RESUMEN
We describe 4 cases of lysinuric protein intolerance, which all fulfilled the diagnostic criteria for hemophagocytic lymphohistiocytosis. Mature histiocytes and neutrophil precursors participated in hemophagocytosis in the bone marrow. Moreover, serum levels of ferritin and lactate dehydrogenase were elevated, hypercytokinemia was present, and soluble interleukin-2 receptor levels were increased up to 18.6-fold. The diagnosis of lysinuric protein intolerance should therefore be considered in any patient presenting with hemophagocytic lymphohistiocytosis.
Asunto(s)
Errores Innatos del Metabolismo de los Aminoácidos/complicaciones , Errores Innatos del Metabolismo de los Aminoácidos/diagnóstico , Histiocitosis de Células no Langerhans/complicaciones , Histiocitosis de Células no Langerhans/diagnóstico , Errores Innatos del Metabolismo de los Aminoácidos/inmunología , Arginina/metabolismo , Células de la Médula Ósea , Niño , Preescolar , Diagnóstico Diferencial , Citometría de Flujo , Histiocitosis de Células no Langerhans/inmunología , Humanos , Lactante , Subgrupos Linfocitarios , Lisina/metabolismo , Ornitina/metabolismoRESUMEN
We report a pediatric case of a mucosa-associated lymphoid tissue lymphoma of the labial minor salivary gland in an immunocompetent patient. Chronic gastritis with Helicobacter pylori was disclosed and managed with antibiotic therapy. With eradication of Helicobacter pylori there was complete remission of the lymphoma.
Asunto(s)
Gastritis/complicaciones , Infecciones por Helicobacter/complicaciones , Helicobacter pylori , Linfoma de Células B de la Zona Marginal/complicaciones , Neoplasias de las Glándulas Salivales/complicaciones , Niño , Gastritis/tratamiento farmacológico , Gastritis/microbiología , Infecciones por Helicobacter/tratamiento farmacológico , Humanos , Inmunocompetencia , Labio , Linfoma de Células B de la Zona Marginal/inmunología , Masculino , Inducción de Remisión , Neoplasias de las Glándulas Salivales/inmunologíaRESUMEN
We retrospectively analyzed the frequency and nature of infections occurring in 48 patients with chronic granulomatous disease. The long-term use of trimethoprim-sulfamethoxazole and ketoconazole as a preventive therapy for infections has also been evaluated. Lymphadenitis, lung infections, dermatitis, enteral infections, and hepatic abscesses were the most frequent infections. Staphylococcus aureus, Salmonella, and Aspergillus were the main microorganisms encountered. Twelve patients died: five from lung aspergillosis, three from hepatic abscesses, two from pneumonopathy of unknown origin, one from salmonellosis, and one from another probable infection that could not be proved. The actuarial survival rate was 50% at 10 years of age, with a prolonged plateau thereafter. There was no difference in survival rates between patients with X-linked and those with autosomal recessive chronic granulomatous disease. The 8-year actuarial survival rate was significantly higher for patients born in 1978 or afterward than for patients born before 1978 (92.9% vs 70.5%). A retrospective analysis of the occurrence of bacterial and fungal infections in patients who received trimethoprim-sulfamethoxazole and ketoconazole as infection prophylaxis indicated that the former was effective against bacterial infections but that ketoconazole provided no protection against Aspergillus infections.