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      Pulmonary Interstitial Glycogenosis: An Unrecognized Etiology of Persistent Pulmonary Hypertension of the Newborn in Congenital Heart Disease?

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          Abstract

          Background

          Pulmonary interstitial glycogenosis (PIG) arises from a developmental disorder of the pulmonary mesenchyme and presents clinically with reversible neonatal respiratory distress and/or persistent pulmonary hypertension of the newborn (PPHN).

          Objective

          We report two cases of PIG in patients with congenital heart disease (CHD) and evidence of PPHN.

          Results

          Both cases demonstrated the hallmark PIG histologic finding of diffuse, uniform interstitial thickening due to the presence of immature interstitial cells containing abundant cytoplasmic glycogen.

          Conclusions

          We report the second and third patients with PIG associated with CHD. Because histologic examination is required to establish the diagnosis, we speculate that PIG, although rare, may be underrecognized in neonates presenting with PPHN in the setting of CHD.

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          Inhaled nitric oxide and persistent pulmonary hypertension of the newborn. The Inhaled Nitric Oxide Study Group.

          R Polin, Stephen F. Morin, W Zapol … (1997)
          Persistent pulmonary hypertension of the newborn causes systemic arterial hypoxemia because of increased pulmonary vascular resistance and right-to-left shunting of deoxygenated blood. Inhaled nitric oxide decreases pulmonary vascular resistance in newborns. We studied whether inhaled nitric oxide decreases severe hypoxemia in infants with persistent pulmonary hypertension. In a prospective, multicenter study, 58 full-term infants with severe hypoxemia and persistent pulmonary hypertension were randomly assigned to breathe either a control gas (nitrogen) or nitric oxide (80 parts per million), mixed with oxygen from a ventilator. If oxygenation increased after 20 minutes and systemic blood pressure did not decrease, the treatment was considered successful and was continued at lower concentrations. Otherwise, it was discontinued and alternative therapies, including extracorporeal membrane oxygenation, were used. Inhaled nitric oxide successfully doubled systemic oxygenation in 16 of 30 infants (53 percent), whereas conventional therapy without inhaled nitric oxide increased oxygenation in only 2 of 28 infants (7 percent). Long-term therapy with inhaled nitric oxide sustained systemic oxygenation in 75 percent of the infants who had initial improvement. Extracorporeal membrane oxygenation was required in 71 percent of the control group and 40 percent of the nitric oxide group (P=0.02). The number of deaths was similar in the two groups. Inhaled nitric oxide did not cause systemic hypotension or increase methemoglobin levels. Inhaled nitric oxide improves systemic oxygenation in infants with persistent pulmonary hypertension and may reduce the need for more invasive treatments.
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            Low-dose nitric oxide therapy for persistent pulmonary hypertension of the newborn. Clinical Inhaled Nitric Oxide Research Group.

            M Keszler, G Southgate, T Kueser … (2000)
            Inhaled nitric oxide improves gas exchange in neonates, but the efficacy of low-dose inhaled nitric oxide in reducing the need for extracorporeal membrane oxygenation has not been established. We conducted a clinical trial to determine whether low-dose inhaled nitric oxide would reduce the use of extracorporeal membrane oxygenation in neonates with pulmonary hypertension who were born after 34 weeks' gestation, were 4 days old or younger, required assisted ventilation, and had hypoxemic respiratory failure as defined by an oxygenation index of 25 or higher. The neonates who received nitric oxide were treated with 20 ppm for a maximum of 24 hours, followed by 5 ppm for no more than 96 hours. The primary end point of the study was the use of extracorporeal membrane oxygenation. Of 248 neonates enrolled, 126 were randomly assigned to the nitric oxide group and 122 to the control group. Extracorporeal membrane oxygenation was used in 78 neonates in the control group (64 percent) and in 48 neonates in the nitric oxide group (38 percent) (P=0.001). The 30-day mortality rate in the two groups was similar (8 percent in the control group and 7 percent in the nitric oxide group). Chronic lung disease developed less often in neonates treated with nitric oxide than in those in the control group (7 percent vs. 20 percent, P=0.02). The efficacy of nitric oxide was independent of the base-line oxygenation index and the primary pulmonary diagnosis. Inhaled nitric oxide reduces the extent to which extracorporeal membrane oxygenation is needed in neonates with hypoxemic respiratory failure and pulmonary hypertension.
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              Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany

              Matthias Griese, Melanie Haug, Frank Brasch … (2009)
              Background Diffuse parenchymal lung diseases (DPLD) in children represent a rare and heterogeneous group of chronic pulmonary disorders. Despite substantial advances in genetics and pathomechanisms, these often lethal diseases are still under-diagnosed. This is due to the fact that (i) the incidence is low, and (ii) clinical presentation, (iii) disease classification and (iv) specific treatment options are largely unknown. Methods Here we systematically assessed the incidence, the presentation, the diagnostic yield and treatments of pediatric DPLD in Germany, using the Surveillance Unit for Rare Paediatric Disorders (ESPED). Results The incidence of DPLD was 1.32 new cases per 1 million of children per year. The majority of these children were diagnosed within the first year of life. Overall survival was 87%. Using centralized data entry and stratification tools, the patients were categorized into an advanced classification system based on diagnostic algorithms, including clinical presentations, genetics and/or histology. Combining molecular and clinical information, this survey provides an etiological overview and specific diagnostic recommendations for children with DPLD. Conclusions Standardized surveys and systematic classifications are valuable tools for the clinical handling of children with DPLD and aim to improve the disease understanding and the prognosis of these rare detrimental lung diseases.
              Article 0 comments Cited 31 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Jeffrey R. Fineman: jeff.fineman@ucsf.edu
                Journal
                Journal ID (nlm-ta): Pediatr Cardiol
                Journal ID (iso-abbrev): Pediatr Cardiol
                Title: Pediatric Cardiology
                Publisher: Springer-Verlag (New York )
                ISSN (Print): 0172-0643
                ISSN (Electronic): 1432-1971
                Publication date (Electronic): 22 May 2012
                Publication date PMC-release: 22 May 2012
                Publication date (Print): June 2013
                Volume: 34
                Issue: 5
                Pages: 1254-1257
                Affiliations
                [ ]Department of Pediatrics, University of California, 513 Parnassus Avenue, Box 1346, San Francisco, CA 94143-1346 USA
                [ ]Cardiovascular Research Institute, University of California, San Francisco, CA 94143-1346 USA
                [ ]Department of Pathology, University of California, San Francisco, CA 94143-1346 USA
                [ ]Department of Surgery, University of California, San Francisco, CA 94143-1346 USA
                [ ]Department of Pediatrics, University of Alberta, Edmonton, AB Canada
                Article
                Publisher ID: 371
                DOI: 10.1007/s00246-012-0371-z
                PMC ID: 3651824
                PubMed ID: 22614905
                SO-VID: 2334517f-7957-4742-814c-c06af08ba4e7
                Copyright © © The Author(s) 2012
                History
                Date received : 28 February 2012
                Date accepted : 8 May 2012
                Categories
                Subject: Case Report
                Custom metadata
                issue-copyright-statement © Springer Science+Business Media New York 2013

                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: congenital heart defects,glycogen storage diseases,interstitial lung diseases,neonatal respiratory distress syndrome,persistent pulmonary hypertension of the newborn
                Data availability:
                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: congenital heart defects, glycogen storage diseases, interstitial lung diseases, neonatal respiratory distress syndrome, persistent pulmonary hypertension of the newborn

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