Difference between revisions of "Mucormycosis"

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{{Infobox_Disease |
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__NOTOC__
  Name           = {{PAGENAME}} |
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<small>
  Image         = Mucormycosis.jpg |
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{{Infobox Disease |
  Caption       = Periorbital fungal infection known as mucormycosis or phycomycosis|
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Name = Mucormycosis |
  DiseasesDB    = 31759 |
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Image = Mucormycosis.png |
  ICD10          = {{ICD10|B|46|0|b|35}}-{{ICD10|B|46|5|b|35}} |
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Caption = Necrotic tissue in rhinocerebral mucormycosis. |
  ICD9          = {{ICD9|117.7}} |
 
  ICDO          = |
 
  OMIM          = |
 
  MedlinePlus    = |
 
  eMedicineSubj  = med |
 
  eMedicineTopic = 1513 |
 
  MeshID        = D009091 |
 
 
}}
 
}}
{{DiseaseDisorder infobox |
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</small>
  Name          = Phycomycosis |
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{{Mucormycosis}}
  Image          = |
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'''For patient information click [[{{PAGENAME}} (patient information)|here]].'''
  Caption        = |
 
  ICD10          = {{ICD10|B|46||b|35}} |
 
  ICD9          = {{ICD9|117.7}} |
 
  ICDO          = |
 
  OMIM          = |
 
  DiseasesDB    = 31329 |
 
  MedlinePlus    = |
 
  eMedicineSubj  = |
 
  eMedicineTopic = |
 
  MeshID        = D020096 |
 
}}
 
{{Infobox_Disease |
 
  Name          = Basidiobolomycosis |
 
  Image          = |
 
  Caption        = |
 
  DiseasesDB    = |
 
  ICD10          = {{ICD10|B|46||b|35}} |
 
  ICD9          = {{ICD9|117.7}} |
 
  ICDO          = |
 
  OMIM          = |
 
  MedlinePlus    = 000649 |
 
  eMedicineSubj  = med |
 
  eMedicineTopic = 2735 |
 
  eMedicine_mult = {{eMedicine2|med|1513}} |
 
  MeshName      = Mucormycosis |
 
  MeshNumber    = D009091 |
 
}}
 
{{SI}}
 
{{CMG}}
 
  
{{EH}}
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{{CMG}}; {{AE}}{{HK}}
  
'''Mucormycosis''' (also known as zygomycosis) is a rare but serious infection of fungi of the [[Mucorales]] order.<ref>{{cite web |url=http://www.emedicine.com/med/topic1513.htm |title=eMedicine - Mucormycosis : Article by Nancy F Crum-Cianflone, MD MPH |accessdate=2007-09-30 |format= |work=}}</ref> The most common fungi responsible for mucormycosis in humans are ''[[Mucor]]'' and ''Rhizopus''. 
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{{SK}} Mucor infection, rhino-cerebral mucormycosis, pulmonary mucorycosis, cutaneous mucormycosis, gastrointestinal mucormycosis
  
==Signs and symptoms==
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==[[Mucormycosis overview|Overview]]==
Zygomycosis frequently involves the [[Paranasal sinus|sinuses]], [[brain]], or [[lungs]] as the sites of infection. While oral or [[cerebral]] zygomycosis are the most common types of the disease, this infection can also manifest in the gastrointestinal tract, skin, and in other organ systems. In rare cases, the [[maxilla]] may be affected by zygomycosis. The rich vascularity of maxillofacial areas usually prevents fungal infections, although more prevalent fungi, such as those responsible for zygomycosis, can often overcome this difficulty.
 
  
There are several key signs which point towards zygomycosis. One such sign is fungal invasion into the [[vascular]] network which results in [[thrombosis]] and [[necrosis|death]] of surrounding tissue by loss of blood supply.<ref name=cmrasm/> If the disease involves the [[cerebrum|brain]] then symptoms may include a [[unilateral|one-sided]] [[headache]] behind the eyes, facial pain, [[fevers]], nasal stuffiness that progresses to black discharge, and acute [[sinusitis]] along with swelling of the eye. Affected skin may appear relatively normal during the earliest stages of infection. This skin quickly progresses to an erythemic (reddening, occasionally with [[edema]]) stage, before eventually turning black due to necrosis.<ref name=cmrasm>{{cite journal |author=Spellberg B, Edwards J, Ibrahim A |title=Novel perspectives on mucormycosis: pathophysiology, presentation, and management |journal=Clin. Microbiol. Rev. |volume=18 |issue=3 |pages=556–69 |year=2005 |pmid=16020690 |doi=10.1128/CMR.18.3.556-569.2005| url=http://cmr.asm.org/cgi/content/full/18/3/556}} {{PMC|1195964}}</ref> In other forms of zygomycosis, such as [[pulmonary]], [[cutaneous]], or disseminated zygomycosis, symptoms may also include [[dyspnea]] (difficulty breathing), and persistent [[cough]]; in cases of necrosis, symptoms include [[nausea]] and [[vomiting]], [[hemoptysis|coughing blood]], and [[abdominal pain]].
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==[[Mucormycosis historical perspective|Historical Perspective]]==
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==[[Mucormycosis classification|Classification]]==
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==[[Mucormycosis pathophysiology|Pathophysiology]]==
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==[[Mucormycosis causes|Causes]]==
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==[[Mucormycosis differential diagnosis|Differentiating Mucormycosis from other Diseases]]==
  
==Presentation==
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==[[Mucormycosis epidemiology and demographics|Epidemiology and Demographics]]==
  
It that frequently involves the [[sinuses]], [[brain]], or [[lungs]] and most commonly presents in [[immunocompromised]] patients. 
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==[[Mucormycosis risk factors|Risk Factors]]==
 
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==[[Mucormycosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
While orbitorhinocerebral mucormycosis is the most common type of the disease, this infection can also manifest in the [[gastrointestinal tract]], [[skin]], and in other organ systems. 
 
 
 
==Associated conditions==
 
Some 50-75% of patients diagnosed with mucormycosis are estimated to have underlying poorly controlled [[diabetes mellitus]] and [[ketoacidosis]].
 
  
 
==Diagnosis==
 
==Diagnosis==
  
As swabs of tissue or discharge are generally unreliable, the diagnosis of zygomycosis tends to be established by a [[biopsy]] specimen of the involved tissue.
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[[Mucormycosis diagnostic criteria|Diagnostic Criteria]] | [[Mucormycosis history and symptoms|History and Symptoms]] | [[Mucormycosis physical examination|Physical Examination]] | [[Mucormycosis laboratory findings|Laboratory Findings]] | [[Mucormycosis x ray|X Ray]] | [[Mucormycosis CT|CT]] | [[Mucormycosis MRI|MRI]] | [[Mucormycosis other imaging findings|Other Imaging Findings]] | [[Mucormycosis other diagnostic studies|Other Diagnostic Studies]]
 
 
Diagnosis for phycomycosis is through a biopsy or [[microbiological culture|culture]], although an [[ELISA]] test has been developed for ''Pythium insidiosum'' in animals.<ref name=Hensel>{{cite journal |author=Hensel P, Greene C, Medleau L, Latimer K, Mendoza L |title=Immunotherapy for treatment of multicentric cutaneous pythiosis in a dog |journal=J Am Vet Med Assoc |volume=223 |issue=2 |pages=215–8, 197 |year=2003 |pmid=12875449 |doi=10.2460/javma.2003.223.215}}</ref>  Computerised imaging techniques such as [[MRI]]s, [[CT scan]]s and [[X-ray]]s may be useful in the diagnosis of specific areas.<ref name=HealthAtoZ>{{cite web|title=Mucormycosis|author=Rebecca J. Frey, PhD|url=http://www.healthatoz.com/healthatoz/Atoz/clients/haz/general/custom/default.jsp|publisher=Health A to Z|accessdate=2008-05-19}}</ref>
 
 
 
Diagnosis is often difficult because basidiobolomycosis is a rare disease and therefore often not recognised.  The lesions often look like [[tumour]]s rather than infection, so often no sample is sent for microbiology, however, the histopathology is characteristic:  the "Splendore-Hoeppli phenomenon" describes the presence of fungal [[hyphae]] (which may exist only as faded streaks on the film) surrounded by eosinophilic material. Basidiobolomycosis is usually a superficial infection of skin, but may very rarely cause lesions of the bowel or liver, mimicking bowel cancer,<ref>{{cite journal | title=A fatal pseudo-tumour: Disseminated basidiobolomycosis | author=Van den berk GEL, Noorduyn LA, van Ketel RJ, ''et al.'' | journal=BMC Infect Dis | year=2006 | volume=6 | pages=140 | doi=10.1186/1471-2334-6-140 }}</ref> or [[Crohn's disease]].<ref name="clininfect">{{cite journal | journal=Clin Infect Dis | year=1999 | volume=28 | issue=6 | pages=1244&ndash;8 | title=Gastrointestinal zygomycotic infection caused by ''Basidiobolus ranarum:'' case report and review | author=Zavasky DM, Samowitz W, Loftus T, Segal H, Carroll K | doi=10.1086/514781 }}</ref> In patients with deep involvement, the [[eosinophil]] count may be raised, falsely suggesting a parasitic infection. Zygomycosis also has similar symptoms to other diseases including [[anthrax]], [[aspergillosis]] and [[cellulitis]].
 
 
 
[[Image:Mature_sporangium_of_a_Mucor_sp._fungus.jpg|thumb|left|200px|This photomicrograph reveals a mature sporangium of a Mucor sp. fungus, which can be responsible for zygomycosis]]
 
  
 
==Treatment==
 
==Treatment==
  
'''Surgical resection of the "fungus ball" and intravenous [[amphotericin B]] is the recommended therapy.'''
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[[Mucormycosis medical therapy|Medical Therapy]] | [[Mucormycosis surgery|Surgery]] | [[Mucormycosis primary prevention|Primary Prevention]] | [[Mucormycosis secondary prevention|Secondary Prevention]] | [[Mucormycosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Mucormycosis future or investigational therapies|Future or Investigational Therapies]]
 
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[[Category:Emergency mdicine]]
If zygomycosis 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 wall]]s 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 zygomycosis, 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". The disease must be monitored carefully for any signs of reemergence.
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[[Category:Disease]]
 
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[[Category:Primary care]]
Surgical therapy can be very drastic, 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#Three_layers|eye structure]]s. 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 [[Neutrophil granulocyte|neutrophils]] to kill the organism.
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[[Category:Up-To-Date]]
 
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[[Category:Infectious disease]]
Treatment for phycomycosis is very difficult and includes surgery when possible.  Postoperative recurrence is common.  Antifungal drugs show only limited effect on the disease, but [[itraconazole]] and [[terbinafine|terbinafine hydrochloride]] are often used for two to three months following surgery.  Humans with ''Basidiobolus'' infections have been treated with [[amphotericin B]] and [[potassium iodide]]. For pythiosis and lagenidiosis, a new drug targeting water moulds called [[caspofungin]] is available, but it is very expensive. [[Immunotherapy]] has been used successfully in humans and horses with pythiosis. Treatment for skin lesions is traditionally with potassium iodide,<ref>{{cite journal | journal=Ann Trop Paediatr | year=1997 | volume=17 | issue=2 | pages=161&ndash;4 | title=Invasive retroperitoneal infection due to ''Basidiobolus ranarum'' with response to potassium iodide&mdash;case report and review of the literature | author=Nazir Z, Hasan R, Pervaiz S, Alam M, Moazam F. | pmid=9230980 }}</ref> but itraconazole has also been used successfully.<ref>{{cite journal |author=Wasim Yusuf N, Assaf HM, Rotowa NA |title=Invasive gastrointestinal Basidiobolus ranarum infection in an immunocompetent child |journal=Pediatr. Infect. Dis. J. |volume=22 |issue=3 |pages=281–2 |year=2003 |pmid=12664879 |doi=10.1097/00006454-200303000-00017 |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0891-3668&volume=22&issue=3&spage=281}}</ref><ref>{{cite journal |author=Mathew R, Kumaravel S, Kuruvilla S, ''et al'' |title=Successful treatment of extensive basidiobolomycosis with oral itraconazole in a child |journal=Int. J. Dermatol. |volume=44 |issue=7 |pages=572–5 |year=2005 |pmid=15985026 |doi=10.1111/j.1365-4632.2004.02419.x |url=http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0011-9059&date=2005&volume=44&issue=7&spage=572}}</ref>
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[[Category:Gastroenterology]]
 
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[[Category:Otolaryngology]]
==Prognosis==
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[[Category:Nephrology]]
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 zygomycosis presents with the highest mortality rate in an otherwise healthy patient, with a mortality rate of up to 90%.<ref name=cmrasm/> Patients with AIDS have a mortality rate of almost 100%.<ref name=HealthAtoZ/> Possible complications of zygomycosis include the partial loss of neurological function, blindness and clotting of brain or lung vessels.
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[[Category:Dermatology]]
 
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[[Category:Pulmonology]]
==Epidemiology==
 
 
 
Zygomycosis is a very rare infection, and as such it is hard to note histories of patients and incidence of the infection. However, one American oncology center revealed that zygomycosis was found in 0.7% of [[autopsy|autopsies]] and roughly 20 patients per every 100,000 admissions to that center.<ref name=HealthAtoZ/> In the United States, zygomycosis was most commonly found in rhinocerebral form, almost always with [[hyperglycemia]] and [[metabolic acidosis]].<ref name="pmid16080086"/> In most cases the patient is immunocompromised, although rare cases have occurred in which the subject was not; these are usually due to a [[Physical trauma|traumatic]] [[inoculation]] of fungal [[spores]]. Internationally, zygomycosis was found in 1% of patients with [[acute]] leukemia in an Italian review.
 
 
 
Predisposing factors for zygomycosis include [[AIDS]], malignancies such as [[lymphoma]]s, [[renal failure]], [[organ transplant]], long term [[corticosteroid]] and immunosuppressive therapy, [[cirrhosis]] and energy [[malnutrition]]. Despite this, however, there have been cases of zygomycosis reported with no apparent predisposing factors present.<ref name="pmid16080086">{{cite journal |author=Roden MM, Zaoutis TE, Buchanan WL, ''et al'' |title=Epidemiology and outcome of zygomycosis: a review of 929 reported cases |journal=Clin. Infect. Dis. |volume=41 |issue=5 |pages=634–53 |year=2005 |month=September |pmid=16080086 |doi=10.1086/432579 |url=http://www.journals.uchicago.edu/doi/abs/10.1086/432579 |issn= |accessdate=2008-05-19}}</ref>
 
 
 
==Case Example: Carotid Artery Mucormycosis==
 
 
 
===Clinical Summary===
 
 
 
A 63-year-old white male was in his usual state of good health until eight weeks before his death when he developed sudden onset of [[shortness of breath]]. A [[thoracotomy]] was performed for plication of ruptured emphysematous blebs.
 
 
 
Following improvement and discharge from the hospital he developed [[weakness]], [[lethargy]], and a left lower lobe lung infiltrate. The patient's condition soon deteriorated further, with almost every organ system having failed. The patient developed [[DIC]] and peripheral embolic phenomena, including [[gangrene]] of his extremities and face.
 
 
 
A single antemortem blood culture grew [[Staphylococcus aureus]].
 
 
 
===Postmortem Findings===
 
 
 
Autopsy revealed severe [[emphysema]], severe widespread abscessiform and necrotizing [[pneumonia]], and [[bacterial endocarditis]] ([[Staphylococcus aureus]]) of the [[pulmonic valve]]. The right internal carotid artery was occluded by a thrombus and there were areas of necrosis (due to CVAs) in the brain.
 
 
 
[http://www.peir.net Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]
 
 
 
<div align="left">
 
<gallery heights="175" widths="175">
 
Image:Carotid Artery Mucormycosis 1.jpg|This is a low-power photomicrograph of a section of carotid artery containing a mural thrombus.
 
Image:Carotid Artery Mucormycosis 2.jpg|This is a higher-power photomicrograph of the wall of the carotid artery (1) and the thrombus (2).
 
</gallery>
 
</div>
 
 
 
 
 
 
 
<div align="left">
 
<gallery heights="175" widths="175">
 
Image:Carotid Artery Mucormycosis 3.jpg|This is an even higher-power photomicrograph of the wall of the carotid artery (1) and the thrombus (2). Within the wall of the artery and in the thrombus there are multiple variably shaped clear areas (3). At this magnification and with this stain, it is impossible to determine what these clear spaces represent.
 
Image:Carotid Artery Mucormycosis 4.jpg|This is a higher-power photomicrograph of just the wall of the carotid artery. Note the ribbon-like clear structure with roughly parallel walls (non-septate hyphae) and right-angle branching (arrow). This is the Mucor organism.
 
</gallery>
 
</div>
 
 
 
 
 
 
 
<div align="left">
 
<gallery heights="175" widths="175">
 
Image:Carotid Artery Mucormycosis 5.jpg|This is another high-power photomicrograph of the wall of the artery and the thrombus. Within the thrombus there are multiple variably-shaped clear areas that represent longitudinal sections and cross sections of the Mucor organisms (arrows).
 
Image:Carotid Artery Mucormycosis 6.jpg|This medium-power photomicrograph shows the thrombus stained to outline the Mucor organisms (arrows). Note again the ribbon-like morphology and the wide-angle branching.
 
</gallery>
 
</div>
 
 
 
 
 
 
 
<div align="left">
 
<gallery heights="175" widths="175">
 
Image:Carotid Artery Mucormycosis 7.jpg|This is an even higher-power photomicrograph of the thrombus stained to outline the Mucor organisms (arrows).
 
Image:Carotid Artery Mucormycosis 8.jpg|This is another high-power photomicrograph of the thrombus stained to outline the Mucor organisms (arrows).
 
</gallery>
 
</div>
 
 
 
==Mucormycosis meningoencephalitis==
 
 
 
<youtube v=un6CqeDPuH0/>
 
 
 
==References==
 
{{Reflist|2}}
 
 
 
==External links==
 
*[http://www.uoflhealthcare.org/f_pressrelease.asp?id=58][http://www.mold-help.org/content/view/544/] - Articles on Mark Tatum, a victim of the disease.
 
*[http://www.newscientist.com/channel/earth/tsunami/dn6938-tsunami-survivors-risk-deadly-fungal-infections.html Tsunami survivors risk deadly fungal infections]
 
 
 
[[Category:Diseases]]
 
[[category:Parasitic fungi]]
 
 
 
[[pl:Mukormykoza]]
 
[[pt:Mucormicose]]
 
 
 
{{Mycoses}}
 
{{SIB}}
 
 
 
{{WH}}
 
{{WS}}
 

Latest revision as of 18:48, 21 September 2017


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