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Chest radiograph demonstrated diffuse bilateral granular opacities&#46; An echocardiogram revealed no evidence of anatomic heart disease with suprasystemic levels of pulmonary artery pressure&#46; Over the next days&#44; her gas exchange worsened&#44; needing intubation and mechanical ventilation&#46; She developed progressive hypoxic respiratory failure that needed high frequency oscillatory ventilation&#44; and nitric oxide administration&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The infant was treated with antibiotics but infectious causes for PH were ruled out with negative blood cultures&#46; Chest computer tomography &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; at 15 days of life showed bilateral granular opacities and ground-glass opacification&#59; two doses of surfactant were administered without improvement&#46; Bronchoscopic bronchoalveolar lavage detected PAS positive material&#46; With this information&#44; a lung biopsy trough video thoracoscopy was performed&#46; There were marked alveolar epithelial hyperplasia and mild widening of alveolar walls and the suspicion of a genetic disorder of surfactant dysfunction was considered&#46; She still needed mechanical ventilation and take away a treatment with monthly high intravenous doses of methylprednisolone in association with oral daily hydroxychloroquine and every other day azithromycin&#46; Genetic testing showed a nonsense mutation in ABCA3 gene&#44; c&#46;4681C&#62;T or p&#46;R1561X&#46; This mutation was present on both maternal and paternal alleles&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">At 7 months of age the infant was transferred to a pediatric lung transplant unit where she underwent bilateral lung transplantation at 10 months of age&#46; Currently she is 2 years old needing home mechanical ventilation support because of tracheal and right main bronchus malacia&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Interstitial lung diseases &#40;ILD&#41; are a heterogeneous group of pathological processes that affect pulmonary parenchyma and&#44; in most cases&#44; lead to an impairment of gas transfer and reduction of the lung capacity&#46; There are no reliable estimates&#44; but prevalence is likely &#60;1 per 100<span class="elsevierStyleHsp" style=""></span>000&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">1</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The definition requires at least three of the four following criteria in the absence of other know lung disorders&#58; &#40;1&#41; respiratory symptoms &#40;cough&#44; rapid and&#47;or difficult breathing&#44; or exercise intolerance&#41;&#44; &#40;2&#41; signs &#40;resting tachypnea&#44; adventitious sounds&#44; retractions&#44; digital clubbing&#44; failure to thrive&#44; or respiratory failure&#41;&#44; &#40;3&#41; hypoxemia&#44; and &#40;4&#41; diffuse abnormalities on chest X-ray or CT scan&#46; Thus&#44; establishing 3 of 4 criteria is a sensitivity method for recognizing patients that could benefit from and ILD evaluation&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The earliest presentation of ILD is shortly after birth&#44; with unexplained respiratory distress in a term neonate&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">An organized classification scheme for ILD in children less was published by the chILD Research Network<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">3&#8211;5</span></a> &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">Given the non-specific presentation of ILD&#44; difficulty is frequently experienced in discerning ILD&#46; Excluding these conditions prior to proceeding to more invasive test is important<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1&#44;2&#44;6</span></a>&#58;<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">-</span><p id="par0060" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">Infection screen</span>&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">-</span><p id="par0065" class="elsevierStylePara elsevierViewall">An <span class="elsevierStyleBold">echocardiography</span> to rule out structural cardiovascular disease and pulmonary hypertension&#46;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">-</span><p id="par0070" class="elsevierStylePara elsevierViewall">Baseline <span class="elsevierStyleBold">chest X-ray</span>&#46;</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">-</span><p id="par0075" class="elsevierStylePara elsevierViewall">Thin-<span class="elsevierStyleBold">section CT</span> scanning&#46; Ground glass opacification and air trapping are classical features detected&#46;</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">-</span><p id="par0080" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">Flexible bronchoscopy with BAL</span> to exclude infection or airway abnormalities&#46;</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">-</span><p id="par0085" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">Genetic testing</span>&#46; Surfactant protein mutations produce recognizable clinical phenotypes of varying severity&#46;</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">-</span><p id="par0090" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">Lung biopsy</span> is the gold standard&#46;</p></li></ul></p><p id="par0095" class="elsevierStylePara elsevierViewall">The gene for ABCA3 is expressed in alveolar type II cells&#44; and the protein is localized to lamellar bodies&#46; ABCA3 mutations have been associated with lethal neonatal respiratory distress and surfactant metabolism dysfunction&#46; Outcomes in patients with ABCA3 mutations are variable&#44; ranging from severe irreversible respiratory failure in early infancy to chronic static or progressive ILD&#46;<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">7&#44;8</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">There have been no controlled trials of therapeutic interventions in ILD syndrome&#46; Case reports of improvement have been recorded with use of glucocorticoids&#44; hydroxychloroquine&#44; azathioprine&#44; bronchodilators&#44; mycophenolate&#44; and other immune modulators&#46;<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">9&#44;10</span></a> Lung transplantation is an option in end-stage lung disease&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">11</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">The North American Children study<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">12</span></a> found a mortality rate of 30&#37;&#44; with 50&#37; of patients experiencing on-going morbidity&#46; It has become clear that some ILD entities are associated with very high mortality&#44; whereas others have a favorable outcome&#46;</p></span>"
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                  \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Diffuse developmental disorders&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Acinar dysplasia&#44; congenital alveolar dysplasia&#44; alveolar-capillary dysplasia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Growth abnormalities&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pulmonary hypoplasia&#44; structural pulmonary changes with chromosomal abnormalities&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Specific conditions of undefined etiology&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Surfactant dysfunction mutations&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Disorders of the normal host&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Disorders masquerading as interstitial disease&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Arterial hypertensive vasculopathy&#44; congestive vasculopathy&#44; lymphatic disorders&nbsp;\t\t\t\t\t\t\n
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