Primary ciliay dyskinesia (PCD)is a kind of monogenic disorder.Young patients often present recurrent respiratory tract infection, even progress to bronchiectasis, and some children are combined with situs inversus.The pathogenesis is closely related to the abnormality of cilia structure and/or function.So far, over 40 genes that are associated with PCD has been confirmed in humans.About 70% of the cases are correlated with these genes.Although the relationship between genotype and phenotype is not fully clear, some genetic defects and clinical manifestations might have certain relevance.The severity of the clinical phenotype may also be related to a specific genotype.With the maturity of gene sequencing technology and the reduction of the cost, gene sequencing can also be applied in clinical practices to make up for the shortcomings of traditional diagnostic methods.

Wheezing in children is a common problem.Approximately 30 percent of infants will have at least one wheezing episode.Although asthma is the condition most commonly associated with wheezing,the high prevalence of wheezing as a common presenting complaint requires consideration of a broad and age-dependent differential diseases.A high index of suspicion of cardiovascular anatomic anomalies should be maintained in infants and children with recurrent wheezing and minimal improvement despite reasonable treatment.Compression of the pediatric airway is a relatively common and often unrecognized complication of a variety of cardiovascular diseases.The most common congenital anomalies associated with airway compression are the vascular rings.Vascular ring abnormalities are aortic arches abnormalities,aberrant left pulmonary artery (pulmonary sling) or aberrant i nnominate artery (innominate artery syndrome).Aortic arches abnormalities include double aortic arch,right/left sided aortic arch with ligamentum arteriosum and aberrant left subclavian artery or cervical aortic arch.Other rare reasons could cause the compression of airway are absent pulmonary valve,aneurysm,left atrial enlargement or massive cardiomegaly.Imaging techniques are usually required for diagnosis.First-tier evaluation of the child with recurrent wheeze should include a chest radiograph.Echocardiography,multi detector computed tomography (MDCT) or magnetic resonance imaging (MRI) are performed as the diagnostic pathway.Bronchoscopy is currently the techniques to assess the degree of bronchial malacia and narrowing in pre-operation.Treatment of these abnormalities usually is surgical.

Objective To investigate the effects of serotonin(5-HT) on the ionic channels currents and membrane potentials of single guinea pig proximate colon myocytes. Methods Cell images before and after contraction were captured and analyzed with an imag e analysis software. Channels currents, action potential (AP) and resting potent ial (RP) were recorded with an EPC-9 amplifier. Results The direct actions of 10 ?mol/L 5-HT to myocytes resulted in followin g effects: Firstly, the exogenous stimulated peak values of AP reduced to 60%?7 .3%(n=7,P

Bronchiectasis is a kind of chronic lung disease which threatens the health of children in China.It can affect the lung function,life quality and development of the patients.There are varied causes of bronchiectasis,and some of the causes have the special intervention.So the etiology diagnosis of bronchiectasis is important.The aims of the treatment of bronchiectasis is removing the causes,releasing the symptoms,preventing the acute exacerbation,sustaining the lung function and life quality.It needs a long tern combine therapy.

Since Middle East respiratory syndrome( MERS)was first reported in 2012,the severe clinical symptoms and high mortality have been concerned. The epidemiology,possible pathogenesis,clinical manifestations(es-pecially in children),treatment and counter measures of MERS were reviewed in this paper.

AIMTo investigate the effects of ovarian hormone on th e expression of 5-HT 3 receptors in the rats colon. METHODS24 adult female SD rats were randomly divided into three groups:sham operation(Sham ) group;ovariectomy(OVX) group and ovariectomy with estrogen and progesterone(OV X+E 2+P) group. The 1 hour fecal pellets and the time of glass pellets output w ere observed 4 weeks after operation;and the expression of 5-HT 3 receptors mR NA in the colon tissues was studied by RT-PCR. RESULTSIn contra st with the Sham group and OVX+E 2+P group,OVX group showed increase in the fec al pellets output and decrease in the time of glass pellets output(P

Anaphylaxis is the most severe clinical presentation of acute systemic allergic reactions.The occurrence of anaphylaxis has increased in recent years, and subsequently, there is a need to continue disseminating knowledge on the diagnosis and management, so every healthcare professional is prepared to deal with such emergencies.This updated position document keep guidance aligned with the current state of the art of knowledge in anaphylaxis management including definition, diagnostic criteria, severity grading, elicitors and cofactors, acute treatment and long-term management of anaphylaxis.Intramuscular Adrenaline continues to be the first-line treatment for anaphylaxis.After an anaphylaxis occurrence, patients should be referred to a specialist to assess the potential cause and to be educated on prevention of recurrences and self-management.

Objective To investigate the protective mechanism of Salvianolic acid B (Sal B) on experimental in-vivo myocardial ischemia-reperfusion injury (MIRI)in rats. Methods Rats model of myocardial ischemia-reperfusion injury was induced by ischemia for 60 min and then reperfusion for 60 min. After treatment,endothelin (ET) levels in plasma and the homogenate of myocardial tissue,and serum levels of nitric oxide (NO) and NO synthase were measured. Meanwhile,the pathological changes of myocardial tissue were also observed. Results Compared with the model group,middle-dose and high-dose Sal B could depress the ET levels in the plasma and the myocardial tissue,and increase the content of serum NO and NOS (P

Aim To investigate the effects of emodin on the contraction of gallbladder smooth muscle(GBSM)and the L-type calcium current in GBSM cells.Methods Gallbladder muscle strips were obtained from adult guinea pigs and the resting tension was recorded.Gallbladder smooth muscle cells were isolated by enzymatic digestion,and calcium current was recorded by the whole-cell patch clamp method.Results Emodin-induced contraction of GBSM was significantly attenuated by pretreatment with nifedipine.Emodin increased the L-type calcium current in a dose-dependent manner.When 10 ?mol?L-1 emodin was applied to GBSM cells,the amplitude of L-type calcium current at +10 mV was enhanced by(45.2?2.26)%.In the presence of PKC inhibitor,staurosporine,emodin did not significantly affect the calcium current.Conclusion Emodin enhances L-type calcium current via PKC-dependent pathway and promotes gallbladder contraction.

AIM To study the effects of progesterone on contractile activity of smooth muscle stripes and the large conduction Ca 2+ activated K + currents (BK Ca ) of single colonic myocytes in female guinea pig proximal colons METHODS Stripes and single cells were separated acutely from guinea pig proximal colon Contractions of stripes were measured by isonic transducer, and Ca 2+ activated K + currents were recorded with an Axopatch 1D amplifier under conventional whole cell patterns RESULTS 64 8 ?mol?L -1 progesterone produced longitudinal stripes significantly re laxation(0 1792 g?0 0873 g, n =6, P