RESUMEN
Sickle cell disease is the most common inherited disorder in African-Americans. Although the primary defect is hematological, the changes in the erythrocytes lead to a vasculopathy with multiorgan injury. The pulmonary complications, i.e., acute chest syndrome and chronic sickle cell lung disease, are significant causes of morbidity and mortality. The pulmonary manifestations result from a unique constellation of factors which come into play in sickle cell disease. Based on the growing understanding of the molecular and cellular biology of sickle cell disease, new therapies are being developed that are likely to ameliorate the natural history of this disease and its complications.
Asunto(s)
Anemia de Células Falciformes/fisiopatología , Enfermedades Pulmonares/etiología , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/terapia , Embolia Grasa/etiología , Eritrocitos Anormales/fisiología , Hemoglobina Falciforme/química , Hemoglobina Falciforme/fisiología , Humanos , Enfermedades Pulmonares/terapiaRESUMEN
BACKGROUND: There is controversy over whether colonization with drug-resistant organisms is a contraindication to lung transplantation. METHODS: We undertook a retrospective review of the results of lung transplantation for patients with cystic fibrosis (CF) at Duke University Medical Center. RESULTS: As of May 1996, 21 patients with CF underwent bilateral lung transplantation. The first patient died within 24 h of transplantation from sepsis due to Stenotrophomonas maltophilia. Of the remaining 20 patients, 17 (85%) are alive and in stable condition. The three deaths were related primarily to bronchiolitis obliterans at 4 and 18 months in two patients and to cytomegalovirus pneumonitis at 5 months in the other patient. The 17 surviving patients have been followed up for a mean of 13 months (range, 0.5 to 34 months). Most of them were colonized and infected with multidrug-resistant organisms before transplantation. Following transplantation, 11 patients had complications from infections. One patient had bacteremia due to a panresistant Burkholderia cepacia and was treated successfully. Two patients had bacteremia and wound infection due to Burkholderia gladioli, previously thought to be pathogenic only in plants. Both patients were treated successfully. Of the six patients with Aspergillus fumigatus isolated from cultures before transplantation, only one had invasive disease following transplantation and responded to treatment. CONCLUSION: The organisms present before transplantation were not the primary cause of mortality in our patient population. Our findings suggest that lung transplantation should be considered in CF patients infected with multidrug-resistant organisms.
Asunto(s)
Fibrosis Quística/cirugía , Enfermedades Pulmonares/microbiología , Trasplante de Pulmón , Adolescente , Adulto , Aspergilosis/microbiología , Aspergillus fumigatus , Bacteriemia/microbiología , Bronquiolitis Obliterante/etiología , Burkholderia , Infecciones por Burkholderia/microbiología , Burkholderia cepacia , Causas de Muerte , Niño , Contraindicaciones , Fibrosis Quística/microbiología , Infecciones por Citomegalovirus/etiología , Farmacorresistencia Microbiana , Resistencia a Múltiples Medicamentos , Femenino , Estudios de Seguimiento , Rechazo de Injerto/etiología , Infecciones por Bacterias Gramnegativas/microbiología , Humanos , Enfermedades Pulmonares/virología , Trasplante de Pulmón/efectos adversos , Masculino , Neumonía Viral/etiología , Estudios Retrospectivos , Sepsis/microbiología , Infección de la Herida Quirúrgica/etiología , Tasa de Supervivencia , Xanthomonas/efectos de los fármacosRESUMEN
Extracorporeal membrane oxygenation (ECMO) is widely used in the treatment of respiratory and cardiovascular failure in neonatal patients. The authors present a case of a child with hemoglobin SS disease who was treated with ECMO after acute chest syndrome and acute respiratory distress syndrome developed. They also present data from the Extracorporeal Life Support Organization on this use of ECMO from other centers. To date, there have been 15 pediatric patients with acute chest syndrome treated with ECMO. Survival rate has been 26%. In selected patients with severe disease, ECMO can provide support at a lower mean airway pressure, allow for aggressive pulmonary lavage, and maintain adequate tissue oxygen delivery until the patient is more stable. Patients who might benefit include those with poor ventilation secondary to mucous plugging and barotrauma. The best success with these patients might be anticipated from venoarterial ECMO. Patients with severe cardiac or neurologic deterioration may constitute a group less likely to survive.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Oxigenación por Membrana Extracorpórea , Síndrome de Dificultad Respiratoria/terapia , Insuficiencia Respiratoria/terapia , Población Negra , Preescolar , Humanos , Masculino , Consumo de Oxígeno/fisiología , Radiografía Torácica , Síndrome de Dificultad Respiratoria/complicaciones , Insuficiencia Respiratoria/complicaciones , SíndromeRESUMEN
Infants with inborn errors of fatty acid metabolism may present with apnea, periodic breathing, and sudden infant death syndrome (SIDS). Recognition of these disorders and initiation of appropriate therapy may prevent SIDS. Metabolic pathways develop during gestation and post-natally. We report three siblings with apnea and periodic breathing, as well as biochemical defects consistent with a non-specific abnormality of beta oxidation. One infant died a witnessed sudden infant death. The two survivors were treated with L-carnitine supplementation resulting in rapid resolution of both respiratory and metabolic abnormalities. Enzyme activity for short, medium, and long chain acyl coenzyme A dehydrogenases was normal in these two infants. Although a unique enzymatic deficiency was not identified, our experience with this family supports the need for routine biochemical evaluation of infants with "near miss" SIDS, also called acute life-threatening events (ALTE), as well as those who have died of SIDS.
Asunto(s)
Apnea/complicaciones , Apnea/genética , Ácidos Grasos/metabolismo , Errores Innatos del Metabolismo Lipídico/complicaciones , Errores Innatos del Metabolismo Lipídico/genética , Apnea/metabolismo , Carnitina/metabolismo , Carnitina/uso terapéutico , Enfermedades en Gemelos , Femenino , Humanos , Recién Nacido , Errores Innatos del Metabolismo Lipídico/terapia , Muerte Súbita del Lactante/genéticaRESUMEN
A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography and confirmed by lung biopsy. Superior mesenteric arteriogram revealed hypoplasia of the intrahepatic portal vein branches and a portosystemic shunt. The possible etiologies of PAVMs are discussed.
Asunto(s)
Malformaciones Arteriovenosas/diagnóstico , Cardiopatías Congénitas/diagnóstico , Arteria Pulmonar/anomalías , Bazo/anomalías , Malformaciones Arteriovenosas/patología , Biopsia , Preescolar , Femenino , Cardiopatías Congénitas/patología , Humanos , Hígado/patología , Pulmón/patología , Arteria Pulmonar/patología , SíndromeRESUMEN
A familial lymphoproliferative disorder presented in three male siblings with primary pulmonary involvement manifested as either lymphoid interstitial pneumonia or an angiodestructive polymorphous infiltrate morphologically resembling lymphomatoid granulomatosis. The polymorphous infiltrate consisted chiefly of mature T-cells with a few B-cells and plasma cells, and gene rearrangement studies failed to show clonality. Epstein-Barr virus, frequently associated with proliferative lesions in males in the X-linked lymphoproliferative syndrome, was not demonstrated in any of the pulmonary lesions. An HLA haplotype shared among the affected siblings was A1, B8, DR4. The unusual clinical presentation plus the lack of involvement by EBV in the pulmonary lesions suggests that this is a previously undescribed familial lymphoproliferative disorder.
Asunto(s)
Enfermedades Pulmonares/complicaciones , Trastornos Linfoproliferativos/genética , Adolescente , Anticuerpos Antivirales/análisis , Niño , Femenino , Antígenos HLA/análisis , Herpesvirus Humano 4/inmunología , Humanos , Mononucleosis Infecciosa/complicaciones , Pulmón/diagnóstico por imagen , Pulmón/patología , Enfermedades Pulmonares/diagnóstico por imagen , Enfermedades Pulmonares/patología , Granulomatosis Linfomatoide/complicaciones , Granulomatosis Linfomatoide/diagnóstico por imagen , Granulomatosis Linfomatoide/patología , Trastornos Linfoproliferativos/complicaciones , Trastornos Linfoproliferativos/inmunología , Trastornos Linfoproliferativos/microbiología , Masculino , Fibrosis Pulmonar/complicaciones , Fibrosis Pulmonar/diagnóstico por imagen , Fibrosis Pulmonar/patología , RadiografíaRESUMEN
Preparation of an experimental emollient wax-gelled ointment base by two processes differing only in cooling rate produced material with markedly different physical properties. Differential scanning calorimetry showed that a major endotherm, possibly related to a phase change in a major triglyceride wax component, Synchrowax HGLC, was different in the two products. Mean enthalpies for this major endotherm for the two products were 7.36 J g-1 (s.d. = 0.49, n = 5) in slow cooled samples and 4.35 J g-1 (s.d. = 0.21, n = 5) in fast cooled samples. The degree of order of the Synchrowax HGLC in the ointment is suggested as being different in the two preparations and it is this that controls the physical properties of the ointment.
Asunto(s)
Rastreo Diferencial de Calorimetría/métodos , Química Farmacéutica , Bases Oleosas/química , Química Farmacéutica/métodos , Aceite Mineral/síntesis química , Propilenglicol , Glicoles de Propileno/química , Aceites de Silicona/químicaRESUMEN
Bedside measurement of respiratory frequency is commonly performed in a cursory manner and judged to be of little clinical importance. However, in a recent study of patients being weaned from mechanical ventilation, we found that tachypnea was quite accurate in predicting an unsuccessful weaning outcome. The present study was undertaken to examine the relationship between nonobtrusive measurements of respiratory frequency, using a calibrated inductive plethysmograph, and detailed measurements of pulmonary function in 11 adult patients with cystic fibrosis of varying severity. Respiratory frequency was increased in the patients with cystic fibrosis compared with a group of healthy control subjects, as was minute ventilation and mean inspiratory flow. Respiratory frequency was a sensitive predictor of respiratory dysfunction, being significantly (p less than 0.05) correlated with airway obstruction (r = 0.76), hyperinflation (r = 0.52), arterial oxygenation (r = -0.59), rib cage-abdominal discoordination (r = 0.54), and maximum ventilation during exercise (r = 0.66). Despite the presence of tachypnea, the patients did not display shallow breathing; indeed, tidal volume was not correlated with any of the above abnormalities. In conclusion, respiratory rate was a useful indicator of respiratory dysfunction in this group of patients with cystic fibrosis.
Asunto(s)
Fibrosis Quística/fisiopatología , Trastornos Respiratorios/diagnóstico , Respiración/fisiología , Adulto , Fibrosis Quística/complicaciones , Prueba de Esfuerzo , Femenino , Humanos , Masculino , Pletismografía , Trastornos Respiratorios/etiología , Pruebas de Función Respiratoria , EspirometríaRESUMEN
Photographic evidence of the process of proliposome hydration is provided together with comprehensive particle size analysis of both hydrated dimyristoylphosphatidylcholine: dimyristoylphosphatidylglycerol:ergosterol:amphotericin B proliposomes and egg lecithin:ergosterol:amphotericin B proliposomes using photon correlation spectroscopy and Coulter Counter analysis. Proliposome particle size and the temperature during hydration have been shown to have little effect on subsequent liposome size. A short term stability study of proliposomes indicated that only minor changes in the size distribution profile of the hydrated product are apparent after storage at 20 degrees C for 9 months. Furthermore, no drop in amphotericin B potency was noticed over a 6-month period.