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{{SK}} Lymphangioleiomyomatosis; LAM; pulmonary lymphangioleiomyomatosis; pulmonary lymphangiomyomatosis
{{SK}} Lymphangioleiomyomatosis; LAM; pulmonary lymphangioleiomyomatosis; pulmonary lymphangiomyomatosis


==[[Lymphangiomyomatosis overview|Overview]]==
__NOTOC__
 
{{SI}}
==[[Lymphangiomyomatosis historical perspective|Historical Perspective]]==
{{CMG}} {{AE}} Your Name
 
{{SK}} Synonym 1; Synonym 2; Synonym 3
==[[Lymphangiomyomatosis classification|Classification]]==
==Overview==
 
==Historical Perspective==
==[[Lymphangiomyomatosis pathophysiology|Pathophysiology]]==
*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
 
*In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
==[[Lymphangiomyomatosis causes|Causes]]==
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
 
==Classification==
==[[Lymphangiomyomatosis differential diagnosis|Differentiating Lymphangiomyomatosis from other Diseases]]==
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
 
:*[group1]
==[[Lymphangiomyomatosis epidemiology and demographics|Epidemiology and Demographics]]==
:*[group2]
 
:*[group3]
==[[Lymphangiomyomatosis risk factors|Risk Factors]]==
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
 
==Pathophysiology==
==[[Lymphangiomyomatosis screening|Screening]]==  
*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
 
*The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
==[[Lymphangiomyomatosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
 
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
==Diagnosis==
==Causes==
[[Lymphangiomyomatosis history and symptoms|History and Symptoms]] | [[Lymphangiomyomatosis physical examination|Physical Examination]] | [[Lymphangiomyomatosis laboratory findings|Laboratory Findings]] | [[Lymphangiomyomatosis electrocardiogram|EKG]] | [[Lymphangiomyomatosis chest x ray|Chest X ray]] | [[Lymphangiomyomatosis CT|CT]] | [[Lymphangiomyomatosis MRI|MRI]] | [[Lymphangiomyomatosis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Lymphangiomyomatosis other imaging findings|Other Imaging Findings]] | [[Lymphangiomyomatosis other diagnostic studies|Other Diagnostic Studies]]
* [Disease name] may be caused by either [cause1], [cause2], or [cause3].
 
* [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
==Treatment==
* There are no established causes for [disease name].
 
==Differentiating [disease name] from other Diseases==
[[Lymphangiomyomatosis medical therapy|Medical Therapy]] | [[Lymphangiomyomatosis surgery|Surgery]] | [[Lymphangiomyomatosis primary prevention|Primary Prevention]] | [[Lymphangiomyomatosis secondary prevention|Secondary Prevention]] | [[Lymphangiomyomatosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Lymphangiomyomatosis future or investigational therapies|Future or Investigational Therapies]]
*[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
 
:*[Differential dx1]
==Case Studies==
:*[Differential dx2]
[[Lymphangiomyomatosis case study one|Case #1]]
:*[Differential dx3]
 
==Epidemiology and Demographics==
[[Category: Disease]]
* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
[[Category: Hematology]]
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
 
===Age===
{{WH}}
*Patients of all age groups may develop [disease name].
{{WS}}
*[Disease name] is more commonly observed among patients aged [age range] years old.
*[Disease name] is more commonly observed among [elderly patients/young patients/children].
===Gender===
*[Disease name] affects men and women equally.
*[Gender 1] are more commonly affected with [disease name] than [gender 2].
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
===Race===
*There is no racial predilection for [disease name].
*[Disease name] usually affects individuals of the [race 1] race.
*[Race 2] individuals are less likely to develop [disease name].
==Risk Factors==
*Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
== Natural History, Complications and Prognosis==
*The majority of patients with [disease name] remain asymptomatic for [duration/years].
*Early clinical features include [manifestation 1], [manifestation 2],and [manifestation 3].
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]
=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Symptoms of [disease name] may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]
=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]
=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].
*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].
*[Imaging study 1] is the imaging modality of choice for [disease name]. *On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.
*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].
=== Prevention ===
*There are no primary preventive measures available for [disease name].
*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
*Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1], [test 2], and [test 3].
==References==
{{Reflist|2}}
[[Category:Oncology]]

Revision as of 17:20, 13 May 2016

Lymphangiomyomatosis
CT scan of a lung with LAM.
OMIM 606690
DiseasesDB ddb30755
MeSH D018192

For patient information, click here.

Template:Lymphangiomyomatosis Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Varun Kumar, M.B.B.S. [2]

Synonyms and keywords: Lymphangioleiomyomatosis; LAM; pulmonary lymphangioleiomyomatosis; pulmonary lymphangiomyomatosis


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3] Associate Editor(s)-in-Chief: Your Name Synonyms and keywords: Synonym 1; Synonym 2; Synonym 3

Overview

Historical Perspective

  • [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Causes

  • [Disease name] may be caused by either [cause1], [cause2], or [cause3].
  • [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
  • There are no established causes for [disease name].

Differentiating [disease name] from other Diseases

  • [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Patients of all age groups may develop [disease name].
  • [Disease name] is more commonly observed among patients aged [age range] years old.
  • [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

  • [Disease name] affects men and women equally.
  • [Gender 1] are more commonly affected with [disease name] than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for [disease name].
  • [Disease name] usually affects individuals of the [race 1] race.
  • [Race 2] individuals are less likely to develop [disease name].

Risk Factors

  • Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

  • The majority of patients with [disease name] remain asymptomatic for [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2],and [manifestation 3].
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

  • The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • [Disease name] is usually asymptomatic.
  • Symptoms of [disease name] may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with [disease name] usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]
  • [finding 5]
  • [finding 6]

Laboratory Findings

  • There are no specific laboratory findings associated with [disease name].
  • A [positive/negative] [test name] is diagnostic of [disease name].
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
  • Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

  • There are no [imaging study] findings associated with [disease name].
  • [Imaging study 1] is the imaging modality of choice for [disease name]. *On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
  • [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

  • [Disease name] may also be diagnosed using [diagnostic study name].
  • Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1], [test 2], and [test 3].

References