Objective To investigate the association of excessive daytime sleepiness(EDS) and the quality of life in patients with moderate to severe obstructive sleep apnea and hypoventilation syndrome (OSAHS).Methods 123 patients with moderate to severe OSAHS were diagnosed by polysomnography(PSG),and 52 cases ruled out by PSG were controls.Patients and controls were completed the Calgary sleep apnea quality of life index (SAQLI) and the Epworth sleepiness score(ESS).Results There were statistical significance of body mass index (BMI),respiratory disturbance index(RDI),the lowest oxygen saturatioo( LSpO2 ),the longest apnea time( Tmax),the percentage of recording time with oxygen saturation of 90％ or less(T90) between the patients and the controls.Daily Functioning,Social Interactions,Emotional Functioning,Symptoms,and total scores of SAQLI in patients with moderate to severe OSAHS were(3.88 ± 1.05),(4.48 ± 1.39),(4.76 ± 1.59),(2.98 ± 1.10),(3.94 ± 1.22),which were less than that of controls respectively.The ESS scoree of patients with moderate to severe OSAHS were higher than that of controls[ ( 13.98 ± 4.05 ) vs (5.78 ± 2.45) ],( P ＜ 0.01 ).ESS were related to the daily Functioning,social interactions,emotional functioning,symptoms,and total scores of SAQLI in patients with moderate to severe OSAHS,and the coecient of correlation were - 0.52,- 0.48,- 0.40,- 0.48 and - 0.44,respectively,and all p value were less than 0.01.Conclusion Patients with moderate to severe OSAHS have the EDS,which associate with the impairment of quality of life.The higher ESS may indicate the decrease of quality of life in patients with moderate to severe OSAHS.

Pseudoxanthoma elasticum(PXE) is a rare, genetic, metabolic disease characterized by ectopic calcification of connective tissue that primarily affects the skin, retina and cardiovascular system, which characteristic histopathology is calcification and fragmentation of elastic fibers in dermis.PXE is mainly caused by ABCC6 gene mutation, which is one of the important regulators of the serum inorganic pyrophosphate (PPi) homoeostasis, a main inhibitor of ectopic calcification and the deficiency of PPi can lead to ectopic calcification. The clinical features are highly heterogeneous.Typical skin lesions of PXE are yellowish flat papules and plaques, and the symptoms of skin relaxation and shrinkage can be manifested in the later stage.Retina, cardiovascular and other complications seriously affect the health and quality of life of patients. The current therapy of PXE include symptom improvement, systemic anti-ectopic calcification medicine, gene therapy and so on.We review the pathogenesis, clinical manifestations, diagnosis and treatment of PXE.