Q:

4/8/2013
I am caring for an 18 month old male with Heiner Syndrome diagnosed at 13 months by another allergy/pulmonology team. He recently moved and is now under my care. His history is bloody stools at 1 month of age while drinking cow milk formula. This resolved with transition to hypoallergenic formula. At 11 months, cow milk products were reintroduced and apparently tolerated until 13 months of age when he presented with hemoptysis. Pulmonary evaluation at that time was reportedly positive for infiltrates and "blood in the lung" per dad. I am still waiting on records. Percutaneous skin testing to cow milk extract and fresh cow milk was negative. He is thriving on a milk-free diet. Dad's question is whether he could introduce baked milk products. I cannot find anything in the literature with respect to introducing processed milk in the diet for Heiner Syndrome and am concerned that baking/processing would not alter the protein significantly enough to prevent antigen complexes from forming. If we do choose to proceed, are there any laboratory studies I should obtain serially to monitor? His prior pulmonologist advised waiting until three years of age before re-introducing.

A:

Thank you for your inquiry.

I am not sure there is any definitive answer to your question; at least I could find none in the literature. An abstract of the most recent review of Heiner syndrome which I could find is copied for you below. As you can see, at least in this study, the attempt to reintroduce milk into the diet was unsuccessful.

Because I do not have an answer for you, I am going to ask for help from Dr. Anna Nowak-Wegrzyn who, as you know, is an internationally known expert in food allergy, especially allergy to milk. When we receive Dr. Nowak-Wegrzyn’s response, we will forward it to you.

Thank you again for your inquiry.

Pediatr Allergy Immunol. 2005 Sep;16(6):545-52.
Milk-induced pulmonary disease in infants (Heiner syndrome).
Moissidis I, Chaidaroon D, Vichyanond P, Bahna SL.
Source
Allergy and Immunology Section, Louisiana State University Health Sciences Center, Shreveport, LA 71130-3932, USA.
Abstract
Heiner syndrome (HS) is a food hypersensitivity pulmonary disease that affects primarily infants, and is mostly caused by cow's milk (CM). Only a few reports have been published, which may be due to its misdiagnosis. We review here a series of eight cases. When first diagnosed they were 4-29 months of age. They were fed CM from birth and their chronic respiratory symptoms began at age 1-9 months. The symptoms were in the form of cough in seven, wheezing in three, hemoptysis in two, nasal congestion in three, dyspnea in one, recurrent otitis media (OM) in three, recurrent fever in four, anorexia, vomiting, colic or diarrhea in five, hematochezia in one, and failure to thrive (FTT) in two. All had radiologic evidence of pulmonary infiltrates. High titers of precipitating antibodies to CM proteins were demonstrated in six of six and milk-specific immunoglobulin E (IgE) was positive in one of two. Pulmonary hemosiderosis (PH) was confirmed in one patient who showed iron-laden macrophages (ILM) in the bronchoalveolar lavage (BAL), gastric washing, and open lung biopsy. Additional findings, in a descending frequency, were eosinophilia, anemia, and elevated level of total IgM, IgE or IgA. Milk elimination resulted in remarkable improvement in symptoms within days and clearing of the pulmonary infiltrate within weeks. Parents consented to milk challenge in only three cases, all of whom developed recurrence of symptoms. After 2 yr of milk avoidance in one patient, milk challenge was tolerated for 2 months, and then the patient developed symptoms, serum milk precipitins, pulmonary infiltrate, and ILM. The HS should be suspected in young children with chronic pulmonary disease of obscure cause. The diagnosis is supported with a positive milk precipitin test and improvement on a trial of milk elimination. Severe cases may be complicated with PH, which should be suspected in the presence of anemia or hemoptysis and be confirmed with the demonstration of ILM.

Sincerely,
Phil Lieberman, M.D.

We received a response from Dr. Anna Nowak-Wegrzyn regarding your inquiry. Thank you again for your inquiry and we hope this response is helpful to you.

Phil Lieberman, M.D.

Response from Dr. Anna Nowak-Wegrzyn:
As you well know, there is very little published evidence regarding Heiner syndrome. There are certainly no data on extensively heated milk in this condition. Pulmonary symptoms tend to be persistent, with relapses described in 6 and 8-year olds but the natural history of food protein-induced pulmonary hemosiderosis is unknown. Considering the seriousness of pulmonary hemorrhage, diagnostic oral food challenges must be done with extreme caution under close physician supervision in the hospital setting and only when potential benefits out-weigh the risks, such as identification of an offending food in a patient with ongoing symptoms or determination of tolerance after a long period of food avoidance without accidental reactions. Based on the young age of the child, the recent [5 months ago] active disease, my advice would be against trying baked milk at this time, unless there is a pressing need, eg nutritional deficiency. The conventional allergy tests are not helpful but milk precipitins were reported at high titers in symptomatic children with milk-induced pulmonary hemosiderosis.

Kind regards,
Anna Nowak-Wegrzyn, MD
Associate Professor of Pediatrics
Mount Sinai School of Medicine
Jaffe Food Allergy Institute

As of last year, this was the complete list of refs for Heiner syndrome.

Heiner DC, Sears JW. Chronic respiratory disease associated with multiple circulating precipitins to cow's milk. Am J Dis Child 1960; 100:500-502.

Boat TF, Polmar SH, Whitman V, et al. Hypereactivity to cow milk in young children with pulmonary hemosiderosis and cor pulmonale secondary to nasopharyngeal obstruction. J Pediatr 1975; 87:23-29.

Lee SK, Kniker WT, Cook CD, Heiner DC. Cow's milk-induced pulmonary disease in children. Adv Pediatr 1978; 25:39-57.

Fossati G, Perri M, Careddu G, Mirra N, Carnelli V. [Pulmonary hemosiderosis induced by cow's milk proteins: a discussion of a clinical case]. Pediatr Med Chir 1992; 14(2):203-207.

Cohen GA, Hartman G, Hamburger RN, O'Connor RD. Severe anemia and chronic bronchitis associated with a markedly elevated specific IgG to cow's milk protein. Ann Allergy 1985; 55(1):38-40.

Moissidis I, Chaidaroon D, Vichyanond P, Bahna SL. Milk-induced pulmonary disease in infants (Heiner syndrome). Pediatr Allergy Immunol 2005; 16(6):545-552.

Cohen GA, Berman BA. Pulmonary hemosiderosis. Cutis 1985; 35(2):106, 108-106, 109.

Agata H, Kondo N, Fukutomi O, Takemura M, Tashita H, Kobayashi Y et al. Pulmonary hemosiderosis with hypersensitivity to buckwheat. Ann Allergy Asthma Immunol 1997; 78:233-237.

Kondo N, Fukutomi O, Agata H, Yokoyama Y. Proliferative responses of lymphocytes to food antigens are useful for detection of allergens in nonimmediate types of food allergy. J Investig Allergol Clin Immunol 1997; 7(2):122-126.

AAAAI - American Academy of Allergy Asthma & Immunology