Recurrent hemoptysis in pediatric bronchial Dieulafoy’s disease with inferior phrenic artery supply:A case report

BACKGROUND Bronchial Dieulafoy’s disease(BDD)is characterized by the erosion of an anomalous artery in the submucosa of the bronchus.The etiology of pediatric BDD is mainly congenital dysplasia of bronchus and pulmonary arteries,which is different from chronic inflammatory injury of the airway in adult patients.The internal thoracic artery,subclavian artery,and intercostal artery are known to be involved in the blood supply to the BDD lesion in children.CASE SUMMARY We report a case of BDD in a 4-year-old boy with recurrent hemoptysis for one year.Selective angiography showed a dilated right bronchial artery,and anastomosis of its branches with the right lower pulmonary vascular network.Bronchoscopy showed nodular protrusion of the bronchial mucosa with a local scar.Selective embolization of the bronchial artery was performed to stop bleeding.One month after the first intervention,the symptoms of hemoptysis recurred.A computed tomography angiogram(CTA)showed another tortuous and dilated feeding artery in the right lower lung,which was an abnormal ascending branch of the inferior phrenic artery(IPA).The results of angiography were consistent with the CTA findings.The IPA was found to be another main supplying artery,which was not considered during the first intervention.Finally,the IPA was also treated by microsphere embolization combined with coil interventional closure.During the one-year follow-up,the patient never experienced hemoptysis.CONCLUSION The supplying arteries of the bleeding lesion in children with BDD may originate from multiple different aortopulmonary collateral arteries,and the IPA should be considered to reduce missed diagnosis.CTA is a noninvasive radiological examination for the screening of suspected vessels,which shows a high coincidence with angiography,and can serve as the first choice for the diagnosis of BDD.

Role of protein kinase C α and cyclin D1 in the proliferation of airway smooth muscle in asthmatic rats

Background Airway smooth muscle （ASM） is suspected to be a determining factor in the structural change of asthma. However, the role of protein kinase C α （PKCα） and cyclin D1 involved in the dysfunction of ASM leading to asthmatic symptoms is not clear. In this study, the central role of PKCα and cyclin D1 in ASM proliferation in asthmatic rats was explored. Methods Thirty-six pathogen-free male Brown Norway （BN） rats were randomly divided into 2 groups： control groups （group N1, N2 and N3） and asthmatic groups （group A1, A2, and A3）. Groups A1, A2 and A3 were challenged with ovalbumin （OA） for 2 weeks, 4 weeks and 8 weeks respectively. Control animals were exposed to an aerosolized sterile phosphate buffered saline （PBS）. The ASM mass and nucleus numbers were studied to estimate the degree of airway remodeling by the hematoxylin-eosin staining method. PKCα and cyclin D1 expression in the ASM cells was detected by reverse transcription polymerase chain reaction （RT-PCR） and immunohistochemistry. The relation between PKCα and cyclin D1 was assessed by linear regression analysis. PKC agonist phorbol 12-myristate 13-acetate （PMA）, PKC inhibitor Ro31-8220 and an antisense oligonucleotide against cyclin D1 （ASOND） were used to treat ASM cells （ASMCs） obtained from the 2 weeks asthmatic rats. The cyclin D1 protein expression level was detected by Western blotting.Results Compared with the control group, the PKCα and cyclin D1 mRNA levels were increased in the asthmatic group. Similar to RT-PCR results, immunohistochemistry analysis for PKCα and cyclin D1 expression revealed an increased production in ASMCs after allergen treatment for 2, 4 and 8 weeks compared with the respective control groups. No difference in expression of PKCα and cyclin D1 in ASM were found in the 2, 4 or 8 weeks asthmatic rats. There were significant positive correlations between PKCα and cyclin D1 expression, both transcriptionally （r=0.944, P 〈0.01） and translationally （r=0.826, P 〈0.01）, in ASM. The content of cyclin D1 in asthmatic ASMCs increased after being stimulated by PMA, and decreased when induced by Ro31-8220. ASOND targeting for cyclin D1 lowered the expression of cyclin D1 induced by PMA.Conclusions Increased expression of PKCα and cyclin D1 in ASM along with smooth muscle structure changes might implicate PKCα and cyclin D1 participation in the proliferation of ASM and contribute to the pathogenesis of asthma after repeated allergen exposure in rats. The results suggested that cyclin D1 might be downstream of PKC signal transduction pathway.