OBJECTIVE：To evaluate the price and affordability of rare disease drugs in China and provide the suggestions for the improvement of rare disease drug affordability in order to provide reference for the relevant decision-making of government departments. METHODS ：According to the List of the First Batch of Rare Diseasesand Diagnosis and Treatment Guideline for Rare Diseases（2019 edition），rare disease drugs were selected. The median price ratio （MPR）was used to evaluate the drug price level ， and the ratio of the annual drug costs to the annual disposable income of urban or rural residents was used to evaluate the affordability，and the impact of price management policieson drug prices and affordability. RESULTS and CONCLUSIONS ：A total of 71 kinds of rare disease drugs were included ，and the median MPR of them was 0.83；among them ，the median MPR of the original drugs was 1.13，and the median MPR of the generic drugs was 0.37. The annual cost of 71 rare disease drugs was 0.001-178.43 times the average annual income of urban residents ，and 0.003-456.57 times the average annual income of rural residents. There were 21 and 28 drugs whose annual cost exceeded the annual income of urban and rural residents.After the implementation of national medical insurance negotiation or volume based procurement of 14 drugs，the median MPR of these drugs decreased from 1.71 to 0.46. For urban residents ，the number of unaffordable drugs had been reduced from 8 to 0；for rural residents，it had been reduced from 10 to 5. In summary ，the price level of orphan drugs in China was slightly lower than international reference price ，but the price level of original drugs was higher than the international reference price ，and the cost of some drugs far exceeded the residents ’ability to pay. It is suggested that the government should strengthen the price management of original drugs ，promote the substitution of high-quality generic drugs for original drugs ，and improve the payment system for high-value rare disease drugs.

Dysregulated crosstalk between different signaling pathways contributes to tumor development, including resistance to cancer therapy. In the present study, we found that the mitogen-activated extracellular signal-regulated kinase (MEK) inhibitor trametinib failed to suppress the proliferation of PANC-1 and MGC803 cells by activating the Janus kinase 2 (JAK2)/signal transducer and activator of transcription 3 (STAT3) signaling pathway, while the JAK2 inhibitor fedratinib failed to inhibit the growth of the PANC-1 cells upon stimulation of extracellular signal-regulated kinase (ERK) signaling. In particular, the most prominent enhancement of the anti-proliferative effect resulted from the concurrent blockage of the JAK2/STAT3 and ERK signaling pathways. Furthermore, the combination of the two inhibitors resulted in a reduced tumor burden in mice. Our evidence suggests novel crosstalk between JAK2/STAT3 and ERK signaling in gastric cancer (GC) and pancreatic ductal adenocarcinoma (PDAC) cells and provides a therapeutic strategy to overcome potential resistance in gastrointestinal cancer.

Objective: To report the first case of lymphomatoid gastropathy in China, and to demonstrate the clinical characteristics, diagnostic approach, treatment and prognosis in this kind of patients. Methods: One patient was diagnosed as lymphomatoid gastropathy at Peking Union Medical College Hospital, and her clinical characteristics, lab data, treatment and follow-up outcomes were reviewed. Results: A case of a 51-year-old female was presented, who underwent esophagogastroduodenoscopy (EGD) due to slight epigastric discomfort. EGD revealed multiple ulcers and erosions. Biopsies showed atypical lymphocytes infiltration with CD3(+), CD56(+), CD20(-), CD8(-), TIA(+), Granzyme B(-) and Ki-67 (75%). Epstein-Barr virus-encoded RNA in situ hybridization was negative. Four months later, repeated EGD examination showed regression of the lesions without specific treatment. Conclusion Lymphomatoid gastropathy was a unique disease entity mimicking NK/T-cell lymphomas in pathology, with the quite different profile of treatment and prognosis. It’s important to consider this issue during the differential diagnosis to avoid any excessive treatment.

Objective To assess the efficacy and clinical resuhs of thoracic endovascular aortic repair (TEVAR) in Stanford type B aortic dissection,and to explore the therapentic strategy and prevention of complication.Methods Fifty-eight patients with Stanford type B aortic dissection underwent TEVAR were enrolled,and were followed up regularly.Results The technical feasibility and success with deployment proximal to the entry tear was 100.0％(58/58),requiring partial coverage of the left subclavian artery in 20 patients and total coverage in 4 patients,and 2 patients used chimney stents.Hospital mortality was 5.2％ (3/58).Type Ⅰ endoleak was found in 11 patients (19.0％,11/58).In 55 survival patients,48 patients occurred thrombosis in pseudocoele and 2 patients' endoleak disappeared.Seven patients' endoleak appeared blood flow.Forty-eight patients(87.3 ％,48/55) were followed up for 3-68 months.Two patients died,and 2 patients' endoleak disappeared,with total suberization in pseudocoele.Other 5 patients' endoleak relieved,with partial suberization in pseudocoele.One patient had new intimal tear at the distal end of the stent-graft and required a second stent implantation.Conclusion TEVAR is safe and effective for the treatment of Stanford type B aortic dissection.

Objective To investigate the treatment methods of tissue defects in nose and lower eyelid area.Methods Based on the impaired area,combined pre-expansion of forehead and facial region was adopted.The volume of soft tissue expander was determined.150-170 ml expander was implanted between frontalis muscle and periosteum of forehead,while at the facial region,and the volume of expander should be determined by the normal skin area as large as possible.The excision of impaired tissue was performed after sufficient expansion,and then skin flap or transposition skin flap were advanced at the facial region.At forehead,ortho-position skin flap based on supratrochlear vessels was designed for repairing the nose and palpebra inferior region.Three weeks later division of the ortho-position skin flap was carried out.With the flattened pedicle,the rest part of impaired area was replaced.Results Six cases were performed with this method.For all the cases,the survival rate of flap was 100％.Esthetic appearance and satisfying color-match were achieved,without appearance of lower eyelid ectropion.Conclusions Combined pre-expansion of forehead and facial region is an ideal method in treatment of the defects in nose and lower eyelid area.

OBJECTIVETo investigate the clinical application of expanded flap based on cutaneous branch of transverse cervical artery for reconstruction of cervical cicatricial contracture.

METHODSBased on the clinical anatomy of cutaneous branch of transverse cervical artery flap, we design the corresponding subclavicular area for expansion. The incision was usually located at the anterior axillary fold, 5-8 cm in length. The expander was implanted under the deep fascial layer, without injury of the vascular pedicle. Fixation sutures were put about 1 cm apart from the incision to prevent the expander from transposition and exposure. After expansion, the cervical cicatricial contracture was excised and released. According to the defect, the expanded flap based on the cutaneous branch of transverse cervical artery was designed, with the pedicle located at the posterior margin of sternocleidomastoid and 1. 8 cm above median point of clavicle. "S" shape incision was made at the location of vascular pedicle. Subcutaneous dissection was performed 1.5 cm in width along the incision on both sides. Then the flap was harvested under the deep fascial layer and rotated to cover defect without tension. It was not necessary to dissect the vascular pedicle further. The defect at donor site was closed directly.

RESULTS17 cases were treated with the island flap. The contracture of the cervical scar was corrected completely with aesthetic appearance.

CONCLUSIONSExpanded flap based on cutaneous branch of transverse cervical artery has reliable blood supply. It' s an ideal flap for the treatment of cervical cicatricial contracture.

Arteries ; Cicatrix ; surgery ; Clavicle ; Contracture ; surgery ; Dissection ; methods ; Fasciotomy ; Humans ; Neck ; blood supply ; Neck Muscles ; anatomy & histology ; Surgical Flaps ; blood supply

Objective To analyze the effect of EVAR in the treatment of octogenarian patients with abdomi-nal aortic aneurysm.Methods 16 octogenarian patients aged over 80 years who treated with EVAR were collected. Mean age was (87.0 ±6.2)years and included 11 cases in male,2 cases in emergency.All the cases were infrarenal abdominal aortic aneurysm and 11 cases (69%)were the complex aortic anatomy.According to ASA classification, 11 cases (69%)were class Ⅲ and 5 cases (31%)were class Ⅳ.All patients were followed up with CT for 1 month to 3 years.We evaluated technical success rate,perioperative complications and the application characteristics of EVAR in the treatment of octogenarian patients with abdominal aortic aneurysm were analyzed.Results All the patients were operated with EVAR,the success rate was 100%,12 Endurant endografts and 4 Talent endografts were used.Mean operation time was (92 ±25)min and mean hospital stay length was (17 ±9.5)days.1 patient died with heart failure in 3 days after operation.1 patient died due to the Ⅰ endoleak and 4 patients died due to other fators. Conclusion For the octogenarian abdominal aortic aneurysm patients,EVAR is the preferred treatment with accepta-ble procedural success and perioperative morbidity.Those patients have characteristics in the physiological function and anatomy,so we should take measures to prevent the complications.The medium and long -term results suggest that EVAR may be of limited in some patients who are aged over 80 years.

Objective To analyze the characteristic of auditory brainstem responses (ABR) evoked by chirp in normal hearing subjects .Methods Fifteen cases (30 ears) with normal hearing young people were recorded ABR by stimulation with two kinds of sounds :chirp and click .The data were compared .Results The response threshold of chirp-ABR were lower than that of click -ABR .The average difference was 8 .59 dB .At 90 dB nHL ,the wave V amplitude yield no significant difference between chirp -ABR and click-ABR .The wave V amplitude had a signifi-cant difference between chirp -ABR and click-ABR at 60 dB nHL .At 90 and 60 dB nHL ,the wave V amplitude of chirp-ABR had not a significant difference .The occurence rate was 40% for the wave I of chirp -ABR ,obvi-ously less than that of click -ABR .At 90 dB nHL ,the wave V latency of chirp -ABR was shorter than that of click-ABR .Conclusion The wave V response threshold of chirp -ABR is less than that of the click -ABR .The chirp-ABR is more advantageous than the click -ABR for assessing hearing threshold .

Objective To explore the repair method for wounds after excision of facial benign tumors.Methods Unilateral or bilateral deltopectoral skin flaps were expanded depending on the area of the facial benign tumor.Expander was implanted underneath deltopectoral flap region through an incision inferior to the clavicle.When expansion was completed,all or part of the benign tumor was excised before designing the flap according to the area of the skin defect.The area of the skin flap should be more than that of skin defect with 10％ to 15％.The pedicle wound could be sealed by rolling it around to form a tube or a hinge using the benign tumor and pedicle.The flap was delayed three weeks later and the pedicle was divided one week after flap delaying.Results All 20 cases got the satisfactory results with treatment of pedicled expanded deltopectoral skin flaps for repair of wounds after excision of facial benign tumor.Conclusions It is a better option to repair a large area wound after excision of facial benign tumor with an expanded deltopectoral skin flap.