An overview of patients with haemophilia A in China: Epidemiology, disease severity and treatment strategies.

INTRODUCTION: Haemophilia A (HA) is a rare X chromosome-linked bleeding disorder resulting in missing or defective clotting factor VIII (FVIII) and causes large disease burden. AIM: As a member of World Federation of Hemophilia, China seeks to understand the current epidemiology, disease profile and treatment landscape of patients with HA through the Hemophilia Treatment Center Collaboration Network of China (HTCCNC). METHODS: The HTCCNC enabled data collection on patients with HA from 166 member hospitals (2007-2019) across China. The distribution of patients across 31 divisions was summarized using a heat map. Patient demographics, disease severity and clinical and treatment information were summarized using descriptive statistics. RESULTS: HTCCNC identified 17,779 patients with HA during 2007-2019. Patients were predominantly male (99.99%), and 28.3% had a known family history of haemophilia. Among patients with lab-measured disease severity (N = 13,116), 6,519 had severe HA (49.7%), 4,788 had moderate HA (36.5%), and 1,809 had mild HA (13.8%). Among patients with information on the delays, delays in diagnosis and in treatment initiation were observed in 1,437 (28.8%) and 1,750 (39.2%) patients, respectively. On average, those patients had an 8.4 years gap between the first bleed and HA diagnosis and a delay of 8.6 years from the first bleed to treatment initiation. Additionally, 44.33% of patients relied solely on episodic treatments, and 16.2% received any prophylaxis treatments. CONCLUSIONS: Using data from the largest haemophilia registry in China, this study indicated that delayed diagnosis and treatment, together with low utilization of prophylaxis, are key challenges for patients with HA.

Three-dimensional soft tissue analysis for the evaluation of facial asymmetry in normal occlusion individuals.

OBJECTIVE: To identify the right and left difference of the facial soft tissue landmarks three-dimensionally from the subjects of normal occlusion individuals. MATERIALS AND METHODS: Cone-beam computed tomography (CT) scans were obtained in 48 normal occlusion adults (24 men, 24 women), and reconstructed into 3-dimensional (3D) models by using a 3D image soft ware. 3D position of 27 soft tissue landmarks, 9 midline and 9 pairs of bilateral landmarks, were identified in 3D coordination system, and their right and left differences were calculated and analyzed. RESULTS: The right and left difference values derived from the study ranged from 0.6 to 4.6 mm indicating a high variability according to the landmarks. In general, the values showed a tendency to increase according to the lower and lateral positioning of the landmarks in the face. Overall differences were determined not only by transverse differences but also by sagittal and vertical differences, indicating that 3D evaluation would be essential in the facial soft tissue analysis. CONCLUSIONS: Means and standard deviations of the right and left difference of facial soft tissue landmarks derived from this study can be used as the diagnostic standard values for the evaluation of facial asymmetry.

[Hard and soft tissue changes following the treatment of Class II division 1 malocclusion using twin-block appliance].

OBJECTIVE: To investigate the hard and soft tissue changes following the treatment of Class II division 1 malocclusion using Twin-block appliance. METHODS: 50 Class II division 1 malocclusion subjects whose handwrist radiographs were in FG-G stage were selected. One group (27 patients) was treated with Twin-block appliance, the other group (23 patients) was observed without treatment The acquired data of cephalometric of two groups were analyzed statistically with SPSS 11.0. RESULTS: Soft tissue changes, Ls-E, Li-E, U1-Stms, Stms-Stmi, NsLs-FH, LsNsLi, LsNsPg', the angle of H decreased. Sn-Stms, Stmi-Me', Ns-Me', Sn-Me', NsLi-FH, NsPg'-FH, A'Ls-FH, B'Li-FH, LiB' Pg', CmSnLs, GSnPg', the angle of Z increased, there was statistically significant difference (P < 0.05). Hard tissue changes, SNB, L1-NB, IMPA increased, ANB, U1-SN, U1-NA, FMIA decreased, there was statistically significant difference (P < 0.05). CONCLUSION: Sagittal relationship between upper- and lower-jaws is effectively improved after orthopedics with Twin-block appliance. Lower face height increases. Soft tissue profile tends to be straight-styled.

[Soft tissue changes following the treatment of Class II division 1 malocclusion using Headgear-activator appliance].

PURPOSE: To investigate the soft tissue changes following the treatment of Class II division 1 malocclusion using Headgear-activator appliance. METHODS: Fifty Class II division 1 malocclusion subjects who were at peak high velocity on the growth curve were divided into two groups. Standardized lateral cephalogram was taken for each patient in the two groups before and after treatment. SPSS11.0 software package was used to calculate the mean and standard deviation, matched t-test was used to evaluate the changes of the soft tissues. RESULTS: The angle of CmSnLs,LiB'Pg' and Z increased, the angle of H, LsNsLi and LsNsPg' decreased, there was statistically significant difference between the two groups. CONCLUSIONS: Using Headgear-activator appliances, soft tissue profile is effectively improved. Soft tissue chin is moved forward obviously. Lip protrusion is decreased.