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[T1 maps and O2-enhanced MRT of the diseased lung. Emphysema, fibrosis, mucoviscidosis].

PURPOSE: Gas exchange is the primary function of the lung and the transport of oxygen plays a key role in pulmonary physiology and pathophysiology. MATERIALS AND METHODS: Molecular oxygen is weakly paramagnetic, so that an increase in oxygen concentration results in shortening T1 relaxation time and thus increasing signal intensity in T1 weighted images. The calculation of parameter maps may allow deeper insights into relaxation mechanisms. T1 maps based on a snapshot FLASH sequence obtained during the inhalation of various oxygen concentrations allow the creation of an oxygen transfer function, providing a measurement of local oxygen transfer. T1 weighted single shot TSE sequences demonstrate the signal changing effects during inhalation of pure oxygen. RESULTS: The average of the mean T1 values over the entire lung during inspiration was 1,199+/-117 ms, the average of these values during expiration was 1,333+/-167 ms. T1 maps of patients with emphysema and lung fibrosis show fundamentally different values and respiratory dependence compared to healthy individuals. Oxygen enhanced MR has the potential to assess reduced diffusion capacity and decreased transport of oxygen in patients with emphysema and cystic fibrosis. DISCUSSION: Results published in the literature indicate that T1 mapping and oxygen enhanced MR are promising new methods in functional imaging of the lung.

Administration, Inhalation↗

Native lung complications after single lung transplantation for emphysema.

We reviewed the impact of the presence of the native diseased contralateral lung on the outcome after single lung transplantation for emphysema. Twenty consecutive recipients of single lung transplants for emphysema were reviewed for complications related to the native lung. Five patients (25%) suffered major complications arising in the native lung and resulting in serious morbidity and mortality. The timing of onset varied from 1 day to 43 months after transplantation. We conclude that the susceptibility of the native lung to complications such as those described in this report is an additional fact to be considered in choosing the ideal transplant procedure for patients with obstructive lung disease.

Adult↗

Lung transplantation versus lung volume reduction as surgical therapy for emphysema.

There are currently two surgical therapies aimed at crippling, end-stage emphysema: lung transplantation and lung volume reduction surgery (LVRS). Unfortunately, most emphysema patients are poor candidates for any surgical intervention. The authors favor a meticulous selection process in which indications and contraindications are considered and the best solution is devised for each patient. Patients with ideal circumstances for LVRS--hyperinflation, heterogeneous distribution of disease, FEV1 of more than 20%, and normal PCO2--are offered LVRS. Patients with diffuse disease, low FEV1, hypercapnia, and associated pulmonary hypertension are directed toward transplantation. LVRS has not been a satisfactory option for patients with a1-antitrypsin deficiency, and we prefer a transplant in most of these patients. With these considerations, we find that few patients are serious candidates for both procedures. Combinations of lung volume reduction and lung transplantation, simultaneously or sequentially, are possible but rarely necessary.

Contraindications↗

Ball valve obstruction of a bronchus causing lobar emphysema in a neonate.

Lobar emphysema in the neonate is usually congenital, resulting from cartilage deficiency causing bronchomalacia and distal air trapping. Acquired forms are usually associated with chronic lung disease or endobronchial obstruction such as mucus plugging. We report a pedunculated endobronchial polyp in a 2-month old ex-premature infant causing intermittent hyperinflation of the right middle lobe. The polyp was seen prolapsing in and out of the bronchus intermedius at bronchoscopy. Possible aetiological links with mechanical ventilation are discussed. We also emphasise the value of bronchoscopy prior to lobectomy in cases of congenital lobar emphysema (CLE).

Bronchial Diseases↗

The change in body composition after bilateral lung volume reduction surgery for underweight patients with severe emphysema.

The aim of this study was to examine the short-term effect of lung volume reduction surgery (LVRS) on body composition and other nutritional indicators in 28 patients with emphysema underwent thoracoscopic LVRS. Functional tests, body weight (BW), and body composition were measured before and 6 months after surgery. Mean daily caloric intake (CI) was estimated by 3-day dietary record as well. Fat-free mass (FFM) and fat mass (FM) were assessed by bioelectrical impedance analysis (BIA). FEV1.0 and VO2max have improved after LVRS by 35.2% and 23.8%, respectively. Preoperatively, 75% of patients were underweight (% ideal body weight (%IBW) <90) with the mean %IBW at 84.5%. BW, CI, and FFM increased significantly after LVRS, whereas FM was unchanged. The change in BW correlated significantly with the change in FEV1.0, MVV, and VO2max (p < 0.01) but not with CI. Bilateral LVRS results in an increase in FFM and functional improvement for underweight patients with severe emphysema, and it may contribute to the improvement in maximal exercise capacity.

Aged↗

Pulmonary storage with emphysema as a sign of Niemann-Pick type C2 disease (second complementation group). Report of a case.

A case is described of Niemann-Pick type C2 disease presenting an infantile pneumopathic phenotype known to occur in this recently established, second, minor complementation group of Niemann-Pick type C (NPC) disease. However, the pulmonary involvement was unique, being dominated, in addition to the usual storage macrophage infiltration of the alveolar and septal compartments, by irregular emphysema attributed to storage cell migration into the bronchiolar lumen. The latter modified considerably the X-ray findings and hindered the initial clinical diagnosis. Otherwise, the storage phenotype, including the range of stored lipids, storage distribution, and cell and organ pathology, was found to be identical to that in the whole Niemann-Pick type C disease group dominated by NPC1. The biochemical findings (cholesterol esterification level) corresponded to the classical biochemical phenotype. Emphysema should thus be considered as a variant of the pulmonary NPC2 storage process, governed most probably by an epigenetic mechanism responsible for storage macrophage migration into the bronchiolar compartment.

Emphysema↗

Persistent interstitial pulmonary emphysema-like cyst associated with metastatic synovial sarcoma.

Interstitial pulmonary emphysema is characterized by the presence of gas dissecting the interstitial tissues of the lung. Clinically, it may be acute or persistent, and the latter can be further categorized as localized or diffuse. Usually, it appears in preterm neonates with a history of assisted ventilation or respiratory distress. Although far from frequent, the localized variety of persistent interstitial pulmonary emphysema (PIPE) can develop spontaneously in full-term babies or infants without any obvious underlying pulmonary disease. Histologically, PIPE is characterized by the presence of uni- and multinucleated histiocytes lining the inner surface of the cysts. In this report, we describe a 15-year-old male with synovial sarcoma (SS) of the right ankle diagnosed 4 years previously who developed pulmonary metastasis, one of which presented cystic changes with features of PIPE.

Adolescent↗

Overestimated frequency of a possible emphysema-susceptibility allele when microsomal epoxide hydrolase is genotyped by the conventional polymerase chain reaction-based method.

A recent association study suggested that the His113 variant of microsomal epoxide hydrolase (mEPHX) may confer a risk for development of emphysema, presumably by increasing susceptibility to smoking injury. Before considering a possible role of this enzyme in pulmonary disease, we attempted to characterize the genetic polymorphism further. The Tyr/His113 polymorphism within exon 3 of mEPHX was initially examined in 62 healthy individuals by conventional methods involving polymerase chain reaction (PCR)-based determination of a restriction fragment length polymorphism (RFLP). Genomic nucleotide sequences, including the polymorphic site and the downstream primer sequence, were further analyzed in 95 unrelated, healthy Japanese volunteers by single-stranded conformation polymorphism (SSCP) analysis and direct sequencing. Genotyping by the first method (PCR-RFLP) revealed that the allelic distribution in our test population apparently deviated from Hardy-Weinberg equilibrium. Sequence analysis showed that a synonymous nucleotide substitution, AAG to AAA (Lys119), was located just within the published primer site. The AAA at codon 119 was present only in alleles with Tyr113, and its frequency reached 0.31 in our panel of 190 Japanese alleles. This substitution potentially hampered PCR amplification because of the nucleotide mismatch, with the result that the frequency of the Tyr113 variation was underestimated. The frequency of His113, a possible emphysema susceptibility allele of the mEPHX gene, was thus overestimated when human DNA samples were genotyped in the conventional way. Depending on the population(s) tested, this anomaly could represent a pitfall for PCR-based association studies.

Alleles↗

Bilateral orbital emphysema and pneumocephalus as a result of accidental compressed air exposure.

Orbital emphysema is a rare condition in the absence of trauma or sinus disease. A 22-year-old man suffering from left orbital trauma due to sudden exposure to compressed air tube was admitted with severe pain in the left eye, swelling, and mild periorbital ecchymosis. Physical examination revealed a large conjunctival laceration in the left orbit. Multislice computed tomographic scanning of the head and orbits showed extensive radiolucencies consistent with the air in both orbits, more prominent in the left. There was also subcutaneous air in the left periorbital soft tissue extending through fronto-temporal and zygomatic areas. Air was also demonstrated adjacent to the left optic canal and within the subarachnoid space intracranially. There was no evidence of any orbital, paranasal sinus, or cranial fracture. Visual acuity was minimally decreased bilaterally. The conjunctiva was sutured under local anesthesia. After 3 weeks of follow-up, the patient completely recovered without visual loss. Bilateral orbital emphysema with pneumocephalus can occur from a high-pressure compressed air injury after unilateral conjunctival trauma without any evidence of fracture.

Adult↗

Evolving strategies in lung transplantation for emphysema.

Evolving strategies of pulmonary preservation, bronchial revascularization, immunosuppression, and infectious disease management were used in 15 initial consecutive patients undergoing lung transplantation for emphysema. There were 10 women and 5 men with a mean age of 49 years (range, 36 to 60 years). All patients required supplemental oxygen therapy. One bilateral, 9 left, and 5 right transplantations were performed. Mean preoperative forced expiratory volume in 1 second and total lung capacity were 16% and 146%, respectively, of predicted. Quadruple drug immunosuppression was used. Actuarial 1-year survival in this initial series is 93.3% +/- 6.4% (Kaplan-Meier) with one early cardiac death at day 71. Mean forced expiratory volume in 1 second and diffusing capacity for carbon monoxide at discharge were 43% and 62%, respectively, of predicted. Rehabilitation has been excellent, and all survivors are active and free of supplemental oxygen. During the study, the following treatment strategies have evolved: (1) University of Wisconsin solution has replaced Euro-Collins' solution for pulmonary preservation; (2) direct bronchial revascularization with the internal thoracic artery now is used; (3) an algorithm-based variable dose OKT3 induction regimen has resulted in a major reduction in dosage; and (4) infectious disease management focuses on the prophylaxis of cytomegalovirus and fungal infection using prolonged ganciclovir and early itraconazole therapy as well as the avoidance of Epstein-Barr virus mismatches. Single-lung transplantation for emphysema has excellent early results with continuing evolving management strategies.

Adenosine↗

Pacemaker failure due to pocket emphysema.

Pocket emphysema is an unusual cause of failure of a permanent pacemaker. Reported herein is temporary failure of a unipolar DDD pacemaker caused by pocket emphysema in a pediatric patient.

Equipment Failure↗

Thoracoscopic laser pneumoplasty in the treatment of diffuse bullous emphysema.

BACKGROUND: Thoracoscopic laser pneumoplasty in the treatment of diffuse bullous emphysema by means of a contact neodymium:yttrium-aluminum garnet laser was evaluated by a retrospective analysis of the first consecutive 500 procedures in 443 patients. The indication for thoracoscopic laser pneumoplasty was intractable dyspnea. METHODS: Advanced age (mean age, 67 years), high oxygen dependency (70%), steroid use (46%), and markedly diminished physical capacity (2% bedridden and 27% wheelchair-bound) were noted. Thoracoscopic laser pneumoplasty was carried out under general anesthesia and one-lung ventilation. Type 3 bullae (381 procedures) were contracted by contact neodymium:yttrium-aluminum garnet laser and type 4 bullae (199 procedures) excised. The operative mortality rate was 4.8%. RESULTS: Subjective improvement was reported by 87% of the patients. Follow-up functional evaluation was available in 229 patients, which showed highly significant improvement. A comparison of preoperative and postoperative functional tests between type 3 and 4 bullae patients showed no significant difference, except the latter had higher decrease in airway resistance, residual volume, and total lung capacity. CONCLUSIONS: Thoracoscopic laser pneumoplasty is an effective treatment for both type 3 and 4 bullous emphysema with an acceptable risk.

Adult↗

Use of the Heimlich valve to shorten hospital stay after lung reduction surgery for emphysema.

BACKGROUND: Prolonged air leak is the major complication after lung reduction surgery for emphysema and the major determinant of hospital length of stay. METHODS: Twenty-five of 107 patients (24%) (mean age, 66 years) with an average forced expiratory volume in 1 second of 0.55 L experienced a prolonged air leak (>5 days) after lung reduction surgery. These persistent air leaks were treated by replacing the chest drainage system with Heimlich valves to facilitate earlier hospital discharge even though 64% of the patients had apical air spaces that measured 1 to 7 cm. RESULTS: These patients had a mean postoperative stay of 9.1 days. Chest tubes were then removed an average of 7.7 days later. All apical air spaces resolved, and there were no deaths, empyemas, or pneumonias. CONCLUSIONS: In conclusion, the use of the Heimlich valve after operation for emphysema was associated with minimal morbidity and shortened the mean hospital stay for patients with prolonged air leaks by 46%. This study demonstrates an important concept in the postoperative management of these patients--do not use suction on severely emphysematous lung.

Aged↗

Serum antielastase deficiency in tight-skin mice with genetic emphysema.

The tight-skin (Tsk) mouse is a model of genetically determined emphysema. The cause for the development of the lung lesion is unknown. In the present study we investigated the lung morphometry and the serum elastase inhibitory capacity (EIC) of Tsk mice. Mean interalveolar distance was significantly greater (+60%) in Tsk mice than in C57 Bl/6J, NMRI, and Balb/c mice, which have similar values. Serum of Tsk mice against mouse leukocyte elastase (MLE) has significantly lower EIC values than that of NMRI, Balb/c (-64%), and C57 Bl/6J (-50%) mice. Similar results were obtained when porcine pancreatic elastase (PPE) was used. Against human leukocyte elastase (HLE), however, there was no difference among the strains, all of which had high EIC values. Preincubation of mouse (C57 Bl/6J) serum with chloramine-T (CT) resulted in an almost complete inhibition of EIC against MLE and PPE but only in a 20% inhibition against HLE using a synthetic substrate. Using elastin Congo Red as substrate, CT inhibited EIC against MLE and PPE by approximately 70% but did not affect the EIC against HLE. These results indicate that (1) the Tsk mouse can be considered a model of severe inborn deficiency of serum antielastase activity which is associated with emphysema; and (2) MLE and PPE can be considered interchangeable in studies of serum EIC in the mouse. On the other hand, the differences between MLE and HLE preclude the use of HLE for EIC determination in this species.

Animals↗

Thoracoscopic carbon dioxide laser treatment of bullous emphysema.

A new technique of thoracoscopic laser ablation of pulmonary bullae suitable for patients with multiple bullae and diffuse emphysema was developed and assessed in 22 patients. 20 of 22 patients survived. Pre-operative and postoperative functional evaluation is available for the 11 patients followed up for more than a month; at 1 to 3 months postoperatively there were increases in FVC (mean 2.0 litres pre-operatively to 2.7 litres postoperatively, p less than 0.001), in FEV1 (0.74 to 1.06 litres, p = 0.01), and in maximum exercise treadmill times (5.4 min to 8.0 min, p less than 0.01). Postoperative air leaks lasted a mean of 13 days and usually resolved spontaneously. Other complications were bleeding (1 patient) and unilateral acute lung injury (1 patient). These results suggest that selected patients with diffuse emphysema and pulmonary bullae may benefit from thoracoscopic carbon dioxide laser ablation.

Cysts↗

Congenital lobar emphysema: a case with bronchial septum.

Congenital lobar emphysema has been described under various designations. Although the etiology is not clear, some form of bronchial obstruction has been documented in about 50% of the patients. A rare case of congenital lobar emphysema with a membranous septum in the left main bronchus is described. Although prompt lobectomy has been the treatment of choice in most cases, this case demonstrates the importance of early bronchoscopy to exclude treatable intra luminal abnormalities.

Bronchi↗

Differentiation of poor R wave progression of old anteroseptal myocardial infarction from that due to emphysema.

The R/S ratio was calculated in all the six conventional precordial leads in 27 cases of emphysema and 23 cases of old anteroseptal myocardial infarction, all having poor R wave progression in the precordial leads. An R/S ratio greater than or equal to 3.5 in lead V5 was found to be most sensitive (87%), specific (83%) and accurate (85%) in differentiating poor R wave progression of old anteroseptal myocardial infarction from that due to emphysema.

Diagnosis, Differential↗

Tension mediastinal emphysema: emergency percutaneous drainage with CT guidance.

Four patients aged 8 to 54 years, under mechanical ventilation, presented with sudden tension mediastinal emphysema and exhibited typical clinical symptoms of compromised venous return. Diagnosis of tension mediastinal emphysema was confirmed by CT. A percutaneous catheter with a diameter varying from F12 to F20 was inserted in the anterior mediastinal compartment with CT control and connected to a waterseal aspiration. Emergency treatment was efficient in all patients. Percutaneous aspiration was continued for 3 to 20 days.

Adult↗