Delayed anaphylaxis due to Alpha-gal allergy: A modified desensitization protocol with red meat in an adult patient.

Alpha-gal allergy is the sensitization to Alpha-gal present in saliva when a tick bites and the development of an IgE-mediated reaction to Alpha-gal also present in red meat by cross-reactivity. In contrast to other food allergies, symptoms occur as late as 2-6 hours after a meal. Prick to prick testing with nonmammalian meat in combination with cooked mammalian meat is recommended for diagnosis. However, the main diagnostic test is Alpha-gal sIgE> 0.1 IU/mL. The primary recommendation in patients with Alpha-gal syndrome is to prevent new tick bites and avoid all mammalian meats. Since most of the dishes in our country's food culture contain red meat, elimination diet may adversely affect patients quality of life. In the management of these patients, the option of desensitization with red meat can be considered by evaluating the benefit-risk ratio together with the patient. Our patient with a history of tick bites and a reaction pattern ranging from urticaria to anaphylaxis two hours after meat consumption was evaluated for Alpha gal allergy. The patient was found to be positive by prick-to-prick with cooked red meat. In addition, the high level of Alpha-gal specific IgE (27.3 Ku/L) confirmed the Alpha-gal allergy, and the decision to apply desensitization with red meat was taken. There are only two literatures on this subject, one of which includes two adult cases and the other a single pediatric case. Since a reaction developed in the fifth step of the 27-step desensitization scheme (Ünal et al.), which we took as a reference, which led to a dose increase of more than 100 times, we modified the protocol by using an intermediate steps. We repeated the prick-to-prick test with red meat after desensitization in our case who successfully completed the modified desensitization protocol. Observation of more than half reduction in test edema diameter concretely supports the success of our modified desensitization protocol.

The coexistence of two rare diseases thought to use the same pathologic pathway: cystic fibrosis and Niemann-Pick disease.

BACKGROUND: Cystic fibrosis (CF) is a multisystemic, autosomal recessive disease, which is caused by a mutation in the transmembrane conduction regulator protein (CFTR) gene. We present a patient who was diagnosed with CF and later diagnosed with Niemann-Pick type-A (NPA) disease, which is an autosomal recessive lysosomal lipid storage disease. CASE: A 2-month-old Syrian refugee patient was diagnosed with CF due to a high sweat test and two homozygous CFTR-related pathogenic gene mutations in our pediatric pulmonology clinic, where she was referred due to a high immunoreactive trypsinogen (IRT) value as a result of newborn screening. As the patient had neurological symptoms and hepatosplenomegaly that could not be explained by CF in the clinical follow-up, the patient was diagnosed with NPA was made with a cherry red spot on eye examination, foam cells in the bone marrow, and low sphingomyelinase activity, in addition to CF. CONCLUSIONS: Although CF and NP have common systems of involvement in both diseases, pathological symptoms have different origins. If a patient with CF has simultaneous neuromotor delay, other autosomal recessive diseases that may accompany it should be suspected. In studies, similar pathological pathways related to abnormal cholesterol accumulation in the cell were detected between NP type C and CF. But our case was NPA. As case reports on the coexistence of the two diseases increase, we believe that a better understanding of similar pathological pathways may lead to new therapeutic targets for both diseases.