THE MILK PROBLEM IN HEINER SYNDROME

Abstract

<h3>Introduction</h3> Heiner syndrome (milk induced pulmonary hemosiderosis) is a rare, non-IgE-mediated hypersensitivity to cow's milk. Symptoms include pulmonary hemorrhage, iron deficiency anemia, and failure to thrive. Serum precipitins to cow's milk can be positive. The mainstay of treatment was historically dairy avoidance, now with the addition of corticosteroids and sometimes immunosuppressants. <h3>Case Description</h3> An 18-month-old boy with Trisomy 21 was admitted with hypoxia, hemoptysis, pulmonary infiltrates, and anemia. Prior to admission, he had failure to thrive, constipation, bloody stools and cough. His BAL showed pulmonary hemosiderosis. He was started on an amino acid-based formula. The family was counseled about a dairy-free diet, on which he gained weight gain and cough resolved. He was not sensitized to milk based on skin and serum testing. Precipitins were positive to milk and 3 component proteins, and he was diagnosed with Heiner syndrome. He had 3 additional episodes of pulmonary hemorrhage. Two were likely viral-induced, while eating ice cream was the culprit for one. Given recurrent pulmonary hemorrhage, he was started on rituximab with a good response. <h3>Discussion</h3> This case adds to the limited literature regarding Heiner syndrome and highlights the potential role for immunomodulators like rituximab, as the presence of IgG to milk proteins suggests a humoral defect. Additionally, this case supports the recent description of an increased risk for pulmonary hemorrhage with Trisomy 21. Heiner syndrome, although rare, should be considered with pulmonary hemosiderosis especially in patients with Trisomy 21, as a dairy-free diet may aid in management.