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[囊性纤维化的腹部影像学检查]

[Abdominal imaging in cystic fibrosis].

作者信息

Mentzel Hans-Joachim, Renz Diane Miriam

机构信息

Sektion Pädiatrische Radiologie, Institut für Diagnostische und Interventionelle Radiologie, Universitätsklinikum Jena, Am Klinikum 1, 07747, Jena, Deutschland.

Arbeitsbereich Kinderradiologie, Institut für Diagnostische und Interventionelle Radiologie, Medizinische Hochschule Hannover, Hannover, Deutschland.

出版信息

Radiologe. 2020 Sep;60(9):831-838. doi: 10.1007/s00117-020-00703-4.

Abstract

CLINICAL ISSUE

Abdominal complications are often the first indications for cystic fibrosis (CF), a multiorgan disease. A broad range of abdominal manifestations are associated with the disease, including gastrointestinal abnormalities (such as meconium ileus in newborns and distal intestinal obstruction syndrome in older children) and hepatobiliary alterations (e.g., cholelithiasis, microgallbladder, hepatosteatosis, biliary cirrhosis). A characteristic finding is pancreatic involvement, which leads to exocrine and over the course of time to endocrine insufficiency.

STANDARD RADIOLOGICAL METHODS

Ultrasonography is the preferred and often sole modality for a precise diagnosis of abdominal CF manifestations. However, all imaging modalities can be used, depending on the pathology: X‑ray, fluoroscopic examinations, computed tomography, magnetic resonance imaging (also with application of magnetic resonance cholangiopancreatography).

METHODICAL INNOVATIONS/PERFORMANCE: Scoring systems are useful for standardized diagnostics. Sonographic findings, described using a scoring system, correlate with clinical symptoms, such as pancreatic lipomatosis with abdominal pain (p = 0.018), flatulence (p = 0.006), and gastroesophageal reflux (p = 0.006).

EVALUATION/PRACTICAL RECOMMENDATIONS: A standardized approach with structured reporting is important due to the numerous abdominal CF manifestations. To enable precise follow-up analyses, scoring systems based on sonographic findings are excellent.

摘要

临床问题

腹部并发症往往是囊性纤维化(CF)这一多器官疾病的首要表现。该疾病伴有广泛的腹部表现,包括胃肠道异常(如新生儿胎粪性肠梗阻和大龄儿童的远端肠梗阻综合征)以及肝胆改变(如胆石症、小胆囊、肝脂肪变性、胆汁性肝硬化)。一个特征性表现是胰腺受累,这会导致外分泌功能不全,随着时间推移还会导致内分泌功能不全。

标准放射学方法

超声检查是精确诊断腹部CF表现的首选且通常是唯一的检查方式。然而,根据病理情况,所有成像方式均可使用:X线、透视检查、计算机断层扫描、磁共振成像(也可应用磁共振胰胆管造影)。

方法学创新/性能:评分系统有助于标准化诊断。使用评分系统描述的超声检查结果与临床症状相关,如胰腺脂肪化生与腹痛(p = 0.018)、肠胃胀气(p = 0.006)和胃食管反流(p = 0.006)相关。

评估/实际建议:由于腹部CF表现众多,采用结构化报告的标准化方法很重要。基于超声检查结果的评分系统非常适合进行精确的随访分析。

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