关键词: Allied disorders of Hirschsprung’s disease Clinical characteristics Emergency department Misdiagnosis and mistreatment Timely diagnosis

来  源:   DOI:10.4240/wjgs.v14.i7.656   PDF(Pubmed)

Abstract:
BACKGROUND: In the past years, only a few studies with a limited number of adult patients analyzed clinical features of allied disorders of Hirschsprung\'s disease (ADHD), most of which were individual case reports or lacked detailed clinical information. Although many studies have reported patients presenting to the emergency department (ED) with recurrent abdominal symptoms for a number of disorders, there are few data involving ADHD. However, owing to a lack of awareness of the disease, misdiagnoses and mistreatments are common. Severe complications such as perforation, bleeding, malabsorption, and even death in ADHD had been reported by many studies.
OBJECTIVE: To assist ED clinicians in having a more comprehensive understanding of this disease and making an early suspected diagnosis of ADHD more effectively.
METHODS: We enrolled 53 patients who visited the ED and were eventually diagnosed with ADHD over the past 11 years in our hospital. Their basic information, clinical manifestations, and imaging findings were analyzed. Blood indices were compared between the ADHD and irritable bowel syndrome (IBS) groups.
RESULTS: Adult patients with ADHD had a mean age of 48.8 ± 14.3 years, and 77.4% had been treated before admission. The transverse colon was the most common dilated part (73.6%), and constipation (67.9%) was the most common symptom. ADHD patients can present with uncommon symptoms and false-negative imaging findings. Logistic regression analysis indicated that body mass index (BMI) [odds ratio (OR) = 0.786, P = 0.013], cholinesterase (per 1000 units; OR = 0.693, P = 0.008), and blood chlorine (OR = 0.816, P = 0.022) were determined to be independent related factors between the ADHD and IBS groups. The area under the receiver operating characteristics curve of these three indices combined was 0.812 (P < 0.001).
CONCLUSIONS: Emergency physicians should be vigilant regarding patients with chronic constipation, abdominal pain, or abdominal distension, and consider the possibility of ADHD despite its rarity. Abdominal computed tomography examination is recommended as a useful tool in the suspected diagnosis of ADHD. BMI, cholinesterase, and blood chlorine have good discriminative abilities between ADHD and IBS. The nutritional status of adult patients with ADHD is worthy of further attention. Surgical treatment for adult patients with ADHD is important and inevitable.
摘要:
背景:在过去的几年中,只有少数研究对数量有限的成人患者进行了分析,分析了先天性巨结肠病(ADHD)相关疾病的临床特征,其中大部分为个案报告或缺乏详细的临床资料.尽管许多研究报告了急诊(ED)就诊的患者因多种疾病而反复出现腹部症状,涉及多动症的数据很少。然而,由于对这种疾病缺乏认识,误诊和虐待是常见的。严重的并发症,如穿孔,出血,吸收不良,许多研究报告甚至多动症患者死亡。
目的:帮助临床医师更全面地了解本病,更有效地对多动症进行早期疑似诊断。
方法:我们招募了53名在我院就诊并在过去11年中最终诊断为ADHD的患者。他们的基本信息,临床表现,和影像学检查结果进行了分析。比较ADHD和肠易激综合征(IBS)组之间的血液指标。
结果:成年多动症患者的平均年龄为48.8±14.3岁,77.4%在入院前接受过治疗。最常见的扩张部位是横结肠(73.6%),便秘(67.9%)是最常见的症状。ADHD患者可出现罕见症状和假阴性影像学表现。Logistic回归分析显示体重指数(BMI)[比值比(OR)=0.786,P=0.013],胆碱酯酶(每1000个单位;OR=0.693,P=0.008),血氯(OR=0.816,P=0.022)是ADHD组和IBS组的独立相关因素。这三个指标组合的受试者工作特征曲线下面积为0.812(P<0.001)。
结论:急诊医师应警惕慢性便秘患者,腹痛,或者腹胀,并考虑多动症的可能性,尽管它的稀有性。建议将腹部计算机断层扫描检查作为可疑诊断ADHD的有用工具。BMI,胆碱酯酶,血氯对ADHD和IBS有很好的辨别能力。成人ADHD患者的营养状况值得进一步关注。成人ADHD患者的手术治疗是重要且不可避免的。
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