Acta Pa3diatr 81: 849-50. 1992


Neonatal pericarditis as a presenting manifestation of chronic granulomatous disease M Wilschanski’, A Abrahmov’, M Weintraub’, B Ferber2, J Glaser2 and M Kaplan3 Departments of Paediatrics’. Cardiology2 and Neonatology’, Division of Paediatrics, Shaare Zedek Medical Center. Hebrew University Medical School, Jerusalem, Israel

Chronic granulomatous disease (CGD) is a rare inherited disorder of neutrophil function, which usually manifests itself in early childhood with multiple bacterial and fungal infections, but in up to 50% of cases no pathogen can be isolated (1, 2). We report an infant with CGD who presented at birth and subsequently at four months of age with pericarditis. To our knowledge such a manifestation of CGD has not been described previously. A 3.6-kg female infant was delivered at 37 weeks gestation after an unremarkable pregnancy. She was the offspring of a consanguineous marriage, and two older siblings had CGD. Immediately after delivery the infant

suffered respiratory distress. Hepatomegaly, and distant heart sounds were noted. White cell count was 37000 cells/mm3 with a marked left shift. Blood and CSF cultures were sterile. Chest X-ray demonstrated an enlarged cardiac silhouette and an ECG showed small QRS complexes and ST elevation. Echocardiography revealed a large pericardial effusion (Fig. l). Pericardiocentesis yielded a small volume of sterile fluid containing numerous neutrophils; viral serology was negative. The child improved rapidly, with conservative management only, and was discharged at 10 days of age after a course of iv antibiotics. CGD was

Fig. I . Echocardiogram at two days of age showing a large pericardial effusion (PE= pericardial effusion, LV = left ventricle, RV = right ventricle, LA = left atrium, RA = right atrium).


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ACTA P E D I A T R 81 (1992)

Fig. 2. Echocardiogram at four months of age showing a large pericardial effusion with signs of cardiac pre-tamponade:systolic collapse of the right atrial wall (arrow).

definitively diagnosed by the zitroblue tetrazolium and negligible cytochrome c reduction tests (3). At four months of age the infant presented with a twoday history of increasing dyspnoea, decreased appetite and lethargy. Physical examination revealed mild cyanosis with marked respiratory distress, clear lungs and distant heart sounds. A chest X-ray showed marked cardiomegaly with clear lung fields. An ECG showed elevation of ST segments and small QRS 6omplexes. Echocardiography again revealed a large collection of pericardial fluid with signs of cardiac pre-tamponade (Fig. 2). Pericardiocentesis was performed surgically; 80 ml of purulent material were aspirated. The infant received a 10-daycourse of iv cefuroxime and recovered. Gram stain of the pericardial fluid revealed numerous neutrophils with no bacteria. Viral and fungal culture was negative. CGD and pericarditis are rarely diagnosed in the neonatal period. While the diagnosis of CGD was facilitated by the fact that the siblings had the condition, the fact that both entities were diagnosed so early makes this case unique. The association of pericarditis and CGD does not of course prove a causative relationship, but the fact that pericarditis was recurrent with signs of a

bacterial inflammatory response, certainly would make this relationship seem more than coincidental. Pericarditis in the neonate is rare, life-threateningand treatable. We are not advocating a search for CGD in every neonate with pericarditis. Its appearance in this age group in the absence of any other aetiology, for example congenital heart disease or serious arrhythmia, together with its recurrent nature, should prompt a search for unusual underlying disorders such as CGD.

References 1. Johnston RB, Newman SL. Chronic granulomatous disease. Pediatr Clin North Am 1977;24:365-76

2. Gallin JI. Recent advances in chronic granulomatousdisease. Ann Intern Med 1983;99657-74 3. Segal SW. Testing neutrophil function. Clin Immunol Allergy 1985;5:491-5 12 4. Noren GR, Staley NA, Kaplan EL. Pyogenic pericarditis. In: Adams FH, EmmanouilidesGC,Riemenschneider TA eds Moss’ Heart Disease in Infants, Children and Adolescents 4th Edn. Baltimore: Williams and Wilkins, 1989:74&5 Received Sep. 7, 1991. Accepted March 9, 1992

Neonatal pericarditis as a presenting manifestation of chronic granulomatous disease.

Acta Pa3diatr 81: 849-50. 1992 SHORT COMMUNICATION Neonatal pericarditis as a presenting manifestation of chronic granulomatous disease M Wilschansk...
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