Lymphangiomyomatosis

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

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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

WikiDoc Resources for Lymphangiomyomatosis
Articles
Most recent articles on Lymphangiomyomatosis Most cited articles on Lymphangiomyomatosis Review articles on Lymphangiomyomatosis Articles on Lymphangiomyomatosis in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Lymphangiomyomatosis Images of Lymphangiomyomatosis Photos of Lymphangiomyomatosis Podcasts & MP3s on Lymphangiomyomatosis Videos on Lymphangiomyomatosis
Evidence Based Medicine
Cochrane Collaboration on Lymphangiomyomatosis Bandolier on Lymphangiomyomatosis TRIP on Lymphangiomyomatosis
Clinical Trials
Ongoing Trials on Lymphangiomyomatosis at Clinical Trials.gov Trial results on Lymphangiomyomatosis Clinical Trials on Lymphangiomyomatosis at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Lymphangiomyomatosis NICE Guidance on Lymphangiomyomatosis NHS PRODIGY Guidance FDA on Lymphangiomyomatosis CDC on Lymphangiomyomatosis
Books
Books on Lymphangiomyomatosis
News
Lymphangiomyomatosis in the news Be alerted to news on Lymphangiomyomatosis News trends on Lymphangiomyomatosis
Commentary
Blogs on Lymphangiomyomatosis
Definitions
Definitions of Lymphangiomyomatosis
Patient Resources / Community
Patient resources on Lymphangiomyomatosis Discussion groups on Lymphangiomyomatosis Patient Handouts on Lymphangiomyomatosis Directions to Hospitals Treating Lymphangiomyomatosis Risk calculators and risk factors for Lymphangiomyomatosis
Healthcare Provider Resources
Symptoms of Lymphangiomyomatosis Causes & Risk Factors for Lymphangiomyomatosis Diagnostic studies for Lymphangiomyomatosis Treatment of Lymphangiomyomatosis
Continuing Medical Education (CME)
CME Programs on Lymphangiomyomatosis
International
Lymphangiomyomatosis en Espanol Lymphangiomyomatosis en Francais
Business
Lymphangiomyomatosis in the Marketplace Patents on Lymphangiomyomatosis
Experimental / Informatics
List of terms related to Lymphangiomyomatosis

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

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].