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מבחן גמר בילדים > ראומטולוגיה > Flashcards

ראומטולוגיה Flashcards

1
Q

Dgx of JIA?

A

Arthritis < 16 yrs of age
symptoms > 6 wks

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2
Q

Lines of Tx for JIA?

A

1st line- NSAIDS + steroids to joint
2nd line- MTX

in Systemic JIA- 1st line MTX

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3
Q

most common subtype of JIA?

and what is the peak of age

A

oligoarticular < 5 joints involved, a-symmetrical

age 2-4. girls > boys

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4
Q

Clinical and Ab of oligo articular

A

ANA ab- 60%
anterior uveitis - 30%

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5
Q

oligo JIA

how many will have full remission?

which things are indicated for risk for chronic ant. uveitis?

A

90% full remmison

risk for chronic ant. uveitis?
pt with
* ANA+
* Onset < 6 age
* disease prolong > 3years

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6
Q

in polyarthritis JIA, Reumatic nodules are a/w which type of Ab?

A

RF

seen more at adolesence and not young kids

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7
Q

Which Ab is involve in bad prognosis of polyarthritis JIA?

A

RF +
anti-CCP

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8
Q

which type of JIA will be seen with:
leukocytosis
high ferritin, CRP, ESR
Thrombocytosis

and solomon rash?

A

Systemic JIA- present with fever
*salomon rash can accompnied the fever

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9
Q

Which extra-joints menefistation can be seen in systemic JIA?

A

Serositis 50% (pleuritis, pericarditis)
Hepatosplenomegaly- 70%

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10
Q

Which life therathing complication can be seen in systemic JIA?

and which lab valuse with right clinical presenation will raise our suspicion

A

Macrophage activation syndrome (MAS)
encephalopathy,multi organ damage Liver issues, prolong PT+PTT

Low ESR

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11
Q

How to dgx macrophage activation syndrome (MAS)?

and how can we confirm our Dgx?

A

Criteria- extremly eleveted ferritin + 2 of the following:
1. Thrombocytopenia
2. eleveted Liver enzym
3. Hypofibrinogemia
4. Hyper TG

confirm Dgx
Bone merrow present with hemophagocytosis

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12
Q

Tx for MAS (macrophage activation syndrome)

A

high dose IV methylprednisone
+
cyclosporine or anakirna

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13
Q

What is the duration and joint involvment of post Sterp arthritis?

A

Could last for months.
mainly oligo ( < 5 joints)

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14
Q

Which pathogens are a/w reactive arthritis?

A

Salmonella
Shigella
Yarsinea
Compylobacter
Clamydia
jiardia

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15
Q

How many of pt with Reactive arthritis are positive for HLA-B27

A

75%

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16
Q

Which disease is a/w
אפיזודות של חום כאבי בטן ותפליט פלאורלי פריחה דמוית אריסיפלס ברגל התחתונה
, מונוארתריטיס ודלקת באשכים

A

FMF

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17
Q

Which mutation and regio is a/w FMF?

A

M694V

טורקים, עירקים, ארמנים, ערביפ ואיטלקים

AR

18
Q

What are the 2 most common menefistation of FMF?

A

Fever- most common
Abdominal pain 90%

also:
arthritis
orchditis
erysipelas
serositis- mainly pleuralitis

19
Q

Tx for FMF?

1st and 2nd line + benefit of 1st line tx

A

1st line- Colcihicine- reduce frequency , duration and severity of attecks + prevent amyloidosis development

2nd lnine- anti- IL1

20
Q

Most common cause of death and complication of FMF?

A

Amyloidosis

Dgx by- kidney / rectal biopsy

21
Q

What is the clinical presentation of PFAPA

Which ages and duration of episodes?

A

like its name:
PF- Periodic Fever
A- aphtous somatitis
P- pahyngitis
A- adenitis (cervial LAP)

age 2-5
duration of episodes- 4-6 days
episodes in a year- 8-12

22
Q

Tx for PFAPA episode?

A

one dose Prednisone 1-2 mg/kg in the start of an episodes.

most clinical symptoms pass within 24hrs

Tonsillectomy can heal in some caseses

23
Q

What is the prognosis of PFAPA?

A

Spontenous remission in age 4-8 without consequences

24
Q

Which is the first most common vasculitis in kids and which is the second?

A

1st- HSP
2nd- Kawasaki

25
Q

what are the criteria for Dgx of kawasaki disease?

A

at least 4/5
rememebr- CRASH and BURN
C- conjuctuvitis bi-lateral
R- Rash (maculopappular of scalded)
A- adenitis (cervical unilaterl LN, at least one is > 1.5 cm)
S- somatitis- Struabrry toungh with red and crackled lips
H- Hands & feet- swollow red hands and feets

BURN- Fever > 5 days

26
Q

What is a-typical kawasaki?

A

Fever > 5 days + 2-3 kawasaki criteria

27
Q

How we Diagnose A-typical kawasaki?

A

labs reasults after 7 days showing the following:
**eleveation of ESR + CRP + 3/6 of the following: **
1. WBC > 15K
2. anemia
3. PLT > 450 (thrombocytosis)
4. Hypoalbuminemia (albumin < 3)
5. eleveted ALT
6. urine > 10 WBC/HPF (Sterile Pyurea)

28
Q

Tx for Kwasaki disease?

A

IVIG high dose
Aspirin- high dose until fever resolve for 48h &raquo_space; then low dose apsirin for 6 wks

if no respond:
another course of IVIG , steorids and consider infliximab
severly ill pt- Cyclosporine or cyclophosphamide

29
Q

After IVIG there is a contraindication for which vaccination and for how ling?

A

11 month
no MMR or Varicella

no live vaccine

30
Q

In which time frame IVIG treatment for kawasaki is recommended?

A

up to 10 days since onset of disease

in severe / non response disease- 2nd dose IVIG, Sterodis and or infliximab

31
Q

Which type of vasculitis is HSP?

what is the age prevelance?

A

IgA vasculitis - most common type in childrens

small veseel vasculitis

32
Q

What we expect the PLT count to be in HSP?

A

normal or eleveted

33
Q

How to Dgx HSP?

A

palpable purpura + 1 of the following:
1. abdominal pain
2. Arthritis / arthralgia
3. Biopsy with IgA
4. Kidney involvment- protenuria > 3 gr, hematuria or RBC casts

34
Q

Tx for HSP and complications?

A

Supportive
Complications- steorids (severe pain + renal invovlment)
arthritis - NSAIDS, but use with caution

35
Q

Prognosis of HSP:
* reslove?
* reccurence?
* CKD?
* ESRD?

A
  • reslove- 4 wks 80%
  • reccurence- 4-6 months 60%
  • CKD- 2%
  • ESRD- out of 2% , 5% will end with ESKD
36
Q

What is the HSP tetrad?

A
  1. joint pain - w/o effusion
  2. Palbable purpura
  3. abdominal pain- mainly colicky
  4. Renal involment - microscopic hematuria (in severe cases protenuria)
37
Q

Which disease is a/w symmetrical proximal muscle weakness, with diffrent types of rash and gottorn paples on DIP + PIP, Haliothropic rash

A

Juvenile Dermatomyositis

like adult but in childrens more GI involvment and calcifications

38
Q

how many of pt with Juvenile dermatomyositis will present with calcinosis?

A

40%

complication

39
Q

Helitrope rash + Gottorns nodules are two pathognemonic features of?

A

dermatomyositis

Helitrope- פריחה סגלגלה מעל העיניים
Gottorn nodules- פריחה ורודה אדמדמה במפרקים בעיקר של הידיים

40
Q

what are the criteria to Dgx of Juvenile dermatomyositis?

tx?

A

Typical rash (Helitrhope or gottorn papules) + 3 /4:
1. proximal symmeric muscle weakness
2. Eleveted liver enzyme- CPK, ALT, AST, LDH, aldolase
3. typical EMG
4. muscle biopsy

Tpoical Steroids + calcinurin inhibitors

41
Q

Which Abs and complement are in correlation of SLE severity?

A

C3, C$
CH50
anti- dsDNA

42
Q

Tx of choice for SLE?

A

Steroids pulse + hydroxycholoquine

במקרה של פגיעה כלייתית או מוחית- תוספת ציקולופוספמיד /מיקופנולט / מתוטרקסט /אזתיופורין

מבחן גמר בילדים (23 decks)

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