Flow cytometric analysis of duodenal intraepithelial lymphocytes (celiac lymphogram): A diagnostic test for celiac disease.

Celiac disease (CD) diagnosis in adults and certain cases of children mainly relies on the assessment of histopathological features in duodenal biopsies. However, none of the histological findings that characterize CD are pathognomonic. This, in addition to the clinical heterogeneity of the disease and the presence of seronegative forms, makes the diagnosis of CD still a challenge. A hallmark of the celiac mucosa is the elevated number of TCRγδ intraepithelial lymphocytes (IEL) in the epithelium, which may remain increased even long after gluten withdrawal. Active disease is also characterized by the decreased CD3- IEL subset. The use of flow cytometry enables a precise cell counting and phenotyping, allowing the ascertainment of both TCRγδ+ and CD3- IEL subsets, what is known as the "IEL lymphogram." Although determination of this lymphogram has become a routine evaluation tool in numerous hospitals, standardization of the technical method will guarantee an accurate performance in order to become a pivotal technique for CD diagnosis. Here we describe the protocol to process duodenal biopsies in order to obtain the IELs from the mucosa and to characterize lymphocyte populations by flow cytometry to obtain the IEL lymphogram.

El vólvulo gástrico en la edad pediátrica: ¿cuándo sospecharlo y cómo manejarlo? / Gastric volvulus in children: when to suspect and how to manage

INTRODUCCIÓN Y OBJETIVO: El vólvulo gástrico es una entidad que puede manifestarse con un amplio espectro clínico, lo que puede llevar a un retraso en el diagnóstico y tratamiento. El objetivo de nuestro trabajo es definir los tipos de vólvulo gástrico, analizando los factores predisponentes, la clínica, los hallazgos radiológicos y el tratamiento de cada uno de ellos. MATERIAL Y MÉTODOS: Se realizó un estudio descriptivo retrospectivo de los vólvulos gástricos diagnosticados en un hospital terciario durante un periodo de 10 años (2006-2016). Se incluyeron 32 pacientes, que se clasificaron en tres grupos en función de la presentación clínica: 7 con la forma aguda, 12 con la forma crónica y 13 con la forma neonatal-lactante. Se recogieron datos epidemiológicos, clínicos, diagnósticos y de manejo de cada caso. RESULTADOS: El vólvulo gástrico órgano-axial fue el subtipo más frecuente al analizar la totalidad de los pacientes (81%), aunque en la presentación aguda el más prevalente fue el mesentérico-axial (86%). En la forma aguda, la clínica más frecuente fue los vómitos no biliosos (86%), siendo en la forma crónica la sintomatología más variada. El diagnóstico se realizó con tránsito gastroduodenal en la mayoría de los casos. Las opciones de tratamiento fueron cirugía o medidas conservadoras, en función del tipo de vólvulo gástrico. CONCLUSIONES: Existen distintas formas de presentación del vólvulo gástrico (aguda, crónica, neonatal-lactante), cada una con una sintomatología y manejo diferentes. Dada la clínica inespecífica, es importante conocer los factores predisponentes y los hallazgos que aumentan su sospecha en la radiografía. La prueba de elección para su diagnóstico es el tránsito gastroduodenal

INTRODUCTION AND OBJECTIVE: Gastric volvulus is an entity with a broad clinical spectrum, which can lead to a delay in diagnosis and treatment. The purpose of this study is to define the types of gastric volvulus, analyzing the predisposing factors, clinical, radiological findings and treatment of each one. MATERIALS AND METHODS: A retrospective, descriptive study of the gastric volvulus diagnosed at a tertiary hospital was carried out during a period of 10 years (2006-2016). Thirty-two patients were included, which were classified into three groups according to the clinical presentation: 7 with the acute form, 12 with the chronic form and 13 with the neonatal form. Epidemiological, clinical, diagnostic and management data were collected for each case. RESULTS: Organo-axial gastric volvulus was the most frequent subtype of all the sample (81%), although in the acute presentation, the most prevalent subtype was the mesentero-axial (86%). In the acute form, the most common symptom was non-bilious vomiting (86%), whereas in the chronic form the clinical presentation was more varied. The diagnosis was made with upper gastrointestinal series in most cases. Surgical or conservative treatment were chosen depending on the type of gastric volvulus. CONCLUSIONS: There are different forms of presentation of gastric volvulus (acute, chronic, neonatal), with different clinical features and implications with respect to treatment options. Due to its nonspecific clinical presentation, it is important to know the predisposing factors and the common radiological findings highly suggestive of the diagnosis. An upper gastrointestinal series is the study of choice for its diagnosis

Severe tracheobronchial stenosis in a patient with Crohn's disease.

Tracheobronchial involvement in Crohn's disease is rare, usually associated with symptoms of tracheobronchitis, and typically responds well to steroids. The authors report a case of a 29-yr old patient with Crohn's disease, who presented with dyspnoea, fever, and a productive cough. Computed tomography of the chest revealed extensive nodular tracheobronchial stenosis, that was accompanied by severe mucosal inflammation at bronchoscopy. High-dose oral steroids diminished the mucosal inflammation, but had limited efficacy on the underlying tracheobronchial stenosis. It is speculated that this relative ineffectiveness of steroids may be due to the persistence of the untreated inflammatory process.

[Role of surgery in the treatment of obstructive respiratory insufficiency]. / Place de la chirurgie dans le traitement de l'insuffisance respiratoire obstructive.

Within 15 years, the prognosis of chronic obstructive respiratory failure has been deeply modified by advances in surgical treatment. In case of emphysema, lung volume reduction permits an improvement in the functional status in a significant number of patients, at least for several years. Moreover, lung transplantation, mainly single lung transplantation, provide currently an actuarial survival rate of 50% at five years.

Myosin heavy chain gene expression changes in the diaphragm of patients with chronic lung hyperinflation.

In striated muscle, chronic increases in workload result in changes in myosin phenotype. The aim of this study was to determine whether such changes occur in the diaphragm of patients with severe chronic obstructive pulmonary disease, a situation characterized by a chronic increase in respiratory load and lung volume. Diaphragm biopsies were obtained from 22 patients who underwent thoracic surgery. Myosin was characterized with electrophoresis in nondenaturing conditions, SDS-glycerol PAGE, and Western blotting with monoclonal antibodies specific for slow and fast myosin heavy chain (MHC) isoforms. Flow volume curves, total lung capacity, and functional residual capacity were measured before surgery in 20 patients. We found that the human diaphragm is composed of at least four myosin isoforms, one slow and three fast, resulting from the combination of three MHC species. Chronic overload was associated with an increase in the slow beta-MHC species at the expense of the fast species (beta-MHC, 78.2 +/- 4.6 and 50.0 +/- 6.5% in emphysematous and control patients, respectively; P < 0.005). Linear correlations were found between beta-MHC percentage and forced expiratory volume in 1 s (r = -0.52; P < 0.02), total lung capacity (r = 0.44; P < 0.05), and functional residual capacity (r = 0.65; P < 0.003). The human adult diaphragm is composed of a balanced proportion of slow and fast myosin isoforms. In patients with chronic obstructive pulmonary disease, the proportion of fast myosins decreases, whereas that of slow myosin increases. This increase appears to be closely related to lung hyperinflation and may reflect an adaptation of the diaphragm to the new functional requirements.

Early release of proinflammatory cytokines after lung transplantation.

BACKGROUND: Systemic hypotension may complicate the early postoperative period after lung transplantation. A release of proinflammatory cytokines secondary to lung ischemia/reperfusion injury could be involved in the pathogenesis of this early hemodynamic failure (EHF). STUDY OBJECTIVE: To assess prospectively whether the occurrence of EHF is associated with a release of cytokines in the systemic circulation. DESIGN: Blood samples were taken daily during the first postoperative week in 26 patients who underwent a double or a single-lung transplantation. These patients were divided into three groups: 7 patients who experienced EHF and subsequently died (EHF group); 15 patients without EHF (control group); and 4 patients without EHF but with an identified sepsis (sepsis group). The serum levels of interleukin (IL)-1beta, tumor necrosis factor-alpha (TNF-alpha), IL-6, and IL-8 were compared among the three groups. RESULTS: In the EHF group, the levels of each cytokine peaked at day 1 postoperatively. Cytokine levels at day 1 were significantly higher in the EHF group than in the control group (p<0.0006) or in the sepsis group (p<0.003 except for TNF-alpha). CONCLUSION: We conclude that EHF is associated with a massive release of proinflammatory cytokines that could play a determinant role in the pathogenesis of this complication.

[Lung volume reduction surgery in emphysema]. / Chirurgie de réduction de volume dans l'emphysème.

Lung volume reduction surgery in emphysema has, as an objective, the reduction of dyspnoea and an increase in the exercise tolerance in patients with respiratory insufficiency suffering from diffuse emphysema. In principle the resection of the most diseased areas of emphysema leads to improvement in the mechanical properties of the emphysematous lung and correct pulmonary hyperinflation. The respiratory function benefits both objective and subjective, produced by surgery are real but transitory and inconstant depending in particular on the evolutionary profile of the emphysematous disease. The indications should be further refined and an objective comparison of different surgical techniques has not been achieved. The impact on the quality of life for these patients is unknown.

Expiratory flow limitation in stable asthmatic patients during resting breathing.

Application of negative pressure at the mouth during tidal expiration (NEP) provides a simple, rapid, noninvasive method for detecting expiratory flow limitation during spontaneous breathing. Patients in whom NEP elicits an increase in flow throughout expiration are not flow-limited (FL). In contrast, patients in whom application of NEP does not elicit an increase in flow during most or part of tidal expiration are considered FL. We have used the NEP technique to assess the prevalence of expiratory flow limitation during resting breathing in sable asthmatic patients in both the seated and supine positions. In patients in the sitting position, we have also assessed flow limitation with the conventional method, based on comparison of tidal and maximal expiratory flow-volume (MEFV) curves. We studied 13 patients (FEV1 range: 48 to 94% predicted) with both the NEP and conventional techniques. According to the NEP technique, none of the patients was FL in the seated and only two were FL in the supine position. By contrast, on the basis of the conventional method, six of the patients would have been classified as FL in the sitting position. We conclude that: (1) most stable asthmatic patients do not exhibit tidal expiratory flow limitation during resting breathing; and (2) the conventional method for assessing flow limitation may lead to erroneous conclusions.

[Pulmonary transplantations. Current assessment]. / Transplantations pulmonaires. Le bilan aujourd'hui.

After fifteen years of experience, 3-year survival rates after lung transplantation have reached 60% for heart-lung, two-lung and single-lung procedures. At 7 years, the rate is currently 41%. Lung transplantation has thus become the ultimate treatment for end-stage respiratory failure, and, as we were able to establish in 1988, single lung transplantation is now the indication of choice. Most of the early complications after transplantation, including edema and post-operative shock, though relatively frequent, can generally be controlled. There is however the problem of acute rejection during the first three months following transplantation due to herpes or cytomegalovirus infections which respond poorly to antiviral therapy. Immunosuppressive therapy generally can control acute rejection, but subsequent chronic episodes may account for 25% of long-term failures. Episodes of chronic rejection usually occur after nine months but onset may be retarded beyond 3 years. The real problem today is the small number of donors and the long waiting lists. In France, where 200 lung transplantations are needed annually, the number of donors is dramatically insufficient.

Bronchial Kaposi's sarcoma after single lung transplantation.

Kaposi's sarcoma (KS) has been reported in 6% of malignancies of solid organ transplant recipients. Most of the observations have been in recipients of renal allografts but, so far, KS has not been described in lung transplantation. We report a case of bronchial KS occurring in a black patient 6 months after single lung-transplantation. Skin lesions were absent and, interestingly, KS lesions were observed solely in the trachea and the native lung. Following reduction of the immunosuppressive regimen a complete remission was obtained 1 year later. Up to the present time, this clinical remission is very encouraging, but close surveillance remains necessary to detect rejection episodes or the reappearance of KS following manipulation of the immunosuppressive therapy.

[Tuberculosis: epidemiological, bacteriological and therapeutic changes]. / Tuberculose: des modifications épidémiologiques, bactériologiques et thérapeutiques.

The regular decline in the incidence of tuberculosis up to 1985 left us with the hope that the disease might one day be totally irradicated. But from 1985 on the number of cases has remained unchanged at about 8000 new cases per year in France. Resistant strains have also been identified, requiring new treatment strategies. Polyresistant strains may develop because of non-compliance to standard treatment or, particularly in immunosuppressed patients with AIDS, infection with atypical mycobacteria such as Mycobacterium avium. In developed countries, prophylactic measures for tuberculosis should be based on early diagnosis, rapid initiation of a proven treatment protocol not only to obtain cure but also to reduce contagion, and avoiding contract between high-risk patients. Chemoprophylaxy should be prescribed in case of doubt about contact as the tuberculin test is no longer discriminate due to widespread vaccination. For AIDS patients, the treatment protocol is the same as for normal subjects but should be prolonged. In case of M. avium infection, the most effective treatment combines pyrazinamide, clarithromycin and a third anti-tuberculosis drug.

Expiratory flow limitation in COPD patients after single lung transplantation.

Expiratory flow limitation and dyspnea during resting breathing are common in patients with severe chronic obstructive pulmonary disease (COPD). Although single lung transplantation (SLT) is used to treat end-stage COPD, its effects on flow limitation and dyspnea are not well established. We assessed expiratory flow-limitation and dyspnea in 13 COPD patients after SLT at rest in the sitting and supine positions by applying negative pressure at the mouth during tidal expiration (negative expiratory pressure [NEP] technique). If NEP increases flow throughout the control tidal volume (VT), flow limitation is absent (not flow limited [NEL]). If NEP does not increase flow during part of the control VT, flow limitation is present. After SLT, lung function improved in all but one patient. Twelve patients were NFL during resting breathing in both positions studied. The patient whose lung function did not improve after SLT was flow-limited (FL) both when seated and supine. This patient also exhibited moderately severe chronic dyspnea (Medical Research Council [MRC] score = 3). In the nine other patients in whom dyspnea was assessed, it was slight (MRC score = 1). In conclusion, after SLT for end-stage COPD, expiratory flow limitation at rest is uncommon in both the seated and supine positions. This is consistent with the finding that after SLT the degree of chronic dyspnea is generally slight.

Progressive obstructive lung disease associated with microscopic polyangiitis.

Small airway involvement and progressive severe airflow obstruction are unexpected features in patients with microscopic polyangiitis. We report the case of a patient with microscopic polyangiitis and circulating anti-neutrophil cytoplasmic antibodies (ANCA), who developed pulmonary hyperinflation and airflow obstruction over a 7-yr period. Systemic manifestations of this vasculitis improved under corticosteriods and cyclophosphamid therapy, a treatment that did not influence either the very high level of anti-myeloperoxidase antibodies or the ventilatory impairment. Small airway involvement was suspected on the basis of pathologic small airway lesions and a mild emphysematous pattern on computed tomography (CT) scan, which was out of proportion with the severity of the obstructive lung disease.

[Pneumopathy caused by minocycline]. / Pneumopathie à la minocycline.

BACKGROUND: Minocycline has been identified as a pathogenic agent in drug-induced pneumonia. We report a new case. CASE REPORT: A 38-year-old female asthmatic patient was given minocycline (100 mg/j) for facial acne. She was allergic to penicillin. Other treatments were theophylline, salbutamol and inhaled budesonide. Severe bilateral hypoxemia pneumonia developed with high eosinophil blood counts within a few days of treatment onset. The lung disease regressed with minocycline withdrawal. DISCUSSION: This case is exceptional because the minocycline-induced lung disease continued to progress despite high-dose corticosteroids in this asthmatic patient.

[Selection criteria of candidates for lung transplantation]. / Critères de sélection des candidats à la transplantation pulmonaire.

Amongst the elements which contributed to the success of the early lung transplants at the beginning of the 1980's we feel that the careful selection of candidates probably played a predominant role. If some of the selection criteria initially described remain somewhat intangible, others have either been eased or have been invalidated. The experience acquired over the last 15 years has enabled to precise the optimal moment to include patients on the waiting list and to refine the choice for the type of surgical procedure according to the underlying disease. This article aims to review the different selection criteria for candidates for transplantation and stresses those which have recently undergone change.

Lung volume reduction in patients with severe diffuse emphysema. A retrospective study.

BACKGROUND: For most authors, surgery of emphysema is restricted to resection of large bullae, whereas resection of small bullae or lung volume reduction is generally considered to have poor results. STUDY OBJECTIVE: To report our experience of lung volume reduction in patients with severe emphysema without large bullae. PATIENTS: Thirteen patients were operated on from 1982 to 1992. Before surgery, they all had severe diffuse emphysema with a dyspnea grade 4 or 5 and mean FEV1 values of 18 +/- 5% of predicted. Seven patients had a PaCO2 greater than 42 mm Hg. On radiologic evaluation, they had either small bullae or, most often, areas of destroyed lung. INTERVENTION: The surgical procedure was unilateral in 11 patients and bilateral in 2. MEASUREMENTS AND RESULTS: Postoperative assessment included dyspnea grading, FEV1 measurements, and blood gas analysis followed at 6- to 12-month intervals. There was no perioperative mortality and the morbidity was limited. At 6, 12, 18, 24, and 36 months postoperatively, a symptomatic improvement was observed in 92%, 85%, 54%, 31%, and 31% of the patients, respectively, with FEV1 increasing by at least 20% in 92%, 46%, 46%, 31%, and 24% of the patients, respectively. CONCLUSION: Our data show that lung volume reduction may result in symptomatic and spirometric improvement in patients with severe emphysema without large bullae.

[Pulmonary emphysema: surgical indications]. / Emphysème pulmonaire: indications chirurgicales.

Surgery for pulmonary emphysema, with the exception of lung transplantation, is limited at present to resection of the emphysematous areas. The resection of a unique bulla within an otherwise healthy parenchyma can be indicated in case of complications but rarely in asymptomatic patients. When the bullae are large (i.e. volume greater than one-third of the hemithorax) in a patient suffering from diffuse emphysema, bullectomy is the ideal indication. Mortality varies from 0 to 10%, essentially due to infection or acute respiratory failure. In most patients, the subjective improvement in terms of dyspnea and the objective improvement as measured by spirometry remains significative up to 5 years after surgery. Inversely, surgical resection is classically considered to be contraindicated in patients with small poorly-limited bullae. Recent data would however question this idea since subjective and objective improvement after reduction of the lung volume is still present 1 year after surgery in most patients, even those with severe obstruction. The mechanism is probably related to increased elastic recoil. Even if only temporary improvement can be achieved for a few years, the persisting course of emphysema would suggest that volume reduction should always be entertained as an alternative before lung transplantation.

Refractory hypoxemia during liver cirrhosis. Hepatopulmonary syndrome or "primary" pulmonary hypertension?

We report an uncommon mechanism of severe hypoxemia in two cirrhotic patients under long-term beta-blocker therapy. Our patients presented with profound hypoxemia refractory to oxygen therapy, normal lung radiography and pulmonary function tests, and evidence of right-to-left anatomic shunt. Although these features are highly suggestive of hepatopulmonary syndrome, pulmonary hypertension was present, and a right-to-left shunt through a patent foramen ovale was demonstrated by contrast-enhanced echocardiography. No cause of pulmonary hypertension other than portal hypertension was identified. Pulmonary hypertension and intracardiac right-to-left shunt eventually regressed after discontinuation of beta-blocker therapy. We conclude that "primary" pulmonary hypertension associated with portal hypertension may because of severe hypoxemia during liver cirrhosis. Differential diagnosis of hepatopulmonary syndrome relies upon contrast-enhanced echocardiography and may be of critical importance because of possible therapeutic implications.