Aims and objectives
Neuroendocrine cell hyperplasia of infancy (NEHI) is a rare form of childhood interstitial lung disease (chILD) [1].
The exact incidence and prevalence of NEHI remain unknown.
NEHI was first described in 2005 [2]and since then all known data is derived from small to moderate-size studies[3].
Typical symptoms of NEHIincludepersistent tachypnea together with hypoxemia,
retractions,crackles [2]and frequently but not always failure to thrive [4,5].
There is an insidious onset of symptoms during the first year of life and usually an eventual improvement of the condition without...
Methods and materials
Study Population
This is a single center retrospective study.
49 infants,
children under the age oftwo,
with a history of diagnostic lung biopsy and HRCT during 2004-2017,
were initially includedin the study population.Patients with a clinical presentation obviously non-consistent with NEHI were thenexcluded(e.g.extremely preterm patients withbronchopulmonarydysplasia,
and samples taken from infants with prolonged respiratory distress after surgery for complex congenital heart defects).The final study population comprised of 32 patients.
Lung biopsy samples weregathered from the pathology information system.Lung HRCT studies were gathered from the regional...
Results
The radiologists agreedsubstantially(Table 1).A measure of inter-rater agreement,
Cohen's kappa coefficient,
was 0.75 with a 0.55 lower bound of the 95 % confidence interval.
After joint reanalysis of images Cohen’s kappa value 1 was reached.
On imaging13 patients showed definite NEHI,
4 patients showed possible NEHI and 2 patients showednon-NEHIfindings.The sensitivity of HRCT for the diagnosis of NEHI was 89% and thespecificitywas77% (Table 2).
Conclusion
This study shows that the sensitivity and specificity of HRCT in NEHI diagnostics is 89% and77% respectively.
With ourcurrent data it seems that HRCTshows lower specificity than previously reported.
Personal information
Päria Miraftabi,
M.D.,
Department of Radiology,
HUS Medical Imaging Center,
Porvoo Hospital/Finland;
[email protected]
Laura Martelius,
M.D.,
Ph.D.,
Pediatric radiologist,
Department of Radiology,
HUS Medical Imaging Center,
Helsinki University Central Hospital and University of Helsinki,
Helsinki/Finland;
[email protected]
Anna Föhr,
M.D.
Pediatric radiologist,
Department of Radiology,
HUS Medical Imaging Center,
Helsinki University Central Hospital and University of Helsinki,
Helsinki/Finland;
[email protected]
Jouko Lohi,
M.D.,
Ph.D.
Department of Pathology,
HUS Medical Center of Pathology,
Helsinki University Central Hospital and University of Helsinki,
Helsinki/Finland;
[email protected]
Turkka Kirjavainen,
M.D.,
Ph.D.
Pediatric pulmonologist,...
References
1.Kurland G,
Deterding RR,
Hagood JS,
Young LR,
Brody AS,
Castile RG,
et al.
An official American Thoracic Society clinical practice guideline: classification,
evaluation,
and management of childhood interstitial lung disease in infancy.
Am J Respir Crit Care Med.
2013 Aug 1;188(3):376–94.
2.Deterding RR,
Pye C,
Fan LL,
Langston C.
Persistent tachypnea of infancy is associated with neuroendocrine cell hyperplasia.
Pediatr Pulmonol.
2005 Aug;40(2):157–65.
3.Young LR.
Neuroendocrine cell hyperplasia of infancy (NEHI) [Internet].
UpToDate.
2018 [cited 2018 Sep 13].
Available from:https://www.uptodate.com/contents/neuroendocrine-cell-hyperplasia-of-infancy-nehi
4.Nevel RJ,
Garnett ET,...