Neuroendocrine cell hyperplasia of infancy (NEHI) has recently been described as an obstructive airway disease that affects infants aged 1-24 months, and presents typically with tachypnoea, crackles and hypoxia. The pathogenesis of the disease is unknown. We describe the clinical course of nine infants with radiologically and histologically confirmed NEHI. Host or environmental factors were not associated with the disease development. All infants with lung function tests demonstrated findings consistent with severe irreversible peripheral airway obstruction, assessed with whole body plethysmography (6/6) or the rapid thoracoabdominal compression technique (5/5). While the symptoms abated in all infants, six infants developed a non-atopic asthma during the follow-up. Systemic or inhaled corticosteroid treatment did not affect the duration of the symptoms. NEHI may mimic severe asthma and thus this entity should be taken into account when evaluating infants with chronic respiratory symptoms.

Infants 1 to 6 months of age with APN caused by bacteria other than E. coli or by papG-negative E. coli strain, positive blood culture and abnormal renal US carry an increased risk for significant urinary tract abnormalities and need enforced follow-up.

High prevalence of bronchiectasis in patients with cartilage-hair hypoplasia To the Editor: Cartilage-hair hypoplasia (CHH, MIM #250250) is a metaphyseal chondrodysplasia with variable immunodeficiency and increased risk of malignancy. We previously showed that in patients with CHH with chronic respiratory symptoms, the prevalence of bronchiectasis (BE) is 52%. 1 The prevalence of BE and the contributing risk factors in the overall CHH population remain unknown. High-resolution computed tomography (HRCT) is the criterion standard investigation for assessing BE; however, radiation exposure is a concern. Pulmonary changes in patients with CHH have not been examined systematically and the applicability of lung magnetic resonance imaging (MRI) in assessing their lung pathology is unclear. We investigated the prevalence of BE in a random cohort of 34 Finnish patients with genetically confirmed CHH (6 men, 28 women; median age, 39 years, range, 13-68 years). All patients or caregivers gave their written informed consent. The Institutional Research Ethics Committee approved the study. We collected clinical data, obtained blood tests, and performed lung HRCT (34 of 34) and MRI (16 of 34) (for detailed imaging protocols, see Appendix E1 in this article's Online Repository at www. jacionline.org). We analyzed the clinical and immunologic correlates with BE with the Fisher exact test, the Mann-Whitney test, and multiple logistic regression analysis. Performance of MRI compared with HRCT was determined by the Spearman rank correlation coefficient (rho). Table I presents the patients' clinical and laboratory characteristics. HRCT showed BE in 10 of 34 patients (29%), aged 29 to 68 years. BE was unilateral (n 5 2) or bilateral (n 5 8) and was located most commonly in the lower lobes and right middle lobe, but was also found in all other lobes (Fig 1). Additional findings on HRCT were acute inflammation (n 5 3), fibrosis-like changes (n 5 6), and nonspecific subpleural nodules of less than 0.5 cm in size (n 5 8). MRI showed lung abnormalities in 8 of 16 patients and had a good correlation with HRCT (overall r, 0.820; P < .001) (see Table E1 in this article's Online Repository at www.jacionline.org). Patients with BE had significantly higher serum IgG and white blood cells, total lymphocyte, and CD16/56 1 cell counts (Table I). However, these parameters correlated significantly with age (see Fig E1 in this article's Online Repository at www.jacionline.org). Patients with BE tended to have more sinus infections, pneumonia, and chronic cough (defined as ongoing daily cough lasting > _1 year). BE was diagnosed in 4 of 6 patients with a history of smoking, but when controlled for age, this association became insignificant. In the multiple logistic regression analysis, only higher CD16/56 1 cell counts remained associated with the presence of BE (P 5 .026; B