Sellouti Mohamed, Agadr Aomar, Abilkassem Rachid
Neonatology Division. Departement of Pediatrics, Mohammed V Military Teaching Hospital, Rabat, Morocco.
Mohammed V Military Teaching Hospital, Faculty of Medicine and Pharmacy, Casablanca, Morocco.
Radiol Case Rep. 2024 Oct 19;20(1):187-190. doi: 10.1016/j.radcr.2024.09.098. eCollection 2025 Jan.
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.
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。