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=Pulmonary alveolar proteinosis=
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==Overview==
==Overview==
'''Pulmonary alveolar proteinosis''' -(PAP) is a rare [[lung]] [[disease]] in which abnormal accumulation of [[surfactant]] occurs within the [[alveoli]], interfering with [[gas exchange]].  PAP can occur in a primary form or secondarily in the settings of malignancy (especially in myeloid [[leukemia]]), pulmonary infection, or environmental exposure to dusts or chemicals.  Rare familial forms have also been recognized, suggesting a [[genetics|genetic]] component in some cases. <ref>Rosen SH, Castleman B, and Liebow AA. Pulmonary alveolar proteinosis. New England Journal of Medicine 1958; 258: 1123-1142.</ref> <ref>Seymour JF and Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. American Journal of Respiratory and Critical Care Medicine 2002; 166: 215-235.</ref> <ref>Shah PL, Hansell D, Lawson PR, et al. Pulmonary alveolar proteinosis; clinical aspects and current concepts on pathogenesis. Thorax 2000; 55: 67-77.</ref> <ref>Stanley E, Lieschke GJ, Grail D, et al. Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology. Proc. Natl. Acad. Sci. USA 1994; 91: 5592-5596.</ref>


==Historical Perspective==
==Historical Perspective==
PAP was first described in 1958 by the physicians Samuel Rosen, Benjamin Castleman, and Averill Liebow.  In their case series published in the [[New England Journal of Medicine]] on June 7th of that year, they described 27 patients with pathologic evidence of [[periodic acid Schiff]] positive material filling the alveoli.  This [[lipid]] rich material was subsequently recognized to be [[surfactant]]. 
 
==Classification==


==Pathophysiology==
==Pathophysiology==
Although the cause of PAP remains obscure, a major breakthrough in the understanding of the [[etiology]] of the disease came by the chance observation that mice bred for experimental study to lack a hematologic [[growth factor]] known as [[Granulocyte-colony stimulating factor|granulocyte-macrophage colony stimulating factor (GM-CSF)]] developed a pulmonary [[syndrome]] of abnormal surfactant accumulation resembling human PAP.  The implications of this finding are still being explored, but significant progress was reported in February, 2007.  Researchers in that report discussed the presence of anti-[[GM-CSF]] [[autoantibodies]] in patients with PAP, and duplicated that [[syndrome]] with the infusion of these [[autoantibodies]] into mice. <ref>{{cite journal |author=Uchida K, Beck D, Yamamoto T, Berclaz P, Abe S, Staudt M, Carey B, Filippi M, Wert S, Denson L, Puchalski J, Hauck D, Trapnell B |title=GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis |journal=N Engl J Med |volume=356 |issue=6 |pages=567-79 |year=2007 |pmid=17287477}}</ref>


==Causes==
==Causes==
Pulmonary alveolar proteinosis is an idiopathic disease, but studies have linked causes to production of antibodies that neutralize GM-CSF, [[granulocyte-macrophage colony-stimulating factor]]. Occasionally, development of pulmonary alveolar proteinosis is related to exposure of toxic substances, such as inorganic dusts, infection with [[Pneumocystis jirovecii]], certain cancers, and immunosuppressants. It rarely occurs in newborns.


There are several types of clinical forms of [[Pulmonary alveolar proteinosis]]; primary, secondary, and congenital. Primary PAP involves high levels of antibodies that neutralize GM-CSF, [[granulocyte-macrophage colony-stimulating factor]]. Secondary PAP occurs from clinical conditions that reduce the numbers and functions of alveolar macrophages. Congenital PAP is due to genetic mutations in the genes encoding surfactant proteins of the receptor for [[granulocyte-macrophage colony-stimulating factor]].<ref name="pmid14695413">{{cite journal| author=Trapnell BC, Whitsett JA, Nakata K| title=Pulmonary alveolar proteinosis. | journal=N Engl J Med | year= 2003 | volume= 349 | issue= 26 | pages= 2527-39 | pmid=14695413 | doi=10.1056/NEJMra023226 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14695413  }} </ref>
==Differentiating {{PAGENAME}} from Other Diseases==


==Causes==
==Epidemiology and Demographics==
===Common Causes===
*[[Pneumocystis jirovecii]]
*[[Respiratory failure]]
*Uncontrolled infection
*Respiratory Pathogens
*Opportunistic Pathogens: [[Nocardia]]
*[[Sirolimus]]
*[[Prednisone]]
*Inorganic dusts
*Inhalation of [[silica]] dust
*Exposure to [[insecticides]]
*aluminum dust
*[[titanium dioxide]]
*[[Haematological]] malignancies
*[[HIV]] infection.


===Causes by Organ System===
==Risk Factors==
{|style="width:80%; height:100px" border="1"
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" |No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Chemical / poisoning'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Dermatologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Drug Side Effect'''
|bgcolor="Beige"|[[Sirolimus]], [[Prednisone]]
|-
|-bgcolor="LightSteelBlue"
| '''Ear Nose Throat'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Endocrine'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Environmental'''
|bgcolor="Beige"| Inorganic dusts, Inhalation of [[silica]] dust, Exposure to [[insecticides]], aluminum dust, [[titanium dioxide]]
|-
|-bgcolor="LightSteelBlue"
| '''Gastroenterologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Genetic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Hematologic'''
|bgcolor="Beige"| [[Haematological]] malignancies
|-
|-bgcolor="LightSteelBlue"
| '''Iatrogenic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Infectious Disease'''
|bgcolor="Beige"| Uncontrolled infection, Respiratory Pathogens, [[Nocardia]], [[HIV]] infection
|-
|-bgcolor="LightSteelBlue"
| '''Musculoskeletal / Ortho'''
|bgcolor="Beige"| [No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Neurologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Nutritional / Metabolic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Obstetric/Gynecologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Oncologic'''
|bgcolor="Beige"| [[Haematological]] malignancies
|-
|-bgcolor="LightSteelBlue"
| '''Opthalmologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Overdose / Toxicity'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Psychiatric'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Pulmonary'''
|bgcolor="Beige"|[[Pneumocystis jirovecii]], [[Respiratory failure]]
|-
|-bgcolor="LightSteelBlue"
| '''Renal / Electrolyte'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Rheum / Immune / Allergy'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Sexual'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Trauma'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Urologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Dental'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Miscellaneous'''
|bgcolor="Beige"| No underlying causes
|-
|}


===Causes in Alphabetical Order===
==Screening==


{{col-begin|width=80%}}
==Natural History, Complications, and Prognosis==
{{col-break|width=33%}}
===Natural History===


*Aluminum dust
===Complications===
*[[Haematological]] malignancies
*[[HIV]] infection


*Inorganic Dusts
===Prognosis===
*[[Insecticides]]
*Opportunistic Pathogens: [[Nocardia]]
*[[Pneumocystis jirovecii]]
*[[Prednisone]]


*[[Respiratory failure]]
==Diagnosis==
*Respiratory Pathogens
===Diagnostic Criteria===
*[[Silica]] dust
*[[Sirolimus]]
*[[Titanium dioxide]]
*Uncontrolled infection


{{col-end}}
===History and Symptoms===


==Natural History==
===Physical Examination===
The clinical course of PAP is unpredictable. Spontaneous remission is recognized; some patients have stable symptoms.
==Complications==
Death may occur due to progression of PAP or due to the underlying disease associated with PAP. Individuals with PAP are more vulnerable to [[pneumonia|infection of the lung]] by [[bacterium|bacteria]] or [[fungi]].
==Prognosis==


==History and Symptoms==
===Laboratory Findings===
The [[symptoms]] of PAP include:
*[[Dyspnea]]<ref name="pmid14695413">{{cite journal| author=Trapnell BC, Whitsett JA, Nakata K| title=Pulmonary alveolar proteinosis. | journal=N Engl J Med | year= 2003 | volume= 349 | issue= 26 | pages= 2527-39 | pmid=14695413 | doi=10.1056/NEJMra023226 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14695413  }} </ref>
*[[Cough]]
*[[Sputum]]
*[[Fatigue]]
*[[Fever]]
*[[Anorexia]]
*[[Hemoptysis]]
*[[Crackles]]
*[[Cyanosis]]
*[[Clubbing]]
 
==Chest X Ray==
Classic radiographic finding is bilateral, symmetric [[alveolar]] [[consolidation]] or ground-glass opacity, particularly in a [[perihilar]] or [[hilar]] distribution resembling [[pulmonary edema]].
 
==CT==
* CT typically shows diffuse ground-glass attenuation with superimposed crazy-paving pattern (intra- and interlobular septal thickening, often in polygonal shapes representing the secondary pulmonary lobule).
'''Patient#1'''
<gallery>
Image:
Pulmonary-alveolar-proteinosis-001.jpg
Image:
Pulmonary-alveolar-proteinosis-002.jpg</gallery>
'''Patient #1: Proven PAP but not quite the classic crazy-paving pattern'''
<gallery>
Image:
Pulmonary alveolar proteinosis 201.jpg
Image:
Pulmonary alveolar proteinosis 202.jpg
Image:
Pulmonary alveolar proteinosis 203.jpg
Image:
Pulmonary alveolar proteinosis 204.jpg
</gallery>
 
==Other Diagnostic Studies==
[[Diagnosis]] is generally made by surgical or endoscopic [[biopsy]] of the lung, revealing the distinctive pathologic finding.
=== Histopathological Findings: Pulmonary alveolar proteinosis===
 
{{#ev:youtube|L9fVRzcPkEQ}}
 
==Medical Therapy==
The use of [[GM-CSF]] injections has also been attempted, with variable success.
 
==Surgery==
The standard treatment for PAP is whole-lung [[lavage]], in which sterile fluid is instilled into the lung and then removed, along with the abnormal [[surfactant]] material.  This is generally effective at ameliorating symptoms, often for prolonged periods. Lung [[transplantation]] can be performed in refractory cases.


===Imaging Findings===


===Other Diagnostic Studies===


==Treatment==
===Medical Therapy===


===Surgery===


===Prevention===


==References==
==References==
{{reflist|2}}
{{reflist|2}}


{{WS}}
{{WH}}


=Encephalitis Table=
=Encephalitis Table=
Line 255: Line 86:
| style="padding: 5px 5px; background: #F5F5F5;" | [[Ataxia]], [[lethargy]]
| style="padding: 5px 5px; background: #F5F5F5;" | [[Ataxia]], [[lethargy]]
| style="padding: 5px 5px; background: #F5F5F5;" |[[Multiple sclerosis]]: clinically, [[nystagmus]], [[internuclear ophthalmoplegia]], [[Lhermitte's sign]]; on imaging, well-demarcated ovoid lesions with possible T1 hypointensities (“black holes”)
| style="padding: 5px 5px; background: #F5F5F5;" |[[Multiple sclerosis]]: clinically, [[nystagmus]], [[internuclear ophthalmoplegia]], [[Lhermitte's sign]]; on imaging, well-demarcated ovoid lesions with possible T1 hypointensities (“black holes”)
[[Acute disseminated encephalomyelitis]]: ; on imaging, diffuse or multi-lesion enhancement, with indistinct lesion borders
[[Acute disseminated encephalomyelitis]]: clinically, [[somnolence]], [[myoclonic]] movements, and [[hemiparesis]]; on imaging, diffuse or multi-lesion enhancement, with indistinct lesion borders
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Substance abuse]]'''
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Substance abuse]]'''
Line 278: Line 109:
|-
|-
|}
|}
==Treatment==
Shown below is an algorithm summarizing the treatment of <nowiki>[[disease name]]</nowiki> according the the [...] guidelines.
{{familytree/start |summary=PE diagnosis Algorithm.}}
{{familytree | | | | | | | | A01 |A01= Do I know how this works?}}
{{familytree | | | | |,|-|-|-|^|-|-|-|-|.| | | }}
{{familytree | | | B01 | | | | | | | | B02 | | |B01=Yes, I'm an Honors Student |B02=No shot, this is wicked hard. }}
{{familytree | | | |!| | | | | | | | | |!| }}
{{familytree | | | C01 | | | | | | | | |!| |C01= Are you smart enough to split it off?}}
{{familytree | |,|-|^|.| | | | | | | | |!| }}
{{familytree | D01 | | D02 | | | | | | D03 |D01= Yes, I'm an Honors Student |D02= No, I guess I'm a pleb. |D03= Well wait, maybe some practice will help}}
{{familytree | |!| | | |!| | | | |,|-|^|.| }}
{{familytree | E01 | | |!| | | E02 | | E03 |E01= Good job. Way to live up to the hype. |E02= Yes! Practice Always Helps! |E03= Have you not seen this?}}
{{familytree | |!| | | |!| | | | |!| | | |!| }}
{{familytree | |!| | | |!| | F01 |'| | F02 |F01= You can learn anything in an hour! |F02= Go home and go to sleep. }}
{{familytree | |!| | | |`|-|-|-|!|-|.| | |!| }}
{{familytree | |`|-|-| G01 |-|-|'| |`|-| G02 | |G01= HONORS STUDENTS ARE NUMBER #1! |G02=RIP My GPA. }}
{{familytree/end}}




==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Latest revision as of 20:20, 8 August 2016

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Sandbox AG from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

References

Template:WS Template:WH

Encephalitis Table

Reference list includes:[1][2]

Disease Similarities Differentials
Meningitis Classic triad of fever, nuchal rigidity, and altered mental status Photophobia, phonophobia, rash associated with meningococcemia, concomitant sinusitis or otitis, swelling of the fontanelle in infants (0-6 months)
Brain abscess Fever, headache, hemiparesis Varies depending on the location of the abscess; clinically, visual disturbance including papilledema, decreased sensation; on imaging, a lesion demonstrates both ring enhancement and central restricted diffusion
Demyelinating diseases Ataxia, lethargy Multiple sclerosis: clinically, nystagmus, internuclear ophthalmoplegia, Lhermitte's sign; on imaging, well-demarcated ovoid lesions with possible T1 hypointensities (“black holes”)

Acute disseminated encephalomyelitis: clinically, somnolence, myoclonic movements, and hemiparesis; on imaging, diffuse or multi-lesion enhancement, with indistinct lesion borders

Substance abuse Tremor, headache, altered mental status Varies depending on type of substance: prior history, drug-seeking behavior, attention-seeking behavior, paranoia, sudden panic, anxiety, hallucinations
Electrolyte disturbance Fatigue, headache, nausea Varies depending on deficient ions; clinically, edema, constipation, hallucinations; on EKG, abnormalities in T wave, P wave, QRS complex; possible presentations include arrhythmia, dehydration, renal failure
Stroke Ataxia, aphasia, dizziness Varies depending on classification of stroke; presents with positional vertigo, high blood pressure, extremity weakness
Intracranial hemorrhage Headache, coma, dizziness Lobar hemorrhage, numbness, tingling, hypertension, hemorrhagic diathesis
Trauma Headache, altered mental status Amnesia, loss of consciousness, dizziness, concussion, contusion

Treatment

Shown below is an algorithm summarizing the treatment of [[disease name]] according the the [...] guidelines.

 
 
 
 
 
 
 
Do I know how this works?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes, I'm an Honors Student
 
 
 
 
 
 
 
No shot, this is wicked hard.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Are you smart enough to split it off?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes, I'm an Honors Student
 
No, I guess I'm a pleb.
 
 
 
 
 
Well wait, maybe some practice will help
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Good job. Way to live up to the hype.
 
 
 
 
 
 
Yes! Practice Always Helps!
 
Have you not seen this?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
You can learn anything in an hour!
 
 
 
Go home and go to sleep.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
HONORS STUDENTS ARE NUMBER #1!
 
 
 
 
 
 
 
 
RIP My GPA.
 
 
 
 
 
 
 
 
 


References

  1. ↑ Eckstein C, Saidha S, Levy M (2012). "A differential diagnosis of central nervous system demyelination: beyond multiple sclerosis". J Neurol. 259 (5): 801–16. doi:10.1007/s00415-011-6240-5. PMID 21932127.
  2. ↑ De Kruijk JR, Twijnstra A, Leffers P (2001). "Diagnostic criteria and differential diagnosis of mild traumatic brain injury". Brain Inj. 15 (2): 99–106. doi:10.1080/026990501458335. PMID 11260760.
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