RESUMO
We report on two sisters affected by congenital alopecia, nail dystrophy, and a severe T-cell immunodeficiency, presumably inherited as an autosomal-recessive disorder. The T-cell defect was characterized by severe functional impairment, as shown by the lack of proliferative response and upregulation of activation markers following mitogen stimulation. The functional abnormality occurred in spite of the presence of phenotypically mature of the defect. This is the first observation reported on an ectodermal disorder, characterized by alopecia and nail dystrophy, observed at birth, in association with a primary immunodeficiency. The hypothesis that these two events may be casually related is discussed.
Assuntos
Alopecia/congênito , Síndromes de Imunodeficiência/complicações , Doenças da Unha/complicações , Doenças da Unha/imunologia , Imunodeficiência Combinada Severa/complicações , Linfócitos T/patologia , Alopecia/complicações , Alopecia/imunologia , Linfócitos B/patologia , Transplante de Medula Óssea , Complexo CD3 , Feminino , Humanos , Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/terapia , Lactente , Recém-Nascido , Células Matadoras Naturais/patologia , Doenças da Unha/genética , Unhas/patologia , Gravidez , Imunodeficiência Combinada Severa/genética , Imunodeficiência Combinada Severa/imunologiaRESUMO
The purpose of this study was to compare hospital charges for carotid endarterectomy on a surgeon-specific basis. The cost of carotid endarterectomy is influenced by preoperative evaluation, operating time, use of the intensive care unit, length of hospital stay, and surgical results. Length of stay and average hospital charges for 18 doctors performing 344 carotid endarterectomies at three hospitals were analyzed. Outcome data were also reviewed. The results demonstrated a wide variation in hospital charges among surgeons. Surgeons using the most cost-effective measures achieved comparable or superior outcomes. In an era of managed care, severe cost constraints mandate that surgeons perform similar studies in their own communities so that cost-effective clinical pathways can be developed. With the use of appropriate guidelines, carotid endarterectomy can be performed at relatively low cost without sacrificing quality.
Assuntos
Endarterectomia das Carótidas/economia , Preços Hospitalares , Hospitais Comunitários/economia , California , Análise Custo-Benefício , Humanos , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Persistent sciatic artery (PSA) is a rare congenital malformation that is complicated by aneurysm formation in more than 25% of the reported cases. Two cases of aneurysm of the PSA are presented. Twenty-six aneurysms of the PSA, including our two cases, have been reported in the English literature in the last three decades. Early surgical treatment is warranted to decrease the 25% amputation rate associated with thromboembolic complications. The posterior, transgluteal repair of this aneurysm affords excellent exposure and avoids a long bypass graft, multiple incisions, and staged procedures. Magnetic resonance imaging may be helpful in preoperative evaluation of the feasibility of proximal, extrapelvic vascular control.
Assuntos
Aneurisma/cirurgia , Artérias/anormalidades , Idoso , Aneurisma/diagnóstico , Aneurisma/etiologia , Angiografia , Artérias/cirurgia , Nádegas/irrigação sanguínea , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Nervo Isquiático/irrigação sanguíneaRESUMO
The efficacy of carotid endarterectomy in preventing stroke is clearly related to appropriate patient selection and low surgical morbidity and mortality. It has been suggested that since results at some centers are better than nationwide statistics, perhaps the operation should be limited to those institutions. In this paper we present an experience with carotid endarterectomy over the past twelve years. These 566 consecutive cases were performed by two vascular surgeons in a large metropolitan area using thirteen different hospitals ranging from 150 to 500 beds. Our mortality of 0.5% and permanent stroke incidence of 1.6% did not vary significantly from hospital to hospital. Where the results of surgical audits were available from the individual hospitals, the overall complication rates were significantly higher. We conclude that individual surgeons, not institutions, determine the efficacy of carotid endarterectomy in community practice.