Fibrous dysplasia

Jump to navigation Jump to search

WikiDoc Resources for Fibrous dysplasia

Articles

Most recent articles on Fibrous dysplasia

Most cited articles on Fibrous dysplasia

Review articles on Fibrous dysplasia

Articles on Fibrous dysplasia in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Fibrous dysplasia

Images of Fibrous dysplasia

Photos of Fibrous dysplasia

Podcasts & MP3s on Fibrous dysplasia

Videos on Fibrous dysplasia

Evidence Based Medicine

Cochrane Collaboration on Fibrous dysplasia

Bandolier on Fibrous dysplasia

TRIP on Fibrous dysplasia

Clinical Trials

Ongoing Trials on Fibrous dysplasia at Clinical Trials.gov

Trial results on Fibrous dysplasia

Clinical Trials on Fibrous dysplasia at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Fibrous dysplasia

NICE Guidance on Fibrous dysplasia

NHS PRODIGY Guidance

FDA on Fibrous dysplasia

CDC on Fibrous dysplasia

Books

Books on Fibrous dysplasia

News

Fibrous dysplasia in the news

Be alerted to news on Fibrous dysplasia

News trends on Fibrous dysplasia

Commentary

Blogs on Fibrous dysplasia

Definitions

Definitions of Fibrous dysplasia

Patient Resources / Community

Patient resources on Fibrous dysplasia

Discussion groups on Fibrous dysplasia

Patient Handouts on Fibrous dysplasia

Directions to Hospitals Treating Fibrous dysplasia

Risk calculators and risk factors for Fibrous dysplasia

Healthcare Provider Resources

Symptoms of Fibrous dysplasia

Causes & Risk Factors for Fibrous dysplasia

Diagnostic studies for Fibrous dysplasia

Treatment of Fibrous dysplasia

Continuing Medical Education (CME)

CME Programs on Fibrous dysplasia

International

Fibrous dysplasia en Espanol

Fibrous dysplasia en Francais

Business

Fibrous dysplasia in the Marketplace

Patents on Fibrous dysplasia

Experimental / Informatics

List of terms related to Fibrous dysplasia

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Fibrous dysplasia is a disorder of bones which may occur with or without endocrinological and skin disorders. It may cause bony pain, deformity, fracture and / or entrapment of nerves, It is basically the acquired mis-sense mutation of gene coding for the α-subunit of the stimulatory G-protein, Gs, in the guanine nucleotide binding, alpha stimulating (GNAS) complex locus in chromosome 20q13. It leads to immature or poor differentiation of body tissue at the time of ossification and replacement of bone by fibrous tissue. Diagnosis depends on radiology and biopsic specimen.

Historical Perspective

  • Fibrous dyspepsia was first observed in bone radiography by Von Recklinghausen in 1891.
  • It was then entitled as separate entity by american pathologist Dr. Louis Lichtenstein in 1938 as fibrous dyspepsia polyostotic.
  • In 1942, Dr Lichtenstein and Dr. Jaffe together labeled syndrome named McCune-Albright syndrome (fibrous dysplasia-café au lait spots-endocrine dysfunction) or Mazabraud syndrome (fibrous dysplasia-myxomas). 

Classification

  • Fibrous dyspepsia may be classified according to number of bony sites involved into two groups:
  • Monoostiotic
  • Poluostiotic
  • Other variants of FD include McCune-Albright syndrome, and Mazabraud syndrome.

Pathophysiology

  • 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].

Clinical Features

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 action 1].
  • 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 followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

References

Template:WS Template:WH