Atresia of the gastrointestinal tract: imaging evaluation. A wide spectrum of congenital anomalies may affect the gastrointestinal tract at any level from the esophagus to the anus. Atresia is an important cause of gastrointestinal obstruction with high morbidity rate in neonates. Different pathogenetic mechanisms could cause this malformation and the two classical explanations are: a defect of recanalization of the intestinal tube or an interruption of blood supply during intrauterine life. The authors present a literature review with an iconographic essay of imaging findings in children with gastrointestinal atresia. Key words: Atresia; Children; Pediatrics; Imaging.
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The exploratory laparotomy in the first day of life identified meconium ileus and secondary peritonitis. Ileal resection and ileostomy were performed, followed by reconstruction of the bowel transit at 20 days of life.
At 35 days of age he was discharged with referrals to primary care and to a special clinic for CF for the determination of sweat chloride. He was received in the outpatient clinic for neonatal screening for CF at 65 days of life presenting malnutrition and respiratory distress. This case illustrates the rapid evolution of CF in a premature patient with complex meconium ileus as the first clinical manifestation.
Cystic Fibrosis CF is the most prevalent lethal autosomal recessive disorder, affecting , caucasians. It is caused by the alteration of a gene located on the long arm of chromosome 7 that encodes a protein of 1, amino acids, the cystic fibrosis transmembrane conductance regulator CFTR , which functions as a chloride channel on the apical membrane of epithelial cells.
There are more than 1, known genetic mutations, as well as disease modifying genes. The classic presentation of CF is chronic lung disease recurrent pulmonary infections , exocrine pancreatic insufficiency diarrhea and malnutrition , loss of salt, and obstructive azoospermia syndrome.
This clinical picture is caused by obstruction of the terminal ileus with thick meconium containing high amounts of protein. Complex MI is a severe condition, significantly more frequent in patients without CF of lower gestational age and birth weight than in patients with CF.
MI is classified as complex when associated with ileal perforation. The objective of the present communication is to report the case of a child with complex MI who had a poor early evolution despite the clinical suspicion of CF. A male infant was received in the outpatient clinic for neonatal screening for CF at 65 days of life. He was born by vaginal delivery, weighing 2,g at 33 weeks and 5 days of gestational age, with Apgar scores of 6 and 9.
His mother was a year-old primigravida who had attended nine prenatal consultations with negative serology for vertical infections and normal obstetrical ultrasound. Immediately after birth, the infant was referred to the neonatal critical care unit due to early respiratory distress and severe abdominal distension.
A plain abdominal radiograph revealed absence of air in the lower abdomen. The patient underwent exploratory laparotomy in the first day of life, which identified MI and secondary peritonitis due to intestinal perforation. He had early and late neonatal sepsis during the hospital stay, requiring prolonged antibiotic therapy. At 35 days of age he was weighing 2, g and was discharged with referrals to primary care and to a special clinic for CF for the determination of sweat chloride.
That same day, the patient was attended at the follow-up clinic for neonatal screening for CF. According to his mother, the same guidelines were maintained regarding the volume of infant formula and the dosage of pancreatic enzymes, which she was instructed to offer mashed in water.
He weighed 2, g and his length was 48cm. His chest X-ray revealed pulmonary hyperinflation and condensation compatible with pneumonic disease. The patient was hospitalized and the laboratory tests performed during hospitalization are shown in Table 1.
The C-reactive protein CRP test performed at admission was Laboratory tests for respiratory syncytial virus, adenovirus and influenza, as well as blood cultures were negative.
A pulmonary CT scan was performed on the 6th day of hospital stay Fig. The dose of pancreatic enzymes was adjusted to 5, U before feeding, every 3 hours. A transfusion of red blood cells was performed. Due to ileal resection, vitamin B12 mcg was administered intramuscularly. On the seventh day of hospital stay, due to progressive worsening of the respiratory pattern, noninvasive ventilation CPAP was applied for two weeks. After stabilization of pulmonary disease he progressed with satisfactory weight gain.
It is known that the confirmation of CF may be difficult during the first days of life, but in the presence of meconium ileus this differential diagnosis should be compulsorily considered. The sweat chloride test can be performed after 48 hours of life, but more reliable results are obtained after the second week of life, with the patient weighing more than 2 kg, showing adequate hydration status and without significant systemic disease.
Cystic fibrosis transmembrane conductance regulator CFTR genetic analysis also helps the diagnosis of CF when it detects two known mutations. Neonates with MI should receive specific treatment for pancreatic insufficiency while pending confirmation of CF by the sweat chloride test. Weight gain during hospital stay indicates a good response. This procedure illustrates the importance of an early and frequent monitoring of children with a suspected chronic, rare and severe disease by the reference service for CF treatment, both regarding support to the family and the refinement of the guidelines offered, so that they will be closely followed.
It should be emphasized that this type of care involves, but does not replace patient monitoring by a general pediatrician. The management of pancreatic insufficiency involves the oral ingestion of intact microspheres of pancreatic enzymes immediately before breastfeeding, ranging from 2, to 4,IU lipase for each mL of formula or breastfeeding.
Pulmonary inflammation or infection may already be present, with Staphylococcus aureus being the microorganism most frequently detected, followed by Pseudomonas aeruginosa , with significant respiratory symptoms. Researchers observed that infants with cystic fibrosis detected by newborn screening may have lung disease with bacterial infection since the first days of life. The present case report illustrates the rapid multisystem evolution of CF in a premature patient with complex MI as the first clinical manifestation.
Furthermore, it emphasizes the importance of a rapid referral of patients with MI and suspected CF to a team of experts. Funding This study did not receive funding. O paciente nasceu por parto vaginal, pesava 2. Aos 35 dias de idade, ele pesava 2. Ele pesava 2. Evitam-se doses mais elevadas do que 2. Read article at publisher's site DOI : To arrive at the top five similar articles we use a word-weighted algorithm to compare words from the Title and Abstract of each citation.
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Affiliations 7 authors 1. Share this article Share with email Share with twitter Share with linkedin Share with facebook. This case illustrates the rapid evolution of CF in a premature patient with complex Meconium ileus as the first clinical manifestation. Free full text. Rev Paul Pediatr. PMID: Author information Article notes Copyright and License information Disclaimer. E-mail: moc. Del Ciampo. Conflicts of interest The authors declare no conflicts of interest.
Received Oct 3; Accepted Dec Copyright notice. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Go to:. Keywords: Cystic fibrosis, Meconium ileus, Prematurity. Table 1. Exams performed at the beginning and at the end of hospitalization of the patient at the referral service for CF management. Open in a separate window.
Figure 1. Pulmonary computer scanner images.
ILEO MECONIAL PEDIATRIA PDF
Johanna Pacheco A. INTA, Chile. Dos factores que probablemente contribuyen al DIOS son la renuencia a seguir el esquema con base en enzimas, y el control inapropiado de la esteatorrea. Como se puede ver, para el manejo de DIOS en pacientes con FQ son necesarias futuras investigaciones que sustenten estas propuestas bajo evidencia. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Intestinal obstruction syndromes in cystic fibrosis: Meconium ileus, distal intestinal obstruction syndrome, and constipation.
Early manifestations of cystic fibrosis in a premature patient with complex meconium ileus at birth
Since , it publishes original contributions, case reports and review of clinical research with methodological approach in the areas of health and disease of neonates, infants, children and adolescents. The objective is to disseminate worldwide the Brazilian research with methodological quality on issues that comprise the health of children and adolescents. All articles are available in Portuguese and in English. To report a case of a preterm infant with complex meconium ileus at birth and cystic fibrosis.. A male infant was born by vaginal delivery at 33 weeks and 5 days of gestational age with respiratory distress and severe abdominal distension. The exploratory laparotomy in the first day of life identified meconium ileus and secondary peritonitis. Ileal resection and ileostomy were performed, followed by reconstruction of the bowel transit at 20 days of life.
To report a case of a preterm infant with complex meconium ileus at birth and cystic fibrosis. A male infant was born by vaginal delivery at 33 weeks and 5 days of gestational age with respiratory distress and severe abdominal distension. The exploratory laparotomy in the first day of life identified meconium ileus and secondary peritonitis. Ileal resection and ileostomy were performed, followed by reconstruction of the bowel transit at 20 days of life. At 35 days of age he was discharged with referrals to primary care and to a special clinic for CF for the determination of sweat chloride. He was received in the outpatient clinic for neonatal screening for CF at 65 days of life presenting malnutrition and respiratory distress.