118 Undefined left ventricular arrhythmogenic cardiomyopathy: the value of genotyping and histological characterization

Aims Etiology identification and risk stratification represent major issues for patients presenting with undefined left ventricular arrhythmogenic cardiomyopathy (ULVACM). To investigate the role of genotyping and histology for ULVACM classification, management, and risk assessment. Methods and results We retrospectively analysed a multicentre cohort of patients (screened n = 1037) with ULVACM defined by ventricular arrhythmia (VA) onset, nonischaemic late gadolinium enhancement (LGE) limited to the LV, and no manifest dilated cardiomyopathy (LVEF ≥ 40%). We selected patients undergoing both next generation sequencing (NGS) genotyping and endomyocardial biopsy (EMB) for etiology definition. When feasible, immunosuppressive therapy (IST) was used to target active myocardial inflammation (AMI). The study endpoint was a composite of cardiac death, heart transplantation and malignant VAs (VT, VF, appropriate ICD treatment). The study cohort is composed by 135 ULVACM patients (age 43 ± 14 years, 63% males, LVEF 55 ± 7%). NGS identified pathogenic or likely-pathogenic variants (PVs/LPVs) consistent with ACM in 21 cases (16%), whereas EMB showed AMI in 78 patients (58%), including 13/21 PVs/LPVs + (62%). IST was started in 41/78 AMI patients (53%), including 9/13 PVs/LPVs + (69%). Twenty patients (15%) met the study endpoint by 12 months, and 36 (27%) by the end of the study (60 ± 27 months). Beyond malignant VT onset, AMI was the only predictor of events by 12 months (HR: 5.0, 95% CI: 1.4–18.1, P = 0.007). No prognostic role was found for PVs/LPVs, except for the subgroup (n = 77) with nonsustained VT onset. Among AMI patients, those treated by IST had a significantly lower occurrence of events, both by 12-months (1/41 vs. 16/37, P < 0.001) and later (HR: 0.05, 95% CI: 0.01–0.21, P < 0.001). Results were independently confirmed in PVs/LPVs+ and PVs/LPVs- cases. Excluding the IST population, the association of multiple factors among VT onset, PVs/LPVs, and AMI, resulted in an improved discrimination of arrhythmic risk profiles. Conclusions Despite its limited diagnostic yield, the combined genetic and histological workup substantially contributed into ULVACM prognostic assessment. Furthermore, EMB identified suitable candidates for IST, who showed better outcomes irrespectively of their genotype.

The Path of Legalization of E-Cigarette Supervision

In recent years, although people have paid more and more attention to the negative effects of e-cigarettes on people's health, and China has gradually strengthened the supervision of e-cigarettes in order to protect people, especially minors, from the infringement of e-cigarettes, the legalization of e-cigarettes supervision cannot be achieved overnight, and there are still some problems such as the lack of national industry standards, disputes over regulatory subjects, unclear classification management, etc. Therefore, China should speed up the introduction of industry standards, determine the regulatory body with the aim of protecting national health, especially the rights and interests of minors, scientifically define the categories of e-cigarettes and formulate different regulatory measures, so as to safeguard national health while taking into account the healthy development of the e-cigarette industry and stimulate the innovative spirit of private enterprises.

Papillary tumor of the pineal region: Is stereotactic radiosurgery efficient for this rare entity?

Background: Papillary tumors of the pineal region are rare neuroepithelial lesions that were described for the 1st time in the WHO 2007 classification. Management of such lesions remains controversial. Case Description: We describe the case of a 26-year-old female who presented with intracranial hypertension syndrome secondary to a 1.9 cm3 lesion of the pineal region causing hydrocephalus. The patient benefited from an endoscopic third ventriculocisternostomy and a biopsy of her lesion in favor of a papillary tumor of the pineal region. After discussion of the surgical risks, the patient refused the surgical option and a stereotactic radiosurgery (SRS) was performed. She improved both clinically (allowing her to regain autonomy) and radiologically (reduction of 60% of tumor volume) at 1 year follow-up. Conclusion: Because of the rarity of the lesion, literature is yet not able to find consensus concerning management of such lesion, but SRS has proven efficiency for these Grades II or III lesions with high recurrence rates. Therefore, it should be considered as a primary therapeutic option allowing good outcome with low risks for the patient.

FUNCTIONAL OUTCOME OF DISTAL FEMORAL FRACTURES AND THEIR MANAGEMENT

Background: Fractures of distal end of femur are complex injuries which are difficult to manage. These fractures often are unstable and comminuted. Despite advances in surgical techniques and improvement in implants, treatment of distal femoral fractures remains a challenge in many situations. This study was done to analyse the functional outcome and this management of distal femoral fractures. Material & Methods: This study has been done between August 2008 to March 2010 on patients coming to Orthopaedics Department at Jawaharlal Nehru Hospital and Research Center, Bhilai. It is a prospective study which includes 25 patients with 10 Type A, 7 Type B and 8 Type C fractures of distal femur after fulfilling the inclusion and exclusion criteria. Results: Mean age of 47.08 years with sex incidence of 3.17:1(M:F). Mode of injury was RTA in 16, simple fall in 6, fall from height in 2 and railway track accident in 1 patient. There were 5-A1, 1-A2, 4-A3, 3-B1, 1-B2, 3-B3, 1-C1, 4-C2 and 3-C3 fractures. Results were found to be excellent in 17, good in 1, moderate in 3 and poor in 4 patients. Conclusion: We conclude that minimally invasive surgical techniques and with the availability of locking type of plates distal femoral fractures can now be dealt with more precessions and more satisfactory results can be produced. Keywords: Distal fractures, Muller Classification, Management, Femoral fractures

To Study Surgical Management of Distal Femoral Fractures & its Complication

Background: Fractures of distal end of femur are complex injuries which are difficult to manage. These fractures often are unstable and comminuted. Despite advances in surgical techniques and improvement in implants, treatment of distal femoral fractures remains a challenge in many situations. This study was done to analyse the functional outcome and this management of distal femoral fractures with its complication. Material & Methods: This study has been done between August 2008 to March 2010 on patients coming to Orthopaedics Department at Jawaharlal Nehru Hospital and Research Centre, Bhilai. It is a prospective study which includes 25 patients with 10 Type A, 7 Type B and 8 Type C fractures of distal femur after fulfilling the inclusion and exclusion criteria. Results: Mean age of 47.08 years with sex incidence of 3.17:1(M:F). Mode of injury was RTA in 16, simple fall in 6, fall from height in 2 and railway track accident in 1 patient. There were 5-A1, 1-A2, 4-A3, 3-B1, 1-B2, 3-B3, 1-C1, 4-C2 and 3-C3 fractures. Complication in form of varus angulation was found in 5 cases, shortening occurred in 2 patients, backing out of distal femoral locking screw occurred in 1 patient. Deep infection occurred in 1 patient. Implant failure and varus angulation at fracture site occurred in 1 patient. Conclusion: We conclude that minimally invasive surgical techniques and with the availability of locking type of plates distal femoral fractures can now be dealt with more precessions and more satisfactory results can be produced. Keywords: Complication, Muller Classification, Management, Femoral fractures.

Study on Compulsory Classification Management and Behavior Synergy of Municipal Solid Waste

There is a general phenomenon of incoordination between garbage classification management and participation behavior, which seriously affects the sustainable management efficiency of domestic garbage. In order to solve this problem, this paper introduced the subject-object-process model into the waste classification management system, and constructed a mandatory classification management model of municipal solid waste and a comprehensive evaluation index system. Taking Beijing, China, as an example, the coupling coordination degree of garbage classification behaviors of residents in different was compared, and the coordination status of household waste management and behaviors was obtained. The results show that the synergy between government management and residents’ household waste classification behavior is between 0.40 and 0.68, and the synergy between enterprises’ participation in governance and residents’ behavior is between 0.45 and 0.75. The coordination degree between domestic waste management and residents’ participation behavior is generally in primary coordination or slight imbalance. The synergy degree between the secondary indicators of domestic waste management and residents’ behavior is higher than that of the tertiary indicators. Superposition effect of integrated management measures is better; among the psychological factors affecting residents’ classification behavior, the awareness rate and recognition degree of waste classification are very high, the awareness of environmental responsibility and social pressure are lagging behind. The study of synergy under the framework of subject-object-process not only quantifies the overall synergy between management and residents’ behavior, but also provides a method to further implement garbage classification management in a targeted manner. Based on the synergy analysis, according to the weak links of various regions, classified management is carried out around publicity and education, supervision and management, assessment, rewards and punishments.