How could hypoglycemia-inducing glycogen storage disease lead to hyperglycemia-induced mucormycosis?

Mucormycosis is an increasingly frequent, difficult to diagnose, difficult to treat, often fatal infection, especially in patients with hyperglycemia from uncontrolled diabetes. Type I (von Gierke) glycogen storage disease is due to inherited deficiency of enzymes in glycogen metabolism, which causes hypoglycemia. This report is the case of a patient with von Gierke disease and a missed diagnosis of pulmonary mucormycosis. This report illustrates the importance of having a high index of suspicion for mucormycosis in the appropriate clinical context.

Pulmonary histoplasmoma: a disguised malady

Histoplasmosis is a mycosis caused by the dimorphic fungus, Histoplasma capsulatum, which is transmitted via dust and aerosols. Lung involvement is the most common, with a varied clinical presentation. Although it is not the only source of infection, H. capsulatum is frequently found in bat guano, which is the reason why it is highly prevalent among caving practitioners. The solitary histoplasmoma of the lung is an unusual and chronic manifestation of this entity, which mimics, or at least is frequently misconstrued, as a malignancy. Almost invariably, the diagnosis of this type of histoplasmosis presentation is achieved after lung biopsy. The authors present the case of a young woman who sought medical care because of chest pain. The diagnostic work-up revealed the presence of a pulmonary nodule. She was submitted to a thoracotomy and wedge pulmonary resection. The histologic analysis rendered the diagnosis of histoplasmoma. Thisreport aims to call attention to this diagnosis as the differential diagnosis of a pulmonary nodule.

Clinical analysis of pulmonary cryptococcosis in non-human immunodeficiency virus infection patients / 北京大学学报(医学版)

OBJECTIVE@#To improve the understanding of the clinical features of pulmonary cryptococcosis in non-human immunodeficiency virus (non-HIV) infection patients and reduce delay in diagnosis, or misdiagnosis.@*METHODS@#The clinical features, imaging characteristics, laboratory examinations, treatment and prognosis of 34 cases of pulmonary cryptococcosis were retrospectively analyzed. The data were collected from Peking University First Hospital from June 1997 to June 2016.@*RESULTS@#There were 34 cases diagnosed with pulmonary cryptococcosis, including 22 males and 12 females, aged from 20 to 75 years [average: (50.1±15.0) years]. There were 16 cases with host factors and (or) underlying diseases named immunocompromised group. In the study, 67.6% patients had clinical symptoms while 32.4% patients had no symptoms. The most common symptoms included cough, fever, chest pain, shortness of breath, and hemoptysis in sequence. Common chest imaging findings were patchy infiltrates, consolidation, single or multiple nodular or masses shadows. Among the 20 cases with cryptococcal capsular polysaccharide antigen detection, 19 were positive. Eleven cases underwent routine cerebrospinal fluid examination, and 3 cases complicated with central nervous system cryptococcal infection. At first visit, 24 cases were misdiagnosed, among which, 11 cases were misdiagnosed as lung cancer. The diagnosis of 15 cases was proved by percutaneous lung biopsy and 11 were confirmed by surgery, while 8 were diagnosed clinically. Then 11 cases were treated by surgical resection, and in median 4 years' followp, there was 1 case of recurrence. And 23 cases were treated with antifungal therapy, and in median 8 years' follow-up, 3 cases lost to the follow-up and 1 case of recurrence. Compared with normal immune group, immunocompromised patients had higher ages (P=0.017), more crackles (P=0.006) and more percentage of increase of peripheral white blood cells or neutrophils (P=0.003), but no significant difference in symptoms, imaging characteristics or hospitalization time.@*CONCLUSION@#There were no specific clinical symptoms and signs for pulmonary cryptococcosis in non-HIV patients. Diagnosis of pulmonary cryptococcosis depends on pathology. Percutaneous lung biopsy was mostly recommended for clinical highly suspected patients. Cryptoeoccal capsular polysaccharide antigen detection had a high sensitivity for the clinical diagnosis. Antifungal drug therapy was the major treatment, and the prognosis of the most patients was good.

Coccidioidomycosis and the skin: a comprehensive review

AbstractCoccidioidomycosis is a highly prevalent disease in the Western hemisphere. It is considered one of the most virulent primary fungal infections. Coccidioides species live in arid and semi-arid regions, causing mainly pulmonary infection through inhalation of arthroconidia although many other organs can be affected. Primary inoculation is rare. Since the first case of coccidioidomycosis was reported in 1892, the skin has been identified as an important target of this disease. Knowledge of cutaneous clinical forms of this infection is important and very useful for establishing prompt diagnosis and treatment. The purpose of this article is to provide a review of this infection, emphasizing its cutaneous manifestations, diagnostic methods and current treatment.

Mucormicosis: Una micosis emergente / Mucormycosis: An emergent mycosis

El objetivo de este trabajo es presentar la incidencia, frecuencia, características clínicas y evolución de los pacientes con mucormicosis atendidos en el Hospital de Clínicas José de San Martín, Universidad de Buenos Aires, entre los años 1982 y 2010. Durante ese período se diagnosticaron 10 casos de mucormicosis. Los tres primeros entre 1982 y 2004 y los últimos 7 entre 2005 y 2010. La incidencia y frecuencia de esta enfermedad, para el período 1980-2004 fue 0.13 pacientes/año y 0.1 casos/10 000 egresos (IC 95%: 0.00 a 0.3) respectivamente. En el período 2005-2010 la incidencia fue 0.86 pacientes/año y la frecuencia de 1.1 casos/10 000 egresos (IC 95%: 0.5 a 2.4). Hubo nueve casos de mucormicosis rinosinuso-orbitaria, siete en pacientes con diabetes mellitus, uno en una paciente con una hemopatía maligna y neutropenia, y el restante en un paciente con HIV/sida que además estaba neutropénico y con un síndrome hemofagocítico. En una paciente se realizó el diagnóstico post mortem de mucormicosis pulmonar. El diagnóstico se efectuó por la observación de filamentos cenocíticos en los diez casos. Hubo desarrollo de mucorales en los cultivos de 8/9 pacientes; cinco Rhizopus spp y tres Mucor spp. Todos los pacientes recibieron un tratamiento inicial con anfotericina B deoxicolato, que en tres de ellos fue continuado con anfotericina B liposomal, y cirugía. Tres enfermos recibieron además un tratamiento adyuvante con oxigeno hiperbárico. La mortalidad fue 30%.

Mucormycosis is an opportunistic infection caused by fungi of the order Mucorales. It is characterized by rapid progression and high morbidity and mortality in the absence of early diagnosis and prompt treatment. It was an infrequent disease, but in recent years, its incidence appears to have increased. The aim of this paper is to report the cases of mucormycosis diagnosed from 1982 to 2010 at the Hospital de Clinicas José de San Martín, University of Buenos Aires. We diagnosed 10 cases of mucormycosis; the first three between 1982 and 2004 and the last 7 between 2005 and 2010. The incidence from 1980 to 2004 was 0.13 patient-years and the frequency 0.1/10 000 discharges (95% CI 0.00- 0.3). In the period 2005 to 2010, the incidence was 0.86 patients per year with 1.1/10 000 discharges (95% CI 0.5-2.4). There was a pulmonary mucormycosis case (in a patient treated with corticosteroids) and nine rhinocerebral cases, two in neutropenic and seven in diabetic patients. The diagnosis was made by observation of cenocytic hyphae in 10/10 patients. Mucorales were recovered in 8/9 cultures (5 Rhizopus spp and 3 Mucor spp.). In one case diagnosis of pulmonary mucormycosis was made post-mortem. Nine patients were treated with amphotericin B deoxycholate (in 3 patients supplemented with liposomal amphotericin B) and surgery. Three patients underwent hyperbaric chamber. Seven patients had favorable outcome. In conclusion, mucormycosis is a rare disease, but its incidence has increased over the past five years. A good evolution of the patients is linked to early diagnosis and treatment.

Criptococcose cutânea primária em paciente imunocompetente / Primay cutaneous cryptococcosis in an immunocompetent patient

Os autores relatam caso de Criptococcose cutânea primária, causada pelo Cryptococcus neoformans, em paciente imunocompetente, fazendeiro que desenvolveu extensivas lesões, no antebraço, após injúria provocada por galináceo, quando fazia limpeza de seu celeiro. Tratamento oral com fluconazol resultou em cura total. A literatura relata raridade de criptococcose cutânea primária em imunocompetentes e sua relativa frequência em imunodeprimidos.

The authors report a primary cutaneous cryptococcosis, caused by Cryptococcus neoformans in immunocompetent patient, a farmer who developed extensive lesions at the site of an injury caused by one of the chickens on his right forearm, while he was cleaning out his barn. Oral treatment with fluconazole was totally successful. A review of the literature showed the rarity of cutaneous cryptococcosis in immunocompetent patients and in contrast, that skin lesions frequently occur in immunocompromised patients.

Histoplasmose pulmonar cavitária crônica / Chronic cavitary pulmonary histoplasmosis

A histoplasmose é uma micose sistêmica causada pelo fungo dimórfico térmico Histoplasma capsulatum, que pode ser isolado a partir de solo contaminado com excrementos de aves e morcegos. Dentre as apresentações clínicas dessa doença, a histoplasmose pulmonar cavitária crônica (HPCC) é uma manifestação rara. O diagnóstico diferencial com tuberculose deve ser realizado em pacientes que apresentam lesões cavitadas nos segmentos pulmonares superiores. É relatado um caso de uma paciente com HPCC que apresentou dispneia progressiva e piora do padrão radiológico em quatro anos de evolução da doença.

Histoplasmosis is a systemic mycosis caused by the thermally dimorphic fungus Histoplasma capsulatum, which can be isolated from soil contaminated with droppings from birds or bats. Chronic cavitary pulmonary histoplasmosis is one of the rarest clinical presentations of this disease. The differential diagnosis with tuberculosis should be made in patients presenting with cavitated lesions in upper lung segments. We report the case of a female patient with chronic cavitary pulmonary histoplasmosis who had presented with progressive dyspnea and worsening of the radiological pattern over a four-year period.

Adiaspiromicose pulmonar humana / Human pulmonary adiaspiromycosis

A adiaspiromicose é uma doença fúngica sistêmica que acomete usualmente roedores e raramente atinge o homem. É causada pelo fungo Emmonsia crescens e ocorre após a inalação da forma contagiante (conídios). Embora estas formas não se multipliquem nem se disseminem no organismo humano, induzem uma reação inflamatória crônica granulomatosa de padrão miliar que pode levar a falência respiratória e morte. Apresentamos aqui um caso de adiaspiromicose pulmonar humana em paciente imunocompetente que exibia infiltrado intersticial pulmonar difuso ao exame de imagem e fora diagnosticado mediante biópsia pulmonar.

Adiaspiromycosis is a systemic fungal disease that usually affects rodents and rarely infects humans. It is caused by the fungus Emmonsia crescens and occurs after inhalation of its contagious form (conidia). Although these forms neither multiply nor spread in the human body, they cause a chronic granulomatous inflammatory reaction of miliary pattern, which may lead to respiratory failure and death. In this study we present a case of human pulmonary adiaspiromycosis in an immunocompetent patient that showed a diffuse pulmonary interstitial infiltrate diagnosed by pulmonary biopsy.

Técnicas histopatológicas no diagnóstico de criptococose por Cryptococcus deficiente de cápsula: relato de caso / Histopathological techniques for diagnosing cryptococcosis due to capsule-deficient Cryptococcus: case report

Caso de criptococose por Cryptococcus deficiente de cápsula, no qual cultivo do espécime clínico e pesquisa do antígeno capsular no líquor e soro foram negativos. As técnicas histopatológicas foram: Hematoxilina-eosina, Grocott, Mucicarmim de Mayer e Fontana-Masson. Confirmou-se o diagnóstico do Cryptococcus deficiente de cápsula pela técnica de Fontana-Masson e pela imunofluorescência direta. É discutida a potencialidade das técnicas histoquímicas.

A case of cryptococcosis due to capsule-deficient Cryptococcus is presented, in which culturing of the clinical specimen and tests for capsular antigen in cerebrospinal fluid and serum were negative. The histopathological techniques evaluated were hematoxylin-eosin, Grocott methenamine silver, Mayer’s mucicarmine and Fontana-Masson. The diagnosis of cryptococcosis due to capsule-deficient Cryptococcus was confirmed by means of the Fontana-Masson technique and by direct immunofluorescence. The potential of the histochemical techniques is discussed.

Candida en el departamento de neonatologia de la Maternidad "Concepción Palacios" enero-junio 2006 / Candida in the department of neonatalogy of the Maternity

En los últimos años la candidemia se ha incrementado en las centros hospitalarios, siendo las áreas de cuidados intensivos donde se observa con mayor frecuencia, ya que los pacientes presentan condiciones que favorecen la instauración de infecciones fúngicas. El objetivo de este estudio fue identificar y conocer la sensibilidad de las especies de Candida predominantes en el Departamento de Neonatología de la Maternidad “Concepción Palacios”. De 349 hemocultivos positivos recibidos del 01 de enero al 30 de junio del 2006; 74 (21.3%) desarrollaron Candida no albicans, con predominio de Candida pelliculosa, una levadura emergente involucrada como agente causal de fungemias, que presentó un patrón de sensibilidad variable.

In the last years the candidemia has increased in the hospital centers, being the areas of intensive care where it appears with major frequency, due to those patients present the conditions for the establishment of fungal infections. The goal of this study was to identify and to know the sensibility of Candida predominant species in the Department of Neonatology of the Maternity “Concepción Palacios”. Of 349 hemocultivos positivos received from january 01 to june 30, 2006; 74 (21.3%) desarrollaron Candida not albicans, with predominance Candida pelliculosa an emergent yeast involved as causal agent of fungemias, that there presented a pattern of changeable sensibility.

Virulence profile of ten Paracoccidioides brasiliensis isolates: Association with morphologic and genetic patterns

Dez isolados de P. brasiliensis foram avaliados em relação à patogenicidade por inoculação intravenosa em camundongos e associação com morfologia miceliana e padrão genético por amplificação genônica do DNA polimórfico (RAPD). A patogenicidade, avaliada por recuperação de fungos viáveis a partir de tecido pulmonar e por lesões histopatológicas em diferentes órgãos, mostrou que os isolados apresentaram quatro graus de virulência: alta virulência, virulência intermediária, baixa virulência e não virulência. A técnica de RAPD agrupou os isolados em dois grupos com 56% de similaridade genética. Amostras com baixa virulência Pb265 ou não virulência Pb192 apresentaram morfologia glabra/cerebriforme e alta similaridade genética (98,7%) quando comparadas com os outros isolados estudados. O mesmo foi observado com os isolados Bt79 e Bt83, que compartilharam 96% de semelhança genética, colônias cotonosas e alta virulência. Essa técnica pode discriminar apenas isolados com morfologia glabra da cotonosa e com alta e baixa virulência. Isolados com virulência intermediária como Pb18, Pb18B6, Bt32 e Bt54 mostraram variabilidade no coeficiente de similaridade, sugerindo que a técnica de RAPD permite mostrar variabilidade genética nessa espécie fúngica. O estudo do perfil de virulência das amostras de P. brasiliensis demonstrou que os dois fenótipos extremos de morfologia miceliana podem ser associados com a virulência do fungo e com o tempo de subcultivo in vitro. Assim, a análise de RAPD, utilizada em conjunto com aspectos de virulência, morfológicos e imunológicos pode ser considerada adequada para detectar diferenças entre isolados de P. brasiliensis.

Histoplasmoma pulmonar no Rio Grande do Sul / Pulmonary histoplasmoma in Rio Grande do Sul

Foram revisados dezesseis prontuários de pacientes que apresentavam nódulo pulmonar à radiografia de tórax diagnosticados como histoplasmoma através de achado histopatológico demonstrando elementos fúngicos compatíveis com Histoplasma capsulatum var capsulatum no Rio Grande do Sul. Revisada a literatura brasileira. Todos os pacientes eram brancos, nove eram homens, a média de idade foi de 50,8 anos. Seis (37,5 por cento) pacientes tinham história epidemiológica sugestiva de contato com fungo. Os nódulos variaram de 0,7 a 2,7 cm de diâmetro, apresentavam-se como granuloma com necrose caseosa. Doze (75 por cento) pacientes apresentavam nódulo solitário. O histoplasmoma como regra se apresenta como nódulo solitário com até três centímetros de diâmetro em paciente assintomático. O diagnóstico é realizado por exame histopatológico com coloração especial para visualização do fungo, método de Gomori-Grocott com metenamina argêntica. Cultivo ou testes soromicológicos não contribuem para o diagnóstico.

Enfermedad de Lutz-Splendore-De Almeida sin manifestaciones mucocutáneas: a propósito de un caso / Disease Lutz-Splendore-De Almeida without mucocutaneous manifestations: a purpose of a case

La enfermedad de LUTZ-SPLENDORE-DE ALMEIDA se debe al Paracoccidiodes brasiliensis, presente en Latinoamérica. Al principio la infección pulmonar produce pocos síntomas y la diseminación hasta mucosas, ganglios linfáticos y otros; hace que el paciente acuda al médico. Los signos son: úlceras induradas en mucosas de orofaringe, y nariz, adenomegalias, lesiones cutáneas y genitales, tos productiva, pérdida de peso, disnea y fiebre. Radiológicamente muestra una neumonía con focos dispersos bilaterales, el cultivo de secreciones, las pruebas serológicas confirman el diagnóstico; se trata con Itraconazol o Trimetopín-Sulfametoxazol. Se describe caso de un masculino de 49 años, agricultor, quien consultó por tos y pérdida de peso, confirmado mediante estudios imagenológicos, serológicos, histológicos y fibrobroncospia, durante su evolución no cursó con manifestaciones cutáneo-mucosas, siendo divergentes al común de los casos, ya que dichas lesiones preceden o acompañan la forma pulmonar. Es importante integrar al pensamiento médico la existencia del Paracoccidioides brasiliensis, como agente etiológico de infecciones respiratorias aun sin manifestaciones cutáneas.

The first imported case of pulmonary coccidioidomycosis in Korea

Coccidioidomycosis is an endemic disease found in the southwestern part of North America. Travellers who visit the endemic area may carry the infection. We report a case of pulmonary coccidioidomycosis in a 74-year-old woman. She was healthy before visiting Arizona, U.S.A twice. After returning home, she began to complain of intermittent dry coughing. The symptom was mild, however, and she was treated symptomatically. Later a chest radiograph, which was taken 4 years after the onset of the symptom, showed a solitary pulmonary nodule in the right upper lobe. By percutaneous needle aspiration, a few clusters of atypical cells were noted in the necrotic background. A right upper and middle lobectomy was done. A 1.5 x 1.5 x 1.2 cm sized tan nodule was present in otherwise normal lung parenchyma. Microscopically, the nodule consisted of aggregates of multiple solid granulomas inside of which was mostly necrotic. Neutrophils and nuclear debris were scattered along the periphery of the necrotic foci. Numerous multinucleated giant cells were associated with the granulomas. In the necrotic area, mature spherules of Coccidioides immitis, which were 30-100 microm in diameter, were present. They contained numerous endospores which ranged from 5 to 15 microm and were also noted in multinucleated giant cells. The diagnosis of coccidioidomycosis was made. She is doing well after the resection.

Massive pulmonary cryptococcosis in an immunocompetent patient.

A 64-year-old man presented with progressive dyspnea. The symptom of severe hypoxia requiring mechanical ventilator, and bilateral pulmonary infiltrates on the chest film led to the clinical diagnosis of adult respiratory distress syndrome. Autopsy demonstrated widespread cryptococci and mucinous material in alveoli with mild inflammatory response.

Adiaspiromicose humana: relato de um caso tratado com cetoconazol / Human adiaspiromycosis. A report of a case treated with ketoconazole

É descrito um caso de adiaspiromicose humana, da forma pulmonar disseminada, no qual se empregou o cetoconazol. O paciente, oriundo de Goianésia, GO, referia tosse produtiva, dispnéia e emagrecimento. Doente há dois meses, já fizera uso do esquema tríplice tuberculostático, devido a um radiograma do tórax ter acusado lesões sugestivas de tuberculose miliar. Esse tratamento não surtiu o efeito esperado, pelo que foi ele submetido a uma biópsia pulmonar a céu aberto. A medida permitiu o reconhecimento da natureza fúngica da doença. Passou-se, então, ao emprego do cetoconazol, quando o processo já completara três meses de evolução. Dois meses depois, foi o paciente novamente visto, para controle do tratamento: todas as manifestações respiratórias haviam cessado e um novo radiograma mostrou regressão completa das alterações pulmonares. Apesar disso, é discutida a eficácia do cetoconazol contra C. parvum var crescens, tendo-se em conta o fato de o microrganismo não se reproduzir no hospedeiro.

A case of human disseminated pulmonary adiaspiromycosis is reported. The patient, from Goianésia, GO, was admitted to the Brasilia University Hospital, in November 1992, with wet cough, dyspnea and weight loss--manifestations that had appeared two months before. Prior to admission, he had been treated for a suspected miliary tuberculosis, because a chest roentgenogram had shown a diffuse reticulonodular infiltrate in both lungs. This therapy brought no improvement to the patient status. An open chest biopsy was then performed, and the microscopic examination of the lung tissue revealed the fungal nature of the disease. Ketoconazole, 400mg/day, was started and the patient discharged from the hospital. He was seen again two months later: the respiratory manifestations had disappeared and a new chest roentgenogram showed complete resolution of the pulmonary lesions. The usefulness of ketoconazole is, however, questioned, since, as there is no multiplication of the fungus in the host organism--adiaspiromycosis is believed to be, usually, a self-healing disease--the efficacy of this imidazole derivative against the agent in animal tissues remains to be confirmed.

Adiaspiromicose pulmonar disseminada: relato de caso / Disseminated pulmonary adiaspiromycosis: a case report

Relata-se caso de adiaspiromicose causando infiltrado pulmonar retículo-nodular difuso e bilateral, em lavrador de 26 anos. O diagnóstico etiológico foi estabelecido através de biópsia por toracoscopia. Tratado com cetoconazol, o paciente evoluiu bem, retornando às atividades profissionais um mês após a alta.

A case of thoracoscopic lung biopsy proven diffuse human adiaspiromycosis is reported. The patient, a 26-year-old male farm worker presented with a three-week history of fever, sweating, dyspnea and unproductive cough. Radiographic findings were those of granulomatous pulmonary interstitial disease. Treated with ketoconazole he improved very well, resuming work normal activities a month later.

La infección pulmonar y sistémica causada por el Paracoccidioides brasiliensis. Avances recientes y perspectivas terapéuticas / Pulmonary and systemic infection caused by Paracoccidioides brasiliensis. Recent advances and therapeutic perspectives

La paracoccidioidomicosis es una infección fúngica subaguda o crónica adquirida por la inhalación del Paracoccidioides brasiliensis, caracterizada por la presencia de infiltados pulmonares "en mancha", y/o lesiones ulcesosa de la mucosa oronasal y la piel. La linfadenopatía es frecuente. En los casos diseminados, todas las vísceras pueden ser afectadas, pero las glándulas suprarrenales son especialmente susceptibles. El diagnóstico se confirma con el examen microscópico directo de los productos patológicos, la histopatología o el cultivo del agente infeccioso. Las técnicas de seroinmunología pueden ser de utilidad en el diagnóstico. Es endémica en las regiones tropicales y subtropicales de Sudamérica, Centroamérica y México. Los trabajadores con mayor riesgo son aquellos en contacto con el suelo, principalmente los campesinos, agricultores y obreros de la construcción; la incidencia más alta se ha registrado entre los adultos de 30 a 50 años de edad, siendo mucho más prevalente en los hombres que en las mujeres. El reservorio probablemente sea el suelo o la tierra cargada con las esporas del hongo. El medicamento de elección es el itraconazol. La anfotericina B seguida de una terapia prolongada con sulfas, resulta ser más barata pero menos efectiva. Sin embargo, los casos diseminados más graves, y la insuficiencia respiratoria residual representan un reto significativo a la terapéutica disponible.