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一名患有新月体性肾小球肾炎、心律失常、急性胃肠道出血和神经并发症的青少年的罕见且严重的过敏性紫癜表现。

A Rare and Severe Presentation of Henoch-Schönlein Purpura in an Adolescent With Crescentic Glomerulonephritis, Arrhythmia, Acute Gastrointestinal Bleed, and Neurological Complications.

作者信息

Shah Siddharth, Hata Jessica

机构信息

Pediatric Nephrology, Norton Children's Hospital, Louisville, USA.

Pathology, Norton Children's Hospital, Louisville, USA.

出版信息

Cureus. 2021 Mar 29;13(3):e14169. doi: 10.7759/cureus.14169.

Abstract

Henoch-Schönlein purpura (HSP) is a childhood vasculitis disorder that involves the skin, joints, gastrointestinal (GI) tract, and kidneys. It is related to immunoglobulin A (IgA) antibody deposition in small blood vessels. HSP is a self-limiting disorder, but its morbidity is primarily associated with renal involvement. GI pathologies like intussusception, gastritis, duodenitis, ileitis, or ulcer have been reported to be associated with this disease. However, cardiac and neurological complications are rarely reported. We present the case of a 16-year-old, previously healthy male who was diagnosed with HSP after presenting with a non-blanching purpuric rash in the lower extremities. The patient also had joint and abdominal pain, and swelling in the extremities. There was renal dysfunction at presentation with blood urea nitrogen (BUN) of 67 mg/dL and serum creatinine of 1.9 mg/dL. The serum albumin was low at 2 g/dL, and the patient had nephrotic range proteinuria. Urine microscopy showed red blood cell casts. A renal biopsy was performed, which showed IgA deposition in glomeruli. He was started on intravenous (IV) pulse methylprednisolone and was later prescribed oral steroids. Four weeks after the treatment initiation, he presented with syncope and acute anemia (hemoglobin of 3.5 g/dL). The fecal occult blood was positive. Esophagogastroduodenoscopy (EGD) was not suggestive of gastritis, duodenitis, or ulcer. The pill-cam capsule endoscopy revealed GI bleeding from the terminal ileum near Meckel's diverticulum. He subsequently required blood transfusions, and the bleeding eventually improved with symptomatic management. Six weeks after treatment initiation, he presented with dizziness and palpitations. The EKG showed the presence of atrial fibrillation, and he had an episode of non-sustained ventricular tachycardia on telemetry. Arrhythmia was diagnosed secondary to HSP cardiac vasculitis, and we initiated treatment with metoprolol and amiodarone. Seven weeks after the initial treatment, he had neurological clinical findings of proximal muscle weakness, tremors, and upper and lower extremity clonus. A second renal biopsy was then performed due to the presence of persistently elevated serum creatinine, which showed 75% of glomeruli with cellular crescents. He was treated with IV cyclophosphamide. Subsequently, the renal function improved. There were no other GI, cardiac, or neurological complications after six months of follow-up. The presentation of HSP can be more severe in adolescents, and they need to be closely monitored for GI, cardiac, renal, and neurological complications after the disease occurrence. Bleeding from Meckel's diverticulum or an episode of non-sustained ventricular tachycardia with HSP has not been previously reported to our knowledge. Arrhythmia is an exceptionally unusual occurrence in HSP, and it is usually treated with anti-arrhythmic drugs and intensification of the immunosuppressive regimen.

摘要

过敏性紫癜(HSP)是一种儿童血管炎疾病,累及皮肤、关节、胃肠道(GI)和肾脏。它与免疫球蛋白A(IgA)抗体在小血管中的沉积有关。HSP是一种自限性疾病,但其发病率主要与肾脏受累有关。据报道,肠套叠、胃炎、十二指肠球炎、回肠炎或溃疡等胃肠道病变与该疾病有关。然而,心脏和神经并发症很少被报道。我们报告了一例16岁、既往健康的男性病例,该患者在出现下肢非苍白性紫癜皮疹后被诊断为HSP。患者还伴有关节和腹痛以及四肢肿胀。就诊时存在肾功能不全,血尿素氮(BUN)为67mg/dL,血清肌酐为1.9mg/dL。血清白蛋白低至2g/dL,患者有肾病范围的蛋白尿。尿显微镜检查显示有红细胞管型。进行了肾活检,结果显示肾小球中有IgA沉积。他开始接受静脉注射(IV)甲泼尼龙冲击治疗,随后被开了口服类固醇药物。治疗开始四周后,他出现晕厥和急性贫血(血红蛋白为3.5g/dL)。粪便潜血呈阳性。食管胃十二指肠镜检查(EGD)未提示胃炎、十二指肠球炎或溃疡。胶囊内镜检查发现靠近梅克尔憩室的回肠末端有胃肠道出血。他随后需要输血,经对症治疗后出血最终得到改善。治疗开始六周后,他出现头晕和心悸。心电图显示存在心房颤动,遥测发现他有一次非持续性室性心动过速发作。心律失常被诊断为继发于HSP心脏血管炎,我们开始用美托洛尔和胺碘酮进行治疗。初始治疗七周后,他出现近端肌无力、震颤以及上下肢阵挛等神经学临床表现。由于血清肌酐持续升高,于是进行了第二次肾活检,结果显示75%的肾小球有细胞性新月体形成。他接受了静脉注射环磷酰胺治疗。随后,肾功能得到改善。随访六个月后未出现其他胃肠道、心脏或神经并发症。HSP在青少年中的表现可能更严重,疾病发生后需要密切监测其胃肠道、心脏、肾脏和神经并发症。据我们所知,此前尚未报道过梅克尔憩室出血或HSP伴非持续性室性心动过速发作的情况。心律失常在HSP中极为罕见,通常用抗心律失常药物治疗并强化免疫抑制方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1f9/8006498/97a0890a8c17/cureus-0013-00000014169-i01.jpg

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