ABSTRACT
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Subject(s)
Humans , Female , Young Adult , Axilla/injuries , Fox-Fordyce Disease/diagnosis , Laser Therapy/adverse effects , Hair Removal/adverse effects , Tretinoin/therapeutic useABSTRACT
INTRODUCTION: Since detection of a series of cases of a new variant of Creutzfeldt Jakob disease (CJD) in the United Kingdom, prionic diseases are frequently mentioned by the media. The neurologists always consider them in daily clinical practice, although the incidence is relatively low (0.5-2 cases/1,000,000 inhabitants/year for CJD). In Spain, in recent years, we have seen an increased number of probable and possible cases, perhaps due to it always being included in the differential diagnosis, and to the greater use of diagnostic tests such as 14-3-3 protein in cerebrospinal fluid (CSF). There is also a reduction in the number of definite cases, probably due to the reticence to carrying out necropsies. In the differential diagnosis, one must remember structural, infectious, degenerative, toxic, vascular and metabolic alterations, including hepatic encephalopathy (HE). CLINICAL CASE: We report the case of a woman in whom CJD and EH co existed. This made diagnosis difficult. It was finally confirmed on necropsy.
Subject(s)
Creutzfeldt-Jakob Syndrome/complications , Creutzfeldt-Jakob Syndrome/pathology , Hepatic Encephalopathy/complications , Hepatic Encephalopathy/pathology , Aged , Autopsy , Electroencephalography , Fatal Outcome , Humans , MaleABSTRACT
La Linfadenitis Necrotizante Histiocitaria o Enfermedad de Kikuchi-Fujimoto es un proceso benigno del sistema linfático caracterizado por linfadenopatías y que puede acompañarse de síndrome febril prolongado, rash cutáneo, alteraciones hematológicas leves como anemia o trombopenia. Suele aparecer en personas de edad joven, generalmente mujeres. Aunque inicialmente se había descrito en países orientales, en los últimos años se han publicado varios casos en Europa. En este trabajo incluimos los datos de seis pacientes que fueron estudiados en nuestro Hospital entre los años 1986 y 1996. Los seis pacientes estaban siendo estudiados por la presencia de fiebre y tumefacciones cervicales. La prueba diagnóstica en todos ellos fue la realización de una biopsia de una de las adenopatías. Ésta mostraba en todos los casos la histología característica de la enfermedad, que consiste en la observación de focos de necrosis dispersos por el ganglio, además de la presencia de histiocitos atípicos y células plasmocitoides. También es un dato característico en esta enfermedad la ausencia de polimorfonucleares neutrófilos. El diagnóstico de la Linfadenitis Necrotizante Histiocitaria es, por tanto, anatomo-patológico. En cuanto a la evolución y pronóstico, es un proceso autolimitado, benigno, que hasta la fecha no se ha relacionado con la aparición posterior de procesos linfoproliferativos, infecciosos u otros. El tratamiento de esta entidad, cuando es necesario, es puramente sintomático. En nuestros pacientes la evolución fue a la remisión completa de los síntomas, sin que ninguno de los pacientes presentaran recaídas posteriores. Con este trabajo pretendemos llamar la atención sobre esta entidad benigna en todos aquellos pacientes que se presentan con adenopatías y fiebre (AU)
Subject(s)
Adolescent , Adult , Female , Male , Humans , Biopsy , Lymph Nodes/pathology , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/pathologyABSTRACT
OBJECTIVES: Neuropenic enterocolitis (NEC) is a destructive lesion of the ileocecal region occurring in cancer patients treated with chemotherapy. Its clinical picture is one of febrile acute abdominal extension with bloody diarrhea and low neutrophil counts. Our aim was to determine the incidence of NEC in children with cancer and to review the indications of surgery in these cases. MATERIAL AND METHODS: The records of children with cancer treated with chemotherapy in the last 6 years at Hospital Infantile La Paz were reviewed. We selected those patients who had abdominal pain and neutropenia and whose physical examination and radiological findings were consistent with NEC. RESULTS: Twelve cases of NEC were diagnosed during this period among 432 malignancies. The symptoms most frequently seen were abdominal pain and distension, nausea and vomiting. The neutrophil count was consistently below 500/ml. All patients were receiving chemotherapy before the onset of the clinical picture. Five children were operated upon. In three of these we found various ileocecal perforations, in one a gastric perforation and in the remaining one a diffuse inflammation of the ileocecal area. Two non-operated patients died from NEC. The remaining children recovered without problems with medical therapy. CONCLUSION: Pediatric surgeons treating neutropenic cancer patients should be familiar with this condition, that must be suspected early in granulocytopenic patients with acute abdominal extension. Aggressive surgical management is indicated in cases with severe peritonitis, bowel perforation or massive lower gastrointestinal bleeding, irrespective of the degree of neutropenia. Medical support should aim at reestablishing normal neutrophil counts.
