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一名患有囊性纤维化的男性新生儿的复杂胎粪性肠梗阻:病例报告。

Complicated meconium ileus in a male neonate with cystic fibrosis: Case report.

作者信息

Sellouti Mohamed, Agadr Aomar, Abilkassem Rachid

机构信息

Neonatology Division. Departement of Pediatrics, Mohammed V Military Teaching Hospital, Rabat, Morocco.

Mohammed V Military Teaching Hospital, Faculty of Medicine and Pharmacy, Casablanca, Morocco.

出版信息

Radiol Case Rep. 2024 Oct 19;20(1):187-190. doi: 10.1016/j.radcr.2024.09.098. eCollection 2025 Jan.

DOI:10.1016/j.radcr.2024.09.098
PMID:39502278
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11535884/
Abstract

In about 10%-15% of instances, meconium ileus (MI) is the first sign of cystic fibrosis (CF). If a newborn exhibits signs of intestinal obstruction and does not pass meconium within a short period of time after birth, MI is suspected. The cystic fibrosis transmembrane conductance regulator gene (CFTR), which is found on chromosome 7q31, is mutated in CF patients. A premature baby, 5 days old, with clinical signs that were typical of MI. These attempts failed in spite of conservative therapies, such as rectal injection of gastrografin. An ileostomy was made and meconium was manually evacuated after an exploratory laparotomy revealed the presence of a typical MI. At ≥1300 µg/L (reference value ≤1000 µg/L), immunoreactive trypsinogen (IRT) levels were increased, which prompted the start of oral pancreatic enzyme replacement treatment. Discharge at 65 days of age included referrals to primary care and a specialized CF clinic. The baby has since shown normal growth and development. This case highlights the rapid onset of CF in a premature infant with complex MI as the initial clinical presentation.

摘要

在大约10%-15%的病例中,胎粪性肠梗阻(MI)是囊性纤维化(CF)的首发症状。如果新生儿出现肠梗阻迹象且出生后短时间内未排出胎粪,则怀疑为MI。位于7号染色体q31上的囊性纤维化跨膜传导调节基因(CFTR)在CF患者中发生突变。一名5天大的早产儿,具有典型的MI临床症状。尽管采取了保守治疗,如直肠注入泛影葡胺,但这些尝试均失败。在剖腹探查发现典型的MI后,进行了回肠造口术并手动排出胎粪。免疫反应性胰蛋白酶原(IRT)水平升高,≥1300µg/L(参考值≤1000µg/L),这促使开始口服胰酶替代治疗。65日龄出院时包括转诊至初级保健机构和一家专门的CF诊所。此后,该婴儿生长发育正常。本病例突出了以复杂的MI为初始临床表现的早产儿中CF的快速发病情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fe32/11535884/9ff4eb356936/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fe32/11535884/c9782c2b0ae2/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fe32/11535884/9ff4eb356936/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fe32/11535884/c9782c2b0ae2/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fe32/11535884/9ff4eb356936/gr2.jpg

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本文引用的文献

1
Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene.胎粪性肠梗阻的患病率标志着囊性纤维化跨膜传导调节因子(CFTR)基因突变的严重程度。
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The influence of genetics on cystic fibrosis phenotypes.遗传对囊性纤维化表型的影响。
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CFTR, mucins, and mucus obstruction in cystic fibrosis.囊性纤维化中的 CFTR、黏蛋白和黏液阻塞。
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Intestinal obstruction syndromes in cystic fibrosis: meconium ileus, distal intestinal obstruction syndrome, and constipation.囊性纤维化中的肠梗阻综合征:胎粪性肠梗阻、远端肠梗阻综合征和便秘。
Curr Gastroenterol Rep. 2011 Jun;13(3):265-70. doi: 10.1007/s11894-011-0185-9.
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Lymphocytic leiomyositis and myenteric ganglionitis are intrinsic features of cystic fibrosis: studies in distal intestinal obstruction syndrome and meconium ileus.淋巴细胞性平滑肌瘤病和肌间神经节炎是囊性纤维化的内在特征:远端肠梗阻综合征和胎粪性肠梗阻的研究
J Pediatr Gastroenterol Nutr. 2009 Jul;49(1):42-51. doi: 10.1097/MPG.0b013e318186d35a.
7
Meconium ileus in patients with cystic fibrosis is not a risk factor for clinical deterioration and survival: the Israeli Multicenter Study.囊性纤维化患者的胎粪性肠梗阻不是临床恶化和生存的危险因素:以色列多中心研究。
J Pediatr Gastroenterol Nutr. 2010 Feb;50(2):173-8. doi: 10.1097/MPG.0b013e3181a3bfdd.
8
An official ATS statement: grading the quality of evidence and strength of recommendations in ATS guidelines and recommendations.美国胸科学会官方声明:对美国胸科学会指南及建议中的证据质量和推荐强度进行分级
Am J Respir Crit Care Med. 2006 Sep 1;174(5):605-14. doi: 10.1164/rccm.200602-197ST.
9
Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes.囊性纤维化的手术考量:32年疗效评估
Surgery. 2005 Oct;138(4):560-71; discussion 571-2. doi: 10.1016/j.surg.2005.06.049.
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Cystic fibrosis -- therapeutic challenge in cystic fibrosis children.囊性纤维化——囊性纤维化患儿面临的治疗挑战。
Eur J Endocrinol. 2004 Aug;151 Suppl 1:S77-80. doi: 10.1530/eje.0.151s077.