Search

Sea-blue histiocytosis is one of the six types of Niemann-Pick disease. It is characterized by childhood onset of hepatosplenomegaly, lack of neurological involvement and diminished sphingomyelinase activity. Pulmonary system is rarely involved sea-blue histiocytosis. In this paper, we present a 39-years-old male who had previously diagnosed as sea-blue histiocytosis at the age of 15. He was admitted to our clinic due to productive cough, hemoptysis, fever and weight loss. His symptoms did not resolve with the antibiotic treatment and further investigations revealed pulmonary involvement of sea-blue histiocytosis. After diagnostic bronchoalveolar lavage, his symptoms were improved, interestingly. This rare entity was discussed with literature survey.

Introduction: Obstructive sleep apnea syndrome is characterized by episodic narrowing of the upper respiratory ways accompanied by an oxygen desaturation during sleep. REM-related obstructive sleep apnea syndrome, comprises a subgroup of sleep disordered breathing with a 10-36% frequency. The aim of the present study is to analyze the frequency, the antropometric and polygraphic features of REM-related obstructive sleep apnea syndrome., Materials and Methods: A total of 427 cases [332 males (77.4%) and 95 females (22.6%)] with definitive obstructive sleep apnea syndrome diagnosis with polysomnography records were included into the study., Results: Of all the cases, 108 (25.3%) were REM-related sleep respiratory disorder. There was no difference between the two groups with regard to age and sex. However, the obstructive sleep apnea syndrome group non-related to REM demonstrated a more severe obstructive sleep apnea syndrome and a longer neck circumference. Superficial sleep was longer in the REM non-related group, while deep refreshing sleep (Non-REM 3) was longer in the REM-related group. The subgroup analysis of the REM-related group with regard to sex revealed a higher body mass index in the female subgroup., Conclusion: REM-related sleep respiratory disorder is more often in mild-to-moderate obstructive sleep apnea syndrome cases, the male/female ratio of REM-related sleep respiratory disorder is not different from the obstructive sleep apnea syndrome prevalence in the community.

A 78-years-old man presented with weight loss, dyspnea, cough and hemoptysis. He was an ex-smoker with a smoking history of 30 pack-years. Imaging studies revealed an endoluminal carinal mass and a tumoral mass arising from the posterior wall of the trachea was observed bronchoscopically. Endoscopic argon plasma coagulation was performed to take biopsies and to regain the airway passage. Recanalisation of the right and left main bronchi and the trachea was achieved together with resection of more than 90% of the tumoural mass. Microscopically, the tumor was composed of fascicular pattern of spindle cells with atypical mitotic figures and nuclear pleomorphism, positively reactive to epithelial membrane antigen supporting a diagnosis of tumour with epithelial differentiation. These findings supported the diagnosis of a spindle cell sarcomatoid carcinoma of trachea. Spindle cell carcinoma cases are reported in many sites, such as head and neck region, larynx, digestive tract, breast, kidney, genital tract, skin and lung, but to our knowledge, not in trachea. Primary malignant tracheal tumours consist mainly of squamous cell carcinoma and adenoid cystic carcinoma and generally have an aggressive course with poor prognosis. We report the first presentation of a spindle cell sarcomatoid carcinoma of trachea together with the clinical course of the patient.

In our study we aimed to examine the sleep structure, oxygenation and breathing pattern in interstitial lung disease (ILD) patients. We also aimed to determine whether relevance between the advanced disease and the sleep disorders exists and whether polysomnography is necessary in those patients. A total of 37 patients were examined in the study and whole night standard polysomnography was performed to all. Polysomnography results revealed that, total sleep time, time spent in NREM sleep stage III and IV, and in REM sleep were decreased. The patients had poor sleep efficiency and they spent more time as wake after sleep onset (WASO). Severe oxygen desaturations were detected during sleep and statistically significant positive correlations were found between mean awake O2 saturation and mean and lowest sleep O2 saturations. Clinical, Radiological and Physiological (CRP) scoring system was used to assess the disease stage, whether advanced or not, and statistically significant negative correlations were found between CRP score and awake and sleep O2 saturations. Obstructive sleep apnea syndrome (OSAS) was diagnosed in 24 (64.9%) patients. In those patients it was found that not the apneas but the hypopneas predominate. No difference was found among body mass indices (BMI) between the patients with and without OSAS. As a result it was concluded that a sleep study should be considered as part of the overall assessment in managing patients with ILD, and is especially indicated if there is incipient pulmonary hypertension, cor pulmonale and nocturnal arrhythmia despite normal awake blood gas tensions and symptoms as snoring and excessive day time sleepiness.