{"doi":"10.1002/ppul.27410","title":"Xe Ventilation and Gas‐Exchange MRI Abnormalities in Children with Neuroendocrine Cell Hyperplasia of Infancy (NEHI)","abstract":"To the Editor, Neuroendocrine cell hyperplasia of infancy (NEHI) is one of the most common childhood interstitial and diffuse lung diseases (chILD) and is characterized by tachypnea and increased work of breathing in infants. The characteristic finding of NEHI on chest computed tomography (CT) scans is ground-glass opacification (GGO), specifically in the right middle lobe and lingula. Magnetic resonance imaging with inhaled hyperpolarized xenon-129 gas (Xe MRI) as a contrast agent is emerging as a new, recently FDA-approved modality to assess regional lung ventilation. Xe MRI is feasible in children as young as 5-years-old [1]. This technique is particularly advantageous for the pediatric population as it has the potential to provide valuable diagnostic imaging information without the radiation exposure of a CT scan. Using Xe gas-exchange MRI, the unique diffusion and chemical shift properties of Xe gas are leveraged to quantitatively map Xe gas within the lung interstitial tissue and blood plasma (“membrane”) and affiliated with the red-blood cells (RBC), akin to a 3-dimensional diffusion-capacity measurement. The regional Xe signal intensities in each of these three functional compartments (i.e., ventilation, membrane, and RBC) as well as the RBC:Membrane ratio are quantified relative to healthy-reference distributions to identify regions of the lung with abnormally-low and -high Xe signal in each compartment [2, 3]. Xe gas-exchange MRI has been reported in many adult lung diseases [4, 5]. For instance, in adults with chronic obstructive pulmonary disease (COPD), Xe MRI was characterized by decreases in ventilation (suggestive of ventilation deficits related to regional obstruction) but relatively normal membrane uptake and RBC transfer. To contrast, in adults with idiopathic pulmonary fibrosis (IPF), ventilation from Xe MRI was relatively normal; however, increases in the membrane uptake (likely related to fibrotic changes) with decreases in the RBC transfer from Xe MRI were associated with progression to lung transplantation or death [6]. Despite its safety and feasibility in the pediatric population, the use of this novel imaging technique has not been as well-described in chILD. We hypothesized Xe MRI would reveal diffuse ventilation heterogeneity and interstitial-tissue abnormalities in children with NEHI. Five pediatric patients with previously diagnosed NEHI (9 ± 4 years) underwent a research Xe MRI study after informed consent and assent for participants ages 11 and older (CCHMC IRB 2014-5279). Xe MRI was performed on a 3 T MRI scanner (Phillips Ingenia Elition, Philips Healthcare, Netherlands) with a flexible Xe transmit-receive chest coil (Clinical MR Solutions, Brookfield, WI) with Xe images acquired during a coached inhalation and breath-hold maneuver. Xe gas was dosed at 1/6th of the height- and sex-predicted total lung capacity and inhaled from functional residual capacity. Gas exchange images were acquired during a 15 s breath-hold of Xe gas using a 3D-radial MRI sequence as described previously [1]. Images of Xe membrane uptake and RBC transfer were normalized to ventilation images. To quantify abnormalities, the Xe signal intensity in each of these gas-exchange compartments were compared to distributions derived from an age-matched healthy-reference population [2, 3]. Briefly, this method uses means (µ) and standard deviations (σ) of the Xe signal intensities derived from Xe MRI scans in a healthy-reference population to determine thresholds for abnormalities. For this analysis, regions with abnormally low Xe signal were defined as signal less than the reference µ-σ, and abnormally high Xe signal was defined as greater than µ + σ. Clinical characteristics for patients were obtained from electronic medical records (Table 1). Four patients remained on nocturnal oxygen at the time of MRI, while one had successfully weaned from all supplemental oxygen support. Four of the five patients (all except Patient 4) co","journal":"Pediatric Pulmonology","year":2024,"id":506325,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9695,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1357565,"name":"Ismail Bilal Masokano","orcid":"0000-0001-7777-9428","position":1,"is_corresponding":false},{"id":489815,"name":"Joseph W. Plummer","orcid":"0000-0002-0200-7869","position":2,"is_corresponding":false},{"id":319689,"name":"Matthew M. Willmering","orcid":"0000-0002-4356-9622","position":3,"is_corresponding":false},{"id":1357566,"name":"Katelyn Krivchenia","orcid":"0000-0002-9817-2710","position":4,"is_corresponding":false},{"id":680096,"name":"C. Towe","orcid":"0000-0002-1129-5671","position":5,"is_corresponding":false},{"id":319687,"name":"Laura L. Walkup","orcid":"0000-0002-5060-6401","position":6,"is_corresponding":false},{"id":1357874,"name":"Melanie A. Anderson","orcid":null,"position":0,"is_corresponding":true}],"reference_count":10,"raw_metadata":null,"created_at":"2026-07-19T02:10:54.646711Z","pmid":"39555716","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}