Long-term evolution of neuroendocrine cell hyperplasia of infancy: the FRENCHI findings.

Only few studies report long-term evolution of patients with neuroendocrine cell hyperplasia of infancy (NEHI). We report data from a 54-patient cohort followed up in the French network for rare respiratory diseases (RespiRare). Demographic characteristics and respiratory and nutritional evolution were collected at the time of the patient's last scheduled visit. The mean duration of follow-up was 68 months (5 months to 18 years). Fifteen patients (27.8%) were considered clinically cured. During follow-up, hospitalizations for wheezy exacerbations were reported in 35 patients (55%), and asthma diagnosed in 20 (37%). Chest CT scan improvement was noted in 25/44 (56.8%). Spirometry showed a persistent obstructive syndrome in 8/27 (29.6%). A sleep disorder was rare (2/36, 5.5%). Oxygen weaning occurred in 28 of the 45 patients initially treated (62.2%) and was age-dependent (35.7% under 2 years, 70.5% between 2 and 6 years, and 100% after 7 years). Oxygen duration was linked to a biopsy-proven diagnosis (p = 0.02) and to the use of a nutritional support (p = 0.003). Corticosteroids were largely prescribed at diagnosis, with no evident respiratory or nutritional effect during follow-up. Among 23 patients with an initial failure to thrive, 12 (52.2%) had no weight recovery. Initial enteral feeding (17/54, 31.5%) was stopped at a mean age of 43 months (3 to 120), with no effect on cure and oxygen liberation at the last visit.  Conclusion: Our results show that NEHI has a globally positive, but unequal, improvement over time. Further prospective studies are needed to better clarify the different trajectories of patients with NEHI. What is Known: • Neuroendocrine cell hyperplasia of infancy (NEHI) is an interstitial lung disease whose long-term outcome is considered positive from very few studies including heterogeneous populations. What is New: • The 68-month follow-up of our 54-patient cohort showed respiratory/nutritional symptom persistence in 72.2%, oxygen requiring in 34%, and asthma in 37%. When controlled, radiological or functional improvement was noted in 56.8 and 40.7%. Further prospective studies are needed to better clarify the different trajectories of patients with NEHI.

French national cohort of neuroendocrine cell hyperplasia of infancy (FRENCHI) study: diagnosis and initial management.

Early diagnosis of neuroendocrine cell hyperplasia of infancy (NEHI) is crucial as, conversely to the other causes of intersititial lung disease, corticosteroids are not recommended. Diagnosis is historically based on lung biopsy (NEHI), but in current practice, a clinical and radiological approach is more and more preferred (NEHI syndrome). This national study aimed to address diagnosis and initial management of patients followed up for a NEHI pattern in pediatric centers for rare lung diseases (RespiRare, France). Data on neonatal and familial events, symptoms at diagnosis, explorations performed and results, and therapeutic management were collected by questionnaire. Fifty-four children were included (boys 63%). The mean onset of symptoms was 3.8 ± 2.6 months. The most frequent symptoms at diagnosis were tachypnea (100%), retraction (79.6%), crackles (66.7%), and hypoxemia (59.3%). The mean NEHI clinical score, evocative when ≥ 7/10, was 7.9 ± 1.4 (76% with a score ≥ 7). All chest CT-scans showed ground glass opacities evolving at least the middle lobe and the lingula. Lung biopsy was performed in 38.9% of the cases and was typical of NEHI in only 52.4%, even when the clinical presentation was typical. Initial treatments were oxygen (83.6%) and more curiously intravenous pulses of steroids (83.3%) and azithromycin (70.2%). CONCLUSION: This national cohort of patients underlines diagnosis difficulties of NEHI. A composite clinical and radiological score should help clinicians for limiting the use of anti-inflammatory drugs. WHAT IS KNOWN: •Neuroendocrine cell hyperplasia of infancy (NEHI) is an interstitial lung disease whose diagnosis is essential to limit corticosteroids therapy. WHAT IS NEW: •In this national cohort of 54 patients with a NEHI pattern, diagnosis is mainly based on clinical symptoms and chest CT-scan results. The newly proposed clinical score and, when performed, the lung biopsies are faulted in 25 and 50% of the cases, respectively. •Corticosteroids are widely used. Such results plead for a new composite score to formally diagnose NEHI.

Use of high-flow nasal cannula in infants with viral bronchiolitis outside pediatric intensive care units.

High-flow nasal cannula (HFNC) is frequently used in infants with acute viral bronchiolitis outside pediatric intensive care units (PICU). A structured questionnaire was sent out to pediatricians of all public French hospitals with pediatric emergency and/or general pediatric departments on their use of HFNC outside PICU (department using HFNC, number of available devices, monitoring, criteria for initiating or stopping HFNC, and personal comments on HFNC). Of the 166 eligible hospitals, 135 answered (96 general and 39 university hospitals; 81.3%), for a total of 217 answering pediatricians. Seventy-two hospitals (53.3%) used HFNC in acute bronchiolitis outside PICU, particularly, general hospitals (59.4% vs 38.5%), and mostly in pediatric general departments (75%). Continuous patient monitoring with a cardiorespiratory monitor was usual (n = 58, 80%). Nursing staff was responsible for 2.7 children on HFNC and checked vital signs 8.6 times per day. Criteria for HFNC initiation and withdrawal were not standardized. Pediatricians had a positive opinion of HFNC and were willing to extend its use to other diseases.Conclusion: Use of HFNC outside PICU in infants with acute bronchiolitis is now usual, but urgently requires guidelines. What is Known: • Acute viral bronchiolitis treatment is only supportive • High-flow nasal cannula (HFNC) is a respiratory support accumulating convincing clinical evidence in bronchiolitis • This latter treatment is usually proposed in pediatric intensive care unit (PICU) What is New: • HFNC are increasingly used outside PICU in bronchiolitis, particularly, in general hospitals and in pediatric general departments • Pediatricians are enthusiastic about this device, but validated criteria for initiation and withdrawal are lacking • Guidelines for the use of HFNC outside PICU are urgently required.

Hyperechogenicity of lenticulostriate vessels: A poor prognosis or a normal variant? A seven year retrospective study.

BACKGROUND: Lenticulostriate vasculopathy (LSV) is a hyperechogenicity of the lenticulostriate branches of the basal ganglia and/or thalamus' middle cerebral arteries and is frequently seen in neonatology. Our study primarily describes the perinatal data and long-term follow-up of newborns with lenticulostriate vessel hyperechoic degeneration. Secondly, it describes the cerebral imaging data as a function of perinatal factors and neurodevelopmental follow-up of these newborns. METHODS: This retrospective study assesses the outcome of newborns with LSV hyperechogenicity on cerebral ultrasound (two grades). These children were born between January 2008 and September 2015 and were treated in a large level III neonatal intensive care unit. Thirty-four term-equivalent age children underwent MRIs using a standardized protocol of T2, T1 3D, diffusion and spectro-MRI sequences. The MRIs retrospectively measured the white matter and basal ganglia apparent diffusion coefficients (ADC). RESULTS: Fifty-eight neonates, ranging from 25 to 42 weeks gestational age (GA), were diagnosed with LSV. There was a significantly increased high-grade LSV when accompanied by fetal heart rate abnormalities (p = 0.03) and the neonate's need for respiratory support at birth (P = 0.002). The mean ADC score was substantially superior in the high-grade versus the low-grade LSVs (p = 0.023). There were no noteworthy outcome differences between a high and low grade LSV. The mean ADC for basal ganglions was appreciably higher in children with a severe prognoses (death or developmental disorder) as compared to children with no abnormalities (p < 0.01). CONCLUSION: From the results of our study, it appears that a low-grade LSV could be considered as a normal variant. There are no unifying diagnostic criteria for LSV on cerebral ultrasound. With a cerebral MRI, the use of ADC values of basal ganglia may well underscore the importance of such data in predicting long-term outcomes.