First report of lung transplantation in a patient with active pulmonary Mycobacterium simiae infection.

Mycobacterium simiae is a slow-growing mycobacteria that in rare cases can cause chronic pulmonary infection. We report the first case of lung transplantation in a patient with active M simiae infection at the time of transplantation. A 56-year-old immunocompetent nonsmoking woman underwent bilateral lung transplantation for end-stage idiopathic bronchiectasis and chronic M simiae infection. The disease proved manageable on a regimen of clarithromycin, moxifloxacin, and cotrimoxazole with a successful outcome 1-year posttransplantation. There is increasing evidence that nontuberculous mycobacterium infection should no longer be an absolute contraindication for lung transplantation.

Vitamin D deficiency as a risk factor for cystic fibrosis-related diabetes in the Scandinavian Cystic Fibrosis Nutritional Study.

AIMS/HYPOTHESIS: Many cystic fibrosis patients are vitamin D-insufficient. Cystic fibrosis-related diabetes is a major complication of cystic fibrosis. The literature suggests that vitamin D might possess certain glucose-lowering properties. We aimed to assess the relationship between vitamin D and cystic fibrosis-related glucose intolerance. METHODS: We enrolled 898 cystic fibrosis patients from Sweden, Norway and Denmark. Vitamin D intake was assessed using a seven-day food record. Serum 25-hydroxyvitamin D (s25OHD) and HbA(1c) were measured, and an OGTT was carried out. Multiple linear and logistic regressions were used for HbA(1c) and cystic fibrosis-related diabetes/OGTT result as outcome variables, respectively. Each model was controlled for country, and for known cystic fibrosis-related diabetes risk factors: age, sex, genotype, liver dysfunction, long-term corticosteroid treatment, and lung and pancreatic function. RESULTS: Degree of vitamin D insufficiency (OR 1.36; p = 0.032) and s25OHD < 30 nmol/l (OR 1.79; p = 0.042) were significant risk factors for cystic fibrosis-related diabetes. Accordingly, HbA(1c) value was positively associated with s25OHD < 30 nmol/l and < 50 nmol/l, as well as with degree of vitamin D insufficiency (adjusted R (2) = 20.5% and p < 0.05 in all). In subgroup analyses, s25OHD < 30 nmol/l determined the HbA(1c) value in paediatric patients (adjusted R (2) = 20.2%; p = 0.017), but not in adults. CONCLUSIONS/INTERPRETATION: Vitamin D status is associated with HbA(1c) and diabetes in cystic fibrosis, particularly in children. The study justifies prospective studies on the proposed role of vitamin D deficiency in the pathophysiology of diabetes mellitus.

Inverse relation between vitamin D and serum total immunoglobulin G in the Scandinavian Cystic Fibrosis Nutritional Study.

BACKGROUND/OBJECTIVES: The hallmark of cystic fibrosis (CF) is chronic lung inflammation. The severity of lung disease is closely correlated with immunoglobulin G (IgG) levels. Beyond its contribution to the bone health, the importance of vitamin D has not been fully recognized owing to the lack of human studies providing evidence of its benefit. In the context of the recently described immunomodulatory functions of vitamin D, we aimed to assess the relationship between vitamin D and IgG levels. SUBJECTS/METHODS: Eight hundred and ninety-six CF patients were included (0.53-65.9 years) from seven centers in Denmark, Norway and Sweden. Serum 25-hydroxyvitamin D (25OHD) and total IgG were measured, spirometry was carried out and vitamin D intake data were gathered using a 7-day dietary food record. Multiple linear regression analyses were performed for IgG and forced expiratory volume in 1λs (FEV1) as dependent variables, and serum 25OHD, daily food and supplemented vitamin D sources of intake as independent variables. The model was controlled for age, gender, genotype, CF-related diabetes, season, infection/colonization status, long-term oral corticosteroid treatment, long-term treatment with macrolide antibiotics, pancreatic insufficient phenotype and body mass index z-score. RESULTS: Serum total IgG levels were negatively associated with serum 25OHD (adjusted R (2) = 0.376; beta = -0.02; P<0.001), supplemented vitamin D intake per kg bodyweight (adjusted R (2) = 0.375; beta = -0.82; P < 0.001) and total vitamin D intake per kg bodyweight (adjusted R (2) = 0.398; beta = -0.60; P = 0.002). Serum 25OHD was positively associated with FEV1 (adjusted R (2) = 0.308; beta = 0.0007; P = 0.025). CONCLUSIONS: Increasing vitamin D intake may positively modulate inflammation in CF. This study supports the proposed role of vitamin D in the immune system during infection and substantiates prospective studies.

Pharmaceutical intervention in the care of cystic fibrosis patients.

In a prospective, randomised, cross-over study including cystic fibrosis patients with indications for HIVAT (home intravenous antibiotic treatment) the prospect of pharmaceutical intervention was investigated. A comparison between the use of disposable infusion devices with antibiotics from the pharmacy and when the patients prepared the drugs themselves was performed. During a first treatment course the patients received either infusion devices during 5 days or reconstituted the drugs themselves during 5 days, or vice versa. During a second treatment course the order was the reversed. Eight patients were included, out of which six completed the original design as a cross-over study, yielding a total of 550 doses of antibiotics. The patients preferred infusion devices from the pharmacy prepared according to GMP (Good Manufacturing Practice) as opposed to reconstituting the antibiotics themselves. Points of view presented included no anxiety over the correct dosage of drugs and less disruption of family and social life. In a practical sense, portable devices are more expensive than the preparation of the drugs by the patients themselves. However, when comparing with in-hospital treatment the direct costs for a hospital stay exceed that of the devices. Another part of the study evaluated the quality of life using a modified form of SEIQoL-DW (Schedule for the Evaluation of Individual Quality of Life - Direct Weighting). Twenty patients took part in the study and the overall quality of life scores increased significantly when patients received infusion devices compared to reconstituting the drugs themselves.

Lung transplantation at the University of Lund 1990-1995. Analysis of the first 39 consecutive patients.

Between 1990 and 1995 39 patients were lung transplanted at the University Hospital in Lund. This is a retrospective review of survival and lung function in these patients. There were 17 single-lung transplants (SLT), 21 double-lung transplants (DLT) and 1 heart-lung transplant (HLT). Seven patients died during the period, giving an overall survival of 82%. One-year survival according to Kaplan-Meier survival analysis was 87%, and 2-year survival was 83%. Vital capacity and forced expiratory volume in 1 s (FEV1) 1 year after transplantation were 91% and 100% of predicted, respectively, in the DLT group and 60% and 50% in the SLT group. Bronchiolitis obliterans syndrome (BOS) developed in 11 of the 35 patients (31%) surviving more than 6 months, 2/21 in the DLT group and 8/13 in the SLT group and in the patient with HLT. The median time until detection of BOS was 11 months after the operation (range 6-18 months). Working capacity 1 year after transplantation was 60% of predicted in the DLT group and 47% of predicted in the SLT group. Ventilatory capacity was no longer function limiting. Lung transplantation today is a therapeutic option with a good medium-term survival and good functional results in selected patients with severe lung disease.