Mucormycosis overview: Difference between revisions

	Mucormycosis Microchapters
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Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Mucormycosis from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Criteria
History and Symptoms
Physical Examination
Laboratory Findings
X Ray
CT
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies

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Revision as of 16:13, 8 June 2017

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Historical Perspective

Mucormycosis in humans was was first reported in 1885 by a German pathologoist named Paltauf. Since its discovery, it has been known to be a lethal infection that mainly affects immune compromised and debilitated individuals. Recenttly, there has been an increase in its incidence across the world possibly due to increased incidence of diabetes, which is a major risk factor for development of the disease. Diagnosing mucormycosis has been a challenge since its discovery and early disgnosis is one of the keys to successful treatment. Much needs to be done regarding the early diagnosis and optimal treatment for this lethal infection.

Classification

Mucormycosis may involve various organ systems including brain, lungs, skin, GIT, bones, liver, spleen and can be classified based on the organ system involvement. Disseminated infection is associated with high mortality.

Pathophysiology

Mucormycosis is a fatal fungal infection occuring usually in immunocompromised and diabetic patients. Impairment of host defense mechanisms leads to development of the fungus within the human body. Iron is important for growth of the mucorales fungus. Thrombosis with eventual necrosis is the end point in mucormycosis infection. Glucose regulated protein 78 receptor plays a vital part in helping the organism attach to endothelial cells and for subsequent vascular invasion and dissemination. On microscopic examination the hyphae of mucorales is found to have few septations, non-pigmented and branches at right angle.

Causes

Rhizopus and mucor species are by far the most common causes of mucormycosis but there may be other fungi that lead to development of the disease.

Differentiating mucormycosis from other diseases

Mucormycosis can be difficult to diagnose and a high degree of clinical suspicion is required. Patient history is an important part of the diagnosis and aids in ruling out other differentials. Other features which help differentiate conditions with similar presentations are radiological and histopathological appearence.

Epidemiology and Demographics

Mucormycosis has limited data which outlines the epidemiology and demographics of the disease. Once the disease develops, it has a high mortality rate. Early diagnosis and management are the keys to improved survival.

Risk Factors

In humans mucormycosis is most prevalent in immunocompromised patients (HIV/AIDS, the elderly, SCID, etc) and patients in acidosis (diabetes, burns), particularly after barrier injury to the skin or mucus membranes, malignancies such as lymphomas and leukemias, renal failure, organ transplant, long term corticosteroid and immunosuppressive therapy, cirrhosis, burns and energy malnutrition. Some 50-75% of patients diagnosed with mucormycosis are estimated to have underlying poorly controlled diabetes mellitus and ketoacidosis.

Natural History, Complications and Prognosis

In most cases, the prognosis of zygomycosis is poor and has varied mortality rates depending on its form and severity. In the rhinocerebral form, the mortality rate is between 30% and 70%, whereas disseminated mucormycosis presents with the highest mortality rate in an otherwise healthy patient with a mortality rate of up to 100%. Patients with AIDS have a mortality rate of almost 100%. Possible complications of mucormycosis include the partial loss of neurological function, blindness and clotting of brain or lung vessels.

Diagnosis

An established criteria exists which can help in diagnosing mucormycosis. The criteria is provided by the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) Consensus Group. It is based on microscopic findings, clinical criteria and characteristics of the fungus.

History and Symptoms

Signs and symptoms of mucormycosis differ according to the organ system involvement. Severe infection of the facial sinuses, which may extend into the brain, is the most common presentation leading to proptosis, redness of skin above sinuses, mental status changes, dark scabbing in nasal cavities, fever and headache. Pulmonary mucormycosis may lead to development of cough, hemoptysis with or without chest pain and fever. Gastrointestinal mucormycosis presents as abdominal pain, hematemesis, diarrhea or constipation.

Physical Examination

Patients with mucormycosis usually appear lethargic, weak and debilitated owing to its development in immune compromised patients. Physical examination of patients with mucormycosis is usually remarkable for skin necrosis with a black eschar, fever, chills, myalgias, sore throat, non-productive cough, abdominal pain.

Laboratory Findings

When a clinician suspects invasive mucormycosis infection, an early diagnostic procedure performed within 16 days from the onset of symptom and early initiation of antifungal therapy will lead to successful management of this highly fatal disease.[1] Biopsy with H&E staining of the specimen and PCR may confirm the diagnosis in suspected cases.

Chest X-ray

CT scan Findings

MRI Findings

Treatment

Medical Therapy

If mucormycosis is suspected, prompt amphotericin B therapy should be administered due to the rapid spread and mortality rate of the disease. Amphotericin B (which works by damaging the cell walls of the fungi) is usually administered for a further 4-6 weeks after initial therapy begins to ensure eradication of the infection. Posaconazole has been shown to be effective against mucormycosis, perhaps more so than amphotericin B, but has not yet replaced it as the standard of care. After administration the patient must then be admitted to surgery for removal of the "fungus ball".

Surgery

Surgical therapy can be very drastic for mucormycosis, and in some cases of rhinocerebral disease removal of infected brain tissue may be required. In some cases surgery may be disfiguring because it may involve removal of the palate, nasal cavity, or eye structures. Surgery may be extended to more than one operation. It has been hypothesised that hyperbaric oxygen may be beneficial as an adjunctive therapy because higher oxygen pressure increases the ability of neutrophils to kill the organism.

Prevention

Mucormycosis may be prevented by avoiding the conditions leading to its development. If we reduce the risk factors, the chance of getting the disease is decreased significantly. Posaconazole has been found to be an effective agent for secondary prevention of mucormycosis.

@@ Line 1: / Line 1: @@
-Introduction to the Overview Page[edit | edit source]
+__NOTOC__
-The Overview page is a summary of all the other microchapters. The overview page for a microchaptered page will show the main subtitles of the page, with a brief overview of the content of each microchapter within each subtitle. This page is best created last, and aims to summarize the important aspects of the disease. To see an example of an Overview page on Pericarditis within a microchaptered page, click here. The aim is to convey the most important information for a "quick glance" at the disease. Below is the general template to follow when creating an Overview for a microchaptered page. Whether all the subheadings are needed and how extensive they are, will depend on the complexity of the disease page you are creating.
+{{Mucormycosis}}
+{{CMG}}; {{AE}}{{HK}}
-Overview[edit | edit source]
+==Overview==
-This section is the general overview statement for the disease. It should include the name of the main page in the first sentence.
-This section will be an overview statement of all the overview statements below it.
+==Historical Perspective==
-It should not contain any synonyms or keywords (as these should be at the top of the page listed next to Synonyms and Keywords: ).
+Mucormycosis in humans was was first reported in 1885 by a German pathologoist named Paltauf. Since its discovery, it has been known to be a lethal infection that mainly affects immune compromised and debilitated individuals. Recenttly, there has been an increase in its incidence across the world possibly due to increased incidence of diabetes, which is a major risk factor for development of the disease. Diagnosing mucormycosis has been a challenge since its discovery and early disgnosis is one of the keys to successful treatment. Much needs to be done regarding the early diagnosis and optimal treatment for this lethal infection.
-It should be aimed to be written at a medical student or intern level of understanding. To view an example of an overview section on an overview page, click here.
+==Classification==
-Historical Perspective[edit | edit source]
+Mucormycosis may involve various organ systems including brain, lungs, skin, GIT, bones, liver, spleen and can be classified based on the organ system involvement. Disseminated infection is associated with high mortality.
-Historical perspective of a disease discusses the initial discovery of the disease, the major outbreaks/events associated with the disease, and the initial diagnostic and therapeutic discoveries related to the disease.
+==Pathophysiology==
-This section should contain the name of the disease you are describing in the first sentence.
+Mucormycosis is a fatal fungal infection occuring usually in immunocompromised and diabetic patients. Impairment of host defense mechanisms leads to development of the fungus within the human body. Iron is important for growth of the mucorales fungus. Thrombosis with eventual necrosis is the end point in mucormycosis infection. Glucose regulated protein 78 receptor plays a vital part in helping the organism attach to endothelial cells and for subsequent vascular invasion and dissemination. On microscopic examination the hyphae of mucorales is found to have few septations, non-pigmented and branches at right angle.
-The overview of the historical perspective of a disease should be a short description of the landmark discoveries associated with the disease. It is ideally written after the main historical perspective microchapter is written, to summarize the key points of the microchapter. It can be the same as the overview that is seen on the historical perspective microchapter.
+==Causes==
-To view a template and examples of the Historical Perspective overview statement, click here.
+Rhizopus and mucor species are by far the most common causes of mucormycosis but there may be other fungi that lead to development of the disease.
-Classification[edit | edit source]
+==Differentiating mucormycosis from other diseases==
-Classification of a disease varies based on the type of disease. For example, certain cancers may be classified based on stage and grade, whereas a drug allergy may be classified based on the type of drug reaction.
+Mucormycosis can be difficult to diagnose and a high degree of clinical suspicion is required. Patient history is an important part of the diagnosis and aids in ruling out other differentials. Other features which help differentiate conditions with similar presentations are radiological and histopathological appearence.
-This section should contain the name of the disease you are describing in the first sentence.
+==Epidemiology and Demographics==
-The overview of the classification of a disease should be a short description of the way in which the disease is classified. It is ideally written after the main classification microchapter is written, to summarize the key points of the microchapter. It can be the same as the overview statement that is seen on the classification microchapter.
+Mucormycosis has limited data which outlines the epidemiology and demographics of the disease. Once the disease develops, it has a high mortality rate. Early diagnosis and management are the keys to improved survival.
-To view a template and examples of the Classification overview statement, click here.
+==Risk Factors==
-Pathophysiology[edit | edit source]
+In humans mucormycosis is most prevalent in immunocompromised patients (HIV/AIDS, the elderly, SCID, etc) and patients in acidosis (diabetes, burns), particularly after barrier injury to the skin or mucus membranes, malignancies such as lymphomas and leukemias, renal failure, organ transplant, long term corticosteroid and immunosuppressive therapy, cirrhosis, burns and energy malnutrition. Some 50-75% of patients diagnosed with mucormycosis are estimated to have underlying poorly controlled diabetes mellitus and ketoacidosis.
-Pathophysiology is the study of the biological and physical manifestations of a disease as they correlate with the underlying abnormalities and physiological disturbances.
+==Natural History, Complications and Prognosis==
-This section should contain the name of the disease you are describing in the first sentence.
+In most cases, the prognosis of zygomycosis is poor and has varied mortality rates depending on its form and severity. In the rhinocerebral form, the mortality rate is between 30% and 70%, whereas disseminated mucormycosis presents with the highest mortality rate in an otherwise healthy patient with a mortality rate of up to 100%. Patients with AIDS have a mortality rate of almost 100%. Possible complications of mucormycosis include the partial loss of neurological function, blindness and clotting of brain or lung vessels.
-The overview of the pathophysiology of a disease should be a short description of the basic disease process. It is ideally written after the main pathophysiology microchapter is written, to summarize the key points of the microchapter. It can be the same as the overview statement that is seen on the pathophysiology microchapter.
+==Diagnosis==
-To view a template and examples of the Pathophysiology overview statement, click here.
+An established criteria exists which can help in diagnosing mucormycosis. The criteria is provided by the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) Consensus Group. It is based on microscopic findings, clinical criteria and characteristics of the fungus.
-Causes[edit | edit source]
+===History and Symptoms===
-This section summarizes the main causes of the disease.
+Signs and symptoms of mucormycosis differ according to the organ system involvement. Severe infection of the facial sinuses, which may extend into the brain, is the most common presentation leading to proptosis, redness of skin above sinuses, mental status changes, dark scabbing in nasal cavities, fever and headache. Pulmonary mucormycosis may lead to development of cough, hemoptysis with or without chest pain and fever. Gastrointestinal mucormycosis presents as abdominal pain, hematemesis, diarrhea or constipation.
-The overview for causes of a disease should ideally be written after the main causes microchapter is written, to summarize the key points of the microchapter. It can be the same as the overview statement found on the main causes microchapter for the disease.
+===Physical Examination===
-To view a template and examples of the Causes (Non-microbiology) overview statement, click here.
+Patients with mucormycosis usually appear lethargic, weak and debilitated owing to its development in immune compromised patients. Physical examination of patients with mucormycosis is usually remarkable for skin necrosis with a black eschar, fever, chills, myalgias, sore throat, non-productive cough, abdominal pain.
-To view a template and examples of the Causes (Microbiology) overview statement, click here.
+===Laboratory Findings===
-Differentiating (Disease name) from other Conditions[edit | edit source]
+When a clinician suspects invasive mucormycosis infection, an early diagnostic procedure performed within 16 days from the onset of symptom and early initiation of antifungal therapy will lead to successful management of this highly fatal disease.[1] Biopsy with H&E staining of the specimen and PCR may confirm the diagnosis in suspected cases.
-In this section, give a brief description of the main diseases that need to be differentiated from the disease you are describing.
+===Chest X-ray===
-The overview of the differentiation of a disease should ideally be written after the main microchapter is written. It can be the same as the overview statement found on the main "differentiating disease from other conditions" microchapter for the disease.
-To view a template and examples of the Differential Diagnosis overview statement, click here.
+===CT scan Findings===
-Epidemiology and Demographics[edit | edit source]
-Epidemiology is the scientific study of the causes, distribution, and control of disease populations. Demographics are the objective characteristics of a population age, marital status, family size, racial origin, present or prior disease, religion, income, and education and how they relate to a specific disease.
+===MRI Findings===
-This section should contain the name of the disease you are describing in the first sentence.
-The overview of the epidemiology and demographics of a disease should ideally be written after the main epidemiology and demographics microchapter is written. It can be the same as the overview statement found on the main epidemiology and demographics microchapter for the disease.
-To view a template and examples of the Epidemiology and Demographics overview statement, click here.
+==Treatment==
-Risk Factors[edit | edit source]
-Risk factors are variables associated with an increased risk of disease or infection.This section should outline the risk factors that have the highest correlation with the disease.
+===Medical Therapy===
-The overview of the risk factors of a disease should ideally be written after the main risk factors microchapter is written, to summarize the key points of the microchapter. It can be the same as the overview statement found on the main risk factors microchapter for the disease.
+If mucormycosis is suspected, prompt amphotericin B therapy should be administered due to the rapid spread and mortality rate of the disease. Amphotericin B (which works by damaging the cell walls of the fungi) is usually administered for a further 4-6 weeks after initial therapy begins to ensure eradication of the infection. Posaconazole has been shown to be effective against mucormycosis, perhaps more so than amphotericin B, but has not yet replaced it as the standard of care. After administration the patient must then be admitted to surgery for removal of the "fungus ball".
-To view a template and examples of the Risk Factors overview statement, click here.
+===Surgery===
-Natural History, Complications and Prognosis[edit | edit source]
+Surgical therapy can be very drastic for mucormycosis, and in some cases of rhinocerebral disease removal of infected brain tissue may be required. In some cases surgery may be disfiguring because it may involve removal of the palate, nasal cavity, or eye structures. Surgery may be extended to more than one operation. It has been hypothesised that hyperbaric oxygen may be beneficial as an adjunctive therapy because higher oxygen pressure increases the ability of neutrophils to kill the organism.
-The natural history of a disease describes how the disease would progress without treatment. The complications describe the negative consequences of the disease and treatment, and the prognosis describes the outcomes of the disease.
+===Prevention===
-This section should contain the name of the disease you are describing in the first sentence.
+Mucormycosis may be prevented by avoiding the conditions leading to its development. If we reduce the risk factors, the chance of getting the disease is decreased significantly. Posaconazole has been found to be an effective agent for secondary prevention of mucormycosis.
-The overview of the natural history, complications and prognosis is ideally written after the main microchapter is written, to summarize the key points of the microchapter. It can be the same as the overview statement that is seen on the natural history, complications and prognosis microchapter page.
-To view a template and examples of the Natural History, Complications and Prognosis overview statement, click here.
-Diagnosis[edit | edit source]
-The diagnosis of a disease details the most important signs, symptoms, tests, and other studies that lead to the diagnosis of a disease.
-This section should contain the name of the disease you are describing in the first sentence.
-The overview of the diagnosis of a disease should ideally be written after the main diagnosis microchapters are written, to summarize the key points of the microchapters.
-History and Symptoms[edit | edit source]
-Describe the main aspects of the patient history that should be focused on, and the symptoms that lead to, or exclude the diagnosis of the disease you are describing. You should use the name of the disease in the first sentence. For an example of this subsection, click here.
-This section can be the same as the overview section on the history and symptoms page.
-To view a template and examples of the History and Symptoms overview statement, click here.
-Physical Examination[edit | edit source]
-Describe the main physical examination findings that can lead to or exclude the diagnosis of the disease you are describing. You should include the name of the disease in the first sentence. For an example, click here
-This section can be the same as the overview section physical examination page.
-To view a template and examples of the Physical Examination overview statement, click here.
-Laboratory Findings[edit | edit source]
-List the main laboratory studies that can lead to or exclude the diagnosis of the disease you are describing. You should include the name of the disease in the first sentence.
-This section should be the same as the overview statement on the laboratory findings page.
-To view a template and examples of the Laboratory Findings overview statement, click here.
-Electrocardiogram[edit | edit source]
-If EKG findings are pertinent to the diagnosis of the disease you are describing, you can provide the findings here.
-This section can be the same as the overview statement found on the Electrocardiogram page.
-To view a template and examples of the Electocardiogram overview statement, click here.
-Chest X Ray[edit | edit source]
-If chest x ray findings are pertinent to the disease page you are making, you can briefly describe them here.
-This can be the same as the overview statement on the chest x ray page.
-To view a template and examples of the Chest X Ray overview statement, click here.
-CT Scan[edit | edit source]
-If CT findings are pertinent to the page you are making, you can briefly describe them here.
-This section can be the same as the overview section on the CT page.
-To view a template and examples of the CT Scan overview statement, click here.
-Echocardiography or Ultrasound[edit | edit source]
-If echocardiography or ultrasound findings are pertinent to the page you are making, you can describe them here.
-This section can be the same as the overview section on the echocardiography and ultrasound page.
-To view a template and examples of the Echocardiography or Ultrasound overview statement, click here.
-Other Imaging Findings[edit | edit source]
-List the most important diagnostic studies, such as imaging and other studies, that can lead to or exclude the diagnosis of the disease you are describing. You should name any "gold standard" studies here, and include the name of the disease in the first sentence.
-To view a template and examples of the Other Imaging Findings overview statement, click here.
-Treatment[edit | edit source]
-Treatment describes the various, most commonly used methods in treating the disease you are describing.
-This section should contain the name of the disease you are describing in the first sentence.
-The overview of the treatments for a disease should ideally be written after the main treatment microchapter is written, to summarize the key points of the microchapter. It can be the same as the overview statement found on the main risk factors microchapter for the disease.
-Medical Therapy[edit | edit source]
-Medical therapy describes all non-surgical therapies that are provided for the patient.
-This section should contain the name of the disease you are describing in the first sentence followed by the indication to treat the patient (if applicable) and the name of the therapy.
-To view a template and examples of the Medical Therapy overview statement, click here.
-Surgery[edit | edit source]
-Surgery describes all surgeries and therapeutic procedures that are provided for the patient.
-This section should contain the name of the disease you are describing in the first sentence followed by the indication to surgically manage the patient (if application) and the name of the surgery.
-To view a template and examples of the Surgery overview statement, click here.
-Prevention[edit | edit source]
-Prevention describes all strategies that prevent from the occurrence of the disease. Prevention may be either primary (prevent occurrence of the disease), secondary (diagnose and treat existent disease in early stages), tertiary (reduce the negative impact of extant disease), and quaternary (methods to avoid results of unnecessary interventions). At least primary and secondary prevention are usually discussed in each chapter.
-This section should contain the name of the disease you are describing in the first sentence. The availability or lack of vaccine availability of a vaccine against the disease should be clearly written. Other strategies for the prevention of the disease should be outlined and classified as either primary, secondary, tertiary, or quaternary.
-To view a template and examples of the Prevention overview statement, click here.