Evaluation of the dual-process approach for in-situ groundwater arsenic removal.

While the worldwide distribution of geogenic arsenic (As)-affected groundwater is highly overlapped with the areas with abundant groundwater, utilization of As-contained groundwater is an inevitable compromise in those areas where surface water is not enough for irrigation. Since the occurrence of As in groundwater is often accompanied by high iron (Fe) contents, the facilitation of As and Fe precipitation without adding additional oxidizers and adsorbents is considered an environmental-friendly approach to removing As in groundwater. In the present study, the oxidation/filtration dual-process with sprinkling height of 25 cm and 120 kg filter media efficiently increased the dissolved oxygen (DO) concentration (0.36-1.52 mg/L) and oxidation-reduction potential (ORP) (24-63 mV), which facilitated the formation of Fe oxides and As co-precipitation. The correlation of As removal efficiencies with their respective flow rates indicated that a decrease in groundwater Fe and an increase of Fe in sands and gravels filters as the flow rate increased evidenced the rapid oxidation of Fe to form the Fe hydroxides. In a 40-hour continuous aeration/filtration operation, As and Fe concentrations in groundwater were reduced by 79.5% and 64.88% within 40 hrs, respectively. The ease of filter replacement and cost-effectiveness in operation can be the major attractions and innovations for future field practices.

Pharmacologic interventions for Kawasaki disease in children: A network meta-analysis of 56 randomized controlled trials.

BACKGROUND: Although the current consensus recommends a standard treatment of high-dose intravenous immunoglobulin with high-dose aspirin to manage Kawasaki disease (KD), the use of different adjunctive therapies remains controversial. The aim of the current network meta-analysis (NMA) was to compare the efficacy and tolerability of different existing interventions for the initial and refractory stages of KD. METHODS: An NMA of randomised controlled trials (RCTs) was conducted using the frequentist model applied after electronic searches in PubMed, Embase, ScienceDirect, ProQuest, ClinicalTrials.gov, ClinicalKey, Cochrane CENTRAL, and Web of Science. The main outcomes were reduced fever duration/diminished severity of fever subsided. The initial stage of KD was defined as the first stage to treat patients with KD; the refractory stage of KD represents KD patients who failed to respond to standard KD treatment. The cut-off points for intravenous immunoglobulin (IVIG) were low (100-400 mg), medium (1 g), and high (at least 2 g). FINDINGS: A total of fifty-six RCTs with 6486 participants were included. NMA demonstrated that the medium-dosage IVIG + aspirin + infliximab [mean difference=-1.76 days (95% confidence intervals (95% CIs): -3.65 to 0.13 days) compared to high-dosage IVIG + aspirin] exhibited the shortest fever duration; likewise, the medium-dosage IVIG + aspirin + infliximab [odds ratio (OR)=0.50, 95% CIs: 0.18-1.37 compared to high-dosage IVIG + aspirin] exhibited the smallest incidence of coronary artery lesion (CAL) in the initial-stage KD. In the refractory-stage KD, the high-dosage IVIG + pulse steroid therapy (OR=0.04, 95% CIs: 0.00-0.43 compared to the high-dosage IVIG only) had the best rate of decline of fever; likewise, the high-dosage IVIG + ciclosporin [OR=0.05 (95% CIs: 0.00-1.21) compared to the high-dosage IVIG only] exhibited the smallest incidence of CAL. Infliximab significantly improved resolution compared to the high-dosage IVIG only group (OR=0.20, 95%CIs: 0.07-0.62) in refractory-stage KD. INTERPRETATION: The NMA demonstrated that the combination therapy with the standard therapy of IVIG and aspirin might have an additional effect on shortening the duration of fever and lowering the CAL incidence rate in patients with acute KD. Moreover, the combination therapy with high-dose IVIG and pulse steroid therapy or cyclosporine therapy might have an additional effect on improving the rate of decline of fever and lowering the incidence rate of CAL in children with refractory KD. Because some of the findings of this NMA should be considered hypothesis-generating rather than confirmatory, further evidence from de novo randomised trials is needed to support our results. FUNDING: None.

Increased Risk of Asthma and Allergic Rhinitis in Patients With a Past History of Kawasaki Disease: A Systematic Review and Meta-Analyses.

Background: Allergic diseases are frequently observed in children with Kawasaki disease (KD). However, the evidence supporting the association between KD and allergies has been conflicting. The objective of the current study is to examine the association between KD and allergic diseases. Methods: We conducted an electronic search using PubMed, Embase, and the Cochrane through 24 July 2021. The inclusion criteria consisted of studies that examined the prevalence of allergic diseases in children with a previous diagnosis of KD and in a comparison group. We pooled studies by using a random effects model. The effects of KD on the subsequent risk of allergic diseases were expressed as odds ratio (OR) with 95% confidence intervals (CI). Results: We included a total of four studies that assessed the effect of KD on asthma, allergic rhinitis, and atopic dermatitis vs. non-KD children (KD individuals for asthma, four studies, n = 8,474; allergic rhinitis, four studies, n = 8,474; atopic dermatitis, three studies, n = 8,330). The overall prevalence of asthma, allergic rhinitis, and atopic dermatitis was 9.12, 27.63, and 6.55% among patients with previous KD. The meta-analysis showed a significantly increased risk of asthma (OR:1.437, CI: 1.067-1.937) and allergic rhinitis (OR: 1.726, CI: 1.291-2.307) in patients with KD, compared with the control groups. However, patients with KD did not have a significantly different level of risk of atopic dermatitis (OR: 1.243, 95% CI: 0.857-1.802). Conclusion: This meta-analysis supports that individuals with KD are more likely to have asthma and allergic rhinitis compared to controls.

Enhancement of PVDF Sensing Characteristics by Retooling the Near-Field Direct-Write Electrospinning System.

This research aimed to develop a direct-write near-field electrospinning system (DW-NFES) with three-axis positioning of controllable speed, torque and position to produce sizable and high-quality piezoelectric fibers for sensing purposes. Sensor devices with high electrical response signals were developed and tested. To achieve DW-NFES purpose, a servo motor controller was designed to develop a high response rate, accurate positioning, and stable mobile device through the calculation of bandwidth and system time delay. With this retooled system of DW-NFES, controllable and uniform size fibers in terms of diameters, stretching force, and interspaces can be obtained. Sensor devices can be made selectively without a complicated lithography process. The characteristics of this DW-NFES platform were featured by high response rate, accurate positioning, and stable movement to make fibers with high piezoelectric property. In this study, polyvinylidene fluoride (PVDF) was used to explore and enhance their sensing quality through the platform. The parametric study of the process factors on piezoelectric sensing signals mainly included the concentration of electrospinning PVDF solution, high voltage electric field, and collection speed. Finally, the surface morphology and piezoelectric properties of the as-electrospun PVDF fibers were examined by scanning electron microscopy (SEM) and characterized by electrical response measurement techniques. The results showed that the fiber spinning speed of the DW-NFES system could be increased to ~125 from ~20 mm/s and the accuracy precision was improved to ~1 from ~50 µm, compared to conventional step motor system. The fiber diameter reached ~10 µm, and the electrospinning pitch reached to as small as ~10 µm. The piezoelectric output voltage of the electrospun fibers was increased ~28.6% from ~97.2 to ~125 mV; the current was increased ~27.6% from ~163 to ~208 nA, suggesting that the piezoelectric signals can be enhanced significantly by using this retooled system. Finally, an external control module (Arduino-MAGE) was introduced to control the PVDF piezoelectric fiber sensors integrated as a sensing array. The behavior of long-term sedentary patients can be successfully detected by this module system to prevent the patients from the bedsores.

Clinical study of children with cryofibrinogenemia: a retrospective study from a single center.

BACKGROUND: This study aimed to evaluate the demographic, clinical features, laboratory data, pathology and other survey in pediatric patients with cryofibrinogenemia. METHODS: A 12-year retrospective chart review identified eight pediatric patients at Mackay Memorial Hospital, Taipei, Taiwan. RESULTS: The female-to-male ratio was 3:1. The mean age at symptom onset and of diagnosis was 10.3 ± 4.6 years and 12.3 ± 4 years, respectively. One child (12.5%) had primary cryofibrinogenemia. The common symptoms were purpura, arthralgia, and muscle weakness (100%). On laboratory examination, cryofibrinogen was positive in all patients. All patients had increased anti-thrombin III while 87.5% and 62.5% had abnormal protein S and protein C, respectively. All eight also complained of neurologic symptoms. One had vertebral artery narrowing, two showed increased T2-weighted signal intensity on the thalamus or white matter, and one had acute hemorrhagic encephalomyelitis on brain magnetic resonance imaging. CONCLUSIONS: This study reports on the presentations of cryofibrinogenemia, which is rare in children. Most cases are associated with autoimmune disease and have severe and complex presentations. Central nervous system involvement is common.

Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database.

BACKGROUND: The recurrence rate of Henoch-Schönlein purpura (HSP) is 2.7%-30%, with varied average intervals between the first and second episodes. Few studies have explored the incidence and risk factors for recurrent HSP. METHODS: We used a 16-year nationwide database to analyze the incidence of recurrent HSP. Patients with HSP were identified, and risk factors for recurrent HSP were explored. Kaplan-Meier and Cox regression model analyses were performed, and covariates were adjusted in the multivariate model. RESULTS: From January 1, 1997 to December 31, 2012, among 2,886,836 individuals in the National Health Insurance Research Database, 1002 HSP patients aged < 18 years were identified. Among them, 164 had ≥2 HSP episodes (recurrence rate, 16.4%; incidence of recurrent HSP, 7.05 per 100 person-years); 83.6% patients with one HSP episode remained free of secondary HSP. The average time intervals between the first and second and second and third HSP episodes were 9.2 and 6.4 months, respectively. After adjusting for demographic parameters, comorbidities, and socioeconomic status, recurrent HSP was found to occur more frequently in patients who had renal involvement (adjusted hazard ratio, 2.41; 95% confidence interval [CI], 1.64-3.54; p < 0.001), were receiving steroid therapy for > 10 days (adjusted hazard ratio, 8.13; 95%CI, 2.51-26.36; p < 0.001), and had allergic rhinitis (adjusted hazard ratio, 1.63; 95%CI, 1.06-2.50; p = 0.026). CONCLUSIONS: The annual incidence of recurrent HSP was low. However, children who had underlying allergic rhinitis, presented with renal involvement, and received steroid treatment for > 10 days should be notified regarding the possibility of recurrence.

The risk of hospitalization for respiratory tract infection (RTI) in children who are treated with high-dose IVIG in Kawasaki Disease: a nationwide population-based matched cohort study.

BACKGROUND: Kawasaki disease (KD) is an immune-mediated systemic vasculitis, and infection plays an important role in the pathophysiology of KD. The susceptibility to infectious disease in patients with KD remains largely unclear. This study aimed to investigate the risk of respiratory tract infection (RTI)-related hospitalizations in children with KD. METHODS: Data from the Taiwanese National Health Insurance Research Database was analyzed. We excluded patients with history of congenital abnormality, allergic diseases, or hospitalization history. Children with KD were selected as KD group and age- and sex-matched non-KD patients were selected as control group with 1:4 ratio. Both cohorts were tracked for one year to investigate the incidences of RTI-related hospitalizations. Cox regression hazard model was used to adjust for confounding factors and calculate the adjusted hazard ratio (aHR). RESULTS: Between January 1996 and December 2012, 4,973 patients with KD were identified as the KD group and 19,683 patients were enrolled as the control group. An obviously reduced risk of RTI-related hospitalizations was observed in KD patients (aHR: 0.75, 95% CI [0.66-0.85]). The decreased risk persisted through the first six-months follow-up period with a peak protection in 3-6 months (aHR: 0.49, 95% CI [0.37-0.64]). CONCLUSIONS: KD patients had approximately half reduction of risk for RTI-related hospitalizations. The protective effects persisted for at least six months. Further studies are warranted to elucidate the entire mechanism and investigate the influences of intravenous immunoglobulin.

Exhaled nitric oxide helps discriminating asthmatic children with and without positive specific IgE to aeroallergens.

BACKGROUND: Aeroallergen sensitization may predict higher fractional exhaled nitric oxide (FeNO) levels. OBJECTIVE: We evaluate cut-off values of FeNO in asthmatic children with and without positive specific immunoglobulin E (IgE) to at least one of 5 aeroallergens (Dermatophagoides pteronyssinus, Dermatophagoides farinae, cat, dog, and cockroach). METHODS: 564 patients with asthma and allergic rhinitis (AR) aged 5 to 18 years were enrolled into two groups. Sensitized group included 378 children with positive IgE to at least one of 5 inhaled allergens. Non-sensitized group included 186 children. Pulmonary function tests, FeNO, eosinophil counts, and IgE levels were examined. Patients were divided into preschool age (5~6 years old), elementary school children (7~11 years old) and adolescents (12~18 years old). RESULTS: In preschool children, FeNO≥15.5 ppb differentiates between non-sensitized and sensitized groups. (sensitivity 54.3%; specificity 87.5%; positive predictive value (PPV) 86.2%; negative predictive value (NPV) 57.1%; area under receiver operating characteristic curve (AUC) 0.72) Among elementary school children, the cut-off value of FeNO≥19.5 ppb showed sensitivity 66.4%; specificity 85.8%; PPV 90.5%; NPV 55.7%; AUC 0.81. In adolescents, FeNO≥27.5 ppb showed sensitivity 60.2%; specificity 85.4%; PPV 91.2%; NPV 46.1%; AUC 0.76. CONCLUSION: In asthmatic children, aeroallergen sensitization appears to contribute to higher FeNO levels than those not sensitized. Cut-off values of FeNO which well discriminate asthmatic children with and without aeroallergen sensitization should be chose according to different ages.

Isotopic evidence of nitrogen sources and nitrogen transformation in arsenic-contaminated groundwater.

High concentrations of naturally occurring arsenic (As) are typically found in young alluvial and deltaic deposits, and high concentrations of ammonium (NH4+) and nitrate (NO3-) are often present in groundwater affected by anthropogenic activities. In this study, on the basis of physicochemical characteristics of groundwater and the nitrogen and oxygen isotope composition of NO3-, it was inferred that the main sources of NO3- in the proximal fan of the Choushui River alluvial fan are likely to be ammonium fertilizers, manure, and septic waste; that in the mid-fan and the distal fan, the possible sources are nitrate fertilizers and marine nitrate. In the proximal fan, the oxidative state obviously promotes microbial nitrification. Denitrification occurs from the upstream region to the downstream region of the Choushui River, and therefore, the decrease in NO3- concentration along streams connecting the Choushui River to the ocean appears plausible. High DO concentrations and relatively low values of δ18ONO3 in the deeper aquifer of the proximal fan may be attributed to unconfined granular nature and groundwater pumping by agricultural activities. In the mid-fan, NO3- assimilation is the dominant response to NO3- attenuation, and denitrification is insignificant; however, high concentrations of As, NH4+ and Fe and depletion of δ15NNO3 imply the occurrence of feammox process. By contrast, denitrification evidently occurs in the distal fan, through assimilation, mineralization, and dissimilatory NO3- reduction to NH4+, resulting in depletion of NO3- and increase in NH4+ in groundwater. Feammox in the mid-fan and denitrification in the distal fan may be the main processes leading to the release of As from As-bearing Fe oxyhydroxides into groundwater.

Health risk assessment of groundwater arsenic pollution in southern Taiwan.

Residents of the Pingtung Plain, Taiwan, use groundwater for drinking. However, monitoring results showed that a considerable portion of groundwater has an As concentration higher than the safe drinking water regulation of 10 µg/L. Considering residents of the Pingtung Plain continue to use groundwater for drinking, this study attempted to evaluate the exposure and health risk from drinking groundwater. The health risk from drinking groundwater was evaluated based on the hazard quotient (HQ) and target risk (TR) established by the US Environmental Protection Agency. The results showed that the 95th percentile of HQ exceeded 1 and TR was above the safe value of threshold value of 10-6. To illustrate significant variability of the drinking water consumption rate and body weight of each individual, health risk assessments were also performed using a spectrum of daily water intake rate and body weight to reasonably and conservatively assess the exposure and health risk for the specific subgroups of population of the Pingtung Plain. The assessment results showed that 0.01-7.50 % of the population's HQ levels are higher than 1 and as much as 77.7-93.3 % of the population being in high cancer risk category and having a TR value >10-6. The TR estimation results implied that groundwater use for drinking purpose places people at risk of As exposure. The government must make great efforts to provide safe drinking water for residents of the Pingtung Plain.

Characterization and risk assessment of PAH-contaminated river sediment by using advanced multivariate methods.

This study applied advanced multivariate methods and risk assessment to evaluate the characteristics of polycyclic aromatic hydrocarbons (PAHs) in the sediment of the severely polluted Erjen River in Taiwan. High-molecular-weight PAHs (HPAHs) dominated in the rainy season. The ecological risk of PAHs in the sediment was low, whereas the total health risk through ingestion and dermal contact was considerably high. The SOM (self-organizing map) analysis clustered the datasets of PAH-contaminated sediment into five groups with similar concentration levels. Factor analysis identified major factors, namely coal combustion, traffic, petrogenic, and petrochemical industry factors, accounting for 88.67% of the variance in the original datasets. The major tributary and the downstream of the river were identified as PAH-contamination hotspots. The PMF (positive matrix factorization) was combined with toxicity assessment to estimate the possible apportionment of sources and the associated toxicity. Spills of petroleum-related products, vehicle exhaust, coal combustion, and exhaust from a petrochemical industry complex constituted respectively 12%, 6%, 74%, and 86% of PAHs in the sediment, but contributed respectively 7%, 15%, 22%, and 56% of toxicity posed by PAHs in the sediment. To improve the sediment quality, best management practices should be adopted to eliminate nonpoint sources of PAHs flushed by storm water into the major tributary and the downstream of the Erjen River. The proposed methodologies and results provide useful information on remediating river PAH-contaminated sediment and may be applicable to other basins with similar properties that are experiencing resembled river environmental issues.

Pachydermodactyly: Three new cases in Taiwan.

Pachydermodactyly (PDD), Greek for thick-skin-finger, is an infrequently recognized benign disorder characterized by painless fusiform swelling of the soft tissues around the proximal interphalangeal joints of the hands. Histopathologic features include increased dermal accumulation of collagen fibers. Young males are predominantly affected. PDD is quite rare with approximately 90 cumulative cases reported worldwide. We report three new cases of PDD in Taiwan, including two female patients. Except for patient 1 having the habit of cracking the knuckles, and patient 2 having a history of patent ductus ateriosus post catheterization, the histories of all three patients were unremarkable. X-ray of bilateral hands revealed no abnormal finding except for soft tissue swelling around proximal interphalangeal joints. Laboratory examinations all showed negative results. No local or systemic treatment was given to these patients to treat PDD, with the exception of non-steroidal anti-inflammatory drugs prescribed to one patient for a short period. Skin care with local irritation avoidance was explained to all three patients. The long-term outcome of PDD was benign.

Unusual manifestations of Kawasaki disease with retropharyngeal edema and shock syndrome in a Taiwanese child.

We report a 3-year-old girl with Kawasaki disease who presented with retropharyngeal edema and shock syndrome. This is the first reported case in Taiwan. The patient initially presented with fever, cough, and pyuria followed by rapidly progressive enlarged bilateral cervical lymphadenopathy. On the third day of the fever, computed tomography for airway compression sign found widening of the retropharyngeal space mimicking a retropharyngeal abscess. Later, an endotracheal tube was inserted for respiratory distress. A skin rash over her trunk was also noted. On the fifth day of the fever, the clinical course progressed to hypotension and shock syndrome. Because of more swelling of bilateral neck lymph nodes, computed tomography was arranged again and revealed partial resolution of the edematous changes in the retropharyngeal space. Edema of the hands and feet, bilateral bulbar conjunctivitis, and fissured lips were subsequently found. The diagnosis of Kawasaki disease was confirmed on the eighth day of fever. There was no evidence of bacterial infection. She was administered intravenous immunoglobulin (2 mg/kg) and high dose aspirin (100 mg/kg/day). One day later, the fever subsided, and her blood pressure gradually became stable. Heart echocardiography on the Day 13 revealed dilated left coronary artery and mitral regurgitation. Follow-up echocardiography six months later showed normal coronary arteries. To date, the patient has not experienced any complications. This case illustrates that retropharyngeal edema and shock syndrome can be present in the same clinical course of Kawasaki disease. Clinicians and those who work in intensive care units should be aware of unusual presentations of Kawasaki disease to decrease rates of cardiovascular complications.

Cystic fibrosis: experience in one institution.

Cystic fibrosis (CF) is one of the most common autosomal recessive inherited disorders among Caucasians. Comparatively, it is considered to be a rare disease among Asians. To date, only a few cases of Taiwanese CF have been published. We report four CF cases from three families. Case 1 was the first report of CF associated with a homozygosity for the CF transmembrane conductance regulator gene (CFTR gene) mutation 3849+10kb C->T in a Taiwanese patient. Cases 2 and 3 had heterozygous c. 1898+5 G->T and heterozygous p. I1023R (novel mutation) for the CFTR gene mutation. Case 4 was homozygous for the CFTR gene mutation R553X being reported in 2005 and complicated with cor pulmonale. These four patients had received 300 mg bid aerosolized tobramycin treatment every other month.

Clinical manifestations and BTK gene defect in 4 unrelated Taiwanese families with Bruton's disease.

BACKGROUND AND OBJECTIVE: X-linked agammaglobulinemia (XLA, also called Bruton's disease) is is an X-linked recessive disorder characterized by recurrent bacterial infections, usually occurring in the first few years of life. Here, we report the results of a BTK gene mutation screening study that was performed in Taiwanese families with the BTK gene defect to further understand the inheritance patterns of XLA patients in Taiwan and to avoid new cases of XLA within families. MATERIALS AND METHODS: In this study, 52 members of 4 unrelated Taiwanese families with the BTK gene defect were enrolled. We studied the immunologic reports of 6 symptomatic living male patients with confirmed BTK gene defects and correlated the findings with their clinical symptoms. The genomic DNA of the subjects was subjected to direct sequencing mutation analysis. RESULTS: We screened 52 members of 4 unrelated Taiwanese families with the BTK gene defect for BTK gene mutation and found that there were 6 symptomatic living patients with a confirmed defect, 7 symptomatic deceased patients highly suspected to have had the defect and 11 asymptomatic female carriers. CONCLUSIONS: This is the first report in a series of the thorough screening for the BTK mutation and its carrier status in 4 unrelated Taiwanese families. One pedigree of our study comprises 4 generations. A complete BTK gene mutation study for the patient's family members is strongly suggested.

Biogeochemical cycling of arsenic in coastal salinized aquifers: Evidence from sulfur isotope study.

Arsenic (As) contamination of groundwater, accompanied by critical salinization, occurs in the southwestern coastal area of Taiwan. Statistical analyses and geochemical calculations indicate that a possible source of aqueous arsenic is the reductive dissolution of As-bearing iron oxyhydroxides. There are few reports of the influence of sulfate-sulfide redox cycling on arsenic mobility in brackish groundwater. We evaluated the contribution of sulfate reduction and sulfide re-oxidation on As enrichment using δ(34)S([SO(4)]) and δ(18)O([SO(4)]) sulfur isotopic analyses of groundwater. Fifty-three groundwater samples were divided into groups of high-As content and salinized (Type A), low-As and non-salinized (Type B), and high-As and non-salinized (Type C) groundwaters, based on hydro-geochemical analysis. The relatively high enrichment of (34)S([SO(4)]) and (18)O([SO(4)]) present in Type A, caused by microbial-mediated reduction of sulfate, and high (18)O enrichment factor (ε([SO(4)-H(2)O])), suggests that sulfur disproportionation is an important process during the reductive dissolution of As-containing iron oxyhydroxides. Limited co-precipitation of ion-sulfide increased the rate of As liberation under anaerobic conditions. In contrast to this, Type B and Type C groundwater samples showed high δ(18)O([SO(4)]) and low δ(34)S([SO(4)]) values under mildly reducing conditions. Base on (18)O mass balance calculations, the oxide sources of sulfate are from infiltrated atmospheric O(2), caused by additional recharge of dissolved oxygen and sulfide re-oxidation. The anthropogenic influence of extensive pumping also promotes atmospheric oxygen entry into aquifers, altering redox conditions, and increasing the rate of As release into groundwater.

Type I hereditary angioedema in Taiwan -- clinical, biological features and genetic study.

BACKGROUND: Hereditary angioedema (HAE) is a rare, autosomal dominant inherited disease which is caused by a genetic deficiency of C1 esterase inhibitor (C1 INH). There have only been a few case reports in Taiwan to date. OBJECTIVE: To describe the clinical features of type I HAE in Taiwanese patients. METHODS: Three unrelated Taiwanese families with type I HAE are reported, and one case of a family from a review of PubMed was reviewed. Clinical manifestations, diagnostic examinations, management and genetic studies were analyzed. RESULTS: Including this report, 19 patients had low C1 INH and low C4 levels and were diagnosed with type I HAE. Only 11 (57.9%) patients were symptomatic. Recurrent skin swelling and edema over the four extremities or trunk were reported in all symptomatic patients (100%). 45.5% of the patients recalled laryngeal attacks and one patient died from asphyxia. 18.2% of the patients experienced abdominal symptoms. The age at the beginning of clinical symptoms ranged from 5 to 30 years (mean +/- SD: 20.82 +/- 7.88 years). The diagnosis tended to be delayed (range from 1 to 39 years; mean +/- SD: 8.45 +/- 11.04 years). Nine patients had a mutant C1 INH gene, and two patients received long-term prophylaxis with danazol. CONCLUSION: The prevalence of hereditary angioedema in Taiwan is low. Persons with low levels of C1 INH who were clinically symptomatic accounted for only 57.9% of the cases in our study, which is far lower than previous reports from other countries. Ethnic differences may be the reason for this finding. Further genomic studies are needed to elucidate the genetic penetrance of C1 INH deficiency in Taiwan.

Factors for high-risk asthma in Taiwanese children.

BACKGROUND: Asthma is one of the major causes of death in otherwise healthy young individuals. However, many of these deaths may have been prevented by more aggressive treatment. To determine factors correlated with a high risk of death in Taiwanese children with atopic asthma. METHODS: Taiwanese children aged 5-18 years, diagnosed with atopic asthma were enrolled in the study. Atopic asthma was diagnosed and immunoglobulin E (IgE) specific to antigens from any 1 of 8 allergens was measured (i.e. Dermatophagoides pteronyssinus, Dermatophagoides farinae, cat and dog dander, cockroach, egg white, milk and fish). High-risk asthma was defined as asthma requiring admission to a hospital or a visit to an emergency department. The study tried to determine the association of high-risk asthma with allergy-related parameters (e.g. asthma severity, asthma score, total serum IgE levels, serum levels of allergen-specific IgE, eosinophil count) and pulmonary function in Taiwanese children. RESULTS: One thousand one hundred and twenty-two Taiwanese children were evaluated. Those with higher asthma severity, asthma symptom score, serum levels of IgE specific to D. pteronyssinus and D. farinae, higher total serum IgE levels, and lower FEF25-75% (forced expiratory flow, 25-75%) values were considered to be members of the high-risk asthma group. CONCLUSIONS: The characterization of risk factors has enabled us to identify high-risk asthma in Taiwanese children, which will facilitate the treatment of these children in the future.