Increased augmentation index in patients with cystic fibrosis.

Increased large artery stiffness occurs in a range of inflammatory conditions indicating an ageing of the vasculature and additionally being an independent risk factor for cardiovascular events. We determined large artery parameters in adults with cystic fibrosis (CF). 50 clinically stable adult patients with CF (mean+/-sd age 28.0+/-8.2 yrs) and 26 controls matched for age, sex and body mass index were studied. Central aortic blood pressure, augmentation index (AIx) and aortic pulse wave velocity (PWV) were determined using applanation tonometry. Lung function, diabetic status and C-reactive protein (CRP) were also determined. Mean+/-sd AIx was greater in patients than controls, 8.5+/-11.1% and -1.8+/-13.1%, respectively (p<0.001), while PWV was similar. Although AIx was greatest in the sub-group with CF-related diabetes (CFRD), it was also increased in the non-CFRD sub-group when compared with controls. In patients, AIx was related to log(10) CRP (r = 0.33) and forced vital capacity (r = -0.34; both p<0.05), and CRP remained predictive in multiple regression. AIx is increased in adults with CF, in the presence of a normal blood pressure and independent of diabetic status. AIx was related to the systemic inflammatory status. These findings have implications for management and require further exploration so that cardiovascular health can be maintained.

A novel approach to central venous catheter thrombosis in a patient with cystic fibrosis.

Catheter directed thrombolysis has been described as a treatment for large pulmonary emboli resistant to systemic therapy [Kelly P, Carroll N, Grant C, Barrett C, Kocka V. Successful treatment of massive pulmonary embolism with prolonged catheter-directed thrombolysis. Heart Vessels 2006;21:124?6]. We now describe a case in which local catheter directed thrombolysis, via a peripherally inserted central catheter (PICC), was used to treat a large thrombus surrounding the tip of an indwelling central venous line that was causing superior vena cava obstruction (SVCO), in a patient with cystic fibrosis.

Predictive factors for the development of scoliosis in Duchenne muscular dystrophy.

OBJECTIVE: Scoliosis is a frequent complication (68-90%) of Duchenne muscular dystrophy (DMD). Prevention of limb deformities, rehabilitation in knee-ankle-foot-orthoses (KAFOs) and glucocorticoids prolong walking and standing, and might reduce scoliosis. We evaluated possible predictive factors for scoliosis development in a large DMD population. METHODS: Case notes of 123 DMD boys, > or = 17 years, followed at our centre between 1992 and 2002 were reviewed. Univariate analysis was used to relate two outcome measures (age at onset of scoliosis and severity at 17 years) with (i) glucocorticoids treatment; (ii) ages at (a) loss of independent ambulation, (b) rehabilitation into KAFOs, (c) loss of standing, (iii) forced vital capacity (FVC) (%) between 11 and 12 years and (iv) lower limb contractures. RESULTS: In total, 37/123 boys (30%) received intermittent prednisolone (0.75 mg/kg/day, 10 day/month) for a median 1-year (2 months-9 years), starting between 7.7 and 12.4 years (mean 9.5). About 96/123 (78%) were rehabilitated into KAFOs at 10.2+/-1.6 years. Age at loss of ambulation in KAFOs was 12.3+/-1.9 years and at loss of standing 12.8+/-2.1 years. About 95/123 (77%) boys developed scoliosis (Cobb angle >30 degrees ). Mean age+/-S.D. at scoliosis onset was 12.7+/-1.6 years. Forty-three boys (35%) had scoliosis surgery by 15+/-1.2 years. Later age at loss of ambulation (p<0.0001) and longer duration of prednisolone treatment (p=0.01) related to later scoliosis onset. Ages at loss of ambulation and standing were inversely related to scoliosis severity at 17 years (p<0.005). Hip asymmetry and %FVC at 11-12 years were directly related to scoliosis severity (p=0.02). CONCLUSIONS: Our data indicate a significant association between prolonged ambulation and a reduced risk of scoliosis development. Glucocorticoid administration, in our series, appear to be associated with a later onset of scoliosis, but did not alter the severity at 17 years, probably reflecting the shorter overall glucocorticoid exposure in this population.

Small bowel transplantation in the rat. Effect of pretransplant blood transfusions and cyclosporine on host survival.

We investigated the effect of pretransplant conditioning as a way to reduce the need for the aggressive immunosuppressive therapy reportedly required in small bowel (SB) allograft recipients. LEW rats were conditioned with (1) a donor-specific blood transfusion (DST) on day -8 and a concurrent 5-day course of CsA (10 mg/kg/day); (2) a nonspecific blood transfusion and CsA; (3) CsA alone. A 10-cm segment of the host native bowel was then replaced with an equivalent segment of SB obtained from ACI rats. Postoperative treatment consisted of CsA at 2.5 mg/kg/day for 30 days. Rats conditioned with a nonspecific transfusion and CsA or with CsA alone survived for 14.1 +/- 5.8 and 18.3 +/- 5.7 days, respectively. In contrast, rats conditioned with DST and CsA survived for 60.3 +/- 36.2 days (P less than 0.001 vs. both controls). Biopsies taken from long-term survivors showed a normal bowel architecture. The function of the allografts was studied in a group of animals totally deprived of their native bowel and transplanted with a 30-cm segment of ACI SB. CsA-DST-treated recipients survived an average of 90 +/- 43 days and grew at a rate comparable to isografted animals. Treated allograft recipients had maltose absorption indistinguishable from isografted controls at all times tested. In contrast, maltose absorption was severely impaired in recipients rejecting their grafts. This study demonstrates that long-term survival of SB allograft recipients can be achieved with good functional results with low doses of CsA in recipients conditioned with DST and CsA.

Lung mucociliary clearance in patients wit Young's syndrome.

Lung mucociliary clearance was measured in 14 patients with primary obstructive azoospermia and chest involvement (Young's syndrome) using the objective, in vivo radioaerosol technique. Lung mucociliary transport was significantly reduced in patients with Young's syndrome compared to 14 control subjects matched for physical characteristics, tobacco consumption and initial topographic distribution of tracer particles within the lungs. This finding indirectly supports the hypothesis that congenital abnormality in the propulsion of sperm in the ciliated epididymis results in the absence of sperm in the ejaculate.

Adenocarcinoma arising in a retained esophageal remnant.

Fistulation between the trachea and an infected mucocele arising from an excluded retained esophageal segment is an interesting and unusual complication of esophageal bypass surgery. We present such a case in which there was also malignant transformation in the cyst wall, manifested by invasive adenocarcinoma and widespread high-grade glandular dysplasia.

Pericardial effusion complicating breast cancer.

Thirty-five patients with metastatic breast cancer and pericardial effusions are described. They were treated with specific endocrine or chemotherapy. Twenty-two (63%) had presented in cardiac tamponade and were rapidly relieved by pericardiocentesis. Eighteen (82%) of these required no further local treatment, 2 obtained relief from a second aspiration and 2 needed additional treatment (intrapericardial bleomycin instillation and surgical pleuropericardial window). The median survival was 13.2 months. No patient relapsed again in the pericardium, suggesting that their prognosis was that of the underlying disease. Cardiac tamponade should be considered in any breathless patient with malignancy. In breast cancer, periocardiocentesis is lifesaving and systemic treatment can usefully prolong life.

Fibreoptic bronchoscopy. Part I: TECHNIQUE AND APPRAISAL.

Fibreoptic bronchoscopy is a simple, useful examination suitable for the investigation of outpatients without general anaesthesia. It considerably extends the range of vision and diagnostic ability that is achieved with a rigid bronchoscope. Further, it lends itself to the diagnosis and management of many medical lung diseases in addition to carcinoma. The applications of fibreoptic bronchoscopy will be discussed in more detail in Part II.

Haemoptysis in eosinophilic granuloma.

Eosinophilic granuloma of bone may involve the lung in about 20% of cases (Lewis 1964); and may present in the lung without other organ involvement. Haemoptysis occurs in about 10% of cases for unknown reasons. I document here a case with typical presentation and course, but which was complicated by haemoptysis of increasing severity leading to death. The cause of haemoptysis was diagnosed at fibreoptic bronchoscopy.

An analysis of the first 300 fibreoptic bronchoscopies at the Brompton Hospital.

We report the results of the first 300 fibreoptic bronchoscopies carried out at the Brompton Hospital. Positive cell typing was possible in only 36% of the visible carcinomas in the first 150 patients, but in 74% of the second 150. The reasons for this difference are discussed. We suggest that fibreoptic bronchoscopy be carried out in district referral hospitals where sufficient experience can be gained to produce good results. Our results suggest that the trap specimen should be examined routinely for acid-fast bacilli, for malignant cells only if biopsy is negative and not at all for other bacteria. With experience good results can be obtained by physicians and the expertise gained is invaluable in the investigation and treatment of a wide range of patients, many of whom do not need surgical treatment.

Nephelometry compared with differential antibody titre in routine rheumatoid factor measurements.

The comparative merits of agglutination techniques (differential agglutination titre (DAT)/latex slide test) and nephelometry for the routine measurements of IgM rheumatoid factors have been studied in this 2-part paper. The first part investigates the errors inherent in standard DAT measurements, and the second determines the rate of false positive results in an elderly nonrheumatoid population. It was found that the errors in agglutination techniques are due to differences in setting up the tests rather than in interpreting the results, and that the coefficient of variance was consistently around 20%. Approximately 1 in 5 of DAT results have such a high error as to be clinically valueless. The false positive rate in the elderly nonrheumatoid population was only 2% with nephelometry compared with 9% on the latex slide test. A previous study had demonstrated the advantages of nephelometry over DAT in routine rheumatological use, showing not only greater reproducibility but also a more accurate positive detection rate than the DAT in rheumatoid arthritis. The results also suggest that the traditional spectrum of rheumatoid factors of low titre detectable in the general population is probably an artefact inherent in agglutination techniques and that a definitive cut-off point is more likely. As nephelometers are generally available in biochemistry laboratories there is much to recommended their routine use for the measurement of IgM rheumatoid factors.

Fibreoptic bronchoscopy and diagnosis of pulmonary lesions in lymphoma and leukaemia.

Fifty-six fibreoptic bronchoscopies were performed on 42 patents with Hodgkin's disease, lymphoma, or leukaemia and pulmonary complications which did not respond to conventional antibiotics. All these patients had received chemotherapy, radiotherapy, or both for the treatment of their underlying conditions. Twenty-two bronchoscopic procedures were complicated by thrombocytopenia and neutropenia, requiring platelet transfusion before bronchoscopy, and many patients were hypoxaemic. Visual examination of the tracheobronchial tree, alveolar lavage, bronchial brushing, and transbronchial biopsy were carried out as approximate. Three patients had minor pulmonary haemorrhage, and three developed a pneumothorax after transbronchial biopsy. A specific diagnosis was obtained in 14 of 18 patients (78%) with diffuse chest radiographic abnormalities, in seven of 11 patients (64%) with lobar or segmental (focal) abnormalities, in two of eight patients with small (local) lesions, and in three of five patients with hilar abnormalities. In only three patients were infections diagnosed. It is concluded that fibreoptic bronchoscopy is a useful and safe diagnostic procedure in this situation but its value depends upon the type of radiological abnormality.

Spontaneous migration of totally implanted venous catheter systems from subclavian into jugular veins.

Two cases of migration of totally implanted catheter systems are described. The complication is potentially life threatening.