propranolol in children with Kostmann syndrome: causes, symptoms, treatment.

Agranulocytosis in children with Kostmann's syndrome manifests itself already in the neonatal period by recurrent purulent infections, such as: recurrent skin lesions with the formation of abscesses and inflammatory infiltrates; ulcerative stomatitis, infectious periodontitis and periodontitis, gingival hypertrophy and bleeding; pathology of ENT organs (otitis media, mastoiditis, inflammation of the mucous membrane of the nasopharynx); protracted pneumonia, prone to abscess formation. Characterized by increased body temperature, generalized enlargement of the lymph nodes, often splenomegaly (enlargement of the spleen). In severe cases, septicemia develops with the formation of abscesses in the liver.

The leukocyte formula in Kostmann's syndrome undergoes specific changes. In typical cases, extreme neutropenia is characteristic (neutrophils may not be detected at all), eosinophilia and monocytosis with a normal number of lymphocytes. Until recently, the prognosis for Kostmann's syndrome was very unfavorable, especially during the neonatal period (with age, the manifestations of agranulocytosis become less pronounced), but recently treatment with granulocyte colony-stimulating factor (G-CSF) has shown encouraging results. This drug, originally isolated from the plasma of mice, stimulates the proliferation of stem cells of the granulocyte series, increases the rate of their maturation and, according to some reports, improves the quality of maturing neutrophils.

Buy propranolol online in children with cyclic neutropenia. Cyclic neutropenia is a hereditary disease transmitted in an autosomal dominant manner (a child is born sick only when at least one of the parents suffers from this disease). This pathology is characterized by rhythmic fluctuations in the level of neutrophils in the blood - from a normal amount to deep agranulocytosis (may not be detected in peripheral blood).

The mechanism of the development of pathology has not yet been clarified, it is assumed that a genetically determined defect in precursor cells of neutrophils is. Cyclically appearing agranulocytosis is characterized by attacks of recurrent fever with ulcerative lesions of the oral cavity and pharynx, accompanied by an increase in regional lymph nodes and severe symptoms of propranolol.