Pediatric Annals

Editorial 

Pulmonology: Horses and Zebras

Stanford T. Shulman, MD

Abstract

The current issue of Pediatric Annals, which is devoted to pediatric pulmonary topics related to chronic lung problems, spans the spectrum from relatively common disorders, such as asthma, cystic fibrosis, and obstructive sleep apnea, to very rare causes of diffuse interstitial lung disease. The latter include the illnesses resulting from mutations of the genes encoding for surfactant protein B, surfactant protein C, ATP binding cassette A3, and thyroid transcription factor-1 (or the NKX2-1 gene). Additional rare causes of interstitial lung disease in children are developmental disorders, such as acinar dysplasia and alveolar-capillary dysplasia, with misalignment of the pulmonary veins, as well as neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis. (I must confess that I’ve not heard of some of these disorders.)

Abstract

The current issue of Pediatric Annals, which is devoted to pediatric pulmonary topics related to chronic lung problems, spans the spectrum from relatively common disorders, such as asthma, cystic fibrosis, and obstructive sleep apnea, to very rare causes of diffuse interstitial lung disease. The latter include the illnesses resulting from mutations of the genes encoding for surfactant protein B, surfactant protein C, ATP binding cassette A3, and thyroid transcription factor-1 (or the NKX2-1 gene). Additional rare causes of interstitial lung disease in children are developmental disorders, such as acinar dysplasia and alveolar-capillary dysplasia, with misalignment of the pulmonary veins, as well as neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis. (I must confess that I’ve not heard of some of these disorders.)

The current issue of Pediatric Annals, which is devoted to pediatric pulmonary topics related to chronic lung problems, spans the spectrum from relatively common disorders, such as asthma, cystic fibrosis, and obstructive sleep apnea, to very rare causes of diffuse interstitial lung disease. The latter include the illnesses resulting from mutations of the genes encoding for surfactant protein B, surfactant protein C, ATP binding cassette A3, and thyroid transcription factor-1 (or the NKX2-1 gene). Additional rare causes of interstitial lung disease in children are developmental disorders, such as acinar dysplasia and alveolar-capillary dysplasia, with misalignment of the pulmonary veins, as well as neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis. (I must confess that I’ve not heard of some of these disorders.)

A 1992 Postcard and Stamp from Macedonia, Issued for the Fight Against TB, Show a Child Undergoing Spirometry/pulmonary Function Testing.

A 1992 Postcard and Stamp from Macedonia, Issued for the Fight Against TB, Show a Child Undergoing Spirometry/pulmonary Function Testing.

The detailed paper by Rock and Sharp (see page 759) provides comprehensive information regarding cystic fibrosis newborn screening programs and how these programs affect early diagnosis and improved survival of CF patients. They also discuss the newly recognized group of patients with what has been termed CRMS (CFTR-related metabolic syndrome). These are asymptomatic infants with hypertrypsinogenemia (manifested by a positive newborn CF screen) and either a sweat chloride in the intermediate (30–59 mM/L range) and/or 1–2 mutations in the CFTR gene, which are not clearly associated with CF.

Issued in 1973, This Stamp Honors the 25th Anniversary of the World Health Organization and Shows the Reading of a Chest X-Ray.

Issued in 1973, This Stamp Honors the 25th Anniversary of the World Health Organization and Shows the Reading of a Chest X-Ray.

Kim Watts and Michael Schechter (see page 793) have analyzed the reasons for the substantial disparities in outcomes among children with asthma or CF. These include poverty, race, access to healthcare, environmental exposures, and genetic factors — some influencing responses to treatment and others potentially serving as modifier genes that impact disease outcomes in CF.

Most stamps that have a connection to pulmonary disease actually are more related to tuberculosis (TB), one of the topics not covered in this issue. The blue stamp from Ghana was issued in 1973 to honor the 25th anniversary of the World Health Organization and shows the reading of a chest X-ray. The Malaysian stamp honors “100 years of X-ray,” showing an X-ray in progress on the right and half of a chest X-ray on the left. The 1992 postcard and stamp from Macedonia (see page 740) were issued for the fight against TB and show a child undergoing spirometry/pulmonary function testing.

The Malaysian Stamp Honors “100 Years of X-Ray,” Showing an X-Ray in Progress on the Right and Half of a Chest X-Ray on the Left.

The Malaysian Stamp Honors “100 Years of X-Ray,” Showing an X-Ray in Progress on the Right and Half of a Chest X-Ray on the Left.

10.3928/00904481-20101122-01

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