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At least 667 records · Page 37Linked to original sources
PubMed1994

[A case of bronchiectasis accompanied by ulcerative colitis (UC) and HTLV-1 associated myelopathy (HAM)].

We report a case of bronchiectasis with marked thickening of the respiratory tract wall occurring in a 37-year-old man with UC and HAM. He was diagnosed as UC at age 20. HTLV-1 was presumably transmitted to this patient by means of a blood transfusion he received at around age 30. On admission, chest X-ray films, tomography and CT-scan revealed dilated lumens and thickened airway walls extending from the trachea to subsegmental bronchi. Pulmonary function tests showed hypoxemia and mixed ventilatory disturbance with a predominantly obstructive component. HAM was diagnosed on the basis of neurological examination and cerebrospinal fluid analysis. A biopsy specimen from the carinal mucosa showed marked T cell infiltration. In these T cells, we detected polyclonal integration of HTLV-1 proviral DNA. Some of the infiltrating T cells showed atypia. In recent times, respiratory diseases other than infiltration of adult T-cell leukemia cells or opportunistic infection have been reported in HTLV-1 carriers and new clinical entities designated as HABA (HTLV-1 associated bronchiolo-alveolar disorder) and HBA (HTLV-1 associated bronchopneumopathy) have been proposed. This case is classified among these new entities, in a broad sense, and is a rare case in that the respiratory disorder is apparently related to UC.

Adult↗
PubMed1993

[Bronchiectasis in early childhood. Its surgical treatment].

We discuss the measures used, including physical examination, radiology, scintigraphy, T.A.C. and bronchography, to diagnoses 34 patients with bronchiectasis. In addition, the treatment used up until the time where the decision to perform surgery is also presented. Ten patients were submitted to surgical intervention and their operations, as well as the effected lobes, are described. The guidelines for surgical preparation, surgical difficulties encountered, postoperative care and the results of the surgeries are all discussed. Due to the age of some of these patients, obvious difficulties prevented functional testing. In those patients in whom it was possible, no loss in respiratory capacity has been observed.

Bronchi↗
PubMedDate not supplied

[Left pneumonectomy in a child with congenital bronchiectasis].

A case of diffuse bronchiectasis concerning an entire lung in an eight-year-old boy is described. The disease in this case was widespread in the entire left lung and was considered as consequent to a defect in the development of the left bronchial wall. Because of the frequent respiratory infections, occurring since birth, and given the good anatomical and functional conditions of the right lung, we recommended the surgical excision of the sick lung. After one year, the operation showed good results with regard to respiratory functions. The functioning of the remaining pulmonary parenchyma is quite satisfactory and allows the boy an excellent quality of life.

Bronchi↗
PubMed1977

[Postural drainage and breathing gymnastics--conditiones sine qua non-before lung resections for bronchiectasis (author's transl)].

Report on 150 lung resections in 143 patients with bronchiectasis in the period of 1958-1974. The intensive preparation of the patients before surgery is of highest importance for a small risk and rare complications after operation. Postural drainage and breathing gymnastics are able to increase significantly FEV1 and VC and to reduce RV in the preoperative phase. The complication rate amounted to 8%, lethality after surgery to 1.3%.

Breathing Exercises↗
PubMedDate not supplied

CFTR gene mutations in adults with disseminated bronchiectasis.

The severity and type of clinical manifestations are variable in patients with cystic fibrosis (CF). The respiratory syndromes in these patients consist of lung infections associated with disseminated bronchiectasis (DB), asthma, and chronic obstructive pulmonary disease. To investigate the possible involvement of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in chronic pulmonary disease in adults, we studied 32 DB patients with a clinically isolated respiratory syndrome. Careful analysis of all the CFTR gene exons and their flanking regions revealed a significantly increased frequency of CFTR gene mutations in these patients. Thirteen CFTR gene mutations were identified in sixteen different alleles. Six of these mutations, which have previously been reported as CF defects, were found on nine alleles. A further four, two of which had not previously been described (D192N and 406-2 AdeltaC), are potentially disease-causing mutations. We also identified three rare substitutions (R31C, L997F, T1220I), which could be involved in mild CFTR gene disease. Four patients were compound heterozygotes, one carried two CFTR gene mutations (possibly allelic) and six were heterozygous for a mutation. These results indicate that CFTR gene mutations may play a role in bronchiectatic lung disease, possibly in a multifactorial context. These findings have implications for genetic counselling of DB patients and their families.

Adult↗
PubMed1976

Bronchiectasis in yearling feedlot cattle.

Thoughout all of 1974, we surveyed, for illnesses and deaths, about 407,000 yearling feedlot cattle. From the 3,943 dead cattle, 1,988 necropsies were made; of the cattle necropsied, 32 (1.6%) had bronchiectasis. In this disease, the permanently dilated small bronchi and bronchioles, located in ventral parts of the lungs, were filled with accumulations of exudate and microorganisms, including Pasteurella hemolytica, Pasteurella multocida, Corynebacterium pyogenes, Escherichia coli, Salmonella anatum, Staphylococcus spp, and Mycoplasma arginini.

Animals↗
PubMed1998

Chronic bronchiectasis and anti-myeloperoxidase antibody related rapidly progressive necrotizing glomerulonephritis.

We reported two cases of chronic bronchiectasis and rapidly progressive necrotizing glomerulonephritis/severe renal failure which were also positive for anti-myeloperoxidase antibody, and followed their treatment and outcome. Immunosuppressive therapy was complicated by superimposed chest infection in both cases. Nonetheless, cautious use of immunosuppressive and antibiotic therapy reversed dialysis-dependent renal failure in one of the two cases.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2003

Situs inversus, bronchiectasis, and sinusitis and its relation to immotile cilia: history of the diseases and their discoverers-Manes Kartagener and Bjorn Afzelius.

The relationship of Kartagener's syndrome to immobile cilia syndrome is a fascinating merging of clinical observations and basic science in Zurich, Stockholm, and Toronto. In 1933, Manes Kartagener, a Zurich pulmonary physician, reported four patients with the triad of sinusitis, bronchiectasis, and situs inversus. In the following decades, he reviewed reports of hundreds of cases, but the fact that the male patients with the condition never had offspring eluded his notice. In the 1970s, Bjorn Afzelius, a Ph.D. ultrastructuralist from Stockholm, reported cilia immotility in infertile males, some of the cases occurring in families. Half of the cases had Kartagener's triad. The observation of Afzelius was soon applied to children by Jennifer Sturgess, a Ph.D. ultrastructuralist, and her medical colleagues in Toronto. With over 500 MEDLINE references since 1966 on Kartagener's and over 1,000 references on immotile cilia, the causes of the pulmonary infections have become clearer as the patients demonstrate impaired clearance of mucus with resultant sinus and bronchial disease. The cause of the situs inversus remains elusive to this day. It is appropriate to call the condition Kartagener-Afzelius syndrome.

Canada↗
PubMed2003

Macrolides as biological response modifiers in cystic fibrosis and bronchiectasis.

For 50 years, macrolide antibiotics have been used to treat community acquired pneumonia and atypical infections such as Chlamydia pneumonia and Mycoplasma. In the late 1960s it was noted that when the 14-member ring macrolide antibiotic troleandomycin was given to asthma patients who required large doses of systemic corticosteroids, they could often reduce their steroid dose or even stop steroids completely without exacerbation of their asthma. Because of this experience, Prof. S. Kodoh and colleagues first used erythromycin as an immunomodulatory agent to treat diffuse panbronchiolitis (DPB). DPB is a cystic fibrosis (CF)-like condition seen predominantly in young, nonsmoking adults in Japan and Korea. The introduction of erythromycin profoundly improved survival, and in many of these very ill patients the illness disappeared. Since then, research has focused attention on many non-antibacterial, disease modifying effects of this class of compounds. These include downregulation of proinflammatory cytokines via an effect on nuclear transcription factors, reduction in adhesion molecule expression, suppression of inducible nitric oxide synthase (iNOS), reduced neutrophil chemotaxis and degranulation, inhibition of neutrophil elastase, cytoprotection against bioactive phospholipids, improvement in the rheological properties of mucus, reduction in bronchial hyperreactivity, and, perhaps, modulation of neutrophil death by apoptosis pathways, and in the end, airway remodeling. Additionally, they have unconventional effects on microorganisms, including inhibiting Pseudomonas aeruginosa twitching motility and thus biofilm formation. There are small case series and three large randomized controlled trials that have established unequivocal evidence of benefit in CF. There is less evidence for an immunomodulatory effect in bronchiectasis. Future work is likely to focus on the development of macrolides with disease-specific modes of action.

Journal Article↗