Evaluating RANKL and OPG levels in patients with Duchenne muscular dystrophy.

RANKL-OPG should be explored in DMD patients to potentially provide targeted therapy. We quantified RANKL and OPG levels in DMD patients compared with controls. RANKL, OPG, and RANKL:OPG significantly declined with age in DMD patients suggesting some bone turnover markers are difficult to assess or use as therapeutic indicators. INTRODUCTION: Osteoporosis in Duchenne muscular dystrophy (DMD) is multi-factorial in nature with high prevalence of fractures. RANKL-OPG should be explored to potentially provide targeted therapy for these patients. We quantified RANKL, OPG, and RANKL:OPG levels in DMD patients compared with controls and analyzed the influence of age, glucocorticoid use, ambulatory status, bone density, and fracture history. METHODS: DMD patients were enrolled at CHLA. Controls were recruited from general pediatric clinic and in collaboration with samples from a previously completed study. Free soluble RANKL and OPG levels were quantified using a sandwich ELISA. RESULTS: Fifty DMD patients and 50 controls were enrolled. DMD patients had a significant decline in RANKL, OPG, and RANKL:OPG with age (p = < 0.0001, p = 0.026, and p = 0.002, respectively) while healthy controls showed no significant change. RANKL trended lower in patients on glucocorticoids (p = 0.05), attributed to the significantly older age in the treatment group. RANKL and RANKL:OPG levels were significantly lower in the non-ambulatory group compared with the ambulatory group (p = 0.010 and 0.036 respectively), again likely due to their older age. There was no correlation of RANKL, OPG, or RANKL:OPG with DXA Z-score or presence of vertebral fractures. CONCLUSION: There was significant decline in RANKL, OPG, and RANKL:OPG with age in DMD patients compared with controls, potentially due to disease severity or worsening osteoblastic function. This suggests some bone turnover markers may be difficult to assess or use as therapeutic indicators in DMD patients. Larger studies are needed to evaluate the role of RANKL-OPG in DMD patients to provide better targeted therapy.

[Acute poisoning by chemical warfare agent: sulfur mustard]. / Intoxication aiguë par arme chimique vésicante: l'ypérite.

OBJECTIVE: To review story, mechanism of action, clinical and therapeutic bases of a sulfur mustard poisoning, by accidental, terrorism or war exposure. DATA SOURCES: References were obtained from computerised bibliographic research (Medline), from personnel data (academic memoir, documents under approbation of the National Defense Office) and from the Library of Military Medical Service. DATA SYNTHESIS: Sulfur mustard is a chemical warfare agent with peace time results: leak, accidental handling, acts of terrorism. Sulfur mustard is a vesicant agent, an organochlorine agent, who alkylate DNA. Under liquid or gas form its main target are skin and lungs. Clinical effects are like burns with loss of immunity, with respiratory failure, ophthalmic, gastrointestinal and haematological signs. The last studies have improved knowledge about the mechanism of action, detection, protection and treatment. Methods for determination of sulfur mustard are based on gas chromatographic method and mass spectrometry. During sulfur mustard contamination the first priorities of treatment are to remove victims from the contaminated place and to initiate decontamination. Emergency workers and materials must take protection to avoid secondary contamination of emergency unit. With treatment of vital functions and respiratory failure, the new ways of treatment are about N-acetyl cysteine for lung injury, poly (ADP-ribose) polymerase inhibitors, calmodulin antagonists and Ca(++) chelators. Interactions between sulfur mustard and anaesthetic agents are not well known and are based on clinical observations. CONCLUSION: Emergency care unit can be confronted with sulfur mustard during accidental contamination or acts of terrorism. First and most efficacy priorities of treatment are to remove and to decontaminate victims. New means of detection and treatment are studied since several years but are not still appropriate to human victims or mass treatment.

[Does a psychosocial group intervention program alter the quality of life of cancer patients?]. / Verändert ein psychosoziales Gruppeninterventionsprogramm die Lebensqualität von Krebspatienten?

BACKGROUND: Structured, multicentre psychosocial support programmes are rare in Switzerland. The Swiss Cancer League has therefore designed a programme of this kind for cancer patients, and the effect on quality of life (QL) is investigated in the present study. METHODS: The programme consisted of 10 weekly 2-hour sessions and was carried out, on the basis of a manual, in 10 cities of German-speaking Switzerland. QL was assessed by standardised tools (HAD, EORTC, QLQ-C30, SELT-M, LASA) before, immediately after, and 9 months post baseline. Patients' personal impressions of the programme were gathered by telephone. RESULTS: 134 patients (25 males, 109 females), mean age 51.9 years, took part in the programme. 50% had breast cancer and the other half a variety of tumours. 25 patients attended only the educative part of the programme, while of the remaining 109, 41% attended all 10 sessions, 50% 7-9 and 9% 4-6. 80-90% had a favourable impression of the individual sessions and their content. Significant QL changes in the desired direction were observed in regard to emotional functioning (Wilcoxon p = 0.011), anxiety (p = 0.0005), depression (p < 0.0001), general life orientation (p = 0.006) and spiritual QL (p = 0.012). More optimistic patients showed a more positive change in physical well-being (Kurskall-Wallis p = 0.0005) and QL in general (p = 0.0006). CONCLUSION: A psychosocial support programme is well received by cancer patients after adjuvant treatment and leads to favourable QL changes, although independent time effects cannot be ruled out. Similar programme should be considered more often in standard oncological treatment regimens.

[A cause of intubation failure: a subglottic bridge]. / Une cause d'intubation impossible: la bride sous-glottique.

We report the case of a patient with a history of facio-thoracic burns, the treatment of which included prolonged intubation, whose trachea could not be intubated because of a subglottic obstacle. The ventilation was easily controlled with a laryngeal mask. At the end of surgery for postburn cheloids, laryngoscopy through the laryngeal mask showed a transversal subglottic laryngeal band, a probable sequela of the previous prolonged intubation. The band was resected one week later. The conventional indicators for difficult intubation cannot detect the laryngotracheal obstacles to tracheal tube insertion.

[Experimental study of extra-corporeal circulation with membrane oxygenators: effects on the lung structures of dogs and monkeys (author's transl)]. / Etude expérimentale de la circulation extra-corporelle avec oxygénateurs à membrane. Effets sur les structures pulmonaires du chien et du singe.

Six dogs and 5 monkeys underwent extra-corporeal circulations with membrane oxygenators for variable time periods (from 4 to 57 hours). The development and evolution of the lesions, which were similar in the two animal species, were controlled by electron microscopic examination of lung samples. From the 2nd h, abnormalities of the capillary endothelium were observed; but a 6 h, all the inner lung structures were reached and the lesions grew worse with time. Thus, the intrinsic toxicity of membrane oxygenators on lung structures emphasizes that this method should be used with utmost precaution in man.

Experimental modifications induced by membrane oxygenators on lung ultrastructure in dogs and baboons.

Extracorporeal circulation with membrane oxygenator (E.C.M.O.) was performed in 6 dogs and 5 baboons during 4 to 57 hours. Examination of pulmonary samples showed progressive lesions in the two species: at the second hour of E.C., alteration of capillary endothelium, at the sixth, extensive lesions of all the lung structures, worsening later on. We discussed whether these lesions are related to the E.C.M.O., their reversibility and the questionable benefit of E.C.M.O. for management of respiratory distress syndrome.

[Experimental study of the effect of membrane oxygenators on the pulmonary structure of the dog and monkey (author's transl)]. / Etude expérimentale de l'effet des oxygénateurs à membrane sur les structures pulmonaires du chien et du singe.

Six dogs and 5 monkeys were subject to extracorporeal circulations with membrane oxygenators for various time periods (from 4 to 57 hours). The pulmonary samples, examined with electron microscopy, enabled us to control the appearance of lesions (similar for the two animal species) and to follow their evolution. From the second hour, abnormalities of the capillary endothelium were observed; but at the 6th hour, the lesions had already deeply affected the whole pulmonary structure and became more and more serious. This intrinsic toxicity of membrane oxygenators on the pulmonary structure should incite extreme caution in the use of this method in man.