Lunatomalacia infantil o enfermedad de Kienböck con conformación tipo VISI en un niño: Descripción de un caso y revisión de la literatura / Childhood Lunatomalacia Or Kienböck Disease With VISI Conformation In A Child: A Case Report And Literature Review

La osteonecrosis del semilunar o enfermedad de Kienböck es una entidad de baja prevalencia, más frecuente en varones jóvenes entre 20 y 40 años. Todavía más exclusiva en pacientes en edad infantil. De etiología variable, se baraja la teoría traumática vascular y la teoría no traumática causada por otros diversos mecanismos. La afectación del semilunar puede presentarse con edema óseo, fragmentación, desalineación escafo-lunar y progresar hacia el colapso. Presentamos el caso de un adolescente con enfermedad de Kienböck, sin antecedente traumático atribuible que desarrolló una lesión de compleja clasificación en la escala de Litchman. Se consiguió mejoría parcial de la clínica con tratamiento conservador, pero sin eliminar el dolor de forma definitiva. El planteamiento quirúrgico se debate actualmente teniendo en cuenta su madurez esquelética y evolución fisiopatológica de la lesión (AU)

Semilunar osteonecrosis or Kienböck's disease has a low prevalence, usually occurring in young men aged between 20 and 40 years. This disease is even less common in childhood. The aetiology varies, with postulation of the vascular trauma theory and the non-traumatic theory, in which the disease is caused by various other mechanisms. Semilunar involvement can occur with bone oedema, fragmentation, and scapholunate misalignment and progresses towards collapse. We present the case of an adolescent boy with Kienböck's disease, with no attributable traumatic antecedent, who developed a complex classification injury on the Litchman scale. Partial improvement of symptoms was achieved with conservative treatment but without definitive pain elimination. Future surgery for this patient is currently being discussed, bearing in mind his skeletal maturity and the pathophysiological progression of the injury (AU)

[Childhood Lunatomalacia Or Kienböck Disease With VISI Conformation In A Child: A Case Report And Literature Review]. / Lunatomalacia infantil o enfermedad de Kienböck con conformación tipo VISI en un niño: Descripción de un caso y revisión de la literatura.

Semilunar osteonecrosis or Kienböck's disease has a low prevalence, usually occurring in young men aged between 20 and 40 years. This disease is even less common in childhood. The aetiology varies, with postulation of the vascular trauma theory and the non-traumatic theory, in which the disease is caused by various other mechanisms. Semilunar involvement can occur with bone oedema, fragmentation, and scapholunate misalignment and progresses towards collapse. We present the case of an adolescent boy with Kienböck's disease, with no attributable traumatic antecedent, who developed a complex classification injury on the Litchman scale. Partial improvement of symptoms was achieved with conservative treatment but without definitive pain elimination. Future surgery for this patient is currently being discussed, bearing in mind his skeletal maturity and the pathophysiological progression of the injury.

Radiofrecuencia pulsada en el tratamiento de la epicondilitis / Pulsed radiofrequency in the treatment of epicondylitis

No disponible

Sedación paliativa en un Hospital Universitario: experiencia tras la puesta en marcha de un protocolo específico / Palliative sedation in a University Hospital: experience after introducing a specific protocol

Pacientes y métodos. Estudio descriptivo prospectivo del registro de sedación paliativa (SP) durante un año tras la puesta en marcha del protocolo de SP. Criterios de inclusión. Pacientes incluidos en el protocolo de SP del Hospital Universitario Virgen Macarena y para los que se había completado la «Hoja de registro de datos». Fuentes de información. Hojas de registro incluidas en el protocolo de SP como anexo n.° 5, incluidas como anexo 1 en este trabajo. Variables. Datos de filiación, datos relativos a la enfermedad de base, pronóstico, nivel de información. Síntoma refractario: tipo, tiempo de evolución y tratamientos aplicados. Participación del enfermo y/o sus representantes en la toma de decisiones. Tipo de sedación, continuidad, profundidad, fármacos utilizados, duración y resultados y reajuste del resto de medidas terapéuticas. Análisis estadístico. Se analizaron las variables mediante SPSS 14.0: para variables cualitativas se estudiaron frecuencias absolutas y proporciones. Para cuantitativas con distribución normal, medias y desviaciones típicas, y con distribución no normal, medianas y rangos. Resultados. En el periodo del estudio constatamos 90 casos de SP. Ello representa el 27,6% de los pacientes atendidos por el equipo de soporte de cuidados paliativos (ESCP) hospitalario, y el 7,03% de los enfermos fallecidos en el hospital. La edad media de los pacientes tratados con SP fue de 59,22 años y rango entre 40,86 y 77,58 años. Un 68,1% fueron hombres. Todos se encontraban en fase avanzada o terminal de su enfermedad, y el 49,5% en fase agónica. En el 90,4% de los casos la patología fundamental fue oncológica. Respecto a la indicación de SP, esta fue realizada de manera conjunta por el médico del ESCP y su médico responsable en 60 casos (66,6%); Los síntomas que motivaron la sedación fueron mayoritariamente: disnea, delirium y dolor. En 5 casos se consultó al Comité de Ética Asistencial. La duración media de la sedación fue de 134,02 h (5,5 días). En el 90% de los casos, la duración osciló entre 50 y 218,04 h (2-9 días). La profundidad de la SP, tras inducción, quedó registrada en el 88,8% de los casos. El consentimiento informado (CI) fue explícito en el 11,2%, otorgado antes de presentar síntomas refractarios y/o fase agónica. En el 88,8% de las sedaciones el CI fue por representación. Respecto a los fármacos empleados como opción inicial, fue midazolam en el 87,8%, y levomepromacina en el 10,2%. Hasta un 15% de sedaciones precisaron cambio o asociación de fármacos. Conclusión. 1) La aplicación de un protocolo de SP y la disponibilidad del ESCP hospitalario y el Comité de Ética asistencial favorecieron el uso seguro (correcto) de la SP en un hospital sin unidad de cuidados paliativos (UCP), y el registro homogéneo de datos para su análisis, con criterios de calidad similares a los que ofrece una UCP. 2) Hemos detectado áreas de mejora que se aplicarán en la segunda versión de nuestro protocolo(AU)

Patients and methods. A descriptive prospective study of palliative sedation (PS) records during a one year period after starting the PS protocol. Inclusion criteria. Patients included in the PS protocol and those who had completed the «data registry form». Data sources. Registry forms included in the PS protocol as Annex 5 (included as Annex 1 in this study). Variables. Personal data, data regarding basal disease, prognosis, level of information. Refractory symptoms: type, time of evolution and treatments employed. Involvement of the patient and/or representatives in decision making. Type of sedation, continuity, depth, employed, duration and results, and readjustment of other therapeutic measures. Statistical analysis. The SPSS 14.0 was used. For qualitative variables we studied absolute frequencies and proportions. For quantitative variables with normal distribution, we used means and standard deviations, and for non-normal distribution, medians and ranges. Results. In the study period 90 cases of PS were counted. This represented 27.6% of the patients treated in the hospital palliative care support team (PCST), and 7.03% of the total deceased patients in our hospital. Mean age of patients undergoing PS was 59.22 years old, range between 40.86 and 77.58 years, and 68.1% were men. All patients were in an advanced or terminal stage of their condition, and 49.5% in an agonal phase. In 90.4% of the cases, the main pathology was oncological. Regarding PS indication, this was jointly made between the PCST physician and the patient's usual doctor in 60 cases (66.6%). Symptoms leading to sedation were mainly dyspnea, delirium and pain. The Ethics committee was consulted in five cases. Mean time under sedation was 134.02 hours (5.5 days). In 90% of the cases, duration ranged from 50 to 218.04 hours (2-9 days). Depth of PS after induction was registered in 88.8% of the cases. Informed consent (IC) was explicit in 11.2% of the cases,and given prior to the appearance of refractory symptoms and/or agonal phase. In 88.8% of sedation cases, the IC was given by a representative. Midazolam was the most used drug of choice in 87.8% of the cases, and levomepromazine in 10.2%. Up to 15% of sedations required drug changes or combinations. Conclusion. 1) The application of a PS Protocol and the availability of a hospital PCST and an Ethical Care Committee favored a safe (correct) use of PS in a hospital lacking a palliative care unit (PCU), and a homogeneous data registry for their analysis, with quality criteria similar to those offered by a PCU. 2) We have detected areas of improvement that will be applied in the second version of our protocol(AU)

[Palliative sedation in a university hospital: experience after introducing a specific protocol]. / Sedación paliativa en un Hospital Universitario: experiencia tras la puesta en marcha de un protocolo específico.

PATIENTS AND METHODS: A descriptive prospective study of palliative sedation (PS) records during a one year period after starting the PS protocol. INCLUSION CRITERIA: Patients included in the PS protocol and those who had completed the "data registry form". DATA SOURCES: Registry forms included in the PS protocol as Annex 5 (included as Annex 1 in this study). VARIABLES: Personal data, data regarding basal disease, prognosis, level of information. Refractory symptoms: type, time of evolution and treatments employed. Involvement of the patient and/or representatives in decision making. Type of sedation, continuity, depth, employed, duration and results, and readjustment of other therapeutic measures. STATISTICAL ANALYSIS: The SPSS 14.0 was used. For qualitative variables we studied absolute frequencies and proportions. For quantitative variables with normal distribution, we used means and standard deviations, and for non-normal distribution, medians and ranges. RESULTS: In the study period 90 cases of PS were counted. This represented 27.6% of the patients treated in the hospital palliative care support team (PCST), and 7.03% of the total deceased patients in our hospital. Mean age of patients undergoing PS was 59.22 years old, range between 40.86 and 77.58 years, and 68.1% were men. All patients were in an advanced or terminal stage of their condition, and 49.5% in an agonal phase. In 90.4% of the cases, the main pathology was oncological. Regarding PS indication, this was jointly made between the PCST physician and the patient's usual doctor in 60 cases (66.6%). Symptoms leading to sedation were mainly dyspnea, delirium and pain. The Ethics committee was consulted in five cases. Mean time under sedation was 134.02 hours (5.5 days). In 90% of the cases, duration ranged from 50 to 218.04 hours (2-9 days). Depth of PS after induction was registered in 88.8% of the cases. Informed consent (IC) was explicit in 11.2% of the cases,and given prior to the appearance of refractory symptoms and/or agonal phase. In 88.8% of sedation cases, the IC was given by a representative. Midazolam was the most used drug of choice in 87.8% of the cases, and levomepromazine in 10.2%. Up to 15% of sedations required drug changes or combinations. CONCLUSION: 1) The application of a PS Protocol and the availability of a hospital PCST and an Ethical Care Committee favored a safe (correct) use of PS in a hospital lacking a palliative care unit (PCU), and a homogeneous data registry for their analysis, with quality criteria similar to those offered by a PCU. 2) We have detected areas of improvement that will be applied in the second version of our protocol.

Clinical experience of bendamustine treatment for non-Hodgkin lymphoma and chronic lymphocytic leukemia in Spain.

Bendamustine is a alkylating agent with a purine-like benzamidazole ring currently approved in Europe for indolent non-Hodgkin lymphoma (NHL), chronic lymphocytic leukemia (CLL) and multiple myeloma. Our aim was to analyze retrospectively the efficacy and toxicity of bendamustine in NHL and CLL in Spain in the bendamustine Compassionate Use Program. Patients with relapsed/refractory NHL or CLL were eligible. Any regimen containing bendamustine was eligible. 109 patients were included from 22 institutions. Forty-nine patients had indolent NHL, 18 aggressive NHL and 42 CLL, being 44 patients (40%) refractory to previous treatment. 63% of patients had adverse events grade 3-4, mainly hematological. Overall response rate (ORR) was 66%, complete responses 30%. ORR observed in refractory patients was 45%. The median progression-free survival (PFS) was 13 months. Outcome was influenced by histology, number of previous treatments, resistance to previous chemotherapy and type of response achieved with bendamustine. Alone or in combination, bendamustine shows a meaningful clinical antitumor activity in patients with relapsed or refractory NHL or CLL, with an acceptable toxicity profile.

Infusion of lymphocytes obtained from a donor immunised with the paraprotein idiotype as a treatment in a relapsed myeloma.

A 48-year-old patient with IgA k multiple myeloma received a BMT from his HLA-matched sibling. After transplantation, the disease relapsed. Melphalan therapy followed by reinfusion of haemopoietic blood stem cells collected from the patient led to the improvement of the clinical status, although mixed chimerism and an elevated serum IgA persisted. Successful donor immunisation against an immunogenic preparation of the recipient monoclonal protein was performed before the infusion of donor T lymphocytes (DLI) into the patient. Ten weeks after the lymphocyte infusions, no monoclonal band was evidenced and donor complete chimerism was detected. The patient did not develop GVHD. Once complete remission was achieved, the idiotype vaccine was administered to the patient. Nineteen months after DLI, the patient remains in remission. Bone Marrow Transplantation (2000).

Rhinocerebral mucormycosis following donor leukocyte infusion: successful treatment with liposomal amphotericin B and surgical debridement.

A 24-year-old male developed cytogenetic relapse of chronic myeloid leukemia (CML) four years after allogeneic BMT. After a year of treatment with IFN-alpha, he achieved a partial cytogenetic response. Treatment with donor leukocyte infusions (DLI) was given (total dose 1 x 10(8) T lymphocytes/kg). Two months later, he developed acute GVHD (skin and liver), that improved with CsA and methylprednisolone and resulted in cytogenetic remission with complete donor chimerism. One month later he developed rhinocerebral mucormycosis and was successfully treated with surgical debridement and liposomal amphotericin B (total dose 12 g). This is the first case of mucormycosis described after DLI.

Mucormycosis and hemopoietic transplants.

Mucormycosis is becoming recognized as a serious complication in patients undergoing hemopoietic transplantation (HT), because it is a major cause of morbidity and mortality. In our institution 4 cases of mucormycosis in post-HT period among 345 patients undergoing HT were diagnosed between 1984 and 1997. We studies the clinical characteristics of these cases and we conclude that mucormycosis is not a common infection in patients undergoing HT but that it is followed by a high morbidity and mortality. Maintained neutropenia is the most important risk factor.

Splenic peliosis with spontaneous splenic rupture in a patient with immune thrombocytopenia treated with danazol.

We present a 79-year-old man diagnosed with immune thrombocytopenia (ITP), treated with danazol, who died as a result of a spontaneously ruptured spleen. The histopathological diagnosis was splenic peliosis. This patient presents a chronological association between the treatment with danazol and the development of peliosis, which suggests a clear cause-effect relationship. Facing an individual patient with ITP, clinicians should weigh the potential benefits of danazol with the possible development of serious complications, such as hepatic failure or splenic rupture due to peliosis.

De novo smoldering paroxysmal nocturnal hemoglobinuria: a flow cytometric diagnosis.

An asymptomatic 26-year-old woman with mild macrocytic anemia (11.6 g Hb/dl) was studied. All biochemical parameters, bone marrow histology and cytogenetics were normal. The Ham's and sucrose tests were negative. A flow cytometric analysis revealed that CD55 and CD59 staining was absent in 20% and 21% of the granulocytes, but erythrocytes and CD34-positive bone marrow cells were CD55 and CD59 positive. Seven months after the initial study, the patient suffered an episode of hemoglobinuria, with mild anemia, moderate thrombocytopenia and a weak positive sucrose lysis test. A new flow cytometric analysis disclosed an increased percentage of CD55 and CD59 negative granulocytes and a 25% of erythrocytes with an intermediate pattern of fluorescence after CD59 labelling. At fourteen months, a population of CD55-deficient erythrocytes was detected and the Ham's test became positive. The present report is, to our knowledge, the first case of smoldering paroxysmal nocturnal hemoglobinuria in a patient with no previous aplastic anemia, or evident pancytopenia. The diagnosis was established by flow cytometry of peripheral blood granulocytes, with apparently phenotipically normal progenitor cells in an early stage of the disease. Flow cytometry appears to be a useful tool in our knowledge of paroxysmal nocturnal hemoglobinuria evolution.

[The influence of a prior appointment on primary care consultations in the Murcia region]. / Influencia de la cita previa en las consultas de atención primaria en la región de Murcia.

AIM: Analysis of how the prior appointment system affects various indicators of attendance. DESIGN: A multi-centered intervention study. SITE. Faculty of Medicine. Department of Social-Health Sciences. PATIENTS AND OTHER PARTICIPANTS: Patients seen in the general consulting-rooms of urban and rural Health Centres during the same months in two successive years, i.e. before and after setting up the prior appointment system. INTERVENTIONS: Introduction of the prior appointment programme (a consultation, with the hour and date arranged beforehand). Questionnaire given to the patient at the end of the consultation. MEASUREMENTS AND MAIN FINDINGS: The personal variations of age and sex, and also of those relating to the medical side were compared by means of the questionnaire, before and after the introduction of the prior appointment system. Significant differences were only seen in relation to hospital referral, which tripled; and to the number of prescriptions per person, which increased. CONCLUSIONS: The prior appointment system has meant less waiting-time for patients and a rationalisation of consultation time; but it has not substantially altered the content of the consultation.

[Celiac disease, autoimmune cirrhosis, chronic pancreatitis: apropos of a triple association]. / Maladie coeliaque, cirrhose auto-immune, pancréatite chronique: à propos d'une triple association.

We report the case of a 60 year old lady suffering from celiac disease and autoimmune cirrhosis who developed exocrine pancreatic insufficiency with canalicular lesions consistent with chronic pancreatitis. Celiac disease is known to be associated with either pancreatic insufficiency or liver disease, but association of all three diseases has not yet been described. We suggest that chronic pancreatitis be added to the list of idiopathic inflammatory pancreatitis of possible autoimmune origin, enabling to explain the pathophysiology of all three disorders with one hypothesis.

[Evaluation of protein catabolism in multi-injured patients]. / Evaluation du catabolisme protéique chez les polytraumatisés.

The urinary excretion of nitrogen was calculated, using the daily urinary urea nitrogen output in 19 patients with multiple trauma. The patients were followed for at least two weeks in an intensive care unit. The urinary excretion for each patient was expressed as the average of 7 day periods and the differences were evaluated with the Student's test for paired samples. Protein catabolism (defined as a urinary nitrogen excretion higher than 0.250 g per kilo and per day) was found in 18 of 19 patients during the first week, and in 16 during the second week. Catabolism was still present in 8 of 11 the third week and in 3 of 6 the fourth week after the trauma. Nitrogen excretion remained at the same level for two weeks and decreased significantly afterwards. Protein catabolism was an important contribution to weight loss. The cumulative loss of lean body mass revealed approximately 70% of weight loss over four weeks. The role of factors involved in catabolism and a therapeutic approach are discussed.