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Biomedical subjects

R Lama

Publications and source records attributed to R Lama.

At least 19 recordsLinked to original sources
PubMed2006

Evaluation of patients referred for lung transplantation: fourteen years experience.

We present a descriptive study of patients referred as candidates for lung transplantation in the last 14 years. The 837 requests were evaluated stepwise in three phases: phase I, derivation report; phase II, outpatient evaluation; and phase III, inpatient evaluation. Chronic obstructive pulmonary disease was the most common reason for referral (31%). Cystic fibrosis was the referral disease with the best transplanted/referred relation (57%) and pulmonary fibrosis was the disease that had the highest mortality (39.7% of all deaths). Forty-three percent of all patients reached phase III and 29% were transplanted. Mortality on the waiting list was 3.7%. The most important causes of exclusion were inadequate indications and the presence of severe associated diseases. The mean study was 44 days. Knowledge of the natural history, local factors that influence organ availability, expected time on the waiting list, and disease progression allow optimization of this therapeutic option.

Graft Rejection↗
PubMed2005

Long-term results of lung transplantation for emphysema.

INTRODUCTION: We sought to assess the differences between lung transplantation (LT) to treat emphysema, versus other pulmonary diseases. METHODS: This retrospective review of lung transplantations (LTs) performed from October 1993 to September 2003, included donor and recipient demographic data, pulmonary function, oxygenation, postoperative complications, incidence of rejection and pneumonia, and survival. RESULTS: Of 159 LTs performed the 39 transplanted to treat emphysema (24.4%), were in 33 men and 6 women of age 50.9 +/- 8.7 years (men, 25 to 65 years). There were differences between the emphysema vs other groups in terms of age (P < .001), gender (P = .001), need for bypass (P = .004), and immediate posttransplantation oxygenation index (P = .001). Perioperative mortality tended to be lower among patients with emphysema (2.7% vs 10.8%; P = .131). The incidences of complications and acute rejections was similar. Forced vital capacity, forced expiratory volume in 1 second, arterial oxygen tension, and arterial carbon dioxide tension improved significantly post-Tx. Actuarial survivals were 82%, 68%, and 63%, respectively, at 1, 3, and 7 years posttransplantation for emphysema patients vs 60%, 53%, and 42%, respectively, at 1, 3, and 7 years posttransplantation for non-emphysema patients (P = .049). CONCLUSION: Lung transplantation in patients with emphysema offers good long-term survival, with significant improvement in functional status and low morbidity. The older age of emphysema patients was not associated with a higher incidence of postoperative complications.

Adult↗
PubMed2005

Pediatric lung transplantation.

INTRODUCTION: Pediatric lung transplantation (LT) was started in Spain in 1996 at our institution. We compare the results of pediatric LT with those in adult patients. METHODS: A retrospective review of LT patients from 1993 to 2003 included demographic donor and recipient data, pulmonary function, gas exchange parameters, complications, episodes of rejection and pneumonia, as well as survival. Patients were divided into 2 groups: pediatric (<16 years) and adult (>16 years) LT patients. RESULTS: Of 165 LTs performed, 23 recipients were pediatric patients (10 boys, 13 girls; mean age, 11.9 +/- 2.9 years [range, 5-16 years]). The indications were cystic fibrosis (n = 21), pulmonary fibrosis (n = 1), and Kartagener syndrome (n = 1). The actuarial survival rate was 73%, 67%, and 62% at 1, 3, and 8 years post-LT in children, versus 67%, 56%, and 41% at 1, 3, and 8 years post-LT in adult patients (P = NS). Of the pediatric patients, 35% required mechanical ventilation preoperatively (P < .001). Pediatric patients showed a higher incidence of pneumonia (P < .01) and acute rejection episodes (P = .02) during the first month post-LT, and longer stays in the intensive care unit (P = .02). Pediatric patients displayed more immunosuppression-related adverse effects: diabetes (P = .04), neuropathy (P < .01), and hirsutism (P < .001). In children, arterial oxygen tension improved, from 51 mm Hg pre-LT to 93 mm Hg at 5 years post-LT. Forced expiratory volume in 1 second improved from 28% pre-LT to 84% at 5 years post-LT. CONCLUSION: In children, LT is a high-risk procedure because of the critical status of these patients. However, the results of pediatric LT are similar to those in adults, but with better long-term survival.

Adolescent↗
PubMed2005

Analysis of lung transplant recipients surviving beyond 5 years.

INTRODUCTION: We started lung transplantation (LT) in October 1993 and review the status of recipients who have survived beyond 5 years. METHODS: A retrospective review of patients undergoing LT from October 1993 to October 1998 included pulmonary function data, incidence of bronchiolitis obliterans syndrome (BOS), functional status, and survival. RESULTS: Of 73 transplantations 41 (56%) patients have survived beyond 5 years (study group), including 23 men and 18 women of age 33.2 +/- 15.6 years. Indications for LT were as follows: cystic fibrosis (n = 16), emphysema (n = 13), pulmonary fibrosis (n = 8), and other (n = 4). Actuarial survival at 5, 7, and 9 years was 56%, 53%, and 43%, respectively. Freedom from BOS was 63%, 56%, and 50% at 5, 7, and 9 years, respectively. The median percent predicted FEV1 was 67%, 56%, and 56%, respectively. Also, 79% of recipients had no limitations in their daily activities; 65% were active and working. Only 5% of patients showed some degree of limitation at 5 years posttransplantation. When survivors beyond 5 years were compared with nonsurvivors beyond 5 years, differences were observed: nonsurvivors more frequently required bypass (P = .01), experienced longer postoperative intubation times (P = .01), and exhibited lower PaO2 at 12 months posttransplantation (P < .01). CONCLUSION: Our data show good survival rates among patients surviving beyond 5 years after LT, with a moderate incidence of BOS at 9 years posttransplantation. Despite the incidence of BOS, these patients have good pulmonary function and activity status.

Actuarial Analysis↗
PubMed2005

Respiratory disorders and quality of sleep in patients on the waiting list for lung transplantation.

BACKGROUND: The purpose of this study was to assess the quality of sleep and extent of respiratory disorders in patients awaiting lung transplantation as compared with a control group. METHODS: From September 2003 to November 2003, 17 clinically stable patients on the waiting list for lung transplantation and 14 healthy controls (with similar age, gender, and body mass index) were studied. Diagnostic polysomnography (PSG) was carried out for all subjects. RESULTS: Seventeen patients were included, 15 men and 2 women, aged 51 +/- 14 years. The indication for lung transplantation was emphysema in 7 cases, pulmonary fibrosis in 6, and "other" in 4. Patients awaiting lung transplantation had the following respiratory values: mean FEV1, 1105 mL (34% of predicted); PaO2, 54 mm Hg; and PaCO2, 44 mm Hg. Significant differences were found among the waiting-list patients in terms of predominance of light sleep, wakeful periods, and phase changes per sleep-hour, as compared with the control group. The recording of the respiratory events showed an apnea-hypopnea index of 6.13, sleeping time with SaO2 <90% of 1.80%, and a mean number of significant desaturations (<4%) of 6.38. There were no statistically significant differences with respect to the control group. CONCLUSIONS: Poor quality of sleep was observed in patients awaiting lung transplantation as compared with a healthy control group. There was no evidence of more respiratory events or significant desaturations in these patients, probably due to the provision of supplementary oxygen therapy during the PSG.

Emphysema↗
PubMed2005

Pulmonary tailoring and lobar transplantation to overcome size disparities in lung transplantation.

Size matching between donors and recipients represents one of the organ distribution criteria widely accepted by lung transplant teams. However, in some cases it is not possible to allocate a donor to the corresponding size-compatible recipient. To avoid possible complications derived from the implantation of oversized lungs into smaller recipients, surgical procedures such as pulmonary tailoring and lobar transplantation have been advocated. We review our experience in 13 patients undergoing volume reduction of the lung graft at the time of transplantation, either by nonanatomical lung volume reduction or by lobar transplantation. There were no significant differences between lung-downsized patients and standard lung transplantation patients in terms of donor characteristics, surgical and postoperative complications, functional outcome, and survival. We conclude that downsizing the lung graft either by nonanatomical resection or lobar transplantation is safe and reliable to overcome size disparities between donor and recipients, with no additional morbidity and with similar early and midterm outcomes to those in standard lung transplants.

ABO Blood-Group System↗
PubMed2005

Urgent lung transplantation in Spain.

BACKGROUND: Lung donors are scarce and lung transplantation resources are limited. Because urgent lung transplantation (ULT) is assumed to yield poor results, its use is controversial. We assessed the outcome of patients who received ULT seeking to determine effectiveness and risk factors. PATIENTS AND METHOD: We collected data from every ULT performed in Spain during 5 years (1998-2002). The survival of patients was studied using Kaplan-Meier, Cox regression, and chi-square statistical analyses. We compared outcomes and perioperative mortality (over 30 days) for ULT procedures, analyzing the influence of certain variables (age, type of transplant, diagnosis, indication, and time on waiting list). RESULTS: Among 109 patients proposed for the procedure, 73 ULT were performed during the period. The most frequent indications were pulmonary fibrosis (19 cases) and cystic fibrosis (19 cases), showing the worst and the better survival rates, respectively. The bad prognosis, determined mainly by per operative mortality rate (35.62%), was significantly affected by age (worse for patients older than 40 years) and type of LT (single worse than double; P < .05). A longer time waiting for ULT also showed a worse prognosis (P < .005). CONCLUSIONS: Long-term survival after ULT shows that the procedure is effective and efficient for a select group of patients, despite the high per operative risk. ULT should be reserved for younger patients. It also requires performance in a short period (just a few days), initially rejecting a single lung transplant, provided that the patient is adequately monitored.

Humans↗
PubMed2004

Allergy, asthma and markers of infections among Albanian migrants to Southern Italy.

BACKGROUND: Studies of immigrants represent an useful tool to determine the relative relevance of environmental vs genetic factors in causing the reported rapid increase of the prevalence of sensitization and allergic diseases. METHODS: A total of 152 Albanian migrants to Southern Italy responded to a questionnaire based on the European Community Respiratory Health Survey (ECRHS) and 139 of them underwent skin prick test, and 61 serological assays for total IgE and IgG antibodies against Toxoplasma gondii (TG), herpes simplex virus 1 (HSV-1), hepatitis A virus (HAV) and Helicobacter pylori (HP). RESULTS: Reported asthma was rare (2/152; 1.3%) and reported nasal allergies rather frequent (24/152; 15.8%). Sensitization to common inhalant allergens occurred in 27/139 (19.4%) subjects. The frequency of skin sensitization to pollen (P = 0.003) and that of hay fever (P = 0.004) increased with the time spent in Apulia. All the 61 sera had antibodies against HAV, 59/61 (96.7%) against HSV-1, 48/61 (78.7%) against HP and 34/61 (55.7%) against TG. The prevalence of skin sensitization and hay fever symptoms were correlated to the duration of residence in Southern Italy. CONCLUSIONS: Data presented indicate that Albanian migrants to Italy, in spite of the low prevalence of allergic diseases and sensitization in their country of origin, manifest with time an increasing prevalence of sensitization to local allergens and nasal symptoms after immigration to Italy. This would suggest a permanent role of allergen exposure and lifestyle factors in influencing the appearance of sensitization and symptoms of allergic diseases.

Albania↗
PubMed2003

Lung transplants with tacrolimus and mycophenolate mofetil: a review.

Traditionally, immunosuppressive maintenance therapy in solid organ transplantation has consisted of cyclosporine (CsA), azathioprine, and prednisone. However, lung transplant recipients are far more frequently affected by acute rejection, especially during the first 6 months after the transplantation, than patients with other transplanted organs. Further, they display a greater risk for chronic transplant dysfunction and ultimate graft loss. Bronchiolitis obliterans syndrome (BOS) is the major cause of morbidity and mortality among long-term survivors after lung transplantation. Acute pulmonary allograft rejection has been identified as the major risk factor for the development of BOS. Based on favourable results in kidney, liver, and heart transplantation, tacrolimus and mycophenolate mofetil have been used as primary prophylaxis and as rescue therapy for recurrent or persistent acute rejection and BOS. A secondary indication is CsA toxicity. This review focuses on reported results of the combination of tacrolimus and mycophenolate mofetil in lung transplantation. These new immunosuppressive drugs have markedly improved the efficacy profiles without additional detrimental toxicities, and appear to be a safe alternative to CsA and azathioprine in patients following lung transplantation. However, at present, BOS is not influenced by these new drugs. The optimal long-term immunosuppressive regimen remains to be established.

Clinical Trials as Topic↗
PubMed2003

[Activity of a pediatric intestinal transplantation program in Spain].

AIM: To analyze the outcome of children with intestinal failure (IF) included as candidates for intestinal transplantation (IT). Patients, Eight out of 23 children with IF assessed since July 1997 met criteria for IT and were included on the waiting list. The causes of IT were Short Bowel Syndrome (SBS) in 6 and Microvillus Inclusion Disease (MID) in 2. The indication of IT were end stage liver disease (ESLD) in 5 (related to total parenteral nutrition administration, TPN), progressive hepatic fibrosis in 2 and loss of venous access in 1. The patients with ESLD were included for combined liver-small bowel transplantation (LSBT) and the remaining for isolated intestinal transplantation (IIT). RESULTS: Two children died waiting for LSBT, 4 patients are on the waiting list, 2 for LSBT and 2 for IIT (length of stay: 4-11 months). Two children were transplanted, one with IIT in a 2.5 years old boy with MID, and one with LSBT in a 22 months girl with SBS and ESLD. Both patients recovered intestinal function after transplantation and are a live (follow-up of 19 and 10 months respectively). The LSBT's patient is under treatment for postransplant lymphoproliferative disease (PTLD). CONCLUSIONS: The lack of suitable donors for the small children candidate to IT explains the long period of stay on the waiting list and the high pretransplant mortality. Two strategies are possible; early referral of children with IF to a transplant center and surgical techniques like ex vivi-hepatic reductions of the LSB graft.

Child↗
PubMed2001

Airway complications after lung transplantation: a review of 151 anastomoses.

OBJECTIVE: To analyze the incidence, treatment and follow up of airway complications after lung transplantation. METHODS: From October 1993 to April 2000, 104 lung transplants were performed in 101 patients. One hundred and fifty one bronchial anastomoses at risk were included in the study (29 single lung and 61 sequential double lung). Donor lungs were flushed both antegradely and retrogradely with Eurocollins. In the recipients, either a single or a sequential bilateral lung transplantation was performed when indicated. The bronchial anastomosis was telescoped and covered with peribronchial tissue in all cases. Postoperative fiberoptic bronchoscopic examinations were dictated by clinical grounds. Recipient variables were recorded and analyzed to assess possible differences between both complicated and non-complicated groups. RESULTS: Eight bronchial anastomotic complications (5.3%) occurred in six patients (6.8%). All complicated cases developed in sequential bilateral lung recipients (P=0.08): stenosis (n=5), granulation tissue (n=2), and bronchial dehiscence (n=1). Treatment consisted of lobectomy and subsequent completion pneumonectomy in one patient, rigid bronchoscopy dilation in two, balloon bronchodilation in two, laser debridement and stenting in one, and conservative therapy in two cases. One patient with severe sepsis and bronchial dehiscence died on day +30. The rest of the patients remain well so far. Airway complications were related to longer intubation periods (P<0.01). Other perioperative donor and recipient factors including the incidence of infections and acute rejection episodes, and actuarial survival, did not differ between groups. CONCLUSION: In our experience, the incidence of airway complications after lung transplantation is 5.3%. The careful surgical technique and organ preservation, the close surveillance of rejection and infection, and early postoperative extubation might play a role in reducing this incidence. Either surgical therapy or bronchoscopic dilation and stenting methods may contribute to resolve these complications.

Adolescent↗
PubMed2000

Potential relationship between genotype and clinical outcome in propionic acidaemia patients.

Propionic acidaemia (PA) is an autosomal recessive disorder caused by mutations in either of the PCCA or PCCB genes which encode the alpha and beta subunits, respectively, of the mitochondrial enzyme propionyl-CoA carboxylase (PCC). In this work we have examined the biochemical findings and clinical outcome of 37 Spanish PA patients in relation to the mutations found in both PCCA and PCCB genes. We have detected 27 early-onset and 101 late-onset cases, showing remarkably similar biochemical features without relation to either the age of onset of the disease or the defective gene they have. Twenty-one of the patients have so far survived and three of them, now adolescents, present normal development. Different biochemical procedures allowed us to identify the defective gene in 9 PCCA deficient and 28 PCCB deficient patients. Nine putative disease-causing mutations accounting for 77.7% of mutant alleles were identified among PCCA deficient patients, each one carrying a unique genotypic combination. Of PCCB mutant alleles 98% were characterised. Four common mutations (ins/del, E168K, 1170insT and A497V) were found in 38/52 mutant chromosomes investigated, whereas the remainder of the alleles harbour 12 other different mutations. By examining the mutations identified both in PCCA and PCCB genes and the clinical evolution of patients, we have found a good correlation between certain mutations which can be considered as null with a severe phenotype, while certain missense mutations tend to be related to the late and mild forms of the disease. Expression studies, particularly of the missense mutations identified are necessary but other genetic and environmental factors probably contribute to the phenotypic variability observed in PA.

Adult↗