Lysozyme amyloid related amyloidosis

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

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Homa Najafi, M.D.[2]

Synonyms and keywords:Lysozyme amyloidosis, ALys, Hereditory amyloidosis, autosomal dominant amyloidosis, Hereditary lysozyme amyloidosis, Familial amyloid nephropathy due to lysozyme variant, Familial renal amyloidosis due to lysozyme variant, Hereditary amyloid nephropathy due to lysozyme variant, Hereditary renal amyloidosis due to lysozyme variant

Overview

Four different mutations in lysozyme which are associated with amyloidosis have been defined . Each is associated with adult-onset renal failure due to glomerular deposition of amyloid. The entire 14 500-Dalton lysozyme protein has been found in amyloid deposits and, in some studies, its function as a bacteriolytic enzyme has been preserved after isolation from amyloid-laden tissues.22 Clinical presentation as renal failure may occur in the third or fourth decades, and kidney transplantation has been found to be an effective mode of therapy for a number of patients.

Historical Perspective

• In 1639, Nicolaus Fontanus autopsied a young man who had ascites, jaundice, liver abscess, and splenomegaly and his report has been the first description of amyloidosis.
• In 1854, Rudolph Virchow introduced the term "amyloid" as a macroscopic abnormality in some tissues.
• In 1867, Weber reported the first case of amyloidosis associated with multiple myeloma.
• In 1922, Bennhold introduced Congo Red staining of amyloid that remains the gold standard for diagnosis.
• In 1959, Cohen and Calkins used ultrathin sections of amyloidotic tissues and assessed by electron microscopy, explained the presence of non-branching fibrils with indeterminate length and variable width.
• In 1993, Pepys introduced that lysozyme form as an an amyloid fibril protein is a variant of hereditary systemic amyloidosis.

Classification

Apolipoprotein AI amyloidosis is one of the subtypes of familial amyloidosis. Familiar amyloidosis may be classified according to the type of mutant protein into 6 subtypes:

• Transthyretin (TTR)
• Apolipoprotein AI
• Apolipoprotein AII
• Fibrinogen Aa
• Lysozyme
• Gelsolin
• Cystatin C

Familial amyloidosis subtypes

Transthyretin (TTR)

Apolipoprotein AI

Gelsolin

Lysozyme

Cystatin C

Fibrinogen Aa-chain

Apolipoprotein AII

Pathophysiology

The exact pathogenesis of [disease name] is not fully understood.

OR

It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].

OR

[Pathogen name] is usually transmitted via the [transmission route] route to the human host.

OR

Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

OR

[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].

OR

The progression to [disease name] usually involves the [molecular pathway].

OR

The pathophysiology of [disease/malignancy] depends on the histological subtype.

Causes

Disease name] may be caused by [cause1], [cause2], or [cause3].

OR

Common causes of [disease] include [cause1], [cause2], and [cause3].

OR

The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].

OR

The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.

Differentiating ((Page 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], and [differential dx3].

OR

[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].

Epidemiology and Demographics

The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.

OR

In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.

OR

In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.

Patients of all age groups may develop [disease name].

OR

The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.

OR

[Disease name] commonly affects individuals younger than/older than [number of years] years of age.

OR

[Chronic disease name] is usually first diagnosed among [age group].

OR

[Acute disease name] commonly affects [age group].

There is no racial predilection to [disease name].

OR

[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].

[Disease name] affects men and women equally.

OR

[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.

The majority of [disease name] cases are reported in [geographical region].

OR

[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].

Risk Factors

There are no established risk factors for [disease name].

OR

The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].

OR

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

OR

Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.

Screening

There is insufficient evidence to recommend routine screening for [disease/malignancy].

OR

According to the [guideline name], screening for [disease name] is not recommended.

OR

According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].

Natural History, Complications, and Prognosis

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Diagnosis

Diagnostic Study of Choice

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], and [criterion 4].

OR

The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].

OR

The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].

OR

There are no established criteria for the diagnosis of [disease name].

History and Symptoms

The majority of patients with [disease name] are asymptomatic.

OR

The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].

Physical Examination

Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].

OR

Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

The presence of [finding(s)] on physical examination is diagnostic of [disease name].

OR

The presence of [finding(s)] on physical examination is highly suggestive of [disease name].

Laboratory Findings

An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].

OR

Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

OR

[Test] is usually normal among patients with [disease name].

OR

Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].

OR

There are no diagnostic laboratory findings associated with [disease name].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

References

Template:WikiDoc Sources