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先天性中胚层肾瘤伴大量血尿及失血性休克1例报告

Congenital mesoblastic nephroma presenting with massive hematuria and hemorrhagic shock: report of one case.

作者信息

Hu Jui-Ming, Wu Trang-Tiau, Chan Si-Wa, Cheng Sun-Long, Chen Shan-Ming, Sheu Ji-Nan

机构信息

Department of Pediatrics, Chung Shan Medical University Hospital, No. 110 Chien-Kuo North Road, Section 1, Taichung 402, Taiwan.

出版信息

Acta Paediatr Taiwan. 2006 May-Jun;47(3):135-8.

Abstract

Congenital mesoblastic nephroma (CMN) is a rare benign tumor that occurs during the neonatal period and early infancy. The vast majority of these tumors present as asymptomatic palpable abdominal masses. We describe an unusual presentation of a CMN in a 10-month-old male infant who presented with massive hematuria and the development of hemorrhagic shock. Abdominal ultrasound showed a heterogeneous solid complex mass measuring 4.8 x 3.5 cm arising from the upper pole of the left kidney. The patient was resuscitated using intravenous fluids and blood transfusions because persistent massive bloody urine leading to progressive shock occurred the night of the admission day. Preoperative diagnosis was possible Wilms tumor of the left kidney. The histopathological findings were consistent with the character of a cellular variant of CMN. The patient was free of recurrence and metastasis at the 2-year follow-up examination. Our case report suggests that CMN is a rare benign renal tumor during infancy and may present with unusual massive hematuria and shock.

摘要

先天性中胚层肾瘤(CMN)是一种罕见的良性肿瘤,发生于新生儿期和婴儿早期。这些肿瘤绝大多数表现为无症状的可触及腹部肿块。我们描述了一名10个月大男婴CMN的不寻常表现,该患儿出现大量血尿并发展为失血性休克。腹部超声显示一个大小为4.8×3.5 cm的不均匀实性复合肿块,起源于左肾上极。入院当晚,由于持续大量血尿导致进行性休克,患者通过静脉输液和输血进行复苏。术前诊断可能为左肾威尔姆斯瘤。组织病理学检查结果与CMN细胞变异型的特征一致。在2年的随访检查中,患者无复发和转移。我们的病例报告表明,CMN是婴儿期罕见的良性肾肿瘤,可能表现为不寻常的大量血尿和休克。

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