Fatal course of a benign mediastinal lipoblastoma in a 20-year-old woman.

Lipoblastoma is a rare benign, but highly proliferative tumor most commonly seen in early childhood. Recurrence rates are high when complete resection is unfeasible and systemic therapy is necessary. We report the case of a large, aggressive thoracic and mediastinal lipoblastoma in a 20-year-old woman, which was surgically not resectable. The tumor has been characterized extensively including molecular pathology, molecular karyotyping, conventional chromosomal analysis and in vitro-chemosensitivity testing in search for alternative therapies. Nevertheless, this did not reveal treatable targets and systemic therapies, which were based on chemosensitivity testing proved ineffective. Despite all treatment attempts, the disease showed a progressive fatal course.

Fatal course of clofazimine-induced pulmonary crystal deposition in a patient with Melkersson-Rosenthal syndrome.

A wide variety of drugs and substances have the potential to damage the respiratory system by different mechanisms. Clofazimine is an anti-leprosy drug that is normally only prescribed for a few years. It has a very long half-life, and crystalline deposition of the drug in various tissues has been documented. But up to now, no fatalities due to pulmonary damage have been described. We report the case of a patient who took clofazimine for almost 27 years as off-label treatment for Melkersson-Rosenthal syndrome. He suffered from progressive dyspnea, productive cough, and occasional hemoptysis. X-ray and CT of the thoracic organs revealed extensive multilocular, compact, tumor-like infiltrates with central necrosis in both lungs. Pulmonary function tests showed restrictive impairment and manifest hypoxemia. Histology of lung biopsies revealed intense interstitial accumulation of histiocytes and marked deposition of crystalline foreign material. The patient died from progressive respiratory failure. Autopsy revealed crystalline deposition and a histiocytic reaction in many other parenchymal organs. Conclusion: Pulmonary parenchymal deposition of drug crystals is a rare mechanism of drug-induced pulmonary diseases. Long-standing, off-label use of clofazimine may cause severe destruction of the lungs and can be fatal.

Prevention of leakage due to mouth opening through applying an oral shield device (Sominpax™) during nasal CPAP therapy of patients with obstructive sleep apnea.

BACKGROUND/OBJECTIVE: The first line treatment for obstructive sleep apnea (OSA) is nasal continuous positive airway pressure (nCPAP), for which a variety of masks are available. While nasal masks (NM) are the first choice; oronasal masks (ONM) are also frequently used to prevent mouth dryness resulting from mouth opening. Our cross-sectional, prospective, randomized, un-blinded study addressed the efficacy of wearing an oral shield in addition to NM in preventing mouth leakage METHODS: Patients with OSA and established therapy using NM and complaining about mouth dryness (n = 29) underwent three polysomnographies (PSGs) using NM, ONM or a nose mask in combination with an oral shield (NMS). Mask leakage was continuously documented and objective sleep quality was assessed. RESULTS: There were significant differences in the apnea-hypopnea-index (AHI) between ONM (8.5/h; SD 6,7) and NM/nasal mask combined with oral shield device (NMS) (2.6/h; SD 2,3; 2.7/h; SD 2,6) (p < 0,05) as well as in leakage [ONM (39.7 l/min SD 12,4); NM (34.6 l/min SD 9,4); NMS (33.1 l/min SD 9,6)] (p = 0.011). Furthermore, analysis of sleep quality (NREM3) favored NM and NMS over ONM (p = 0.02). There were no significant differences between NM and NMS in any objective outcome. CONCLUSIONS: Our data consistently confirmed the NM as the first choice for continuous positive airway pressure (CPAP) therapy of OSA. Notably, we demonstrated a high potential of the oral shield for patients with mouth opening to achieve additional comfort and thereby possibly compliance, without affecting nCPAP therapy effectiveness.

Fecal calprotectin: a marker for clinical differentiation of microscopic colitis and irritable bowel syndrome.

BACKGROUND: The aim of this study is to compare two methods for measuring fecal calprotectin (FC) concentration and to evaluate the possibility of differentiation between microscopic colitis (MC) and irritable bowel syndrome (IBS). METHODS: Twenty-three patients with MC (six patients with active disease and 17 patients retested in remission) and 20 patients with IBS were prospectively included in this study. Active disease state of MC was determined by clinical symptoms of >3 bowel movements per day and histological correlate. All patients underwent ileocolonoscopy, including segmental biopsy samples for histology. FC levels in stool samples were analyzed using a rapid test system (Quantum Blue(®)) and an enzyme-linked immunosorbent assay (ELISA). RESULTS: FC levels were significantly higher in patients with active MC (median 48 µg/g [23-106]) compared to patients with IBS (median 2 µg/g [1-111.83]), P=0.0001 using an ELISA. FC level of patients with MC in remission was 22 µg/g (1-106.4), which is similar to those identified in patients with IBS. The difference of FC levels between active MC and IBS was not detected by the FC rapid test (P=0.635). DISCUSSION: FC levels might serve as parameter for differentiation between patients with active MC and IBS. Since there is no surrogate marker available at present for MC, FC appears to be a candidate for differentiating MC from IBS. CONCLUSION: High FC levels, which were analyzed by ELISA, are a potential marker for patients with active MC compared to those with IBS. The FC rapid test was less suitable for this purpose.