Dyspnea assessment in myotonic dystrophy type 1.

In myotonic mystrophy type 1 (DM1), combining respiratory symptom screening and respiratory function testing, is crucial to identify the appropriate time for ventilatory support initiation. Dyspnea has been little investigated in DM1. To provide a multidimensional description of dyspnea, questionnaires assessing dyspnea were administered to 34 consecutive adult patients with DM1 (median (25th-75th centile) age of 36 (28-49), Vital Capacity (VC) of 74 (64-87)% of predicted value). Dyspnea scores were low whatever the questionnaire used: Multidimensional Dyspnea Profile score of 2(0-4.7)/50 for dyspnea sensory descriptor and of 0 (0-4.7)/60 for the emotional descriptor, Visual Analogue Scale score of 0 (0-0)/10 in sitting and supine position and Borg score after six-minute walk test (6MWT) of 2.2 (1.8-4.2)/10. Eleven patients (32%) reported disabling dyspnea in daily living (modified Medical Research Council (mMRC) score ≥ 2). In comparison with patients with mMRC score < 2, patients with mMRC score ≥ 2 had a more severe motor handicap (Muscular Impairment Rating score of 4.0 (4.0-4.0) vs 3.0 (2.0-3.5), p<0.01), a lower 6MWT distance (373 (260-424) vs 436 (346-499)m, p = 0.03) and a lower VC (64 (48-74)% vs 75 (69-89)%, p = 0.02). These data suggest that the mMRC scale might be an easy-to-use and useful tool to assess dyspnea in daily living in DM1 patients. However, the interest of integrating the mMRC dyspnea scale in clinical practice to guide therapeutic management of DM1 patients remains to be assessed in further studies.

[Systemic lupus erythematosus presenting as severe alveolar hypoventilation and the shrinking lung syndrome]. / Hypoventilation alvéolaire sévère révélant un shrinking lung syndrome lupique.

INTRODUCTION: The shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus. CASE REPORT: A 69-year-old man presented with exertional dyspnoea, muscle weakness, and weight loss of 15kg in 6months. Pulmonary function tests revealed a restrictive lung disorder, with a dramatic decrease in maximal inspiratory pressure (17% of theoretical value), and alveolar hypoventilation (pH 7.43; PaCO2 55mmHg). A thoracic CT-scan showed bilateral diaphragmatic elevation. The creatinine phophokinase level was increased at 280U/L. Progress was marked by a rapidly increasing respiratory acidosis (pH 7.24, PaCO2 109mmHg) requiring invasive ventilation. Auto-immune studies revealed positive anti-nuclear antibodies (1/800) and positive anti-native DNA antibody at 45U/L. Treatment with systemic corticosteroids led to an initial improvement but it was not possible to discontinue mechanical ventilation. The outcome was fatal. Autopsy did not reveal any other cause and a diagnosis of the SLS associated with lupus was confirmed. CONCLUSION: The interesting features of this case report consist of: 1) the presentation of the SLS as an alveolar hypoventilation with a fatal outcome, 2) the presentation of systemic lupus as SLS.

Phase II trial of paclitaxel and carboplatin in metastatic small-cell lung cancer: a Groupe Français de Pneumo-Cancérologie study.

PURPOSE: To evaluate the efficacy and safety of paclitaxel and carboplatin in the treatment of previously untreated patients with metastatic small-cell lung cancer (SCLC). PATIENTS AND METHODS: Eligible patients were aged 18 to 75 years with an Eastern Cooperative Oncology Group (ECOG) score < or = 2 and life expectancy > or = 12 weeks. Paclitaxel (200 mg/m(2)) was infused over 3 hours, before carboplatin (area under the curve [AUC] 6; Calvert formula) infused over 1 hour, once every 3 weeks for six cycles maximum. Prednisolone, dexchlorpheniramine, and ranitidine were standard premedication. Response to treatment was assessed every two cycles, and nonresponding patients were withdrawn from the trial to receive standard chemotherapy. RESULTS: Of the 50 patients entering the study, 48 and 46 patients were assessable for toxicity and response, respectively. The overall response rate was 65%, with complete responses in three patients. Five patients had stable disease (11%) and 11 patients experienced progressive disease (24%). Median survival was 38 weeks, and median duration of response was 20 weeks. One-year survival was 22.5%. For a total of 232 cycles, grade 3 and 4 toxicity was 33% for neutropenia, 3.5% for thrombocytopenia, and 4% for anemia. Four patients had neutropenic fever (one toxic death). Nonhematologic toxicity was mainly grade 1 and 2 paresthesia (21% of patients); grade 3 myalgia/arthralgia was observed in 6.5% of patients. CONCLUSION: First-line chemotherapy with paclitaxel and carboplatin in metastatic SCLC achieved a response rate and survival similar to standard regimens. With 1-day administration and a tolerable toxicity profile, this combination merits further investigation.

[Fire-eater's lung]. / Pneumopathie des cracheurs de feu.

INTRODUCTION: Fire-eaters use kerdan, a petroleum-derivative, during their performances. Its accidental inhalation produces respiratory symptoms known as fire-eater's lung. CASES: We report 8 cases of fire-eater's lung and compare their clinical and radiologic data and outcome with those of 44 cases from the French and English-language literature. Presentation is highly characteristic: i) occurrence in young inexperienced subjects; ii) immediate phase characterized by coughing and digestive symptoms; iii) after a latency period, pseudoinfectious lung disease frequently associated with extra-respiratory symptoms, usually digestive; iv) generally favorable clinical and radiological outcome, rarely complicated by pneumatocele or pneumothorax. CONCLUSION: Diagnosis of fire-eater's lung is based on history-taking and characteristic clinical and radiological presentation. Treatment is symptomatic. Prevention relies on educational campaigns about the risks of fire-eating.

Combination chemotherapy with cisplatin, etoposide and gallium chloride for lung cancer: individual adaptation of doses.

Twelve inoperable lung cancer patients were treated with a combination chemotherapy of cisplatinum (CDDP) and etoposide (VP16), as a continuous infusion for 5 days, every 21 days, and with a daily oral administration of GaCl3. Dosages of CDDP and VP16 were adapted in order to obtain an area under the curve (AUC) of 80,000 micrograms l-1.h for plasma total platinum and of 200 mumol.l-1 h for plasma VP16 during each 120 h infusion. GaCl3 was given at the dosage of 400 mg/24h from the time of diagnosis at least until the evaluation after 3 courses of chemotherapy. An objective response was observed in 5 non small cell (NSCLC) lung cancer patients (group 1) and 3 small cell (SCLC) lung cancer patients (group 2). In the other 4 patients with a NSCLC no partial response was noted (group 3). No significant difference in area under the curve (AUC) was noted between the 3 groups, either for plasma total platinum (group 1 = 89,598 +/- 20,843 micrograms l-1.h; group 2 = 88,081 +/- 15,431 micrograms l-1.h; group 3 = 83,820 +/- 13,455 micrograms l-1.h), or for VP16 (group 1 = 227 +/- 41 mumol.l-1 h; group 2 = 217 +/- 29 mumol.l-1.h and group 3 = 211 +/- 30 mumol.l-1.h). The maximal plasma Ga concentrations were 244 +/- 34 micrograms/l in group 1, 112 +/- 57 micrograms/l in group 3 (p less than 0.005) and 243 +/- 132 micrograms/l in group 2. It was then decided to increase the dose of GaCl3 in the further non-responding patients. In 6 responders, 3 additional courses of this combination chemotherapy could have been given without major toxicity, allowing a much more important decrease in the tumor volume in 4 of them. This schedule of treatment should permit the chemotherapy to continue for longer than 6 courses, in order to improve the survival time.

[Importance of serum TPA determination in bronchopulmonary cancer. A comparative study of CEA, CA 19.9 and NSE]. / Intérêt du dosage du TPA sérique dans le cancer broncho-pulmonaire. Etude comparative avec l'ACE, le CA 19.9 et la NSE.

This study concerns 45 patients group one suffering from broncho-pulmonary cancer, the diagnosis was obtained by bronchial biopsies or by transparietal puncture using a scanner: there were 35 non-small cell bronchial carcinomas (CNPC) and 10 small cell bronchial cancers (CPC). The control patients (99 patients) were divided up as follows: 44 pleuro-pulmonary infections (group two) and 55 with respiratory failure of various causes other than infectious episodes (group three). In group one the level for TPA was positive in 30 cases (the threshold value was 90 units per litre), 9 for CA 19.9, 7 for ACE and 9 for NSE. The overall sensitivity was thus better for TPA. There was no correlation between TPA and type of tumour histology nor between the different markers. Their association did not improve the sensitivity. The NSE however, remained the most sensitive test for the diagnosis of CPC with six positive tests out of ten. In the control population, the specificity of TPA (66%) was less than that of ACE (100%) or of CA 19.9 (94%) and the false positives were significantly more numerous in group two: 21 patients had a positive test compared to only 12 in group three. Finally we noticed an increase in the level of TPA contrary to other markers, as a function of the extent of the disease from the carcinoma (CNPC unique). The TPA is thus the most sensitive and it turns out to be better reflector to the extent of the tumour disease than either ACE, CA 19.9 or NSE but this applies uniquely to non-small cell carcinoma.

[Carcinoid thymus tumor at an advanced age: diagnostic value of mediastinal needle biopsy with computerized tomography]. / Tumeur carcinoïde du thymus à un âge avancé: intérêt diagnostique de la ponction-biopsie médiastinaie sous tomodensitométrie.

Carcinoid tumour of the thymus is a rare neuroendocrine tumour particularly at an advanced age. The authors report a case of a mediastinal mass in a man aged 85, the mass had remained asymptomatic for a long time. It was decided to achieve a diagnosis because the tumour was causing local compression: a mediastinal needle biopsy under computerised tomographic control confirmed that this was a carcinoid tumour and a study of the biopsy material using an electron microscope showed neurosecretory granules. A sternotomy enabled the tumour to be excised but a post-operative Pseudomonas pneumonia led to the death of the patient. This case underlines the diagnostic place of mediastinal needle biopsy in the presence of a mediastinal tumour. The technique can be carried out under computerised tomography or ultrasonography and this can be associated with a study of the biopsy specimen using electron microscopy which enables the diagnosis to be made before any therapeutic decisions. The treatment of choice of a carcinoid tumour of the thymus is surgery which confirms the tumour limits and also its thymic origin. Tumour excision can be completed using radiotherapy or even chemotherapy.

[Bronchial biopsy, curative treatment for cancer in situ?]. / Le prélèvement biopsique bronchique, traitement curatif des cancers in situ?

Bronchial carcinoma in situ is an intra-epithelial proliferation of tumour which does not cross the basement membrane and is asymptomatic. The evidence for this cancer often rests on a biopsy carried out on a bronchus which may show simple inflammation or may even be normal. We report a new observation on a bronchial carcinoma in situ which was completely ablated after a bronchial biopsy. However surgery remains the first form of treatment for bronchial cancer. If the patient is inoperable, endobroncho-cryotherapy, radiotherapy or phototherapy may be tried but tumour recurrence remains a possibility.

[Detergent enzymes: has the risk of occupational sensitization disappeared?]. / Enzymes des lessives: le risque de sensibilisation professionnelle a-t-il disparu?

Occupational allergy to enzyme detergents had almost disappeared by the use of preventive measures, both individually and in industries. The occurrence of 8 new cases of asthma in a detergent factory has led to a prospective clinical and aetiological enquiry. Although there was no documentation using the specific provocation test or by measuring respiratory function whilst at work, the occupational character of this asthma seemed possible after taking a history. Sensitization to the enzymes used (Savinase, Maxatase and Biozym P 300 S) was shown by skin tests (8 positive to all the enzymes) and the level of specific IgE to Rast (8 times greater than class 3 for Savinase and Maxitase) and for HBDT for Maxatase and Biozym P 300 S (greater than 50% in 8 patients). From another stand point the incomplete application of preventive measures within the factory did not seem to totally explain the origin of this sensitization. In fact, it was the study of the morphology of the enzyme grains, which had been introduced into the factory which helped us to explain the mechanism. In effect it was the capsules of the grain which were fissured and thus allowed allergen contact. The replacement of these grains whose envelope was intact, led to the disappearance of the symptoms and confirmed our hypothesis. There remains a continuing risk from occupational sensitization to asthma and it is suggested that there is continued collaboration between the medical profession and industry.

[Bronchial epidermoid carcinoma with unusual disclosure]. / Carcinome épidermoïde bronchique de révélation inhabituelle.

Dissemination has often occurred before the diagnosis of bronchopulmonary cancer. Pancreatic metastases are exceptional and are very rarely the revealing manifestation. The authors report a case of a tumour of the pancreas which led to the discover of an bronchogenic epidermoid carcinoma. The relationship between these two tumours lead to the proposed hypothesis of pancreatic metastasis of a bronchogenic carcinoma. Although this situation is rare, the preoperative work-up for bronchogenic carcinoma should include computed tomography of the pancreas if the abdominal echography does not allow complete visualization of the pancreas in cases with suggestive digestive disorders. If a tumour is observed in the pancreas, scan-guided needle biopsy should be performed to enable the histological examination.

[Primary myxoid mediastinal liposarcoma]. / Liposarcome myxoïde primitif du médiastin.

Mediastinal liposarcomas (LPS) are rare tumours. We report a case of primary myxoid LPS in a 22-year-old woman suffering from cough, dyspnoea on exercise and asthenia for 3 weeks. Thoracic MRI showed a large tumour on the right side. After neoadjuvant chemotherapy, a complete resection was performed, followed by adjuvant thoracic irradiation. Eighteen months after the diagnosis, no sign of recurrence was detected. Mediastinal LPS include a heterogeneous group of bulky tumours, the progression of which depends on the histological type. The prognosis is dominated by the operability of the tumour. Adjuvant therapies are not established.

Baker's asthma related to soybean lecithin exposure.

We report two cases of soybean-lecithin-induced asthma in bakers. The patients experienced clinical symptoms in relation to an occupational exposure to this additive. Skin tests were positive with soybean lecithin, RAST showed a sensitization to soybean, and bronchial challenge tests were positive for a dilution of 10(-3) with this allergen. The same tests remained negative among healthy and asthmatic controls. Soybean lecithin, a common additive in bakery, must be added to the list of numerous aeroallergens involved in baker's asthma.

Erythema multiforme due to pyrazinamide.

Drug-induced erythema multiforme is reported with many antimicrobial and antipyretic medications. We present one patient who experienced erythema multiforme and urticaria after administration of pyrazinamide for treatment of cutaneous tuberculosis. The side-effects of pyrazinamide include hepatic or dermatologic disorders, but erythema multiforme has not yet, to our knowledge, been described. Clinical features and discontinuation and reintroduction of the drug led us to consider pyrazinamide the cause of the erythema multiforme, but allergologic studies remained negative, except for the presence of circulating immune complexes. The mechanism of this eruption remains obscure, and definite withdrawal of pyrazinamide seems to be the best therapeutic choice.