[Thyroid manifestations of sarcoidosis: a case report]. / Manifestations thyroïdiennes de la sarcoïdose: à propos d'un cas.

Sarcoidosis is a systemic disease with many localizations. Thyroid involvement has been often described but rarely confirmed histologically. A common immune mechanism appears to be the cause. Thyroid sarcoidosis should be envisaged in patients with a thyroid nodule and mediastino-pulmonary involvement. We report the case of a 63-year-old woman with no past history who was hospitalized for dyspnea. Explorations evidenced a cold thyroid nodule associated with diffuse interstitial lung disease and mediastinal node enlargement. Pathology examination disclosed the sarcoid nature of the thyroid nodule and the lung lesions.

Acute Q fever pneumonia: a review of 80 hospitalized patients.

STUDY OBJECTIVES: To emphasize epidemiologic, clinical, or radiologic characteristics whose detection could lead to an early diagnosis and to enhance therapeutic efficacy. PATIENTS: Eighty hospitalized patients from 1982 to 1996. DESIGN: The diagnosis of Q fever infection was serologically confirmed in all the patients (phase II Coxiella burnetii antibody) using the complement fixation test and/or the indirect immunofluorescence antibody test. RESULTS: Patients from rural and urban areas were noted in the same proportion; however, the usual epidemiologic factors such as contact with cats or farm animals were found in 40% of the patients. Mean age+/-SD was 49+/-20 years, and there was a higher sex ratio of male to female patients (1:3.44). We found a specific seasonal distribution since 80% of the cases occurred between February and May. Delay before referring to hospital was 8.2+/-7.8 days, while 69.3% of the patients received an antibiotic treatment that was mainly penicillin or cephalosporin. The dominant clinical features were dry cough and high fever, as the maximal temperature reached more then 40 degrees C in 58% of the patients. Digestive symptoms were rare. WBC count remained within normal range in 80% of the cases with a low proportion of lymphocytes in half of the patients, and the sedimentation rate was usually elevated (55+/-34 mm). Altered liver function consisted more frequently in an elevated level of alkaline phosphatase (70% of the cases) than transaminases, while hyponatremia was frequently mentioned (28.2% of the patients). We found radiologic evidence of unique lobar or segmental alveolar opacity involving more likely the lower lobes in 55 patients, and multiple or interstitial opacities in the others. Chest radiographs were considered normal in eight patients. The clinical response was favorable in all the patients with a reduction in fever 4.8+/-3.9 days after the start of treatment with the second antibiotic that included mainly erythromycin or quinolones, and chest radiographs returned to normal in 81% of the patients within the first month.

Influence of correction of flow limitation on continuous positive airway pressure efficiency in sleep apnoea/hypopnoea syndrome.

We evaluated the efficiency of two different treatment procedures with continuous positive airway pressure (CPAP) on sleep, nocturnal breathing characteristics and daytime vigilance in 18 newly diagnosed patients with untreated sleep apnoea/hypopnoea syndrome (SAHS) randomly allocated to two different groups. In group I, the positive pressure (PP) level was set to suppress flow limitation (PFL), while in group II the PP was set at a level that eliminated only apnoea/hypopnoea and snoring (PAHS). At the end of a 3 week period of home CPAP therapy, a follow-up sleep study, vigilance and cognitive tests were made. Overall, PFL was significantly higher than PAHS values (PFL: 10.42.6 cmH2O; PAHS: 8.9+/-2.6 cmH2O; p<0.01, mean+/-SD). We found no difference in sleep quality, nocturnal saturation and apnoea/hypopnoea index, or in daytime vigilance tests between the two groups at the end of the treatment period. However, there was a significantly greater scattering in the changes of sleep latency in group II than in group I. This was associated with a significant difference in the daily duration of nasal CPAP use between the two groups (group I: 7.29+/-0.95 h x day(-1); group II: 6.01+/-0.94 h x day(-1); p=0.01) and with a positive correlation between final maintenance of wakefulness test values and the duration of CPAP use (p<0.05; r=0.55). These results tend to show that correcting flow limitation is associated with a higher observance and a more important efficiency in normalizing daytime vigilance than with conventional nasal continuous positive airway pressure.

[Desquamative interstitial pneumopathy: a rare form of diffuse interstitial pneumopathy]. / La pneumopathie interstitielle desquamative: une forme rare de pneumopathie interstitielle diffuse.

Desquamative interstitial pneumonia (PID) is a rare disorder initially described by Liebow in 1965. It is characterised by the presence of an interstitial pneumonia consisting of minimal lesions of interstitial fibrosis and by the accumulation of a rich intraalveolar cellular material principally composed of macrophages. Numerous aetiologies have been found particularly that due to long term Nitrofurantoin. We report the observation of a 57 year old patient, a cigarette smoker, in whom the diagnosis of PID was made after histological examination of a lung biopsy. Based on this observation the pathogenesis and possible different aetiologies of PID are discussed.

Long-term evolution of daytime somnolence in patients with sleep apnea/hypopnea syndrome treated by continuous positive airway pressure.

We demonstrated in a previous study that excessive daytime sleepiness (EDS) in patients who have sleep apnea/hypopnea syndrome (SAHS) and are undergoing continuous positive airway pressure (CPAP) treatment improved differently in two groups of patients: in group I, multiple sleep latency tests (MSLT) were normalized after 50 days of treatment (individual value > 10 minutes), whereas they remained low (individual value < 10 minutes) in group II, with a significant difference between groups. To evaluate the long-term evolution of daytime somnolence under nasal CPAP treatment, five patients from group I and seven patients from group II underwent a new polysomnography and MSLT 4 years after the previous study. Clinical, polysomnographic and MSLT results obtained at baseline before treatment (T1), after the initial 50-day period of CPAP treatment (T2), and after 4 years (T3) were compared. The significant difference in mean MSLT value between the two groups previously observed at T1 and T2 disappeared at T3 (group I: 12.4+/-5.9 minutes; group II: 9.7+/-5.9 minutes). We found that this long-term improvement in excessive daytime somnolence was independent of the initial MSLT value, the severity of SAHS, and the initial MSLT changes under nasal CPAP. Long-term MSLT evolution was significantly correlated to CPAP compliance. These results demonstrate that even in the absence of a significant increase in MSLT at the beginning of CPAP treatment, further improvement is still possible several years later, which may encourage the regular, long-term use of nasal CPAP by patients.

[Differences in efficiency of continuous positive pressure respiration on the diurnal somnolence of patients with sleep apnea/hypoapnea syndrome]. / Inégalité d'efficacité de la pression positive continue (PPC) sur la somnolence diurne des patients atteints de syndrome d'apnées/hypopnées du sommeil (SAHS).

Diurnal hypersomnia was studied in sixteen patients suffering from the sleep apnoea/hypopnoea syndrome (SHAS) Group I, and seventeen snorers Group II. The groups were studied in their basal state then after 51 +/- 14 days of continuous positive pressure (CPP) in Group I with the aid of clinical scores and of multiple latency tests of sleeping episodes (TLME). The patients in Group I presented with increased and pathological diurnal somnolence (TLME = 3.9 +/- 2.45 min) associated with disturbances of oxygen saturation during the course of sleep (a significant reduction of the mean SaO2) and of the pattern of sleep (significant reduction in light slow sleep at the expense of deep slow sleep, with a significant increase in the index of brief arousals). We have found a positive correlation between the initial TLME and the mean SaO2 during the course of sleep (p < 0.05; R = 0.51) without any correlation with other polysomnographic parameters. At the time of the final assessment, the improvement in TLME in the patients of Group I was significant (p < 0.001). However, in a sub-group of patients (BR: n = 7) the TLME were returned to normal using CPP (16.4 +/- 2.21 min) while those in patients in the second sub-group (MR: n = 9) the TLME remained below ten minutes on CPP (6.18 +/- 1.88 min). In the BR subgroup which presented with the most elevated mean initial TLME levels the amplitude of the rise of TLME between the two groups was significantly larger. No initial polysomnographic difference existed between the two sub-groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Anaphylactic reaction to local administration of rifamycin SV.

BACKGROUND AND OBJECTIVE: Systemic reactions during anesthesia are commonly attributed to muscle relaxants, hypnotics, macromolecular solutions, latex, or parenteral antibiotics. After exclusion of these different components as causes, we were interested in the potential implication of rifamycin in the systemic reaction, which occurred during anesthesia, and in the immunologic mechanism by which it can trigger this reaction. METHODS: We report four cases of systemic reactions occurring after local administration of rifamycin. Three patients needed orthopedic surgery, and the fourth needed a urethrotomy. Severe systemic reactions occurred in all four patients when the surgeon washed the incision area with a rifamycin solution. All patients correctly responded to appropriate treatment and recovered. Skin tests were performed 2 months after the incident with the drugs used during anesthesia, latex, and rifamycin. To assess the relationship with a possible IgE-mediated mechanism, two in vitro tests were concomitantly performed to evaluate the cell reactivity to rifamycin: (1) determination of histamine release from peripheral basophils and (2) platelet cytotoxicity test, which explored the presence on platelets of specific IgE antibodies bound to the low-affinity receptor for IgE. RESULTS: Skin tests were performed with different drugs used during surgery, and results were only positive for rifamycin in the four cases, accompanied in two cases by a systemic reaction. Histamine release from basophils was positive in three of four patients. The platelet cytotoxicity test results were positive in all four cases. CONCLUSION: It appears that rifamycin, used locally during surgery, is apt to trigger severe systemic anaphylactic reactions, which are linked to an IgE-related process. This situation is worth pointing out, especially in patients who undergo repeated orthopedic operations during which, at least in Europe, rifamycin is commonly used for the prevention of local sepsis.

Auditory brain-stem responses (ABRs) in sleep respiratory disorders.

Neurophysiological repercussions of sleep respiratory disorders (SRDs) are still unclear. It has been shown that SRDs induce disturbance on auditory event-related potentials with a delayed latency of the cognitive P300 potentials. Since the Auditory Brainstem Responses (ABRs) explore the auditory brainstem structures and are independent on cognition, we evaluated the neurological impact of SDRs on central nervous system by ABRs in these patients. Four groups of patients were studied: snorers (S, 17 subjects), sleep apnoea syndrome (SAS, 48 subjects), apnoeic patients with respiratory insufficiency (SAI, 17 patients) and patients with respiratory insufficiency alone (RI, 12 subjects). A standard polysomnographic study was done in each patient with further quantitative analysis of sleep, respiratory events, and oxygen arterial saturation. ABRs were recorded before the study night and interpeak latencies (IPLs) between waves I, III and V were measured. Results were expressed as the mean of each group for all the different parameters. ABR IPL latencies (IPLI-III, IPLIII-V, IPLI-V) of the four groups were within the normal range. However, comparing groups between themselves, there were significant differences (ANOVA) between groups. Longer IPLIII-V and IPLI-V (which explored the central conduction time) were observed in group SAI compared to group SAS. No evidence on the origin of this elongation was found; the level of hypoxia, in particular, was not related to IPL values. These results show that SAS alone do not affect lower brainstem auditory function, in contrast to the association of SAS and RI.

Predictive factors of long-term compliance with nasal continuous positive airway pressure treatment in sleep apnea syndrome.

The long-term acceptability of treatment with nasal continuous positive airway pressure (CPAP) was studied prospectively in 44 patients with obstructive sleep apnea syndrome. At 14 months on the average after starting treatment with CPAP, 30 patients (68 percent) were found to be compliant (characterized by use of the apparatus every night throughout the night, for more than 5 h per night). The daily use of nasal CPAP was significantly correlated to the initial apnea/hypopnea index (p = 0.013; r = 0.37), as well as to the percentage of light sleep (p = 0.045; r = 0.30) and slow-wave sleep (p = 0.037; r = -0.31) during the initial polygraphic recording. We found a strong correlation between the daily use of nasal CPAP and the difference in the apnea/hypopnea index (p = 0.025; r = -0.34), the difference in mean oxygen saturation during sleep (p = 0.013; r = 0.38), and the difference in hypersomnia scores (p = 0.006; r = -0.40) obtained before and after treatment by nasal CPAP. Thus, patients used CPAP much more if they had an initial significant clinical handicap and if they were aware of the beneficial effects of CPAP. Under these conditions, patients tended to use the apparatus for the optimal length of time, regardless of the side effects linked to the treatment. This ensured efficacy and the maintenance of good compliance. This study confirms the importance of supervision of the time counter, as well as regular encouragement of patients to use the treatment as long as possible each night, in order to extract a maximum benefit from treatment by nasal CPAP.

[Vitelline tumor: a rare tumor of the mediastinum]. / La tumeur vitelline: une tumeur rare du médiastin.

A young patient presented with a rare tumour of the mediastinum. The first sign was liquid effusion in the right hemithorax. A major rise in alpha-foeto-protein level to 34,000 ng/ml rapidly led to diagnosis. Histological confirmation was obtained on a surgical biopsy specimen of the lung. Management included primary polychemotherapy followed by complete surgical exeresis. A second post-operative cycle of chemotherapy was given but only led to temporary normalization of alpha-foeto-protein levels and intensive chemotherapy followed by autograft to peripheral stem cells was performed. The vitellin tumour is a primary germ cell tumour usually observed in young men. It originates in extraembryonary primary mesenchymal tissue. Like other germ cell tumours, the diagnosis should be entertained for all tumours of the anterior mediastinum. Tumour markers useful for diagnosis and prognosis are beta-HCG, AFP and CEA. Levels above 500 ng/ml confirm vitellin tumour. Chemotherapy should be instituted rapidly using a BEP protocol (bleomycine, etoposide, cisplatinium) or a PVB protocol (cisplatinium, vinblastin, bleomycine). Using these new drugs and current therapeutic protocols, the rate of complete remission has risen from 13% to 40%.

[Sacral tuberculous osteitis]. / Ostéite tuberculeuse sacrée.

The authors report a case of a 5 year old child suffering from isolated sacral tuberculosis which presented as sudden and total loss of function of the left leg. The rarity of this bony site, and the recurrence at nine months of a presacral abscess after 9 months of anti-tuberculous treatment in an HIV negative child, vaccinated with BCG, justify the presentation of this observation.

Usher syndrome type I associated with bronchiectasis and immotile nasal cilia in two brothers.

Usher syndrome type I is an autosomal recessive disease characterised by congenital sensorineural deafness, involvement of the vestibular system, and progressive visual loss owing to retinitis pigmentosa. Here we report the association of this disease with bronchiectasis, chronic sinusitis, and reduced nasal mucociliary clearance in two sibs and we suggest Usher syndrome type I could be a primary ciliary disorder.

Atrial arrhythmia as a complication of nasal CPAP.

We report a case of supraventricular rhythm disorder occurring as a complication of nasal continuous positive airway pressure (CPAP) in sleep apnea syndrome treatment. The investigations showed a preexisting atrial vulnerability revealed by positive pressure ventilation, hemodynamic changes with decreased cardiac output, and a rise in the right atrial pressure. Such complications may develop further studies to evaluate cardiovascular repercussions of nasal CPAP.