Síndrome de embolia grasa tras fractura ósea / Fat embolism syndrome after bone fractures

Objetivos. Revisar la incidencia, las características clínicas, diagnósticas y terapéuticas, así como la mortalidad del síndrome de embolia grasa (SEG) en la última década. Pacientes y métodos. Estudio retrospectivo y descriptivo de los pacientes diagnosticados de SEG postraumático entre enero de 2001 y diciembre de 2011 en un solo centro. Resultados. Se evalúan 19 pacientes, 16 varones y 3 mujeres, con edad media de 27 años. Todos presentaban fracturas de huesos largos como consecuencia de un politraumatismo, múltiple en el 78,9%. La clínica respiratoria fue la más frecuente (89,5%), seguida de la neurológica (68,4%) y del exantema petequial (63,2%). El tiempo medio de presentación desde el ingreso fue de 42h. En todos se realizó estabilización precoz de la fractura previa al episodio embólico. En ningún caso se utilizaron corticoides profilácticos. El tratamiento quirúrgico definitivo tuvo una demora media de 7 días y la estancia media hospitalaria fue de 34 días. La incidencia de SEG fue de 0,14% y la mortalidad del 10,5%. Conclusiones. El SEG postraumático afectó fundamentalmente a pacientes jóvenes, politraumatizados, con fracturas de huesos largos. Presentaron manifestaciones de la tríada clínica clásica (respiratorias, neurológicas, exantema), tras un periodo asintomático inicial de menos de 2 días. Su incidencia global fue baja(AU)

Objectives. To review the incidence, clinical features, diagnosis, therapy and mortality rates of fat embolism syndrome (FES) in a tertiary referral hospital in the last decade. Patients and methods. Retrospective and descriptive study of patients diagnosed with post-traumatic FES between january 2001 and december 2011. Results. A total of 19 patients, 16 men and 3 women, with an average age of 27 years were evaluated. All had long bone fractures, multiple in 78.9%, as a result of multiple injuries. Respiratory symptoms were the most frequent (89.5%), followed by neurological symptoms (68.4%) and petechial rash (63.2%). The average time of presentation of the syndrome after admission was 42 hours. All patients underwent early stabilisation of the fracture prior to the embolic event. Steroids prophylaxis was not used in any of the cases. Definitive surgical treatment had mean delay of 7 days. The mean hospital stay was 34 days. The overall incidence of FES was 0.14%, and mortality was 10.5%. Conclusions. Post-traumatic FES mainly affected young patients with multiple injuries and long bone fractures. They all had symptoms of the classic clinical triad (respiratory, neurological, rash) after an initial asymptomatic period of less than 2 days. The overall incidence was low(AU)

[Fat embolism syndrome after bone fractures]. / Síndrome de embolia grasa tras fractura ósea.

OBJECTIVES: To review the incidence, clinical features, diagnosis, therapy and mortality rates of fat embolism syndrome (FES) in a tertiary referral hospital in the last decade. PATIENTS AND METHODS: Retrospective and descriptive study of patients diagnosed with post-traumatic FES between january 2001 and december 2011. RESULTS: A total of 19 patients, 16 men and 3 women, with an average age of 27 years were evaluated. All had long bone fractures, multiple in 78.9%, as a result of multiple injuries. Respiratory symptoms were the most frequent (89.5%), followed by neurological symptoms (68.4%) and petechial rash (63.2%). The average time of presentation of the syndrome after admission was 42 hours. All patients underwent early stabilisation of the fracture prior to the embolic event. Steroids prophylaxis was not used in any of the cases. Definitive surgical treatment had mean delay of 7 days. The mean hospital stay was 34 days. The overall incidence of FES was 0.14%, and mortality was 10.5%. CONCLUSIONS: Post-traumatic FES mainly affected young patients with multiple injuries and long bone fractures. They all had symptoms of the classic clinical triad (respiratory, neurological, rash) after an initial asymptomatic period of less than 2 days. The overall incidence was low.

Fístulas mandibulares en una mujer de 75 años / Jaw fistulas in 75 years-old woman

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[Skeletal manifestations of Gauchers disease. A report of two cases]. / Manifestaciones óseas de la enfermedad de Gaucher. A propósito de dos casos.

Gauchers disease is the most frequent hereditary lysosomal deposit storage disorder. It is characterized by a deficiency of the enzyme glucocerebrosidase that leads to an accumulation of substrate in the interior of the macrophage lysosomes. It is classified in three types, according to the presence of central nervous system involvement (type 2 and 3) or not (type 1). It is a multisystemic disease and in the majority of patients there is hepatosplenomegaly, anemia and thrombocytopenia. Skeletal involvement is also important and it is frequently the most disabling manifestation. We present two cases of Gauchers with skeletal manifestations and we review the literature.

Manifestaciones óseas de la enfermedad de Gaucher. A propósito de dos casos / Skeletal manifestations of Gaucher´s disease. A report of two cases

La enfermedad de Gaucher es la enfermedad hereditaria de depósito lisosomal más frecuente. Se caracteriza por una deficiencia de la enzima glucocerebrosidasa que conduce a la acumulación de substrato en el interior de los lisosomas de los macrófagos. Se clasifica en tres tipos según exista afectación de sistema nervioso central (tipos 2 y 3) o no (tipo 1).Es una patología multisistémica y en la mayoría de pacientes se aprecia hepatoesplenomegalia, anemia y trombopenia. La afectación esquelética también es importante y a menudo constituye el aspecto más discapacitante. Presentamos dos casos de enfermedad de Gaucher con manifestaciones óseas y realizamos una revisión de la literatura (AU)

Dorsalgia en varón de 35 años / Back pain in a 35-year old male

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La enfermedad de Rendu-Osler-Weber. Estudio descriptivo de 17 casos / Rendu-Osler-Weber disease. Descriptive study of 17 cases

Objetivo. Estudiar la prevalencia y formas de presentación de la telangiectasia hemorrágica hereditaria (THH) en la población de nuestro ámbito hospitalario.Material y métodos. Se realizó un estudio descriptivo retrospectivo de los pacientes diagnosticados de THH entre los años 1972 y 1999 en el Hospital La Fe de Valencia. Se valoró la presencia de antecedentes familiares, epistaxis de repetición, telangiectasias cutaneomucosas y lesiones viscerales, así como los tratamientos administrados y la evolución de la enfermedad. Resultados. Se diagnosticaron 17 pacientes en dicho período con edades comprendidas entre los 24 y los 80 años. Ocho pacientes presentaban antecedentes familiares. Los síntomas más frecuentes fueron la epistaxis y la anemia ferropénica, seguidas en frecuencia por las telangiectasias cutaneomucosas. Seis pacientes presentaron manifestaciones neurológicas, cinco pulmonares, cinco gastrointestinales y tres pacientes hepáticas, generalmente secundarias a malformaciones vasculares a esos niveles.Conclusiones. La prevalencia de la enfermedad puede ser más elevada de lo que había sido descrito anteriormente. Ante pacientes con epistaxis de repetición y/o anemias ferropénicas hay que tener en cuenta esta enfermedad y buscar la presencia de telangiectasias que puedan orientar al diagnóstico. El diagnóstico precoz de la THH puede ayudar al control temprano de malformaciones viscerales asociadas. El tratamiento de la THH de ser individualizado según las manifestaciones clínicas del paciente (AU)

Hipertiroidismo e hipertensión pulmonar

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[Adult celiac disease: study of 21 cases and review of the bibliography]. / Enfermedad celíaca del adulto: estudio de 21 casos y revisión de la bibliografía.

Celiac sprue is a chronic disease characterized by a typical lesion in the small intestine and nutrient malabsorption that improves on withdrawal of dietary gluten. Adult celiac disease (or gluten enteropathy) is a common and under-diagnosed disease since presentation varies among a) symptomatic or clinically active forms (including mono- or oligosymptomatic forms), b) asymptomatic, subclinical or silent forms, and c) latent forms. We present a descriptive study of a retrospective series of patients diagnosed with symptomatic or clinically active adult celiac disease who required hospitalization between January 1993 and June 2000. The form of symptomatic expression, biological alterations, investigations that confirm the diagnosis, associated diseases, complications and response to treatment are described.

[Rendu-Osler-Weber disease. Descriptive study of 17 cases]. / La enfermedad de Rendu-Osler-Weber. Estudio descriptivo de 17 casos.

OBJECTIVE: To study the prevalence and presentation forms of hereditary hemorrhagic telangiectasia (HHT) among the population in our hospital area. MATERIALS AND METHODS: Descriptive, retrospective study of patients with the diagnosis of HHT from to 1999 at La Fe Hospital, Valencia. Family history, repeated epistaxis, mucocutaneous telangiectasis and visceral lesions, as well as administered treatments, and clinical course were evaluated. RESULTS: Seventeen patients were diagnosed during such time period, with ages ranging from 23 to 80 years. Eight patients had family histories. The most common symptoms included epistaxis and iron-deficiency anemia, followed by muco-cutaneous telangiectasis. Six patients had neurological, five pulmonary, five gastrointestinal, and three hepatic manifestations, usually secondary to vascular malformations at those levels. CONCLUSIONS: The prevalence of the disease maybe higher than previously reported. This disease should be considered in patients with repeated epistaxis and/or iron-deficiency anemia. The presence of telangiectasis that can suggest the diagnosis should be investigated. An early diagnosis of HHT can be useful for the early control of associated visceral malformations. Treatment of HHT should be tailored according to the clinical manifestations of the patient.

[Association of multiple myeloma and solid neoplasms: analysis of 13 cases]. / Asociación de mieloma múltiple y neoplasias sólidas: análisis de trece casos.

BACKGROUND: The diagnosis of second malignancies in patients with multiple myeloma is uncommon. It is debatable whether this tumor is in itself a risk factor for the incidence of second malignancies. Etiopathogenic factors which might account for this association were analyzed. MATERIALS AND METHODS: Retrospective analysis of a series of 210 patients with myeloma controlled by a Medical Oncology Department from 1984 to 1998. After searching for the diagnosis of a second malignancy, thirteen patients were identified with both diagnoses. A descriptive statistical study was elaborated as well as an analysis of survival. RESULTS: There were seven males and six females, with a mean age at diagnosis of 69 years (57-80). The Durie-Salmon stages at diagnosis were: I-A (3), I-B (1), II-A (4), III-A (4). Associated solid malignancies included: hepatocarcinoma (2), prostate adenocarcinoma (2), soft tissue sarcoma (2), lung adenocarcinoma (1), cholangiocarcinoma (1), breast carcinoma (1), endometrial carcinoma (2) and bladder carcinoma (1). At diagnosis, seven of the solid malignancies were metastatic. At the time of this analysis, eleven patients had died, eight because of progression of the solid cancer, two because of progression of myeloma and one because of therapy toxicity. Two patients are still alive with stability of the myeloma and solid malignancy controlled after surgical resection. CONCLUSIONS: The association was observed in 6.2% of patients with myeloma and occurs at an advanced age. IgG myelomas and in early stages predominated. Solid malignancies were diagnosed in advanced stages in most cases. A short term high mortality rate was observed due to progression of the solid malignancy. The frequency of the association did not seem to be higher than the overall incidence of second malignancies in patients with cancer.

Oestrogen and progesterone receptors in the human vagina during the menstrual cycle, pregnancy and postmenopause.

Oestrogen receptor (ER) and progesterone receptor (PR) concentrations were measured by enzyme immunoassays in human vaginal tissues. There were no significant differences in either case between the follicular and luteal phases, or in ER concentrations between regularly cycling and postmenopausal women. ER levels were significantly lower in pregnant than in non-pregnant women. PR levels were significantly lower in both postmenopausal and pregnant women than in regularly cycling women. A positive correlation between ER and PR was found in pregnant women and there were correlations between ER and both oestradiol and prolactin in postmenopausal women. This is the first study to compare both ER and PR in the human vagina during the menstrual cycle, pregnancy and postmenopause.