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| {{ Paroxysmal nocturnal hemoglobinuria }} | | {{Paroxysmal nocturnal hemoglobinuria}} |
| {{CMG}} | | {{CMG}}; {{AE}} |
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| ==Overview== | | ==Overview== |
| '''Paroxysmal nocturnal hemoglobinuria''' is a rare, acquired, potentially life-threatening disease of the blood characterised by[[hemolytic anemia]], [[thrombosis]] and red [[urine]] due to breakdown of [[red blood cell]]s. [[PNH]] is the only hemolytic anemia caused by an ''acquired'' intrinsic defect in the [[cell membrane]]. | | '''Paroxysmal nocturnal hemoglobinuria''' is a rare, acquired, potentially life-threatening disease of the blood characterised by[[hemolytic anemia]], [[thrombosis]] and red [[urine]] due to breakdown of [[red blood cell]]s. [[PNH]] is the only hemolytic anemia caused by an ''acquired'' intrinsic defect in the [[cell membrane]]. |
| | ==Historical Perspective== |
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| ==Historical Perspective==
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| *[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
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| *In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
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| *In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
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| ==Classification== | | ==Classification== |
| *[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
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| :*[group1]
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| :*[group2]
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| :*[group3]
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| *Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
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| ==Pathophysiology== | | ==Pathophysiology== |
| *The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
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| *The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
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| *On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
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| *On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
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| ==Causes== | | ==Causes== |
| * [Disease name] may be caused by either [cause1], [cause2], or [cause3].
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| * [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
| | ==Differentiating Xyz from Other Diseases== |
| * There are no established causes for [disease name].
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| ==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:
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| :*[Differential dx1]
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| :*[Differential dx2]
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| :*[Differential dx3]
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| ==Epidemiology and Demographics== | | ==Epidemiology and Demographics== |
| * The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
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| * In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
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| ===Age===
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| *Patients of all age groups may develop [disease name].
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| *[Disease name] is more commonly observed among patients aged [age range] years old.
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| *[Disease name] is more commonly observed among [elderly patients/young patients/children].
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| ===Gender===
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| *[Disease name] affects men and women equally.
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| *[Gender 1] are more commonly affected with [disease name] than [gender 2].
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| * The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
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| ===Race===
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| *There is no racial predilection for [disease name].
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| *[Disease name] usually affects individuals of the [race 1] race.
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| *[Race 2] individuals are less likely to develop [disease name].
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| ==Risk Factors== | | ==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].
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| == Natural History, Complications and Prognosis==
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| *The majority of patients with [disease name] remain asymptomatic for [duration/years].
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| *Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
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| *If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
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| *Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
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| *Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
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| == Diagnosis ==
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| ===Diagnostic Criteria===
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| *The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
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| :*[criterion 1]
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| :*[criterion 2]
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| :*[criterion 3]
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| :*[criterion 4]
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| === Symptoms ===
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| *[Disease name] is usually asymptomatic.
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| *Symptoms of [disease name] may include the following:
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| :*[symptom 1]
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| :*[symptom 2]
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| :*[symptom 3]
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| :*[symptom 4]
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| :*[symptom 5]
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| :*[symptom 6]
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| === Physical Examination ===
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| *Patients with [disease name] usually appear [general appearance].
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| *Physical examination may be remarkable for:
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| :*[finding 1]
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| :*[finding 2]
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| :*[finding 3]
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| :*[finding 4]
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| :*[finding 5]
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| :*[finding 6]
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| === Laboratory Findings ===
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| *There are no specific laboratory findings associated with [disease name].
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| *A [positive/negative] [test name] is diagnostic of [disease name].
| | ==Screening== |
| *An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
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| *Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
| | ==Natural History, Complications, and Prognosis== |
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| ===Imaging Findings=== | | |
| *There are no [imaging study] findings associated with [disease name].
| | ==Diagnosis== |
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| | ===Diagnostic Study of Choice=== |
| *[Imaging study 1] is the imaging modality of choice for [disease name].
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| *On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
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| *[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
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| === Other Diagnostic Studies === | |
| *[Disease name] may also be diagnosed using [diagnostic study name].
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| *Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
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| == Treatment == | |
| === Medical Therapy ===
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| *There is no treatment for [disease name]; the mainstay of therapy is supportive care.
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| *The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
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| *[Medical therapy 1] acts by [mechanism of action 1].
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| *Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
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| === Surgery === | |
| *Surgery is the mainstay of therapy for [disease name].
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| *[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
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| *[Surgical procedure] can only be performed for patients with [disease stage] [disease name].
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| === Prevention === | |
| *There are no primary preventive measures available for [disease name].
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| *Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
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| *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].
| | ===History and Symptoms=== |
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| ==References== | | ===Physical Examination=== |
| {{Reflist|2}}
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| | ===Laboratory Findings=== |
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| | ===Electrocardiogram=== |
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| | ===X-ray=== |
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| | ===Echocardiography and Ultrasound=== |
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| | ===CT scan=== |
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| | ===MRI=== |
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| | ===Other Imaging Findings=== |
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| | ===Other Diagnostic Studies=== |
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| | ==Treatment== |
| | ===Medical Therapy=== |
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| | === Interventions === |
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| | ===Surgery=== |
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| | ===Primary Prevention=== |
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| | ===Secondary Prevention=== |
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| [[Category:Hematology]]
| | ==References== |
| [[Category:Rheumatology]]
| | {{reflist|2}} |
| [[Category:Mature chapter]]
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| {{WH}} | | {{WikiDoc Help Menu}} |
| {{WS}} | | {{WikiDoc Sources}} |
| | [[Category: (name of the system)]] |