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1.
Ital J Pediatr ; 49(1): 6, 2023 Jan 13.
Artículo en Inglés | MEDLINE | ID: mdl-36639823

RESUMEN

BACKGROUND: Pediatric Mastocytosis is a rare and heterogeneous disease, characterized by accumulation of mast cells in the skin (Cutaneous Mastocytosis) and/or, less frequently, in other organs, mainly liver, spleen, bone marrow, lymph nodes and gastrointestinal tract (Systemic Mastocytosis). Patients affected by Systemic Mastocytosis show symptoms caused by  a massive release of mast cell mediators: itching, flushing, abdominal pain, generalized weakness, fatigue and neuropsychiatric disorders. Moreover, children with Systemic Mastocytosis are at greater risk of anaphylactic/anaphylactoid reactions, often poorly controlled by the conventional therapy with antihistamines, mast cells stabilizers and steroids. As a result, children affected by Systemic Mastocytosis have a poor quality of life and suffer the consequence of prolonged steroidal treatment. CASE PRESENTATION: A child with Systemic Mastocytosis and severe symptoms, refractory to symptomatic and steroidal therapy, has been successfully treated with Omalizumab, an anti-IgE monoclonal antibody usually employed in allergic patients with severe asthma and orticaria. The onset of clinical benefit of Omalizumab therapy was extraordinarily rapid, but proved to be strictly dependent on drug administration. The child has become completely and steadily asymptomatic. No other anaphylactic episodes have been reported. Steroid treatment could be definitively withdrawn after the second dose of Omalizumab, and all the other medications were later reduced. Twenty months after beginning, Omalizumab therapy is still ongoing with good symptomatology control; no side effects have been observed so far. CONCLUSIONS: In our experience, Omalizumab is an effective treatment for children affected by Systemic Mastocytosis not responding to conventional medical treatments. The main strengths of this therapy are its rapid and extraordinary efficacy to control the severe mast cells mediator-related symptoms, the lack of side effects and its steroid-sparing effect. However, more extensive and controlled studies in pediatric patients affected by Systemic Mastocytosis are needed to substantiate these promising findings.


Asunto(s)
Mastocitosis Sistémica , Mastocitosis , Humanos , Niño , Omalizumab/uso terapéutico , Mastocitosis Sistémica/complicaciones , Mastocitosis Sistémica/diagnóstico , Mastocitosis Sistémica/tratamiento farmacológico , Calidad de Vida , Mastocitosis/inducido químicamente , Mastocitosis/diagnóstico , Mastocitosis/tratamiento farmacológico , Antagonistas de los Receptores Histamínicos/uso terapéutico
2.
Int J Infect Dis ; 110: 338-340, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-34332085

RESUMEN

During the coronavirus disease 2019 (COVID-19) pandemic, patients with humoral immunodeficiency are at higher risk of developing chronic infection and having a negative outcome. Few data are available on therapeutic options for this population. This case report discusses the treatment of disease relapse with remdesivir and monoclonal antibodies in an adult patient with X-linked agammaglobulinaemia.


Asunto(s)
Tratamiento Farmacológico de COVID-19 , SARS-CoV-2 , Adenosina Monofosfato/análogos & derivados , Adulto , Agammaglobulinemia , Alanina/análogos & derivados , Anticuerpos Neutralizantes , Anticuerpos Antivirales , Enfermedades Genéticas Ligadas al Cromosoma X , Humanos , Recurrencia
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