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Correspondence

Indian Pediatr 2012;49: 69-70

Unusual Cause of Neonatal Respiratory Distress


Eloisa Tiberi and Enrico Zecca

Department of Neonatology, Catholic University of the Sacred Heart – Rome,
Largo A. Gemelli 8 00168 Roma – Italia.
Email: [email protected]



We described a female infant weighing 2000 g born by vaginal delivery at 35+5 weeks’ gestation to a woman with urine culture and vaginal swab positive for E. Coli . At 54 hours of age, the baby developed respiratory distress syndrome associated with persistent rhinitis and productive cough. At the beginning we suspected sepsis with pneumonia and antibiotic treatment was commenced. Despite more courses of antibiotic treatment no significant radiological improvement happened, and rhinitis and productive cough were persistent. Since immunodeficiencies, cystic fibrosis, and pulmonary malformation were ruled out earlier, we performed a nasal brush biopsy in the attempt to diagnose primary ciliary dyskinesia (PCD). Findings consistent with PCD were found. After being discharged at the age of 37 d, the baby was followed up in our outpatient respiratory facility. During this time, physiotherapy has been performed twice a day, and just an episode of pneumonia occurred at the age of 6 month of life. Nasal brush biopsy was repeated at the age of 12 months and confirmed the ultrastructural defect. The baby is now 13 months old, has a satisfactory growth and a normal neurodevelopmental outcome.

PCD is an extremely rare cause of neonatal respiratory distress. It is usually an autosomal recessive disease with a prevalence of 1:15-30000 live births, but this is likely to be underestimated because underdiagnosis is common [1]. PCD is characterized by recurrent infections of upper and lower respiratory tract such as pneumonia, sinusitis, otitis media, and in almost half of the cases is associated with situs inversus (Kartagener syndrome) [2]. PCD diagnosis is rarely made in the newborn infant, and is often delayed until late childhood or even adulthood despite a history of unexplained respiratory distress in the neonatal period [1-5]. The association of PCD with neonatal respiratory distress suggests that motile cilia are critical for effective clearance of fetal lung fluid [5].

In our case, respiratory distress syndrome was associated with persistent rhinitis and productive cough. The early diagnosis of PCD is difficult and requires a high index of suspicion. We want to emphasize the diagnostic role of rhinitis and productive cough, that are both very rarely seen in normal neonates, but are common from the first few days of life in patients with PCD. These two clinical symptoms should increase the suspect especially when they occur simultaneously in a single patient and/or in an healthy newborn without respiratory risk factors. Early diagnosis allows an adequate program of treatment and follow-up, consisting of physiotherapy for airway clearance and microbiological surveillance with aggressive treatment of inter-current infections, in order to preserve the lung function in this genetic condition as long as possible [1].

References

1. Coren ME, Meeks M, Morrison I, Buchdahl RM, Bush A. Primary ciliary dyskinesia: age at diagnosis and symptom history. Acta Paediatr. 2002;91:667-9.

2. Bush A, Chodhari R, Collins N, Copeland F, Hall P, Harcourt J, et al. Primary ciliary dyskinesia: current state of the art. Arch Dis Child. 2007;92:1136-40.

3. Hossain T, Kappelman MD, Perez-Atayde AR, Young GJ, Huttner KM, Christou H. Primary ciliary dyskinesia as a cause of neonatal respiratory distress: implications for the neonatologist. J Perinatol. 2003;23:684-7.

4. Bessaci-Kabouya K, Egreteau L, Motte J, Morville P. Neonatal diagnosis of primary ciliary dyskinesia: report of one case Arch Pediatr. 2005;12:555-7.

5. Ferkol T, Leigh M. Primary ciliary dyskinesia and newborn respiratory distress. Semin Perinatol. 2006;30:335-40.
 

 

 

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