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__NOTOC__
[[Syncope]] is classified into three types:
{{Familial mediterranean fever}}
* [[Cardiac]]
{{CMG}}; {{AE}}
* [[Neurogenic]]
== Overview ==
* [[Vasovagal syncope|vasovagal]]
 
== Diagnostic Study of Choice ==
 
=== Study of choice ===
Familial Mediterranean fever is primarily diagnosed based on the clinical presentation.
 
==== The comparison of various diagnostic studies for familial Mediterranean fever====
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! style="background: #4479BA; color: #FFFFFF; text-align: center;" | Test
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sensitivity
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Specificity
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Tel Hashomer criteria
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Livneh criteria
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|}
<small> [Name of test with higher sensitivity and specificity] is the preferred investigation based on the sensitivity and specificity</small>
==== The Tel Hashomer for diagnosing familial Mediterranean fever====
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! style="background: #4479BA; color: #FFFFFF; text-align: center;" | Type
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Criteria
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Criteria
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Major
| style="background: #DCDCDC; padding: 5px; text-align: center;" |
| style="background: #DCDCDC; padding: 5px; text-align: center;" |
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Minor
| style="background: #DCDCDC; padding: 5px; text-align: center;" |
| style="background: #DCDCDC; padding: 5px; text-align: center;" |
|}
===Tel Hashomer criteria===
[Disease name] may be diagnosed at any time if one or more of the following criteria are met:
* Criteria 1
* Criteria 2
* Criteria 3
The diagnosis of Familial Mediterranean fever is made when at least 2 of the following diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
 
==References==
{{Reflist|2}}
 
 
__NOTOC__
 
===Management options of Retinoblastoma===
:*
{| class="wikitable"
|+
! colspan="2" |Treatment options for Intraocular tumor<ref name="urlRetinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#_13 |title=Retinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute |format= |work= |accessdate=}}</ref>
|-
|Unilateral retinoblastoma
|
* Enucleation followed by chemotherapy
*Conservative ocular salvage approaches:
**Chemoreduction with either systemic or ophthalmic artery infusion chemotherapy with or without intravitreal chemotherapy
**Local treatments (cryotherapy, thermotherapy, and plaque radiation therapy)
|-
|Bilateral retinoblastoma
|
* Enucleation for large intraocular tumors, followed by risk-adapted chemotherapy when the eye and vision cannot be saved
* Conservative ocular salvage approaches when the eye and vision can be saved:
**Chemoreduction with either systemic or ophthalmic artery infusion chemotherapy with or without intravitreal chemotherapy
**Local treatments (cryotherapy, thermotherapy, and plaque radiation therapy)
**EBRT
|-
|Cavitary retinoblastoma
|
* Systemic and/or intra-arterial chemotherapy
|-
|Progressive or recurrent intraocular retinoblastoma
|
* Enucleation
* Radiation therapy (EBRT or plaque radiation therapy)
* Local treatments (cryotherapy or thermotherapy)
* Salvage chemotherapy (systemic or intra-arterial)
* Intravitreal chemotherapy
|+
! colspan="2" |Treatment options for Extraocular tumor<ref name="urlRetinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#_13 |title=Retinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute |format= |work= |accessdate=}}</ref>
|-
|Orbital and locoregional retinoblastoma
|
* Chemotherapy
* Radiation therapy
|-
|CNS disease
|
* Systemic chemotherapy and CNS-directed therapy
* Systemic chemotherapy followed by myeloablative chemotherapy and stem cell rescue
|-
|Trilateral retinoblastoma
|
* Systemic chemotherapy followed by surgery and myeloablative chemotherapy with stem cell rescue
* Systemic chemotherapy followed by surgery and radiation therapy
|-
|Extracranial metastatic retinoblastoma
|
* Systemic chemotherapy followed by myeloablative chemotherapy with stem cell rescue and radiation therapy
|-
|Progressive or recurrent extraocular retinoblastoma
|
* Systemic chemotherapy and radiation therapy for orbital disease
* Systemic chemotherapy followed by myeloablative chemotherapy with stem cell rescue, and radiation therapy for extraorbital disease
|}
 
 
 
 
 
 
{| border="3"
|+ Intraocular classifications of retinoblastoma and their features
!  !! International Intraocular Retinoblastoma Classification (IIRC) !! Intraocular Classification of Retinoblastoma (ICRB)
|-
! Group A
(very low
risk)
|Small intraretinal tumors away from foveola and optic nerve<br>3mm or smaller in the greatest dimension, confined to the retina<br>Located further than 3 mm from the foveola and 1.5 mm from the optic disc
| Tumors ≤ 3 mm (in basal dimension or thickness)
|-
!Group B
(low risk)
|Tumors confined to the retina<br>Not in the group A<br>Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
|Tumors > 3 mm (in basal dimension or thickness) or<br>Macular location (≤ 3 mm to foveola)<br>Juxtapapillary location (≤ 1.5 mm to disc)<br>Additional subretinal fluid (≤3 mm from margin)
|-
! Group C
(moderate
risk)
|Local disease with minimal subretinal or vitreous seeding with following characteristics:<br>Discrete<br>Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina<br>Local fine vitreous seeding may be present close to the discrete tumor<br>Local subretinal seeding less than 3 mm (2 DD) from the tumor
|Tumor with:<br>Subretinal seeds ≤ 3 mm from tumor<br>Vitreous seeds ≤ 3 mm from tumor<br>Both subretinal and vitreous seeds ≤ 3 mm from retinoblastoma
|-
!Group D
(high risk)
|Diffuse tumor with significant vitreous or subretinal seeding<br>Maybe massive or diffuse<br>Subretinal fluid present or past without seeding, involving up to total retinal detachment<br>The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses<br>Diffuse subretinal seeding may include subretinal plaques or tumor nodules
|Tumor with:<br>Subretinal seeds > 3 mm from tumor<br>Vitreous seeds > 3 mm from tumor<br>Both subretinal and vitreous seeds > 3 mm from retinoblastoma
|-
!Group E
(very high
risk)
|Presence of any one or more of the following poor prognosis features<br>Tumor touching the lens<br>Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment<br>Diffuse infiltrating retinoblastoma<br>Neovascular glaucoma<br>Opaque media from hemorrhage<br>Tumor necrosis with aseptic orbital cellulitis<br>Phthisis bulbi
|Extensive tumor filling >50% globe or with<br>Neovascular glaucoma<br>Opaque media from hemorrhage in the anterior chamber, vitreous or subretinal space<br>Invasion of the post-laminar optic nerve<br>choroid (>2 mm), sclera, orbit, anterior chamber
|}
{| style="border: 0px; font-size: 90%; margin: 3px; width: 800px" align=center
|valign=top|
|+
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Groups}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Features}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
:Group A
| style="padding: 5px 5px; background: #F5F5F5;" |
*Small intraretinal tumors away from foveola and optic nerve
**3mm or smaller in the greatest dimension, confined to retina
**Located further than 3 mm from the foveola and 1.5 mm from the optic disc.
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
:Group B
| style="padding: 5px 5px; background: #F5F5F5;" |
*Tumors confined to the retina.
**Not in the group A
**Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
|-
| style="padding: 5px 5px; background: #DCDCDC;font-All remaining discrete tumors confined to the retina.weight: bold" |
:Group C
| style="padding: 5px 5px; background: #F5F5F5;" |
*Local disease with minimal subretinal or vitreous seeding with following caracteristics:
**Discrete
**Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina
**Local fine vitreous seeding may be present close to the discrete tumor
**Local subretinal seeding less than 3 mm (2 DD) from the tumor
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
:Group D
| style="padding: 5px 5px; background: #F5F5F5;" |
*Diffuse tumor with significant vitreous or subretinal seeding
**May be massive or diffuse
**Subretinal fluid present or past without seeding, involving up to total retinal detachment
**The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses
**Diffuse subretinal seeding may include subretinal plaques or tumor nodules
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
:Group E
| style="padding: 5px 5px; background: #F5F5F5;" |
*Presence of any one or more of the following poor prognosis features
**Tumor touching the lens
**Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment
**Diffuse infiltrating retinoblastoma
**Neovascular glaucoma
**Opaque media from hemorrhage
**Tumor necrosis with aseptic orbital cellulitis
**Phthisis bulbi
|}
 
 
{|
! colspan="2" style="background:#DCDCDC;" align="center" + |Diagnostic algorithm for Infantile onset glyogen storage disease type II
|-
 
 
 
*On [[microscopic]] [[histopathological]] analysis, [[carotid body]] [[tumor]] composed of:
**The chief or [[paraganglionic]] cells composing the predominant part of the [[tumor]] and contain [[eosinophilic]] granular materials and oval or round nuclei.<ref name="PatetsiosGable2002">{{cite journal|last1=Patetsios|first1=Peter|last2=Gable|first2=Dennis R.|last3=Garrett|first3=Wilson V.|last4=Lamont|first4=Jeffrey P.|last5=Kuhn|first5=Joseph A.|last6=Shutze|first6=William P.|last7=Kourlis|first7=Harry|last8=Grimsley|first8=Bradley|last9=Pearl|first9=Gregory J.|last10=Smith|first10=Bertram L.|last11=Talkington|first11=C.M.|last12=Thompson|first12=Jesse E.|title=Management of Carotid Body Paragangliomas and Review of a 30-year Experience|journal=Annals of Vascular Surgery|volume=16|issue=3|year=2002|pages=331–338|issn=08905096|doi=10.1007/s10016-001-0106-8}}</ref>
**The supporting or sustentacular cells responsible for the [[chemoreceptor]] activity of the [[carotid body]]
*The characteristic finding of this [[tumor]] is:
:*Chief cells Arranged in distinctive pattern called [[cell]] balls (zellballen)
:*Separated by fibrovascular stroma and surrounded by [[sustentacular]] cells
:*The [[cytoplasm]] is [[pale]] and diffuse with occasional presence of the [[eosinophilic]] [[granules]].<ref>{{cite book | last = Bibbo | first = Marluce | title = Comprehensive cytopathology | publisher = Saunders/Elsevier | location = Philadelphia, PA | year = 2008 | isbn = 978-1-4160-4208-2 }}</ref>
:*The nuclei are round to spindle shape.
*The [[tumor]] is highly [[vascular]].
*Although there is no well-accepted [[histologic]] criteria for the [[diagnosis]] of [[malignant]] [[tumors]], worrisome [[histologic]] features include:<ref name="WienekeSmith2009">{{cite journal|last1=Wieneke|first1=Jacqueline A.|last2=Smith|first2=Alice|title=Paraganglioma: Carotid Body Tumor|journal=Head and Neck Pathology|volume=3|issue=4|year=2009|pages=303–306|issn=1936-055X|doi=10.1007/s12105-009-0130-5}}</ref>
**[[Necrosis]]
**Extensive [[vascular]] or capsular [[invasion]]
**Increased [[mitotic]] activity
**Atypical [[mitotic]] figures
 
{| {{table}} cellpadding="4" cellspacing="0" style="border:#c9c9c9 1px solid; margin: 1em 1em 1em 0; border-collapse: collapse;"
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|''' Features on Gross Pathology'''}}
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Image'''}}
|-
|Characteristic findings of [[carotid body]] [[tumor]], include:<ref name="WienekeSmith2009">{{cite journal|last1=Wieneke|first1=Jacqueline A.|last2=Smith|first2=Alice|title=Paraganglioma: Carotid Body Tumor|journal=Head and Neck Pathology|volume=3|issue=4|year=2009|pages=303–306|issn=1936-055X|doi=10.1007/s12105-009-0130-5}}</ref>
**Well-circumscribed with psudocapsule
**The size of the [[tumor]] varies greatly and it may be as large as 10 cm
**The cutting surface is solid with a smooth, rubbery texture||
|[[File:Carotid body tumor.jpg|thumb|300px|Contributed by Paweł Kuźniar in wikimedia.commons]]
|-
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{Family tree/start}}
{{Family tree | | | | | | | | | | | | A01 | | | |A01= Patient with carotid body tumor}}
{{Family tree | | | | | | | | | | | | |!| | | | | }}
{{Family tree | | | | | | | | | | | | B01 | | | |B01= History, Physical examination, and evaluation of cnotralateral side}}
{{Family tree | | | | | | | | | |,|-|-|^|-|-|.| | }}
{{Family tree | | | | | | | | | C01 | | | | C02 |C01= Patients with age < 50 years<br>Patients with multiple paraganglioma<br>Patients with a positive family history| C02= The rest of the patients}}
{{Family tree | | | | | | | | | |!| | | | }}
{{Family tree | | | | | | | | | D01 | | | | | |D01= SDHD genetic testing}}
{{Family tree | | | | | |,|-|-|-|^|-|-|.| | }}
{{Family tree | | | | | E01 | | | | | E02 |E01= Presence of SDHD mutation |E02= Absence of SDHD mutation}}
{{Family tree | | | | | |!| | | | | | |!| | | | | }}
{{Family tree | | | | | |!| | | | | | F01 | | | |F01= SDHC and SDHB genetic testing}}
{{Family tree | | | | | |!| | | |,|-|-|^|-|-|.| | }}
{{Family tree | | | | | |!| | | G01 | | | | G02 |G01= Presence of SDHC/B mutation |G02= Absence of SDHC/B mutation}}
{{Family tree | | | | | |!| | | |!| | }}
{{familytree  | | | | | | H02 |-|'| | | |H02=All the relatives should be evaluated for the presence of paragnaglioma}}
{{Family tree | | | | | | |!| | | | }}
{{Family tree | | | | | | I01 | | | |I01= whole-body 18F-dihydroxyphenylalanine (F-DOPA) positron emission tomography to assess the presence of other paragangliomas}}
{{Family tree | | | |,|-|-|^|-|-|.| |}}
{{Family tree | | | J01 | | | | J02 |J01= Presence of other paraganglioma |J02= Absence of other paraganglioma}}
{{Family tree | | | |!| | | | | |!| | | }}
{{Family tree | | | K01 | | | | K02 |K01= 24-hour urine catecholamines and MRI for biochemical screening|K02=surveillance screening every 5 years}}
{{Family tree/end}}

Latest revision as of 00:32, 12 August 2021

Syncope is classified into three types:

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