RESUMO
BACKGROUND. Factors such as environment, income status, as well as access to proper healthcare influence the survival and quality of life of people affected by chronic diseases including cystic fibrosis (CF). Survival factors in Mexican patients with CF have not been reported before, even when it has been estimated that this disease could not be negligible in the Mexican population. OBJECTIVE. To compare the influence of the mutant allele ΔF508 and environmental factors on the survival of Mexican CF patients. MATERIAL AND METHODS. We collected epidemiological data of 40 patients molecularly tested between 1987 and 2008 in the Clínica de Fibrosis Quística from the Hospital Universitario of the Universidad Autónoma de Nuevo León in Northeastern México. Kaplan-Meier plots and survival statistics were estimated and compared. RESULTS. Survival analysis revealed statistical significance for low-income status (p = 3.13 x 10-6), cor pulmonale (p = 0.00169), severe pulmonary disease (p = 0.00136), and BMI ≤15 kg/m2 (p = 0.00678). Statistical significance was not observed for the predominant allele ΔF508 considering two (p = 0.992), one (p = 0.503) or no (p = 0.403) mutant allele. CONCLUSIONS. Low income status was the most detrimental factor; followed by cor pulmonale, severe pulmonary disease and BMI ≤ 15 kg/m2 for the survival in North East Mexican patients with CF. Carrying the ΔF508 allele did not influence survival.
Assuntos
Fibrose Cística/mortalidade , Renda , Adolescente , Adulto , Índice de Massa Corporal , Criança , Pré-Escolar , Fibrose Cística/genética , Genótipo , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , México/epidemiologia , Doença Cardiopulmonar/mortalidade , Fatores de Risco , Adulto JovemRESUMO
STUDY DESIGN: Retrospective chart and complementary study review. OBJECTIVES: To describe the features of adult patients with spinal deformity and respiratory failure and to analyze the results of surgical treatment. SUMMARY OF BACKGROUND DATA: Many authors have studied the relation between spinal deformities and cardiorespiratory failure, but there exists little information about the benefits of reconstructive surgery in severely compromised patients. METHODS: The charts and complementary studies of 35 adult patients surgically treated between January 1, 1978, and December 31, 1994, were reviewed. The patients were 18 years old or older (average age, 36 years). They had spinal deformity of any etiology with respiratory insufficiency as evidenced by vital capacity of less than 60% of predictive normal, PaO2 less than 80 mm Hg, or PaCO2 more than 45 mm Hg. All had reconstructive spinal surgery in an attempt to improve their respiratory problem. RESULTS: Seven patients died within the first postoperative year, and one patient was lost to follow-up at 6 months. The other 27 patients had a mean follow-up time of 72 months. The 34 patients were divided into three groups: good, fair, and poor evolution. The patients in the good evolution group had a better preoperative general condition, had more correction of their deformities, had more improvement in their respiratory function, and had fewer complications than those in the other groups. The patients in the poor evolution group were older, had more cardiac problems, and had less correction at surgery. CONCLUSION: The results of surgery varied from extremely good to extremely bad. The seven patients who died within the first year had no benefit, but the 27 others did very well, usually gaining significant improvement of their respiratory function. Because the alternative to surgical correction is death, this study shows that, under the right circumstances, correction of spinal deformity and, therefore, correction of respiratory function can be life-saving.
Assuntos
Doença Cardiopulmonar/etiologia , Insuficiência Respiratória/etiologia , Curvaturas da Coluna Vertebral/complicações , Curvaturas da Coluna Vertebral/cirurgia , Adulto , Fixadores Externos , Feminino , Seguimentos , Humanos , Masculino , Doença Cardiopulmonar/mortalidade , Doença Cardiopulmonar/prevenção & controle , Testes de Função Respiratória , Insuficiência Respiratória/mortalidade , Insuficiência Respiratória/prevenção & controle , Estudos Retrospectivos , Curvaturas da Coluna Vertebral/terapia , Fatores de Tempo , Tração/instrumentação , Resultado do TratamentoRESUMO
Se practicó cateterismo cardíaco a 83 niños carentes de cardiopatía intrínseca, que desarrollaron cor pulmonale secundario. La presión pulmonar media registrada fue de cuando menos el doble de la normal. Se identificaron las siguientes anormalidades anatómicas: a) Alteraciones de las estructuras torácicas. b) Obstrucción de vías aéreas. c) Anormalidades de los vasos arteriales pulmonares. d) Alteraciones del parénquima pulmonar. Los padecimientos asociados fueron muy diversos: tumor intratorácico, parálisis diafragmática, hipertrofia de amígdalas y de adenoides, enfermedad por reflujo gastroesofágico, colangiopatías, neumonías de diferente causa, displasia broncopulmonar, cuerpo extraño en vías aéreas, laringomalacia, talasemia, síndromes de Kartagener, Pierre Robin, Hurler, anillo vascular y otros. Se analizan los mecanismos que en estos casos llevaron a hipoxemia, hipertensión pulmonar o a ambas, y que culminaron en sobrecarga ventricular derecha, con insuficiencia cardíaca en muchos y con la muerte en otros. Si bien el cor pulmonale puede ser reversible cuando su evolución es corta, en ocasiones tiene un curso fatal cuando tales mecanismos han operado intensamente y por tiempo prolongado.