidney Int 2005:67:1692-1703
Etiology Pathology
Chadban et al Lancet 2005:356:1797-1806
Classification of glomerular diseases
Classification of glomerular diseases
Etiology
Yan et al Chin J Med 2018:131:731-735
Classification of glomerular diseases
Etiology
Classification of glomerular diseases
Pathology
Chadban et al Lancet 2005:356:1797-1806
Classification according to the clinical manifestation
is the simplest and most effective tool for the clinical. Chadban et al Lancet 2005:356:1797-1806
Classification of glomerular diseases
Clinical
Glomerular
injury
Hereditary
Acquired
Primary
(idiopathic,
immune)
Secondary
Nephrotic
Nephritic
RPGN
Pulmonary-Renal Syndromes
MCD
FSGS
MN
MPGN
Glomerular deposition diseases
IC
ANCA small vessel vasculitis
Anti-GBM
Primary (idiopathic)
Secondary
IgAN Primary
Secondary
Nephrotic
Nephritic
Congenital nephrotic syndrome
Alport syndrome
Thin-basement menbrant
Asymptomatic hematuria or proteinuria
Classification of glomerular diseases
Classification of glomerular diseases
Classification of glomerular diseases
Definition heavy proteinuria, minimal hematuria, hypoalbuminemia,
hypercholesterolemia, edema, and hypertension
Non-immune treatment:
Hypercholesterolemia: lipid-lower agents
Edema: diuretics
Hypercoagulable state: anticoagulants
Proteinuria: RAS system inhibitors
Immune treatment: depends on individual diseases
Six common entities: (> 90% in adults)
Minimal change disease (MCD), Focal segmental glomerulosclerosis (FSGS)
Membranous nephropathy (MN), Membranoproliferative glomerulonephritis
(MPGN), glomerular deposition diseases, and diabetic nephropathy
Nephrotic syndrome
Incidence Nephrotic syndrome: 70-90% in children ( <16 years)
10-15% in adults
Clincial pictures
Nephrotic syndrome: typical, selective proteinuria
Hematuria: 20-30%
Hypertension and renal failure: rare
Renal patholocial findings
EM: diffuse effacement of the foot processes of visceral epithelial cell
Minimal Change Disease
Nephrotic syndrome常見疾病
Genetic predisposition: HLA-B12 Immune-related mutation in nephrin, α-actinin-4, and podocin
Etiology
Minimal Change Disease
Nephrotic syndrome常見疾病
Spontaneous remission: 30-40% in children
Rare in adults
Highly steroid-responsive
8 weeks high-dose oral glucocorticoid
Children: 60 mg/m2 qd x 4 weeks
40 mg/m2 qod x 4 weeks
--- 90% remission
Adult: 1 mg/kg/day x 4 weeks
1 mg/kg qod x 4 weeks
--- 50% remission
steroid dependent: relapse during or shortly after withdrawal of steroid
frequently relapse: relapse more than three times per years
added cyclophosphamide, chlorambucil, and cyclosporine
Treatment
Minimal Change Disease
Nephrotic syndrome常見疾病
Incidence One-thirds of cases of nephrotic syndrome in adults
Sclerosis with hyalinosis involving portions (segmental) of fewer than 50% (focal)
of glomeruli on a tissue section.
Clinical pictures
Nephrotic syndrome: 66% of patients, non-selective
Subnephrotic proteinuria: 33%
Hypertension, abnormal urine sediment (RBC & WBC)
Renal pathological findings
Focal Segmental Glomerulosclerosis
Nephrotic syndrome常見疾病
Etiology
Primary FSGS: unclear, immunologic (partly), mutation, urokinase receptor
Focal Segmental Glomerulosclerosis
Nephrotic syndrome常見疾病
Spontaneous remission: rare
Renal prognosis is relatively poor
Glucocorticoid therapy: 16-24 weeks
poor prognostic factors: hypertension, abnormal renal function,
black race, and persistent heavy proteinuria
adjuvant therapy: cyclosphosphamide, cyclosporine,
and mycophenolate mofetil
Renal transplantation: recurrence 50%, graft loss 10%
poor prognostic factors: a short time interval between the onset of
the FSGS and ESRD, young age at onset,
presence of mesangial hypercellularity on
renal biopsy
Treatment
Focal Segmental Glomerulosclerosis
Nephrotic syndrome常見疾病
Incidence Leading cause of idiopathic nephrotic syndrome in adults
(30-40%, aged: 30-50 years)
A rare cause in children: < 5%
Male: Female = 2:1.
Clinical pictures Nephrotic syndrome:80%, non-selective
Hematuria: 50%, associated with hypertension
Renal pathological findings
Membranous nephropathy
Nephrotic syndrome常見疾病
Idiopathic: incompletely understood, phospholipase A2 receptor, neutral endopeptidase
and cationic bovine serum albumin
Etiology
Membranous nephropathy
Nephrotic syndrome常見疾病
Spontaneous remission: 40%
ESRD: 10-20%
poor prognostic factors: male, older age, hypertension, severe
proteinuria, and hyperlipidemia, and
impaired renal function
Immunosuppressive agents: glucocorticoid, cyclophosphamide,
chlorambucil, and cyclosporine.
Kidney transplantation: a successful treatment for ESRD
Treatment
Membranous nephropathy
Nephrotic syndrome常見疾病
Also known a mesangiocapillary GN or lobar GN
Thickening of the GBM and proliferative changes
Clinical pictures 70%: hypocomplementemia
subdivided pathologically into type I, type II, and type III disease. Type I: heavy proteinruia, nephrotic syndrome. active urinary sediments
Type II and III: nephrotic syndrome, nephritic syndromen and RPGN
Membranoproliferative glomerulonephritis
Renal pathological findings
Nephrotic syndrome常見疾病
Type I MPGN:
secondary to glomerular deposition of circulating immune complexes or
their in situ formation.
Types II and III MPGN:
may be related to “nephritic factors”
Membranoproliferative glomerulonephritis
Nephrotic syndrome常見疾病
Type I Type II
Membranoproliferative glomerulonephritis
Nephrotic syndrome常見疾病
World J Nephrol 2016 July 6; 5(4): 308-320
Membranoproliferative glomerulonephritis
Nephrotic syndrome常見疾病
Treatment: Non-immune:
RAS inhibitors for proteinuria and hypertension
Dipyridamole, Coumadin or cyclophosphamide: not strongly established
Immune: steroid, plasma exchange and immunosuppressive agents
Prognosis: 50% ESRD, 10 years later
90% CKD, 20 years later
Poor prognostic factors: hypertension, nephrotic syndrome
and renal insufficiency
Plasma cell dyscrasias -> excess light chain immunoglobulin -> glomerular and tubular
deposits (nonorganized, granular and organized, fibril) -> heavy proteinuria renal
failure
Light chain deposition, Renal amyloidosis, Fibrillary-imunotactoid glomerulopathy
Glomerular deposition diseases
Clin J Am Soc Nephrol. 2016 Dec 7;11(12):2260-2272
Nephrotic syndrome常見疾病
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