Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy

Tsao, KuoJen; Hawgood, Samuel; Vu, Lan; Hirose, Shinjiro; Sydorak, Roman M.; Albanese, Craig T.; Farmer, Diana L.; Harrison, Michael R.; Lee, Hanmin

doi:10.1053/jpsu.2003.50089

Cited by 147 publications

(75 citation statements)

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“…Patients with CCAM and nonimmune hydrops fetalis with predicted mortality did not undergo fetal intervention, and were treated instead with prenatal betamethasone to increase lung maturity. After a course of prenatal steroids during the second trimester, patients had resolution of hydrops and were delivered without respiratory distress in most cases (28,29). The exact mechanism of the improvement in these patients' pulmonary status and resolution of CCAM after steroid administration is unknown.…”

Section: Discussionmentioning

confidence: 99%

Genetic Analysis of Congenital Cystic Adenomatoid Malformation Reveals a Novel Pulmonary Gene: Fatty Acid Binding Protein-7 (Brain Type)

et al. 2008

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ABSTRACT:The pathogenesis of congenital cystic adenomatoid malformation (CCAM) is unknown and its natural history is unpredictable. Fatty acid binding protein-7 (FABP-7) has been previously described in brain and breast development, but never before in the lung. We investigate gene expression in CCAM, and hypothesize that CCAM results from an aberration in the signaling pathway during lung development. Under IRB approval, tissue specimens of fetal CCAM, fetal control, postnatal CCAM, and postnatal control were examined and microarray analysis was performed. Candidate differentially expressed genes were selected with log-odds ratio (B) Ͼ0 and false discovery rate Ͻ0.05. Validation of differential expression was achieved at the RNA and protein levels. FABP-7 was underexpressed in fetal CCAM compared with fetal lung in both the microarray and by RT-PCR. Findings were duplicated by Western Blot analysis and immunohistochemistry. This is the first description of FABP-7 in the human lung. Decreased expression of FABP-7 in fetal CCAM compared with normal fetal lung at both the RNA and protein levels suggests FABP-7 may have a role in pulmonary development and in the pathogenesis of CCAM. (Pediatr Res 64: 11-16, 2008) C ongenital cystic adenomatoid malformation (CCAM) is a multicystic pulmonary mass characterized by an abnormal proliferation of tissue resembling terminal respiratory bronchioles. The relatively rare lesions usually occur unilaterally and involve only one lobe of the lung. Diagnosis is often made by prenatal ultrasonography.CCAM has a variable natural history. Some lesions regress in utero with no residual abnormality at birth (1-2), others are associated with fetal polyhydramnios, mediastinal shift, and nonimmune hydrops fetalis resulting in the risk of fetal demise (3). Mediastinal shift and hydrops fetalis are indications for fetal intervention.Stocker et al.'s original classification of CCAM included three subtypes based predominantly on the cyst size. Type I lesions are composed of single or multiple large cysts (Ͼ2 cm in diameter); type II lesions are multiple, smaller cysts (Ͻ1 cm in diameter); and type III are more solid, microcystic lesions. Two variants were later added to the original classification system: type 0, composed of bronchial-like histology and type IV, a more distal acinar lesion (4).The pathogenesis of CCAM is unclear. Its original description was that of a hamartomatous lesion. The five Stocker CCAM types have also been described as each being distinct malformations, with unique etiologies resulting from a localized obstruction or atresia (2). The parenchymal maldevelopment characterizing CCAM has been associated with bronchial atresia (5). The bronchial atresia may result from a localized arrest in the bronchial tree during the branching phase of lung development, the pseudoglandular phase.Lung development during the pseudoglandular phase involves extensive epithelial-mesenchymal interaction of growth factors, transcription factors, and signaling molecules. Previous studies evalu...

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Section: Discussionmentioning

confidence: 99%

Genetic Analysis of Congenital Cystic Adenomatoid Malformation Reveals a Novel Pulmonary Gene: Fatty Acid Binding Protein-7 (Brain Type)

et al. 2008

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“…Clinically, cardiogenic hydrops typically presents with impaired diastolic ventricular filling (Bellini et al 2009), as seen in GR K/K foetuses (though not in foetuses with cardiomyocytespecific GR knock-out). Glucocorticoids are an effective treatment in hydrops foetalis associated with congenital cystic adenomatoid malformations (CCAM; Tsao et al 2003, Curran et al 2010, although the therapeutic mechanism remains to be ascertained (Leung et al 2005) and may relate to reduced mediastinal shift secondary to glucocorticoid-induced maturation or involution of the lung lesions, restoring cardiac venous return and improving contractility (Knox et al 2006).…”

Section: Glucocorticoids Through Gr Promote Foetal Heart Maturationmentioning

confidence: 99%

Glucocorticoids and foetal heart maturation; implications for prematurity and foetal programming

Rog-Zielinska¹,

Richardson²,

Denvir³

et al. 2013

Journal of Molecular Endocrinology

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Glucocorticoids are steroid hormones, essential in mammals to prepare for life after birth. Blood levels of glucocorticoids (cortisol in most mammals including humans; corticosterone in rats and mice) rise dramatically shortly before birth. This is mimicked clinically in the routine administration of synthetic glucocorticoids to pregnant women threatened by a preterm birth or to preterm infants to improve neonatal survival. Whilst effects on lung are well documented and essential for postnatal survival, those on heart are less well known. In this study, we review recent evidence for a crucial role of glucocorticoids in late gestational heart maturation. Either insufficient or excessive glucocorticoid exposure before birth may alter the normal glucocorticoid-regulated trajectory of heart maturation with potential life-long consequences.

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“…This rate has since been confirmed by others (Knox et al, 2006). A recent advance is the use of maternal steroids, but its efficacy and wider place in management remains to be demonstrated (Tsao et al, 2003;Peranteau et al, 2007).…”

Section: Cystic Lung Lesionsmentioning

confidence: 73%

The making of fetal surgery

et al. 2010

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Fetal diagnosis prompts the question for fetal therapy in highly selected cases. Some conditions are suitable for in utero surgical intervention. This paper reviews historically important steps in the development of fetal surgery. The first invasive fetal intervention in 1963 was an intra-uterine blood transfusion. It took another 20 years to understand the pathophysiology of other candidate fetal conditions and to develop safe anaesthetic and surgical techniques before the team at the University of California at San Francisco performed its first urinary diversion through hysterotomy. This procedure would be abandoned as renal and pulmonary function could be just as effectively salvaged by ultrasound-guided insertion of a bladder shunt. Fetoscopy is another method for direct access to the feto-placental unit. It was historically used for fetal visualisation to guide biopsies or for vascular access but was also abandoned following the introduction of high-resolution ultrasound. Miniaturisation revived fetoscopy in the 1990s, since when it has been successfully used to operate on the placenta and umbilical cord. Today, it is also used in fetuses with congenital diaphragmatic hernia (CDH), in whom lung growth is triggered by percutaneous tracheal occlusion. It can also be used to diagnose and treat urinary obstruction. Many fetal interventions remain investigational but for a number of conditions randomised trials have established the role of in utero surgery, making fetal surgery a clinical reality in a number of fetal therapy programmes. The safety of fetal surgery is such that even non-lethal conditions, such as myelomeningocoele repair, are at this moment considered a potential indication. This, as well as fetal intervention for CDH, is currently being investigated in randomised trials.

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Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy

Cited by 147 publications

References 20 publications

Genetic Analysis of Congenital Cystic Adenomatoid Malformation Reveals a Novel Pulmonary Gene: Fatty Acid Binding Protein-7 (Brain Type)

Genetic Analysis of Congenital Cystic Adenomatoid Malformation Reveals a Novel Pulmonary Gene: Fatty Acid Binding Protein-7 (Brain Type)

Glucocorticoids and foetal heart maturation; implications for prematurity and foetal programming

The making of fetal surgery

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