BACKGROUND Splenic rupture associated with Behçet’s syndrome(BS)is extremely rare,and there is no consensus on its management.In this case report,a patient with BSassociated splenic rupture was successfully trea...BACKGROUND Splenic rupture associated with Behçet’s syndrome(BS)is extremely rare,and there is no consensus on its management.In this case report,a patient with BSassociated splenic rupture was successfully treated with splenic artery embolization(SAE)and had a good prognosis after the intervention.CASE SUMMARY The patient was admitted for pain in the left upper abdominal quadrant.He was diagnosed with splenic rupture.Multiple oral and genital aphthous ulcers were observed,and acne scars were found on his back.He had a 2-year history of BS diagnosis,with symptoms of oral and genital ulcers.At that time,he was treated with oral corticosteroids for 1 month,but the symptoms did not alleviate.He underwent SAE to treat the rupture.On the first day after SAE,the patient reported a complete resolution of abdominal pain and was discharged 5 d later.Three months after the intervention,a computed tomography examination showed that the splenic hematoma had formed a stable cystic effusion,suggesting a good prognosis.CONCLUSION SAE might be a good choice for BS-associated splenic rupture based on good surgical practice and material selection.展开更多
Behçet’s disease (BD) is a multi-systemic vasculitis characterized by a triad made up of oral aphthosis, genital aphthosis and uveitis which may or may not be associated with visceral involvement. Other times de...Behçet’s disease (BD) is a multi-systemic vasculitis characterized by a triad made up of oral aphthosis, genital aphthosis and uveitis which may or may not be associated with visceral involvement. Other times described in the ancient silk road, BD is increasingly found in countries south of the Sahara with the mixing of populations. Our objective was to describe the epidemiology and clinical characteristics of BD in an African country. Methodology: This was a five-year retrospective study in the internal medicine department of Donka National Hospital. We used international criteria finding BD. Urinary dipstick was use for finding kidney disease. Result: During the period, five cases of Behçet’s disease were collected. The average age was 35 years old. The male sex was more represented with 3 out of 5 cases. The most common clinical manifestation was oral aphthosis in all patients (100%), followed by genital aphthosis in 4 cases (80%). The other clinical manifestations observed were uveitis in 3 cases (60%), joint manifestations such as arthralgia in 3 cases (60%) and neurological manifestations such as chronic headache in one case (20%). HLA-B51 was found in two cases. The pattergic test done in two patients came back positive (100%). The patients received colchicine and oral corticosteroid therapy. Two patients were lost to sight. We deplored a death probably in a neuro-Behçet table before the rebellious headaches. Conclusion: Behçet’s disease is a multi-systemic vasculitis that must be evoked and managed quickly in a tropical environment, especially since the prognosis, vital and functional prognosis can be engaged.展开更多
Aseptic abscesses are an emergent entity and have been described in inflammatory bowel disease,especially in Crohn's disease,and in other diseases.However,aseptic abscesses associated with Beh et's disease are...Aseptic abscesses are an emergent entity and have been described in inflammatory bowel disease,especially in Crohn's disease,and in other diseases.However,aseptic abscesses associated with Beh et's disease are extremely rare.We report a Japanese male diagnosed with an incomplete type of Beh et's disease who developed multiple aseptic abscesses of the spleen and liver.In 2002,the spleen abscesses were accompanied by paroxysmal oral aphthous ulcers and erythema nodosum.As the patient's response to antibiotic treatment was inadequate,a splenectomy was performed.Severe inflammatory cell infiltration,largely of polymorphonuclear neutrophils,was observed without evidence of bacterial or fungal growth.Although the patient had no history of ocular symptoms or genital ulcers,a diagnosis of incomplete Beh et's disease was made according to the Japanese diagnostic criteria because of the presence of paroxysmal arthritis and epididymitis since 2002.In 2005,multiple liver abscesses developed with right hypochondrial pain and seemed to be attributed to Beh et's disease because the abscesses yielded negative results during a microbiologic investigation and failed to go into remission under antibiotic therapy.Oral prednisone(15 mg/d) was started in May 2006,and the abscesses dramatically disappeared 4 wk after treatment.Although the patient had a relapse of the liver abscesses in association with the tapering of prednisone,the augmentation of prednisone dosage yielded a response.The abscesses of the liver and spleen were strongly suggested to be attributed to Beh et's disease.Clinician should be aware of the existence of aseptic abscesses as uncommon manifestations of Beh et's disease.展开更多
TO THE EDITOR I have read with great interest the very recent article rifled ＂Intestinal Behcet＇s disease with pyoderma gangrenosum： A case report＂ of Nakamura T et al that was published in your journal. The autho...TO THE EDITOR I have read with great interest the very recent article rifled ＂Intestinal Behcet＇s disease with pyoderma gangrenosum： A case report＂ of Nakamura T et al that was published in your journal. The authors stated that they presented a very rare case of intestinal Behcet＇s disease with pyoderma gangrenosum in a 16-year old patient. However, I would like to make some important contributions and suggestions to the presented case and have a few questions to ask the authors.展开更多
BACKGROUND In Behçet’s disease(BD),very few cases of muscular involvement have been reported previously.The natural history and therapeutic protocol for muscular involvement in BD are obscure due to the low inci...BACKGROUND In Behçet’s disease(BD),very few cases of muscular involvement have been reported previously.The natural history and therapeutic protocol for muscular involvement in BD are obscure due to the low incidence of peripheral neuropathy or myopathy in BD.The purpose of our study was to report a rare case of BD with chronic,focal forms of neuromyopathy and review the relevant literature.CASE SUMMARY We herein report the case of a 54-year-old man who presented with progressive muscular atrophy and weakness of both thighs 2 years after the presentation of the cardinal symptoms of BD.The past medical history,electrophysiological study,neurological examination,blood tests,magnetic resonance imaging study,and histological exam were performed for the differential diagnosis.Relevant literature on muscular involvement in BD was reviewed.Neurological examination revealed that muscular involvement was predominantly localized in the proximal parts of the lower extremities.Heterogeneous enhancement of several thigh muscles was observed on magnetic resonance imaging,which corresponded with the clinical manifestations.Histological study of one of the enhanced muscles showed denervation atrophy of the muscle with superimposed myopathic changes,while electrophysiological studies only suggested denervation.CONCLUSION To our knowledge,this is the first case of neurogenic muscular atrophy with a specific set of clinical,radiological,electrophysiological,and histological findings reported in BD.展开更多
Purpose: To analyze the clinic manifestation and prognosis of Behet disease. Method: Twenty patients requiring inpatient treatment with Beh?觭et disease were retrosp-ectively analyzed. Results: The morbidity of Beh?...Purpose: To analyze the clinic manifestation and prognosis of Behet disease. Method: Twenty patients requiring inpatient treatment with Beh?觭et disease were retrosp-ectively analyzed. Results: The morbidity of Beh?觭et disease is 5.5/100 000. In the systemic damage, stom-atocace and skin lesion are 95%, eye lesion and genital ulcer 50%, joint lesion 45%, gastrointestinal lesion 35%, Uveitis is the major disease in eye lesion, and followed in order by retinal vasculitis and obstruction of retinal artery. Attack age average 30.3 years old. Diagnosis age average 34.8 years old. The patients stay in hospital for 41 days on the average. Cure rate is 55%, improvement rate 40%, blinding rate of eye lesion is 36%. Conclusions: Beh?觭et disease is a multisystem lesion disease. Stomatocace and skin lesion is the major lesion, the next in common occurrence are eye and genital lesions. Repeated attack of uveitis, complicated cataract and secondary glaucoma are the major causes of blindness.展开更多
<strong>Background:</strong> Beh<span style="white-space:nowrap;">ç</span>et’s disease (BD) is a multisystemic, chronic inflammatory disorder with a broad range of manifes...<strong>Background:</strong> Beh<span style="white-space:nowrap;">ç</span>et’s disease (BD) is a multisystemic, chronic inflammatory disorder with a broad range of manifestations including within the cardiovascular system. Cardiac involvement like intracardiac thrombus (ICT) and valvular involvement in BD are rarely seen entities and often associated with poor prognosis. <strong>Case Presentation: </strong>We present the case of a young patient with vascular-Beh<span style="white-space:nowrap;">ç</span>et diagnosed by the presence of intracardiac thrombus in the right ventricle, unresponsive to medical treatment. Even though the intracardiac lesion was successfully treated by surgical excision, he presented a severe tricuspid dysfunction some years later due to the extension of fibrosis with no surgical therapeutic option. <strong>Conclusion:</strong> Intracardiac thrombosis is a rare but early manifestation of Beh<span style="white-space:nowrap;">ç</span>et’s disease, which is difficult to diagnose and has a poor prognosis.展开更多
Having considered the impact of the function of TLR2 in there cognition of several microorganisms that are thought to havean association with disease(BD),we aimed to determinea possible association between the TLR2 Ar...Having considered the impact of the function of TLR2 in there cognition of several microorganisms that are thought to havean association with disease(BD),we aimed to determinea possible association between the TLR2 Arg753Gln polymorphism and susceptibility to BD.We genotyped 83 patients with BD,95 ethnically matched healthy controls,12patients with recurrent aphthous stomatitis(RAS)and 21 patientswith rheumatoid arthritis(RA)by restriction fragment length polymorphism after PCR amplification of the genomicregion encompassing the polymorphic site.Comparison of the TLR2 Arg753Gln A allele and A/G genotype frequencies did not show a significant difference between patients with BD andhealthy controls(1.2%vs.0.6%,and 2.1%vs.1.1%,respectively).None of the patients from the RAS and RA groups had the A allele or A/G genotype.Our results indicate that the TLR2 Arg753Gln polymorphism does not play a role in the aetiopathogenesis of BD.展开更多
Introduction:disease is a rare,chronic disorder.The cause of disease is unknown.It is believed to be caused by an autoimmune reaction.As in other chronic autoimmune diseases,disease may show a subclinical adrenal fail...Introduction:disease is a rare,chronic disorder.The cause of disease is unknown.It is believed to be caused by an autoimmune reaction.As in other chronic autoimmune diseases,disease may show a subclinical adrenal failure and some changes in cortisol levels.We aimed to evaluate adrenal gland function in disease patients.Material and method:This study included 18 disease patients and 15 healthy controls.Patient and control groups were administered i.v.1 μg low dose test(LDT)and 250 μg standarddose test(SDT)adrenocorticotropic hormone(ACTH)stimulation test after 12 h of night fasting with an interval of 3-days and cortisol responses in the 0th,30th and 60th minutes were evaluated.Results:There was no statistically significant difference between basal cortisol values of disease and control groups.Cortisol values in the 60th minute in LDT were significantly lower in disease group than in the control group.In the peak cortisol responses to LDT,a significant decrease was found in disease group.Conclusion:These findings suggest that hypothalamo-pituitary adrenal axis is partially suppressed in disease.展开更多
Background:disease(BD)is a systemic inflammatory vasculitis of young adults with unknown etiology.Objective:The aim of this study was to determine the clinical features and outcome of BD in childhood.Patients and meth...Background:disease(BD)is a systemic inflammatory vasculitis of young adults with unknown etiology.Objective:The aim of this study was to determine the clinical features and outcome of BD in childhood.Patients and methods:Seventeen patients with BD within a pediatric age group were studied since 1997.Results:Boys :girls ratio was 12:5.Familial cases were very frequent(45%).Oral aphthous ulcers were presentin all patients(100%),genital aphthous ulcers were present in 16(94%),ocular lesions in 4(24%),erythema nodosum in 3(18%),papulopustules in 8(47%),joint symptoms in 13(76%),mild gastrointestinal symptoms in 2(12%),neurological involvement in 2(12%),positive pathergy reactions in 13(76%),and thrombophlebitis in 1(6%).We used colchicine,system iccorticosteroids,cyclosporine,nonsteroidal anti-inflammatory drugs,sulfasalazine,and topical agents for the treatment of children with BD.Conclusion:The findings of BD in children are similar as in adults,but the frequency of familial cases is significantly higher.展开更多
目的系统评价白塞病(BD)患者血浆同型半胱氨酸水平与动静脉血栓形成间的关联性。方法检索1950年1月至2014年10月Pubmed、EMBASE、Cochrane Library、Web of Science、CNKI等中外文数据库上所有关于BD合并动静脉血栓形成的研究。按照纳...目的系统评价白塞病(BD)患者血浆同型半胱氨酸水平与动静脉血栓形成间的关联性。方法检索1950年1月至2014年10月Pubmed、EMBASE、Cochrane Library、Web of Science、CNKI等中外文数据库上所有关于BD合并动静脉血栓形成的研究。按照纳入和排除标准纳入文献,并采用Rev Man5.2软件进行Meta分析。结果最终纳入11个研究,分析结果显示同型半胱氨酸血症在血栓组较无血栓组更普遍(比值比2.61,95%置信区间1.46~4.67,P=0.001),血栓组同型半胱氨酸的平均水平显著高于无血栓组(均数差=2.99,95%置信区间1.81~4.16,P【0.00001]。结论血浆高同型半胱氨酸增加BD患者的动静脉血栓形成风险。展开更多
文摘BACKGROUND Splenic rupture associated with Behçet’s syndrome(BS)is extremely rare,and there is no consensus on its management.In this case report,a patient with BSassociated splenic rupture was successfully treated with splenic artery embolization(SAE)and had a good prognosis after the intervention.CASE SUMMARY The patient was admitted for pain in the left upper abdominal quadrant.He was diagnosed with splenic rupture.Multiple oral and genital aphthous ulcers were observed,and acne scars were found on his back.He had a 2-year history of BS diagnosis,with symptoms of oral and genital ulcers.At that time,he was treated with oral corticosteroids for 1 month,but the symptoms did not alleviate.He underwent SAE to treat the rupture.On the first day after SAE,the patient reported a complete resolution of abdominal pain and was discharged 5 d later.Three months after the intervention,a computed tomography examination showed that the splenic hematoma had formed a stable cystic effusion,suggesting a good prognosis.CONCLUSION SAE might be a good choice for BS-associated splenic rupture based on good surgical practice and material selection.
文摘Behçet’s disease (BD) is a multi-systemic vasculitis characterized by a triad made up of oral aphthosis, genital aphthosis and uveitis which may or may not be associated with visceral involvement. Other times described in the ancient silk road, BD is increasingly found in countries south of the Sahara with the mixing of populations. Our objective was to describe the epidemiology and clinical characteristics of BD in an African country. Methodology: This was a five-year retrospective study in the internal medicine department of Donka National Hospital. We used international criteria finding BD. Urinary dipstick was use for finding kidney disease. Result: During the period, five cases of Behçet’s disease were collected. The average age was 35 years old. The male sex was more represented with 3 out of 5 cases. The most common clinical manifestation was oral aphthosis in all patients (100%), followed by genital aphthosis in 4 cases (80%). The other clinical manifestations observed were uveitis in 3 cases (60%), joint manifestations such as arthralgia in 3 cases (60%) and neurological manifestations such as chronic headache in one case (20%). HLA-B51 was found in two cases. The pattergic test done in two patients came back positive (100%). The patients received colchicine and oral corticosteroid therapy. Two patients were lost to sight. We deplored a death probably in a neuro-Behçet table before the rebellious headaches. Conclusion: Behçet’s disease is a multi-systemic vasculitis that must be evoked and managed quickly in a tropical environment, especially since the prognosis, vital and functional prognosis can be engaged.
文摘Aseptic abscesses are an emergent entity and have been described in inflammatory bowel disease,especially in Crohn's disease,and in other diseases.However,aseptic abscesses associated with Beh et's disease are extremely rare.We report a Japanese male diagnosed with an incomplete type of Beh et's disease who developed multiple aseptic abscesses of the spleen and liver.In 2002,the spleen abscesses were accompanied by paroxysmal oral aphthous ulcers and erythema nodosum.As the patient's response to antibiotic treatment was inadequate,a splenectomy was performed.Severe inflammatory cell infiltration,largely of polymorphonuclear neutrophils,was observed without evidence of bacterial or fungal growth.Although the patient had no history of ocular symptoms or genital ulcers,a diagnosis of incomplete Beh et's disease was made according to the Japanese diagnostic criteria because of the presence of paroxysmal arthritis and epididymitis since 2002.In 2005,multiple liver abscesses developed with right hypochondrial pain and seemed to be attributed to Beh et's disease because the abscesses yielded negative results during a microbiologic investigation and failed to go into remission under antibiotic therapy.Oral prednisone(15 mg/d) was started in May 2006,and the abscesses dramatically disappeared 4 wk after treatment.Although the patient had a relapse of the liver abscesses in association with the tapering of prednisone,the augmentation of prednisone dosage yielded a response.The abscesses of the liver and spleen were strongly suggested to be attributed to Beh et's disease.Clinician should be aware of the existence of aseptic abscesses as uncommon manifestations of Beh et's disease.
文摘TO THE EDITOR I have read with great interest the very recent article rifled ＂Intestinal Behcet＇s disease with pyoderma gangrenosum： A case report＂ of Nakamura T et al that was published in your journal. The authors stated that they presented a very rare case of intestinal Behcet＇s disease with pyoderma gangrenosum in a 16-year old patient. However, I would like to make some important contributions and suggestions to the presented case and have a few questions to ask the authors.
文摘BACKGROUND In Behçet’s disease(BD),very few cases of muscular involvement have been reported previously.The natural history and therapeutic protocol for muscular involvement in BD are obscure due to the low incidence of peripheral neuropathy or myopathy in BD.The purpose of our study was to report a rare case of BD with chronic,focal forms of neuromyopathy and review the relevant literature.CASE SUMMARY We herein report the case of a 54-year-old man who presented with progressive muscular atrophy and weakness of both thighs 2 years after the presentation of the cardinal symptoms of BD.The past medical history,electrophysiological study,neurological examination,blood tests,magnetic resonance imaging study,and histological exam were performed for the differential diagnosis.Relevant literature on muscular involvement in BD was reviewed.Neurological examination revealed that muscular involvement was predominantly localized in the proximal parts of the lower extremities.Heterogeneous enhancement of several thigh muscles was observed on magnetic resonance imaging,which corresponded with the clinical manifestations.Histological study of one of the enhanced muscles showed denervation atrophy of the muscle with superimposed myopathic changes,while electrophysiological studies only suggested denervation.CONCLUSION To our knowledge,this is the first case of neurogenic muscular atrophy with a specific set of clinical,radiological,electrophysiological,and histological findings reported in BD.
文摘Purpose: To analyze the clinic manifestation and prognosis of Behet disease. Method: Twenty patients requiring inpatient treatment with Beh?觭et disease were retrosp-ectively analyzed. Results: The morbidity of Beh?觭et disease is 5.5/100 000. In the systemic damage, stom-atocace and skin lesion are 95%, eye lesion and genital ulcer 50%, joint lesion 45%, gastrointestinal lesion 35%, Uveitis is the major disease in eye lesion, and followed in order by retinal vasculitis and obstruction of retinal artery. Attack age average 30.3 years old. Diagnosis age average 34.8 years old. The patients stay in hospital for 41 days on the average. Cure rate is 55%, improvement rate 40%, blinding rate of eye lesion is 36%. Conclusions: Beh?觭et disease is a multisystem lesion disease. Stomatocace and skin lesion is the major lesion, the next in common occurrence are eye and genital lesions. Repeated attack of uveitis, complicated cataract and secondary glaucoma are the major causes of blindness.
文摘<strong>Background:</strong> Beh<span style="white-space:nowrap;">ç</span>et’s disease (BD) is a multisystemic, chronic inflammatory disorder with a broad range of manifestations including within the cardiovascular system. Cardiac involvement like intracardiac thrombus (ICT) and valvular involvement in BD are rarely seen entities and often associated with poor prognosis. <strong>Case Presentation: </strong>We present the case of a young patient with vascular-Beh<span style="white-space:nowrap;">ç</span>et diagnosed by the presence of intracardiac thrombus in the right ventricle, unresponsive to medical treatment. Even though the intracardiac lesion was successfully treated by surgical excision, he presented a severe tricuspid dysfunction some years later due to the extension of fibrosis with no surgical therapeutic option. <strong>Conclusion:</strong> Intracardiac thrombosis is a rare but early manifestation of Beh<span style="white-space:nowrap;">ç</span>et’s disease, which is difficult to diagnose and has a poor prognosis.
文摘Having considered the impact of the function of TLR2 in there cognition of several microorganisms that are thought to havean association with disease(BD),we aimed to determinea possible association between the TLR2 Arg753Gln polymorphism and susceptibility to BD.We genotyped 83 patients with BD,95 ethnically matched healthy controls,12patients with recurrent aphthous stomatitis(RAS)and 21 patientswith rheumatoid arthritis(RA)by restriction fragment length polymorphism after PCR amplification of the genomicregion encompassing the polymorphic site.Comparison of the TLR2 Arg753Gln A allele and A/G genotype frequencies did not show a significant difference between patients with BD andhealthy controls(1.2%vs.0.6%,and 2.1%vs.1.1%,respectively).None of the patients from the RAS and RA groups had the A allele or A/G genotype.Our results indicate that the TLR2 Arg753Gln polymorphism does not play a role in the aetiopathogenesis of BD.
文摘Introduction:disease is a rare,chronic disorder.The cause of disease is unknown.It is believed to be caused by an autoimmune reaction.As in other chronic autoimmune diseases,disease may show a subclinical adrenal failure and some changes in cortisol levels.We aimed to evaluate adrenal gland function in disease patients.Material and method:This study included 18 disease patients and 15 healthy controls.Patient and control groups were administered i.v.1 μg low dose test(LDT)and 250 μg standarddose test(SDT)adrenocorticotropic hormone(ACTH)stimulation test after 12 h of night fasting with an interval of 3-days and cortisol responses in the 0th,30th and 60th minutes were evaluated.Results:There was no statistically significant difference between basal cortisol values of disease and control groups.Cortisol values in the 60th minute in LDT were significantly lower in disease group than in the control group.In the peak cortisol responses to LDT,a significant decrease was found in disease group.Conclusion:These findings suggest that hypothalamo-pituitary adrenal axis is partially suppressed in disease.
文摘Background:disease(BD)is a systemic inflammatory vasculitis of young adults with unknown etiology.Objective:The aim of this study was to determine the clinical features and outcome of BD in childhood.Patients and methods:Seventeen patients with BD within a pediatric age group were studied since 1997.Results:Boys :girls ratio was 12:5.Familial cases were very frequent(45%).Oral aphthous ulcers were presentin all patients(100%),genital aphthous ulcers were present in 16(94%),ocular lesions in 4(24%),erythema nodosum in 3(18%),papulopustules in 8(47%),joint symptoms in 13(76%),mild gastrointestinal symptoms in 2(12%),neurological involvement in 2(12%),positive pathergy reactions in 13(76%),and thrombophlebitis in 1(6%).We used colchicine,system iccorticosteroids,cyclosporine,nonsteroidal anti-inflammatory drugs,sulfasalazine,and topical agents for the treatment of children with BD.Conclusion:The findings of BD in children are similar as in adults,but the frequency of familial cases is significantly higher.
文摘目的系统评价白塞病(BD)患者血浆同型半胱氨酸水平与动静脉血栓形成间的关联性。方法检索1950年1月至2014年10月Pubmed、EMBASE、Cochrane Library、Web of Science、CNKI等中外文数据库上所有关于BD合并动静脉血栓形成的研究。按照纳入和排除标准纳入文献,并采用Rev Man5.2软件进行Meta分析。结果最终纳入11个研究,分析结果显示同型半胱氨酸血症在血栓组较无血栓组更普遍(比值比2.61,95%置信区间1.46~4.67,P=0.001),血栓组同型半胱氨酸的平均水平显著高于无血栓组(均数差=2.99,95%置信区间1.81~4.16,P【0.00001]。结论血浆高同型半胱氨酸增加BD患者的动静脉血栓形成风险。
