נפרו Flashcards
MCNS - steroids regimen
In children with presumed MCNS, prednisone or prednisolone should
be administered as a single daily dose of 60 mg/m2/day or 2 mg/kg/day
to a maximum of 60 mg daily for 4-6 wk followed by alternate-day
prednisone (starting at 40 mg/m2 qod or 1.5 mg/kg qod) for a period
ranging from 8 wk to 5 mo, with tapering of the dose.
MCNS response define as? remission? relapse? frequently relapsing? steroid dependent? steroid resistance?
Response is defined as the attainment of remission within the initial
4 wk of corticosteroid therapy. Remission consists of a urine
protein:creatinine ratio of < 0.2 or <1+ protein on urine dipstick testing
for 3 consecutive days.
Relapse is an increase in the first morning urine
protein:creatinine ratio > 0.2 or a reading of 2+ and higher for 3 consecutive
days on Albustix testing. Frequently relapsing is two or more
relapses within 6 mo after the initial therapy or four relapses in a 12-mo
period. Steroid dependent is a relapse during steroid tapering or a
relapse within 2 wk of the discontinuation of therapy
Managing the Clinical Sequelae of
Nephrotic Syndrome
Edema
Children with severe symptomatic edema, including large
Edema.
pleural effusions, ascites, or severe genital edema, should be hospitalized.
In addition to sodium restriction (<1,500 mg daily), water/fluid restriction
may be necessary if the child is hyponatremic.
administration of loop diuretics - possible although extreme caution should be exercised (afraid of intravascular volume depletion > Renal failure, thrombosis.
when will we administer albumin in Nephrotic syndrome
When a patient has severe generalized edema with evidence of intravascular Volume depletion (e.g., hemoconcentration, hypotension, tachycardia), intravenous administration of 25% albumin (0.5-1.0 g
albumin/kg) as a slow infusion followed by furosemide (1-2 mg/kg/dose intravenously) is sometimes necessary
close monitoring of volume status, blood pressure, serum electrolyte, balance, and renal function. (afraid of overload volume)
Dyslipidemia. nephrotic syndrome
Dyslipidemia should be managed with a low-fat diet.
INfections nephrotic syndrome - treatment
which antibiotics should be administered
If there is a suspicion of infection,
a blood culture should be drawn prior to starting empirical antibiotic therapy. In the case of spontaneous bacterial peritonitis, peritoneal fluid should be collected if there is sufficient fluid to perform a paracentesis
and sent for cell count, Gram stain, and culture. The antibiotic provided
must be of broad enough coverage to include Pneumococcus and Gram negative
bacteria. A third-generation cephalosporin is a common choice of intravenous antibiotic
To reduce the risk of serious infections in children with nephrotic syndrome, give the full pneumococcal vaccination (with the 13-valent conjugant vaccine and 23-valent polysaccharide vaccine) and influenza vaccination annually to the child and their household contacts
Thromboembolismnephrotic syndrome - treatment
suspicion > DI > studies to delineat specific underlying hypercoagublable state > Anticoagulation therapy > heparin,
low-molecular-weight heparin, and warfarin are therapeutic options
Obesity and Growth. nephrotic syndrome - treatment
Growth may be affected in children
who require long-term corticosteroid therapy. Steroid-sparing strategies
may improve linear growth in children who require prolonged courses
of steroids
when is relapse of nephrotic syndrome is common & treatment
Relapses are common,
especially in younger children, and are often triggered by upper respiratory
or gastrointestinal infections
Alternative Therapies to Corticosteroids in the Treatment of
Nephrotic Syndrome.
Cyclophosphamide
prolongs the duration of remission and reduces the number
of relapses in children with frequently relapsing and steroid-dependent
nephrotic syndrome. The potential side effects of the drug (neutropenia,
disseminated varicella, hemorrhagic cystitis, alopecia, sterility, increased
risk of future malignancy) should be carefully reviewed with the family
before initiating treatment.Cyclophosphamide (2 mg/kg) is given as a
single oral dose for a total duration of 8-12 wk. Alternate-day prednisone therapy is often continued during cyclophosphamide administration
Calcineurin inhibitors (cyclosporine or tacrolimus) are recommended
as initial therapy for children with steroid-resistant nephrotic syndrome.
Children must be monitored for side effects, including hypertension,
nephrotoxicity, hirsutism, and gingival hyperplasia. Mycophenolate can
maintain remission in children with steroid-dependent or frequently
relapsing nephrotic syndrome.
rituximab, the chimeric monoclonal antibody against CD20-targeting
B cells, in children with steroid-dependent and/or steroid-resistant
nephrotic syndrome.
Mesangial proliferation is characterized by a diffuse increase in
mesangial cells and matrix on light microscopy
Immunofluorescence
microscopy might reveal trace to 1+ mesangial IgM and/or IgA staining.
Electron microscopy reveals increased numbers of mesangial cells and
matrix as well as effacement of the epithelial cell foot processes.
Approximately 50% of patients with this histologic lesion respond to
corticosteroid therapy.
focal segmental glomerulosclerosis (FSGS)
Immunofluorescence microscopy is
positive for IgM and C3 staining in the areas of segmental sclerosis. Electron microscopy demonstrates segmental scarring of the glomerular
tuft with obliteration of the glomerular capillary lumen.
what is the most common chronic glomerular disease in
children?
IgA nephropathy
It is characterized by a predominance of IgA within mesangial
glomerular deposits in the absence of systemic disease. Its diagnosis
requires a renal biopsy
IgA nephropathy clinical and lab manifestations
male>female
usually clinical presentationd in children is benign (VS adults)
uncommon cause for end-stage renal failure during childhood
gross(west) hematuria /micro(japan) hematuria+ proteinuria
gross hematuria (at most cases) - usually 1-2d after respiratory/GI infection
Proteinuria is
often < 1,000 mg/24 hr in patients with asymptomatic microscopic
hematuria.
Mild to moderate hypertension is most often seen in patients
with nephritic or nephrotic syndrome, but is rarely severe enough
Normal serum levels of C3 in IgA
nephropathy help to distinguish this disorder from postinfectious
glomerulonephritis
Serum IgA levels have no diagnostic value
IgA nephropathy - Prognosis
progressive disease develops in 20–30% of adult patients
15-20 yr after disease onset
Poor prognostic
indicators at presentation or follow-up include persistent hypertension,
diminished renal function, and significant, increasing, or prolonged
proteinuria.
A more severe prognosis is correlated with histologic evidence
of diffuse mesangial proliferation, extensive glomerular crescents,
glomerulosclerosis, and diffuse tubulointerstitial changes, including
inflammation and fibrosis.
IgA nephropathy treatment
1 appropriate BP contro & mgmt of significant proteinuria
ACEi ARB - effective in reducing proteinuria and retarding the rate of disease
progression when used individually or in combination
Fish oil, which
contains antiinflammatory omega-3 polyunsaturated fatty acids, may
decrease the rate of disease progression in adults
if RAS bloclade ineffective > addition immunosuppression - corticosteroids -reduce proteinuria and improve renal
function in those patients with a glomerular filtration rate > 60 mL/
min/m2.
Acute Poststreptococcal
Glomerulonephritis
nephrotic/nephritic?
Clinical meanifestations
acute nephritic syndrome
sudden onset of gross hematuria, edema, hypertension, and renal
dysfunction. It
The severity of kidney involvement varies from asymptomatic
microscopic hematuria with normal renal function to gross hematuria
with acute renal failure.
patients can develop various degrees of edema, hypertension, and oliguria.
Patients are at risk for developing encephalopathy and/or heart failure
secondary to hypertension or hypervolemia. Hypertensive encephalopathy
must be considered in patients with blurred vision, severe headaches,
altered mental status, or new seizures
Respiratory distress, orthopnea, and cough
may be symptoms of pulmonary edema and heart failure. Peripheral
edema typically results from salt and water retention and is common
Respiratory distress, orthopnea, and cough
may be symptoms of pulmonary edema and heart failure. Peripheral
edema typically results from salt and water retention and is common;
nephrotic syndrome develops in a minority (<5%) of childhood cases.
Atypical presentations of APSGN include those with subclinical disease
and those with severe symptoms but an absence of initial urinary
abnormalities; in individuals who present with a purpuric rash, it is
difficult to distinguish APSGN from Henoch-Schönlein purpura without
a renal biopsy
The acute phase generally resolves within 6-8 wk. Although urinary
protein excretion and hypertension usually normalize by 4-6 wk after
onset, persistent microscopic hematuria can persist for 1-2 yr after the
initial presentation
APSGN - diagnosis
UA – RBC, blood casts, proteinuria, and polymorphonuclear leukocytes
proteinuria, and polymorphonuclear leukocytes. A mild
normochromic anemia may be present from hemodilution and low-grade hemolysis.
The serum C3 level is significantly reduced in > 90% of
patients in the acute phase, and returns to normal 6-8 wk after the
onset. Although serum CH50 is commonly depressed, C4 is most often
normal in APSGN, or only mildly depressed.
Magnetic resonance imaging of the brain is indicated in patients with
severe neurologic symptoms and can demonstrate posterior reversible
encephalopathy syndrome in the parietooccipital areas on T2-weighted
images. Chest x-ray is indicated in those with signs of heart failure or
respiratory distress, or physical exam findings of a heart gallop, decreased
breath sounds, rales, or hypoxemia
DD - Acutte nephritic syndrome, evidence of recent strep infection and low level of C3 ?
However, it is important to
consider other diagnoses such as systemic lupus erythematosus, endocarditis,
membranoproliferative GN, and an acute exacerbation of chronic
GN.
Renal biopsy should be considered only in the presence of acute
renal failure, nephrotic syndrome, absence of evidence of streptococcal
infection, or normal complement levels
Henoch-Schönlein Purpura Nephritis
an idiopathic systemic immune
complex–mediated vasculitis associated with IgA deposition within
small-vessel walls. The current terminology for HSP is IgA vasculitis
It is the most common small-vessel vasculitis in children, with a peak
incidence in early childhood (4-6 yr of age).
about half the cases preceded by an upper
respiratory infection.
Hemolytic-Uremic Syndrome
Hemolytic-uremic syndrome (HUS) is a common cause of community
acquired
acute kidney injury in young children. It is the most common
form of thrombotic microangiopathy (TMA) in children. Like all
TMAs, HUS is characterized by the triad of microangiopathic hemolytic
anemia, thrombocytopenia, and renal insufficiency. HUS has clinical
features in common with thrombotic thrombocytopenic purpura (TTP)
(see Chapter 511.05). The etiology and pathophysiology of the more
common forms of HUS clearly delineate typical childhood HUS as
separate from idiopathic TTP.
Typical HUS - most common cause?
The most common form of HUS is caused by Shiga
toxin–producing Escherichia coli (STEC), which causes prodromal acute
enteritis and is commonly termed STEC-HUS or diarrhea-associated
HUS.
STEC-HUS accounts for about 90% of all HUS cases in childhood.
reservoir of STEC is the intestinal tract of domestic animals, usually
cows. - transmitted uncooked meat, unpasteurized milk and apple cider.
second major category of HUS cause
genetic forms, atypical nondiarrheal
inherited deficiencies of either von Willebrand factor–cleaving protease (ADAMTS13) or
complement factor H, I, or B can cause HUS.
