Xantomatosis cerebrotendinosa: Reporte de un caso en el Perú: Evaluación clínica integral y estudios de neuroimágenes / Cerebrotendinous Xanthomatosis: Report of a case in Peru: Comprehensive clinical evaluation and neuroimaging studies

Se presenta el caso de una paciente de 73 años de edad que, a los 30 años aproximadamente, comenzó a quejarse de dolor al caminar, localizando la molestia a nivel de las regiones aquilianas, con subsecuente aumento de volumen; al paso del tiempo, estas molestias la obligaron a efectuar consulta médica. Los análisis de laboratorio mostraron severa dislipidemia mixta. Al lado de información de significativa declinación cognitiva, provista por familiares (vgr., (i.e., olvidos frecuentes, desorientación, atención disminuida, concentración pobre), hubo evidencia de ánimo fluctuante, labilidad emocional, crisis ansiosas evolucionando hacia ataques de pánico. El test minicognitivo de Folstein, mostró severo estado demencial y en el examen neurológico se constataron ataxia cerebelosa y signos de piramidalismo parcial. El examen oftalmológico puso en evidencia xantelasmas, cataratas y un denso arco senil. El estudio del cerebro con resonancia magnética (RM) mostró el daño encefálico y signos sugestivos de depósitos del colastenol en el SNC. La presencia de xantomas , los hallazgos oftalmológicos, la demencia definidamente progresiva y la ataxia cerebelosa fueron hallazgos clínicos que permitieron establecer el diagnóstico de xantomatosis cerebrotendinosa.

The case of a 73 years-old woman that, since approximately the age of 30 years started to complain of pain when walking, is presented. The symptom was mainly located in the acchillean regions which, as time advanced, showed gradual volume increase and, finally, forced her to seek medical evaluation. Accompanying relatives reported a several years’ history of gradually increasing cognitive difficulties (i.e., forgetfulness, disorientation, poor attention and concentration), fluctuating mood (from periods of good humor switching to sudden episodes of sadness and crying spells), emotional lability and anxiety crises evolving into brief panic attacks. The Mini-cognitive Fenton Test confirmed severe dementia and the neurological evaluation showed cerebellar ataxia and partial pyramidalism. The ophthalmological examination revealed xanthelasmas, cataracts and dense arcus senilis. Xanthomas were detected in the Achillean tendons of both lower extremities. Auxiliary laboratory and densitometric tests demonstrated mixed dyslipidemia and dorsal-lumbar osteoporosis, respectively, and magnetic resonance imaging of the brain (RMC) confirmed SNC damage and suggested deposits of cholestenol, thus confirming the diagnosis of Cerebroitendinous Xanthomatosis.

Familial tularaemia.

Tularaemia is a zoonotic disease caused by Francisella tularensis . In this report, we have presented an early stage case of tularemia with fever and pharyngitis and two cases from the same non-endemic region with typical lymphadenitis. All three patients were treated with non-specific medications in healthcare centres, the treatment being directed towards symptoms resembling those of upper respiratory tract infections. However, there was no regression in their complaints. Because the first case had been treated earlier, his lymphadenopaties regressed and there was no suppuration. The other two cases, which had been suspected to be exposed to the same pathogen based on their histories, were at a mild acute phase and presented to our clinic with typical lymphadenitis. The diagnoses of each of the three patients were made serologically. An early clinical recovery was achieved in the first patient with streptomycin (1 x 1 g/day im) and doxycyline (2 x 100 mg/day peroral) therapy. The therapy was prolonged to 4 weeks in the other two cases according to lymph node response and no complications occurring in their follow-ups. It can be concluded that tularaemia should be considered in the differential diagnosis of patients with fever, pharyngitis, conjunctivitis and cervical lymphadenopathies that do not respond to β -lactam antibiotics.

Nosocomial urinary tract infections: micro-organisms, antibiotic sensitivities and risk factors / Infecciones nosocomiales del tracto urinario: microorganismos, sensibilidades antibióticas y factores de riesgo

OBJECTIVE: Although urinary tract infections (UTIs) are the most common hospital-acquired infections, the epidemiology of these UTIs is not well defined in Turkey. The aim of this surveillance study was to determine micro-organisms responsible for UTI, their antibiotic sensitivities and to describe the incidence and risk factors of nosocomial urinary tract infections (NUTI). SUBJECTS AND METHODS: This was a prospective surveillance study including cases of NUTI in intensive care units and various inpatient clinics. This study was carried out between November 2000 and January 2002. The following information was recorded: patients' age, gender, type of infection (hospital-acquired), presence of urinary catheter, intensive care unit admission, duration of hospital stay, type of organisms isolated and their antimicrobial susceptibility. The diagnosis of NUTI was based on criteria established by the Centers for Disease Control, Atlanta. Mini Api and conventional culture methods were used to determine the causative agents. The agents were isolated on eosin methylene blue agar and 5sheep blood agar Statistical analyses of data were by chi-square test and logistic regression. RESULTS: In this study, 618 (2.1) nosocomial infections (NIs) were determined in 29,778 patients, and 178 of these infections were NUTI (28.8, 178/618). The mean age of NUTI patients was 61.0 +/- 19.4 years (0-91 years) and 82 NUTI patients (46.1) were male and 96 (53.9) were female. The most frequently isolated micro-organism was Escherichia coli (31.4) followed by Candida spp (21.3), Klebsiella spp (10.6) and Enterococcus spp (6.9). Compared to the rate of other NIs, the rate of NUTI increased by 1.011 times per year of age, by 2.052 times in females and by 3.83 times in patients with urinary catheters (p < 0.05). The most effective antibiotics against Gram-negative bacteria were found to be imipenem and meropenem. CONCLUSIONS: Important factors to prevent NUTI are to avoid unneces...

OBJETIVO: Aunque las infecciones del tracto urinario (ITU) son las que con mayor frecuencia se adquieren en los hospitales, su epidemiología no se halla bien definida en Turquía. El objetivo de este estudio de vigilancia fue determinar los microorganismos responsables de la ITU, sus sensibilidades antibióticas, y describir la incidencia y los factores de riesgo de las infecciones del tracto urinario nosocomiales (ITUN). SUJEITOS Y MÉTODOS: Este fue un estudio prospectivo de vigilancia que incluyó casos de ITUN en unidades de cuidados intensivos y en varias clínicas de ingreso. El estudio se llevó a cabo entre noviembre de 2000 y enero de 2002. Se registró la siguiente información: edad del paciente, sexo, tipo de infección (adquirida en el hospital), presencia de catéter urinario, ingreso a la unidad de cuidados intensivos, duración de la estadía hospitalaria, y el tipo de organismo aislado así como su susceptibilidad antimicrobiana. El diagnóstico de ITUN se basó en criterios establecidos por los Centros de Control de Enfermedades, Atlanta. El sistema Mini Api y métodos de cultivo convencionales fueron usados con el propósito de determinar los agentes causantes. Los agentes causantes fueron aislados sobre agar-eosina-azul de metileno y agar sangre de oveja a 5%. El análisis estadístico de los datos se realizó usando la prueba de chi-cuadrado y regresión logística. RESULTS: En este estudio, se determinaron 618 (2.1%) infecciones nosocomiales (IN) en 29 778 pacien-tes, y 178 de estas infecciones resultaron ser INTU (28.8%, 178/618). La edad media de los pacientes de INTU fue 61.0 B1 19.4 años (0–91 años) y 82 pacientes de INTU (46.1%) fueron varones y 96 (53.9%) fueron hembras. El micro-organismo más frecuentemente aislado fue Escherichia coli (31.4%), seguido por Candida spp (21.3%), Klebsiella spp (10.6%) y Enterococcus spp (6.9%). En comparación con la tasa de otras IN, la tasa de INTU aumentó 1.011 veces por año de edad, 2.052 veces en las...

Factores genéticos y ambientales asociados a la hendidura labio-palatina / Factors genetic and ambient assofactors with cleft-palate

El trabajo tiene como objetivo determinar los factores genéticos y ambientales de riesgo en niños tratados con hendidura labio palatina en el Instituto Autonómo Hospital Universitario de Los Andes del Estado Mérida. La población está conformada por los pacientes que asistieron a la consulta entre 1 mes y 5 años de edad, durante el período de un mes, donde asistieron 70 pacientes con la malformación, tomándose como muestra el 50 por ciento (35 pacientes al azar). Los resultados demostraron que el 48,5 por ciento era producto de primigesta, procedían del Distrito Sanitario El Vigía y los Estados limítrofes (22,8 por ciento) donde practican actividades agrícolas y manipulan en forma inadecuada los productos químicos (fertilizantes, insecticidas, plaguicidas), un 62,7 por ciento por parte de los padres y con un alto consumo de alcohol (74,3 por ciento); la administración de vitaminas (37,1 por ciento), ácido fólico (20 por ciento) y de hierro (11,4 por ciento) fue al final de la gestación; sólo un 28,9 por ciento tenía antecedentes familiares, en el 97 por ciento no había parentesco de los progenitores, el 85 por ciento de las madres estaban dentro del parámetro de edad reproductiva y el 57 por ciento sufrieron situaciones de angustia durante el embarazo. En vista que el factor ambiental fue significativo es importante dirigir la Atención Primaria de Salud orientada a la población desde el punto de vista preventivo, disminuyendo los factores de riesgo de adquirir la malformación

Bifid scrotum, perineal hamartoma and high imperforate anus: a case report

This is a case report of a newborn patient with imperforate anus, urethro-colonic fistula, perianal hamartoma, and bifid scrotum. Successful staged repair of these anomalies is described together with review of the embriology related to the case

Solid and papillary neoplasm of the pancreas: a case presentation

Solid and papillary tumors of the pancreas are very rare malignancies, more commonly occurring in young women. They usually present as asymptomatic, large abdominal masses, and different from the most common neoplasm of the pancreas, which is the adenocarcinoma, these tumors have a high percentage of curability when treated by complete surgical resection. (6) For this reason, when the diagnosis of Frantz's tumor is made or strongly suspected, every attempt should be made for complete surgical excision since curability is high and radiotherapy and, or chemotherapy are of no use for its treatment

Anorectal sigmoid myotomy with ileal anastomosis after subtotal colectomy due to Hirschsprung's enterocolitis: case report and review of the literature

We presented a one year old seven months old patients that had undergone a double barreled proximal ileostomy-sigmoidostomy after resection of most of the ileum, ascending, transverse and descending colon due to Hirschsprung's enterocolitis; that was admitted for anorectal-sigmoid myotomy and ileosigmoidostomy. The advantages of Kasai's procedure (anorectal myotomy with coloanal anastomosis) over the pull-through procedures to treat Hirschsprung's disease are discussed along with the more pertinent literature

Transduodenal sphincteroplasty--how we do it

Transduodenal Sphincteroplasty can be performed in a simple reproducible and low morbidity fashion. The surgical technique used by the Department of Surgery of the Mayagüez Medical Center is presented

Characterization of an aggregated polymer from Penicillium sp. (PB-73 strain)

1. We describe a polymeric of magnesium ammonium phosphate associated with linoleic acid and a basic protein produced by Penicillium sp (strain PB-73). 2. Chemical and physical data suggest a structure characterized as an aggregated polymeric form of protein magnesium ammonium phospholinoleate anhydride. 3. The aggregated polymer has an approximate molecular weight of 316 kDa and a phosphate/magnesium ratio of 2.34

Comparison of the antiviral activity and toxicity of a protein magnesium ammonium phospholinoleate anhydride polymer with other antiviral drugs

1. SB-73, a magnesium ammonium phospholinoleate anhydride aggregate, exhibited antiviral action in vitro in the concentration range of 50 to 100 µg/ml against herpes simplex type 1, stomatitis vesicular virus, adenovirus type 5, and in vivo in the dose range of 0.7 to 1.3 mg/Kg against canine parvovirus distemper virus. 2. The lethal dose (LD50) was 2.71 ñ 1.55 g/Kg body weight in mice inoculated intraperitoneally. Oral ingestion of the aggregate up to 30 g/Kg body weight by mice had no lethal effects during the 14 days of observation. 3. In in vitro cytotoxicity experiments with fibroblasts (V-79 Chinese hamster cell line), no toxic effects were observed with SB-73 concentrations (120 µg/ml) having antiviral activity. 4. In a cellular proliferation experimental using hamster V-79 cells, we observed 72% proliferation after treatment of the cells with a high concentration (500 µg/ml) of SB-73. 5. Compound SB-73 showed no genotoxicity for human lymphocytes at concentrations of 100 µg/ml. 6. When the cytoxicity and genotoxicity of SB-73 wee compared with those of acyclovir, idoxuridine and AZT at 500µg/ml concentration the compound was found to have effects similar to those of acyclovir

Effect of growth conditions on production of violacein by Chromobacterium violaceum (BB-78 strain)

1. Chromobacterium violaceum (strain BB-78 isolated in Brazil) produces violacein, a substance potentially useful in phototherapy and with antibiotic and trypanocide activity. Culture conditions were optimized for the production of violacein and change in nutrients, temperature and pØ were correlated to cellular growth. 2. Methionine was the only absolute requirement for growth. alanine, arginine, tryptophan and vitamin B12 and its metabolites were important as a carbon source for violacein production and bacterial growth. 3. The highest yields of violacein were obtained by incubation in liquid medium and in the obsence of light at pH 7.0 and 28-C for 24 h

Some new aspects enzymatic lignin biodegradation

The present review focuses on some aspects of enzymology and on the ligninase mechanism in particular, with a survey of genetic studies carried out during the last few years. Some white-rot fungi are currently known to the most efficient lignin degrading organisms. Among them, Chrysonilia sitophila is emphasized for its high ligninolytic activity. Ligninases are enzymes which need H2O2 for activity and have intrinsic peroxidase activity. A free radical mechanism for ligninase-catalyzed degradation in suggested