Abstract: Pulmonary disease is recognized to occur as a complication of Sjogren's syndrome and we have studied 17 patients, the majority middle-aged women, with this association. Eight had sicca syndrome alone and nine had Sjoren's syndrome in association with a connective tissue disease. Various pulmonary disorders were found: 10 had diseases mainly affecting pulmonary parenchyma (recurrent pneumonia, granulomatous infiltration, pseudolymphoma and fibrosing alveolitis) and six had disorders of the airways (bronchitis, bronchiectasis and asthma). One patient presented with a mediastinal immunoblastic lymphoma. To test the hypothesis that physical factors such as the retention of viscid mucus may play a part in the development of lung disease in Sjogren's syndrome, we have measured mucociliary clearance in patients with Sjogren's syndrome using 99Tc-labelled polystyrene particles. The mucociliary clearance rate was found to be normal from central airways and the whole lung clearance rate was significantly increased in Sjogren's syndrome compared with normal controls. The latter was attributed to the failure of penetrance of the radioaerosol into the periphery of the lung consistent with obstruction of small airways in Sjogren's syndrome. The patients with Sjogen's syndrome and lung disease were characterized by a number of immunological markers irrespective of the presence or type of associated connective tissue disorder. The commonest found were speckled antinuclear antibody by indirect immunofluorescence, antibodies to the acidic nuclear antigen B by counterimmunoelectrophoresis, increased serum DNA binding, increased levels of circulating immunoglubulins, rheumatoid factor in high titre and circulating soluble immune complexes as detected by Clq binding assay.

Abstract: Clear guidelines for the selection of patients with large pulmonary bullae and severely impaired lung function for surgery remain to be defined. Twenty-one such patients operated on between 1971 and 1977 are reviewed in an attempt to shed some light on this difficult problem. Four of six patients with preoperative hypercapnia survived and were improved by surgery. There was no mortality among the remaining 15 patients of whom 14 were improved symptomatically by surgery (with improvement in FEV1 and vital capacity in 9). Preoperative bronchograms were used to help identify patients suitable for surgery. The presence of bronchiectasis was predictive of postoperative complications. Better results were obtained in those patients in whom plication of bullectomy could be performed than in those requiring lobectomy.

Abstract: Kartagener's syndrome is a condition that consists of situs inversus, bronchiectasis, and sinusitis. Some patients have respiratory symptoms that date from early infancy, and electron microscopical examination has shown that adults with this condition lack dynein arms in ciliary microtubules. It has been suggested that an inherited defect in ciliary ultrastructure, the immotile cilia, is the basis for the syndrome. We report 6 patients who presented within the first 24 hours of life with tachypnoea, retraction, rales, and dextrocardia. Five of them had situs inversus. They all responded to physiotherapy after antibiotics had failed to improve their condition. Electron microscopical examination of the cilia in 2 of the infants confirmed deficiency of dynein arms. All 6 infants had recurrent respiratory infections. Four went on to develop recurrent otitis media, 2 had sinusitis, and 1 now has bronchiectasis. The immotile cilia syndrome should be added to the list of causes of respiratory distress in newborn infants. The condition is worth diagnosing because of the good response to physiotherapy, and because of the likelihood of recurrent respiratory problems.

Abstract: Anomalies of the bronchial cilia were studied in 5 children with recurrent pulmonary infections. Case 1 had Kartagener's syndrome and an absence of the inner and outer dynein arms in most cilia, although a few shortened and even some normal arms could be seen. Cases 2 and 3 had unilateral bronchiectasis without family history of Kartagener's syndrome. Serial studies of the bronchial epithelium at times showed a bilateral lack of the inner dynein arms and a partial lack of outer arms. These abnormalities persisted in these 2 children after they had recovered from the acute pulmonary infection but disappeared after 6-8 months of antibiotic treatment. Cases 4 and 5 had recurrent pulmonary infections without bronchiectasis and many shortened outer dynein arms could be seen, but these anomalies disappeared after recovery. In all 5 children such architectural ciliary anomalies were present as megacilia, fused cilia, naked cilia, and completely disorganised axonemas. These architectural defects were particularly numerous in the children without bronchiectasis. Our observations suggest that anomalies of the bronchial ciliary microtubular system may not only be congenital but may also be acquired; this might well help to explain some cases of repeated respiratory tract infection and bronchiectasis.

Abstract: Radiographs of 50 patients age 17 or more with documented cystic fibrosis were reviewed. Peripheral nodular and nonvascular linear densities were common early abnormalities. Specific findings of bronchiectasis were found in 90% of all cases. Hyperinflation was seen in 76% of cases, especially in the lower lobes; atelectasis and all other abnormalities were more common in the upper (and middle) lobes. Cystic air spaces developed in 24% of cases. The severity of abnormalities (including hyperinflation, atelectasis, and bronchial changes) increased in 30 of the 39 patients with follow up for a year or more. Eight of the 15 patients who died came to autopsy. The lungs showed acute and chronic inflammation of airways, including peripheral bronchioles, with adjacent parenchymal inflammation of airways peripheral bronchioles, with adjacent parenchymal infiltrates and fibrosis. The surrounding alveoli were aerated and enhanced the visibility of the thick-walled airways, except in regions of lobar atelectasis, sca...

Abstract: Krypton 81m ventilation and technetium 99m perfusion lung scans in anterior, posterior and oblique views in 86 children (age range 14 days to 15 years) with various paediatric problems were obtained. On reviewing these studies we found four main areas of clinical usefulness, (a) Establishing the diagnosis; in a relatively small number of patients the lung scan was essential for either establishing the exact diagnosis or directing attention to the abnormal area. (b) Refuting a diagnosis: the two main groups in this category include possible bronchiectasis and inhaled foreign body. (c) Establishing the extent of the disease; radionuclide studies enable one to assess and follow up the extent of the disease in children with lower respiratory problems; a lung scan may obviate the need for bronchography in bronchiectatics failing to respond to medical treatment and for whom surgery is being considered. Repeat studies are useful in following the natural history or the response to treatment of various lu...

Abstract: Secondary amyloidosis has been described in association with a large number of chronic diseases. Although viscidosis is associated with chronic suppuration, the presence of amyloidosis as a complication has been reported in only three cases.1.2Here we present a new case, to our knowledge the third in the English literature. Report of a Case.—A male patient received a diagnosis of cystic fibrosis based on repeated respiratory infections, steatorrhea, and a positive sweat test result (chloride content, 110 mEq/L) at 6 years of age. Two siblings died of bronchopulmonary processes when they were a few weeks old. He suffered from frequent and prolonged respiratory infections. The chest roentgenogram gave evidence of the existence of bronchiectasis. When he was 11 years of age, an increase in the thyroid size was noticed, and this led to treatment with strong iodine solution. The thyroid size continued to increase gradually. When the patient

Abstract: The radiographic appearances of inflammatory lung disease in the horse are presented under three major morphologic categories: (a) parenchymal diseases, (b) airway diseases, (c) pleural diseases. Under the category of inflammatory parenchymal disorders, pneumonias of bacterial, viral, mycotic, parasitic, and immunologic origins are discussed. Airway disorders described included bronchitis, bronchiolitis, bronchiectasis, and emphysema. Pleural effusion as well as less common manifestations of pleural disease, are also discussed.

Abstract: Cefuroxime and ampicillin were compared in a randomized double-blind trial in the treatment of severe lower respiratory tract infections. 750 mg of cefuroxime were given to 57 patients and 500 mg of a

Abstract: Sera from patients with chronic lung diseases were tested for the presence of immune complexes (ICs) by the 125I-C1q-binding assay. Contrary to earlier reports, modification of the test system by addition of heparin decreased rather than increased the ability of the test to discriminate between control and pathological sera. Using the unmodified system, elevated C1q-binding activity (C1qBA) was found in patients with asthma (18%), chronic bronchitis (18%), sarcoidosis (18%), fibrosing alveolitis (50%), bronchogenic carcinoma (52%) and bronchiectasis (67%). Studies with the reducing agent 2-mercaptoethanol (2-ME) suggested a role for IgM rheumatoid factor (RF) and/or IgG-containing complexes in the C1q-reactive material of sera from patients with bronchiectasis and bronchogenic carcinoma. In the latter two groups, C1qBA was found to correlate with serum levels of IgG and IgA but not with C3 and C4. A weak condition between levels of C-reactive protein (CRP) and C1qBA was found in the bronchogenic carcinoma group. Carcinoembryonic antigen (CEA) levels were elevated in all groups studied but no correlation with C1qBA was demonstrated, suggesting that CEA and CEA-ICs, if present, do not have an influence on the C1qBA of such sera. The results indicate that elevated serum C1qBA is a concomitant of both chronic inflammatory and neoplastic diseases of the lung but the extent of any similarity in the non-immunoglobulin components of the immune complexes in the respective conditions remains unknown.

Abstract: Five patients with histories of resectional lung surgery presented with recurrent or persistent cough. Lobectomy or pneumonectomy had been done for carcinoma (three patients), bronchial adenoma (one patient), and bronchiectasis (one patient). The cough in each case was first interpreted as being due to recurrence of the original disease. Bronchoscopy excluded recurrent disease and led to the discovery of exposed endobronchial sutures. Elimination of the sutures spontaneously in one patient and removal by bronchoscopy in four patients alleviated the cough. Exposed sutures apparently cause cough by producing local irritation, granuloma formation, and infection. This cause should be considered in the differential diagnosis of cough in the post-thoracotomy patient.

Abstract: The case records of 23 patients with Kartagener Syndrome in the past 10 years were reviewed. For diagnostics bronchoscopy, bilateral bronchography, lung function investigations and angiopulmonography were done. Their immunologic and genetic aspects were studied. Lung resections were performed in 17 patients, 5 of them underwent bilateral pulmonary resections. The lung specimens were investigated morphologically. As top clinico-morphologic features bronchiectasis in patients with situs inversus viscerum did not differ significantly from that without accompanying diseases. Unfavourable hereditary, dyskinesia of bronchial tree, paranasal sinus underdevelopment provoked inflammatory processes of bronchi and development of bronchiectasis.

Abstract: Cefamandole 3 g daily gave poor results in the treatment of severe purulent respiratory infections in 50 patients. A further 71 patients were treated with 4 g daily and the results were excellent with 86% clinical success. Apart from muscle pain with the injection, side-effects were minimal.

Abstract: Four girls born to second cousin parents developed chronic chest infection and bronchiectasis in infancy. Three were studied in detail: they all had the same HLA haplotype, all showed random orientation of cilia or compound cilia in the respiratory tract, and all had low levels of the C1 and C2 components of the complement system. Although the cause of the respiratory disease in this family remains unclear, it is suggested that the low C1 levels may have contributed to the disease in two of the children while the low C2 levels were artefacts and the ciliary abnormalities were secondary to chronic chest infection.

Abstract: Unfamiliar manifestation of pulmonary tuberculosis found in Central and West Africa are presented These include egg shell calcification in parenchyma and glands, calcification of the diaphragmatic pleura resembling asbestosis, honeycombing and B Kerley's lines with or without complicating bronchiectasis There should be an index of suspicion of pulmonary tuberculosis even when other typical tuberculous lesions are not associated with these lesions not normally attributed to the disease

Abstract: Six healthy children, 5 boys and 1 girl aged from 13 months to 4 years had a severe measles bronchopneumonia. The end result is established chronic airflow obstruction. On a background of fairly severe respiratory failure there were paroxysms of breathlessness with fever, wheezes and cyanosis which were little affected by bronchodilators or steroids. The anatomical basis of this syndrome is before all else a bronchiolar obstruction, shown at autopsy in a child dying after four months of the illness. But also there is a disorder of the large bronchi with bronchiectasis and problems with ventilation centrally and/or peripherally. The major radiological signs are airways distension which is always clearly in keeping with emphysema and a thickening of the peri-bronchial walls which are clearly visible on tomography. Thus it appears that measles, as with other respiratory viruses, can lead to permanent sequelae in very young children. It is however possible that the respiratory sequelae may be due to associated viral infections, with adenovirus in particular. On the practical level, the occurrence of an early and severe measles pneumonia in a young child and its persistence with a hypoxia and a lowering of dynamic compliance requires a prolonged follow up and a guarded prognosis. Unfortunately the treatment of this type of chronic airflow obstruction is only symptomatic.

Abstract: Bronchi are visible in the lung field radiologically in various conditions. One special condition manifesting abnormal shadow due to bronchial involvement is the replacement of the air in the bronchi by water-dense material. This type of abnormal shadow has several characteristic features, such as beeing Y or W shaped, pointing its tip toward the hilum, and fanlike distribution, because the abnormalities are based on bronchi themselves. Until recently, only two water-dense materials which produce abnormal shadow were known, namely mucus and pus. When bronchial secretion is retained and inspissated in the bronchial lumen, bronchial mucocele and mucoid impaction is the term applied for this condition. These cases are seen in bronchial atresia, allergic broncho-pulmonary aspergillosis, bronchial stenosis. Mucus retention is also caused by complete bronchial obstruction in bronchogenic carcinoma. When bronchial secretion is infected, such as in bronchiectasis and mucoviscidosis, bronchial pyocele is used for this condition, and gloved-finger or tooth-paste shadow is the roentgenological expression. Recently, we experienced cases with abnormal shadow due to impaction of cellular component in the bronchi and blood retention in the bronchial lumen.

Abstract: Nasal ciliary function and mucociliary clearance were studied in patients with cystic fibrosis and in three control groups. Ciliary beat frequency and nasal clearance time were measured in groups of 10 subjects with cystic fibrosis, sinusitis and bronchiectasis and age and sex-matched control subjects. Ciliary beat frequency was also measured in normal subjects matched as bronchiectasis controls. Cystic fibrosis patients and their controls, patients with sinusitis, and the bronchiectasis controls did not differ in ciliary beat frequency, but it was slower in the patients with bronchiectasis (p less than 0.05). Nasal mucociliary clearance in cystic fibrosis and bronchiectasis was slower than in the cystic fibrosis controls (p less than 0.001) and in patients with sinusitis (p less than 0.01). The finding of a normal beat frequency in cystic fibrosis cilia studied in vitro together with abnormal nasal mucociliary clearance measured in vivo in the same patients suggests the existence of an abnormality of mucus in vivo. The innate function of cystic fibrosis cilia, as measured in vitro by beat frequency, is normal.

Abstract: Seven patients with severe, unexplained chronic bronchial suppuration and inflammatory bowel disease are reported. In three, rapidly progressive bronchiectasis developed within one year of procto-colectomy, in two it developed in association with an exacerbation of colitis and in the other two a milder, limited colitis post-dated the start of the lung disease. The high incidence of arthropathy and skin rashes suggests a systemic disease, and personal and family histories of auto-immune conditions were common. Antinuclear antibodies were detected in six and smooth muscle antibodies in five of the seven patients and there was no evidence of liver disease. Evidence of circulating immune complexes was found in two patients. In the four patients who received corticosteroid therapy there was an impressive improvement in cough, sputum volume and dyspnoea, especially when taking high doses. Because of the time relationships of the bowel and lung symptoms, we suggest that these disorders may be related.

Abstract: Ulcerative colitis has many systemic manifestations, such as erythema nodosum and iritis, and is now recognised as a multi-system disease. A patient is reported in whom total colectomy for ulcerative colitis was followed by development of autoimmune haemolytic anaemia, Hashimoto's disease, and pulmonary bullae and bronchiectasis. The association of these conditions with ulcerative colitis and the similarity of their immunological mechanisms are discussed.