Neonatal Acute Liver Failure Associated with Angioinvasive Hepatic Zygomycosis.

BACKGROUND: Neonatal acute liver failure (NALF) is often a fatal condition. Zygomycosis is a fungal infection that is often fatal in both adults and infants. Only a few cases of hepatic zygomycosis are reported in the literature and they are invariably associated with immunosuppression. MATERIALS AND METHODS: Post-mortem liver biopsy from a 14-day old neonate demonstrated confluent panacinar necrosis with angioinvasive zygomycosis. The limited work-up could not rule out an underlying immunodeficiency. CONCLUSION: Angioinvasive hepatic zygomycosis can present in the neonatal period as NALF.

Hyperbaric oxygen therapy and other adjunctive treatments for zygomycosis.

Zygomycetes are increasingly reported as a cause of life-threatening invasive fungal infections in profoundly immunocompromised patients and in those with diabetic ketoacidosis. Zygomycosis, typically presents as soft tissue, rhino-orbitocerebral, pulmonary or disseminated disease and is characterized by rapid clinical progression and high mortality rates. Treatment with amphotericin B lipid formulations in combination with surgery and, perhaps, the addition of caspofungin offers the best chance for survival; posaconazole, a new antifungal triazole, is increasingly used for consolidation or maintenance therapy. Because of the poor prognosis of zygomycosis, particularly in immunocompromised cancer patients, adjunctive treatments such as hyperbaric oxygen therapy, use of immunomodulatory cytokines, and in vivo iron starvation continue to be explored. However, although each of these modalities is based on a plausible scientific rationale and has been helpful in the management of individual patients, there is no clinical evidence for their general effectiveness as adjunctive treatments in patients with zygomycosis. Further experimental and clinical investigations are necessary to determine whether and how these treatments can impact on outcome and to determine which patients and which types of infection may benefit from them.

ZYGOMYCOSIS: current approaches to management of patients with haematological malignancies.

Zygomycosis is an invasive infection that can occur particularly in patients with haematological malignancy. The causative fungi are members of the order Mucorales, and individual species within this group require a high level of laboratory skill to be identified. Zygomycosis can present as rhinocerebral, pulmonary, or disseminated disease, with a rapid clinical course. The optimal management of these cases requires early diagnosis, aggressive antifungal therapy and, when possible, surgical debridement. Founded on clinical experience, but without the benefit of comparative studies, liposomal amphotericin B has become the therapeutic agent of choice. Posaconazole is an orally administered triazole with a demonstrated in vitro and in vivo activity against most Zygomycetes that is comparable to that of amphotericin B. Studies on salvage therapy with posaconazole have yielded promising results, and successful case reports are also available. As an adjuvant approach, iron chelation with deferasirox has shown promising results, although clinical experience is still limited.

[Zygomycosis].

Zygomycosis is an invasive fungal infection with extremely high mortality caused by filamentous fungi which belong to Class Zygomycetes (Rhizopus spp., Mucor spp., Cunninghamella spp., etc). Despite of the similarities of the ecological characteristics and of the patients' backgrounds, zygomycosis is much rarer than invasive aspergillosis. In addition to well known immunosuppressive risk factors (hematological malignancy, hematopoietic stem cell or solid organ transplant, prolonged neutropenia, corticosteroid, etc), diabetic ketoacidosis, iron overload, and administration of deferoxamine are specific factors predisposing zygomycosis. Rhinocerebral, pulmonary and disseminated disease is characteristic forms. The mainstay of the treatment is surgical resection, reversal of immunosuppressive factors, and administration of high-dose amphotericin B or its liposomal formulation. Because of the difficulty of culture detection and the absence of reliable serological diagnostic methods, premortem diagnosis and no delaying of effective treatment remain a challenge to physicians.

Diagnosis and treatment of cutaneous zygomycosis.

Cutaneous zygomycosis is a rare but severe fungal infection with high risk of dissemination. Early recognition, deep surgical biopsy for diagnosis, aggressive treatment with repeated surgical debridement, and targeted pharmacotherapy are essential and can prevent dissemination and fatal outcome. We present case reports of 2 patients.

Rhinocerebral zygomycosis: an increasingly frequent challenge: update and favorable outcomes in two cases.

Zygomycosis or mucormycosis is an increasingly frequent life-threatening infection caused by opportunistic fungal organisms of the class Zygomycetes. The pathognomonic feature is the presence of invasive aseptate mycelia that are larger than other filamentous fungi with the hyphae exhibiting right angle and haphazard branching. Usually classified as rhinocerebral, disseminated, and cutaneous types, this classification serves as important predictor of pathogenesis and outcome. These occur mostly in immunosuppressed patients including individuals with diabetes (43% exhibit the rhino-cerebral form) and patients with organ transplants and hematologic malignancies. Without early aggressive treatment, the disease follows a dismal and fatal course. The prognosis has not shown any appreciable change in the past 40 years with a stagnant mortality rate of 44%. We present 2 cases of rhinocerebral zygomycosis (RCZ), in a 58-year-old male and a 63-year-old female; both were poorly controlled diabetic patients with maxillary lesions suggestive of osteomyelitis. The patients were leading a near normal life with minimal discomfort or signs and symptoms of underlying mycosis. Most of the health care professionals treating these patients often overlooked the disease or recommended inadequate therapy. Despite long delays and inadequate initial therapy these patients survived with little outward morbidity. The prognosis for this condition may therefore be considered less dire than previously thought.

Zigomicetos y zigomicosis en la era de las nuevas terapias antifúngicas / Zygomycetes and zygomycosis in the new era of antifungal therapies

La zigomicosis o mucormicosis es la tercera infección fúngica invasora tras la candidiasis y la aspergilosis. Tradicionalmente se ha consideradouna enfermedad de adquisición comunitaria, pero se está convirtiendo en una infección de frecuente adquisición nosocomial. En los últimosaños, numerosos estudios en instituciones aisladas apuntan a un aumento del número de casos de zigomicosis invasora a raíz de las nuevasterapias antifúngicas e inmunosupresoras, y al aumento de la población inmunodeprimida. Por otro lado, el diagnóstico de la zigomicosismuchas veces es complicado, sobre todo en las formas pulmonares y diseminadas. Uno de los principales problemas que presenta el aislamientode zigomicetos de muestras clínicas en el laboratorio de microbiología es que con frecuencia los resultados tienen una difícil interpretación.Además, el aumento del número de micosis invasoras por hongos resistentes a los antifúngicos ha llevado al desarrollo de nuevasmoléculas con actividad antifúngica y diferentes perfiles de actividad frente a los zigomicetos(AU)

Zygomycosis or mucormycosis is the third most invasive fungal infection after candidiasis and aspergillosis. Traditionally, it has been considereda community-acquired disease, but it is becoming a frequent nosocomial-acquired disease. Recently, several publications from differentinstitutions have reported an increase in the number of cases of invasive zygomycosis as a result of the new antifungal and immunosuppresivetherapies and the emerging immunocompromised population. In addition, the diagnosis of zygomycosis is elusive, mainly in pulmonaryand disseminated forms. One of the main limitations in isolating Zygomycetes from clinical samples is the interpretation of results. The increasingnumber of invasive fungal infections caused by multiresistant fungi has led to the development of new antifungal drugs with variableactivity against Zygomycetes(AU)

[Zygomycetes and zygomycosis in the new era of antifungal therapies]. / Zigomicetos y zigomicosis en la era de las nuevas terapias antifúngicas.

Zygomycosis or mucormycosis is the third most invasive fungal infection after candidiasis and aspergillosis. Traditionally, it has been considered a community-acquired disease, but it is becoming a frequent nosocomial-acquired disease. Recently, several publications from different institutions have reported an increase in the number of cases of invasive zygomycosis as a result of the new antifungal and immunosuppresive therapies and the emerging immunocompromised population. In addition, the diagnosis of zygomycosis is elusive, mainly in pulmonary and disseminated forms. One of the main limitations in isolating Zygomycetes from clinical samples is the interpretation of results. The increasing number of invasive fungal infections caused by multiresistant fungi has led to the development of new antifungal drugs with variable activity against Zygomycetes.

Hyperbaric oxygen as an adjunctive treatment for zygomycosis.

Zygomycosis is a rare but emerging mycosis. Because of the sub-optimal efficacy of the standard antifungal treatment for this disease, hyperbaric oxygen (HBO) has been used occasionally as an adjunctive therapeutic modality. A review of 28 published cases of zygomycosis indicates that adjunctive HBO may be beneficial in diabetic patients (94% survival), whereas its benefit in the small group of patients with haematological malignancies or bone marrow transplants is doubtful (33% survival; p 0.02). Prolonged courses of HBO were associated with a higher survival (100% survival; p 0.003). Additional studies are required to assess the optimal timing and dose for HBO treatment.