26.10.2020

Heiner’s syndrome treatment

The central link in the treatment of pathology is the elimination of cow’s milk proteins in the body – in some cases only these measures allow achieving complete recovery of the patient. If older patients (more than 1.5-2 years old) do this quite simply, then there may be difficulties in treating infants. Requires the abolition of feeding with artificial mixtures, which in most cases are based on the milk of cows, and the transition to natural feeding or the use of donor breast milk.

The use of analogues (for example, soybean milk) is ambiguous, a number of patients with Heiner’s syndrome have negative reactions and their use.

Auxiliary and symptomatic methods of treatment include the following activities:

  • Reduction of allergic and inflammatory manifestations. Often there is a positive effect from the use of antihistamines – an improvement in the gastrointestinal tract occurs, infiltrates in the lungs disappear more quickly. Severe cases may require the use of corticosteroids to reduce the severity of inflammation.
  • Improved blood counts.  The fight against anemia includes the prescription of iron supplements, the introduction of proteins and vitamins into the diet. When breastfeeding requires a similar correction to the power of the mother.
  • Symptomatic therapy.  A strong cough may be weakened by inhalation, postural drainage – the appointment of expectorant drugs is contraindicated, as this can increase bleeding in the lungs. Treatment of dyspeptic disorders, inflammations of the upper respiratory tract, secondary infections produced by various groups of drugs according to the indications.

Often, as part of the treatment of pathology, correction is made of side effects from previous therapeutic interventions, prescribed on the basis of an incorrect diagnosis of the syndrome. Most often, this includes the correction of intestinal microflora, disturbed as a result of taking antibiotics used to treat bronchopneumonia.

Another frequent consequence of an inappropriate approach to therapy is a delay in physical development that occurs with prolonged consumption of cow’s milk. It is compensated by spa events, medical gymnastics, and classes with a child psychologist.

Heiner syndrome (allergic primary hemosiderosis of the lungs) is a very rare form of allergic enteropathy provoked by milk proteins – mainly cow’s, but cases of similar reactions to goat and even soy milk products are described. Pathology occurs in children aged 3 months to 2-3 years, with the same frequency affects boys and girls. Clinical features (bronchitis and bronchopneumonia of unclear genesis, anemia, vomiting and diarrhea) often lead to incorrect diagnosis and subsequent incorrect treatment. It is assumed that a significant part of cases (from one third to one half) is not diagnosed – the amount of milk in the diet of children decreases with the transition to a normal diet, the symptoms spontaneously disappear.

Causes of Heiner Syndrome

The etiology of the disease remains largely unexplored, research is difficult because of the rarity of the pathology and the large number of unreported cases. It is assumed that the main etiological factor is an abnormal immunological reaction to certain types of milk albumin, which causes the release of antibodies against them.

The effect on the body’s own tissues is explained by cross-reactions – mainly, the proteins of the pulmonary vessels are affected, the effect on the erythrocyte membranes and intestinal epithelium cells is supposed. The influence of genetic factors on the development of Heiner syndrome is discussed, but there is no clear indication of the hereditary nature of the pathological condition.

There is a scientific discussion whether to consider the disease a simple variant of enteropathic allergy or a full-fledged autoimmune state of childhood. Supporters of the allergic version indicate the presence of the main provoking factor – cattle milk, with the exclusion of which the manifestations immediately diminish and almost disappear.

On the other hand, the severity and activity of lesions of its own tissues is uncharacteristic for simple allergies. The lack of data on the preservation of milk intolerance in older age makes it impossible to accurately prove the validity of one or another point of view. Therefore, state studies are being continued in order to more accurately determine its causes.

Pathogenesis of Heiner syndrome

Like etiology, the pathogenesis of Heiner syndrome is studied very poorly, many of its manifestations are explained by assumptions and theoretical ideas. It is believed that when cow’s milk enters the gastrointestinal tract, its proteins are recognized by the child’s immune system as foreign, highly immunogenic agents. The resulting reaction is accompanied by the release of antibodies against these proteins, causing their binding and subsequent elimination.

Since when milk enters the body, it is initially located in the digestive tract organs, the first manifestations of the pathology affect them – the stool changes, pains and motility disorders appear. Absorption of other nutrients, in particular – iron-containing substances, is impaired, which exerts its influence on the subsequent stages of the pathogenesis of the syndrome.

At a certain stage in the development of pathology, a cross-reaction occurs – antibodies against milk proteins begin to attach to certain cells of the lungs and other organs, marking them as foreign and, thereby, stimulating cellular and humoral immunological reactions. Lung tissue infiltration develops with damage to the blood vessels of the pulmonary circulation, which is clinically manifested by symptoms of bronchopneumonia. There is a loss of red blood cells due to their exit from the vessels and absorption by macrophages with the formation of siderophages.

This process, combined with insufficient iron supply from the gastrointestinal tract, leads to hypochromic anemia, which sometimes takes on a threatening character. In severe cases, hepatosplenomegaly occurs. With continued delivery of milk, a combination of pulmonary, gastrointestinal and hematological disorders causes a slowdown in the child’s physical development.

Symptoms of Heiner syndrome

Manifestations of pathology occur at an early age, starting from the third month of life, their development is preceded by the introduction into the diet of the child whole cow milk or mixtures containing it. Initially, non-specific gastrointestinal disturbances can be detected – colic, flatulence, diarrhea, occasionally vomiting. Then there are signs of lung lesions – cough, wheezing, difficulty breathing. An increase in body temperature observed on this background, hemoptysis creates a picture similar to pneumonia or another form of pulmonary infection, which often leads to erroneous diagnosis and treatment.

Lungs in the lungs, abdominal pain, diarrhea and other manifestations increase with continued use of products based on milk protein. There is no response to antibiotics and other anti-pneumonia and gastrointestinal infections. This is one of the important diagnostic features of Heiner syndrome.

Some patients have skin manifestations – rash, urticaria, itching. A common symptom is a disease of the upper respiratory tract – nasal congestion and ear pain due to otitis are recorded. The nature of feces changes as the pathology progresses – from a simple liquid it can become bloody, including first thin streaks of blood, and then an increasing number of it.

Loss of hemoglobin due to hemoptysis and excretion of blood is combined with a violation of iron absorption in the gastrointestinal tract, which leads to severe anemia, which has a hypochromic nature. This, in turn, provokes an increase in the size of the liver and spleen (hepatosplenomegaly). Oxygen starvation of tissues, especially of the brain, which develops due to disruption of the respiratory system and a decrease in the concentration of hemoglobin, leads to a slower development of the child. With long-term consumption of milk and its products, this can lead to vegetative disturbances, problems with concentration of attention and learning.

Heiner syndrome complications

In the acute period of Heiner syndrome, pulmonary disorders are particularly dangerous, which can be complicated by a secondary bacterial infection with the development of life-threatening pneumonia with respiratory failure. Anemia, intestinal dysfunction and the associated impairment of absorption of nutrients, vitamins and trace elements in general, reduces the body’s resistance to infectious agents.

Infiltration of the lung tissue with immunocompetent cells over a long time can turn into its replacement with scar elements (pneumosclerosis). This reduces the capacity of the lungs, complicates the flow of oxygen into the body. According to some data, in older patients, there is an increased incidence of bronchial asthma, food allergies, and diseases of the upper respiratory tract.

Diagnosis of Heiner syndrome

The definition of Heiner syndrome in modern pediatrics poses a number of difficulties. The main role is played by the rarity of pathology, which is why specialists are not always able to recognize its presence in time due to the lack of relevant experience.

Problems are also caused by non-specific manifestations of the disease, their similarity with infectious lesions of the lungs, organs of the digestive system, and anemia. These factors lead to an incorrect diagnosis, which complicates treatment and increases the risk of complications. Therefore, in the process of determining the pathology, attention should be paid to the details of the anamnesis and the clinical picture.

The diagnostic algorithm is as follows:

  • Medical history and symptom analysis. The combination of pulmonary disorders (wheezing, coughing, sputum with streaks of blood) with diarrhea, vomiting and anemic manifestations should arouse suspicion of the presence of this pathology. An additional argument in favor of the disease is the recent transfer of the child to artificial feeding, the inclusion in the diet of cow’s milk products. Frequent colds, otitis, anemia in history also confirm the presence of the syndrome.
  • X-ray examinations. Radiography of the chest organs reveals increased severity of pulmonary pattern, the presence of infiltrates in the form of cloud-like shadows.
  • Lab tests.  In general, a blood test is noted hypochromic microcytic anemia, the severity of which depends on the severity of the patient’s condition. A characteristic feature of Heiner’s syndrome is eosinophilia, especially increasing after consuming dairy products, leukopenia and a significant increase in ESR are also possible. An immunological study of serum detects the presence of antibodies to milk proteins.
  • Microscopic examination.  Microscopy of sputum confirms a significant amount of siderophages – monocytes containing brown hemosiderin grains. The presence of red blood cells, a small number of eosinophils and Charcot-Leiden crystals are also noted. Microscopic examination of feces (coprogram) detects the presence of eosinophils.
  • Immunological tests.  If cow milk intolerance is suspected, an elimination test is prescribed – exclusion of provocative products from the diet when monitoring clinical indicators. Improvement of the condition is registered already 7-10 days after cancellation, which is evidence in favor of the presence of the disease. In the case of a satisfactory condition of the patient, provocative tests related to the introduction of an allergen into the body can be used – while the indicators will worsen.

Indirect signs of the syndrome may be the lack of effect in the treatment of pneumonia with antibiotics, worsening of the patient’s condition with non-compliance of the milk-free diet. Differential diagnosis is made with bronchopneumonia, gastrointestinal toxicoinfection, hematological disorders.

The main distinguishing characteristic of the disease from the above pathologies is the absence of the pathogen in the microscopic study of fecal mucus and sputum.

Prognosis and prevention of Heiner syndrome

Among pediatricians, Heiner syndrome is considered a condition with a relatively favorable prognosis – the simple elimination of dairy products in itself ensures the success of treatment. The potential threat to the health and life of the child is the likelihood of an incorrect definition of the disease and, as a result, improper treatment. It threatens the development of side effects (for example, dysbacteriosis), which further aggravate the patient’s condition, but a further threat is the further intake of milk proteins into the body and the resulting increase in pathological processes.

There is no specific prevention of the disease; in order to reduce the risk of complications, parents are encouraged to remember when and what foods were used to feed the child. In the future, it helps to link the development of the symptom complex with certain components of the diet and more quickly determine the diagnosis.

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