T h e new england journal o f medicine case records of the massachusetts general hospital Founded by Richard C. Cabot Nancy Lee Harris, m.d., Editor Eric S. Rosenberg, m.d., Associate Editor Jo-Anne O. Shepard, m.d., Associate Editor Alice M. Cort, m.d., Associate Editor Sally H. Ebeling, Assistant Editor Christine C. Peters, Assistant Editor Case 37-2011: A 9-Month-Old Boy with Recurrent Tachypnea and Respiratory Distress T. Bernard Kinane, M.D., Randheer Shailam, M.D., and Eugene J. Mark, M.D. Presentation of Case Dr. Sze Man Tse (Pediatrics): A male infant was admitted to this hospital at the age of From the Departments of Pediatrics 5.5 months, because of tachypnea and respiratory distress. (T.B.K.), Radiology (R.S.), and Pathology (E.J.M.), Massachusetts General Hospi- The patient had been well until 2 days earlier, when cough and somnolence de- tal; and the Departments of Pediatrics veloped. The night before admission, the temperature rose to 38.6°C; acetaminophen (T.B.K.), Radiology (R.S.), and Pathology was administered. The next morning, labored breathing was observed, and his fam- (E.J.M.), Harvard Medical School — both in Boston. ily noted blotchy areas on his face and that his “chest was sucking in” more than usual. Oral intake decreased, nonbloody diarrhea (four or five episodes) developed, and N Engl J Med 2011;365:2221-8. urination decreased. On examination by his pediatrician that afternoon, the respira- Copyright © 2011 Massachusetts Medical Society. tory rate was 30 to 50 breaths per minute, with chest retractions, and the oxygen saturation was 85 to 87% while the patient was breathing ambient air. Albuterol was administered by nebulizer, and he was transported to the emergency department at this hospital. The patient was born after a full-term gestation by scheduled repeat cesarean section, and he had been well except for one febrile episode at the age of 89 days. He had received all routine childhood immunizations through 4 months of age, took no medications, and had no known allergies. He lived with his mother, one sibling, and maternal relatives and attended day care. There were no pets in the house. His father, maternal grandmother, paternal grandfather, and sibling had asthma; his mother smoked cigarettes outside the home. On examination, the weight was 7.2 kg (10th percentile), the temperature 36.4°C, the blood pressure 106/57 mm Hg, the pulse 160 beats per minute, the respiratory rate a maximum of 52 breaths per minute, and the oxygen saturation 90% while the patient was breathing ambient air. Nasal flaring and supraclavicular and subcostal retractions with respirations were present; there were coarse expiratory wheezes bilaterally and decreased breath sounds on the right side. The remainder of the ex- amination was normal. A complete blood count, measurements of serum electro- lytes and calcium, tests of renal function, and a urinalysis were normal. Testing for influenza, parainfluenza, adenovirus, and respiratory syncytial viruses was negative. A chest radiograph showed mildly increased lung volumes, bilateral perihilar inter- stitial opacities, and peribronchial cuffing. Oxygen was administered by a nonre- n engl j med 365;23 nejm.org december 8, 2011 2221 The New England Journal of Medicine Downloaded from nejm.org by LUIGI GRECO on February 29, 2012. For personal use only. No other uses without permission. Copyright © 2011 Massachusetts Medical Society. All rights reserved. T h e new england journal o f medicine breather face mask; the oxygen saturation rose 7.5 months of age, testing for influenza A virus to 96 to 98%. Methylprednisolone, 2 mg per antigen was positive (H1N1 presumed), and kilogram of body weight, was administered in- when he was 9 months of age, testing for respi- travenously, and albuterol was given by nebuliz- ratory syncytial virus was positive. On physical er. The patient was admitted to the pediatric examination, there were persistent fine crackles service, and intravenous crystalloid was adminis- in both lungs. Each time, his condition improved tered; prednisolone (2 mg per kilogram per day) with supportive care, and he was discharged was given orally, and ceftriaxone (360 mg) was after 5 to 7 days. administered intravenously. A blood culture was When the patient was 9 months of age, com- sterile. His condition improved, and he was dis- puted tomography (CT) of the chest performed charged on the third day, taking albuterol and after the intravenous administration of contrast budesonide by nebulizer and prednisolone (a material revealed bilateral, symmetric paramedias- 5-day course) orally. tinal ground-glass opacities, with scattered regions During the next week, cough persisted, nasal of air trapping, areas of atelectasis in the depen- congestion increased, and oral intake and urina- dent portions of the lungs, and a few small paren- tion gradually decreased. Eleven days after dis- chymal nodules. Chest radiographs taken 2 weeks charge, the patient returned to the emergency later showed hyperinflated lungs and increasing department. On examination, the pulse was 150 perihilar patchy opacities in the right lung. beats per minute, the respiratory rate 24 breaths One month later, a diagnostic procedure was per minute, and the oxygen saturation 90% while performed. he was breathing ambient air; the temperature was normal. Mild rhinorrhea was present, and coarse Differential Diagnosis breath sounds were heard bilaterally, without wheezing or prolongation of the expiratory phase. Dr. T. Bernard Kinane: May we review the chest ra- The remainder of the examination was normal. A diographs? complete blood count was normal, as were tests of Dr. Randheer Shailam: Obtained at the time of the renal and liver function and measurements of se- first admission, a frontal chest radiograph shows rum electrolytes, glucose, phosphorus, magne- bilateral patchy perihilar opacities (Fig. 1A) and sium, total protein, albumin, and direct and total a lateral chest radiograph shows flattening of bilirubin. Chest radiographs revealed hyperin- the hemidiaphragms, indicating hyperinflation flated lungs, with peribronchial thickening. (Fig. 1B). These findings can be seen in cases of The patient was readmitted to the hospital; reactive airway disease, atypical pneumonia, or supplemental oxygen, prednisolone, intravenous bronchiolitis. fluids, and saline nebulizer treatments were added. Dr. Kinane: I am aware of the diagnosis in this Testing for respiratory viruses was negative. Inter- case. This 9-month-old infant had recurring respi- mittent tachypnea and a supplemental oxygen re- ratory distress that began when he was 5 months quirement persisted. On the sixth day, ausculta- of age. There are no dysmorphic features, and tion of the lungs revealed coarse breath sounds the infant is growing normally. The findings on and fine crackles. Glucocorticoid administration auscultation — namely, rhonchi with persistent was increased (prednisolone from 1 mg to 2 mg fine crackles in the background — are unusual. per kilogram per day orally, and budesonide The chest radiograph shows persistent hyperin- from 0.25 mg to 0.5 mg twice daily), and azithro- flation and an absence of parenchymal infiltrates. mycin was begun. Supplemental oxygen was grad- The disease in this case seems to be confined to ually weaned. The patient was discharged on the the respiratory system; there seems to be no car- ninth day, receiving prednisolone (to taper over diac abnormality. Since the child is growing well, a period of 1 week) and azithromycin (total, an underlying immunodeficiency is unlikely. 5-day course); albuterol, budesonide, and sodium chloride were given by nebulizer. His mother was Reactive airway disease encouraged to stop smoking. Recurrent wheezing is not unusual in infants and At the ages of 7, 7.5, and 9 months, the pa- can be seen in up to 19% of children.1 It has many tient was again admitted to the hospital because names, including reactive airway disease and tran- of fever and respiratory distress. When he was sient early wheeze. The underlying cause is the 2222 n engl j med 365;23 nejm.org december 8, 2011 The New England Journal of Medicine Downloaded from nejm.org by LUIGI GRECO on February 29, 2012. For personal use only. No other uses without permission. Copyright © 2011 Massachusetts Medical Society. All rights reserved. case records of the massachusetts general hospital small airways of infants.1 When a respiratory tract infection develops in an infant, the diam- A B eter of the lumen becomes even smaller and the child wheezes. Reactive airway disease gradu- ally resolves during early childhood. The resolu- tion is thought to be related to growth of the airways. The most characteristic findings in this condition are recurrent wheeze and hyperinfla- tion, and this infant has these findings. How- ever, he had persistent fine crackles on physical examination and prolonged periods of hypox- emia, a pattern that is not consistent with reac- tive airway disease. C D Interstitial lung disease The findings in this patient are consistent with in- terstitial lung disease. In pediatric patients, a diag- nosis of interstitial lung disease is considered when the child has tachypnea, persistent fine crackles, and hypoxemia.2 Wheezing occurs in 20% of pa- tients with pediatric interstitial lung disease, and Figure 1. Imaging Studies. most cases start with a viral illness,2 as in this A frontal chest radiograph obtained when the patient was 5.5 months of age patient. The estimated incidence is 0.36 cases per shows patchy perihilar opacities (Panel A, arrows); a lateral chest radiograph 100,000 children, and there is a male predominance obtained at the same time shows flattening of the hemidiaphragms, indicat- (60:40).3 Three quarters of cases of interstitial lung ing hyperinflation (Panel B, arrows). An axial CT scan at the level of the carina shows centrally distributed ground-glass opacities bilaterally (Panel C, arrows) disease in children are manifested before the pa- and areas of low attenuation anteriorly (arrowheads), indicating air trapping.