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1.
Cereb Cortex ; 33(4): 1403-1411, 2023 02 07.
Artigo em Inglês | MEDLINE | ID: mdl-35368059

RESUMO

It has been shown that transcranial ultrasound stimulation (TUS) is capable of attenuating myelin loss and providing neuroprotection in animal models of brain disorders. In this study, we investigated the ability of TUS to promote remyelination in the lysolecithin (LPC)-induced local demyelination in the hippocampus. Demyelination was induced by the micro-injection of 1.5 µL LPC (1%) into the rat hippocampus and the treated group received daily TUS for 5 or 12 days. Magnetic resonance imaging techniques, including magnetization transfer ratio (MTR) and T2-weighted imaging, were used to longitudinally characterize the demyelination model. Furthermore, the therapeutic effects of TUS on LPC-induced demyelination were assessed by Luxol fast blue (LFB) staining. Our data revealed that reductions in MTR values observed during demyelination recover almost completely upon remyelination. The MTR values in demyelinated lesions were significantly higher in TUS-treated rats than in the LPC-only group after undergoing TUS. Form histological observation, TUS significantly reduced the size of demyelinated lesion 7 days after LPC administration. This study demonstrated that MTR was a sensitive and reproducible quantitative marker to assess remyelination process in vivo during TUS treatment. These findings might open new promising treatment strategies for demyelinating diseases such as multiple sclerosis.


Assuntos
Doenças Desmielinizantes , Esclerose Múltipla , Remielinização , Ratos , Animais , Esclerose Múltipla/diagnóstico por imagem , Esclerose Múltipla/terapia , Esclerose Múltipla/patologia , Doenças Desmielinizantes/induzido quimicamente , Doenças Desmielinizantes/diagnóstico por imagem , Doenças Desmielinizantes/terapia , Lisofosfatidilcolinas/toxicidade , Modelos Animais , Bainha de Mielina , Modelos Animais de Doenças
2.
Int J Mol Sci ; 23(17)2022 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-36077437

RESUMO

Microglia, astrocytes, and oligodendrocyte progenitor cells (OPCs) may serve as targets for remyelination-enhancing therapy. Low-intensity pulsed ultrasound (LIPUS) has been demonstrated to ameliorate myelin loss and inhibit neuroinflammation in animal models of brain disorders; however, the underlying mechanisms through which LIPUS stimulates remyelination and glial activation are not well-understood. This study explored the impacts of LIPUS on remyelination and resident cells following lysolecithin (LPC)-induced local demyelination in the hippocampus. Demyelination was induced by the micro-injection of 1.5 µL of 1% LPC into the rat hippocampus, and the treatment groups received daily LIPUS stimulation for 5 days. The therapeutic effects of LIPUS on LPC-induced demyelination were assessed through immunohistochemistry staining. The staining was performed to evaluate remyelination and Iba-1 staining as a microglia marker. Our data revealed that LIPUS significantly increased myelin basic protein (MBP) expression. Moreover, the IHC results showed that LIPUS significantly inhibited glial cell activation, enhanced mature oligodendrocyte density, and promoted brain-derived neurotrophic factor (BDNF) expression at the lesion site. In addition, a heterologous population of microglia with various morphologies can be found in the demyelination lesion after LIPUS treatment. These data show that LIPUS stimulation may serve as a potential treatment for accelerating remyelination through the attenuation of glial activation and the enhancement of mature oligodendrocyte density and BDNF production.


Assuntos
Doenças Desmielinizantes , Esclerose Múltipla , Remielinização , Animais , Fator Neurotrófico Derivado do Encéfalo/metabolismo , Doenças Desmielinizantes/metabolismo , Modelos Animais de Doenças , Camundongos , Camundongos Endogâmicos C57BL , Esclerose Múltipla/patologia , Bainha de Mielina/metabolismo , Oligodendroglia/metabolismo , Ratos
3.
Insects ; 12(11)2021 Oct 28.
Artigo em Inglês | MEDLINE | ID: mdl-34821776

RESUMO

Aphids are prominent phloem-feeding insect pests. Myzus persicae and Acyrthosiphon pisum are generalist and specialist species, respectively. In this study, these two aphid species were used to investigate host acceptance and plant resistance to aphid feeding. M.persicae survived and reproduced on rape (Brassica rapa), but few individuals (9%) survived on faba bean (Vicia faba). A.pisum survived and reproduced on faba bean, but no A.pisum survived on rape. The probing and feeding behavior of M. persicae and A. pisum on rape and faba bean was examined using an electrical penetration graph (EPG) technique. The results demonstrated the feeding preferences of these two aphid species. The EPG results suggest that the resistance of faba bean to M. persicae and that of rape to A. pisum are likely residing in the phloem and mesophyll tissues, respectively. Due to the distinct probing and feeding behaviors, specialist and generalist aphids would have different impacts on the epidemiology of plant viral diseases. The findings can be applied to the management of viral diseases transmitted by specialist or generalist aphids in crop production.

4.
PLoS One ; 16(3): e0247500, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33657150

RESUMO

Thrips and thrips-transmitted tospoviruses cause significant losses in crop yields worldwide. The melon thrips (Thrips palmi) is not only a pest of cucurbit crops, but also a vector that transmits tospoviruses, such as the watermelon silver mottle virus (WSMoV). Vector transmission of tospoviruses has been well studied in the tomato spotted wilt virus (TSWV)-Frankliniella occidentalis model system; however, until now the transmission mode of WSMoV by T. palmi has not been sufficiently examined. The results of the transmission assays suggest that T. palmi transmits WSMoV in a persistent manner, and that the virus is mainly transmitted by adults, having been ingested at the first-instar larval stage. Complementary RNAs corresponding to the NSm and NSs genes of WSMoV were detected in viruliferous thrips by reverse transcription-polymerase chain reaction; NSs protein was also detected in viruliferous thrips by western blotting, verifying the replication of WSMoV in T. palmi. Furthermore, we demonstrated that in thrips infected with WSMoV at the first-instar larval stage, the virus eventually infected various tissues of the adult thrips, including the primary salivary glands. Taken together, these results suggest that T. palmi transmits WSMoV in a persistent-propagative mode. The results of this study make a significant contribution to the understanding of the transmission biology of tospoviruses in general.


Assuntos
Citrullus/virologia , Doenças das Plantas/virologia , Tisanópteros/virologia , Tospovirus/genética , Animais , Feminino , Larva/virologia , Masculino , RNA Viral/genética , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Glândulas Salivares/virologia , Replicação Viral
5.
Food Technol Biotechnol ; 57(1): 105-112, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31316282

RESUMO

Orally administered fungal vaccines show promise for the prevention of infectious diseases. Edible mushrooms are deemed appropriate hosts to produce oral vaccines due to their low production cost and low risk of gene contamination. However, their low expression level of antigens has limited the potential development of oral vaccines using mushrooms. The low expression level might result from impurity of the transgenic mycelia since dikaryotic mycelia are commonly used as transformation materials. In this study, stable transgenic hepatitis B virus surface antigen (HBsAg) in Flammulina velutipes transformants was obtained by Agrobacterium-mediated transformation, followed by fruiting and basidiospore mating. The formation of HBsAg was detected by western blot analysis. The expression levels of HBsAg in transgenic F. velutipes fruiting bodies were (129.3±15.1), (110.9±1.7) and (161.1±8.5) ng/g total soluble protein. However, the values may be underestimated due to incomplete protein extraction. Two of the four pigs in the experimental group produced positive anti-HBsAg-specific IgG after being fed the HBsAg transgenic F. velutipes fruiting bodies for 20 weeks, while no anti-HBsAg antibody was detected in the control group. One of the positive pigs had HBsAg titres of 5.36 and 14.9 mIU/mL in weeks 10 and 14, respectively, but expression faded thereafter. The other positive pig displayed HBsAg titres of 9.75, 17.86 and 39.87 mIU/mL in weeks 14, 18 and 20, respectively. The successful immunogenicity in pigs fed transgenic F. velutipes fruiting bodies demonstrated the potential of using the fungus as an oral vaccine.

6.
Plant Dis ; 102(3): 600-607, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30673497

RESUMO

The whitefly-transmitted tomato chlorosis virus (ToCV) belonging to the genus Crinivirus (family Closteroviridae) affects tomato production worldwide. ToCV was first recorded in Taiwan in 1998 affecting tomato production. In this study, a local virus isolate XS was obtained, after serial whitefly transmissions from a diseased tomato plant displaying general chlorosis were collected in central Taiwan. The whole genome sequence of XS was determined from cDNA fragments amplified by reverse transcription (RT)-PCR, first using the degenerate primers for viruses of Closteroviridae and followed by degenerate and specific primers designed on available sequences of the ToCV isolates. The nucleotide (nt) sequences of RNA-1 and RNA-2 of the XS shared low identities of 77.8 to 78% and 78 to 78.1%, respectively, with genome segments of other ToCV isolates. Nevertheless, the viral RNA-dependent RNA polymerase (RdRp), heat shock protein 70 homolog (Hsp70h), and major capsid protein (CP) shared 88.3 to 96.2% amino acid (aa) identities with other ToCV isolates, indicating that XS is a new strain of this virus. Phylogenetic analyses of these three proteins indicated that all ToCV isolates from different counties outside Taiwan are closely related and clustered in the same clade, whereas the XS isolate is distinct and forms a unique branch. A one tube RT-PCR assay using primers designed from the genomic sequence of the XS was able to detect the ToCV-XS in infected tomato plants and in individual whiteflies. A field survey during 2013 to 2016 revealed a high ToCV-XS prevalence of 60.5% in 172 tested tomato samples, demonstrating that ToCV-XS is becoming an emerging threat for tomato production in Taiwan.


Assuntos
Crinivirus/isolamento & purificação , Hemípteros/virologia , Insetos Vetores/virologia , Doenças das Plantas/virologia , Solanum lycopersicum/virologia , Animais , Crinivirus/genética , DNA Complementar , Filogenia , Folhas de Planta/virologia , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Taiwan
7.
Pestic Biochem Physiol ; 131: 24-31, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27265823

RESUMO

Compared to other organophosphate-resistant and -susceptible (S) lines of Bactrocera dorsalis, the carboxylesterase (CBE) BdE5 in the naled-resistant (nal-r) line has been found to possess remarkable quantitative elevation. Our study attempts to identify the role of BdE5 in naled resistance, and we discovered several points of interest. Firstly, activity staining on native PAGE revealed that the percentage of flies with intensive BdE5 bands in the nal-r line was substantially higher than in the S line, indicating that the BdE5 band correlates with naled susceptibility. Secondly, in vitro and in vivo inhibition assays showed that BdE5 was inhibited by naled in both lines; under diagnostic doses of naled, the overall extent of inhibition on CBEs was much greater in the S line than in the nal-r line. Thirdly, NanoLC-nanoESi-MS/MS analysis used the NCBI database to identify and annotate BdE5 as an esterase FE4-like (XP_011200445.1) in B. dorsalis. Fourthly, rapid amplification of cDNA ends was used to obtain the 2012-bp full-length BdE5 cDNA, which contained an open reading frame of 1770bp and encoded a putative protein of 590 amino acid residues. Phylogenetic analysis revealed that BdE5 is a secreted ß-esterase (E clade) closely related to CG6414 (NP_570089), a CBE in Drosophila melanogaster. Finally, our relative quantification real-time PCR data showed a significant elevation in transcript levels of the BdE5 gene in nal-r line. Our results confirmed that BdE5 is correlated with naled resistance and provides further understanding about the identification and molecular characteristics of BdE5 in B. dorsalis.


Assuntos
Carboxilesterase/genética , Resistência a Inseticidas/genética , Inseticidas/farmacologia , Naled/farmacologia , Tephritidae/enzimologia , Animais , Carboxilesterase/isolamento & purificação , Clonagem Molecular , Eletroforese em Gel de Poliacrilamida , Feminino , Masculino , Filogenia , Reação em Cadeia da Polimerase em Tempo Real , Tephritidae/efeitos dos fármacos , Tephritidae/genética
8.
J Econ Entomol ; 109(3): 1378-1386, 2016 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-27106222

RESUMO

Pesticide resistance poses many challenges for pest control, particularly for destructive pests such as diamondback moths ( Plutella xylostella ). Organophosphates have been used in the field since the 1950s, leading to selection for resistance-related gene variants and the development of resistance to new insecticides in the diamondback moth. Identifying actual and potential genes involved in resistance could offer solutions for control. This study established resistant diamondback moth strains from two different collections using mevinphos. Two sets of transcriptome sequencing (RNA-Seq) data were generated for pairs of mevinphos-resistant versus susceptible (wild-type) strains. One susceptible strain containing 14 giga base pairs was assembled into a reference-based assembly using published scaffold sequences as reference. Differential expression data between resistant and susceptible strains revealed 944 transcripts (803 with annotations) showing upregulation and 427 transcripts (150 with annotations) showing downregulation. Around 6.8% of the differential expression transcripts (65) could be categorized as associated with well-known resistance mechanisms such as penetration, detoxification, and behavior response; of these 65 transcripts, 38 showed upregulation, and 12 relating to penetration were upregulated when the transcripts of 19 cytochrome P450s, 2 zeta-class glutathione S-transferases, and 4 ATP-binding cassette transporters showed upregulation. In addition, 11 groups of transcripts related to olfactory perception appeared to be downregulated in trade-off situations. Quantitative polymerase chain reaction expression results were consistent with RNA-Seq data. Possible roles of these differentially expressed genes in resistance mechanisms are discussed in this study.

9.
J Microbiol Immunol Infect ; 48(3): 340-4, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23218407

RESUMO

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.


Assuntos
Fibroma/congênito , Dedos/anormalidades , Dedos/patologia , Pele/patologia , Adolescente , Criança , Feminino , Fibroma/diagnóstico , Fibroma/patologia , Humanos , Masculino , Taiwan
10.
J Microbiol Immunol Infect ; 47(4): 358-61, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-22992393

RESUMO

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.


Assuntos
Fibrose Cística/diagnóstico , Fibrose Cística/genética , Adolescente , Adulto , Criança , Fibrose Cística/complicações , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Feminino , Genótipo , Humanos , Masculino , Mutação , Linhagem , Taiwan , Adulto Jovem
11.
PLoS One ; 8(3): e59099, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23516605

RESUMO

Agrobacterium tumefaciens-mediated transformation for edible mushrooms has been previously established. However, the enhancement of heterologous protein production and the expression of multi-target genes remains a challenge. In this study, heterologous protein expression in the enoki mushroom Flammulina velutipes was notably enhanced using 2A peptide-mediated cleavage to co-express multiple copies of single gene. The polycistronic expression vectors were constructed by connecting multi copies of the enhanced green fluorescent protein (egfp) gene using 2A peptides derived from porcine teschovirus-1. The P2A peptides properly self-cleaved as shown by the formation of the transformants with antibiotic resistant capacity and exciting green fluorescence levels after introducing the vectors into F. velutipes mycelia. The results of western blot analysis, epifluorescent microscopy and EGFP production showed that heterologous protein expression in F. velutipes using the polycistronic strategy increased proportionally as the gene copy number increased from one to three copies. In contrast, much lower EGFP levels were detected in the F. velutipes transformants harboring four copies of the egfp gene due to mRNA instability. The polycistronic strategy using 2A peptide-mediated cleavage developed in this study can not only be used to express single gene in multiple copies, but also to express multiple genes in a single reading frame. It is a promising strategy for the application of mushroom molecular pharming.


Assuntos
Flammulina/metabolismo , Expressão Gênica/genética , Vetores Genéticos/genética , Western Blotting , Flammulina/genética , Proteínas de Fluorescência Verde/genética , Proteínas de Fluorescência Verde/metabolismo
12.
Asian Pac J Allergy Immunol ; 29(3): 260-5, 2011 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22053596

RESUMO

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.


Assuntos
Agamaglobulinemia/genética , Povo Asiático/genética , Doenças Genéticas Ligadas ao Cromossomo X/genética , Proteínas Tirosina Quinases/deficiência , Proteínas Tirosina Quinases/genética , Tirosina Quinase da Agamaglobulinemia , Criança , Pré-Escolar , Cromossomos Humanos X , Análise Mutacional de DNA/métodos , Feminino , Ligação Genética , Heterozigoto , Humanos , Lactente , Masculino , Mutação , Taiwan
13.
Asian Pac J Allergy Immunol ; 29(4): 327-31, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22299312

RESUMO

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.


Assuntos
Proteína Inibidora do Complemento C1/genética , Angioedema Hereditário Tipos I e II/genética , Angioedema Hereditário Tipos I e II/fisiopatologia , Adolescente , Adulto , Criança , Pré-Escolar , Proteína Inibidora do Complemento C1/metabolismo , Humanos , Estudos Retrospectivos , Taiwan , Adulto Jovem
14.
Arch Virol ; 155(7): 1085-95, 2010 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-20480192

RESUMO

Melon yellow spot virus (MYSV), a tentative member of the genus Tospovirus, is considered a distinct serotype due to the lack of a serological relationship with other tospoviruses in its nucleocapsid protein (NP). Recently, a virus isolate collected from diseased watermelon in central Taiwan (MYSV-TW) was found to react with a rabbit antiserum (RAs) prepared against the NP of Watermelon silver mottle virus (WSMoV), and a monoclonal antibody (MAb) prepared against the common epitope of the NSs proteins of WSMoV-serogroup tospoviruses, but not with the WSMoV NP-specific MAb, in both enzyme-linked immunosorbent assay (ELISA) and western blotting. In this investigation, both RAs and MAb against MYSV-TW NP were produced. Results of serological tests revealed that the RAs to MYSV-TW NP reacted with the homologous antigen and the crude antigens of members of the WSMoV serogroup, including members of the formal species WSMoV and Peanut bud necrosis virus, and members of three tentative species, Watermelon bud necrosis virus, Capsicum chlorosis virus and Calla lily chlorotic spot virus. The MAb to MYSV-TW NP reacted only with the homologous antigen and the other geographic isolates of MYSV from Japan (JP) and Thailand (TH). Our results of reciprocal tests indicate that the NP and the NSs protein of MYSV are serologically related to those of WSMoV-serogroup tospoviruses. Furthermore, we show that both the MYSV NP MAb and the WSMoV NP MAb are reliable tools for identification of MYSV and WSMoV from single or mixed infection in field surveys, as verified using species-specific primers in reverse transcription-polymerase chain reaction.


Assuntos
Cucurbita/virologia , Doenças das Plantas/virologia , Vírus de Plantas/classificação , Vírus de Plantas/fisiologia , Animais , Anticorpos Monoclonais , Soros Imunes , Camundongos , RNA Viral/química , Coelhos , Sorotipagem
15.
Asian Pac J Allergy Immunol ; 28(4): 250-5, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21337908

RESUMO

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.


Assuntos
Alérgenos/imunologia , Asma/epidemiologia , Asma/imunologia , Eosinófilos/imunologia , Adolescente , Animais , Asma/sangue , Asma/fisiopatologia , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Imunoglobulina E/sangue , Masculino , Testes de Função Respiratória , Fatores de Risco , Taiwan
16.
Asian Pac J Allergy Immunol ; 28(4): 279-86, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21337913

RESUMO

BACKGROUND: Scleroderma is a chronic connective tissue disease characterized by hardened or scaly skin and widespread abnormalities of the viscera, which is rare in the pediatric age group. OBJECTIVE: In this study, we retrospectively reviewed 23 pediatric patients suffering systemic (SSc) and localized (LS) scleroderma. METHODS: Twenty-three patients were enrolled and were diagnosed with SSc or LS from March 1993 to September 2009 in the Department of Pediatrics at Mackay Memorial Hospital in Taipei, Taiwan. These diagnoses were based on the criteria of the American College of Rheumatology and the clinical manifestations of hard skin. Data recorded included sex, age-at-onset, age-at-diagnosis, laboratory data, family history, trauma history, treatment, and outcomes. RESULTS: Three patients suffered SSc and 20 patients had LS, including 16 girls and 7 boys. Mean age-at-onset was 6.55 +/- 3.28 years old. Antinuclear antibodies were positive in 15 patients. Tests for anti-Scl-70 antibodies were positive in 1 patient with SSc. One boy had en coup de sabre combined with a posterior fossa tumor. Twenty-two patients were treated with D-penicillamine. Oral prednisolone and methotrexate were added, if indicated. One girl with LS developed proteinuria after D-penicillamine treatment. All patients with localized disease ultimately documented a softening of their skin lesions. CONCLUSIONS: While scleroderma is rare in children, the prognosis of SSc is poor but better than for adults. The prognosis for LS is usually benign, however, the skin may become progressively indurated and it may not only be a skin disease. No progression from LS to SSc was observed in our study.


Assuntos
Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/epidemiologia , Adolescente , Adulto , Idade de Início , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Prognóstico , Proteinúria , Doença de Raynaud , Estudos Retrospectivos , Esclerodermia Localizada/fisiopatologia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/fisiopatologia , Fatores Sexuais , Taiwan
17.
Asian Pac J Allergy Immunol ; 25(2-3): 163-7, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-18035804

RESUMO

Hereditary angioedema (HAE) is an autosomal dominant disorder caused by a deficiency of C1 esterase inhibitor (C1-INH). Affected individuals have attacks of swelling involving almost any part of the body. We studied a family with 15 living members, including a 16-year-old girl who had 3 attacks of angioedema in 2 years. Her paternal uncle had died of asphyxiation during an attack 15 years previously. We analyzed the blood of each family member for C3, C4, and C1-INH levels and sequenced the SERPING1 (formerly C1NH) gene that codes for C1-INH. Six individuals had decreased serum levels of C4 and C1-INH, and they were all found to have a single nucleotide A deletion at codon 210 of the gene, 1210fsX210, a novel mutation that accounts for the HAE in this family.


Assuntos
Angioedemas Hereditários/genética , Proteínas Inativadoras do Complemento 1/genética , Mutação , Serpinas/genética , Doença Aguda , Adolescente , Sequência de Bases , Proteína Inibidora do Complemento C1 , Complemento C4/análise , Proteínas do Sistema Complemento/análise , Feminino , Humanos , Masculino , Dados de Sequência Molecular , Linhagem , Serpinas/sangue , Taiwan
18.
Ann Allergy Asthma Immunol ; 99(4): 375-9, 2007 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-17941288

RESUMO

BACKGROUND: Hereditary angioedema (HAE) is a rare disorder characterized by recurrent attacks of localized subcutaneous or submucosal edema. It is inherited in an autosomal dominant fashion and caused by a deficiency of C1 inhibitor (C1 INH). Most patients with HAE have an absolute deficiency of C1 INH (type I HAE), whereas the rest (approximately 15%) synthesize a dysfunctional C1 INH protein (type II HAE). Mosaicism is rare in HAE. OBJECTIVE: To describe the clinical manifestations, laboratory findings, and molecular genetic studies in a Taiwanese family with type I HAE with paternal mosaicism. METHODS: A family that included a 34-year-old man (index patient) and his 25-year-old brother who both had recurrent peripheral angioedema was evaluated. A younger sister had died of an unexplained cause at 18 years of age. We analyzed blood levels of C3, C4, and C1 INH and sequenced the SERPING] (C1NH) gene that codes for C1 INH in 5 family members, including the parents and 3 brothers. RESULTS: The 4 men in the family had a novel mutation c.3_73del, p.N1fsX34 in exon 3 of the C1INH gene, resulting in C1 INH deficiency. Although the father carried this mutant gene, he had normal serum levels of C1 INH. Based on quantitative analysis of allele dosage by DNA fragment analysis (GeneScan), the father was determined to have genetic mosaicism. CONCLUSION: Parental mosaicism is a possible explanation for normal C1 INH plasma concentrations in both parents despite clinically apparent HAE in the children.


Assuntos
Angioedema/genética , Proteínas Inativadoras do Complemento 1/genética , Doenças Genéticas Inatas/genética , Mosaicismo , Serpinas/genética , Adulto , Alelos , Sequência de Aminoácidos , Angioedema/sangue , Proteína Inibidora do Complemento C1 , Complemento C3/análise , Complemento C4/análise , Análise Mutacional de DNA , Éxons/genética , Saúde da Família , Feminino , Mutação da Fase de Leitura , Doenças Genéticas Inatas/sangue , Humanos , Masculino , Dados de Sequência Molecular , Pais , Linhagem , Deleção de Sequência , Serpinas/sangue , Taiwan
19.
Acta Paediatr Taiwan ; 48(5): 257-62, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-18254574

RESUMO

BACKGROUND: To evaluate the efficacy of prednisolone sodium phosphate oral solution plus inhaled procaterol in the treatment of acute asthma in children. METHODS: Forty-three patients aged 6 to 12 years with an acute exacerbation of asthma were double-blind randomized into one of two treatment groups in a 1:1 ratio:1) prednisolone oral solution +placebo tablets + procaterol MDI or 2) prednisolone tablets +placebo oral solution + procaterol MDI, all given three times daily for 7 days. Peak expiratory flow rate (PEFR), 24-hour reflective asthma symptom scores, spirometry and pulmonary index score (PIS) were recorded before and after treatment. Net changes in PEFR, symptom score, PIS, Forced Expiratory Volume in the first second (FEV1), FEV1/forced vital capacity (FVC), forced expiratory flow between 25 and 75 percent of the forced vital capacity (FEF(25-75%)) (before and after treatment) and global assessment by the investigator and the subjects or their parents were analyzed. RESULTS: The two groups were statistically similar at baseline values of these parameters. After a 7-day course of treatment, the net change of PEFR before and after treatment was significantly improved in both groups, but there was no significant difference in the net change of PEFR between the two groups (57.27+/-31.44 L/min vs. 54.29 +/-30.04 L/min, difference 2.99 +/-30.76 L/min, mean +/-SD, P=0.752). The net change in PIS and total symptom score did not differ between the two groups (P=0.091 and 0.827, respectively). Similarly, the FEV1, FEV1/FVC and FEF25-75% all improved with either treatment, and neither group was significantly superior to the other group (P=0.162, 0.48 and 0.081, respectively). Global assessment by the investigator and the subjects or their parents at the end of study indicated an essentially comparable result. CONCLUSIONS: Prednisolone sodium phosphate oral solution plus inhaled procaterol is as efficacious as prednisolone tablets plus inhaled procaterol in the management of acute asthma in children.


Assuntos
Asma/tratamento farmacológico , Prednisolona/administração & dosagem , Procaterol/administração & dosagem , Doença Aguda , Administração por Inalação , Administração Oral , Asma/fisiopatologia , Criança , Pré-Escolar , Método Duplo-Cego , Feminino , Volume Expiratório Forçado/efeitos dos fármacos , Humanos , Masculino
20.
Pediatr Dermatol ; 23(6): 560-3, 2006.
Artigo em Inglês | MEDLINE | ID: mdl-17155998

RESUMO

An 8-month-old boy developed a disseminated cutaneous mycobacterial infection at 6 months of age that responded poorly to treatment. Further immunologic study and sequence analysis showed the presence of a missense mutation in the IL2RG gene, and X-linked severe combined immunodeficiency was diagnosed. To identify the strain of the microorganism causing the cutaneous infection, polymerase chain reaction with four pairs of primers was performed on skin biopsy specimens positive for acid-fast bacilli. Sequence analysis showed 99.36% homology with a bacilli Calmette-Guérin positive control. Disseminated bacilli Calmette-Guérin disease must be considered in any infant with cutaneous mycobacterial lesions, especially those with atypical histologic findings or who are immunocompromised. Testing by polymerase chain reaction using the proper primers may help in making a precise diagnosis.


Assuntos
Mycobacterium bovis/genética , Reação em Cadeia da Polimerase , Tuberculose Cutânea/diagnóstico , Tuberculose Cutânea/microbiologia , Doenças por Imunodeficiência Combinada Ligada ao Cromossomo X/diagnóstico , Doenças por Imunodeficiência Combinada Ligada ao Cromossomo X/microbiologia , Vacina BCG/administração & dosagem , Vacina BCG/efeitos adversos , Evolução Fatal , Humanos , Lactente , Masculino , Tuberculose Cutânea/complicações , Doenças por Imunodeficiência Combinada Ligada ao Cromossomo X/complicações
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