Benefit of continuous treatment for responders with newly diagnosed multiple myeloma in the randomized FIRST trial.

The phase 3, randomized Frontline Investigation of Revlimid and Dexamethasone Versus Standard Thalidomide (FIRST) trial investigating lenalidomide plus low-dose dexamethasone until disease progression (Rd continuous) vs melphalan, prednisone and thalidomide for 12 cycles (MPT) and Rd for 18 cycles (Rd18) in transplant-ineligible patients with newly diagnosed multiple myeloma (NDMM) showed that Rd continuous prolonged progression-free survival and overall survival compared with MPT. A subanalysis of the FIRST trial was conducted to determine the benefits of Rd continuous in patients with NDMM based on depth of response. Patients randomized 1:1:1 to Rd continuous, Rd18 or MPT were divided into subgroups based on best response: complete response (CR; n=290), ⩾very good partial response (VGPR; n=679), ⩾partial response (PR; n=1 225) or ⩽stable disease (n=299). Over 13% of patients receiving Rd continuous who achieved ⩾VGPR as best response did so beyond 18 months of treatment. Rd continuous reduced the risk of progression or death by 67%, 51% and 35% vs MPT in patients with CR, ⩾VGPR and ⩾PR, respectively. Similarly, Rd continuous reduced the risk of progression or death by 61%, 54% and 38% vs Rd18 in patients with CR, ⩾VGPR and ⩾PR, respectively. In patients with CR, ⩾VGPR or ⩾PR, 4-year survival rates in the Rd continuous arm (81.1%, 73.1% or 64.6%, respectively) were higher vs MPT (70.8%, 59.8% or 57.2%, respectively) and similar vs Rd18 (76.5%, 67.7% and 62.5%, respectively). Rd continuous improved efficacy outcomes in all responding patients, including those with CR, compared with fixed duration treatment.

Clinical course and predictors of pericardial effusion following cardiac transplantation.

OBJECTIVE: Pericardial effusions occur frequently after orthotopic heart transplantation. There have been conflicting reports describing etiology, prognosis, and outcomes associated with these early postoperative effusions. METHODS: A retrospective review of 91 patients transplanted between January 2001 and September 2004 was performed. Pericardial effusion was defined by serial echocardiography and graded as none, small, moderate, or large. A total of 1088 echocardiograms were evaluated during the first posttransplant year. Perioperative variables were evaluated by logistic regression analysis to define predictors for occurrence of effusions. RESULTS: Echocardiographic data were available for 88 patients. Thirty-one patients (35%) developed moderate to large effusion in the immediate postoperative period. Three patients developed hemodynamic compromise that required immediate intervention. All other effusions resolved within 3 months of heart transplantation without any specific intervention. Only prolonged donor ischemic time was associated with higher risk of occurrence of moderate to large pericardial effusions (odds ratio 1.012, 95% confidence interval 1.001 to 1.019, P = .033). There was no difference in morbidity or early mortality between patients with and without pericardial effusions. CONCLUSION: Moderate to large pericardial effusions occur frequently after heart transplantation. In a vast majority, these effusions are not associated with any adverse clinical outcomes and resolve within 3 months postoperatively. Early postoperative close monitoring is still required to evaluate for tamponade.

Potential renal protective benefits of intra-operative BNP infusion during cardiac transplantation.

BACKGROUND: Recombinant BNP (nesiritide) is known to reduce endothelin levels, cause afferent arteriole vasodilation, and increase natriuresis and diuresis. We hypothesized that intraoperative infusion of BNP may benefit renal function in cardiac transplant patients. METHODS: From June 2003 to September 2005, 22 consecutive heart transplant patients received BNP at a dose of 0.01 microg/kg/min before initiation of cardiopulmonary bypass (group A). BNP infusion was continued for a mean of 3.3 +/- 1.9 days. Hemodynamics, urine output, and serum creatinine levels were prospectively collected and compared with 22 consecutive patients who underwent heart transplantation between May 2002 and June 2003 following the identical transplant protocol, but without BNP infusion (group B). RESULTS: At 24 hours postoperatively, mean blood pressure was comparable between groups (87 +/- 11 mm Hg vs 89 +/- 17 mm Hg, P = .7), but pulmonary artery pressure (18 +/- 5 mm Hg vs 24 +/- 5 mm Hg, P = .001) and central venous pressure (12 +/- 5 mm Hg vs 16 +/- 4 mm Hg, P = .01) were lower with BNP infusion, whereas cardiac index was augmented (2.8 +/- 0.5 vs 2.4 +/- 0.6, P = .03). Requirement of low-dose inotropic and vasopressor support was equally distributed between groups (P > or = .72). Postoperative urine output for the initial 24 hours was higher in group A (84 +/- 15 vs 55 +/- 36 mL/h, P = .01). None of the patients with BNP infusion required additional diuretics or renal replacement therapy during the first week after transplantation. Mean postoperative serum creatinine levels as compared with preoperative values remained unchanged within group A (P = .12), but increased significantly in group B (P < .001). CONCLUSIONS: Intraoperative BNP infusion in heart transplant recipients was associated with favorable postoperative hemodynamics, significantly improved urine output, and stable serum creatinine levels. A prospective, randomized, multicenter trial is warranted to evaluate the potential renal protective benefits of intraoperative BNP infusion in this patient population.

Influence of age and carriage status on salivary IgA to Neisseria meningitidis.

Asymptomatic carriage of Neisseria meningitidis is common (5-35% of individuals) while the incidence of invasive meningococcal disease is fairly low (<1-5 per 100,000 per annum in Europe). Naturally acquired protective immunity may account for this difference. In this study, we investigated the relationship between anti-meningococcal salivary IgA and age and carriage. We showed that salivary IgA to a range of meningococcal antigens increased successively with age with some specificity for commonly circulating serosubtypes. In a group of 258 students 37 (14%) of whom were carriers of N. meningitidis serogroup B, higher levels of specific IgA were associated with carriage. Stratified analysis revealed a positive relationship between smoking and specific anti- N. meningitidis IgA independent of current carriage, weighted odds ratio (OR) 4.1 (95% CI 1.1-18) and OR 3.8 (95% CI 0.96-16) for reference strains B:1:P1.14 and B:4:P1.5,4 respectively. These data implicate IgA as a factor in host defence from meningococcal invasion, although the precise mechanisms remain uncertain.

Nutritional value of a corn containing a glutamate dehydrogenase gene for growing pigs.

Eight female PIC Line 42 pigs (initial BW = 47.5 +/- 1.8 kg) were used in a two-period switchback design (n = 4 per treatment per period) to evaluate the nutritional difference between a genetically modified corn and a similar nontransgenic corn. The genetically altered corn (gdhA+) contained a glutamate dehydrogenase gene isolated from Escherichia coli. The non-transgenic corn was the same variety lacking the transgenic cassette, grown at the same two locations. Pigs were surgically fitted with steered ileocecal valve cannulas for collection of ileal digesta. Diets were made up of primarily one of the two corn sources. Dietary AA profiles were adjusted using crystalline AA to match Illinois Ideal Protein Ratios. Pigs were limit-fed at 8% of metabolic body weight (BW0.75) in two equal feedings at 0600 and 1800 daily throughout the experiment. The study consisted of two 15-d periods. Each period consisted of a 7-d acclimation period, a 3-d total collection of feces and urine, two 12-h ileal collections, and a 3-d adjustment period between ileal collections to ensure adequate hydration. Crude protein, leucine, methionine, alanine, aspartic acid, glutamic acid, and tyrosine concentrations were greater (P < 0.05) in the gdhA+ corn than in the nontransgenic variety. The presence of the gene did not alter (P > 0.17) BW gain. Similarly, DM digestibility, fecal N excretion (grams per day), apparent total-tract N digestibility, N balance, net protein utilization, and N retained as percentages of absorbed were not affected (P > or = 0.32) by the gene modification. Apparent ileal AA digestibility values did not differ (P > 0.31) between the two dietary treatments. Results of this study suggest corn that contains the E coli. gene for glutamate dehydrogenase was nutritionally equivalent to the unaltered variety.

Outcome of bilateral lung volume reduction in patients with emphysema potentially eligible for lung transplantation.

OBJECTIVE: Between January 1993 and May 1998, we performed 200 consecutive bilateral lung volume reduction operations. After initial assessment, 99 of these patients were eligible for lung volume reduction and potentially eligible for immediate or eventual lung transplantation on the basis of age and absence of contraindications. All chose to proceed with lung volume reduction surgery. The outcomes of these 99 patients are reviewed to assess the consequences of proceeding with lung volume reduction surgery on patients potentially eligible for lung transplantation. METHODS: A retrospective study was performed with the use of a prospectively assembled computer database. RESULTS: The 61 men and 38 women were 55 +/- 7 years old at evaluation for lung volume reduction. Mean values for first second expired volume, total lung capacity, and residual volume were 24% +/- 8%, 141% +/- 19%, and 294% +/- 54% predicted. There were 4 operative deaths and 17 late deaths. Two-year and 5-year survival after evaluation for lung volume reduction are 92% and 75%. The 32 patients who have been listed for transplantation after lung volume reduction include 15 who have undergone transplantation, 14 who remain on the list, and 3 who have been removed from the list. All 15 transplant recipients survived transplantation and 3 have subsequently died of rejection or late infection. The 12 living recipients have a median post-transplantation follow-up of 1.7 years. The age at transplantation was 58 +/- 5 years with transplantation occurring 3.8 +/- 1.1 years after lung volume reduction. Sixteen of 99 patients underwent lower lobe volume reduction with an increased rate of listing (63%, P =.008) and transplantation (38%, P =.003) compared with patients undergoing upper lobe volume reduction. Patients listed for transplantation were younger, more impaired, and experienced less benefit from lung volume reduction than patients not yet listed for transplantation. CONCLUSIONS: The preliminary use of lung volume reduction in patients potentially suitable for transplantation does not appear to jeopardize the chances for subsequent successful transplantation.

Endoscopic quadricepsplasty: A new surgical technique.

We present a new surgical subperiosteal endoscopic technique for the release of fibrosis of the quadriceps to the femur caused by gunshot injuries, postsurgical scarring, and fractures, that was developed at the Arthroscopy Group at Hospital Hermanos Ameijeiras in Havana, Cuba. The technique used is a proximal endoscopic subperiosteal extension of the usual arthroscopic intra-articular release of adhesions, using periosteal elevators and arthroscopic scissors placed through medial and lateral superior knee portals to release adhesions and bands of scar tissue beneath the quadriceps mechanism. The technique was used in a prospective case series of 26 male patients aged 19 to 22 years between February 1997 and March 1998 who presented with clinically and ultrasonically documented extra-articular fibrosis resulting in ankylosis of the knee in extension. Only patients who had reached a plateau in their aggressive physiotherapy program with no further progression in knee flexion for 3 months were selected. Those with joint instability, motion-limiting articular surface pathology, and muscle or neurologic injury were excluded. All patients had obtained satisfactory results at 2-year follow-up. The extra-articular release gained at final follow-up was between 30 degrees and 90 degrees of flexion in addition to that obtained at the completion of the standard intra-articular release. Complications included 1 case of deep vein thrombosis, 2 cases of scrotal edema, 5 cases of hemarthrosis, and 2 cases of reflex sympathetic dystrophy. We have found this technique useful in obtaining additional flexion and improved function in a difficult class of patients with ankylosis caused by extra-articular fibrosis of the quadriceps to the femur, allowing immediate aggressive rehabilitation and presenting a useful outpatient alternative with fewer and less severe complications than described with the classic open Thompson's quadricepsplasty.

Minimally invasive selective osteotomy of the knee: A new surgical technique.

We present a simple surgical technique created by the authors to address degenerative chondral lesions of the knee and its application in a limited prospective case series. The technique assumes the concept of beneficial epiphyseal changes caused by disruption of the subchondral bone in improving symptoms, as with drilling, microfracture, periarticular osteotomy, and other invasive procedures. Minimally invasive selective osteotomy (MISO) is an expansion of the arthroscopic treatment of the knee, specifically targeting symptomatic lesions with minimal additional trauma and cost, while avoiding disruption of the articular surface of the subchondral bone. The technique involves a mimimal access approach with selective saw cuts placed with a 1-cm oscillating blade parallel to the joint surface 1 to 1.5 cm deep to identified lesions. The technique does not address malalignment but can address lesions not addressed by classic osteotomies and, as such, may be combined with other corrective alignment procedures as necessary. We present the results of MISO of the knee in a case series of 62 outpatients carried out at the Orthopaedic Division of the Clinical and Surgical Hermanos Ameijeiras Hospital in Havana, Cuba. At 2-year follow-up, there was improvement of symptoms without significant complications.

Endoscopic treatment of calcaneal spur syndrome: A comprehensive technique.

We describe a comprehensive approach to the endoscopic treatment of calcaneal spur syndrome developed by the Arthroscopic Group of the Orthopedic Service of Hospital Hermanos Ameijeiras in Havana, Cuba. The surgical technique involves treatment of the heel spur and plantar fasciitis commonly found in calcaneal spur syndrome, but it also addresses adjacent calcaneal periostitis and allows decompression of the nerve to the abductor digiti quinti. Medial endoscopy and lateral instrumentation are used in a sequential approach with exposure and debridement of the posterior roof of the calcaneal arch, followed by removal of the calcaneal spur, lateral to medial release of the medial 75% of the plantar fascia, and if necessary, debridement of the calcaneal tuberosity periosteum. This technique was used in a prospective case series from June 1997 to May 1998 to treat a select group of 38 feet in 30 patients who reported unacceptable levels of pain despite 5 months of conservative treatment, which included an aggressive 8-week physical therapy program prescribed by the treating physician. Good to excellent results were obtained at 3 months postoperatively in all patients with regard to pain relief and return to normal activity, although 5 patients required a short course of physical therapy to resolve symptoms brought on by sports, trauma, or impact loading before 1-year follow-up, at which time all patients reported good to excellent results. Complications included 3 superficial wound infections cured by oral antibiotics and 2 transient lateral paresthesias that resolved with rest and nonsteroidal inflammatory medications. The described technique may provide a useful method for treating refractory heel spur syndrome and warrants further study.

Arthroscopic decompressive medial release of the varus arthritic knee: Expanding the functional envelope.

We present the rationale and technique for treating medial knee osteoarthritis by dynamically unloading the medial compartment of the knee. Recent advances in kinematic studies indicate a dynamic linkage between differing degrees of freedom in the knee joint. Both the adduction moment and the foot progression angle are important determinants of medial compartment loading. The medially osteoarthritic knee has progressive compromise of free motion in more than 1 plane. Arthroscopic decompressive medial release unloads the medial compartment by release of the medial capsule and medial collateral ligament in the presence of intact cruciate ligaments, which may allow a decreased adduction moment and decrease of the external rotation restraint in extension found in more severely osteoarthritic knees. A case series of 38 patients with medial gonarthrosis was treated by this technique at the Hermanos Ameijeiras Hospital in Havana, Cuba. All patients had good results without postoperative valgus instability or significant complications. We feel that this technique warrants further clinical and biomechanical study for its use in isolation or in combination with high tibial osteotomy or minimally invasive selective osteotomy for the treatment of medial gonarthrosis of the knee. A minimally invasive, selective approach to biomechanical factors in osteoarthritis may be combined with other modulating techniques in efforts to forestall or prevent the need for total joint replacement.

Combined partial arthroscopic synovectomy and radiation therapy for diffuse pigmented villonodular synovitis of the knee.

We present the results of combined partial arthroscopic synovectomy and low-dose external-beam radiation therapy (RT) in the treatment of diffuse pigmented villonodular synovitis (PVNS) of the knee. Mechanical synovectomy is an effective tool in treating PVNS of the knee, but when used alone it may be insufficient to eliminate all affected tissue. Intra-articular radiation or external-beam radiation may be added to mechanical synovectomy to treat recurrence but is not routinely done at the time of initial synovectomy. Combining intra-articular synovectomy with RT at the initial treatment for PVNS of the knee may reduce the recurrence rate. We present a prospective study of the treatment of 22 patients with clinical, ultrasonic, and histologically confirmed findings of diffuse PVNS of the knee. Characteristic clinical findings included pain, swelling, and erythema. These patients were treated by the Arthroscopic Surgery Group of the Orthopaedic Service at the Hospital "Hermanos Ameijeiras" in Havana, Cuba from 1990 to 1998. The protocol included anterior (patellofemoral, medial, and lateral) arthroscopic synovectomy and postoperative RT with a total dose of 2,600 cGy. This combination therapy was effective in reducing symptoms of pain and edema, and in improving overall function of patients. Nineteen patients (86%) had good or excellent results at an average follow-up of 33 months (range, 26 to 76 months). Three patients had residual stiffness and swelling, 2 of whom also had pain. Three had clinically and ultrasonically confirmed recurrence of disease and were treated with repeat arthroscopic synovectomy without harmful effects from RT. In all of the cases requiring repeat arthroscopic synovectomy, we observed fibrous bands secondary to reorganization of synovial inflamed tissue, meniscal retraction, and microscopic findings of fibrosis and cellular paucity. Partial arthroscopic synovectomy combined with low-dose RT in anti-inflammatory doses produced good results in the treatment of PVNS without significant complications in our patient series. Partial arthroscopic synovectomy of the knee for PVNS may be combined with RT to reduce the risk of disease recurrence. Adjuvant RT should also be considered for patients receiving a radical synovectomy to treat inaccessible or hidden disease sites. Rates of recurrence with combined partial (anterior) synovectomy and RT approach that of complete synovectomy in this series. Combining RT with radical arthroscopic synovectomy might further reduce recurrence rates.

Reduced cardiotoxicity and preserved antitumor efficacy of liposome-encapsulated doxorubicin and cyclophosphamide compared with conventional doxorubicin and cyclophosphamide in a randomized, multicenter trial of metastatic breast cancer.

PURPOSE: To determine whether Myocet (liposome-encapsulated doxorubicin; The Liposome Company, Elan Corporation, Princeton, NJ) in combination with cyclophosphamide significantly reduces doxorubicin cardiotoxicity while providing comparable antitumor efficacy in first-line treatment of metastatic breast cancer (MBC). PATIENTS AND METHODS: Two hundred ninety-seven patients with MBC and no prior chemotherapy for metastatic disease were randomized to receive either 60 mg/m(2) of Myocet (M) or conventional doxorubicin (A), in combination with 600 mg/m(2) of cyclophosphamide (C), every 3 weeks until disease progression or unacceptable toxicity. Cardiotoxicity was defined by reductions in left-ventricular ejection fraction, assessed by serial multigated radionuclide angiography scans, or congestive heart failure (CHF). Antitumor efficacy was assessed by objective tumor response rates (World Health Organization criteria), time to progression, and survival. RESULTS: Six percent of MC patients versus 21% (including five cases of CHF) of AC patients developed cardiotoxicity (P =.0002). Median cumulative doxorubicin dose at onset was more than 2,220 mg/m(2) for MC versus 480 mg/m(2) for AC (P =.0001, hazard ratio, 5.04). MC patients also experienced less grade 4 neutropenia. Antitumor efficacy of MC versus AC was comparable: objective response rates, 43% versus 43%; median time to progression, 5.1% versus 5.5 months; median time to treatment failure, 4.6 versus 4.4 months; and median survival, 19 versus 16 months. CONCLUSION: Myocet improves the therapeutic index of doxorubicin by significantly reducing cardiotoxicity and grade 4 neutropenia and provides comparable antitumor efficacy, when used in combination with cyclophosphamide as first-line therapy for MBC.

Lung transplantation is warranted for stable, ventilator-dependent recipients.

BACKGROUND: Lung transplantation for patients on ventilators is a controversial use of scarce donor lungs. We have performed 500 lung transplants in 12 years and 21 of these have been in ventilator-dependent patients. METHODS: A retrospective review of patient records and computerized database was performed. Living patients were contacted to confirm their health and functional status. RESULTS: Patients included 13 men and 8 women with a mean age of 43 years. Sixteen patients were considered stable awaiting lung transplant, whereas 5 patients were unstable with acute graft failure after prior lung transplantation. Stable patients had been ventilated for a mean of 57 +/- 46 days whereas unstable patients had been supported for 10 +/- 9 days. Half of the patients required cardiopulmonary bypass support during the transplant, and there was no statistical difference in the frequency of CPB in stable and unstable patients (p = 0.61). Three hospital deaths included 0 of 16 of the stable patients and 3 of 5 of the unstable patients (p = 0.01). Long-term actuarial survival was significantly better in stable versus unstable patients (p = 0.02), with 5-year survival 40% for stable patients and 0% for unstable patients. CONCLUSIONS: Lung transplantation can be successfully conducted in stable patients who have become ventilator dependent after listing for transplantation. Acute retransplantation for early lung dysfunction is high risk and has produced poor long-term results.

Perioperative complications after living donor lobectomy.

OBJECTIVE: Clinical lung transplantation has been limited by availability of suitable cadaveric donor lungs. Living donor lobectomy provides right and left lower lobes from a pair of living donors for each recipient. We reviewed our experience with living donor lobectomy from July 1994 to February 2000. METHODS: Sixty-two donor lobectomies were performed. The hospital and outpatient records of these 62 donors were retrospectively analyzed to examine the incidence of perioperative complications. RESULTS: Twenty-four (38.7%) of 62 donors had no perioperative complications and had a median length of hospital stay of 5.0 days. Thirty-eight (61.3%) of 62 donors had postoperative complications. Twelve major complications occurred in 10 patients and included pleural effusions necessitating drainage (n = 4), bronchial stump fistulas (n = 3), bilobectomy (n = 1), hemorrhage necessitating red cell transfusion (n = 1), phrenic nerve injury (n = 1), atrial flutter ultimately necessitating electrophysiologic ablation (n = 1), and bronchial stricture necessitating dilatation (n = 1). These 38 donors had 55 minor complications including persistent air leaks (n = 9), pericarditis (n = 9), pneumonia (n = 8), arrhythmia (n = 7), transient hypotension necessitating fluid resuscitation (n = 4), atelectasis (n = 3), ileus (n = 3), subcutaneous emphysema (n = 3), urinary tract infections (n = 2), loculated pleural effusions (n = 2), transfusion (n = 2), Clostridium difficile colitis (n = 1), puncture of a saline breast implant (n = 1), and severe contact dermatitis secondary to adhesive tape (n = 1). There were no postoperative deaths and only 1 donor required surgical re-exploration. CONCLUSIONS: Living donor lobectomy can be performed with low mortality and remains an important alternative for potential recipients unable to wait for cadaveric lung allografts. However, morbidity is high and must be considered when potential living donors are being counseled.

Selective use of extracorporeal membrane oxygenation is warranted after lung transplantation.

OBJECTIVES: Early allograft dysfunction after lung transplantation ranges from subclinical x-ray abnormalities to pulmonary edema, hypoxemia, hypercarbia, and pulmonary hypertension. Management may include extracorporeal circulation to allow recovery of the acute lung injury. We reviewed our experience with extracorporeal membrane oxygenation after lung transplantation to assess the utility of this therapy. METHODS: A retrospective chart review was performed. Single or bilateral lung transplantation was performed in 444 adults from July 1988 to July 1998. Twelve (2.7%) patients experienced allograft dysfunction severe enough to require extracorporeal membrane oxygenation after failure of conventional therapy, including sedation, paralysis, and inhaled nitric oxide. RESULTS: Seven of 12 patients requiring extracorporeal membrane oxygenation were discharged from the hospital. Mean and median times to extracorporeal membrane oxygenation support were 1.2 days and 0 days, respectively. Mean length of support was 4.2 days. Four patients died while receiving extracorporeal membrane oxygenation support. One patient was weaned from extracorporeal membrane oxygenation but died during the hospitalization. Two patients required acute retransplantation while receiving extracorporeal membrane oxygenation, and one survived to discharge. Three patients continued to receive extracorporeal membrane oxygenation support for more than 4 days, and all 3 died. All survivors had begun receiving extracorporeal membrane oxygenation support by post-transplantation day 1. Three of 7 patients discharged from the hospital died 12 months, 13 months, and 72 months after transplantation because of bronchiolitis obliterans syndrome (n = 2) or lymphoma (n = 1). Four patients are alive 2, 12, 25, and 54 months after transplantation. CONCLUSIONS: Extracorporeal membrane oxygenation provides effective therapy for acute post-transplantation lung dysfunction. The frequency and pattern of our extracorporeal membrane oxygenation use reflects bias toward early extracorporeal membrane oxygenation support for isolated graft failure in otherwise intact and uninfected recipients.

Single versus bilateral lung transplantation for idiopathic pulmonary fibrosis: a ten-year institutional experience.

OBJECTIVE: Between July 1988 and July 1998, we performed 433 lung transplants. Forty-five patients had idiopathic pulmonary fibrosis, and operations for these patients included 32 single lung transplants and 13 bilateral sequential lung transplants. This study reviews this experience and compares single lung transplantation and bilateral lung transplantation for pulmonary fibrosis. METHODS: We performed a retrospective review, including inpatient hospital charts, outpatient clinic records, and telephone contact with patients to verify current health status. RESULTS: Perioperative mortality was 4 (8.9%) patients. One patient underwent redo bilateral lung transplantation for reperfusion injury and graft failure after single lung transplantation. The median hospitalization was 22 days. Actuarial survival at 1 and 5 years was 75.5% and 53.5%, respectively, which was not significantly different from our survival for all recipients (85.5% and 56.4%, respectively). Seventeen (41%) of 41 operative survivors have died. Late causes of death included obliterative bronchiolitis with respiratory failure (9), malignancy (3), and cytomegalovirus pneumonitis (2). Hospital mortality was 3 (9.4%) of 32 after single lung transplantation and 1 (7.7%) of 13 after bilateral lung transplantation. There was no difference between single and bilateral lung transplantation with regard to hospital stay. Four (12.5%) of the 32 patients undergoing single lung transplantation required tracheostomy, whereas 3 (23%) of 13 recipients undergoing bilateral lung transplantation required tracheostomy. CONCLUSION: Single or bilateral lung transplantations offer viable therapy for patients with pulmonary fibrosis. We demonstrate no benefit of bilateral over single lung transplantation for patients with this diagnosis. Survival after transplantation appears better than that of historic control subjects receiving standard medical care at other institutions.

Approach to the vaso-occlusive crisis in adults with sickle cell disease.

The vaso-occlusive crisis, or sickle cell crisis, is a common painful complication of sickle cell disease in adolescents and adults. Acute episodes of severe pain (crises) are the primary reason that these patients seek medical care in hospital emergency departments. Frequently, however, the pain is incompletely treated. Despite advances in pain management, physicians are often reluctant to give patients adequate dosages of narcotic analgesics because of concerns about addiction, tolerance and side effects. It is important to recognize a pain crisis early, correct the inciting causes, control pain, maintain euvolemia and, when necessary, administer adequate hemoglobin to decrease the hemoglobin S level. The family physician and the hematologist must work together to treat acute pain episodes promptly and effectively, manage the long-term sequelae of chronic pain and prevent future vaso-occlusive crises.

Acute chest syndrome in sickle cell disease. Crucial considerations in adolescents and adults.

Rapid recognition of acute chest syndrome is essential in patients with sickle cell disease. The condition can be particularly severe in adolescents and adults and often leads to death. In this article, the authors review the challenges of evaluating the syndrome and outline current treatment and supportive care.