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Biomedical subjects

I Bonté

Publications and source records attributed to I Bonté.

4 recordsLinked to original sources

Peripheral gangrene in adult-onset Kawasaki disease.

Kawasaki disease (KD) is an acute vasculitis characterized by marked tropism of the coronary vessels. Usually a childhood disease, KD can occasionally be observed in adults. We report a case of adult-onset KD that presented as a prolonged fever of unknown origin with subsequent development of severe vasculitis, manifested by coronary aneurysms and peripheral gangrene of the lower limbs. Therapy with intravenous immunoglobulins, corticosteroids, aspirin, anticoagulants, and prostacyclin analogue resulted in rapid improvement in the patient's condition but he required partial distal amputation for irreversible gangrene. Peripheral ischaemia leading to gangrene is a very rare feature of KD vasculitis with only 19 cases previously published in the Medline-indexed literature. The outcomes of those patients were poor, being either fatal or requiring distal amputation. All prior cases affected young children less than 1 year old and, to the best of our knowledge, the present case is the first description of peripheral gangrene in adult-onset KD.

Adult↗

[Treatment of systemic mastocytosis].

BACKGROUND: Systemic mastocytosis is a rare disease, characterized by mast cells proliferation in various organs. Two types of clinical manifestations can be distinguished: those related to mast cells mediators release and those related to tumoral proliferation involving different organs, these later defining aggressive systemic mastocytosis. Until recently, treatment was mainly symptomatic, without anti tumoral effect. RECENT FACTS: These last years, advances have been made in the understanding of the disease with the discovery of the c-kit oncogene mutation and the approach of the disease as a myeloproliferative disorder. PERSPECTIVES: Based on experiences acquired in the treatment of this kind of disorders, evaluation of new therapeutics, such as cladribine or combination of interferon-alpha and cytarabine is in progress. At least, tyrosine kinase inhibitors, a new family of molecules, are able of inhibiting some types of the mutated c-kit protein and one of them, imatinib mesylate, has shown a great efficacy in the treatment of gastro intestinal stromal tumors (GIST) which also involves the c-kit mutation. By analogy, treatment of patients with c-kit susceptible mutation might be treated with this molecule.

Adrenal Cortex Hormones↗