Ambrisentan improves exercise capacity and symptoms in patients with portopulmonary hypertension.

INTRODUCTION: Ambrisentan, a selective endothelin receptor antagonist has been approved in several countries for pulmonary arterial hypertension. No data have been published on the efficacy of ambrisentan on improvement of exercise capacity in patients with portopulmonary hypertension (PoPH). PATIENTS AND METHODS: We retrospectively analyzed the safety and efficacy of ambrisentan in patients with PoPH in four German university hospitals. RESULTS: 14 patients with moderate to severe PoPH were included. The median follow-up was 16 months (IQR, 12 - 21). 6 minute walk tests after 6 and 12 months improved from 376 meters (IQR, 207 - 440) at baseline to 415 meters (IQR, 393 - 475; p = 0.011) and 413 meters (IQR, 362 - 473, p = 0.005), respectively. WHO- functional class after 1 year of therapy with ambrisentan also improved significantly (p = 0.014). No significant changes in blood gas analysis and liver function tests (aspartate aminotransferase, alanine aminotransferase, total bilirubin, and international normalized ratio) during therapy with ambrisentan were detectable. CONCLUSIONS: The present study demonstrates significant improvement of exercise capacity and clinical symptoms without relevant safety concerns during ambrisentan treatment in patients with PoPH.

Statistical limitations of percent ideal body weight as measure for nutritional failure in patients with cystic fibrosis.

BACKGROUND: For the past decade, percentage of ideal body weight (%IBW) was recommended by European and US nutrition consensus reports as preferred clinical measure of nutritional status in children with cystic fibrosis (CF). We and others have demonstrated that the %IBW method underestimates the prevalence of nutritional failure in CF, but the underlying mechanism for this methodological flaw remains incompletely defined. DESIGN: We performed model calculations from cross sectional growth data of healthy and CF-children to assess the methodological limitations of %IBW calculation. RESULTS: Here we demonstrate that an intrinsic limitation of %IBW method is that it largely ignores the statistical principle of regression to the mean. The key assumption of %IBW is that ideal weight-for-age is on exactly the same percentile ranking as height-for-age. We show that this assumption is only valid if the individual's height is close to the reference median. When the stature deviates from the median of the reference population, however, the increments of height-for-age and weight-for-age percentiles are not the same. In consequence, %IBW method systematically underestimates the ideal weight for smaller-than-average individuals, like CF-patients, which results in significant underestimation of the prevalence of malnutrition in this group of patients. CONCLUSION: There is increasing scientific evidence that calculation of %IBW as a measure of nutritional status in children with CF is flawed and should be discontinued. It is expected that future guidelines will recommend the use of alternative measures of weight-for-height proportion, e.g. BMI percentiles, to assess underweight and malnutrition in patients with CF.

Concordant genotype of upper and lower airways P aeruginosa and S aureus isolates in cystic fibrosis.

RATIONALE: Lower airway (LAW) infection with Pseudomonas aeruginosa and Staphylococcus aureus is the leading cause of morbidity in cystic fibrosis (CF). The upper airways (UAW) were shown to be a gateway for acquisition of opportunistic bacteria and to act as a reservoir for them. Therefore, tools for UAW assessment within CF routine care require evaluation. OBJECTIVES: The aims of the study were non-invasive assessment of UAW and LAW microbial colonisation, and genotyping of P aeruginosa and S aureus strains from both segments. METHODS: 182 patients with CF were evaluated (age 0.4-68 years, median 17 years). LAW specimens were preferably sampled as expectorated sputum and UAW specimens by nasal lavage. P aeruginosa and S aureus isolates were typed by informative single nucleotide polymorphisms (SNPs) or by spa typing, respectively. RESULTS: Of the typable S aureus and P aeruginosa isolates from concomitant UAW- and LAW-positive specimens, 31 of 36 patients were carrying identical S aureus spa types and 23 of 24 patients identical P aeruginosa SNP genotypes in both compartments. Detection of S aureus or P aeruginosa in LAW specimens was associated with a 15- or 88-fold higher likelihood also to identify S aureus or P aeruginosa in a UAW specimen from the same patient. CONCLUSIONS: The presence of identical genotypes in UAW and LAW suggests that the UAW play a role as a reservoir of S aureus and P aeruginosa in CF. Nasal lavage appears to be suitable for non-invasive UAW sampling, but further longitudinal analyses and comparison with invasive methods are required. While UAW bacterial colonisation is typically not assessed in regular CF care, the data challenge the need to discuss diagnostic and therapeutic standards for this airway compartment. TRIAL REGISTRATION NUMBER: NCT00266474.

Endovascular therapy of symptomatic intracranial stenosis in patients with impaired regional cerebral blood flow or failure of medical therapy.

BACKGROUND AND PURPOSE: Symptomatic intracranial stenoses have a high risk for a recurrent stroke if treated medically. Although angioplasty and stent placement are proposed treatment options, data on longer-term outcome are limited. MATERIALS AND METHODS: We analyzed all endovascular procedures on symptomatic intracranial stenosis at our institution from January 1998 to December 2005. We retrospectively assigned patients to group A (symptoms despite antithrombotic therapy) or group B (impaired regional cerebral blood flow [rCBF]). Primary outcome events were periprocedural major complications or recurrent ischemic strokes in the territory of the treated artery. We used the Kaplan-Meier method to calculate survival probabilities. RESULTS: The procedural technical success rate was 92% (35/38) with periprocedural major complications in 4 cases (10.5%; group A [8.3%, 2/24], group B [14.3%, 2/14]). Median (range) follow-up for the 33 patients with technically successful procedures was 21 (0-72) months. Recurrent ischemic strokes occurred in 15% (3/20) of patients in group A and 0% (0/13) of patients in group B. Overall, there were 21% (7/33) primary outcome events (group A [25%, 5/20], group B [15%, 2/13]). There was a nonsignificant trend for better longer-term survival free of a major complication or recurrent stroke in patients with impaired rCBF compared with patients who were refractory to medical therapy treatment (Kaplan-Meier estimate 0.85 [SE 0.10] vs 0.72 [SE 0.11] at 2 years, respectively). CONCLUSION: Interventional treatment of symptomatic intracranial stenosis carries significant risk for complications and recurrent stroke in high-risk patients. The observation that patients with impaired rCBF may have greater longer-term benefit than medically refractory deserves further study.

From registry to quality management: the German Cystic Fibrosis Quality Assessment project 1995 2006.

Since 1995, the German Cystic Fibrosis Quality Assessment project has collected demographic data and outcome parameters. It aims to develop tools for quality management. The basic data of 6,835 patients has been collected annually by 93 centres. Weight for height and body mass index (BMI) indicated nutritional status, and forced expiratory volume in one second (FEV(1)) served as the central respiratory parameter. Data on mortality and survival were calculated. The mean age of all patients has increased from 13.9 yrs in 1995 to 17.7 yrs in 2005, and the percentage of adult patients has increased from 28.4 to 43.4%. Benchmarking diagrams and centre reports indicated considerable differences between the centres. The achievement of basic aims at the age of 6, 12 and 18 yrs indicated a positive development in 1995 to 2005. In 2005, median age at death was 23.7 yrs and the median cumulative survival was 37.4 yrs. Mortality correlated with a BMI <19 kg x m(-2) and an FEV(1) <80%. No sex gap in mortality was detected. "Learning from the best" is now possible. Further improvements in the system of cystic fibrosis care are required, such as: defining alarm signals for early treatment; involvement of patients and their families in quality management; auditing; benchmarking; and in-house training.

Resistance mechanisms of multiresistant Pseudomonas aeruginosa strains from Germany and correlation with hypermutation.

In this study, we analyzed the mechanisms of multiresistance for 22 clinical multiresistant and clonally different Pseudomonas aeruginosa strains from Germany. Twelve and 10 strains originated from cystic fibrosis (CF) and non-CF patients, respectively. Overproduction of the efflux systems MexAB-OprM, MexCD-OprJ, MexEF-OprN, and MexXY-OprM was studied. Furthermore, loss of OprD, alterations in type II topoisomerases, AmpC overproduction, and the presence of 25 acquired resistance determinants were investigated. The presence of a hypermutation phenotype was also taken into account. Besides modifications in GyrA (91%), the most frequent mechanisms of resistance were MexXY-OprM overproduction (82%), OprD loss (82%), and AmpC overproduction (73%). Clear differences between strains from CF and non-CF patients were found: numerous genes coding for aminoglycoside-modifying enzymes and located, partially in combination with beta-lactamase genes, in class 1 integrons were found only in strains from non-CF patients. Furthermore, multiple modifications in type II topoisomerases conferring high quinolone resistance levels and overexpression of MexAB-OprM were exclusively detected in multiresistant strains from non-CF patients. Correlations of the detected phenotypes and resistance mechanisms revealed a great impact of efflux pump overproduction on multiresistance in P. aeruginosa. Confirming previous studies, we found that additional, unknown chromosomally mediated resistance mechanisms remain to be determined. In our study, 11 out of 12 strains and 3 out of 10 strains from CF patients and non-CF patients, respectively, were hypermutable. This extremely high proportion of mutator strains should be taken into consideration for the treatment of multiresistant P. aeruginosa.

Evaluation of body mass index percentiles for assessment of malnutrition in children with cystic fibrosis.

OBJECTIVE: To compare the performance of recently released body mass index percentiles (BMIp) with standard anthropometric indexes, including height-for-age percentile (HAP), weight-for-age percentile (WAP) and percent ideal body weight (%IBW), as measures for nutritional failure in children with cystic fibrosis (CF). DESIGN: Cross-sectional analysis of growth and lung function data from 4577 children with CF reported to the German CF quality assurance (CFQA) project from 1995 to 2004. RESULTS: Frequency distribution of HAP (mean+/-s.d.: male 30.0+/-27.5; female 31.3+/-27.4) and WAP (male 28.9+/-27.0; female 29.6+/-26.7) were skewed, with significant numbers of patients below the fifth percentiles of a healthy reference population. However, because deficits occurred in both measures simultaneously, mean %IBW (male 97.0+/-12.1; female 98.1+/-12.3) assumed subjects weight close to the nominal weight-for-height at all ages. In contrast, mean BMIp was markedly reduced (male 35.7+/-27.9; female 35.6+/-27.2) and steadily declined with age. Ideal weight-for-age was significantly lower when predicted by %IBW compared with BMIp method, particularly in subjects with shorter-than-average stature. Consequently, less CF children were identified with nutritional failure according to %IBW method (male 20.5%; female 22.7%) compared with BMIp method (male 30.4%; female 28.7%). The clinical relevance of these findings was confirmed by stronger correlation of BMIp with impaired %forced expiratory volume/s, a marker for disease progression in CF. CONCLUSION: BMIp predicts nutritional failure more sensitively and accurately than conventional anthropometric indexes, at least in children with CF. Screening of CF patients by BMIp could provide an early warning sign and allow for timely therapeutic intervention.

[Preoperative hypervolemic hemodilution with 6% hydroxyethyl starch 130/0,4 (HES 130/ 0.4) solution as a way of reducing needs for donor blood transfusion].

UNLABELLED: The efficacy of hypervolemic hemodilution (HHD) in reducing the rate of donor blood transfusion is controversely discussed. The present prospective, randomized, clinical study analyzes the impact of HHD with 6% hydroxyethyl starch (HES) 130/0.4 solution on the rate transfusion, laboratory parameters, and the incidence of complications as compared with those in the control group receiving no preoperative HHD. MATERIAL AND METHODS: 80 patients who had undergone total prostatectomy or cystectomy were randomized into 2 groups. Before anesthesia, the HHD group (n=40) received 15 ml/kg of 6% HES 130/0.4 solution. In the HHD and control (n=40) groups, 6% HES 130/0.4 was intraintraoperatively infused in its maximum dose of 33 ml/kg according to the patients' needs. Indicationsf or blood transfusion trigger were Hb <5 g/dl or packed cell volume < 0.24. Laboratory parameters (Hb, PCV, platelets, prothrombin index, prothrombin time, thrombin time, fibrinogen, antithrombin III were measured before surgery, after HHD and 2, 24, and 48 hours after surgery. The mean blood pressure (MBP), heart rate (HR), and central venous pressure (CVP) were determined before surgery, after HHD, and 2 hours postsurgery. Statistical measurements were made in all patients from the HHD and control groups, as well as in a subgroup, in which intraoperative blood loss was greater than 30% of the total blood volume (70 ml/kg body weight). RESULTS: Demographic data and surgical techniques were similar in both groups. After HHD, CVP rose significantly. The changes in MBP and HR were statistically insignificant. There were no complications after HHD. Of the 40 HHD-group patents, 5 needed donor blood transfusion while in the control group blood was transfused to 10 of the 40 patients in the control group. Comparing both groups showed a lower need for blood transfusion in the HHD-group patients than in the controls (10 versus 24 packed red cells). The postoperative Hb values showed no difference between both groups. CONCLUSIONS: Preoperative HHD in patients undergoing surgery with expected >30% blood loss leads to decreased needs for blood transfusion. The method is safe and easy-to-use.

[Acute pain management in proximal femoral fractures: femoral nerve block (catheter technique) vs. systemic pain therapy using a clinic internal organisation model]. / Akutschmerztherapie bei Patienten mit hüftgelenknahen Frakturen : N.-femoralis-Katheter-Analgesie vs. systemische Schmerztherapie unter Anwendung eines klinikinternen Organisationsmodells.

BACKGROUND: The aim of this study was to compare safety and efficacy of catheter-mediated femoral nerve block analgesia with systemic pain therapy in patients with proximal femoral fractures in the pre-operative and post-operative setting using a protocol for coordinating pain management. METHODS: In a prospective randomised trial of patients attending the emergency department, 100 individuals were selected with a clinically diagnosed proximal femoral fracture. Patients were divided into two equal groups A and B. Group A (n=50) received a catheter-mediated femoral nerve block with 1% prilocaine (40 ml) and post-operatively 0.2% ropivacaine (30 ml) 6 hourly. Group B (n=50) initially received intravenous metamizol (1 g) and a fixed combination of oral tilidine (100 mg) + naloxone (8 mg). Patients aged 90 years or more received a reduced dose (tilidine 75 mg + naloxone 6 mg). In the post-operative period regular oral ibuprofen (400 mg, 8 hourly) in addition to oral tilidine (50 mg) + naloxone (4 mg) was given as required for break through pain. Pain intensity was measured using a verbal rating scale (VRS) from 1 to 5: pain free (=1), mild pain (=2), moderate pain (=3), severe pain (=4), excruciating pain (=5). Pain scores were recorded at rest (R), during passive anteflection (30 degrees) of the hip (PA) on arrival and at 15 and 30 min after initial administration of analgesia. Thereafter, recordings were made 4 times a day up to the third post-operative day. RESULTS: Pain scores were comparable for both groups on admission (VRS in R 2.50 vs. 2.46; VRS during PA 4.30 vs. 4.34). Significant pain relief was achieved in both groups following initial administration of analgesia, but the total pain scores in group A were significantly lower than in group B (VRS in R 1.22 vs. 1.58, p<0.01 and VRS during PA 2.66 vs. 3.26; p<0.001). No difference was noted between the two groups during the first 3 post-operative days. No severe complications occurred as a result of analgesia, however, the catheter was dislodged in 20% of patients in group A resulting in the need for systemically administered analgesia. CONCLUSION: All patients presenting with proximal femoral fractures should receive adequate analgesia within the emergency department even prior to radiographic imaging. Femoral nerve block should be considered as the method of choice. The insertion of a femoral nerve block catheter has the dual advantage of early analgesia permitting repeated clinical examination in addition to continued post-operative pain management. The cumbersome logistics inherent in this technique within the clinical setting limits its practical application. An initial single-shot regional nerve block followed by a systemic post-operative analgesia protocol was considered an appropriate alternative. The execution of safe, consistent and appropriate regional nerve block anaesthesia is reliant on formal guidelines and protocols as agreed by the multidisciplinary teams involved with patient-directed pain management and good clinical practice.

Prospective evaluation of emerging bacteria in cystic fibrosis.

BACKGROUND: Bacteria contribute considerably to the progression of lung disease in cystic fibrosis. In this prospective, multi-centre study, we aimed to evaluate the occurrence of emerging bacteria and the physicians' assessments of the clinical importance of these findings. METHODS: Twelve CF centres (total number of patients: 1419) reported the detection of any Stenotrophomonas maltophilia, Burkholderia cepacia complex, MRSA, Alcaligenes xylosoxidans, Klebsiella species and Mycobacteria during an observation period of 6 months. RESULTS: 213 specimens with emerging bacteria were reported from 145 different patients. The proportion of patients with emerging bacteria differed between centres (3-38%, mean: 12.6%) and increased with age. The predominant bacterium was S. maltophilia (n=106 positive specimens), followed by Klebsiellae (n=36), B. cepacia complex (n=31), A. xylosoxidans (n=16), Mycobacteria (n=11), MRSA (n=11), and others (n=2). In many instances the same microorganisms had already been reported earlier, indicating intermittent or chronic colonisation. The clinical status was reported to be stable in 70% of patients, and antibiotic treatment was anticipated for 46% of positive specimens. Comparison of clinical data to age matched controls did not reveal any significant differences with regard to pulmonary and nutritional status prior to detection of emerging bacteria. CONCLUSION: These data suggest a high variability between centres regarding the prevalence of emerging bacteria. Most patients maintained a stable clinical condition during the 6-month study period despite being colonised with emerging bacteria.

Natural antimicrobial susceptibility patterns and biochemical profiles of Leclercia adecarboxylata strains.

Leclercia adecarboxylata is an opportunistic human pathogen that phenotypically resembles Escherichia coli. The natural susceptibilities of 101 Leclercia strains to 70 antimicrobial agents were investigated. MICs were determined with a microdilution procedure in cation-adjusted Mueller-Hinton broth (all strains) and IsoSensitest broth (some strains). Natural susceptibility patterns were assessed using German (DIN) standards (when applicable). In addition, biochemical properties recommended for the phenotypic identification of L. adecarboxylata were evaluated, applying two commercially available identification systems for Enterobacteriaceae and seven conventional tests. L. adecarboxylata strains were naturally sensitive to tetracyclines, aminoglycosides, all but two beta-lactams, quinolones, folate pathway inhibitors, chloramphenicol, nitrofurantoin and azithromycin. They were naturally resistant to penicillin G, oxacillin, erythromycin, roxithromycin, clarithromycin, ketolides, lincosamides, streptogramins, linezolid, glycopeptides, rifampicin, fusidic acid and fosfomycin. There were only minor medium-dependent differences in susceptibility to most antibiotics. Lysine decarboxylase, malonate assimilation and acid production from arabitol and cellobiose, but not from adonitol and sorbitol, allowed definitive separation of L. adecarboxylata from E. coli. The results of this study form a database that can be applied to validate forthcoming antibiotic susceptibility tests of L. adecarboxylata, and might contribute to its reliable identification. Susceptibility patterns did not indicate obvious therapeutic difficulties for treatment of Leclercia infections. Special attention should be paid to biochemically aberrant leclerciae. Apart from biochemical features, fosfomycin susceptibility might be useful to differentiate between L. adecarboxylata and E. coli.

Natural antibiotic susceptibility of Ewingella americana strains.

The natural susceptibility of 20 Ewingella americana strains to 72 antibiotics was examined. MIC values were determined using a microdilution procedure in cation-adjusted Mueller-Hinton broth. Evaluation of natural antibiotic susceptibility was performed applying the German standard (where applicable). Beta-lactamases were examined with a conventional nitrocefin colony testing procedure, activity and induction assays, and SDS-PAGE. Ewingella strains were naturally resistant or of intermediate susceptibility to cefaclor, loracarbef, cefazoline, cefuroxime, cefoxitin, benzylpenicillin, oxacillin, fosfomycin, erythromycin, roxithromycin, clarithromycin, lincosamides, dalfopristin-quinupristin, ketolides, linezolid, glycopeptides, fusidic acid and rifampicin. Uniform natural sensitivity was found with acylureidopenicillins except for azlocillin, ticarcillin, several cephalosporins, carbapenems, aztreonam, tetracyclines, aminoglycosides, quinolones, azithromycin, folate-pathway inhibitors and chloramphenicol. Strains of E. americana were naturally sensitive or of intermediate susceptibility to aminopenicillins (with and without beta-lactamase inhibitors), azlocillin and nitrofurantoin. All ewingellae yielded beta-lactamases; testing of representative strains revealed that these enzymes belong to Ambler class C. Inducibility of beta-lactamase was shown for E. americana ATCC 33852T, CCUG 35675 and CCUG 42782. The present study describes a database concerning the natural susceptibility of E. americana strains to a range of antibiotics, which can be applied to validate forthcoming antibiotic susceptibility tests of these bacteria. It enlarges the number of Enterobacteriaceae expressing naturally-occurring AmpC beta-lactamases.

Natural antibiotic susceptibility of strains of Serratia marcescens and the S. liquefaciens complex: S. liquefaciens sensu stricto, S. proteamaculans and S. grimesii.

The natural susceptibility of 77 strains of Serratia marcescens and 41 strains of the S. liquefaciens complex (S. liquefaciens sensu stricto (n=21), S. grimesii (n=10), S. proteamaculans (n=10)) to 70 antibiotics was examined using a microdilution procedure in Isosensitest broth (all strains) and cation-adjusted Mueller Hinton broth (some strains). All species were naturally resistant to benzylpenicillin, oxacillin, cefaclor, cefazolin, cefuroxime, numerous macrolides, lincosamides, streptogramins, glycopeptides, rifampicin and fusidic acid. Uniform natural sensitivity was found to most aminoglycosides, several acylureidopenicillins, ticarcillin, newer cephalosporins, carbapenems, aztreonam, quinolones and antifolates. Species-related differences in susceptibility affecting clinical assessment criteria were found for several agents. S. marcescens was less susceptible to some aminoglycosides than species of the S. liquefaciens group. It was the only species that was uniformly naturally resistant to tetracycline, amoxycillin, amoxycillin/clavulanate and loracarbef. Species of the S. liquefaciens group were naturally resistant and intermediate or naturally intermediate to the latter agents. Differences in susceptibility among the species of the S. liquefaciens complex were generally small. S. proteamaculans was most susceptible to sulphamethoxazole. S. liquefaciens sensu stricto was less susceptible than S. grimesii and S. proteamaculans to tetracyclines, chloramphenicol and nitrofurantoin; it was the only species uniformly naturally resistant to fosfomycin. This study suggested that all species examined probably express chromosomally-encoded AmpC beta-lactamases, but the amount of enzyme may vary from species to species. The naturally-occurring low-level expression of the S. marcescens aminoglycoside 6'-acetyltransferase AAC(6')-Ic and its absence in other Serratia spp. was supported by the data. All species of the S. liquefaciens complex should be considered as probable agents of human diseases.

Natural antibiotic susceptibility of Enterobacter amnigenus, Enterobacter cancerogenus, Enterobacter gergoviae and Enterobacter sakazakii strains.

OBJECTIVE: To investigate the natural susceptibility to 69 antimicrobial agents of 107 Enterobacter strains comprising E. amnigenus (n = 18), E. cancerogenus (n = 26), E. gergoviae (n = 28) and E. sakazakii (n = 35). METHODS: Minimal inhibitory concentrations (MICs) were determined with a microdilution procedure in Isosensitest broth and cation-adjusted Mueller-Hinton broth. RESULTS: All the species were naturally sensitive or intermediate to tetracyclines, amino-glycosides, numerous beta-lactams (acylureidopenicillins, ticarcillin, ampicillin/sulbactam, several cephalosporins, carbapenems, aztreonam), quinolones, antifolates, chloramphenicol and nitrofurantoin. Natural resistance was found to penicillin G, oxacillin, several macrolides, lincosamides, streptogramins, glycopeptides, rifampicin and fusidic acid. Species-related differences in natural susceptibility were found to some beta-lactams, azithromycin and fosfomycin. Whereas E. gergoviae was the most susceptible species to azithromycin, E. cancerogenus was most susceptible to fosfomycin and was the only species showing natural resistance to amoxicillin, amoxicillin/clavulanic acid, cefaclor, cefazoline, loracarbef and cefoxitin. There were only minor medium-dependent differences in susceptibility to most antibiotics. CONCLUSIONS: The present study establishes a database concerning the natural susceptibility of recently established Enterobacter species to a wide range of antibiotics, which can be applied for the validation of routine susceptibility test results. beta-Lactam susceptibility patterns indicate the expression of species-specific beta-lactamases expressed at high or low levels in all the species except E. sakazakii.

[Drug-induced toxic epidermal necrolysis with involvement of the intestinal and respiratory tract. A case report]. / Toxisch epidermale Nekrolyse Arzneimittelinduzierte toxisch epidermale Nekrolyse mit Beteiligung des Intestinal- und Respirationstraktes.

Toxic epidermal necrolysis (TEN) is a disease occurring with low-incidence but has a relatively high mortality rate. Sepsis is the predominant cause for life-threatening complications in TEN but severe mucosal damage represents a further complication which may delay convalescence. We report a case of TEN in a 51-year-old man which eventually spread to include the whole skin surface. The long-term and comprehensive treatment focused on support of the organ failure as well as wound treatment. The extent of involvement of the intestinal tract, the sustained laryngeal stenosis and the pronounced saddle-nose were unusual. It appears necessary to treat TEN in facilities which offer intensive care and are able to manage extensive skin damage. Burns units offer the best conditions for its management.

Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project.

BACKGROUND: The German cystic fibrosis (CF) quality assurance (CFQA) project is a patient registry for CF which was founded in 1995. Relevant clinical and laboratory data, respiratory function test results, complications, and CF treatments are entered into the database once a year for each patient. Using the database, a study was undertaken to elucidate the relationship between nutrition and lung function in a large patient cohort by cross sectional and longitudinal analysis. METHODS: A cohort of 3298 patients above 2 years of age was analysed. Patients were grouped according to the presence or absence of malnutrition (wasting and/or stunting). Cross sectional and longitudinal analyses over 2 and 3 years including mixed model analyses were performed. RESULTS: The prevalence of abnormal weight for height (<90% predicted) increased with age from 19% in children aged <6 years to 38% in adults with CF. Patients with malnutrition had significantly lower mean values of vital capacity, arterial oxygen tension (PO(2)), and forced expiratory volume in 1 second (FEV(1)) and higher serum IgG (p<0.05). Pseudomonas aeruginosa infection was also associated with decreased pulmonary function. Malnourished adolescents aged 12-18 years experienced a serious decline in FEV(1) of about 20% predicted, whereas mean FEV(1) values remained stable at above 80% predicted in adolescents of normal weight. Longitudinal follow up showed that malnourished patients of all ages and those with P aeruginosa infection had significantly worse lung function than their normally nourished counterparts and a greater yearly loss of FEV(1) % predicted. During 1 year of observation adolescents who experienced a >5% predicted decrease in weight for height had a concomitant mean loss of FEV(1) of 16.5% predicted during that year, whereas patients who gained relative weight had a parallel increase in FEV(1) of 2.1% predicted. CONCLUSIONS: These data emphasise the close relationship between nutrition, lung function, and clinical course in CF. Normal body weight and absence of P aeruginosa infection was associated with better preservation of lung function.