Multisystem Disease Associated With Hematuria Flashcards

1
Q

Multisystem Disease Associated With Hematuria etiology ……?

A

systemic infections, systemic lupus erythematosus, Henoch-Schönlein purpura nephritis, Goodpasture disease, hemolytic-uremic syndrome, nephrotoxicity, and renal cortical necrosis.

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2
Q

HIV-associated nephropathy features …..?

A

HIV-associated nephropathy, direct viral infection of nephrons occurs because renal cells express a variety of lymphocyte chemokine receptors that are essential for and facilitate viral invasion.

The renal expression of HIV infection is quite variable and includes an immune complex injury and a direct cytopathic effect.

The classic histopathologic lesion of HIV-associated nephropathy is focal segmental glomerulosclerosis.

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3
Q

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by ….?

A

fever, weight loss, dermatitis, hematologic abnormalities, arthritis, and involvement of the heart, lungs, central nervous system, and kidneys

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4
Q

— — is the most important cause of morbidity and mortality in SLE.

A

Lupus nephritis

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5
Q

Lupus nephritis is a result of ….?

A

The deposition of circulating immune complexes, as well as the direct binding of autoantibodies to glomerular components with resultant complement stimulation.

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6
Q

WHO class I nephritis …..

A

Minimal mesangial lupus nephritis

No histologic abnormalities are detected on light microscopy but mesangial immune deposits are present on immunof luorescence or electron microscopy

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7
Q

WHO class II nephritis ……

A

Mesangial proliferative nephritis

Light microscopy shows both mesangial hypercellularity and an increased matrix, along with mesangial deposits containing immunoglobulin and complement

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8
Q

WHO class III nephritis ……?

A

Focal proliferative LN < 50% glomeruli involved
A. Active
A/C. Active and chronic
C. Chronic Lesions

characterized by both mesangial and endocapillary lesions.

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9
Q

WHO class IV nephritis ……..

A

Diffuse proliferative LN (>50% glomeruli involved); all may be with segmental or global involvement (S or G)

A. Active
A/C. Active and chronic
C. Chronic

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10
Q

WHO class V nephritis

A

Membranous lupus nephritis

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11
Q

WHO class VI ……?

A

Advanced sclerosing LN

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12
Q

Characteristics of class 4 lesion in LN

A

Other signs of active disease include capillary walls that are thickened secondary to subendothelial deposits (creating the characteristic wire-loop lesion), necrosis, and crescent formation

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13
Q

Most children with SLE are adolescent male or females …..?

A

females (female-to-male ratio of 5 : 1), and present with extrarenal manifestations

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14
Q

Lupus nephritis affects——% of pediatric patients with SLE………?

A

80%

And although it commonly presents within the first year of diagnosis, may occur at any time during the course of the disease

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15
Q

What is clinical remission in LN ….?

A

Clinical remission, defined as normalization of renal function and proteinuria, and

A serologic remission, defined as normalization of anti-DNA antibody, C3, and C4 levels.

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16
Q

For patients with more severe forms of nephritis (WHO classes III and IV) treatment choices ?..

A

More aggressive immunosuppressive regimens induction phase and a maintenance phase

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17
Q

The most commonly employed induction therapy in

LN 3 & 4 …..?

A

6 consecutive monthly intravenous infusions of cyclophosphamide at a dose of 500-1,000 mg/m2

Pulse intravenous methylprednisolone (1,000 mg/m2) is also used in addition to oral corticosteroids

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18
Q

Induction therapy in LN ?

A

mycophenolate mofetil was as efficacious as, or even superior to, cyclophosphamide, and is increasingly considered for use in children at a dosage of 600 mg/m2 per dose twice daily

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19
Q

Major side effects of mycophenolate mofetil …..?

A

diarrhea, leucopenia, and teratogenicity

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20
Q

Treatment for children with proliferative lupus nephritis refractory to steroids and other immunosuppressants …?

A

Rituximab, a chimeric monoclonal antibody specific for human CD20

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21
Q

Role of plasma paresis in SLE-LN ….?

A

Plasmapheresis is ineffective in lupus nephritis

unless there is accompanying thrombotic thrombocytopenic purpura or antineutrophilic cytoplasmic antibodyassociated disease

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22
Q

New therapies include in LN ?

A

Belimumab, a fully humanized monoclonal antibody against a type II transmembrane protein that functions in the normal survival and differentiation of B cells

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23
Q

Which is prescribed for most patients with SLE for extrarenal manifestations …..?

A

Hydrochloroquine

But is thought to have a beneficial effect in maintaining the remission in lupus nephritis

24
Q

Which is the most common small-vessel vasculitis in children, with a peak incidence in early childhood (4-6 yr of age)

A

Henoch-Schönlein purpura (HSP) and the current terminology is IgA vasculitis

25
Q

Most acceptabed pathophysiology of HSP and I gA nephropathy …?

A

Studies have identified defective glycosylation of the hinge region of IgA1 in patients with both HSP nephritis and IgA nephropathy

Recognition of the exposed hinge region of IgA1 by naturally occurring autoantibodies leads to formation of immune complexes that are deposited in the glomerular mesangium

26
Q

T he classic tetrad of HSP nephritis includes ….?

A

palpable purpura
arthritis or arthralgia,
abdominal pain, and
evidence for renal disease

27
Q

What is severe HSP nephritis and it’s treatment…?

A

severe HSP nephritis (>50% crescents on biopsy)

For children with severe histologic manifestations (>50% glomerular crescents), treatment with intravenous methylprednisolone pulses for 3 days, followed by a combination of oral prednisone (for 3 mo) and azathioprine or mycophenolate mofetil (extended course) may be considered

28
Q

For children with the most severe histology (>75% glomerular crescents) and progressive renal failure in
HSP treatment….. ?

A

intravenous steroids plus plasmapheresis may be considered.

If progression to end-stage renal disease occurs, renal transplantation is the treatment of choice

29
Q

Goodpasture disease is an autoimmune disease characterized by triad of ……

A

pulmonary hemorrhage,

rapidly progressive glomerulonephritis, and

elevated anti–glomerular basement membrane antibody titers

30
Q

Goodpasture disease is an autoimmune disease pathophysiology ….?

A

The disease results from an attack on these organs by antibodies directed against certain epitopes of type IV collagen, located within the alveolar basement membrane in the lung and glomerular basement membrane (GBM) in the kidney

31
Q

Kidney biopsy in Goodpasture disease ….?

A

Kidney biopsy shows crescentic glomerulonephritis in most patients. Immunofluorescence microscopy demonstrates the pathognomonic continuous linear deposition of immunoglobulin G along the GBM

32
Q

What is Presenting complaint of Goodpasture disease …?

A

Patients usually present with hemoptysis from pulmonary hemorrhage that can be life-threatening.

Concomitant renal manifestations include acute glomerulonephritis with hematuria, nephritic urinary sediment with cellular casts, proteinuria, and hypertension, which usually follow a rapidly progressive course

33
Q

Disease that can cause a pulmonary-renal syndrome…?

A

systemic lupus erythematosus,
Henoch-Schönlein purpura,
nephrotic syndrome–associated pulmonary embolism, and
ANCA-associated vasculitis (such as granulomatosis with polyangiitis and microscopic polyangiitis).

34
Q

Treatment for Goodpasture disease ….?

A

The prompt institution of plasmapheresis,
high-dose intravenous methylprednisolone, and cyclophosphamide often induces remission and improves survival times.

Retrospective cohort studies suggest that when this combination of treatments is started early, the majority of patients will have a good renal outcome

35
Q

HUS is characterized by the triad of …?

A

microangiopathic hemolytic anemia,

thrombocytopenia, and

renal insufficiency

36
Q

The most common form of HUS is caused by ….?

A

Shiga toxin–producing Escherichia coli (STEC)

which causes prodromal acute enteritis and is commonly termed STEC-HUS or diarrhea-associated HUS.

37
Q

Toxins implicated in HUS …?

A

In the subcontinent of Asia and in southern Africa, the toxin of Shigella dysenteriae type 1 is causative

In Western countries, verotoxin or Shiga toxin–producing E. coli (STEC) is the usual cause

38
Q

How common STEC-HUS ….?

A

STEC-HUS accounts for about 90% of all HUS cases in childhood.

39
Q

serotypes of E. coli can produce the toxin responsible for

HUS ..?

A

O157:H7 is most common in Europe and the Americas.

A large epidemic of HUS in Europe was caused by Shiga toxin–producing E. coli O104:H4

40
Q

How is STEC transmission occurs ?…?

A

The reservoir of STEC is the intestinal tract of domestic animals, usually cows.

Disease commonly is transmitted by undercooked meat or unpasteurized (raw) milk and apple cider

41
Q

Streptococcus pneumoniae and HUS ….?

A

A rare but distinct entity of infection-triggered HUS is related to neuraminidase producing Streptococcus pneumoniae

typically severe, develops during acute infection with this organism, typically manifesting as pneumonia with empyem

42
Q

What is atypical HUS …..?

A

Genetic forms of HUS (atypical, nondiarrheal)

43
Q

Genetic forms of HUS (atypical, nondiarrheal) due to deficiency of ….?

A

Inherited deficiencies of either von Willebrand factor–cleaving protease (ADAMTS13) or complement factor H, I, or B can cause HUS.

44
Q

A major feature characteristic of genetic forms of HUS ….?

A

absence of a preceding diarrheal prodrome

45
Q

Is pneumococcal-associated HUS anemia Coombs negative or positive …?

A

anemia with a positive direct Coombs test

neuraminidase cleaves sialic acid on membranes of endothelial cells, red cells, and platelets to expose the underlying cryptic ThomsenFriedenreich (T) antigen. Endogenous immunoglobulin M (IgM) antibodies recognize and react with the T antigen to trigger hemolysis and anemia

46
Q

Onset of HUS after how many days of diarrhoea …?

A

onset of HUS occurs 5-7 days after the onset of gastroenteritis with fever, vomiting, abdominal pain, and diarrhea

47
Q

Following the prodromal illness, the sudden onset of pallor, weakness, and lethargy heralds the onset of ……?

A

HUS, and it reflects the development of microangiopathic hemolytic anemia.

48
Q

Dialysis in HUS how long to continue ….

.?

A

2 wks

49
Q

How recovery starts in

HUS …..?

A

Recovery of platelet counts usually occurs first,

followed by renal recovery about 5 days later, and

finally by resolution of anemia

50
Q

Prognosis of HUS ….?

A

the mortality rate for diarrhea-associated HUS is < 5% in most major medical center

51
Q

Renal outcome in HUS ..?

A

Most recover renal function completely, but of surviving patients,

5% remain dependent on dialysis, and

up to 30% are left with some degree of chronic renal insufficiency

52
Q

Role of antibiotics in HUS …?

A

Antibiotic therapy to clear enteric toxigenic organisms (STEC) can result in increased toxin release, potentially exacerbating the disease, and therefore is not recommended

53
Q

Role of plasmapheresis in HUS …..?

A

Plasma infusion or plasmapheresis has been proposed for patients suffering severe manifestations of HUS with serious CNS involvement

54
Q

What is eculizumab and it’s role which type of HUS ….?

A

Eculizumab is an anti-C5 antibody that inhibits complement activation, a pathway that contributes to active disease in some forms of atypical familial HUS; this pathway may also contribute to the process in STECHUS

55
Q

How NSAIDs can cause the nephropathy ..?

A

NSAIDs in febrile children with concomitant dehydration, NSAIDs can inhibit the production of intrarenal vasodilatory prostaglandins, thereby leading to decreased renal perfusion and acute kidney injury.