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Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado

2010, Number 2

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Rev Esp Med Quir 2010; 15 (2)

Wegener’s granulomatosis in children: a review about a case

Vega RZ, Hernández MJ, Huerta RJF, Salamanca GM

Language: Spanish
References: 14
Page: 87-91
PDF size: 283.07 Kb.


ABSTRACT

The Wegener’s granulomatosis is a systemic vasculitis of small vessels, characterized by inflammation and necrosis with formation of granulomas. The etiology is unknown, probably multifactorial. Endothelial damage is determined by an autoimmune process. The difference with others vasculitis is that Wegener’s granulomatosis affects predominantly lung and kidney vessels. It occurs at all ages, although it is rare in the pediatric group. The mortality rate is 80% after 2 years of the clinical onset; and many patients do not survive more than 5 years. We communicate the case of a 10 year-old male patient with a clinical history of lipotimias associated with effort at 5, 7 and 9 years old, and progressive dyspnea of medium efforts. He came to medical consult because of a clinical course of 15 days of evolution with hyaline rhinorrhea, cough initially dry and then productive, sneezing, fever, odynophagia and abdominal pain. Patient was treated with antipyretic and antibiotics. Due to the persisting fever, malaise, insomnia, asthenia, adynamia, hyporexia and cough accesses, he was hospitalized; at the forth day of hospitalization, he suffered a cough access, with desaturation and respiratory arrest that did not respond to cardiopulmonary resuscitation.


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