A conceptual framework to develop a patient-reported experience questionnaire on the cystic fibrosis journey in France: the ExPaParM collaborative study.

BACKGROUND: The objective of the study was to elaborate a conceptual framework related to the domains of patient experience along the cystic fibrosis (CF) journey from the patients and parents of children with CF to inform the design of a patient-reported experience questionnaire. METHOD: A collaborative research group including patients and parents with clinicians and academic researchers was set up. They identified the situations along the CF care pathway from diagnosis to paediatric care, transition to adult care and adult follow-up, transfer to transplant centres and follow-up after transplantation. Participants were recruited by CF centres in metropolitan France and overseas departments. Semi-structured interviews were conducted, transcribed verbatim and subjected to an inductive analysis conducted in duos of researchers/co-researchers using NVivo®. The conceptual framework was discussed with the research group and presented to the CF centres during two video conferences. The protocol obtained a favourable opinion from the Ethics Evaluation Committee of INSERM (IRB00003888-no. 20-700). RESULTS: The analysis led to a conceptual framework composed of domains of the CF journey, each divided into several items. 1. CF care: Management of care by the CF centre team; in-hospital care; quality of care in the community; therapeutic education and self-management support; at-home care; new therapies and research; procreation; 2. Transplant care: management of transplant and CF care; coordination with other specialties; education and self-management support; at-home care; procreation; new therapies and research; 3. Turning points along the journey: diagnosis of CF, transition to adult care, transfer to transplantation; 4. Social life with CF: housing, employment and education, social relations, social welfare and family finances. The number of patients included and the diversity of situations made it possible to achieve a sufficient richness and saturation of codes by domain to develop patient experience questionnaires. CONCLUSION: This conceptual framework, resulting from the participants' experience, will inform the design of a patient-reported experience tool, whose construct will be tested during the next phase of the ExPaParM project to assess its fidelity, intelligibility, and ability to report patient experience of the CF journey.

Collaborative research protocol to define patient-reported experience measures of the cystic fibrosis care pathway in France: the ExPaParM study.

INTRODUCTION: In France, the cystic fibrosis (CF) care pathway is coordinated by multidisciplinary teams from specialised CF centres or transplant centres. It includes the care provided at home or out of hospital, risk prevention in daily life and adjustments to social life, which together contribute to the person's quality of life. Patient experience is used to describe and evaluate the care and life of patients living with the disease. OBJECTIVES: Our collaborative research aims to identify the most significant areas and criteria that characterise the CF pathway. It will lead to the development of a questionnaire to collect patients' experience, which can be administered to all patients or parents of children registered and followed in the centres. The article describes the protocol developed in partnership with patients and parents of children living with the disease. METHOD: A multidisciplinary research group brings together researchers, patients, parents of children with CF and health care professionals. The patient partnership is involved in the 4 phases of the protocol: (1) setting up the study, recruiting patient and parent co-researchers, training them in qualitative research methods, defining the situations and profiles of patients in the study population, elaborating the protocol; (2) selecting the study sites, recruiting participants, carrying out semi-structured interviews, analysing verbatims using the grounded theory approach; (3) co-elaborating Patient-Reported Experience Measures (PREM) questionnaires adapted to the 4 types of participants: parents, adolescents, non-transplanted adults and transplanted adults; (4) validating the construct with participants and professionals from the study centres. RESULTS: The protocol obtained a favourable opinion from the Ethics Evaluation Committee of INSERM (IRB00003888-no. 20-700). Training was provided to the 5 patients and 2 parent co-researchers to enable them to participate effectively in the research. Eleven centres participated in the recruitment of participants in mainland France and Reunion Island. Eighty hours of interviews were conducted. DISCUSSION: The PREM questionnaires to be elaborated will have to undergo psychometric validation before being used by the actors of the CF network to assess the impact on the care pathways of quality approaches or new therapies available in cystic fibrosis. Trial Registration Registry: IRB00003888 - no. 20-700. Issue date: 06/09/2020.

[How can the transition of adolescents from a children's to an adult CF center be improved? Analysis of adolescents' and parents' needs during the post-transfer period]. / Comment améliorer la transition au sein des CRCM : analyse des besoins des patients et de leurs parents lors de l'arrivée en service adulte.

INTRODUCTION: In 2018, 55.4% of the 7180 French cystic fibrosis (CF) patients were adults. Our study was aimed at identifying young adult patients' needs and those of their parents when the young adults arrived in an adult CF center. METHODOLOGY: Semi-structured interviews, conducted between July 2018 and December 2019and involving all the concerned teenagers and their parents, took place at least 6 months after their transfer. The interview guide dwelt on the aspects having had an impact on their experience of the transition. The interviews were recorded, transcribed and analyzed exhaustively. The results were classified by categorizing the contents according to respondent profile. RESULTS: Thirty-eight young adult patients and 16 parents were interviewed. As regards the young adults, analysis of their needs underlined the importance of their continuing to develop their skills in adaptation, communication and self-care. As regards their parents, they needed support in view of defining their role in their children's new care pathway. CONCLUSION: During and also following the transfer, therapeutic education for the parents as well as the young adults requires reinforcement.

Smoothing the transition of adolescents with CF from pediatric to adult care: Pre-transfer needs.

INTRODUCTION: In France, the cystic fibrosis (CF) care pathway is performed in 45 CF centers, the life expectancy of patients has steadily increased, but to date there are no national recommendations for the transition from pediatric to adult care. The transition to an adult CF center still raises questions about the relevance of its organizational arrangements. The "SAFETIM need" study aimed to identify the organizational needs both of patients and of parents before the transfer to an adult CF center. METHODS: This was a prospective, observational, multicenter study conducted between July 2017 and December 2018, involving the three CF centers of a regional network in southeastern France. Each adolescent registered with the center and his or her parents were interviewed individually, on the same day, during the 6 months leading up to transfer. They participated in semi-structured interviews during one of their routine consultations at the CF center. The interview manual, based on literature reviews and targeting national recommendations, was tested and validated by the national CF therapeutic education group (GETheM). All interviews were transcribed and checked by two different people, and analyzed by two researchers individually. The results were classified by topic according to content categorization. RESULTS: Overall, 43 adolescents and 41 parents were interviewed, respectively, who were followed up by CF centers: 14% (n=6) in a mixed CF center (pediatric and adult); 19% (n=8) and 67% (n=29), respectively, in two different pediatric CF centers. Adolescents were between 16 and 19 years old. For adolescents, the average interview time was 5.11min. (standard deviation [SD]: 3.8min; minimum: 2.53min; maximum: 17.14min). For parents, the average interview time was 7.99min (SD: 3.56min, minimum: 3.43min; maximum: 22.50min). DISCUSSION: Our study enquired only about the preparation and organization of the transfer. We identified three areas of actions matching the needs of adolescents and parents before transfer. The first one is to anticipate team change to prepare follow-up in their future CF center: acquire new skills, consider the future CF center according to the adolescent's curriculum, be involved in the transition process. The second area is to accompany the upcoming change. The care team could help by providing information and support during the start of teenagers' transition toward autonomy. And parents were aware that the CF center change will reverse roles. They must provide their own knowledge and manage the ambivalence of this as well as letting go. The third one is to announce the transition process and functioning of the future adult CF center, because the transition would require time to find their place (patients and parents) with the new team. CONCLUSION: The "SAFETIM needs" pre-transfer study results show that we can identify the main criteria to be developed and strengthened, to promote a smooth, high-quality transition from pediatric to adult CF care for patients in France. For most patients, the transition cannot be prepared at the last minute. Caregivers need to develop specific skills in adolescent and young adult care and follow-up. Each team must consider the transition as a normal part of the patient care cycle. While it must be structured, some flexibility must be allowed so as to give everyone the chance to be prepared and to personalize the care.

[Quality criteria for the transition to adult care in French CF centers - results from the SAFETIM APP study?] / Critères de qualité pour la transition dans la mucoviscidose en France (suivi des adolescents, des familles et des équipes, pour une transition idéale en mucoviscidose ­ analyse des pratiques professionnelles).

INTRODUCTION: SAFETIM-APP compiled an inventory of professional practice in the 45 French cystic fibrosis reference centres (CFRC), between February 2015 and December 2016, related to the transition of adolescents with cystic fibrosis to adult centres. METHOD: This multicentre cross-sectional study addressed the modalities of the transition in CFRCs and proposed a list of items that could be used to establish quality criteria. Quantitative analysis of the criteria and a qualitative analysis of the transition procedure were carried out. RESULTS: A total of 77% of the CFRCs that were contacted took part. Transition lasted 3 to 5 years and began at around 15 years of age. Nine criteria were described as fundamental, including: collaboration between teams, taking adolescence into account, having a time for adolescents to speak with the physician alone, defining a program including therapeutic education, involving the family, accompanying the parents. Seven additional criteria were noted to be important, including: re-announcing the diagnosis, identifying a common thread (caregiver) accompanying the family, scheduling adult follow-up from paediatrics onwards, visiting the adult department, organizing a formal departure/reception time, initiating the process early enough, identifying indicators to evaluate practices. CONCLUSION: The transition processes in place in CFRCs can be improved by implementing the use of these quality criteria systematically.

[National French guidelines for management of infants with cystic fibrosis]. / Recommandations nationales pour la prise en charge du nourrisson dépisté atteint de mucoviscidose. Consensus de la fédération des centres de ressources et de compétences de la mucoviscidose.

These guidelines aim to standardize the care of infants diagnosed with a typical form of cystic fibrosis (CF) at neonatal screening. They have been implemented by the National Working Group for Neonatal Screening of the French Federation for CF and have been validated using the Delphi methodology by a large group of clinicians involved in the care of CF infants. These guidelines encompass management and organization of care at diagnosis and describe nutritional, digestive, and respiratory monitoring and treatment during the first 2 years of life.

Aplicación de terapia de vacío en el tratamiento de nevus pigmentario gigante en la infancia / Negative pressure therapy in the treatment of a giant melanocytic nevus in chilhood

Los nevus melanocíticos gigantes congénitos son muy infrecuentes. Su incidencia de malignización varía según las series clínicas entre el 2 y el 45%. Este riesgo de malignización y los problemas de adaptación psicosocial que suelen acarrear hacen recomendable el inicio del tratamiento quirúrgico de este tipo de lesiones antes de la edad escolar. Presentamos el caso de un niño de 7 años de edad connevus melanocítico congénito gigante que afectaba a la espalda y al flanco derecho, de 30 x 19 cm de diámetro. Desarrollamos un protocolo terapéutico mediante programación de intervenciones quirúrgicas seriadas de mínima morbilidad, con postoperatorio simple y poco doloroso. En un primer tiempo realizamos extirpación de la lesión con bisturí ultrasónico y cobertura temporal del defecto creado con dermis artificial. En un segundo tiempo intentamos cobertura definitiva con piel cultivada autóloga, siendo necesario un tercer tiempo de cobertura con autoinjerto de espesor ultrafino. En este caso, el sistema de terapia de presión negativa VAC® mostró su eficacia para inmovilizar los apósitos de forma segura, indolora y en régimen ambulatorio (AU)

Giant congenital melanocytic nevi are very uncommon. Malignization incidence varies according to clinical series between 2 and 45%. This risk of malignization and psychosocial adaptation problems make necessary to begin the surgical treatment before school age. We present a 7-year-old child with a giant melanocytic nevus in the back and right flank, 30 x 19 cm in size. A therapeutic protocol using serial surgical interventions with minimal morbidity and an easy and painless postoperative care were developed. In the first surgery we removed the nevus with ultrasonic scalpel and used artificial dermisas a temporary coverage. In the second time we tried to complete the definitive coverage with autologous cultured skin, but we needed a third time to apply definitive ultrathin skin autograft. In this clinical case, VAC® Therapy System has shown its effectiveness to fix dressings, painless safely and as an outpatient (AU)

Uso de Terapia VAC(R) en paciente pediátrico con exposición ósea postamputación traspúrpura fulminans / VAC(R) Therapy in pediatric patient with bone exposition after purpura fulminans

La sepsis meningocóccica se asocia con un alto índice de mortalidad. Los pacientes afectados pueden mostrar signos de coagulación intravascular diseminada (CID) y necrosis de partes blandas debidas a baja perfusión tisular. Los procedimientos quirúrgicos precoces suelen demorarse en estos casos, pues inicialmente es difícil determinar el grado de la lesión. Una vez que el paciente está estable, es esencial el desbridamiento de todo tejido necrótico, teniendo como objetivo el preservar la máxima cantidad de tejido posible, especialmente las articulaciones. Muchos niños sobreviven a esta patología, pero sufren gran morbilidad: amputación de una o varias extremidades y otras complicaciones derivadas como exposiciones óseas. Existen pocas publicaciones que hagan referencia al tratamiento no quirúrgico de este tipo de lesiones. Presentamos un caso de aplicación del sistema de terapia depresión negativa VAC ® en paciente en edad pediátrica tras pérdida de sustancia como resultado de una púrpura fulminans secundaria a meningococcemia (AU)

Meningococcal sepsis is associated with a high mortality rate. These patients may show severe disseminated intravascular coagulation (DIC) and skin, subcutaneous tissue and muscle necrosis due to poor tissue perfusion. It is rare that early surgical intervention be required, as it is relatively difficult to determine the degree of tissue loss early on. Once the patient is stable, debridement of all necrotic tissue is essential, and the goals are still to preserve as much tissue as possible, specially the joints. Many children survive the disease, but suffer major morbidities such as extremities amputation, which may result of complications like bone exposure. There is very little published experience regarding the non-surgical treatment of this complication. We report anew possible application of the vacuum-assisted closure system (VAC®) in very young patients with loss of tissue as a result of purpura fulminans secondary to meningococcemia (AU)

[Screening and management of glucose metabolism disorders in cystic fibrosis patients. Practices survey in 4 French reference centers]. / Dépistage et prise en charge des troubles du métabolisme glucidique chez les patients atteints de mucoviscidose. Etude de pratiques au sein du réseau EMERA.

OBJECTIVE: Glucose metabolism disorders are a new point of interest in cystic fibrosis (CF) management. Cystic fibrosis-related diabetes mellitus (CFRD) increases alteration of pulmonary function as well as patients' morbidity and mortality. In France, CF patients are exclusively followed up in reference centers. We conducted a practices survey on screening and diagnosis of glucose metabolism disorders at 4 French CF centers. The objective of this study was to assess adherence to practice guidelines developed in 2002 at these centers. METHODS: This study was conducted in 2 sessions: 60 medical records were randomly selected in 2005 and in 2007 for patients aged over 10 years followed up at 4 CF centers. A questionnaire survey was completed for each patient with questions on CFRD screening, diagnosis, monitoring and treatment. Our guidelines recommend random blood glucose (RBG) at each standard biological test, annual glycosylated haemoglobin and an oral glucose tolerance test (OGTT) at 10, 15 and 18 years of age, then every 2 or 3 years. RESULTS: An annual RBG was performed in 82% of patients in 2005 and 91.5% in 2007. HbA1c screening was performed annually for 77% of patients in the 1st session and for 90% of patients for the 2nd session (p<0.10). Adherence to OGTT guidelines was better for adults than children: 96% had an OGTT during the 3 years of the first session and 79% during the second session, while fewer than 50% of children had their OGTT at 15 and 18 years of age. Taking the OGTT at 10 years of age could not be assessed because no patients were 10 years old during the study period. Screening for neurological complications of CFRD was assessed in the majority of diabetic patients, while half or less than half had annual fundus oculi or microalbuminuria dosage. DISCUSSION: There was an improvement in screening for CFRD and glucose metabolism disorders between 2005 and 2007, even though practices could still be improved. This shows that clinical guidelines can be implemented and followed. However, screening and management criteria for glucose metabolism disorders must be consensus-based with higher evidence in order to limit the variability of practices and prevent CFRD-related complications.

Tratamiento del xantogranuloma palpebral en el adulto mediante colgajos en isla del área zigomática / Treatment of adult eyebrow xanthogranuloma with a zygomatic island flap

El término Xantoma, introducido por Frank Smith en1869, significa tumor amarillo. El Xantogranuloma en el adulto es una afección cutánea inusual, de localización única, sin afectación visceral y que no remite espontáneamente. La afectación palpebral representa el 25% de los casos y en la mayoría de los casos suele ser un nódulo solitario. Presentamos el caso de un paciente varón de 58 años que acude a nuestro Servicio de Cirugía Plástica por presentar extensas lesiones palpebrales nodulares y bilaterales, decrecimiento lento y coloración amarillenta. Las lesiones fueron diagnosticadas histopatológicamente como xantogranulomas. El defecto palpebral fue reparado mediante un colgajo en isla del área zigomática, descrito por Heywooden 1991, modificado en su diseño original. Nuestro objetivo es describir el Xantogranuloma en el adulto por tratarse de una patología poco frecuente, sus características histológicas y sus posibles diagnósticos diferenciales. Destacamos la utilidad y versatilidad del colgajo zigomático ampliado como una excelente alternativa para la reparación de amplios defectos cutáneos palpebrales inferiores (AU)

The term anthoma, meaning yellow tumor, was introduced by Frank Smith in 1869. Xanthogranuloma in adults is an unusual single-site, cutaneous tumor, with novisceral involvement and without spontaneous regress. Palpebral xanthomas account for 25% of all cases, most of which usually involve a solitary nodule. We present a58-year-old man who was referred to our Plastic Surgery Service with large, yellowish, slow-growing, bilateral palpebral lesions. The histological diagnosis was xanthogranuloma. The palpebral defect was repaired using an island flap from the zygomatic area, described by Heywood in 1991 and modified from the original design. We describe adult xanthogranulomas, which are unusual lesions, and discuss the histological study and differential diagnosis. We also draw atention to the usefulness and versatility of the extended zygomatic flap as an excellent alternative for the repair of large lower palpebral cutaneous defects (AU)

[Influenza vaccination coverage in asthmatic children in France in 2006-2007]. / Couverture vaccinale contre la grippe chez les enfants asthmatiques en France en 2006-2007.

In France, an annual seasonal influenza vaccination has been recommended since 2000 for patients suffering from chronic respiratory diseases, including asthma. Each year, a free influenza vaccination voucher is sent by the French Public Health Insurance authorities to patients with chronic respiratory disease, including severe asthma. In November 2006, this measure was extended to all asthmatic patients, irrespective of asthma severity. The present paper examines the 2006-2007 influenza vaccination coverage rate in 433 asthmatic children aged six to 17 years (mean age: 9.5 years; male: 61%) who consulted a pediatric pulmonologist between March and September 2007 in eight hospitals throughout France. The influenza vaccination coverage rate was 15.7% for the 2006-2007 season (13.9% for the 2005-2006 season and 10.9% for the 2004-2005 season). General practitioners vaccinated 72.1% of the children. Lack of information (42%) was the most frequently reported reason for non-vaccination. Free vouchers (received by 39.6% of the children) significantly increased the vaccination coverage rate (31% versus 5.9%; p < 0.001). In France, in 2006-2007, the influenza vaccination coverage rate in asthmatic children was far below the national public health objective to achieve for the year 2008 (at least 75%). Concerted action is needed to improve the influenza vaccination coverage rate in asthmatic children.

Low influenza vaccination coverage in asthmatic children in France in 2006-7.

In France, annual seasonal influenza vaccination has been recommended since 2000 for patients suffering from chronic respiratory diseases, including asthma. Since 1988, each year from September to December, a free influenza vaccination voucher is sent by the French Public Health Insurance authorities to patients with chronic respiratory disease, including severe asthma. In November 2006, this measure was extended to all asthmatic patients, irrespective of asthma severity. The present paper examines the 2006-7 influenza vaccination coverage rate (VCR) in 433 asthmatic children aged 6 to 17 years (mean age: 9.5 years; male: 61%) who consulted a paediatric pulmonologist between March and September 2007 in eight hospitals throughout France. The influenza VCR was 15.7% for the 2006-7 season (13.9% for the 2005-6 season and 10.9% for the 2004-5 season). General practitioners vaccinated 72.1% of the children. "Lack of information" (42%) was the most frequently reported reason for non-vaccination. Vouchers (received by 39.6% of the children) significantly increased the VCR (31% versus 5.9%; p<0.001). In France, in 2006-7, the influenza VCR in asthmatic children was far below the national public health objective (at least 75% for the year 2008). Concerted action is needed to improve the influenza VCR in asthmatic children.

Lipomatosis simétrica benigna tipo II (Enfermedad de Madelung): a propósito de un caso / Type II benign symmetric lipomatosis (Madelung’s disease): case report

Presentamos el caso de un varón de 56 años de edad con historia de 35 años de abuso de alcohol y sin otra patología asociada, que presentaba masas en hombros, mamas y flancos que habían aumentado de tamaño de forma progresiva durante los últimos dos años y medio, y que dificultaban su movilidad y su actividad laboral. El estudio mediante Tomografía Axial Computerizada apreció un exceso de deposito graso de distribución homogénea no encapsulado (difuso) sobre los hombros, mamas y cintura abdominal, sin afectación de estructuras profundas. Consideramos importante conocer los dos tipos existentes de Lipomatosis Simétrica Benigna, por sus diferencias tanto en la localización de las masas lipomatosas como en la afectación de estructuras profundas. Es por ello que aportamos este caso de Lipomatosis Simétrica Benigna tipo II sin afectación cervical ni de estructuras profundas (AU)

We report the case of a 56 years-old man with a 35- years history of alcohol abuse, but no other illness. The patient presented masses on shoulders, breasts and flanks that had enlarged progressively over the previous two and a half years, and that hindered his work by restricting mobility. Computed Tomography revealed non-encapsulated excess fat deposits evenly distributed on shoulders, breasts and abdomen, without affecting the deep structures. Awareness of the two types of Benign symmetric lipomatosis is necessary because of their differences, both in location of the lipomatous masses and in the involvement of the deep structures. We therefore report this case of type II Benign symmetric lipomatosis without cervical or deep structure involvement (AU)

[Recurrent pneumonia revealing a bronchial carcinoid tumor: report of two cases]. / Pneumopathies récidivantes révélant une tumeur carcinoïde bronchique : à propos de deux observations.

Carcinoid tumors are the most common endobronchial tumor in the pediatric population, and represent a rare cause of airway obstruction. The authors report two cases of boys aged 10 and 11 years old, who presented with a 12-month history of recurrent pneumonia. Bronchial endoscopy showed an endobronchial tumor. Chest CT-scan identified local extension and lung-associated lesions; octreoscan was performed to detect distant metastases. Histopathological study concluded in typical carcinoid tumor. The outcome after surgical conservative resection is uneventful with a follow-up of 7 and 26 months. Bronchial tumors must be considered in children with recurrent pneumonia or persistant respiratory symptoms, and require CT scan and bronchial endoscopy for their diagnosis.

Ventilator-associated pneumonia due to colistin susceptible-only microorganisms.

Acinetobacter spp. and Pseudomonas aeruginosa are common pathogens of ventilator-associated pneumonia (VAP). The presentation and outcome of VAP due to Acinetobacter spp. and P. aeruginosa susceptible to carbapenems (Carb-S; imipenem and/or meropenem) and to colistin only (Col-S) were compared in the present retrospective study in three intensive care units. A total of 61 episodes of VAP caused by Acinetobacter spp. or P. aeruginosa were studied, of which 30 isolates were Carb-S and 31 were Col-S. Demographics, worsening of renal function and mortality were not different. The univariate analysis showed that a later onset and a previous episode of VAP, prior antimicrobial therapy for >10 days and previous therapy with carbapenems during the present admission were more frequent in patients with Col-S strains. On multivariate analysis, prior antimicrobial therapy for >10 days and a previous episode of VAP remained significantly associated with Col-S VAP. Approximately 41% of the infections caused by Col-S isolates, but none of those due to Carb-S isolates, had received prior carbapenem therapy. Colistin-susceptible ventilator-associated pneumonia episodes can be effectively treated using colistin without significant renal dysfunction. This susceptibility pattern could be suspected in patients with a previous ventilator-associated pneumonia episode or prior antibiotic therapy for >10 days preceding the present ventilator-associated pneumonia episode.

[Environment and asthma in children: controversies]. / Environnement et asthme de l'enfant: controverses et jusqu' où aller?

The increase in asthma prevalence over the past 20 years could be due to modification of exposure to environmental factors (environmental theory) or to the lost of protective factors (hygienist theory). Among environmental factors this paper reviewed the controversial role of exposure to house dust mite (HDM). If exposure to HDM is deleterious in asthmatics known to be sensitised to this allergen, the effect of HDM exposure on asthma incidence has been challenged recently, based on longitudinal studies showing no correlation between level of exposure to HDM and asthma incidence. Exposure to animal dander may have protective effects. This review has shown that, even if some studies have shown potential protective effect of early exposure, the protection seems to be better in families with a low risk of atopy; almost all studies tend to show a deleterious effect of current exposure; exposure to dog dander may be more protective than cat. These studies cannot give definite conclusions to change current advices of early eviction of HDM and animal dander, especially in families with a high risk of atopy. The deleterious effect of passive smoking on asthma prevalence and severity is undisputable and our efforts should concentrate on obtaining effective eviction of passive smoking.

Lutein and zeaxanthin in the eyes, serum and diet of human subjects.

Inverse associations have been reported between the incidence of advanced, neovascular, age-related macular degeneration (AMD) and the combined lutein (L) and zeaxanthin (Z) intake in the diet, and L and Z concentration in the blood serum. We suggest that persons with high levels of L and Z in either the diet or serum would probably have, in addition, relatively high densities of these carotenoids in the macula, the so-called 'macular pigment'. Several lines of evidence point to a potential protective effect by the macular pigment against AMD. In this study we examined the relationship between dietary intake of L and Z using a food frequency questionnaire; concentration of L and Z in the serum, determined by high-performance liquid chromatography, and macular pigment optical density, obtained by flicker photometry. Nineteen subjects participated. We also analysed the serum and retinas, as autopsy samples, from 23 tissue donors in order to obtain the concentration of L and Z in these tissues. The results reveal positive, though weak, associations between dietary intake of L and Z and serum concentration of L and Z, and between serum concentration of L and Z and macular pigment density. We estimate that approximately half of the variability in the subjects' serum concentration of L and Z can be explained by their dietary intake of L and Z, and about one third of the variability in their macular pigment density can be attributed to their serum concentration of L and Z. These results, together with the reported associations between risk of AMD and dietary and serum L and Z, support the hypothesis that low concentrations of macular pigment may be associated with an increased risk of AMD.

Measurements of respiratory system resistance by the interrupter technique in healthy and asthmatic children.

We studied respiratory system resistance by the interrupter technique (Rint) in healthy and asthmatic children, paying special attention to the effect of cheek compliance and the effects of supporting the cheeks, the influence of lung volume at which interruption was performed, the effect of direction of flow before occlusion (inspiration vs. expiration), and short-term reproducibility of this method. One hundred and thirty-two children (36 controls and 96 asthmatics) were included in the study (mean, 9.0 +/- SD 3.6 years). Rint was calculated from the ratio of the alveolar pressure (estimated from moth pressure during occlusion) to the flow prior to interruption. We observed that 1) underestimation of airway resistance due to upper airway compliance can be minimized by supporting the cheeks; Rint was significantly lower when measured without supporting the cheeks than with support of the cheeks in controls (3.9 +/- 0.9 vs. 4.7 +/- 1.2 cmH2O.L-1.s, respectively) and asthmatics (5.2 +/- 1.6 vs 6.9 +/- 2.0 cmH2O.L-1.s); the quantitative differences of Rint with and without cheek support was larger in small children and in the most obstructed children; 2) performing occlusion at mid-tidal volume accurately reflected the respiratory system resistance of the whole respiratory cycle since we observed no difference in Rint when performing occlusion at different volumes during quiet respiration or at the middle of tidal volume; 3) Rint measured during expiration was higher than Rint obtained during inspiration in controls and in asthmatics; moreover, the effect of direction of flow before occlusion was greater in the small children; 4) Rint was closely correlated to height in controls (r = -0.82; P < 0.001); and 5) short-term reproducibility (at 15 min intervals) was satisfactory in controls and asthmatics (coefficients of variation were 9% and 7%, respectively).