Sergio González B

Meningoencefalitis granulomatosa por Balamuthia mandrillaris: Reporte de un caso y revisión de la literatura

Las amebas de vida libre (AVL) de los generos Naegleria y Acanthamoeba son reconocidos agentes causales de infecciones del sistema nervioso central. Durante los ultimos anos, otra ameba de vida libre, Balamuthia mandrillaris, ha sido identificada como agente etiologico de meningoencefalitis granulomatosa amebiana (MGA) en humanos. Presentamos el caso de una escolar de sexo femenino, inmunocompetente en quien se realizo el diagnostico post mortem de MGA por este agente. Consulto por aparicion de lesiones eritematosas e induradas que comprometian la zona centro-facial. En biopsia cutanea se evidencio una lesion granulomatosa con RPC positiva para secuencias genicas de Mycobacterium atipico, por lo que se inicio tratamiento para micobacteriosis atipica extrapulmonar. Evoluciono con compromiso neurologico progresivo, falleciendo aproximadamente un ano despues de iniciar los sintomas. La necropsia revelo una MGA, cuyo estudio posterior demostro la presencia de B. mandrillaris. La infeccion por B. mandrillaris debe ser considerada en el diagnostico diferencial de una enfermedad granulomatosa cronica que evoluciona con compromiso neurologico

Tuberculosis cutánea: reporte de dos casos y revisión de la literatura

Cutaneous tuberculosis is a chronic infectious disease caused by Mycobacterium tuberculosis. It is uncommon (1% of all cases of tuberculosis), but has increased due to the human immunodeficiency virus epidemic and to pharmacologic immunosuppression. It presents a wide variety of clinical forms depending on how bacteria reach the skin and on the immune status of the patient. We present two cases of cutaneous tuberculosis diagnosed in the Hospital Clinico de la Pontificia Universidad Catolica de Chile. We emphasize the difficulty in diagnosis and classification of this disease and briefly discuss on the different diagnostic and therapeutic approaches.

Enfermedad de Hansen: Revisión a propósito de un caso

Leprosy is a granulomatous disease affecting the skin and peripheral nerves caused by Mycobacterium leprae. The range of clinical forms varying from tuberculoid to lepromatous leprosy results from variations in the cellular immune response to the mycobacterium. Despite available combined drug-therapy, it continues to be a significant public health problem, carrying a strong stigma. Although recently there has been no native cases in Chile, a few imported cases have been diagnosed. We present a 56-year-old man who had lived in Paraguay for 8 years, and presented with leprosy 6 years after returning to Chile. The biology of leprosy, clinical features of the disease, current diagnostic criteria and approaches to treatment are discussed.

Evaluación clínico-radiológica y clasificación de la bronquiolitis del adulto

Bronchiolar disorders are generally difficult to diagnose. A detailed clinical history may point toward a specific diagnosis. Pertinent clinical questions include history of smoking, collagen vascular disease, inhalation injury, medication use and organ transplantation. It is important also to evaluate possible systemic and pulmonary signs of infection, evidence of air trapping, and high-pitched expiratory wheezing, which may suggest small airways involvement. Pulmonary function tests and plain chest radiography may demonstrate abnormalities; however, they rarely prove sufficiently specific to obviate bronchoscopic or surgical biopsy. High-resolution CT (HRCT) scanning of the chest is often an important diagnostic tool to guide diagnosis in these difficult cases, because different subtypes of bronchiolar disorders may present with characteristic image findings. Some histopathologic patterns of bronchiolar disease may be relatively unique to a specific clinical context but others are nonspecific with respect to either etiology or pathogenesis. Primary bronchiolar disorders include acute bronchiolitis, respiratory bronchiolitis, follicular bronchiolitis, mineral dust airway disease, constrictive bronchiolitis, diffuse panbronchiolitis, and other rare variants. Prominent bronchiolar involvement may be seen in several interstitial lung diseases, including hypersensitivity pneumonitis, collagen vascular disease, respiratory bronchiolitis-associated interstitial lung disease, cryptogenic organizing pneumonia, and pulmonary Langerhans’ cell histiocytosis. Large airway diseases that commonly involve bronchioles include bronchiectasis, asthma, and chronic obstructive pulmonary disease. The clinical and prognostic significance of a bronchiolar lesion is best determined by identifying the etiology, underlying histopathologic pattern and assessing the correlative clinic-physiologic-radiologic context.

Morfea en niños: Revisión bibliográfica y puesta al día

Introduccion: La Morfea o Esclerodermia localizada es una enfermedad del tejido conectivo de etiologia desconocida. Es asintomatica y de curso variable, generalmente autolimitada, puede dejar secuelas funcionales y esteticas importantes. Objetivos: Describir las caracteristicas clinicas de la enfermedad y el manejo terapeutico actual. Material y Metodo: Revision bibliografica y de Medline. Resultados: La patologia se deberia a un deposito excesivo de colageno, lo que se manifiesta como una atrofia epidermica y un engrosamiento e induracion de la dermis. Diversos mecanismos estarian involucrados, destacando la radiacion, vacunas, infecciones, inmunologicos y traumas. En ninos la presentacion clinica es diversa, y se clasifica en Lineal, en Placa y Generalizada. No existe un examen diagnostico, debiendo correlacionarse la clinica con los diversos metodos de estudio. Los tratamientos mas usados son los corticoides, analogos de vitamina D, antirreumaticos y fototerapia. Conclusiones: Dado lo infrecuente y autolimitado de la enfermedad, no existe acuerdo en cuanto a quienes deben ser tratados, ni en la forma, ni en la duracion del tratamiento. Su pronostico depende del tipo de lesion y de las caracteristicas de cada paciente

Síndrome de Sweet asociado a leucemia mieloide aguda y factor estimulante de colonias de granulocitos: Caso clínico

Sweets syndrome, also known as acute febrile neutrophilic dermatosis, is characterized by fever, neutrophilia, erythematous and tender skin lesions that typically show a diffuse infiltrate of neutrophils in the upper dermis. This disorder has been associated with myeloproliferative syndromes. We report the case of a 53-year-old woman with an acute myeloid leukemia, presenting a Sweets syndrome. A worsening of cutaneous lesions injuries was observed when granulocyte colony stimulating factor was added to treatment.

Necrosis cutánea: un desafío para el médico

Skin necrosis must be considered as a syndrome, because it is a clinical manifestation of different diseases. An early diagnosis is very important to choose the appropriate treatment. Therefore, its causes should be suspected and confirmed quickly. We report eleven patients with skin necrosis seen at our Department, caused by different etiologies: Warfarin-induced skin necrosis, loxoscelism, diabetic microangiopathy, ecthyma gangrenosum, disseminated intravascular coagulation, necrotizing vasculitis, paraneoplastic extensive necrotizing vasculitis, livedoid vasculopathy, necrotizing fasciitis, necrosis secondary to the use of vasoactive drugs and necrosis secondary to the use of cocaine. We also report the results of our literature review on the subject.

Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento

Background: Ciliary dyskinesia (CD) is a low incidence genetic illness, that presents with a wide clinical spectrum. Also, there are transitory conditions that present with ciliary anomalies, secondary to infectious diseases of the airways. Aim: To describe clinical and ultrastructural findings and clinical and therapeutic evolution of these patients. Patients and Methods: Retrospective review of medical records and electron microscopy findings of 33 patients (aged 1 to 21 years, 14 females) with ultrastructural diagnosis of CD. To obtain follow up information, a telephone survey was done. Results: In 30 patients (90%) the inner dynein arm (IDA) was absent in 50 or more percent of the cilia. Twenty two (66%) had absence of the outer dynein arm. Before diagnosis of CD, 19 patients (57%) presented recurrent otitis media, 25 patients (77%), three or more episodes of rhinosinusitis and 18 patients (56%) had recurrent pneumonia. Middle ear ventilation tubes were placed in 19 patients (57%), and during its use, 12 (68%) remained without othorrea. Sixteen patients (48%) with recurrent episodes of rhinosinusitis required adenoidectomy. Seven (21%) required a functional endoscopic sinus surgery (FESS), and 6 (86%) improved after FESS. Conclusions: Our patients with CD presented recurrent infections in different airway locations. In those with a diagnosis of CD and recurrent otological and rhinosinusal infections, IDA was absent in a high percentage of cilia. FESS and the use of ventilation tubes may have a beneficial role in a subgroup of patients with CD (Rev Med Chile 2007; 135: 1147-52). (Key words: Ciliary motility disorders; Otitis; Rhinitis; Sinusitis)

Bursitis Tuberculosa: Caso clínico

We report a previously healthy 51 years old woman with a one year history of pain in the left hip associated with a mass without fever or local inflammatory changes. Magnetic resonance imaging located the mass in the medial gluteal muscle. The pathological examination of the mass disclosed unspecific inflammatory changes. Due to worsening of pain, a left throchanteric bursitis was diagnosed two weeks later and a throchanteric bursectomy was performed, obtaining a second biopsy. Aerobic, anaerobic and fungal cultures were negative. Lowenstein-Jensen culture showed development of mycobacteria and a genetic probe confirmed the presence of Mycobacterium tuberculosis. The second biopsy also identified several granulomas with areas of caseation and the Ziehl-Nielsen stain was positive for acid fast bacilli. Osteoarticular tuberculosis, specially bursitis, shoulds be suspected in the presence of chronic pain associated with swelling (Rev Med Chile 2002; 130: 319-21)

Bronquiolitis obliterante con neumonía en organización secundaria a quimioterapia, en un niño con sarcoma pericárdico primario

The uncommon bronchiolitis obliterans organizing pneumonia can be idiopathic or caused by infection or medications. We report a 5 year old boy with pericardial sarcoma that was treated with chemotherapy (vincristine, doxorubicin, etoposide, cyclophosphamide) and radiotherapy. During the third cycle of chemotherapy, he developed progressive cough and dyspnea, needing oxygen therapy. Chest X-ray examination showed bilteral infiltrates. After discarding infectious etiologies, an open lung biopsy was performed, and the pathological examination showed a bronchiolitis obliterans organizing pneumonia. He was treated with prednisolone for 7 days, followed by prednisone for additional 45 days. He continued with fluticasone propionate as maintenance and remains in good condition with a ches X ray showing mild interstitial images, after seven months of follow up. (Rev Med Chile 2000; 128: 633-40).