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简介：AbstractIntroduction:Behçet disease is a chronic multisystem vasculitis disease, however generalized polymorphous cutaneous lesions are uncommon.Here, we reported a case of Behçet disease with multiple parts of the body and complex lesions, which may lead to misjudging in clinical diagnosis.Case report:A 69-year-old man presented with a seven-year history of recurrent generalized polymorphous mucocutaneous lesions (erythematous papules, nodules, ulcers, and necrosis) over his entire body, and the lesions had been painful for the past three years. Based on the past medical history, clinical presentation, histological examination excluded other diseases, and the 2014-amended International Criteria for Behget disease, the patient had a score of 6 points and was diagnosed as Behçet disease.Discussion:The common clinical feature in patients with Behçet syndrome is the presence of recurrent and usually painful mucocutaneous ulcers. Other clinical manifestations of this disorder are more variable among different patients. A diagnosis of generalized polymorphous cutaneous lesions should remain on the list of differential diagnosis of Behçet disease after excluding other diseases.Conclusion:The mucocutaneous lesions of Behçet disease are often preceded by other manifestations, and timely diagnosis may benefit early treatment and prognosis.

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简介：AbstractIntroduction:Erosive adenomatosis of the nipple is a rare benign lesion involving nipple which can be easily misdiagnosed as more ominous entities such as Pagets disease.Here we reported a case of erosive adenomatosis on the nipple masquerading as paget’s disease.Case presentation:A 21-year-old woman presented with a 6-year history of breast erythema involving her left nipple with tenderness and mild itching. The nipple appeared rough and thickened with areas of erosion and fissuring. Physical examination of other areas of breast as well as imaging studies including ultrasound and mammography showed no abnormalities. The histological examination reveal adiagnosis of erosive adenomatosis of nipple (EAN). The patient was treated by a simple surgical excision without complications and recurrence during 9-month follow-up.Discussion:In the early stage, it can mimic eczema, but in the later stage, more serious diagnosis such as Pagets disease should be differential. It is diagnosed by histopathological examination. Surgical excision is an optimal choice for treament with or without plastic reconstruction.Conclusion:EAN should be considered in the diagnosis of erythema and erosion lesions involving nipple. The gold standard for diagnosis is histopathological examination. A prompt and correct diagnosis of EAN can avoid both unnecessary over treatment and patients emotional stress.

简介：AbstractIntroduction:Coronavirus disease-2019 (COVID-19) presents with a variety of symptoms, but rhabdomyolysis has rarely been reported in children.Case presentation:We report a 10-year-old girl who presented with fever, myalgia, and limping. The patient was tested positive for severe acute respiratory syndrome coronavirus-2. On admission, creatine kinase (CK) level was 13 147 units per liter and the patient was diagnosed with rhabdomyolysis. She was treated with intravenous fluids, which resulted in CK levels decrease. There are currently seven case reports of children with rhabdomyolysis associated with acute COVID-19 infection and two reports with the multisystemic inflammatory syndrome.Conclusion:Children presenting with muscle pain and weakness in the acute phase or following COVID-19 infection, should alert physicians of the possibility of rhabdomyolysis.

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简介：煽动性的肠疾病(IBD)包括二个实体，Crohn的疾病和ulcerative。两个是有经常的复杂并发症和外科的过程的长期的条件，一个伟人影响病人生命的质量。thiopurine抗代谢物azathioprine和6-mercaptopurine广泛地在IBD病人被使用。当前的指示包括维护治疗，类固醇家属疾病，管闭合，infliximabimmunogenicity的预防和Crohn的疾病复发的预防。令人惊讶地，在最后十年的抑制免疫力的药的宽使用没减少外科的需要，可能因为这些治疗在疾病路线在太迟了的阶段被介绍。immunossupressants的更早的使用现在被一些作者倡导。合理包括：(1)修改现在的治疗学的途径的IBD自然科学的失败，(2)那个azathioprine罐头导致粘膜的愈合的示范，为Crohn的疾病和ulcerative的一个相关预示的因素，并且(3)早immunossupression有的示范很积极的影响在上小儿科，最近诊断的Crohn的疾病病人。我们现在正在等候新研究的结果，诊断了成年Crohn的疾病病人(阿兹台克人学习)，与infliximab(声音的学习)相比澄清azathioprine的贡献，并且在最近表明azathioprine的实用性。

简介：AbstractIntroduction:Bowen’s disease is a squamous cell carcinoma in situ. It is more common on the head and neck, less common on the nail units in which it usually involves the second to fourth fingers of both hands. Here, we report a case of Bowen’s disease involving the left fifth finger of a 78-year-old Chinese woman.Case presentation:The patient was treated with nine sessions of topical 5-aminolevulinic acid-photodynamic therapy (5-ALA-PDT), with a one-week interval between sessions. The 5-ALA cream was applied to the lesion and the region surrounding its marginal diameter by 0.5 cm. After 3-4 hours, the lesion was irradiated with 635 ± 10 nm red light at an intensity of 80 mw/cm2 for 20 minutes. An excellent result with a topical 5-ALA-PDT was reached.Discussion:PDT involves the application of a combination of a photosensitizing drug and light to cause selective damage to the target tissue. PDT is more appropriate than surgical excision for lesions located in sites with poor healing ability.Conclusion:PDT appears to be effective, safe and has a better cosmetic outcome in the treatment of Bowen’s disease of the finger unit.

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简介：AbstractBackground:Few data are available regarding the long-term case-fatality rate (CFR) among people living with HIV (PLWH) with nontuberculous mycobacteria (NTM) disease. The aim of this study is to analyze the long-term CFR in patients with NTM disease and to identify risk factors for their death.Methods:A retrospective cohort study of 379 cases of microbiologically confirmed NTM disease in PLWH was conducted from January 1, 2012, to December 31, 2020, in Shanghai, China. We used Kaplan-Meier survival analysis and the log-rank test to compare the long-term CFR in patients with disseminated NTM (DNTM) and localized NTM disease. Univariate Cox proportional hazards regression analysis and a stepwise Cox proportional hazards regression model were used to estimate the predictors of long-term CFR.Results:The cohort was followed up for a median of 26 months. The total CFR was 15.7% by one year and increased to 22.6% at 5 years after the diagnosis of NTM disease. The 5-year CFR of PLWH with DNTM was significantly higher than that of PLWH with localized NTM (26.7% vs 19.6% for DNTM and localized NTM disease, respectively). Older age [hazard ratio (HR)= 1.04, 95% confidence interval (CI): 1.02-1.06, P < 0.001], comorbidity (HR= 2.05, 95% CI: 1.21-3.49, P < 0.01), DNTM (HR= 2.08, 95% CI: 1.17-3.68, P < 0.05), and HIV viral load (HR= 1.32, 95% CI: 1.12-1.55, P < 0.001) were all independent risk factors for long-term CFR. In the subgroup analysis, time to culture positivity was negatively correlated with CFR in patients with DNTM (HR= 0.90, 95% CI: 0.82-0.98, P < 0.05).Conclusions:NTM was associated with a high long-term CFR in PLWH. Further approaches to prevent NTM disease in PLWH are urgently needed.

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简介：Despitetheadvancesincombinatorialorsyntheticchemistryandbioinformatics,recentliteraturehasdemonstratedtherelevanceofnatureandbiomassasasourceofnewmoleculestotreatdifferentpathologies,i.e.,bioactivecompoundsobtainedfromEcteinascidiaturbinatetotreatsometypesofcancerorrapamycinfromStreptomyceshygroscopicustopreventorganrejectionaftertransplant.Thistrend

简介：Complicationsaremorefrequentinelderlypatientswithcoronaryheartdisease(CHD),suchasimpairedglucosetoleranceandconstipations.Itisalwaysdifficulttocurethesecomplicationsinclinicalpractice.Inthiscasewehadsuccessfullycuredaneighty-threeyearsoldmanwithCHDcomplicatingimpairedglucosetoleranceandconstipationbyintegratedChinese-Westernmedicine.

简介：AbstractIntroduction:Celiac disease typically presents with symptoms of malabsorption, but extraintestinal manifestations are increasingly reported. Aplastic anemia as the mode of celiac disease presentation is extremely rare in children.Case presentation:We report a 2-year-old boy who presented with loose stools, loss of appetite, and bicytopenia with severe aregenerative normocytic anemia. Investigations, including bone marrow aspirate and biopsy, revealed aplastic anemia. Screening for malabsorption showed increased plasma concentrations of anti-transglutaminase and anti-gliadin antibodies. A duodenal biopsy confirmed the histologic features of celiac disease. The child received a packed red cell transfusion and was started on a gluten-free diet, with a very good prognosis and normalization of both his blood and histological parameters. To the best of our knowledge, our report is the sixth pediatric case in the literature.Conclusion:Screening for celiac disease should be performed in children with unexplained hematological abnormalities such as aplastic anemia with or without gastrointestinal symptoms.

简介：AbstractObjective:This study aimed to investigate the association between single-nucleotide polymorphisms (SNPs) of PCSK1 (proprotein convertase subtilisin/kexin type 1) related to obesity and nonalcoholic fatty liver disease (NAFLD).Methods:In this case-control observational study, four candidate SNPs (rs6234, rs155971, rs6232, rs3811951) of PCSK1 were genotyped in 732 NAFLD patients and 823 healthy control participants, all of whom were of ethnic Han Chinese descent. All participants came from Shanghai, China, and joined our study during 2015 to 2016. The frequencies of each allele and genotype, paired linkage disequilibrium, and haplotype were calculated on the SHEsis platform. In addition to SHEsis, five different genetic models (codominant, dominant, recessive, overdominant, and log-additive) were employed to identify the correlation between genotype frequency and NAFLD. This study was approved by the Medical Ethics Committee of Shanghai University of Traditional Chinese Medicine (approved No. 2017LCSY069).Results:In a comparison of NAFLD patients and healthy participants, none of the four PCSK1 SNPs were significantly correlated with the occurrence of NAFLD (P>0.05), in either genotypic or allelic distribution. The recessive model of rs3811951 appeared to show a correlation (odds ratio=1.077; 95% confidence interval=0.924-1.256; P=0.04), but there was no statistical significance after Bonferroni correction (Pcorr>0.0125).Conclusions:Four obesity-related PCSK1 SNPs (rs6234, rs155971, rs6232, rs3811951) showed no significant correlation with the development of NAFLD in a Han Chinese population.

简介：AbstractIntroduction:Numerous dermatoses associated with monoclonal gammopathy have been reported in the literature. Subepidermal autoimmune bullous diseases (SABD) are one of them which were not common.Here we report a case of SABD associated with MGUS who had a severe mucosal involvement and unexpected positive direct Nikolsky phenomenon.Case presentation:A 68-year-old male patient was admitted to our clinic with erosions on the oral mucosa, tense blisters, erosions, and ulcers on the trunk and extremities. Subepidermal vesicle formation was detected in the skin biopsy. Clinical examination revealed positivity for the Nikolsky phenomenon. The disease was unresponsive to conventional treatments and dysphagia and hoarseness occurred. The patient was screened for malignancy due to his unresponsiveness to the treatments and his severe oral mucosal involvement. Ig-G MGUS was detected in the patient.Discussion:The Nikolsky sign is an indicator of acantholysis and is known as a specific finding for pemphigus. However, when we look at gammopathy-associated autoimmune bullous dermatoses, skin fragility has been reported in cases. However, the meaning of fragility is not explained. The diagnosis of all these patients was Ig-M MGUS. Our patient was presented because of non-IgM MGUS, direct Nikolsky positivity, and severe mucosal involvement.Conclusion:Nikolsky positivity may be a clue for gammopathy-related subepidermal autoimmune bullous diseases.

简介：AbstractIntroduction:Darier’s disease is a rare autosomal dominant skin disorder caused by mutations in ATP2A2. Recently, the high prevalence of neuropsychiatric symptoms is frequently reported in Darier’s disease patients.We present a case of patient with concurrence of Darier’s disease and depression and detected the mutations in ATP2A2.Case presentation:A 29-year-old man presented with a 15-year history of brown, harsh keratotic papules on his scalp, face, neck, trunk, axilla, ingunia and upper limbs. Meanwhile, he had persistent depression. He was diagnosed as Darier’s disease according to clinical manifestations and result of histological examination. We sequenced all coding regions of ATP2A2. The mutation c.2993_2994del (p. Val998Alafs*33) in exon 20 of ATP2A2 was detected.Discussion:Darier’s disease has been associated with several extracutaneous manifestations, in particular neuropsychiatric morbidity. Likely gene disrupting mutations in ATP2A2 was reported to have a close relationship to Darier’s disease cases with reported co-occurring neuropsychiatric features. Consistent with the previous reports, an likely gene disrupting mutations was detected in our patient with concurrence of DD and depression.Conclusion:The psychiatric disorders in patients with Darier’s disease should be well appreciated .The underlying mechanism in correlation between the mutation in ATP2A2 and neuropsychiatric phenotypes in Darier’s disease remain unclarified and warrants further investigation.

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