Rhinocerebral mucormycosis due to absidia corymbifera

Summary We report two cases of rhinocerebral mucormycosis due toAbsidia corymbifera, an extremely uncommon causal agent. Both occurred in patients with haematological disorders and both had a fatal outcome, despite antemortem diagnosis and aggressive treatment. We discuss the factors which generally contribute to the poor prognosis in this group of patients and suggest another possible mechanism which may contribute to the high mortality.

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Rhinozerebrale Mucormykose durch Absidia corymbifera

Zusammenfassung Wir berichten über zwei Fälle von rhinozerebraler Mukormykose durchAbsidia corymbifera, einen extrem seltenen Erreger. Bei beiden Patienten lag eine hämatologische Grunderkrankung vor, beide Fälle verliefen tödlich, obwohl die Diagnose gestellt und eine aggressive Therapie eingeleitet worden war. Die bei dieser Patientengruppe für eine schlechte Prognose verantwortlichen allgemeinen Faktoren werden diskutiert, wir stellen außerdem einen möglichen Pathomechanismus vor, der die hohe Letalität der Infektion mit verursachen kann.

     

Rhinocerebral mucormycosis: a report of eleven cases

Rhinocerebral mucormycosis (RCM) is a rare, fulminant fungal infection that usually occurs in diabetic or immunocompromised patients. The mortality rate has been reduced recently with the advent of amphotericin B combined with aggressive surgery. Eleven RCM patients have been treated over the past five years at Srinagarind Hospital. Eight had underlying diabetes, five had renal failure and three of them had both. In eight patients, the diagnosis was established by KOH preparation before histological confirmation. Only two cases revealed positive cultures for Rhizopus spp and Cunninghamella spp. All patients underwent surgical treatments (extensive debridement, 8 cases; sphenoidectomy, 7 cases; ethmoidectomy 8 cases; maxillectomy 5 cases and orbital exenteration, 6 cases). Amphotericin B was administered to all patients as soon as the diagnosis of RCM was made. Only three patients survived. Early diagnosis and cooperation among ophthalmologist, otolaryngologist and physician are the most important factors for the survival of patients with mucormycosis.     

Rhinocerebral mucormycosis: premortem diagnosis and therapy.

The diagnosis of rhinocerebral mucormycosis is most often made at autopsy. We report a series of nine patients in whom the diagnosis was established premortem. Six of the patients had underlying diabetes mellitus and three had acute leukemia. Facial or ocular pain was the complaint found in all patients, and frequently was the initial symptom. The diagnosis was established by examination and culture of infected tissue obtained by biopsy. In seven patients, identification of hyphal elements in smears of biopsy material allowed the immediate institution of amphotericin B therapy. Four of the seven patients treated with amphotericin B survived. All surviving patients had underlying diabetes mellitus and had undergone surgical debridement. Early diagnosis leading to immediate institution of appropriate therapy is most important for survival of patients with mucormycosis.     

Rhinocerebral mucormycosis in a patient with cirrhosis and chronic renal failure

Mucormycosis is an opportunistic fungal infection caused by Mucorales. The disease is uncommon and produces serious and rapidly fatal infection in diabetic or immunocompromised patients. The classical presentation of rhinocerebral mucormycosis is involvement of nasal mucosa with invasion of paranasal sinuses and orbit. Early diagnosis is based on (direct) histological examination and computed tomography scan. Unfortunately the clinical signs and symptoms do not occur in all cases. A high index of suspicion is needed not only in typical groups of immunocompromised patients or diabetics, but also in patients with serious chronic diseases. We report a patient who was not diabetic, but she had a history of cirrhosis and well compensated renal failure.     

Rhinocerebral mucormycosis in renal transplant recipients: report of three cases and review of the literature

Mucormycosis is an opportunistic infection caused by fungi of the order Mucorales. The commonest clinical form is rhinocerebral mucormycosis, which has been described as characteristically complicating diabetes mellitus and leukemia. Three patients with rhinocerebral mucormycosis complicating renal transplantation are described, and 11 additional cases recorded in the English-language medical literature are reviewed. The mean age of the 14 patients was 36 years, and the ratio of males to females was 1.8:1. Diabetes mellitus was present in only five patients, and polycystic kidney was the most common underlying renal disease. Most kidney grafts were obtained from cadavers. Eight patients had evidence of graft rejection, and the majority had been receiving corticosteroids and azathioprine. The initial manifestations of infections became evident two days to four years after transplantation (median, two months). Facial swelling, tissue necrosis, and cranial nerve involvement were common. Seven of 14 cases occurred in Israel, a finding suggesting the intervention of local factors. Despite antifungal and/or surgical therapy, nine patients died as a consequence of the infection days to months after diagnosis. Although a rare complication, rhinocerebral mucormycosis remains a serious threat to the kidney transplant recipient.     

Rhinocerebral mucormycosis treated with 32 gram liposomal amphotericin B and incomplete surgery: a case report

Background  

Mucormycosis (or zygomycosis) is the term for infection caused by fungi of the order Mucorales. Mucoraceae may produce severe disease in susceptible individuals, notably patients with diabetes and leukemia. Rhinocerebral mucormycosis most commonly manifests itself in the setting of poorly controlled diabetes, especially with ketoacidosis.     

Microalbuminuria and increased plasma prorenin. Prevalence in diabetics followed up for four years

When urinary albumin excretion was measured by radioimmunoassay, most diabetics excreted more albumin than nondiabetic subjects. Microalbuminuria was defined as an albumin excretion greater than 30 mg/g of urinary creatinine, more than twice the upper limit of normal. Intermittent microalbuminuria was found in 20% of patients with insulin-dependent diabetes mellitus (IDDM) or non-insulin-dependent diabetes mellitus (NIDDM). Continuous microalbuminuria occurred in a similar percentage of patients with NIDDM, but less frequently in patients with IDDM. Rigorous control of glycemia was followed by cessation of microalbuminuria in nearly half of these patients. Microalbuminuria was associated with an increased incidence of other microvascular complications, as well as a distinctly higher plasma prorenin value in IDDM. Hypertension of 160/100 mm Hg or above was accompanied by increased albumin excretion and lower plasma prorenin values than in normotensive diabetics.     

Gastrointestinal mucormycosis

Mucormycosis is an acutely malignant fungal disease causing local thrombosis and infarction. Gastrointestinal infection most commonly occurs in diabetics or in the immunologically compromised. This case demonstrates the radiological appearances of the gastrointestinal lesion and prolonged survival with amphotericin-B therapy.     

Cutaneous mucormycosis

Mucormycosis is an aggressive invasive fungal infection that occurs rarely in immunocompetent but frequently in immunocompromised patients. We present a case of a 68‐year‐old patient with cutaneous mucormycosis due to Rhizopus pusillus. He was initially hospitalized for invasive pulmonary aspergillosis and diabetes mellitus secondary to acute graft‐versus‐host treatment with glucocorticoids after allogeneic hematopoietic stem cell transplantation for acute myeloid leukemia. Treatment with liposomal amphotericin B and posaconazole was initiated but the patient developed septic shock with multiple organ failure and died 5 days later. The risk factors, clinical presentation, treatment, and prognosis of cutaneous mucormycosis in hematopoietic stem cell and solid organ transplant patients are discussed.     

Healthcare-associated mucormycosis

Mucormycosis is a severe emerging invasive fungal infection that occurs as a consequence of environmental exposure. We exhaustively reviewed all the cases of mucormycosis (European Organisation for Research and Treatment of Cancer/Mycoses Study Group 2008 criteria) attributed to healthcare procedures that occurred between 1970 and 2008. A total of 169 cases were studied (29% children, 61% male). Major underlying diseases were solid organ transplantation (24%), diabetes mellitus (22%), and severe prematurity (21%). Skin was the most common localization (57%), followed by gastrointestinal tract (15%). Culture results were available in 75% (92% positive), and results of histological examination were positive in 95%. Rhizopus was the most frequent genus (43%). Infection portal of entry included surgery and presence of medical devices such as catheters or adhesive tape. Outbreaks and clusters were related to adhesive bandages (19 cases), wooden tongue depressors (n = 5), ostomy bags (n = 2), water circuitry damage (n = 2), and adjacent building construction (n = 5). Thorough investigations are mandatory to identify healthcare-associated mucormycosis, notably in neonatology, hematological, and transplantation units.     

Gastric mucormycosis

A case of gastric mucormycosis proven on culture and histologic examination and successfully treated with resectional surgery and Amphotericin B is described.     

Pulmonary mucormycosis

Mucormycosis (formerly zygomycosis) is a life-threatening opportunistic mycosis that infects a broad range of hosts with qualitative or quantitative defects in innate immunity, including patients with severe neutropenia, recipients of corticosteroids or other immunosuppressive medications, poorly controlled diabetes mellitus, and those with iron overload states. Mucormycosis has recently emerged as breakthrough sinopulmonary infection in hematologic patients and recipients of transplantation being on antifungal prophylaxis with Aspergillus-active antifungals that lack activity against Mucorales. Unlike pulmonary aspergillosis, the prognosis and outcome of pulmonary mucormycosis have not improved significantly over the last decade, mainly because of difficulties in early diagnosis and the limited activity of current antifungal agents against Mucorales. Recent evidence suggests a critical role for iron metabolism and fungal-endothelial cell interactions in pathogenesis of mucormycosis, and holds promise for development of novel therapeutic strategies. Currently, prompt initiation of antifungal therapy with a lipid amphotericin B-based regimen, reversal of underlying host factors, and aggressive surgical approach offers the best chances for survival of patients infected with this devastating mycosis.     

肺毛霉菌病的再认识

肺毛霉菌病是由毛霉菌引起的条件致病性感染,好发于免疫抑制人群.起病急,进展快,临床表现无特异性,可表现为发热、咳嗽、呼吸困难、胸痛等.肺部CT表现包括渗出、实变、结节、空洞、胸腔积液.确诊主要依靠支气管镜或肺穿刺病理活检.尽早采用外科手术或抗真菌药两性霉素B治疗能明显改善预后.     

Pathogenesis of mucormycosis

Mucormycosis is a life-threatening infection that occurs in patients who are immunocompromised because of diabetic ketoacidosis, neutropenia, organ transplantation, and/or increased serum levels of available iron. Because of the increasing prevalence of diabetes mellitus, cancer, and organ transplantation, the number of patients at risk for this deadly infection is increasing. Despite aggressive therapy, which includes disfiguring surgical debridement and frequently adjunctive toxic antifungal therapy, the overall mortality rate is high. New strategies to prevent and treat mucormycosis are urgently needed. Understanding the pathogenesis of mucormycosis and the host response to invading hyphae ultimately will provide targets for novel therapeutic interventions. In this supplement, we review the current knowledge about the virulence traits used by the most common etiologic agent of mucormycosis, Rhizopus oryzae. Because patients with elevated serum levels of available iron are uniquely susceptible to mucormycosis and these infections are highly angioinvasive, emphasis is placed on the ability of the organism to acquire iron from the host and on its interactions with endothelial cells lining blood vessels. Several promising therapeutic strategies in preclinical stages are identified.     

Future directions in mucormycosis research

Mucormycosis has emerged as an important opportunistic infection, especially in severely immunosuppressed hosts. The evolving epidemiology, immunopathogenesis, molecular virulence studies, early diagnosis, and pitfalls in designing clinical studies of mucormycosis are discussed in this article.     

Gastric mucormycosis.

Systemic mucormycosis is a rare fatal fungal infection that usually involves the nasopharynx. Gastrointestinal mucormycosis is rare, occurring in immunocompromised conditions and with advanced malignancies. We report a 35-year-old man, an alcoholic, admitted with acute abdomen. Endoscopy revealed an ulcerated plaque-like lesion in the stomach. Histology revealed mucormycosis of the stomach. The patient successfully underwent treatment with amphotericin-B.