[Pulmonary complications of Chronic Granulomatous Disease]. / Complications pulmonaires de la granulomatose septique chronique.

Chronic Granulomatosis Disease (CGD) is an inherited immune deficiency due to a mutation in the genes coding for the subunits of the NADPH oxidase enzyme that affects the oxidative capacity of phagocytic cells. It is characterized by increased susceptibility to bacterial and fungal infections, particularly Aspergillus, as well as complications associated with hyperinflammation and granulomatous tissue infiltration. There exist two types of frequently encountered pulmonary manifestations: (1) due to their being initially pauci-symptomatic, possibly life-threatening infectious complications are often discovered at a late stage. Though their incidence has decreased through systematic anti-bacterial and anti-fungal prophylaxis, they remain a major cause of morbidity and mortality; (2) inflammatory complications consist in persistent granulomatous mass or interstitial pneumoniae, eventually requiring immunosuppressive treatment. Pulmonary complications recurring since infancy generate parenchymal and bronchial sequelae that impact functional prognosis. Hematopoietic stem cell allograft is a curative treatment; it is arguably life-sustaining and may limit the morbidity of the disease. As a result of improved pediatric management, life expectancy has increased dramatically. That said, new challenges have appeared with regard to adults: difficulties of compliance, increased inflammatory manifestations, acquired resistance to anti-infectious therapies. These different developments underscore the importance of the transition period and the need for multidisciplinary management.

Severe Aspergillus endocarditis in a lung transplant recipient with a five-year survival.

We report the case of a patient with cystic fibrosis who underwent lung transplant and developed Aspergillus endocarditis and cutaneous relapse. Long-term survival was achieved with surgical and prolonged antifungal treatment. This case report emphasizes the recommendation of life-long antifungal treatment in transplant recipients who survive an episode of fungal endocarditis.

[Management of the pulmonary complications of haematological malignancy]. / Approche diagnostique d'une pneumopathie chez un malade atteint d'hémopathie maligne.

The high frequency of pulmonary complications of haematological malignancy and the increasing number of patients treated for these disorders make it important that the respiratory physician has a structured diagnostic approach according to: 1 the immune deficiency due to the malignancy and/or the treatment administered; 2 the factors that can modify the risk of infection (anti infection prophylaxis and/or pre-emptive treatment); 3 co-morbidities; 4 extra-pulmonary manifestations. Two main situations can be identified: The patient is aplasic: Initially the pneumonias are predominantly of bacterial origin but may be fungal if the neutropenia is prolonged. The respiratory physician is faced with two problems: 1 the diagnosis of pneumonia; this may be helped by CT scanning; 2 The choice of antibiotics; this will depend on previous investigations. The patient is not aplasic: The lung disease may have many causes, mainly infectious but also drug related, tumoral, haemorrhagic or embolic. The main problem is the correct choice of investigations to establish an aetiological diagnosis. The collection of data according to a pre-established protocol based on simple factors (study of the notes and clinical examination) is one of the key elements for improving the prognosis of these patients whose management should be multidisciplinary following a pre-defined plan.

[Inflammatory pseudotumor: a rare cause of proximal bronchial obstruction]. / Une cause rare d'obstruction bronchique proximale récidivante: la pseudo-tumeur inflammatoire.

Inflammatory pseudotumor of the lung is a rare disease with usual benign course. Relapse after incomplete resection and aggressive forms have already been described. We report the case of a 67-year-old woman with atelectasis of the left lung related to an endobronchial inflammatory pseudotumor. After laser resection, relapse occurred 3 months later, requiring surgical treatment.

Experimental study on the in vivo behaviour of a new collagen glue in lung surgery.

OBJECTIVE: To study the pneumostatic ability of a collagen polymerised with a polysaccharide (GAO) glue in lung surgery; its influence in pleuro-pulmonary adhesion formation; the pulmonary tissue reaction to it, its biodegradability, and the eventual alterations of pulmonary compliance induced by the glue. METHODS: Two groups of ten rabbits (controls and treated) were operated under ventilatory assistance by thoracotomy to promote pleural adhesions, and injury to the lung. Repeated chest X-rays were carried out postoperatively. Lungs were examined histologically at day 40. In vitro tests were performed to study glue effects on pulmonary compliance. RESULTS: Air leaks stopped 2 min after glue application. Persistent pneumothorax were likely seen in treated rabbits (ns). Glue induces a temporary reduction of pulmonary compliance. Glue did not increase adhesion formation, or interfere with the healing process. CONCLUSIONS: For its properties, GAO seems to be a good and well-tolerated tool to reduce air leaks from the lung, without inducing residual pleural symphysis.

Coexistence of acute cellular rejection and lymphoproliferative disorder in a lung transplant patient.

We report the case of a 37-year-old man who underwent bilateral lung transplantation for end-stage cystic fibrosis. Two months after his operation, a computed tomographic scan showed multifocal nodules throughout both lungs. Endobronchial biopsies revealed an Epstein-Barr virus-associated B-cell lymphoproliferation. Transbronchial biopsies revealed perivascular lymphoid infiltrates composed of predominantly small T lymphocytes. These perivascular infiltrates were retrospectively considered to be an acute cellular rejection rather than the periphery of the lymphoproliferative disorder. This opinion was based on several arguments: (a) a decrease in dosage of maintenance immunosuppression led to total regression of the lymphoproliferation but did not affect the perivascular lymphoid infiltrates; (b) the treatment of the acute cellular rejection temporarily induced the disappearance of the perivascular infiltrates; (c) the expression of Epstein-Barr virus was not detected in the perivascular infiltrates; and (d) on autopsy, performed 1 year later, severe obliterative bronchiolitis lesions were discovered, for which acute cellular rejection is the main risk factor. These observations point to the possibility that acute cellular rejection and an Epstein-Barr virus-associated lymphoproliferative disorder may coexist.

Accuracy of cytology vs. microbiopsy for the diagnosis of well-differentiated hepatocellular carcinoma and macroregenerative nodule. Definition of standardized criteria from a study of 100 cases.

OBJECTIVE: To determine the accuracy of ultrasound (US)-guided fine needle aspiration (FNA) for the diagnosis of well-differentiated hepatocellular carcinoma (wd HCC) and macroregenerative nodule (MRN) and to identify the most useful cytologic and histologic criteria to distinguish between those two diagnoses. STUDY DESIGN: Cytologic and histologic specimens of 50 wd HCC and 50 MRN were reviewed blindly and the diagnosis compared to the final clinical diagnosis. Twenty-eight cytologic and 25 histologic criteria were examined and subjected to statistical analysis. RESULTS: Among 100 cases studied, the final diagnosis was available for 43. In those 43 cases, combining analysis of cytologic and histologic specimens, the sensitivity of US-guided FNA was of 75% and the specificity 100%. Cytologic analysis was better than isolated histologic analysis, with a sensitivity of 75% vs. 68%, respectively. Sensitivity of cytologic diagnosis was lower for smaller nodules and for those located in poorly accessible hepatic segments. With the use of stepwise logistic regression analysis, four cytologic features (increased nuclear/cytoplasmic ratio, cellular monomorphism, nuclear crowding, loss of bile duct cells) and four histologic features (increased nuclear/cytoplasmic ratio, decreased Kupffer cells, cellular monomorphism, increased trabeculae thickness) were identified as predictive of HCC.

[Role of guided fine needle punction in the diagnosis of deep-seated Aids-related infections. Report of a case of hepato-nodal histoplasmosis]. / Place de la ponction guidée à l'aiguille fine dans le diagnostic des infections profondes du SIDA. A propos d'un cas d'histoplasmose hépatoganglionnaire.

A case of disseminated histoplasmosis diagnosed by fine needle aspiration biopsy is reported. The patient suffering from acquired immune deficiency syndrome (AIDS) had enlarged liver, spleen and mesenteric lymph nodes. Cytological smears prepared from a CT scan guided fine needle aspiration biopsy of one of the lymph node and the liver, showed numerous free or intrahistiocytic yeasts consistent with Histoplasma capsulatum. Yeasts and protozoars morphologically close to Histo-plasma capsulatum are reviewed. The indications of fine needle aspiration biopsy for the diagnosis of infections in AIDS patients are emphasized. This method enables to send rapidly material for cultures and to start immediately an appropriate treatment.

[How fine needle aspiration biopsy make it possible to avoid extensive surgery. A case of abdominopelvic actinomycosis]. / Comment la ponction guidée à l'aiguille fine peut éviter une chirurgie mutilante.

A case of abdominopelvic actinomycosis diagnosed by fine needle aspiration biopsy is reported. The patient, using an intrauterine device for five years, had a pelvic mass infiltrating the left ovary and five hepatic masses. Cytological smears prepared from an ultrasound-guided fine needle aspiration biopsy of one of the hepatic masses, showed actinomycotic granule. In the literature, few cases of abdominopelvic actinomycosis diagnosed by fine needle aspiration biopsy are reported. This affection, able to simulate a neoplasia on clinical and radiological grounds, is usually diagnosed on the histology of the surgical specimen. Ultrasound and computerized tomography allows to guide fine needle into necrotic areas where the probability to meet actinomycotic granules is maximum and so to avoid an extensive surgery.

[The intratesticular leiomyoma: an unusual location. Report of a case]. / Le léiomyome intratesticulaire: une localisation exceptionnelle. A propos d'un cas.

Intratesticular location of leiomyoma is unusual. A single case has been published in the literature. We report a case of what we consider to be an intratesticular leiomyoma, with a description of its pathology, a discussion of its differential diagnosis and histogenesis.

Detection of telomerase status by semiquantitative and in situ assays, and by real-time reverse transcription-polymerase chain reaction (telomerase reverse transcriptase) assay in bladder carcinomas.

OBJECTIVE: To investigate whether telomerase activity could be used as a diagnostic and/or prognostic marker of bladder carcinoma, by assessing telomerase activity using semiquantitative and in situ methods, and quantifying telomerase reverse transcriptase (hTERT) mRNA expression in a series of bladder carcinomas. MATERIAL AND METHODS: Telomerase activity was detected by the telomeric repeat amplification protocol (TRAP) assay using a telomerase polymerase chain reaction (PCR) enzyme-linked immunosorbent assay (ELISA) kit on a series of 29 bladder carcinomas and on three normal bladder samples. hTERT mRNA levels were quantified using real-time quantitative reverse transcription (RT)-PCR. For the in situ detection of telomerase activity, the same telomerase PCR ELISA kit was used and applied to frozen-tissue sections only for discordant cases between telomerase activity and hTERT mRNA status. RESULTS: Telomerase activity was positive in 15 of the 29 bladder carcinomas (52%) and negative for the three normal bladder samples. hTERT was detected and quantified in all tumour samples, with major differences in hTERT values. None of the three normal bladder samples had quantifiable hTERT mRNA, giving complete sensitivity and specificity for the method in diagnosing bladder carcinoma. Comparing the results of RT-PCR and TRAP assay showed a significant association between the enzyme activity and levels of hTERT mRNA expression, with only five discordant cases, most of them being TRAP-negative and hTERT-positive. Among these cases the in situ results of telomerase activity were concordant with hTERT mRNA levels by RT-PCR and not with TRAP assay results, as nuclear fluorescence of malignant epithelial cells. The semiquantitative evaluation of positive cell numbers showed a heterogeneity of telomerase activity within the tumour tissue. There was a significant correlation between RT-PCR and histopathological variables (grade and stage), and a significant correlation between TRAP assay results and histopathological grade. CONCLUSION: Detecting hTERT mRNA by RT-PCR seems to be a promising method, with a much better sensitivity than the TRAP assay in diagnosing bladder carcinomas, and a better correlation with histopathological variables. False-negative cases on the TRAP assay are explained by the heterogeneity of telomerase activity within tumour cells. Thus evaluating hTERT gene expression levels could be used as a marker of malignant progression, useful in the early diagnosis and follow-up of bladder carcinomas.