[French practical guidelines for the diagnosis and management of IPF - 2021 update, full version]. / Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique ­ Actualisation 2021. Version intégrale.

BACKGROUND: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated. METHODS: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale. RESULTS: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis. CONCLUSION: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis.

[French practical guidelines for the diagnosis and management of IPF - 2021 update, short version]. / Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique ­ Actualisation 2021. Version courte.

BACKGROUND: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated. METHODS: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale. RESULTS: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis. CONCLUSION: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis.

[A case of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) syndrome]. / Un cas clinique de syndrome d'hyperplasie neuroendocrine pulmonaire diffuse idiopathique (DIPNECH).

INTRODUCTION: DIPNECH is a strictly histological entity according to the WHO 2015 classification and is considered to be at pre-neoplastic risk. It has been proposed that DIPNECH syndrome should be used to describe patients have clinical symptoms, an obstructive ventilatory disorder and compatible radiological abnormalities. The diagnosis is histological and usually based on a surgical lung biopsy. CASE REPORT: We report the case of a 58-year-old woman with a chronic cough for over 20years who had an obstructive airway pattern on spirometry. Diagnoses of asthma and COPD had been discussed. After 7years of follow-up, the DIPNECH hypothesis was evoked on the scanning aspect of mosaic attenuation, expiratory trapping and micronodules, which was subsequently confirmed by surgical pulmonary biopsy. CONCLUSION: It is necessary to consider the possibility of this rare disease in order to avoid inappropriate treatments and in the hope that future therapeutic advances (somatostatin analogs, mTOR inhibitors) improve patients' experience and the progression of respiratory function.

[A rare cause of eosinophilic asthma: Hyper-IgG4 syndrome (IgG4-related sclerosing disease)]. / Une cause rare d'asthme éosinophilique : le syndrome hyper-IgG4 (maladie fibro-sclérosante liée aux IgG4).

INTRODUCTION: The hyper-IgG4 syndrome is an emerging disease with various thoracic manifestations: eosinophilic asthma, adenomegalies or mediastinal fibrosis, asthma, infiltrative pneumonia, nodules or pseudo-tumors with bronchial and pleural localizations. CASE REPORT: We report the case of a 38-year-old woman who was admitted for acute exacerbation of eosinophilic asthma with high total IgE levels. A medical history of idiopathic acute pancreatitis, associated with sclerosing cholangitis and renal failure, suggested a diagnosis of hyper-IgG4 syndrome in this woman with late-onset severe and non-atopic asthma. A previous hepatic histology and a reinterpretation of renal tomodensitometry have confirmed this hypothesis. CONCLUSION: The hyper-IgG4 syndrome is responsible of different clinico-radiological patterns that should be evoked because of the pejorative evolution of this steroid sensitive disease.

Room-temperature ferromagnetism of all-epitaxial ß-Fe-Ge/diamond-Ge/ß-Fe-Ge trilayers.

We report on the first all-epitaxial ferromagnet/inorganic semiconductor/ferromagnet hybrid heterostructure that exhibits (i) a Ge barrier of diamond crystal structure, (ii) room-temperature ferromagnetic electrodes and (iii) very smooth interfaces. Both bottom- and top-Fe-Ge electrodes exhibit tiny in-plane magnetic anisotropies dominated by a magnetocrystalline contribution of six-fold symmetry originating from the hexagonal symmetry of the B82 (Ni2In) ß-Fe-Ge phase. A key result is the absence of any magnetic coupling between these soft-magnetic electrodes for Ge barrier thickness as low as ~2.5 nm, which allows us to easily tune the parallel and antiparallel magnetic alignments by applying suitably small magnetic fields. This confirms the beneficial use of H-surfactant in order to drastically reduce the roughness of the Ge barrier, as revealed by our scanning tunneling microscopy and transmission electron microscopy measurements. This new all-epitaxial ferromagnet/semiconductor hybrid system appears, therefore, to be a promising candidate for the realization of magnetic tunnel junctions with a single crystal semiconductor barrier that are fully compatible with Si-based technology.

[Resistance of Streptococcus pneumoniae to penicillin. "Bacteriological" resistance/"clinical" resistance]. / La résistance de Streptococcus pneumoniae à la pénicilline résistance "bactériologique"/résistance "clinique".

From January 1988 to December 1992, 143 Streptococcus pneumoniae strains have been isolated at a significant level from 136 patients hospitalized in the pulmonary care unit of CHI Créteil. The clinical presentations were: 83 pneumonia (P), 48 acute bronchitis (B) and 12 colonizations (C). Fourteen strains were found to be penicillin resistant (MIC > 0.125 mg. l-1): 11 P, 2 B, 1 C. Two patients were lost of view after the beginning of the treatment: a pneumonia (Serotype 23F, MIC = 2 mg. l-1) and a bronchitis (Serotype 23F, MIC = 2 mg. l-1). Treatment was pristinamycine for one and amoxicilline alone or associated to clavulanic acid for the other 13. We observed the failure of the treatment in two. A pneumonia (Serotype 23, MIC = 2 mg. l-1) was treated before hospitalization by josamycine (3 days), then cefpodoxime (4 days) and amoxicillin-clavulanic acid (1.5 gr/d during 7 days). Healing was obtained with the increase to 3 gr/d. The other failure was a pneumonia without improvement after 11 days with 3 gr/d of amoxicilline. The treatment was stopped during 5 days and a protected brushing was performed. A Streptococcus Pneumoniae (Serotype 23F, MIC = 8 mg. l-1) was isolated. The clinical status rapidly improved with erythromycine (3 gr/d) to which the strain was susceptible. "Bacteriological" Resistance to penicillin is demonstrated (10 percent in our sample, 17 percent in France during 1991). However amoxicillin to a sufficient dosage is still enough ("Clinical" Resistance < 1 percent in our sample) for the treatment of respiratory infections except when the MIC is at a high level.