J Postgrad Med
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Muckle-Wells syndrome (MWS) is a rare autosomal dominant disease that belongs to a group of hereditary periodic fever syndromes. It is part of the wider spectrum of the cryopyrin-associated periodic syndrome (CAPS) which has only rarely been described in non-Caucasian individuals. It is characterized by recurrent self-limiting episodes of fever, urticaria, arthralgia, myalgia and conjunctivitis from childhood. ⋯ We report two patients with MWS in an Indian family associated with the p. D303N mutation in the NLRP3 gene. These findings promote awareness of these hereditary periodic fever syndromes as a cause for recurrent fevers from childhood in the Indian population.
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Editorial Comment
Improving peri-operative patient care: the surgical safety checklist.
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Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyper inflammatory condition, if not recognized and treated in time. A high index of suspicion can help identify the condition early. ⋯ In this article, we share our experience with this entity and make an attempt to explore literature about ravenous macrophages which occurs secondary to infections. It is a series of six cases of HLH secondary to infectious disease in our center in a coastal city in South India over last one year with follow up.