Characteristics and trends in diagnosis of Kawasaki disease outside the usual age range.

OBJECTIVE: To explore the trends in diagnosis of Kawasaki disease (KD) and determine the characteristics for patients outside the usual age range of 6 months to 4 years. METHOD: A retrospective review of patients with KD identified in four epidemiological surveys spanning 20 years (1998-2017) in Shanghai was performed. RESULTS: A total of 8416 patients were included. All in all, 223 (2.6%) were aged 0 to 2 months, 639 (7.6%) were 3 to 5 months, 6556 (77.9%) were 6 months to 4 years, 915 (10.9%) were 5 to 9 years, and 83 (1.0%) were ≥ 10 years. The use of intravenous immunoglobulin increased in all extreme age groups, and delayed treatment rates decreased in all patients except in those aged 0 to 2 months and ≥ 10 years. The number of patients outside the usual age increased over time, but the proportion of these patients did not change significantly. They had more incomplete KD and coronary artery aneurysms (CAA), while those aged 0 to 2 months and ≥ 10 years had more delayed diagnoses in their respective age groups. The incidence of CAA was similar in younger and older children, but the former group had more cases of incomplete KD and abnormal laboratory parameters, while the latter group had longer duration of fever and was more difficult to diagnose promptly. CONCLUSIONS: The proportion of KD outside the usual age range did not increase over time. Older and younger children have different clinical and laboratory characteristics. Key Points • The number of patients outside the usual age increased over time, but the proportion of these patients did not increase. • Patients aged 0 to 2 months and ≥ 10 years had more delayed diagnoses in their respective age groups. • Younger children had more cases of incomplete KD and abnormal laboratory parameters, while older children had longer duration of fever and were more difficult to diagnose promptly.

Epidemiologic Features of Kawasaki Disease in Shanghai From 2013 Through 2017.

BACKGROUND: We sought to investigate epidemiologic features of Kawasaki disease (KD) in Shanghai from 2013 through 2017 and identify risk factors for coronary artery lesions (CAL). METHODS: As in our previous three surveys, a set of questionnaires and diagnostic guidelines for KD were sent to 50 hospitals providing pediatric medical care in Shanghai. Medical records of KD patients diagnosed from January 2013 through December 2017 were retrospectively analyzed. Multivariate logistic regression analysis was performed to identify risk factors for CAL. RESULTS: A total of 4,452 cases were enrolled. Male-to-female ratio was 1.7:1. The incidence of KD was 68.8 to 107.3 per 100,000 children aged <5 years from 2013 to 2017. Age at onset ranged from 15 days to 14.0 years (median: 1.8 years). KD occurred more frequently in spring and summer. Of 4,325 patients (97.0%) receiving intravenous immunoglobulin (IVIG), 362 (8.4%) were resistant to initial IVIG. CAL occurred in 406 (9.1%) patients, including 118 (2.7%) with medium aneurysms and 31 (0.7%) with giant aneurysms. Recurrent cases were 60 (1.3%). No death was found in this survey. Higher platelet levels, lower albumin levels, male sex, incomplete KD, IVIG resistance, and receiving initial IVIG ≤4 days or >10 days, were independently associated with CAL. CONCLUSIONS: The incidence of KD in Shanghai had substantially increased while the proportion of CAL had substantially decreased as compared with our previous surveys. Higher platelet levels, lower albumin levels, male sex, incomplete KD, IVIG resistance, and receiving initial IVIG ≤4 days or >10 days, were risk factors for CAL.

Synthesis of poly(1,5-diaminonaphthalene) microparticles with abundant amino and imino groups as strong adsorbers for heavy metal ions.

Poly(1,5-diaminonaphthalene) microparticles with abundant reactive amino and imino groups on their surface were synthesized by one-step oxidative polymerization of 1,5-diaminonaphthalene using ammonium persulfate as the oxidant. The molecular, supramolecular, and morphological structures of the microparticles were systematically characterized by IR and UV-vis spectroscopies, elementary analysis, wide-angle X-ray diffractometry, and transmission electron microscopy. The microparticles demonstrate electrical semiconductivity and high resistance to strong acid and alkali, and strong adsorption capability for lead(II), mercury(II), and silver(I) ions. The experimental conditions for adsorption of Pb(II) were optimized by varying the persulfate/monomer ratio, adsorption time, sorbent concentration, and pH value of the Pb(II) solution. The maximum adsorption capacity is 241 mg·g-1 for particles after a 24 h-exposure to a solution at an initial Pb(II) concentration of 29 mM. The adsorption data fit a Langmuir isotherm and follow a pseudo-second-order reaction kinetics. This indicates a chemical adsorption that is typical for a chelation interaction between Pb(II) and amino/imino groups on the sorbent. Graphical abstract Poly(1,5-diaminonaphthalene) microparticles with abundant functional amino and imino groups have been synthesized by one-step direct polymerization of non-volatile 1,5-diaminonaphthalene in aqueous medium for sustainable preparation of high-performance adsorbents to strongly adsorb lead(II), mercury(II), and silver(I) ions.

Epidemiologic Features of Kawasaki Disease in Shanghai From 2008 Through 2012.

BACKGROUND: This study was to investigate the epidemiologic trends of Kawasaki disease (KD) and coronary arterial lesions (CALs) in Shanghai from 2008 through 2012. METHODS: Data were collected by using the network of the KD research group established during the first survey in Shanghai to conduct the third survey, covering the period from 2008 through 2012. Clinical records of 2304 patients with acute KD were retrospectively reviewed. Epidemiologic features of KD were investigated. Univariate and multivariate analyses were performed to identify the risk factors for CAL in patients with KD. The data were compared with the previous 2 surveys covering the periods from 1998 to 2002 and 2003 to 2007, respectively. RESULTS: The average incidence of KD was 30.3 to 71.9 per 100,000 children aged 0-4 years from 2008 through 2012. Age at onset ranged from 32 days to 11.7 years (median: 2.3 years). The occurrence of KD was more common in summer and spring. A total of 365 (15.9%) cases developed CAL defined as ectasia or aneurysm. Male, age ≤ 1 year, intravenous immunoglobulin (IVIG) unresponsiveness, a smaller administrative dosage and the delayed administration of IVIG (>10 days) were independent risk factors for CAL. The occurrence of CAL seemed less frequent in patients who received IVIG within 5 days after onset of illness. CONCLUSIONS: The incidence of KD in children has increased over time, and the development of CAL decreased in the past 5 years in Shanghai. Earlier treatment with IVIG (<5 days) was associated with reduced CAL among patients with KD.

Coronary artery indexed diameter and z score regression equations in healthy Chinese Han children.

OBJECTIVE: In children with coronary disease, clinical decision should be based on detailed measurements of the coronary arteries by two-dimensional echocardiography. We aimed to establish coronary artery reference indexed diameter and z scores regression equations in a large cohort of Chinese Han children. METHODS: We measured the diameter of the proximal right (RCA), left main (LMCA), left anterior descending, and left circumflex coronary artery, and of the aortic annulus, and calculated the coronary-aorta index (coronary artery-to-aortic annulus ratio) in 506 Chinese Han children with normal hearts whose ages ranged from 1 day to 18 years. Regression analyses were performed, relating the coronary artery dimensions to body surface area (BSA). Several models were used, and the best model (yielding the maximum adjusted R(2) ) was chosen to establish a z score calculator. RESULTS: Based on cubic regression, (M) = ß0 + ß1 × BSA + ß2 × BSA(2) + ß3 × BSA(3) , the adjusted R(2) values were 0.515, 0.553, 0.505, and 0.518 for the RCA, LMCA, left anterior descending, and left circumflex coronary artery models, respectively. RCA/aortic annulus was 0.14 ± 0.02 (range, 0.07-0.24) and LMCA/AOA was 0.15 ± 0.03 (range, 0.10-0.28). CONCLUSIONS: Our results provide reference values of coronary artery z scores, regression equation, and coronary-aorta index as a quick guide to determine coronary dilation in Chinese Han children.

Interfacial chemical oxidative synthesis of multifunctional polyfluoranthene.

A novel polyfluoranthene (PFA) exhibiting strong visual fluorescence emission, a highly amplified quenching effect, and widely controllable electrical conductivity is synthesized by the direct cationic oxidative polymerization of fluoranthene in a dynamic interface between n-hexane and nitromethane containing fluoranthene and FeCl3, respectively. A full characterization of the molecular structure signifies that the PFAs have a degree of polymerization from 22-50 depending on the polymerization conditions. A polymerization mechanism at the interface of the hexane/nitromethane biphasic system is proposed. The conductivity of the PFA is tunable from 6.4 × 10-6 to 0.074 S cm-1 by doping with HCl or iodine. The conductivity can be significantly enhanced to 150 S cm-1 by heat treatment at 1100 °C in argon. A PFA-based chemosensor shows a highly selective sensitivity for Fe3+ detection which is unaffected by other common metal ions. The detection of Fe3+ likely involves the synergistic effect of well-distributed π-conjugated electrons throughout the PFA helical chains that function as both the fluorophore and the receptor units.

Efficient synthesis of oligofluoranthene nanorods with tunable functionalities.

Strongly fluorescence-emitting oligofluoranthene (OFA) nanorods are readily synthesized by a direct template-free chemical oxidative oligomerization of fluoranthene in nitromethane containing ferric chloride as an oxidant. The OFAs likely consist of five fluoranthene units containing cyclic pentamers with crystalline order and tunable electrical conductivity across 12 orders of magnitude. The OFA nanorods are heat-resistant materials and efficient precursors for macroporous carbon materials with high carbon yield in argon at 1100 °C. In particular, the optimal ring-like pentamer shows 12.2 times stronger cyan fluorescence-emission than recognized highly fluorescent fluoranthene under the same conditions, which makes the OFAs into ideal strong fluorescent emitters, tunable conductors, and high carbon-yield precursors for the preparation of sensors and carbon materials. These findings demonstrate an advance in the direct synthesis of oligomers from fused-ring aromatic hydrocarbons and provide a potential direction to optimize the synthesis and functionalities of wholly aromatic nanomaterials.

Synthesis of semiconducting polymer microparticles as solid ionophore with abundant complexing sites for long-life Pb(II) sensors.

Intrinsically electrically semiconducting microparticles of semiladder poly(m-phenylenediamine-co-2-hydroxy-5-sulfonic aniline) structures containing abundant functional groups, like -NH-, -N=, -NH2, -OH, -SO3H as complexation sites, were efficiently synthesized by chemical oxidative copolymerization of m-phenylenediamine and 2-hydroxy-5-sulfonic aniline. The obtained copolymers were found to be nonporous spherical microparticles that were able to achieve greater π-conjugated structure, smaller particle aggregate size, and stronger interaction with Pb(II) ions than poly(m-phenylenediamine) containing only -NH-, -N=, and -NH2. A potentiometric Pb(II) sensor was fabricated on the basis of the copolymer microparticles as a crucial solid ionophore component within plasticized PVC. The sensor exhibited a Nernstian response to Pb(II) ions over a wide concentration range, together with a fast response, a wide pH range capability, a long lifetime of up to 5 months, and good selectivity over a wide variety of other ions and redox species. The process for synthesizing the microparticles and fabricating the Pb(II)-sensor can be facilely scaled-up for use in the straightforward long-term online monitoring of Pb(II) ions in heavily polluted wastewaters. This study develops an understanding of the facile synthesis of conducting microparticles bearing many functional groups and their structures governing the potentiometric susceptibility toward interaction between Pb(II) ions and the microparticles for fabricating robust long-lived Pb(II)-sensor, signifying the potential suitability of such novel materials for inexpensive sensitive detection of Pb(II) ions.

Combinatorial screening of potentiometric Pb(II) sensors from polysulfoaminoanthraquinone solid ionophore.

A potentiometric Pb(II)-selective sensor was fabricated by a combinatorial screening of electrically conducting polysulfoaminoanthraquinone (PSA) nanoparticles as a solid ionophore, ion exchangers (oleic acid (OA) and NaTPB), plasticizers in a polyvinyl chloride (PVC) matrix, membrane thickness, inner filling ion species, and concentration. The membrane sensor with the composition of PSA/PVC/DOP (dioctyl phthalate)/OA (1.0:33:61:5.0) exhibited the best performance, including a slope of 29.3 mV decade(-1) in the concentration range 10(-6.3)-10(-1.6) M, detection limit of 1.6 × 10(-7) M, response time of 16 s, lifetime of five months, and good response reversibility. The proposed sensor has demonstrated good selectivity for Pb(II) over other monovalent, divalent and trivalent interfering ions, and could be used in a pH range of 3.62-5.22. The Pb(II) sensor has been successfully applied for the determination of Pb(II) concentration in real-world samples and also as an indicator electrode for potentiometric titration of lead ions.

[Clinical characteristics and prognosis of infantile-onset glycogen storage disease type II in 16 Chinese patients].

OBJECTIVE: To explore the clinical characteristics and prognosis of infantile-onset glycogen storage disease type II (GSDII) in Chinese patients. METHODS: Sixteen children diagnosed as infantile-onset GSDII in Shanghai Children's Medical Center during Jan 2005 to Dec 2012 were recruited. Their disease history, presenting symptom, physical signs, biochemical tests and examinations of electrocardiogram and echocardiography were analyzed retrospectively. Follow-up data on motor development and survival were also collected and analyzed retrospectively. RESULTS: 16 cases were diagnosed as infantile-onset GSDII (10 males, 6 females), in which the peripheral blood levels of acidic α-glucosidase were remarkably low or completely absent. All of them were complicated with cardiac hypertrophy and left ventricular mass index was 161-616 g/m(2). Severe muscular weakness, hypotonia and development lag were found in all during the follow-up. Creatine kinase was detected in 15 patients and its level became significantly elevated in 14 of them. Alanine aminotransferase and aspartate aminotransferase were detected in 15 patients and their levels became significantly elevated in all of them. The median age was 3.6 (2.0-6.8) months at symptom onset and 6.5 (3.8-9.3) months at diagnosis. And 14 of them died during the follow-up and the median age at death was 9.0 (4.7-18.7) months. CONCLUSIONS: As a fatal disease, infantile-onset GSDIIhas the prominent clinical manifestations of progressive cardiac hypertrophy and muscular weakness or hypotonia. The clinical features and nature history of Chinese patients are similar as those reported in other countries. Detection of acidic α-glucosidase activity in peripheral blood is an effective way of screening for infantile-onset GSDII.

Lead-ion potentiometric sensor based on electrically conducting microparticles of sulfonic phenylenediamine copolymer.

A potentiometric sensor to detect lead ions using newly synthesized conducting copolymer microparticles as an ionophore in self-supporting poly(vinyl chloride) membrane matrix plasticized with dioctyl phthalate was developed. The copolymer microparticles containing many ligating functional groups including amino, imino and sulfonic groups were synthesized by a chemical oxidative copolymerization of m-phenylenediamine (mPD) and p-sulfonic-m-phenylenediamine (SPD) in pure water. Due to the presence of -NH-, -N=, -NH2, and -SO3H ligating groups on the microparticles, a linear Nernstian response is obtained within a Pb(II) activity range from 1.00 × 10(-6) M to 1.00 × 10(-3) M. The Pb(II)-sensor containing the mPD/SPD (95/5) copolymer microparticles with the maximal electrical conductivity demonstrates a superior detection limit down to 1.26 × 10(-7) M, short response time to 14 s, and long lifetime of up to 4 months. The Pb(II)-sensor also exhibits a selective response to Pb(II) over 9 other metal ions and a pH independent plateau between 2.7 and 5.0. These advantages could make for a robust sensor performing credible analysis of Pb(II) concentration in real-world samples at trace levels.

[Transcatheter closure of patent ductus arteriosus with the new Amplatzer Duct OccluderII].

OBJECTIVE: To evaluate the indications, methodology and results of the transcatheter closure of patent ductus arteriosus (PDA) with the new Amplatzer Duct Occluder II (ADO-II). METHOD: Totally 51 patients underwent transcatheter closure of PDA with the new ADO-II. The devices were delivered by 4F or 5F sheath through arterial or venous side respectively. The descending aorta angiography and transthoracic echocardiography was performed to evaluate the device position, residual shunt and complications caused by the device during and after implantation. RESULT: Forty-nine patients had successful transcatheter closure of the PDA without significant residual shunts and artery obstruction during the short-term follow-up. One patient received the ADO-II dislodgment and first generation ADO re-implantation for the obvious descending aortal obstruction caused by ADO-II. Another patient had the ADO-II dislodgment and left pulmonary artery shaping surgery, because the ADO-II implantation led to obstruction of the left pulmonary artery. Both the obstructions in these two patients were ameliorated afterwards. CONCLUSION: The transcatheter closure using the ADO-II is safe and effective for the non-window type PDA with a small size.

Interfacial synthesis and functionality of self-stabilized polydiaminonaphthalene nanoparticles.

A simple and effective template-free synthesis method for nanosized conducting polymers with self-stability and functionality is a main challenge. Herein, a strategy is reported for the facile synthesis of poly(1,5-diaminonaphthalene) nanospherical particles by an interfacial miniemulsion oxidative polymerization of 1,5-diaminonaphthalene at mobile microinterfaces between a stirred biphase without external emulsifiers. The size of the nanospheres was carefully optimized by controlling the polymerization conditions. Formation and self-stabilization mechanisms of the nanoparticles are proposed. The constantly movable and refreshed microinterface is a key to successful synthesis of the nanospheres, for significantly suppressing secondary growth leading to agglomerated particles because vigorous stirring makes as-formed self-stabilized nanospheres instantly leave the microinterfaces. The resulting nanospheres possess several advantages: clean surface, self-stability, redispersibility, semiconductivity, electroactivity, and fluorescence emission. The fluorescence emission can be quenched by specific quenchers, thus enabling low-cost, high-performance chemosensors to be obtained for the sensitive detection of Zn(II) ions in a wide linear concentration range of more than five orders of magnitude with a superior detection limit down to 1 nM.

Ultrasensitive Pb(II) potentiometric sensor based on copolyaniline nanoparticles in a plasticizer-free membrane with a long lifetime.

A newly designed Pb(II) potentiometric sensor based on intrinsically conducting nanoparticles of solid poly(aniline-co-2-hydroxy-5-sulfonic aniline) possessing many ligating functional groups like -NH-, -N=, -OH, -SO(3)H, -NH(2) as ionophores in plasticizer-free vinyl resin solid membranes has been fabricated. A linear Nernstian response is obtained within a wide Pb(II) activity range from 1.0 × 10(-3) to 1.0 × 10(-10) M with a detection limit as low as 2.2 × 10(-11) M. The pH independent plateau ranges between 3.5 and 7.0. After 15 months' usage, the sensor maintains 95% performance parameters. Its anti-interference ability to Cu(II), Cd(II), Ag(I), and Hg(II) is much stronger than other sensors with a detection limit at (sub)nanomolar level. Electrochemical impedance spectroscopy reveals that the solid sensing membrane has a diffusion coefficient of around 5 × 10(-14) to 1 × 10(-13) cm(2) s(-1). The much lower diffusion coefficient for Pb(II) is highly beneficial for the elimination of Pb(II) flux across the membrane. The wide detection concentration range, low detection limit, high selectivity, extensive pH window, and long lifetime make for a robust sensor giving reliable measurement of Pb(II) content with potential application in real-world samples at trace levels.

[Efficacy of the transcatheter closure of perimembranous and muscular ventricular septal defects with the Amplatzer duct occluder II].

OBJECTIVES: To evaluate the feasibility and efficacy of transcatheter closure of perimembranous ventricular septal defects (pmVSD) with aneurysmatic formation and muscular ventricular septal defects (mVSD) with Amplatzer duct occluder II. METHODS: This retrospective analysis included 48 cases received transcatheter closure of pmVSD aneurysmatic formation or mVSD from February 2011 to March 2012 in our hospital (42 pmVSD with aneurysmatic formation and 6 mVSD). Median age was 5.2 years (range: 1.8 - 15 years), and median weight was 20.2 kg (range: 12 - 44 kg). Amplatzer duct occluder II was selected depending on the condition of ventricular septal defect. The device was implanted by antegrade or retrograde approach. Complications such as residual shunt, valvular regurgitation and arrhythmia were evaluated by echocardiography or angiography. Median follow-up was 9.5 months (range: 1 - 13 months). RESULTS: The mean ratio of pulmonary (Qp) to systemic (Qs) blood flow was 1.35 ± 0.15 before transcatheter closure. The diameter of exit hole of ventricular septal defects was (2.46 ± 0.53) mm measured by transthoracic echocardiography, and (2.35 ± 0.40) mm by angiography. Successful implantation of the device was achieved in 46 patients (96%) and unsuccessful in two cases due to acute aortic insufficiency. Forty-two (92%) patients were closed successfully, and trivial residual leak was evidenced in four patients and remained unchanged during follow-up. One patient with mVSD still had trivial residual shunt at 6 months post procedure. New trivial tricuspid insufficiency was observed in 1 patient (2.1%) during follow-up. Two patients developed procedural related left anterior fascicular block and remained unchanged during follow-up. CONCLUSIONS: pmVSD with aneurysm and mVSD could be successfully treated with Amplatzer duct occluder II. However, the long waist and large disc of the device could interfere with tricuspid valve function and cause tricuspid insufficiency.

[Clinical presentation and therapeutic outcomes of carnitine deficiency-induced cardiomyopathy].

OBJECTIVE: Carnitine deficiency has been associated with progressive cardiomyopathy due to compromised energy metabolism. The objective of this study was to investigate clinical features of carnitine deficiency-induced cardiomyopathy and the therapeutic efficacy of L-carnitine administration. METHOD: Between January 2010 and December 2011, filter-paper blood spots were collected from 75 children with cardiomyopathy. Free carnitine and acylcarnitine profiles were measured for each individual by tandem mass spectrometry (MS/MS). For those in whom carnitine deficiency was demonstrated, treatment was begun with L-carnitine at a dose of 150 - 250 mg/(kg·d). Clinical evaluation, including physical examination, electrocardiography, chest x-ray, echocardiography and tandem mass spectrometry, was performed before therapy and during follow-up. RESULT: Of 75 cardiomyopathy patients, the diagnosis of carnitine deficiency was confirmed in 6 patients, which included 1 boy and 5 girls. Their age ranged from 0.75 to 6 years. Free carnitine content was (1.55 ± 0.61) µmol/L (reference range 10 - 60 µmol/L). Left ventricular end-diastolic diameter (LVDd) was (5.04 ± 0.66) cm and left ventricular ejection fraction (LVEF) was (38.5 ± 10.5)%. After 10 - 30 d therapy of L-carnitine, free carnitine content rose to (30.59 ± 15.02) µmol/L (t = 4.79, P < 0.01). LVDd decreased to (4.42 ± 0.67) cm (t = 4.28, P < 0.01) and LVEF increased to (49.1 ± 7.6)% (t = 6.59, P < 0.01). All patients received follow-up evaluations beyond 6 months of treatment. Clinical improvement was dramatic. LVEF returned to normal completely in all the 6 patients. LVDd decreased further in all the 6 patients and returned to normal levels in 3 patients. No clinical signs or symptoms were present in any of the 6 patients. The only complications of therapy had been intermittent diarrhea in 1 patient. CONCLUSION: Tandem mass spectrometry is helpful to diagnose carnitine deficiency and should be performed in all children with cardiomyopathy. L-carnitine has a good therapeutic effect on carnitine deficiency-induced cardiomyopathy.

Lead ion-selective electrodes based on polyphenylenediamine as unique solid ionophores.

A novel membrane electrode for Pb(II) ion detection based on semi-conducting poly(m-phenylenediamine) microparticles as a unique solid ionophore was fabricated. The electrode exhibited significantly enhanced response towards Pb(II) over the concentration range from 3.16×10(-6) to 0.0316 M at pH 3.0-5.0 with a low detection limit of 6.31×10(-7) M, a high sensitivity displaying a near-Nernstian slope of 29.8 mV decade(-1) for Pb(II). The electrode showed a long lifetime of 5 months and a short response time of 14s. A systematical investigation on the effect of anion excluder and various foreign ions on the selectivity of the electrode by a fixed interference method suggests that all other metal ions hardly ever interfere with the determination of Pb(II) except high concentration Hg(II). The electrode was successfully used as an indicator electrode in the potentiometric titration of Pb(II) with EDTA. Furthermore, the electrode has been used to satisfactorily analyze four types of real-world samples like spiked human urine, spiked tap water, and river water containing interfering ions like Na(I), Ca(II), Mg(II), Zn(II), Pd(II), Fe(III), K(I), Cu(II) and Hg(II) up to 8.04×10(-4) M, demonstrating fast response, high selectivity, good recovery (96.6-121.4%), good repeatability (RSD 0.31-6.45%), and small relative error (5.0%).