Case reports of two patients with unusually late initial presentation of chronic granulomatous disease with fulminant Aspergillus pneumonia.Medical notes; retrospective study.Identical pattern of clinical presentation in two patients referred for support with extracorporeal membrane oxygenation (ECMO). Our Institutional Review Board waived the need for consent.Two school-aged boys presented with features of, and were initially treated, for community-acquired pneumonia. However, the disease course was rapidly progressive to fulminant respiratory failure and because both failed conventional intensive care management, they were referred to ECMO support. Although both died of evolving multiorgan failure, ECMO support allowed open lung biopsy leading to diagnosis of invasive Aspergillus pneumonia and chronic granulomatous disease.Failure of adequate therapy for acute community-acquired pneumonia and rapid progression to respiratory failure should lead to the possibility of fungal etiology. Congenital immunodeficiency may present for the first time late in life, so acute invasive pulmonary aspergillosis in the absence of known risk factors should lead to consideration of chronic granulomatous disease regardless of patient age.

To the Editors: In the review article on zygomycosis in children by Zaoutis et al1 it is clearly outlined that this rare but life-threatening infection does occur in individuals with predisposing factors, such as neutropenia in hematopoietic stem cell transplant recipients and patients with hematological malignancies, diabetes mellitus, burns, surgery and trauma, desferoxamine therapy, as well as certain “pediatric-specific” conditions such as high-risk premature newborns, juvenile-onset (type 1) diabetes mellitus with uncontrolled diabetic ketoacidosis, and congenital metabolic aciduria. We have recently been surprised by finding widespread Absidia corymbifera infection on postmortem of a 14-year-old boy who presented 1 month before he died with signs and symptoms of acute community-acquired pneumonia. With worsening respiratory function despite broad-spectrum antibiotics, and with no diagnosis established of the underlying disease despite extensive investigations before developing adult respiratory distress syndrome requiring mechanical ventilation and treatment with high-dose steroids, he was referred for emergency support with extracorporeal mechanical oxygenation (ECMO). Open lung biopsy at that stage revealed Aspergillus fumigatus invasive pneumonia and the dihydrorhodamine neutrophil oxidative burst flowcytometry assay was diagnostic for chronic granulomatous disease (CGD), subsequently confirmed to be a result of p47phox mutation. The past medical history was uneventful except that he helped cleaning gutters of dead leaves the week before he presented. Despite combined antifungal (voriconazole and caspofungin) therapy, γ-interferon, and granulocyte transfusions, he died of multiorgan system failure. This case was reported recently as patient 1 of the series of 9 patients describing the newly recognized emergency presentation of CGD, the “fulminant mulch pneumonitis.”2 Other patients in that series, all exposed to recent inhalation of mulch, had multiple fungal infections besides Aspergillus spp., mainly with Rhizopus spp., another of the Zygomycetes class, and Penicillium spp. However, we could not find any previous reports of A. corymbifera infection in CGD, neither is that mentioned in the recent extensive review on fungal infections in primary immunodeficiency.3 Mario Abinun, MD Department of Paediatric Immunology Chris Wright, FRCPath Department of Histopathology Kate Gould, FRCPath Department of Microbiology Terence J. Flood, MA, MRCPI Department of Paediatric Immunology Jane Cassidy, MRCP Department of Paediatric Intensive Care Newcastle upon Tyne Hospitals NHS Foundation Trust Newcastle upon Tyne United Kingdom

ABSTRACT Adenovirus causes disseminated disease following bone marrow transplantation (BMT). We report a child who underwent T-cell-depleted BMT. Adenovirus subgenus F serotype 41 was detected antemortem by PCR in cerebrospinal fluid and postmortem in other tissues. Serotypes 40 and 41, associated with gastrointestinal disease, have not previously been implicated in disseminated disease.

A 3-year retrospective study of 173 neonates treated with extracorporeal membrane oxygenation in the United Kingdom identified 9 cases of irreversible lung dysplasia, including alveolar capillary dysplasia (n = 5), surfactant protein B deficiency (n = 1), pulmonary hypoplasia (n = 1), pulmonary lymphangiectasis (n = 1), and combined lymphangiectasis and hypoplasia (n = 1).

One year after neonatal repair of esophageal atresia, a boy had intermittent mild dysphagia. Rigid esophagoscopy showed what was thought to be some inflammation at the level of the anastomosis but, at 5 years of age, with flexible esophagoscopy, this was identified as a ring of heterotopic gastric mucosa. This ring of mucosa has remained stable with surveillance up to 9 years of age. Although islands of heterotopic gastric mucosa in the esophagus are well recognized, there are no published reports of this at the level of an anastomosis for esophageal atresia with its associated clinical implications. J Pediatr Surg 37:E14. Copyright 2002, Elsevier Science (USA). All rights reserved.