RESUMO
Mucopolysaccharidosis type II (MPS II) is an X-linked recessive disorder characterized by a deficiency of the enzyme iduronate-2-sulfatase leading to a multisystem involvement by tissue accumulation of glycosaminoglycans heparan and dermatan sulfate. We report a case of a 9-year-old boy diagnosed with mucopolysaccharidosis type II at 4 years of age, classified as severe variant. He presented necrotizing pneumonia associated with influenza A H1N1 infection, requiring mechanical ventilation for 1 month and 10 days. This case emphasizes the importance of healthcare workers to be aware of potentially lethal complications in patients with MPS II, such as influenza A H1N1 infection, which in turn may be associated with necrotizing pneumonia.
Assuntos
Vírus da Influenza A Subtipo H1N1 , Influenza Humana/complicações , Mucopolissacaridose II/complicações , Pneumonia Necrosante/virologia , Criança , Humanos , MasculinoRESUMO
La mucopolisacaridosis tipo II (MPS II) es un desorden recesivo ligado al cromosoma X, caracterizado por una deficiencia de la enzima iduronato-2-sulfatasa, que lleva a una afectación multisistémica por acumulación tisular de los glicosaminoglicanos heparan y dermatan sulfato. Reportamos el caso de un niño de 9 años diagnosticado con MPS II a los 4 años de edad, catalogado como portador de una variante grave. Presentó neumonía necrotizante asociada a infección A H1N1, que requirió ventilación mecánica por 1 mes y 10 días. Este caso enfatiza la importancia de que los trabajadores de la salud estén alerta a las potenciales complicaciones en pacientes con MPS II, tales como la infección por influenza A H1N1, la que, a su vez, puede estar asociada con neumonía necrotizante.
Mucopolysaccharidosis type II (MPS II) is an X-linked recessive disorder characterized by a deficiency of the enzyme iduronate-2-sulfatase leading to a multisystem involvement by tissue accumulation of glycosaminoglycans heparan and dermatan sulfate. We report a case of a 9-year-old boy diagnosed with mucopolysaccharidosis type II at 4 years of age, classified as severe variant. He presented necrotizing pneumonia associated with influenza A H1N1 infection, requiring mechanical ventilation for 1 month and 10 days. This case emphasizes the importance of healthcare workers to be aware of potentially lethal complications in patients with MPS II, such as influenza A H1N1 infection, which in turn may be associated with necrotizing pneumonia.
Assuntos
Masculino , Criança , Mucopolissacaridose II , Vírus da Influenza A Subtipo H1N1 , Pneumonia NecrosanteRESUMO
La mucopolisacaridosis tipo II (MPS II) es un desorden recesivo ligado al cromosoma X, caracterizado por una deficiencia de la enzima iduronato-2-sulfatasa, que lleva a una afectación multisistémica por acumulación tisular de los glicosaminoglicanos heparan y dermatan sulfato. Reportamos el caso de un niño de 9 años diagnosticado con MPS II a los 4 años de edad, catalogado como portador de una variante grave. Presentó neumonía necrotizante asociada a infección A H1N1, que requirió ventilación mecánica por 1 mes y 10 días. Este caso enfatiza la importancia de que los trabajadores de la salud estén alerta a las potenciales complicaciones en pacientes con MPS II, tales como la infección por influenza A H1N1, la que, a su vez, puede estar asociada con neumonía necrotizante.(AU)
Mucopolysaccharidosis type II (MPS II) is an X-linked recessive disorder characterized by a deficiency of the enzyme iduronate-2-sulfatase leading to a multisystem involvement by tissue accumulation of glycosaminoglycans heparan and dermatan sulfate. We report a case of a 9-year-old boy diagnosed with mucopolysaccharidosis type II at 4 years of age, classified as severe variant. He presented necrotizing pneumonia associated with influenza A H1N1 infection, requiring mechanical ventilation for 1 month and 10 days. This case emphasizes the importance of healthcare workers to be aware of potentially lethal complications in patients with MPS II, such as influenza A H1N1 infection, which in turn may be associated with necrotizing pneumonia.(AU)
RESUMO
La mucopolisacaridosis tipo II (MPS II) es un desorden recesivo ligado al cromosoma X, caracterizado por una deficiencia de la enzima iduronato-2-sulfatasa, que lleva a una afectación multisistémica por acumulación tisular de los glicosaminoglicanos heparan y dermatan sulfato. Reportamos el caso de un niño de 9 años diagnosticado con MPS II a los 4 años de edad, catalogado como portador de una variante grave. Presentó neumonía necrotizante asociada a infección A H1N1, que requirió ventilación mecánica por 1 mes y 10 días. Este caso enfatiza la importancia de que los trabajadores de la salud estén alerta a las potenciales complicaciones en pacientes con MPS II, tales como la infección por influenza A H1N1, la que, a su vez, puede estar asociada con neumonía necrotizante.(AU)
Mucopolysaccharidosis type II (MPS II) is an X-linked recessive disorder characterized by a deficiency of the enzyme iduronate-2-sulfatase leading to a multisystem involvement by tissue accumulation of glycosaminoglycans heparan and dermatan sulfate. We report a case of a 9-year-old boy diagnosed with mucopolysaccharidosis type II at 4 years of age, classified as severe variant. He presented necrotizing pneumonia associated with influenza A H1N1 infection, requiring mechanical ventilation for 1 month and 10 days. This case emphasizes the importance of healthcare workers to be aware of potentially lethal complications in patients with MPS II, such as influenza A H1N1 infection, which in turn may be associated with necrotizing pneumonia.(AU)
RESUMO
Mucopolysaccharidosis type II (MPS II) is an X-linked recessive disorder characterized by a deficiency of the enzyme iduronate-2-sulfatase leading to a multisystem involvement by tissue accumulation of glycosaminoglycans heparan and dermatan sulfate. We report a case of a 9-year-old boy diagnosed with mucopolysaccharidosis type II at 4 years of age, classified as severe variant. He presented necrotizing pneumonia associated with influenza A H1N1 infection, requiring mechanical ventilation for 1 month and 10 days. This case emphasizes the importance of healthcare workers to be aware of potentially lethal complications in patients with MPS II, such as influenza A H1N1 infection, which in turn may be associated with necrotizing pneumonia.