The location and tracking of swallowed dental appliances: the role of radiology

BACKGROUND: The effects of beta 2 adrenergic agonists on chemoreceptors remain controversial. This study was designed to examine whether fenoterol, a beta 2 adrenergic agonist, increases the ventilatory responses to hypercapnia (HCVR) and hypoxia (HVR) in normal subjects. METHODS: HCVR was tested with a rebreathing method and HVR was examined with a progressive isocapnic hypoxic method in 11 normal subjects. Both HCVR and HVR were assessed by the slope of occlusion pressure (P0.1) or ventilation (VE) plotted against end tidal carbon dioxide pressure and arterial oxygen saturation, respectively. Respiratory muscle strength, spirometric values and lung volume were measured. After a single oral administration of 5 mg fenoterol or placebo HCVR and HVR were evaluated. RESULTS: Fenoterol treatment did not change the specific airway conductance or forced expiratory volume in one second. Respiratory muscle strength did not change. Fenoterol increased the slope of the HCVR of both P0.1 (from 0.251 (0.116) to 0.386 (0.206) kPa/kPa, average increase 71%) and VE (from 10.7 (3.4) to 15.1 (4.2) l/min/kPa, average increase 52%), and shifted the response curves to higher values. For the HVR fenoterol increased the slopes of both P0.1 and VE (from -4.06 (2.00) x 10(-3) to -7.99 (4.29) x 10(-3) kPa/%, an average increase of 83%, and from -0.221 (0.070) to -0.313 (0.112) l/min/%, a 44.5% increase, respectively), and shifted the response curves to higher values. CONCLUSION: Acute administration of fenoterol increases the ventilatory responses to both hypercapnia and hypoxia in normal subjects.     

Lung transplantation for Williams-Campbell syndrome

Williams-Campbell syndrome is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi leading to distal airway collapse and bronchiectasis. We report the first case of lung transplantation in a patient with end-stage lung disease secondary to Williams-Campbell syndrome. Although the patient did not have proximal airway collapse prior to transplantation, his posttransplant course was complicated by the development of bronchomalacia of the right and left mainstem bronchi. The patient experienced recurrent pulmonary infections and died of bacterial pneumonia 1 year after transplantation. Autopsy revealed cartilage deficiency in both right and left mainstem bronchi. A hypothesis may be made that a combination of proximal cartilage deficiency and posttransplant airway ischemia led to the development of bronchomalacia after lung transplantation. Thus, in contrast to previous reports, the cartilage deficiency in Williams-Campbell syndrome can involve both proximal and distal airways. Consequently, bilateral sequential lung transplantation may not be an effective therapeutic option in patients with this syndrome.     

Diagnosis oligohydramnios-related pulmonary hypoplasia (Potter syndrome): value of portable voiding cystourethrography in newborns with respiratory distress

Potter renal nonfunctional syndrome is an association of facial and limb anomalies, pulmonary hypoplasia, and fetal renal anomalies which lead to marked oligohydramnios, including renal agenesis (true Potter syndrome), renal cystic dysplasia, and obstructive uropathies. Some infants survive long enough to develop severe respiratory distress secondary to pulmonary hypoplasia. The underlying renal disease is often noted only at autopsy. We studied four infants, only one of whom had clinical signs of the renal nonfunction syndrome. Portable voiding cystourethrography revealed a tiny bladder in three infants with cystic dysplasia kidneys (two of these infants had reflux into unused ureters). Bladder hypertrophy and vesicoureteral reflux secondary to posterior uretral valves were noted in the fourth infant.     

Chemoradiotherapy in cancers of the uterine cervix

In order to determine the possible role of the cystic fibrosis transmembrane regulator (CFTR) gene in pulmonary diseases not due to cystic fibrosis, a complete screening of the CFTR gene was performed in 120 Italian patients with disseminated bronchiectasis of unknown cause (DBE), chronic bronchitis (CB), pulmonary emphysema (E), lung cancer (LC), sarcoidosis (S) and other forms of pulmonary disease. The 27 exons of the CFTR gene and their intronic flanking regions were analyzed by denaturing gradient gel electrophoresis and automatic sequencing. Mutations were detected in 11/23 DBE (P = 0.009), 7/25 E, 5/27 CB, 5/26 LC, 5/8 S (P = 0.013), 1/4 tuberculosis, and 1/5 pneumonia patients, and in 5/33 controls. Moreover, the IVS8-5T allele was detected in 6/25 E patients (P = 0.038). Four new mutations were identified: D651N, 2377C/T, E826K, and P1072L. These results confirm the involvement of the CFTR gene in disseminated bronchiectasis of unknown origin, and suggest a possible role for CFTR gene mutations in sarcoidosis, and for the 5T allele in pulmonary emphysema.     

A case of Kartagener's syndrome associated with pulmonary tuberculosis, pneumothorax and pulmonary aspergilloma

A case of Kartagener's syndrome associated with multiple pulmonary complication was presented. A 19-year-old man was admitted to our hospital because of pulmonary tuberculosis in May 1972. He had been diagnosed as Kartagener's syndrome because of the presence of chronic parasinusitis, bronchiectasis and complete situs inversus. His chest radiographs in Dec 1972 revealed left pneumothorax. Chest radiographs in Aug 1975 appeared aspergilloma in the right middle lung field. He was administrated intravenous and oral anti-fungal agent and transbronchial installation of Amphotericin-B because of hemoptysis. Chest radiographs in July 1980 resolved the aspergilloma and his symptom were also resolved. In 1996, he had no pulmonary symptoms and respiratory failure. We consider that the Kartagener's syndrome was good prognosis with adequate pulmonary therapy.     

Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group

STUDY OBJECTIVE: To study the safety and efficacy of aerosolized recombinant human DNase I in the treatment of idiopathic bronchiectasis. DESIGN: Double-blind, randomized, placebo-controlled, multicenter study. POPULATIONS: Three hundred forty-nine adult outpatients in stable condition with idiopathic bronchiectasis from 23 centers in North America, Great Britain, and Ireland. INTERVENTIONS AND MEASUREMENTS: Study patients received aerosolized rhDNase or placebo twice daily for 24 weeks. Primary end points were incidence of pulmonary exacerbations and mean percent change in FEV1 from baseline over the treatment period. RESULTS: Pulmonary exacerbations were more frequent and FEV1 decline was greater in patients who received rhDNase compared with placebo during this 24-week trial. CONCLUSIONS: rhDNase was ineffective and potentially harmful in this group of adult outpatients in stable condition with idiopathic bronchiectasis. This contrasts with previously published results that demonstrated efficacy of rhDNase in patients with cystic fibrosis bronchiectasis.     

Systemic amyloidosis in cystic fibrosis

We report two siblings with cystic fibrosis and systemic amyloidosis. The major clinical problem in both cases was recurrent respiratory infection with pulmonary fibrosis and bronchiectasis prior to death at ages 20 and 22 years. Findings from postmortem examinations disclosed diffuse amyloidosis. In addition, amyloid infiltration developed in both patients, with enlargement of the thyroid gland, and one required thyroidectomy. An autopsy review of 17 additional cases of cystic fibrosis failed to disclose any other instances of systemic amyloidosis.     

1990-1996: the experience of the La Fe Lung Transplant Group (Valencia)

Objective to review the experience of the lung transplantation unit at Hospital La Fe (Valencia). Between February 1990 and March 1996 we performed 40 lung transplants. The following causes were most common: cystic fibrosis (9 cases), emphysema (8), pulmonary fibrosis (8) and bronchiectasis (7). Types of intervention were 27 double lung transplants (25 sequential and 9 blocked), 9 single lung transplants, and 4 heart-lung transplants. We then reviewed the 36 single and double lung transplants. The main exclusion criteria were age over 65 years, malignant disease, kidney or liver disease, severe or non reversible central nervous system disease, and drug addiction. Prior surgery, mechanical ventilation and the presence of Aspergillus were considered lower-order contraindications. Mean patient age was 37.7 years (14-59). Six patients were colonized by Aspergillus before transplantation. Five had undergone earlier surgery and two were mechanically ventilated before the transplant. The most common complication was respiratory infection, which was present in 6 of the 7 patients who died. Other complications in order of frequency were dehiscence and/or bronchial stenosis, corticoid myopathy and postoperative bleeding. The actuarial survival rate of single and double lung transplants was 67.85 after 3 years, and 87.5% in patients with cystic fibrosis. Lung transplantation is a well-established procedure that is gradually being extended to treat more conditions. The main obstacle is the scarcity of donors. The main challenge at present is bronchiolitis obliterans.     

Renal agenesis associated with homolateral ovarian dysplasia. A case report]

This case concerns an arc welder who presented suppurative bronchiectasis and episodes of purulent left side pleurisy in relation to cystic bronchiectasis of the left lower lobe and a very severe stenosis at the origin of the main left bronchus. The medicolegal problem was to assess the causal relationship between these lesions and occupational exposure. They do not come under the heading of table 44 of the General List and we made this the aim of discretionary award in front of a regional committee of compensation for occupational disease.     

Successful treatment of gemcitabine toxicity with a brief course of oral corticosteroid therapy

Gemcitabine is a nucleoside analog that is useful in the treatment of solid tumors. Its use has been postulated to produce lung injury by causing a capillary leak syndrome. We describe a gemcitabine-treated female patient who developed severe dyspnea, diffuse pulmonary infiltrates, and hypoxia, with evidence of interstitial disease on pulmonary function tests. Following the administration of oral corticosteroids, she had complete resolution of all signs and symptoms of gemcitabine toxicity. Physicians should be aware of this treatable complication of gemcitabine therapy.     

The role of fibrinogen degradation products in the pathogenesis of the respiratory distress syndrome

Pulmonary dysfunction in awake rabbits was induced by intravenous infusion of a highly purified human fibrin split product (fragment D). The dose of infused fragment D was chosen to achieve observed plasma concentrations of fibrin split products in hospitalized patients with severe burns or trauma (about 100mug of FSP/ml of blood). Four hours after infusion, the animals displayed a clinical and pathological pattern which closely resembled post-traumatic acute respiratory distress syndrome, including hypoxia, hypocarbia, thrombocytopenia, increased pulmonary capillary permeability to albumin, interstitial edema, hypertrophy of alveolar lining cells, and intra-alveolar hemorrhage. In vivo production of fibrin split products by infusion of thrombin with induction of secondary fibrinolysis produced similar pulmonary changes, although intravascular clots and platelet aggregates also were prominent. Infusion of human fibrinogen and human albumin at identical doses failed to induce pulmonary dysfuction. The results suggest that fibrin split products (fragment D) alone are toxic to the respiratory system and may contribute to the development of acute respiratory distress syndrome in severely traumatized or burned patients.     

Comparison between morphologic changes seen on high-resolution CT and regional pulmonary perfusion seen on SPECT in patients with cystic fibrosis

OBJECTIVE: To evaluate the relationship between morphologic findings seen on high-resolution computed tomography (HRCT) of the lung and regional lung perfusion depicted on single photon-emission computed tomography (SPECT) pulmonary perfusion imaging in patients with cystic fibrosis. MATERIALS AND METHODS: Ten HRCT and 10 technetium-99 m macroaggregated albumin SPECT pulmonary perfusion imaging studies were performed on eight young adult patients who were considered to be clinically well and have mild to moderate cystic fibrosis. HRCT scans of the chest were evaluated using a CT scoring system which included grading of bronchiectasis, peribronchial thickening, hyperlucency, bullae, collapse/consolidation, and mucus plugging. Each lung was divided into six anatomic zones which were independently scored. A lung perfusion score (between 0 and 100), reflecting the percentage of compromised lung, was estimated for each zone. Axial lung perfusion SPECT images were matched anatomically to HRCT images. Lung function was considered compromised when the counts per pixel were less than 25 % of the count level seen in an area of the same patient's lung which was judged to be normal. RESULTS: There was a statistically significant relationship (P = 0.0001) between HRCT total scores and SPECT lung perfusion scores as well as between hyperlucency scores by HRCT and the SPECT lung perfusion scores. However, the HRCT score was a poor predictor of the lung perfusion score in zones with intermediate HRCT scores, which constituted 106 of 120 zones. CONCLUSION: Morphologic changes depicted by HRCT correlate with decreased lung pefusion on SPECT. However, HRCT changes accurately predict regional lung function only in the most normal and severely diseased lung zones.     

Marked pulmonary function abnormalities in a case of HIV-associated pulmonary hypertension

Recent reports have suggested an association between primary pulmonary hypertension and human immunodeficiency virus (HIV) infection. This appears to be an accelerated syndrome, associated with a relatively brief duration of symptoms, yet prominent right ventricular failure and severe pulmonary hypertension on presentation. We present a case of a primary pulmonary hypertension in a 35-year-old HIV-seropositive hemophiliac. His accelerated clinical course is consistent with previously reported cases of HIV-related pulmonary hypertension. However, this patient's pulmonary function tests revealed marked hyperinflation, a decreased diffusing capacity, and no airflow obstruction. To our knowledge, this very usual constellation of pulmonary function changes has not been described previously in this syndrome.     

Cognitive, adaptive, and behavioral characteristics of Williams syndrome

The difference of morphological injury between rabbit aorta and pulmonary artery was compared after the animal was exposed to the altitude 5 km (PO2 = 10.8 kPa) for 24 h. Hypoxia caused subendothelial edema, increased vacuoles and injured mitochondria and endoplasmic reticulums in both kinds of endothelial cells. The impairment of pulmonary artery was obviously more severe than aorta and its smooth muscle cells were also affected. Forthermore, the exposure increased mitochondria in pulmonary artery endothelial cells. Bubbled with a mixture air of 95% N2-5% CO2 (PO2 = 4 kPa) led to an increase of pulmonary in tension, while hypoxia to the same extent induced aorta relaxation. These results indicate that hypoxia produces the differential effects on these two kinds of vessels, providing a possible explanation for the production of hypoxic pulmonary hypertension.     

Lobular low attenuation of the lung parenchyma on CT: evaluation of forty-eight patients

PURPOSE: Our goal was to analyze patients with pulmonary lobular low attenuation (LLA) on thin section CT with regard to the underlying pulmonary diseases and the dynamic changes occurring in the low attenuation regions during respiration. METHOD: Forty-eight consecutive patients with LLA on thin section CT were analyzed retrospectively. Forty-six patients (95%) had symptoms related to respiratory disease, such as productive cough (n = 25) and hemoptysis (n = 18). Only two patients, one with chronic pulmonary embolism and one with Takayasu arteritis combined with bronchiectasis, had pulmonary vascular disease. Six patients, four with bronchiectasis and two with vascular disease, were studied with dynamic CT during forced vital capacity maneuver. Attenuation values for LLA areas and adjacent lung were measured and time-density curves were plotted. RESULTS: Forty-one (85%) patients had bronchiectasis, typically in other than the regions of the LLA. Areas with proximal bronchiectasis showed low attenuation but without notable lobular distribution. Pulmonary vessels in the LLA areas were smaller than those of adjacent normal lung (n = 45). Of 22 patients who underwent pulmonary function tests, 15 had obstructive pattern of impairment. Respiratory dynamic CT showed expiratory air trapping in LLA areas in all six patients. The mean attenuation values of LLA areas were lower than those of the adjacent normal lung by 67 HU at end-inspiration and by 165 HU at end-expiration (p = 0.002). CONCLUSION: The majority of the patients with LLA shown by thin section CT had bronchiectasis elsewhere in the lung, and evidence of air trapping in the LLA was clearly demonstrated. Bronchiolar obstruction may be the most prevalent cause for the development of LLA.     

Spiral CT angiography in an infant with severe hypoplasia of a long segment of the descending aorta

Annuloaortic ectasia due to Shprintzen-Goldberg syndrome (SGS) is reported. A 10-year-old boy was admitted to our hospital for evaluation of chest pain. On admission, he was diagnosed as SGS on the basis of his various anomalies. Two-dimensional echocardiography showed a bicuspid aortic valve and marked annular dilatation, Doppler flow studies revealed severe aortic regurgitation, and retrograde aortography showed severe aortic regurgitation with annular dilatation. Successful aortic root replacement was performed; subsequent histologic examination of the ascending aorta demonstrated cystic medial necrosis. In conclusion, SGS is a generalized connective tissue dysplasia, with clinical manifestations of cardiovascular lesions similar to those in Marfan syndrome. Aortic root replacement was successfully performed; however, recurrence of aortic aneurysms outside of the ascending aorta should be carefully observed. Surgical treatment for cardiovascular disorders may be necessary to save the life of patients with SGS.     

How do cystic fibrosis transmembrane conductance regulator mutations produce lung disease?

In recent years, several functions of the cystic fibrosis transmembrane conductance regulator have been discovered, yet the pathophysiology of the pulmonary disease in cystic fibrosis remains unclear. At the cellular level, functions of this protein include regulation of chloride and sodium transport at the cell membrane and in intracellular organelles, regulation of protein trafficking, and posttranslational processing of glycoconjugates. Elucidation of these functions has led to several hypotheses to account for how defects in the cystic fibrosis transmembrane conductance regulator produce pulmonary disease, but a clear understanding of the pathophysiologic links between the cellular functions of the cystic fibrosis transmembrane conductance regulator and organ dysfunction has been hampered by the lack of ideal model systems. Current evidence suggests that defects in the cystic fibrosis transmembrane conductance regulator lead to alterations in periciliary fluid homeostasis, mucus hydration, mucin secretion, and apical membrane protein structure. In turn, these alterations impair mucociliary clearance and promote bacterial infection, which then leads to chronic airway inflammation and the development of bronchiectasis.     

Indicator cell lines for detection of primary strains of human and simian immunodeficiency viruses

The pulmonary artery pressure (Ppa) responses to short runs of acute hypoxia at two different levels of end-tidal CO2 were measured in nine normal subjects and in 20 patients with moderate to severe obstructive sleep apnea (OSA). In normal subjects the mean increase in Ppa in response to eucapnic hypoxia was 8 +/- 2 mm Hg (SEM) and was not different from the response to hypercapnic hypoxia (9 +/- 2 mm Hg, p > 0.2). In patients with OSA, the mean increase of Ppa was 8 +/- 1 mm Hg to eucapnic hypoxia, and the response to hypercapnic hypoxia was higher at 10 +/- 1 mm Hg (p = 0.01). Pulmonary pressor response to hypoxia was augmented (> 10 mm Hg) by hypercapnia in four of 20 patients with OSA but in none of the normal subjects. Normoxic hypercapnia alone was a weak stimulus, increasing Ppa by > 5 mm Hg in only two of nine patients with OSA studied. In conclusion, Ppa increases in both normal subjects and patients with OSA exposed to a ramp of acute isocapnic hypoxia. There were clear interindividual differences in pulmonary artery response. Hypercapnia did not produce clinical significant changes in Ppa in either group.     

Effects of hypoxia and anisodamine on products of TXA2 and PGI2 in cultured intra-pulmonary arteriolar smooth muscle cells

The changes of contents of TXB2 and 6-Keto-PGF1a were studied in severely acute hypoxic cultured intra-pulmonary arteriolar smooth muscle cells (PASMCs) under the action of anisodamine. The results demonstrated that the contents of TXB2 and 6-Keto-PGF1a and their ratio were significantly increased in severe acute hypoxic PASMCs' medium. The content of TXB2 decreased significantly, but the content of 6-Keto-PGF1a was hardly affected by anisodamine under normoxia and hypoxia. These findings suggest that acute and severe hypoxia results in pulmonary vascular constriction through increased production of PASMCs and liberation of TXA2, or PGI2, and increased TXA2/PGI2 ratio. The latter effect of hypoxia could be prevented by anisodamine, which antagonized the effect of hypoxia induced pulmonary vasoconstriction.     

High seroprevalence of Helicobacter pylori in active bronchiectasis

Helicobacter pylori causes chronic inflammation of the gastric mucosa and has been identified in tracheobronchial secretions. Serum IgG against H. pylori was therefore measured prospectively in consecutive subjects with bronchiectasis (n = 100; mean age +/- SD 55.1 +/- 16.7 yr), active pulmonary tuberculosis (n = 87; age, 57.3 +/- 19.1 yr), and healthy volunteers (n = 94; age, 54.6 +/- 7.6 yr). Seropositivity was found in 76.0% of bronchiectatic subjects, which was significantly higher than that of the control (54.3%, p = 0.001) and tuberculous (52.9%, p = 0.0001) groups. Multiple logistic regression, adjusted for age, sex, occupational social class, and number of persons living in the household, showed that H. pylori IgG levels of the bronchiectatic group were still significantly higher than that of the control (p = 0.0014) and tuberculous (p = 0.0154) groups. Multiple regression analysis revealed associations between H. pylori serology and sputum volume (p = 0.03) and age (p = 0.001) in the bronchiectatic patients, but not lung function indices or causes of bronchiectasis. The H. pylori seroprevalence in bronchiectasis was significantly (p = 0.0002) higher in patients who produced more (83.1%) than those who produced less than 5 ml sputum/24 h (58.6%). This is the first report of a high H. pylori seroprevalence in bronchiectasis which appears to be specific. Further studies are indicated to evaluate the possible pathogenic role of H. pylori in bronchiectasis.