Case report: Aggressive progression of acute heart failure due to juvenile tuberculosis-associated Takayasu arteritis with aortic stenosis and thrombosis.

Background: Takayasu arteritis (TA) is a chronic granulomatous vasculitis with unknown pathophysiology. TA with severe aortic obstruction has a poor prognosis. However, the efficacy of biologics and appropriate timing of surgical intervention remain controversial. We report a case of tuberculosis (TB)-associated TA with aggressive acute heart failure (AHF), pulmonary hypertension (PH), thrombosis, and seizure, who failed to survive after surgery. Case presentation: A 10-year-old boy who developed a cough with chest tightness, shortness of breath, hemoptysis with reduced left ventricular ejection fraction, PH, and increased C-reactive protein and erythrocyte sedimentation rate was hospitalized at the pediatric intensive care unit of our hospital. He had strongly positive purified protein derivative skin test and interferon-gamma release assay result. Computed tomography angiography (CTA) showed occlusion of proximal left subclavian artery and stenosis of descending aorta and upper abdominal aorta. His condition did not improve after administration of milrinone, diuretics, antihypertensive agents, and intravenous methylprednisolone pulse followed by oral prednisone. Intravenous tocilizumab was administered for five doses, followed by two doses of infliximab, but his HF worsened, and CTA on day 77 showed complete occlusion of the descending aorta with large thrombus. He had a seizure on day 99 with deterioration of renal function. Balloon angioplasty and catheter-directed thrombolysis were performed on day 127. Unfortunately, the child's heart function continued to deteriorate and died on day 133. Conclusion: TB infection may be related to juvenile TA. The biologics, thrombolysis, and surgical intervention failed to achieve the anticipated effect in our case with aggressive AHF due to severe aortic stenosis and thrombosis. More studies are needed to determine the role of biologics and surgery in such dire cases.

Short-term effectiveness of baricitinib in children with refractory and/or severe juvenile dermatomyositis.

Objective: To determine the short-term effectiveness safety of baricitinib in children with refractory and/or severe juvenile dermatomyositis (rsJDM) in a real-world setting. Methods: This was a single-center retrospective study, including 20 children with rsJDM. They were all treated using baricitinib combined with steroids and other immunosuppressive agents. The childhood myositis assessment scale (CMAS) and PRINTO remission criteria were used to evaluate the disease severity and treatment outcome at 0, 4, 12, and 24 weeks after initiation of baricitinib. Results: The skin rash improved in 95% of patients (19/20) at week 24, with a significant decrease of skin-DAS at weeks 12 (6.0 vs. 2.0, p < 0.05] and week 24 [6.0 vs. 1.0, p < 0.05) by median statistics. The CMAS score increased significantly at week 12 (41.0 [29.0, 44.0] vs. 46.0 [42.0, 52.0], p < 0.05) and week 24 (41.0 [29.0, 44.0] vs. 50.0 [45.0, 52.0], p < 0.05), as did the manual muscle testing (MMT)-8 score at week 24 (73.0 [610, 76.0] vs. 79.0 [77.0, 80.0], p < 0.05). At 24 weeks, the complete response (CR) and partial response (PR) were achieved in 75% (15/20) and 15% (3/20), respectively. The dose of corticosteroids (CS) decreased by 37% from the baseline (0.53 [0.42, 1.00] mg/kg) to week 12 (0.33 [0.18, 0.40] mg/kg) (p < 0.05), and by 49% at week 24 (p < 0.05). No serious side effects were observed. Conclusion: Baricitinib combined with traditional immunosuppressants treatment was efficacious in rsJDM. Add-on therapy of baricitinib was helpful for tapering CS dose. No serious side effects were observed in this study.

A moisture balanced antibacterial dressing loaded with lysozyme possesses antibacterial activity and promotes wound healing.

Wound moisture management is very important in wound healing. Previous wound management has included dry healing and moist healing, and the theory of wound moisture balance is currently generally accepted. However, current studies have not reported which humidity is suitable for wound healing and how to appropriately use antibacterial compounds when the humidity is suitable. Our study explored the moisture balance of polyurethane foam dressings through a moisture balance test and constructed a safe and effective moisture balanced antibacterial dressing by loading lysozyme onto a polyurethane foam dressing. Wound healing experiments showed that the wound healing speed was the fastest when the humidity was 25%. In vivo and in vitro antibacterial experiments showed the superior antibacterial performance of the dressing after lysozyme loading. We loaded lysozyme on moisture balanced polyurethane dressings by means of dopamine adsorption, and the modified dressings were named PU/DA-LYS (polyurethane/dopamine-lysozyme). Experiments on wound healing in infected mice indicated that PU/DA-LYS helps fight infection while promoting wound healing. Cytotoxicity experiments and in vivo biological safety experiments indicated that PU/DA-LYS was safe for use. Our study found that the lysozyme loaded polyurethane dressing can provide appropriate wound moisture and prevent bacterial infection, which is a future developmental direction for wound dressings.

Haploinsufficiency of A20 (HA20): updates on the genetics, phenotype, pathogenesis and treatment.

BACKGROUND: A20, a protein encoded by the tumor necrosis factor alpha-induced protein 3 gene (TNFAIP3), plays a vital role in the negative regulation of inflammation and immunity. Loss-of-function mutation in TNFAIP3 leads to a new described autoinflammatory disease-haploinsufficiency of A20 (HA20). Since HA20 was first described in 2016, a number of new cases have been described in this literature, however, the disease and its pathogenesis are poorly understood. This review seeks to improve clinical recognition of this disorder, and promote both earlier diagnosis and initiation of targeted therapies to improve patients' outcomes. METHODS: We reviewed 26 papers about A20 and HA20, and we summarized genetic variants and clinical manifestations of a total of 61 reported patients from 26 families identified to have a genetic diagnosis of germline pathogenic variants in TNFAIP3/A20. Additionally, we discussed the pathogenesis and treatment of HA20. RESULTS: A total of 24 pathogenic variants of A20 had been reported. There was significant clinical heterogeneity, even among those with the same variants in TNFAIP3. Prior to receiving a molecular diagnosis of HA20, patients had been diagnosed with Behcet's disease, rheumatoid arthritis, rheumatic fever, juvenile idiopathic arthritis, systemic lupus erythematosus, and even adult-onset Stills' disease. The patients with HA20 that presented with inflammatory signatures in NF-κB signaling were mostly responsive to treatment. CONCLUSIONS: HA20 is a monogenic autoinflammatory disease with highly variable clinical manifestations. This extensive heterogeneity makes it difficult to set a clinical diagnostic criteria, and genetic sequencing is necessary for a definitive diagnosis of HA20.

[Repair of deep wound on the head due to high-voltage electrical burn].

OBJECTIVE: To explore the methods for repairing deep wound on the head due to high-voltage electrical burn (HEB). METHODS: Twenty-six patients with deep wounds on the head due to HEB were hospitalized from June 2002 to May 2012. They were all injured by high-voltage (voltage ranged from 380 V to 300 kV) electric current, involving head and several other parts over the body. The total burn area ranged from 1% to 75% TBSA, and the depth ranged from deep partial-thickness to full-thickness (including muscle or even bone). Scalp defect area ranged from 3 cm×2 cm to 20 cm×18 cm, and the maximum size of skull exposure was 12 cm×9 cm and the maximum size of skull defect was 7 cm×6 cm. The wounds of 26 patients were repaired with 7 local advance flaps, 4 bilateral rotation flaps, 18 local rotation flaps combined with thin split-thickness skin grafts in donor site, and 3 free anterolateral thigh flaps with vascular anastomosis. In four of the 26 patients, expander was used in the early stage after burn and 5 after wound healing (with thin split-thickness grafts). RESULTS: All flaps and skin grafts survived, except for one flap which was complicated by wound infection, and it was healed after dressing and secondary suturing. The implanted expander expanded smoothly. Patients were followed up for 15 days to three years after surgery. Satisfactory results were obtained, and wounds of 15 patients were repaired completely. CONCLUSIONS: Deep wound on the head due to HEB should be repaired with suitable flap combined with skin graft in donor site, and implantation of expander according to the injury area and condition of patient.

[Experience in the treatment of severe pressure sore].

OBJECTIVE: To summarize the experience in the treatment of severe pressure sore. METHODS: From Aug. 2007 to Jun. 2011, 21 cases of severe pressure sore with 43 III-IV degree lesions were treated with combination treatment, including vacuum sealing drainage technique, local fascia flaps, upper or lower gluteus maximus island myocutaneous flaps, lower gluteus maximus myocutaneous flap, neurocutaneous femoris posterior flaps, tensor fascia lata island myocutaneous flaps, free latissimus dorsi myocutaneous flaps, and skin graft, combined with stryker frame and nursing tracking guidance. 13 of 21 cases had multiple pressure sore. Among them, 5 III degree pressure sores were covered by skin grafting and 3 non-caudal III degree pressure sores (< 2 cm in width) were directly closed after debridement. 8 of 21 cases had single IV degree pressure sore. RESULTS: All the 43 wounds healed completely. 5 wounds in 3 cases had effusion under flap which healed after re-drainage. The wounds were not healed in 3 cases with flap transposition which were also healed after re-debridement. All the flaps survived completely. 16 cases were followed up for 2-26 months. Recurrence happened in 4 cases after discharge because of not following the required nursing care. CONCLUSIONS: Comprehensive application of vacuum sealing drainage technique, multiple myocutaneous flaps and skin grafting, combined with stryker frame and nursing tracking guidance after discharge can be used for the treatment of severe pressure sore with satisfactory results.

[Reverse small saphenous vein-sural neurovascular island flap for t he reconstruction ofsoft tissue defect on foot and ankle in children].

OBJECTIVE: To investigate the clinical application of reversed small saphenous vein-sural neurovascular island flap for reconstruction of soft tissue defect on foot and ankle in children. METHODS: From July 2006 to June 2008, 8 children with soft tissue defects on foot, heel or ankle were treated with reversed small saphenous vein-sural neurovascular island flaps. The size of flaps ranged from 6 cm x 5 cm to 9 cm x 7 cm. The upper margin of the flaps reached the upper third of cruris, with 1 case reaching the transverse line of popliteal fossa. RESULTS: All the flaps survived. The patients were followed up for 1 - 17 months with good aesthetic and functional results. The growth of the two legs had no difference. The sensation of the flaps improved with no heel ulcer and no dysfunction at the donor site. The upper boundary of flaps can reach the upper third of the cruris even the reansverse line of popliteal fossa. The rotation point of the flaps located at 4 - 6 cm above the lateral ankle in children. CONCLUSIONS: The reversed small saphenous vein-sural neurovascular island flap in children has a reliable survival area. The operation is easily performed without any obvious influence on the growth of the operated cruris. It is a good reconstructive method for soft tissue defect in foot and ankle.

[Repair of high-voltage electric burn in jaw and neck region with insular pectoralis major myocutaneous flap].

OBJECTIVE: To evaluate the effect of insular pectoralis major myocutaneous flap on repair of jaw and neck tissue defect as a result of high-voltage electric burn. METHODS: Eighteen patients with large area tissue defect in jaw and neck caused by high-voltage electric burn hospitalized from August 2001 to December 2007 were repaired with insular pectoralis major myocutaneous flap. The flaps (from 12 cm x 10 cm to 16 cm x 13 cm) was transplanted in 8 patients after early wound debridement, and in 10 patients on infected wounds. RESULTS: All flaps survived wells except in 4 patients necrotic area (length 2-5 cm, width 1-2 cm) appeared in the distal wound edge, localized dehiscence of wound edge in 2 cases, which were healed after dressing change or secondary suturing. Patients were followed up from 6 months to 3 years, and the appearance and function were satisfactory. CONCLUSIONS: Pectoralis major muscle island myocutaneous flap has the advantage of large tissue mass, with thin and flat muscle belly, and rich blood supply, which is suitable for repair of large deep tissue defect in jaw and neck.

[Clinical observation of the efficacy of MEBO in the treatment of burn patients with burn area over 50% TBSA].

OBJECTIVE: To observe the effectiveness of MEBO in the treatment of burn patients with burn area over 50% TBSA. METHODS: Two hundred and ninety-eight patients hospitalized in our hospital from May of 1991 to December of 2003 with burn area over 50% TBSA, who had MEBO treatment before hospitalization, were enrolled in the study as the experiment (E) group. Another group of 300 burn patients with burn area over 50% TBSA that treated with SD-Ag cream were enrolled in the study as the control (C) group. Bacterial culture results, major changes in injury and mortality were compared between the two groups. RESULTS: There were 1 506 bacteria strains isolated from wounds in E group, and 9 main changes in injury (1679 cases) occurred with 20.8% mortality in this group. There were 353 bacteria strains isolated, with occurrence of 9 changes in injury (518 cases) and 4.7% mortality in the SD-Ag group. CONCLUSION: MEBO is much less effective for the treatment of the burn patients with large burn area compared with SD-Ag cream treatment.

[Effects of comprehensive interventions in community on smoking, chronic bronchitis, and asthma in rural areas of Beijing].

OBJECTIVE: To study the effects of comprehensive interventions in community on smoking, chronic bronchitis, and asthma in rural areas of Beijing. METHODS: Twenty-three villages in rural areas of Beijing were randomly divided into interventional (13 villages) and control villages (10 villages) in 1992. Comprehensive interventions including education of former-smokers and improvement of living environment were carried out in the interventional villages, and none was done in the control villages. In April 2000, surveys on smoking, chronic bronchitis, and asthma were carried out among 34,436 participants aged 15 or more in the interventional and control villages. During the same period, knowledge on prevention from chronic obstructive pulmonary diseases (COPD), living environments, and smoking were assessed among 1658 high-risk individuals of COPD at baseline and following-up period. RESULTS: The scores of knowledge and improvement on living environments in the interventional villages were significantly higher than those in control villages (P < 0.001). The decrease rate of smoking and current smoking rate in the interventional villages were significantly higher than in the control villages (0.4% vs -0.8%, P < 0.001; 2.4% vs 1.3%, P < 0.001) in men, while not different in women (P > 0.05). Among never smokers at baseline, the accumulated incidence of smoking among people aged 15 to 24 from 1993 to 2000 was significantly lower in the interventional villages than in the control villages in men (18.9% vs 23.7%, P = 0.005) and in women (0% vs 0.7%, P = 0.005). Daily cigarettes smoked by smokers in the interventional villages were less than in control villages in both men (14.8 +/- 7.0 vs 17.2 +/- 8.2 cigs daily, P < 0.001) and women (12.8 +/- 6.9 vs 13.4 +/- 7.2 cigs daily, P = 0.088). The increase of prevalence of chronic bronchitis in the interventional villages was less than in the control villages (men: 0.9% vs 1.3%, P = 0.012; women: 0.1% vs 0.3%, P = 0.003). After the age factor is adjusted, odds ratio (OR) for accumulated incidence of chronic bronchitis from 1993 to 2000 in the interventional villages were 0.80 (95%CI: 0.60-1.07) for men, 0.76 (95%CI: 0.45-1.28) in women. The OR for asthma was not significant in both men and women. CONCLUSIONS: Comprehensive interventions in community may improve knowledge of COPD prevention and living environments, decrease the smoking rate, cigarettes smoked per day, and incidence of chronic bronchitis, but have no significant effects on asthma.

Linkage analysis of 2q14-q23 and 5q32 with blood pressure quantitative traits in Chinese sib pairs.

OBJECTIVES: Several genome-wide scans recently accomplished in the ethnic Chinese revealed a number of candidate loci possibly contributing to essential hypertension, and some appeared to be replicable in 2q14-q23 and 5q32. The current study aimed to examine the linkage of qualitative and blood pressure quantitative traits in essential hypertension with these genomic regions in a large sample of Chinese hypertensive families. METHODS: We performed a genetic analysis on 148 randomly ascertained families containing 328 affected sib pairs, grouped into two geographically distinct subsets. Five highly informative microsatellite markers (D2S151, D2S142, D5S2090, D5S413 and D5S2013) were genotyped, and linkage analyses were performed with different genetic models. RESULTS: We did not observe consistent evidence for excess allele sharing identity by descent in either of the qualitative or the quantitative test. However, higher LOD scores were found at D5S2013 in North Group subset with Haseman-Elston and maximum likelihood (ML) variance (no dominance variance, NDV) algorithms. With the ML (NDV) algorithm, the LOD was 1.410 for diastolic blood pressure at this locus, although this was not statistically significant. CONCLUSIONS: These findings provide no evidence to support a significant linkage of 2q14-q23 or 5q32 with essential hypertension or blood pressure quantitative traits in the ethnic Chinese, and indicate the aetiologic diversity and complexity of hypertension. Previous reports implied 2q14-q23 or beta 2- adrenergic receptor gene potentially linked to essential hypertension in the ethnic Chinese. To replicate these results and perform quantitative linkage analysis, we genotyped members of 148 hypertensive families with five highly informative microsatellite markers. We observed no evidence of excess allele sharing identity by descent in sib pairs, revealing a lack of linkage between 2q14-q23 or 5q32 (chromosome region harboring the gene encoding beta 2 adrenergic receptor) and hypertension in our study sample.

[Effects of health promotion for eight years on knowledge, attitude and behavior of cardiovascular diseases in rural Fangshan District, Beijing].

OBJECTIVE: To evaluate the effects of health promotion for eight years on cardiovascular diseases based on the changes in knowledge (K), attitude (A) and behavior (B) (KAB) before and after intervention in rural population of Fangshan, Beijing. METHODS: Five townships in Fangshan District, Beijing were divided into intervention and control communities with cluster sampling. Totally 772 farmers were selected randomly for interviews with a questionnaire of KAB, including 424 in the intervention communities (IC) and 348 in the control communities (CC) in 1992. After community-based health promotion and education on cardiovascular prevention for eight years, 895 farmers, including 431 in IC and 464 in CC, were selected randomly again in 1999 to be interviewed with the same questionnaire of KAB used in 1992. RESULTS: From 1992 to 1999, knowledge of cardiovascular diseases was significantly improved in residents of IC (P < 0.01) and CC. Compared to the residents in CC, there was a net increase in knowledge in residents of IC. Significant improvement was found in 10 and 11 of the 18 items of knowledge in males and females (P < 0.01), respectively. Level of knowledge in cardiovascular diseases was significantly higher in the residents of IC than those of CC in 1999. Proportions of those with positive attitudes and appropriate behaviors were significantly elevated in the residents of IC (P < 0.01), as well as prevalence of smoking and alcohol drinking decreased. However, as compared to CC, attitude and behavior in most aspects in the residents of IC changed positively, but in few aspects changed negatively. It suggested knowledge improved better than attitude and behavior changed. CONCLUSIONS: Community-based health promotion and education for a longer term had obvious effects on improvement of knowledge in cardiovascular disease for rural population, but slower effects on changes in attitude and behavior, which suggested there still existed certain barriers to transform knowledge into healthy behavior.