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Neonatal diseases II
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Brian P. Hackett, Jeffrey Dawson, Akshaya Vachharajani, Barbara Warner, F. Sessions Cole
Unlike the typical respiratory problems of prematurely delivered newborns that result from inadequate surfactant production, the mechanisms of respiratory problems in term infants (‡37 weeks of gestation) are associated with inadequate pulmonary perfusion (e.g., persistent pulmonary hypertension of the newborn) as well as surfactant deficiency due to both inadequate production (e.g., in the late preterm infant) and inactivation [e.g., meconium aspiration syndrome (MAS), congenital pneumonia]. Finally, both spontaneous airleaks and congenital abnormalities of airway, pulmonary parenchyma, or pulmonary vascular development (e.g., congenital diaphragmatic hernia, alveolar-capillary dysplasia) present as respiratory distress in the term newborn. Although term infants are less likely to suffer from respiratory problems than preterm infants, their risk for respiratory distress has been estimated at 0.6% in one large prospective study (1) and their risk for severe respiratory distress that requires mechanical ventilation at ~0.2% (2).
Childhood ILD
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
There is a spectrum of disorders comprising acinar dysplasia and alveolar-capillary dysplasia. Typically these present in a term baby who requires positive pressure ventilation soon after birth and progresses rapidly despite every attempted treatment into terminal respiratory failure. The histological pattern is of disrupted alveolar and capillary growth, with failure of normal apposition of capillaries and airspaces. Mutations in FOXF1 and STRA6 have been found in some patients (19,20). ‘Alveolar-capillary dysplasia with misalignment of pulmonary veins’ is in fact a misnomer; the ‘misaligned’ pulmonary veins have been shown by elegant morphometric studies to be dilated bronchial veins, with the anastomotic communications with the pulmonary venous system and distal pulmonary veins absent or obliterated (21).
Extracorporeal life support for neonatal cardiorespiratory failure
Published in Prem Puri, Newborn Surgery, 2017
William Middlesworth, Jeffrey W. Gander, Charles J. H. Stolar
Every effort should be made to establish a clear diagnosis before the initiation of ECMO. Circumstances arise, however, where clinical decompensation is rapid, and survival depends on immediate provision of mechanical hemodynamic support. In this situation, it is reasonable and appropriate to institute ECMO, with a plan to establish the diagnosis after achieving clinical stability during the course of ECMO. For example, pulmonary vein misalignment, a uniformly fatal anomaly of deficient alveolar capillaries and anomalous veins within the bronchoarterial bundles, presents with symptoms of persistent pulmonary hypertension unresponsive to pharmacologic treatment.10 These infants are often placed onto ECMO. When pulmonary hypertension is intractable despite ECMO support, the diagnosis of alveolar capillary dysplasia should be entertained.11 This diagnosis is made via lung biopsy. If alveolar capillary dysplasia is confirmed, then discussion with the family regarding withdrawal of ECMO support is appropriate. In addition, some important cardiac conditions such as TAPVR and TOGV with intact septum may present as respiratory failure, as mentioned previously. These patients are occasionally placed onto ECMO before an accurate diagnosis can be rendered. Once the diagnosis is established, appropriate treatment should be instituted.
An update on the diagnosis and treatment of pediatric pulmonary hypertension
Published in Expert Opinion on Pharmacotherapy, 2020
The new classification was primarily based on pathological and etiological features and partly based on clinical presentation and treatment modalities. Pediatric PAH needs to be sub-classified on the basis of specific conditions, such as congenital heart disease (CHD), congenital lung disease, and a persistent PH of newborns (PPHN) [6,19]. For example, PAH-CHD is classified as; Eisenmenger syndrome, operable and inoperable left-to-right shunts, coincidental defects, and postoperative defects. PPHN is associated with multiple disorders and antenatal and perinatal events. It is the most common cause of transient PAH. Developmental lung disorders include bronchopulmonary dysplasia (BPD), congenital diaphragmatic hernia, alveolar capillary dysplasia, surfactant abnormalities, lung hypoplasia, and Down syndrome.
Chronic interstitial lung diseases in children: diagnosis approaches
Published in Expert Review of Respiratory Medicine, 2018
Nadia Nathan, Laura Berdah, Keren Borensztajn, Annick Clement
Diffuse developmental disorders are severe and often lethal conditions [61]. Acinar dysplasia and congenital alveolar dysplasia are characterized by an arrested development at the pseudoglandular or canalicular/saccular stages, respectively. Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) associates an aberrant parenchymal development of the lung with thickened interstitium, a poor capillary bed, and in most cases, the presence of pulmonary veins in the bronchovascular axis instead of at the periphery of the lobule. The newborns present with refractory hypoxemia and severe pulmonary hypertension. The diagnosis is usually made on autopsies. Recently, mutations in TBX4 and FGFR2 have been associated with syndromic acinar dysplasia [62,63]. The majority of the described cases of ACD/MPV have been found to be related to FOXF1 mutations or to mutations in the FOXF1 enhancer. Extra-pulmonary disorders are documented in more than half of the cases: congenital heart diseases, digestive malformations or malrotations, and genitourinary malformations [64–66].
Persistent Pulmonary Hypertension Without Underlying Cardiac Disease as a Presentation of Pulmonary Interstitial Glycogenosis
Published in Fetal and Pediatric Pathology, 2018
Gordon Gray Still, Shuo Li, Mark Wilson, Paul Sammut
A lung wedge biopsy at 5 months of age revealed a moderate degree of alveolar simplification and duct widening. There was no alveolar capillary dysplasia or findings suggestive of pulmonary venous hypertension on histology. PIG was represented by mild patchy changes of interstitial widening with a combination of mesenchymal cells (Figure 2). Dilated proximal pulmonary arteries, thickened smaller muscular pulmonary branches, and hypoplastic muscular pulmonary artery branches with mild medial hypertrophy were consistent with pulmonary hypertension. Periodic acid–Schiff (PAS) positive staining of mesenchymal cell granules cleared with diastase. At 18 months of age, she continues to receive low flow oxygen for hypoxemia, Sildenafil and Treprostenil. Bosentan was discontinued secondary to elevation in liver enzymes.