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