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Lung Pathology |

Follicular Bronchiolitis in a Child With 1p36 Deletion Syndrome

Marissa Love, MD; Jane Taylor, MD; Wendy Estrellado, MD
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Department of Pediatrics, University of Kansas Medical Center, Kansas City, KS


Chest. 2015;148(4_MeetingAbstracts):631A. doi:10.1378/chest.2258571
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Abstract

SESSION TITLE: Lung Pathology Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: The rarity of pulmonary manifestations in children with chromosome 1p36 deletion syndrome makes a diagnosis of follicular bronchiolitis (FB) a unique challenge.

CASE PRESENTATION: A one year old female was diagnosed with a chromosome 1p36 deletion after birth. She has hypotonia, developmental delay, visual abnormalities, growth and feeding related problems, which are common features of this disorder. She presented with tachypnea, several months of productive cough and wheezing that were unresponsive to antibiotics, albuterol, and corticosteroids. On exam, she was dysmorphic, hypotonic, with increased respiratory distress, and mild splenomegaly. A chest CT showed compression of the right upper lobe (RUL) bronchus and an ill-defined soft tissue fullness in right hilum. She was admitted with concern for neuroblastoma and immunodeficiency. An extensive autoimmune, infectious, and immunodeficiency work-up was negative, except for a low CD4/CD8 ratio. She was found to have elevated metanephrines; however, her MIBG did not show evidence of an avid lesion. Flexible bronchoscopy disclosed compression of the RUL bronchus. Lung biopsy revealed findings consistent with follicular bronchiolitis, whereas lymph node biopsy showed reactive follicular and interfollicular hyperplasia. She was treated with IV steroids and did not have any recurrence.

DISCUSSION: Notably, our patient has a complex chromosomal anomaly that does not typically present with pulmonary problems; however, deletions in chromosome 1p36 have been linked to reactive lymphoid hyperplasia and follicular lymphoma. FB is a rare disease in children. In adults, most cases are associated with collagen vascular diseases, immunodeficiency, or hypersensitivity reaction.1 This disease is characterized by bronchiolar narrowing by external compression due to lymphoid hyperplasia, which is seen in our patient.2 Affected children may present with tachypnea, respiratory distress and failure to thrive. Lung biopsy is necessary for confirmation of diagnosis. There are no well-established guidelines for the treatment of this condition, but macrolides and steroids have been suggested to have a benefit.3 Immunosuppressive agents are generally reserved for refractory cases.

CONCLUSIONS: To our knowledge, this is the first case of FB reported on a child with chromosome 1p36 deletion. The etiology of this disease in children is unknown; however, diagnosis should be considered in a child with chronic bronchial obstruction.

Reference #1: Yousem S.A., et al. Follicular bronchitis/bronchiolitis. Hum Pathol. 1985 Jul;16(7):700-706.

Reference #2: Kinane B.T., et al. FB in the Pediatric Population. Chest. 1993; 104:1183-1186.

Reference #3: Dias A., et al. FB: A Rare Cause of Persistent Atelectasis in Children. Respiratory Medicine Case Reports. 2013; 10:7-9.

DISCLOSURE: The following authors have nothing to disclose: Marissa Love, Jane Taylor, Wendy Estrellado

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