Enfermedad de Creutzfeldt-Jakob: experiencia de 5 años en un hospital terciario de Chile / Creutzfeldt-Jakob disease, experience in 17 patients

Background: Creutzfeldt-Jakob disease (CJD) is a prion affection that typically produces a rapidly progressive dementia with different neurologic and extra-neurologic manifestations. Aim: To characterize clinical, imaging and electroencephalography findings in patients with a probable CJD. Patients and Methods: A case series study of patients admitted in the Neurology department at a public hospital, between 2014 and 2019. Demographic, clinical, imaging, and electroencephalographic data of patients with probable CJD were analyzed. Results: Seventeen patients aged 63 ± 11 years (53% women) with a probable CJD were gathered. The incidence was 4.7 cases/year per million inhabitants. Twenty four percent of patients had a family history of CJD. The median time between the onset of symptoms and the hospital admission was three months with a survival of four months. The most common clinical manifestations were an amnesic syndrome in 88%, myoclonus in 76%, frontal syndrome and ataxia in 71%. Brain MRI was abnormal in all patients. The preponderant finding was the involvement of the caudate nucleus in 82% of cases. In the EEG, 94% of patients had abnormalities. All had a theta-delta slowing as a base rhythm. The pseudo-periodic pattern was observed in the 29% and status epilepticus in 18%. Conclusions: In this group of patients we observed the heterogeneity of the clinical manifestations of the disease, the frequent imaging and electroencephalographic alterations and the short evolution time leading to death.

Microsecretory Adenocarcinoma of Salivary Glands: An Expanded Series of 24 Cases.

Microsecretory adenocarcinoma (MSA) is a recently described salivary gland tumor with a characteristic histologic and immunophenotypic profile and recurrent MEF2C-SS18 fusions. Because only six cases of MSA have been published, its complete clinicopathologic spectrum is unclear, and its biologic behavior has not been documented. Here, we present an updated and expanded experience of 24 MSA cases. All cases of MSA were obtained from the authors' files. Immunohistochemistry for S100, SOX10, p63, p40, SMA, calponin, and mammaglobin was performed. Molecular analysis was performed by targeted RNA sequencing, SS18 break apart fluorescence in situ hybridization, and/or reverse transcriptase polymerase chain reaction for MEF2C-SS18 fusion. Clinical follow-up was obtained from medical records. A total of 24 MSA cases were collected, from 13 women and 11 men, ranging from 17 to 83 years (mean 49.5 years). The vast majority (23 of 24) arose in the oral cavity, with the palate (n = 14) and buccal mucosa (n = 6) as the most frequent subsites. Tumors showed consistent histologic features including: (1) microcystic tubules, (2) flattened intercalated duct-like cells, (3) monotonous oval hyperchromatic nuclei, (4) abundant basophilic luminal secretions, (5) fibromyxoid stroma, and (6) circumscribed borders with subtle infiltration. The tumors were very consistently positive for S100 (24 of 24), p63 (24 of 24), and SOX10 (14 of 14) and negative for p40 (0 of 21), calponin (0 of 12) and mammaglobin (0 of 16), while SMA (4 of 20) was variable. MEF2C-SS18 fusion was demonstrated in 21 of 24 cases; in the remaining 3 cases with insufficient RNA, SS18 break apart FISH was positive. Treatment information was available in 17 cases, all of which were managed with surgery only. In 14 cases with follow-up (1-216 months, mean 30), no cases recurred or metastasized. MSA is a distinct salivary gland neoplasm with remarkably consistent clinical, histologic, immunophenotypic, and genetic features that generally behaves in an indolent manner following surgery alone. These observations solidify MSA as a unique, low-grade salivary gland carcinoma that warrants inclusion in the next version of the WHO classification of head and neck tumors.

Sialolipoma of minor salivary glands: presentation of five cases and review of the literature with an epidemiological analyze.

UNLABELLED: Sialolipoma is rare benign neoplasm arise from salivary glands (majors and minors) characterized by neoplastic adipose tissue with scattered non-neoplastic salivary gland acinus. To date 60 cases (including 5 cases reported in the present paper) have been reported in scientific literature. This article presents 5 new cases of sialolipoma affecting minor salivary glands (MiSG) and additionally reviews and analyzes the previously published cases to assess possible demographical differences between sialolipoma from minor and from major salivary glands. CASE REPORTS: 5 cases (3 females; 2 males; age means 63.8 years), of sialolipoma from MiSG, are reported. 2 of them were located in buccal mucosa, 1 in upper lip mucosa, 1 in floor of the mouth and 1 in retromolar area. All tumors were composed by neoplastic adipocytes cells interlaced with normal salivary gland acinus cover it by a fibrous tissue capsule. Analyzes of literature showed that MiSG sialolipoma is most frequent in females over 60 years old, therefore and in conclusion this article assess different demographical profile of sialolipoma in respect to their topography.

Indications and procedures for direct immunofluorescence biopsies of the oral mucosa.

A definitive diagnosis is crucial for management of any oral mucosal disease. Direct immunofluorescence (DIF) is a valuable diagnostic aid for immune-mediated blistering diseases and systemic connective tissue diseases of the skin and the mucosa. This paper gives an overview of the DIF biopsy technique for oral lesions and provides a background for the clinician to optimize the utilization of DIF biopsy. The key characteristic diagnostic findings of DIF of specific mucosal diseases are also discussed.

The challenges of defining oral cancer: analysis of an ontological approach.

An important inconsistency currently exists in the literature on oral cancer. Reviewing this literature, one finds that the term oral cancer is defined and described with great variation. In a search in PubMed, at least 17 different terms were found for titles of papers reporting data on oral cancer. The variability of the terms used for designating anatomic regions and type of malignant neoplasms for reporting oral cancer has hampered the ability of researchers to effectively retrieve information concerning oral cancer. Therefore, it is sometimes extremely difficult to provide meaningful comparisons among various studies of oral cancer. Recently, a new ontological strategy that is rooted in consensus-based controlled vocabularies has been proposed to improve the consistency of data in dental research (Smith et al. in J Am Dent Assoc 141:1173-1175, 2010). In this paper, we analyzed the terminology dilemma on oral cancer and explained the current situation. We proposed a possible solution to the dilemma using an ontology-based approach. The advantages for applying this strategy are also discussed.

En búsqueda del Phymatotrichopsis Omnívora, en pobladores de la Colonia Tovar, Venezuela, octubre 2007 / Phymatotrichopsis Omnivore, study search at Aragua Colonia Tovar residents, Venezuela, October 2007

Se realizó un estudio descriptivo, y de corte transversal, con el propósito de investigar la presencia del hongo Phymatotrichopsis omnívora como agente etiológico , del alerta epidemiológica denunciada en un medio de comunicación regional, la cual le atribuyo a este hongo el deterioro de la cosecha del durazno, conocida por los pobladores como secason y algunas lesiones de piel en pacientes adultos y pediátricos de los caseríos, Gabante, área urbana y el Cedral, del Municipio Tovar, Estado Aragua; Venezuela. Se examinaron 144 pacientes con lesiones de piel sospechosos de micosis superficial que acudieron a la consulta de los centros de salud de la zona. Se realizó examen de piel y faneras, examen directo; cultivos para hongos y biopsia de piel los cuales fueron procesados en el laboratorio del Hospital Central de Maracay y en el Servicio de Anatomía Patológica del Instituto Autónomo de Biomedicina, Caracas. Los datos básicos se registraron en ficha epidemiológica; se procedió al análisis estadístico; encontrándose entre las 10 primeras patologías dermatológicas: Pitiriasis alba (18,75%), Queratosis Folicular (11,1%), Quiste Millium (10,4%), Acné (6,25%),Prurigo Infantil (6,25%), Picaduras de Insectos (5,5%),Dermatitis Seborreica (4,9%), Micosis Superficial (4,16%), Urticaria (4,16%), Melasma (3.47%), otras (2.7%). De origen micótico encontramos, dos con Pitiriasis Versicolor cuyo agente etiológico fue Malassezia furfur; dos Onicomicosis, uno Tiña Corporal y uno con Tiña Inguinal; aislándose en los cultivos Trichophytum rubrum. En conclusión: de 144 personas examinadas ninguna presentó lesiones atribuidas al hongo Phymatotrichopsis omnívora.

It was made a cross-sectional descriptive, with the purpose of investigating the presence of the mushroom Phymatotrichopsis omnivore as an etiological agent, of an epidemic alert reported on a regional communications medium; which attributed to this fungus deterioration peach crop, known to residents as 'secasón' and some skin lesions in adult and paediatrics patients in the hamlets, Gabante, urban and Cedral, Tovar Municipality, Aragua; Venezuela. We examined 144 patients with skin lesions suspected of superficial fungal infections and they were seen at the health centers in the area. Examination was made of skin and appendages, direct examination, fungal cultures and skin biopsies which were processed in the laboratory of the Central Hospital of Maracay and the Pathology Department of the Autonomous Institute of Biomedicine, Caracas. Baseline data were recorded in epidemiological record, we proceeded to statistical analysis to be among the top 10 dermatological diseases: Pityriasis Alba (18.75%), follicular keratosis (11.1%), cyst Millium (10.4%) Acne (6.25%), Child Prurigo (6.25%), insect bites (5.5%), seborrheic dermatitis (4.9%) Superficial Mycoses (4.16%), urticaria (4.16 %), melasma (3.47%), other (2.7%). Of fungal origin found, two with Pityriasis Versicolor whose etiologic agent was Malassezia furfur; two onychomycosis, one Corporal and one Tinea jock itch, isolated from cultures Trichophytum rubrum. In conclusion: of 144 people tested had no injuries attributed to the fungus Phymatotrichopsis omnivorous.

Gingival melanoacanthoma: case report and discussion of its clinical relevance.

Oral melanoacanthoma is an uncommon reactive condition that appears as a solitary pigmented lesion and is most often found on the buccal mucosa. For it to appear in the gingiva is extremely rare--only 11 cases are reported in the literature. This article presents a case of gingival melanoacanthoma and reviews the previously published cases. Because gingival melanoacanthoma shares clinical features with other pigmented lesions with diverse genesis, the differential diagnosis of a solitary pigmented lesion on the gingiva is also discussed.

Solitary fibrous tumor with entrapment of minor salivary gland tissue: an unusual presentation that requires exclusion of pleomorphic adenoma.

We report two unusual cases of solitary fibrous tumor (SFT) of minor salivary glands that microscopically mimicked pleomorphic adenoma. One of these lesions presented in the retromolar region and the other in the buccal mucosa. The microscopic features of these two tumors and their intimate relationship with regional mucous minor salivary glands posed a diagnostic challenge. Awareness of the morphological diversity of SFT coupled to a judicious use of appropriate immunohistochemical probes should prove valuable to accurately segregate SFT from pleomorphic adenoma.

Inflammatory response to dental polishing and prophylaxis materials in rats.

OBJECTIVE: To describe the tissue response to implanted polishing and prophylaxis materials using a rat model system. MATERIAL AND METHODS: Two polishing pastes (diamond polishing paste and aluminum polishing paste), two prophylaxis materials (prophylaxis paste with fluoride and air polishing prophylaxis powder) and negative and positive controls were subcutaneously implanted in rats. Tissue specimens obtained after 2 days, 1, 4, 6 and 8 weeks after implantation were processed for routine hematoxylin and eosin staining and polarized light evaluation. RESULTS: Air polishing prophylaxis powder produced a mild inflammatory response. A more intense inflammation was elicited by diamond polishing paste, and the prophylaxis paste with fluoride elicited an even greater response. The aluminum polishing paste produced the most severe and persistent tissue response, which was of the granulomatous type. CONCLUSIONS: This finding suggests that foreign body reaction should be considered in a gingivitis that does not respond to plaque control or does not represent a mucocutaneous lesion.

Estudio de cumplimiento del tratamiento antidepresivo / A study of compliance in the antidepressant treatment

Objetivo. Estudiar las variables asociadas al cumplimiento del tratamiento antidepresivo en un sector de la población peruana y comparar lo hallado con lo reportado en la lietratura mundial. Método. Se incluyeron 133 pacientes de consulta externa, vistos entre marzo y junio de 1999, correspondientes a los siguientes establecimientos de salud: Instituto Nacional de Salud Mental "Honorio Delgado-Hideyo Noguchi", Hospital Hipólito Unanue, Hospital María Auxiliadora y Clínica Ricardo Palma. Resultados. Las variables que se asociaron significativamente con cumplimiento del tratamiento fueron: psicoterapia complementaria (p<0.001), número de consultas (p<0.001), explicación clara por parte del médico de cómo debía tomar la medicación (p<0.001), creencia de que el medicamento ayuda o es beneficioso (p<0.001), repuesta al tratamiento (p=0.002), considerar que la medicación es necesaria para su tratamiento (p=0.003), buen trato por parte del médico (p=0.007), confianza en el médico tratante (p=0.01) cefalea (P=0.026) e insomnio (p=0.041); por otro lado, los factores que se asociaron significativamente al incumplimiento fueron: problemas para cubrir el costo de la medicación (p<0.001), temor a hacerse dependiente de la medicación (p=0.003), considerar que es suficinete tomar la medicación sólo hasta sentirse bien (p=0.044) y dolor abdominal (p=0.03). Los pacientes incumplidores consideraron que el costo de la medicación era la principal causa por la que habían dejado el tratamiento. Conclusiones. Las variables asociadas al cumplimiento del tratamiento son similares a las encontradas en otros estudios, destacando las relacionadas con la información que el paciente recibe, la relación médico-paciente y el costo del fármaco.

Multiple suppurative cystic lesions of the lips and buccal mucosa: a case of suppurative stomatitis glandularis.

Cheilitis glandularis (CG) is a rare inflammatory salivary gland disease that usually affects the lips. Although the etiology of CG is still unknown, it is believed to be a hereditary disease with an autosomal dominant pattern of inheritance. Three clinical presentations of CG are described in the literature: simple, superficial suppurative, and deep suppurative. A case of deep suppurative CG that extended to the buccal mucosa has been previously reported as suppurative stomatitis glandularis (SSG). Here we report a case of SSG in a 64-year-old white female with a history of bilateral renal transplants for adult polycystic kidney disease, who presented with painful swollen lips and bilateral buccal mucosal lesions. The diagnosis and management of the case is discussed. To the best of our knowledge, this is the second report of SSG, a rare condition affecting the minor salivary glands in the oral cavity.

A case of neurofibromatosis-Noonan syndrome with a central giant cell granuloma.

Neurofibromatosis Type 1 (NF1) is one of the most common autosomal dominant diseases affecting multiple systems including the vascular, skeletal, and central nervous system. Noonan syndrome (NS) is an autosomal dominant genetic disorder, associated with musculoskeletal and skin manifestations. Coexistence of central giant cell lesions in patients with both NS and NF1 were reported in the literature. Development of multiple central giant cell lesions in a patient with a Noonan syndrome has been referred to as Noonan-like syndrome. A few cases with features of NF1 and NS have been termed as NF1-NS. Here, we present a case of so-called NF-NS associated with a central giant cell lesion.

Terapia con surfactante exógeno en recién nacidos con enfermedad de membrana hialina / Exogenous surfactant therapy in newborns with hyaline membrane disease

Con el propósito de evaluar el impacto del empleo del surfactante exógeno (colfosceril palmitato con alcohol cetílico y tiloxapol para suspensión intratraqueal) en la letalidad y morbilidad de los recién nacidos con enfermedad de membrana hialina (EMH), se compararon 73 niños atendidos en tres unidades de neonatología de hospitales asociados con universidades de Santiago, Chile (grupo tratado, 1 de diciembre de 1990 a 30 de noviembre de 1991), cuyas medias de peso al nacer y edad gestacional fueron 1.471 g y 30,5 semanas, con 77 niños (grupo control 1 de diciembre de 1989 a 30 de noviembre de 1990) cuyas medias de peso al nacer y edad gestacional eran de 1.493 g y 30,7 semanas. Entre ambas muestras no había diferencias significativas poblacionales ni cambios sustanciales en las variables de tratamiento, normas de manejo e infraestructura de las unidades aistenciales. En el grupo tratado con surfactante artificial se registró disminución de significativa de letalidad (30,1 por ciento ante 57,1 por ciento; p=0,0009), especialmente entre los niños cuyo peso al nacer era entre 1.000 y 1.499 g (27,6 por ciento ante 76,7 por ciento; p=0,004); significativamente mayor proporción de sobrevivientes sin displasia broncopulmonar (53,4 por ciento ante 35,1 por ciento; p=0,03). Los promedios de los días en ventilación mecánica y oxigenoterapia fue similar entre ambos grupos, como tambien la incidencia de rotura alveolar. En morbilidad asociada, sólo se encontró mayor incidencia de apnea en el grupo tratado (21,9 por ciento ante 3,9 por ciento; p=0,0009). Se comprueba que el tratamiento de los recién nacidos con enfermedad de membrana hialina disminuye la letalidad y aumenta la proporción de sobrevivientes libres de displasia broncopulmonar

Carcinoma primario del hígado: apropósito de seis pacientes / Primary liver carcinoma: apropos of 6 cases

Se estudiaron ocho pacientes en quienes se sospechaba hepatocarcinoma, en un período de un año. En seis pacientes se confirmó el diagnóstico histológicamente. Fueron cuatro hombres y dos mujeres con edades entre 27 y 91 años (edad promedio 52 años). Se discuten las hipótesis actuales en la etiopatogenia, hallazgos clínicos y de laboratorio y las tendencias actuales del tratamiento en este tipo de neoplasias. Dos de los seis pacientes se consideraron fuera de tratamiento y fallecieron después del diagnóstico. Se operaron dos sujetos, en uno se realizó hepatectomía derecha y tuvo una sobrevida de un año, al otro se le practicó segmentectomía y laffeció en el postoperatorio inmediato, los dos restantes rechazaron el tratamiento

Sistema de vigilancia por computador del paciente recién nacido grave / Neonatal intensive care unit patient monitoring by computer

Se describe un sistema de vigilancia por computador de la unidad de neonatología del Hospital Clínico de la Universidad Católica, que permite vigilar 8 pacientes en cuidado intensivo, conectando los monitores electrónicos a un computador central de bajo costo, programado por los autores, con propósitos asistenciales y de investigación. Entrega información en pantalla de video y graba registros en "cassete". El sistema lee y calcula estadísticas de las variables vitales de los pacientes y diagnostica condiciones de emergencia: apneas, bradicardia, hipoxia, hiperoxia, hipotensión arterial y combinación de ellas. La validación clínica del sistema se hizo mediante 30 registros realizados en 22 pacientes. Se subraya la importancia del equipo multidisciplinario medicina-ingeniería para progresar en la investigación en el control de procesos de evaluación de las variables vitales de los pacientes en situación crítica. El desarrollo de sistemas y programas locales de computación aplicados a la clínica permite el progreso en el conocimiento tecnológico con cierta independencia de los elevados costos de materiales y programas importados

Falla renal aguda por sobredosis de indometacina en un recién nacido prematuro con un ductus arterioso persistente / Acute renal failure due to overdose of indomethacin in a preterm infant with patent ductus arteriosus

Un recién nacido prematuro con ductus arterioso persistente y signos de insuficiencia cardíaca fue tratado accidentalmente con una sobredosis de indometacina endovenosa. El soplo de ductus desapareció pero inmediatamente después el paciente sufrió oliguria severa acompañada de retención nitrogenada y reducción de la concentración sérica de sodio, sin embargo evolucionó favorablemente recuperándose en 7 días sin mostrar evidencia de secuelas a la edad de 21 días