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[伴有严重多系统受累的过敏性紫癜]

[The Schönlein-Henoch syndrome with severe multisystemic involvement].

作者信息

Bosio M, Ravelli A, Ruperto N, Migliori C, Perotti F, Scotta M S, Martini A

机构信息

Divisione di Pediatria, Ospedale Provinciale G. Fornaroli, Magenta, Milano.

出版信息

Minerva Pediatr. 1993 May;45(5):197-201.

PMID:8366849
Abstract

A case of Henoch-Schönlein purpura (HSP) characterized by several unusual complications and exceedingly prolonged course is reported. A 6-year-old boy, hospitalized with a typical clinical picture of HSP, developed after a few days a severe gastrointestinal vasculitis leading to digestive hemorrhages and food intolerance. The treatment included continuous gastrointestinal infusion, parenteral nutrition, and corticosteroids during the exacerbations of symptoms. Intestinal ultrasonography showed diffuse submucous edema and widespread mural hematomas; hydrops of the gallbladder and edematous swelling of the pancreas were also detected. A gastro-duodenoscopy revealed diffuse and severe mucosal vasculitis with ulcers and petechial lesions. One week after admission the occurrence of weight gain, hyponatremia, and hypoprotidemia in the absence of proteinuria suggested a protein-losing enteropathy. The elevation of serum and urine amylases was consistent with pancreatic involvement. The course was characterized by recurrent exacerbations of gastrointestinal manifestations; symptoms subsided slowly to such a degree that oral feeding could be gradually restarted only seven weeks after admission. The use of high-frequency intestinal ultrasonography proved useful and sensitive in monitoring the evolution of intestinal involvement. During hospitalization the child complained of acute painful scrotal swelling. Ultrasonography showed scrotal hemorrhage and testicular swelling but no signs of testicular torsion, thus helping to avoid surgical exploration. In the recovery phase the child complained of colicky abdominal pain with vomiting. A plain abdominal roentgenogram showed bilateral staghorn pelvic urolithiasis. This finding was confirmed by an intravenous pyelography which also revealed a bilateral pyeloureteritis with edema of the ureteral wall and partial stenosis of the lumen.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

本文报告一例以多种罕见并发症及病程极长为特征的过敏性紫癜(HSP)病例。一名6岁男孩因典型的HSP临床表现入院,数日后出现严重的胃肠道血管炎,导致消化道出血和食物不耐受。症状加重期间,治疗包括持续胃肠内输注、肠外营养和使用皮质类固醇。肠道超声显示弥漫性黏膜下水肿和广泛的壁层血肿;还检测到胆囊积水和胰腺水肿。胃肠十二指肠镜检查显示弥漫性重度黏膜血管炎伴溃疡和瘀点性病变。入院一周后,在无蛋白尿的情况下出现体重增加、低钠血症和低蛋白血症,提示蛋白丢失性肠病。血清和尿淀粉酶升高与胰腺受累相符。病程特点为胃肠道表现反复加重;症状缓解缓慢,以至于入院七周后才逐渐重新开始经口喂养。高频肠道超声检查在监测肠道受累的演变过程中被证明是有用且敏感的。住院期间,患儿主诉阴囊急性疼痛性肿胀。超声检查显示阴囊出血和睾丸肿胀,但无睾丸扭转迹象,从而避免了手术探查。在恢复阶段,患儿主诉腹部绞痛伴呕吐。腹部平片显示双侧鹿角形盆腔尿路结石。静脉肾盂造影证实了这一发现,同时还显示双侧肾盂输尿管炎,输尿管壁水肿,管腔部分狭窄。(摘要截取自250词)

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