Abnormal control of lung branching in experimental esophageal atresia

Fragoso, Ana Catarina; Aras-López, Rosa; Martínez, Leopoldo; Estevão‐Costa, José; Tovar, Juan A.

doi:10.1007/s00383-012-3195-2

Cited by 6 publications

(7 citation statements)

References 25 publications

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“…MID1 (a member of the RING-B box-coiled coil subgroup of RING finger proteins) is expressed in lung and esophageal epithelium of human embryos, and experimental data suggest a negative influence in Shh expression (50, 51). Finally, Gli3 is a transcription factor that mediates the Shh signaling, a critical pathway in foregut and lung morphogenesis (mutant mice for Gli3 exhibit tracheoesophageal and lung defects) (40, 52, 53). …”

Section: Mechanisms Of Ea ± Tefmentioning

confidence: 99%

The Multifactorial Origin of Respiratory Morbidity in Patients Surviving Neonatal Repair of Esophageal Atresia

Fragoso

Tovar

2014

Front. Pediatr.

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Esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) occurs in 1 out of every 3000 births. Current survival approaches 95%, and research is therefore focused on morbidity and health-related quality of life issues. Up to 50% of neonates with EA ± TEF have one or more additional malformations including those of the respiratory tract that occur in a relatively high proportion of them and particularly of those with vertebral, anal, cardiac, tracheoesophageal, renal, and limb association. Additionally, a significant proportion of survivors suffer abnormal pulmonary function and chronic respiratory tract disease. The present review summarizes the current knowledge about the nature of these symptoms in patients treated for EA ± TEF, and explores the hypothesis that disturbed development and maturation of the respiratory tract could contribute to their pathogenesis.

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Section: Mechanisms Of Ea ± Tefmentioning

confidence: 99%

The Multifactorial Origin of Respiratory Morbidity in Patients Surviving Neonatal Repair of Esophageal Atresia

Fragoso

Tovar

2014

Front. Pediatr.

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“…Two of the most relevant FGFs involved in lung morphogenesis are FGF10 and FGF7 (keratinocyte growth factor) that share the same mesenchymal FGFR2IIIb receptor. Indeed, abnormal FGF10 signaling has been reported in the lungs from rat fetuses with EA-TEF [13]. FGF7 stimulates lung fluid secretion that is critical for lung branching and growth and latter on, promotes maturation of fetal alveolar type II cells and consequently surfactant lipid and protein expression [17,18].…”

Section: Discussionmentioning

confidence: 99%

Lung maturity in esophageal atresia: Experimental and clinical study

Fragoso

Martínez

Estevão‐Costa

et al. 2015

Journal of Pediatric Surgery

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“…Although several pathways (e.g. Shh, FGF10) are reported to have been altered in the lungs of fetal rats with EA-TEF [25,26], the precise mechanisms of impaired airway branching are still unclear.…”

Section: Discussionmentioning

confidence: 99%

Impaired VEGF Signaling in Lungs with Hypoplastic Esophageal Atresia and Effects on Branching Morphogenesis

Liu

et al. 2016

Cell Physiol Biochem

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Background/Aims: Patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) often suffer chronic respiratory tract disease. We previously reported that primary lung maldevelopment caused by deficient branching of embryonal airways in experimental EA-TEF was induced by Adriamycin. In this study, we investigated the Vascular endothelial growth factor (VEGF) pathway in the developing lung in an EA-TEF rat model. We further analyzed the effect of recombinant VEGF treatment in vitro on branching morphogenesis of embryo lungs in experimental EA-TEF. Methods: Pregnant rats received either Adriamycin or vehicle on E7, E8 and E9. Lungs were recovered at E15, E18 and E21. Expression of VEGF and receptors (Flk-1 and Flt-1) were assessed by quantitative PCR, immunohistochemistry and immunoblotting. E13 lungs were cultured for 72 hours with 50 ng/mL of recombinant rat VEGF in serum-free medium. The rates of increase in bud count and airway contour were evaluated. Results: Our results showed a significant downregulation of VEGF during pseudoglandular and canalicular stages. In contrast, there were significantly higher levels of the Flt-1 receptor in the canalicular stage, which may represent a compensatory response to decreased VEGF. However, both variables returned to normal levels at the saccular stage. Exogenous VEGF treatment enhanced hypoplastic lung growth, evidenced by the increase in bud count and airway contour. Conclusions: A VEGF signaling defect possibly plays an important role in defective embryonic airway branching. Additionally, VEGF treatment may accelerate lung growth in EA-TEF lungs.

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Abnormal control of lung branching in experimental esophageal atresia

Cited by 6 publications

References 25 publications

The Multifactorial Origin of Respiratory Morbidity in Patients Surviving Neonatal Repair of Esophageal Atresia

The Multifactorial Origin of Respiratory Morbidity in Patients Surviving Neonatal Repair of Esophageal Atresia

Lung maturity in esophageal atresia: Experimental and clinical study

Impaired VEGF Signaling in Lungs with Hypoplastic Esophageal Atresia and Effects on Branching Morphogenesis

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