Hemosiderosis is an uncommon childhood disease characterized by spontaneous pulmonary hemorrhage often associated with iron deficiency anemia. During January 1993-November 1994, eight cases of acute pulmonary hemorrhage/hemosiderosis were diagnosed among infants at a children's referral hospital in Cleveland. In comparison, during 1983-1993, a total of three cases of pulmonary hemosiderosis were diagnosed among infants and children at this hospital. This report summarizes the preliminary results of the ongoing epidemiologic, clinical, and laboratory investigations by pediatric pulmonologists in Cleveland, the Ohio Department of Health, the City of Cleveland Department of Public Health, the Cuyahoga County Board of Health, and CDC.

In 1993, cases were diagnosed in January (one case) and October (one); in 1994, cases were diagnosed in March (one), June (one), July (two), September (one), and November (one). For each of the eight infants (mean age: 10.3 weeks; range: 4 weeks-16 weeks), onset of hemoptysis was associated with pallor and an abrupt cessation in crying; fever was not reported for any of the infants. Other reported symptoms on admission included limpness, lethargy, and grunting. At the time of initial evaluation at the hospital, seven infants required admission to the pediatric intensive-care unit because of hemoptysis and respiratory distress.

All eight infants were black, and seven were male. The median age of their mothers was 20 years (range: 15-29 years). Seven of the pregnancies and deliveries occurred without complications; one infant born at 27 weeks' gestation and weighing 2 lbs, 2 oz (950 g) had complications of severe prematurity. All infants lived within a 6-mile radius of the hospital. No infants were breast fed; before admission, all were fed cow's-milk-based formula.

Laboratory findings on admission included a normal white blood cell count (median=13.8 cells/mm3) and features consistent with a normocytic, normochromic anemia characteristic of acute blood loss with a mean hematocrit of 27.1% (normal: 36.0%-47.0%) and a mean hemoglobin of 9.1 g/dL (normal: 10.0-15.0 g/dL). Red blood cell morphology was suggestive of a microangiopathic process: microscopic examination indicated that five of the eight infants had mild to moderate (1+ to 2+) hemolysis characterized by the presence of microcytes, burr cells, spherocytes, and bizarre fragments. Based on guaiac testing, occult blood was present in the stool of three infants. Results of coagulation studies included normal prothrombin and partial thromboplastin time for all infants. Chest radiographs of all infants showed diffuse, bilateral infiltrates consistent with pulmonary hemorrhage. In six infants, the mean serum magnesium level was 2.1 mg/dL (normal: 1.4-1.9 mg/dL).

Cultures of blood, urine, and bronchoalveolar lavage from seven infants were negative for bacterial, mycotic, and viral pathogens. Cultures of bronchoalveolar lavage from one infant grew Bacillus sp. Hemosiderin-laden macrophages -- indicating continued pulmonary hemorrhage -- were detected in each of the seven infants who underwent bronchoscopy more than 2 weeks after the acute hemorrhage. No other source of bleeding (i.e., gastrointestinal or nasopharyngeal) was identified during endoscopic evaluation. Immunoglobulin G levels to cow's milk proteins were above normal ( greater than 20 U/mL) in five of seven infants.

Five infants required mechanical ventilation for an average of 5 days. All infants survived the first hospitalization and were discharged in stable condition without evidence of hemoptysis after a median length of stay of 10 days (range: 2-35 days). In five infants, acute hemoptysis necessitating readmission recurred within 1 day to 6 months of discharge. One death -- attributed to severe hypoxic encephalopathy secondary to recurring pulmonary hemorrhage- -occurred in a 9-week-old full-term infant.

Local surveillance measures and active case finding have not identified additional cases in the Cleveland area. A case-control study is under way to determine risk factors for acute pulmonary hemorrhage among infants. Reported by: DG Dearborn, MD, MD Infeld, MD, P Smith, DO, C Judge, MD, Rainbow Babies and Childrens Hospital; TE Horgan, MPH, T Allan, MPH, Cuyahoga County Board of Health; JA Zimomra, MPA, Cleveland Dept of Public Health, Cleveland; BK Mortensen, PhD, SA Burkett, MA, K Winpisinger-Slay, MS, S Wagner, MPH, Ohio Dept of Health. Div of Environmental Hazards and Health Effects, Div of Birth Defects and Developmental Disabilities, and Div of Environmental Health Laboratory Sciences, National Center for Environmental Health, CDC.

Editorial Note

Editorial Note: The eight cases of acute pulmonary hemorrhage/hemosiderosis described in this report exceed the number expected at this hospital during a 2-year period. Massive acute pulmonary hemorrhage occurs rarely in infants; it usually is attributed to cardiac or vascular malformations, infectious processes, immune vasculitides, trauma, or known milk protein allergies. Cases for which the etiology is undetermined, such as these eight reported from Cleveland, traditionally have been classified as idiopathic pulmonary hemosiderosis (IPH) and account for less than 5% of all cases of pulmonary hemorrhage during infancy.

The pathologic mechanism for IPH in children is unknown. Recent histomorphologic techniques suggest that the initial histopathologic damage occurs at the alveolar epithelial surface (1). IPH has been associated with circulating antibodies to cow's milk protein; however, this association has not been consistently reproduced (1,2). In addition, some reports have described familial occurrences of pulmonary hemosiderosis, suggesting a possible genetic vulnerability to a toxicant (3,4).

To identify additional cases of acute pulmonary hemorrhage/hemosiderosis, CDC has established the following provisional surveillance case definition: hemoptysis in an infant aged less than 1 year not attributed to cardiac or vascular malformations, infectious processes, or trauma. A case report form is available from CDC. Physicians should report possible cases through state health departments to CDC's Air Pollution and Respiratory Health Branch, Division of Environmental Hazards and Health Effects, National Center for Environmental Health; Internet: rae1@cehdeh1.em.cdc.gov; telephone (404) 488-7320; or fax (404) 488-7335.

References

1. Levy J, Wilmott R. Pulmonary hemosiderosis. In: Hilman BC, ed. Pediatric respiratory disease: diagnosis and treatment. Philadelphia: WB Saunders, 1993:543-9.

2. Heiner DC, Sears JW, Kniker WT. Multiple precipitins to cow's milk in chronic respiratory disease. Am J Dis Child 1962;103:634-

4. Breckenridge RL, Ross JS. Idiopathic pulmonary hemosiderosis: a report of familial occurrence. Chest 1979;75:636-9.

5. Beckerman RC, Taussig LM, Pinnas JL. Familial idiopathic pulmonary hemosiderosis. Am J Dis Child 1979;133:609-11.

   
   

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