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2.
Zhonghua Er Ke Za Zhi ; 58(12): 977-981, 2020 Dec 02.
Artigo em Chinês | MEDLINE | ID: mdl-33256319

RESUMO

Objective: To analyze the clinical course and targeted therapy of pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. Methods: The clinical history of a 6-year-old boy with PAPA syndrome, who was admitted to Hong Kong University Shenzhen Hospital in September 2017, was reviewed. His genetic diagnosis was confirmed by whole exome sequencing. The response to targeted therapy was evaluated by comparing the inflammatory markers (erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) and serum cytokines (interleukin (IL)-1, IL-6 and tumor necrosis factor-α (TNF-α)) before and after biological agents treatment. For literature review, "PAPA syndrome" and"PSTPIP1 gene"were used as keywords to retrieve papers published from January 1997 to December 2019 from Pubmed, Wanfang and CNKI database. Results: The patient was a 6-year-old boy, admitted to the hospital due to recurrent joint swelling and pain for more than 4 years. Before treatment, the CRP (256 mg/L), ESR (105 mm/1 h) and cytokines including serum TNF-α (7.43 ng/L), IL-1 (<5 ng/L), IL-6 (301 ng/L) were significantly elevated. Culture of the joint effusion was negative, but the IL-6 level was above 1 000 ng/L. MRI showed osteomyelitis at the lower end of the right femur. Gene detection found a heterozygous variation of PSTPIP1 gene (c.748G>A, p.E250K). Arthralgia once alleviated after the initiation of tocilizumab and infliximab, but recurred after 1 year of treatment. Thereafter, the anti-IL-1 receptor antagonist (Anakinra) was commenced, followed by a significant improvement of the arthralgia, and a complete remission during the follow-up. Besides, the level of CRP, ESR, serum TNF-α, IL-1 and IL-6 were all decreased to normal on the last followed up in December 2019. Literature review found 29 articles and 87 patients in total. The initial symptoms included those of arthritis (n=58), pyoderma gangrenosum (n=33), and acne (n=24). Among all the cases, 13 genotypes were confirmed, and 47 variations involved amino acid p.E250. Steroid and/or biological agents were used in most patients. Conclusions: PAPA syndrome should be suspected in children with recurrent pyogenic sterile arthritis, and an early diagnosis could be achieved by genetic test. Targeted treatment with biological agent may control the symptoms effectively. Biological agents can control symptoms of this disorder effectively.


Assuntos
Acne Vulgar , Artrite Infecciosa , Pioderma Gangrenoso , Acne Vulgar/tratamento farmacológico , Proteínas Adaptadoras de Transdução de Sinal/genética , Artrite Infecciosa/tratamento farmacológico , Fatores Biológicos/uso terapêutico , Criança , Proteínas do Citoesqueleto/genética , Humanos , Masculino , Pioderma Gangrenoso/tratamento farmacológico
4.
Artigo em Chinês | MEDLINE | ID: mdl-21171453

RESUMO

AIM: To investigate whether or not the lateral septal nucleus (LS) was one of important analgesic areas of BmK venom in the central nervous system (CNS), and, targeting on which receptor, analgesic effect was produced by it. METHODS: Pain threshold of skin was observed by the latent period of tail flick evoked by radiant heat. Glass micropipette placed in Pf was used to record unit discharges of neurons in it, before and after 0.01% BmK venom were injected into LS. Stainless steel cannula placed in the lateral cerebral ventricle (icy) and LS was used for microinjection. RESULTS: After injection of 2 microl 0.01% BmK venom into icy of rat, the pain threshold was apparently raised, which was completely returned by injection of 0.5 microl 0.25% naloxone into icy. After 0.5 microl 0.01% BmK venom was injected into LS, 71% (15/21) nociceptive-on neurons and 83.3% (5/6) nociceptive-off neurons decreased the nociceptive response to tail pinch, but no evident effect was observed in the non-nociceptive neurons. CONCLUSION: The analgesic effect of BmK venom was probably realized mainly by the opiate receptor, and LS was one of important analgesic areas of BmK venom on CNS.


Assuntos
Analgésicos/farmacologia , Sistema Nervoso Central/efeitos dos fármacos , Venenos de Escorpião/farmacologia , Animais , Núcleos Laterais do Tálamo/efeitos dos fármacos , Núcleos Laterais do Tálamo/fisiologia , Neurônios/fisiologia , Limiar da Dor , Ratos , Ratos Wistar , Escorpiões
5.
Vaccine ; 15(9): 944-7, 1997 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-9261939

RESUMO

A study on the possibility of transmission of live hepatitis A vaccine (H2-strain) from vaccinees to nonvaccinees was conducted. As a result, no seroconversion was found among 87 seronegative nonvaccinees, who had a close contact with their 141 subcutaneously vaccinated classmates nor was it found among 101 seronegative children administered the vaccine orally. The above fundings suggest that by losing the ability to be transmitted orally the vaccine virus may result in a decreasing possibility of dissemination among contacts. A 4-year study on the protective efficacy of the H2-strain vaccine was done at 11 primary schools starting at 1991 in Shaoxing County. Since then, there has been no hepatitis A reported among 18102 cumulative person-years in the vaccination group, while 495 cases occurred among 242168 cumulative person-years in the control groups. A large scale vaccination with a cumulative vaccination coverage of 89.45% was carried out in Jiaojiang City among children 1-15 years old. Hepatitis A in this age group in the city, which had 12-87 cases per annum with an average of 32 for 8 years before vaccination, decreased drastically to 0-1 cases after vaccination. The protective efficacy of H2-strain vaccine proved to be satisfactory.


Assuntos
Vírus da Hepatite A Humana/imunologia , Hepatite A/prevenção & controle , Programas de Imunização , Vacinas contra Hepatite Viral/efeitos adversos , Vacinas contra Hepatite Viral/imunologia , Administração Oral , Adolescente , Adulto , Criança , Pré-Escolar , China , Feminino , Seguimentos , Hepatite A/transmissão , Anticorpos Anti-Hepatite A , Vacinas contra Hepatite A , Anticorpos Anti-Hepatite/biossíntese , Humanos , Lactente , Masculino , Sorotipagem , Vacinas Atenuadas/administração & dosagem , Vacinas Atenuadas/efeitos adversos , Vacinas Atenuadas/imunologia , Vacinas contra Hepatite Viral/administração & dosagem
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