Tumor necrosis factor-alpha blockade ameliorates inflammatory response in two children with chronic infantile neurological, cutaneous and articular syndrome.

Chronic infantile neurological, cutaneous and articular (CINCA) syndrome is a rare autoinflammatory disease caused by monogenic defects in the NLRP3 gene. Pro-inflammatory cytokines such as interleukin (IL)-1ß play a crucial role in the pathogenesis, and IL-1 receptor antagonists have been regarded as the mainstay therapy. Endogenous tumor necrosis factor (TNF)-α was found recently to be involved in the onset of the disease. Here, we report two Chinese children with CINCA syndrome who had elevated serum levels of TNF-α, with one carrying a novel mutation of c.1330T/G (p.444Phe/Val) in exon 3 of the NLRP3 gene. Anti-TNF-α (etanercept) therapy successfully alleviated both clinical symptoms and systemic inflammation after 6 months. These results suggest the complexity of the mechanisms of the disease and that TNF-α blockade will broaden the therapeutic approach for a subset of patients.

Increased attention deficit/hyperactivity and oppositional defiance symptoms of 6-12 years old Chinese children with atopic dermatitis.

Atopic dermatitis (AD), a prevalent chronic skin disease in children, has been associated with psychosocial illness and reduced quality of life because of severe itching and sleep deprivation. Previous studies have found a consistent association between AD and attention deficit hyperactivity disorder (ADHD). However, little is known about this relationship in Chinese children with AD.To investigate co-occurrence of ADHD symptoms, oppositional defiant disorder (ODD) symptoms, and the relevant risk factors of AD, as well as its impact on the quality of life in Chinese school-aged children with AD.Outpatients aged 6 to 12 years with confirmed AD and healthy controls matched for age were randomly included in this study from October 2018 to October 2019. AD severity was evaluated using the Severity Scoring of Atopic Dermatitis scale (SCORAD). Inattention, hyperactivity, and oppositional defiant symptoms were evaluated by using the Swanson, Nolan and Pelham IV Teacher and Parent 26-Item Rating Scale (SNAP-IV) questionnaires and quality of life was evaluated using the Children's Dermatology Life Quality Index (CDLQI).The study included 89 AD patients and 184 healthy controls. AD patients were more likely to have ADHD symptoms (10.1% vs. 3.8%; P = .04) and ODD symptoms (5.6% vs 0%; P < .001) than controls, especially hyperactive/impulsive (P = .03). The severity of itching and sleep loss in AD patients were positively correlated with inattention (P = .03; P < .001), hyperactivity/impulsiveness (P = .01; P = .03), and oppositional defiance scores (P < .01; P = .04). Sleep loss in AD patients was independently associated with an increased risk of ADHD symptoms (OR, 1.78; 95% CI, 1.07-2.98; P = .03). The mean CDLQI scores of AD patients were 6.98 ±â€Š5.02, and CDLQI scores were significantly higher in AD patients with ADHD symptoms than in those without ADHD symptoms (11.44 vs. 6.48; P = .01).AD is a prevalent chronic condition associated with an increased likelihood of ADHD symptoms and ODD symptoms in school-aged children. Sleep deprivation caused by AD may be a risk factor for ADHD. AD affects quality of life, especially in patients with ADHD symptoms. AD patients with symptoms of inattention and hyperactivity should be evaluated for ADHD.

Modified Method to Increase the Volume and Stability of Bleomycin Foam: An Experimental Study.

BACKGROUND: Bleomycin foam is an effective sclerotherapy method for venous malformations. The preparation method is rather complicated, and the volume and stability of the foam are limited. OBJECTIVE: To modify the currently used method for preparing bleomycin foam, to simplify the preparation procedure, and to produce foam with greater volume and increased stability. MATERIALS AND METHODS: Experiment 1: 6.0 IU of bleomycin powder was dissolved in different human serum albumin (HSA):saline solution (SS) ratios of 0.5:1.5, 0.75:1.25, 1:1, 1.25:0.75, 1.5:0.5, 1.75:0.25, and 2:0 in volume; then, an air:liquid ratio of 2:1 was used to create foam using the Tessari method. Experiment 2: 6.0 IU of bleomycin was dissolved directly in 2.0 mL of HSA; then, air:liquid ratios of 1:1, 2:1, 3:1, and 4:1 were used to create foam using the Tessari method. The optimum proportions of HSA:SS and air:liquid were screened by comparing the foam half-life (FHL). RESULTS: Experiment 1: the optimum proportion of HSA:SS was 2:0, and the FHL was 7.5 minutes. Experiment 2: the optimum proportion of air:liquid was 3:1, and the FHL was 9.0 minutes. CONCLUSION: The modified method is simpler and could produce more stable bleomycin foam with greater volume.

Preliminary Experience With More Stable Polidocanol Foam in Sclerotherapy of Head and Neck Venous Malformations.

BACKGROUND: Foam sclerotherapy is an effective treatment strategy for venous malformations (VMs). Stability is one of the characteristics of sclerosant foams that determine the sclerosing power. OBJECTIVE: To evaluate the clinical outcomes of sclerotherapy with prolonged half-life of hyaluronic acid (HA)-polidocanol (POL) foam for treating head and neck VMs. MATERIALS AND METHODS: Seventy patients with head and neck VMs were enrolled between October 2013 and January 2015 in Qilu Hospital Shandong University. Sclerotherapy was performed with prolonged half-life POL foam by addition of 0.05% HA. All patients were followed up at 1, 3, and 6 months. Reviews on the site and size of the lesion, times and duration of treatments, therapeutic response, and complications were performed to evaluate the end result. RESULTS: Sites of lesions included face, bucca, parotid region, neck, tongue, floor of mouth, lip, forehead, palate, and nose. An overall average of 2.5 treatments was required. Total response rate was 100%. "Resolution" was achieved in 21 cases (30%) and a significant response in 49 patients (70%). Immediate swelling was the most common complication. Epidermal extravasation was seen in 1 patient. Mucosal ulcer was observed in 1 patient. No generalized complications occurred. CONCLUSION: The prolonged half-life of HA-POL foam sclerotherapy of VMs in the head and neck is safe and effective.

Multistage Sclerotherapy for Extensive Lymphatic Malformations With Airway Involvement in Infant: A Protocol to Prevent Tracheotomy.

PURPOSE: The management of extensive head and neck lymphatic malformations (LMs) in infants is challenging because of life-threatening upper airway compression. The aim of this study was to present a management protocol and evaluate the clinical outcomes for preventing tracheotomy in these patients. MATERIALS AND METHODS: Fifteen infants with extensive head and neck LMs and airway involvement were enrolled from August 2010 through September 2015 at the Qilu Hospital of Shandong University (Jinan, China). According to various key factors associated with airway compression of patients in the perioperative period, different anesthesia types, treatment times, sclerosant concentrations, and sclerotherapy protocols were used. Multistage sclerotherapy was performed with bleomycin A5. All patients were followed at 1, 3, 6, and 12 months. More extended follow-up was offered if patients had a residual lesion requiring supplementary sclerotherapy. Reviews on the site and size of the lesion, times and durations of treatments, therapeutic response, airway complications, and conduction of tracheotomy were performed. RESULTS: LM lesions in the head and neck were located in the floor of the mouth, tongue, and neck. An overall average of 5 treatments was required; a lesion volume decrease of more than 50% was achieved in all patients. For efficacy, morphologic resolution was achieved in 3 of 15 cases (20%), and there was a substantial response in 12 of 15 cases (80%). Eight of 15 patients (53.3%) with microcystic LMs exhibited immediate swelling and had more serious upper airway symptoms than preoperatively, and 2 of 15 patients (13.3%) had feeding difficulty. No upper airway obstruction occurred and no tracheotomy was performed in the patients in this study. CONCLUSIONS: Multistage sclerotherapy with bleomycin A5 is a safe and effective treatment for extensive head and neck LMs in infants with airway involvement. A routine perioperative protocol is essential for decreasing airway complications.

A modified Tessari method for producing more foam.

This study aimed to develop a modified Tessari method for producing more sclerosing foam in treatment of extensive venous malformations. Sclerosing foam was produced by using Tessari method and the modified Tessari method. The procedure of the later was as follows: prepared foam in a sclerosant-air ratio of 1:4; connected three disposable 10 ml syringes to two medical three-way taps; drawn 4 ml of liquid sclerosant into one syringe and 16 ml averagely of air into the other two; then moved the plungers of all syringes back and forth for 20 times to produce sclerosing foam. The volume and foam half time (FHT) of foam produced by the two methods were compared. The average volume of sclerosing foam produced by Tessari method and the modified Tessari method were 9.8 and 19.7 ml, and assessed to have statistical difference. The FHT of foam produced by the two methods were 120 and 150 s, and assessed to have statistical difference. In conclusion, the modified Tessari method could produce more fresh and stable sclerosing foam.

An Investigation on the Influence of Hyaluronic Acid on Polidocanol Foam Stability.

BACKGROUND: Foam sclerotherapy is an effective treatment strategy for varicose veins and venous malformations. Foam stability varies according to foam composition, volume, and injection technique. OBJECTIVE: To evaluate the stability of polidocanol (POL) foam with the addition of hyaluronic acid (HA). MATERIALS AND METHODS: Group A: 2 mL of 1% POL + 0 mL of 1% HA + 8 mL of air; Group B: 2 mL of 1% POL + 0.05 mL of 1% HA + 8 mL of air; Group C: 2 mL of 1% POL + 0.1 mL of 1% HA + 8 mL of air. Tessari's method was used for foam generation. The half-life, or the time for a volume of foam to be reduced to half of its original volume, was used to evaluate foam stability. Five recordings were made for each group. RESULTS: The half-life was 142.8 (±4.32) seconds for 1% POL without the addition of HA, 310.6 (±7.53) seconds with the addition of 0.05 mL of 1% HA, and 390.4 (±13.06) seconds with the addition of 0.1 mL of 1% HA. CONCLUSION: The stability of POL foam was highly increased by the addition of small amounts of HA.

A new method for using radiopaque sclerosing foam to treat venous malformations.

BACKGROUND: Sclerosing foam is recommended to treat venous malformations (VMs). However, determining the precise dosage for satisfactory efficacy and few side effects are still challenging. OBJECTIVE: To develop a new method of preparing sclerosing foam by adding contrast agent to treat patients with VMs visibly and precisely under radiologic guidance. MATERIALS AND METHODS: Sclerosant (polidocanol) and contrast agent (iopromide) were mixed to prepare a new sclerosing foam according to Tessari method, and different ratios of the 2 materials were used to study the stability (half-life) of the produced foams. X-ray absorption of the radiopaque foams was observed during patient treatment with digital subtraction angiography (DSA). RESULTS: The foams generated with the new method demonstrated a high x-ray absorption under DSA. The foam half-life among groups varied. The ratio of iopromide/polidocanol at 1:2 had a significantly longer half-life than the 1:1 or polidocanol-only group. The 1:2 group had a relative high x-ray absorption and was radiopaque to allow visualization of the sclerosing foam and lesion with DSA. CONCLUSION: Mixing contrast agent with sclerosant to prepare radiopaque foams is a new method for treating complex VMs with DSA, and a ratio of 1:2 is recommend for polidocanol and iopromide.

Effects of inorganic electrolyte anions on enrichment of Cu(II) ions with aminated Fe3O4/graphene oxide: Cu(II) speciation prediction and surface charge measurement.

The present work evaluated the effects of six inorganic electrolyte anions on Cu(II) removal using aminated Fe3O4/graphene oxide (AMGO) in single- and multi-ion systems. A 2(6-2) fractional factorial design (FFD) was employed for assessing the effects of multiple anions on the adsorption process. The results indicated that the Cu(II) adsorption was strongly dependent on pH and could be significantly affected by inorganic electrolyte anions due to the changes in Cu(II) speciation and surface charge of AMGO. In the single-ion systems, the presence of monovalent anions (Cl(-), ClO4(-), and NO3(-)) slightly increased the Cu(II) adsorption onto AMGO at low pH, while the Cu(II) adsorption was largely enhanced by the presence of SO4(2-), CO3(2-), and HPO4(2-). Based on the estimates of major effects and interactions from FFD, the factorial effects of the six selected species on Cu(II) adsorption in multi-ion system were in the following sequence: HPO4(2-)>CO3(2-)>Cl(-)>SO4(2-)>NO3(-)=ClO4(-), and the combined factors of AD (Cl(-)×SO4(2-)) and EF (Cl(-)×SO4(2-)) had significant effects on Cu(II) removal.

Effect of aniline on cadmium adsorption by sulfanilic acid-grafted magnetic graphene oxide sheets.

Cd(II) has posed severe health risks worldwide. To remove this contaminant from aqueous solution, the sulfanilic acid-grafted magnetic graphene oxide sheets (MGOs/SA) were prepared and characterized. The mutual effects of Cd(II) and aniline adsorption on MGOs/SA were studied. The effects of operating parameters such as pH, ionic strength, contact time and temperature on the Cd(II) enrichment, as well as the adsorption kinetics and isotherm were also investigated. The results demonstrated that MGOs/SA could effectively remove Cd(II) and aniline from the aqueous solution and the two adsorption processes were strongly dependent on solution pH. The Cd(II) adsorption was reduced by the presence of aniline at pH<5.4 but was improved at pH>5.4. The presence of Cd(II) diminished the adsorption capacity for aniline at pH<7.8 but enhanced the aniline adsorption at pH>7.8. The decontamination of Cd(II) by MGOs/SA was influenced by ionic strength. Besides, the adsorption process could be well described by pseudo-second-order kinetic model. The intraparticle diffusion study revealed that the intraparticle diffusion was not the only rate-limiting step for the adsorption process. Moreover, the experimental data of isotherm followed the Freundlich isotherm model.

Bioaccumulation of zinc, lead, copper, and cadmium from contaminated sediments by native plant species and Acrida cinerea in South China.

This research was conducted to search and identify spontaneously growing heavy metal-tolerant plant species that are potentially useful for phytoremediation in contaminated sediment. Five sites were selected for collection of plants growing on polluted shore (river bank) sediment of the Xiang River, China. The concentrations of Zn, Pb, Cu and Cd in plants, sediments, and grasshoppers were determined using flame atomic absorption spectrophotometer (AAS700, Perkin-Elmer, USA). Considering translocation factor and bioaccumulation factor, Rumex crispus (Polygonaceae), Rumex dentatus (Polygonaceae), and Lagopsis supina (Labiatae) could be potentially useful for phytostabilization of metals. R. crispus can be considered potentially useful for phytoextraction of Cd. In light of the biomagnification factors, grasshoppers are deconcentrators for Pb and Cd, microconcentrators for Zn and macroconcentrators for Cu to the plants, respectively. To the best of our knowledge, the present study is the first report on Zn, Pb, Cu and Cd accumulation in R. crispus and L. supina, providing a pioneer contribution to the very small volume of data available on the potential use of native plant species from contaminated sediments in phytostabilization and phytoremediation technologies.

Responses of Phanerochaete chrysosporium to toxic pollutants: physiological flux, oxidative stress, and detoxification.

The white-rot fungus Phanerochaete chrysosporium has been widely used for the treatment of waste streams containing heavy metals and toxic organic pollutants. The development of fungal-based treatment technologies requires detailed knowledge of the relationship between bulk water quality and the physiological responses of fungi. A noninvasive microtest technique was used to quantify real-time changes in proton, oxygen, and cadmium ion fluxes following the exposure of P. chrysosporium to environmental toxic (2,4-dichlorophenol and cadmium). Significant changes in H(+) and O(2) flux occurred after exposure to 10 mg/L 2,4-dichlorophenol and 0.1 mM cadmium. Cd(2+) flux decreased with time. Reactive oxygen species formation and antioxidant levels increased after cadmium treatment. Superoxide dismutase activity correlated well with malondialdehyde levels (r(2) = 0.964) at low cadmium concentrations. However, this correlation diminished and malondialdehyde levels significantly increased at the highest cadmium concentration tested. Real-time microscale signatures of H(+), O(2), and Cd(2+) fluxes coupled with oxidative stress analysis can improve our understanding of the physiological responses of P. chrysosporium to toxic pollutants and provide useful information for the development of fungal-based technologies to improve the treatment of wastes cocontaminated with heavy metals and organic pollutants.

Coarsening of extracellularly biosynthesized cadmium crystal particles induced by thioacetamide in solution.

A novel coarsening route for extracellularly biosynthesized cadmium nanocrystals was investigated for the first time. In this process, the white rot fungus Coriolus versicolor was employed to take up cadmium ions and synthesize extracellular cadmium crystal particles. The coarsening of the particles was induced by thioacetamide under certain conditions. Scanning electron microscopy showed that the formed cadmium crystal particles were coarsened from about 100nm to 2-3µm. The corresponding energy-dispersive X-ray spectra confirmed the presence of proteins in the particles. The maximum removal efficiency of Cd(II) increased from 17% to 87%, and the corresponding sorption capacity of biomass increased from 4 to 24mgg(-1) with the completion of the coarsening process. The properties of the coarsened particles were also examined using X-ray diffraction (XRD) and transmission electron microscopy (TEM). XRD analysis of fungal mycelial pellets embedded with the coarsened particles confirmed the formation of cubic crystalline cadmium sulfide particles. The TEM results suggest that the coarsened particles were composed of clusters of several smaller particles. The changes in the functional groups on the biomass surface were studied through Fourier transform infrared spectroscopy. Based on the results above, a possible mechanism for the formation and coarsening of cadmium crystal particle is also discussed.

Adsorption of chromium (VI) by ethylenediamine-modified cross-linked magnetic chitosan resin: isotherms, kinetics and thermodynamics.

The adsorption of chromium (VI) ions from aqueous solution by ethylenediamine-modified cross-linked magnetic chitosan resin (EMCMCR) was studied in a batch adsorption system. Chromium (VI) removal is pH dependent and the optimum adsorption was observed at pH 2.0. The adsorption rate was extremely fast and the equilibrium was established within 6-10min. The adsorption data could be well interpreted by the Langmuir and Temkin model. The maximum adsorption capacities obtained from the Langmuir model are 51.813mgg(-1), 48.780mgg(-1) and 45.872mgg(-1) at 293, 303 and 313K, respectively. The adsorption process could be described by pseudo-second-order kinetic model. The intraparticle diffusion study revealed that film diffusion might be involved in the present case. Thermodynamic parameters revealed the feasibility, spontaneity and exothermic nature of adsorption. The sorbents were successfully regenerated using 0.1N NaOH solutions.

[Effect of bone morphogenetic proteins-2 on mandibular distraction osteogenesis in rabbits].

OBJECTIVE: To investigate the Effect of recombinant adenovirus vectors containing human Bone morphogenetic proteins-2 (Ad-hBMP-2) on the for mandibular distraction osteogenesis (DO) in rabbits. METHODS: Twenty-four New Zealand white rabbits were randomly divided into experimental group, and control group and underwent mandibular distraction osteogenesis. After 5 days latency, the distracters were activated at a speed of 0.5 mm every 12 hours for 7 days, then on the first day in the consolidation period, the distraction gaps of experimental group were injected with 0.2 ml Ad-hBMP2 10(12) pfu/L, while the animals of control group were injected with 0.2 ml Ad-EGFP 10(12) pfu/L. At the 7 th and 28 th day of consolidation period, specimens were obtained, X-ray and histomorphology were performed. The bone density and the quantity of new bone formation in the distraction gaps were observed and compared between the two groups at different consolidation period. RESULTS: Ad-hBMP-2 treated specimens demonstrated an increased amount of new bone formation CONCLUSIONS: Adenovirally-mediated delivery of BMP-2 can locally increase bone deposition during DO, which may potentially shorten the consolidation period.

[Mandibular keratocyst induced trigeminal neuralgia: report of one case].

Trigeminal neuralgia is very common in clinic, but the etiology is still unclear. This articles reported a case of trigeminal neuralgia induced by jaw keratocyst. The clinical manifestation, radiographic characteristic, pathologic diagnosis and treatment were discussed. It is concluded that jaw bone cavities may be one of the major etiologic factors of secondary trigeminal neuralgia. Surgical curettage of the lesion is the main treatment for this disease.

[Gene expression profile of refractory acute myeloid leukemia (M2a)].

BACKGROUND & OBJECTIVE: Recurrence is the major cause of treatment failure of acute myeloid leukemia (AML). At recurrence, some patients show changes in immunophenotype and cytogenetics. Drug resistance, the main cause of refractoriness of AML, is related to abnormal genes expression. This study was to detect differential expression of genes in naive and recurrent/refractory AML, and explore potential mechanisms of recurrent/refractory AML. METHODS: Differential gene expressions of bone marrow mononuclear cells between naive and recurrent/refractory diseases in 5 self-paired patients with AML-M(2a) were detected by DNA microarray. RESULTS: In 925 tested genes, 14 were differentially expressed between naive and recurrent/refractory diseases in the 5 self-paired patients. Of the 14 genes, 12 (involved in signal transduction, DNA replication, regulation of transcription, RNA processing, and regulation of cell cycle) were obviously up-regulated in recurrent diseases, and up-regulation of RRM1 (involved in DNA replication) was the most obvious. CONCLUSIONS: Development of recurrent/refractory AML-M(2a) is concerned with various genes. Up-regulation of these genes suggests that proliferation of recurrent/refractory AML-M(2a) blasts may be higher than that of naive AML-M(2a) blasts.

[A serious complication-obstructive sleep apnea hypopnea syndrome after velopharyngeal ring ligation procedure: report of 6 cases].

PURPOSE: To discuss the occurrence and characteristics of obstructive sleep apnea hypopnea syndrome (OSAHS) after velopharyngeal ring ligation procedure (VRLP). METHODS: Seven hundred and eight cleft palate patients underwent velopharyngeal ring ligation procedure from 1997 to 2003. Nine cases were found having some suspicious symptoms of OSAHS after operation and 6 of them were confirmed as OSAHS by polysomnography. Six VRLP patients without OSAHS as controls were examined with polysomnography. Chi-square test and t test were used to determine the statistical differences between the groups. RESULTS: Six cases of 708 patients met the criterion of severe OSAHS and the incidence of OSAHS after VRLP in children below 12 years was 1.8%. Compared with controls, more arousals and longer awakening were found in OSAHS group, the ratio of III and IV phase of NREM and REM in total sleep was significantly decreased (P<0.01). CONCLUSION: OSAHS was likely to occur in VRLP patients below 12 years. Many feature of this kind of OSAHS were different from the ordinary pediatric OSAHS. It showed that OSAHS after VRLP was characterized by more apneas, less partial upper airway obstruction and associated with distinguished staged desaturation.