Síndrome del ocho y medio como manifestación de enfermedad desmielinizante aguda por adenovirus / Eight-and-a-half syndrome as manifestation of acute disseminated adenovirus encephalomyelitis

La encefalomielitis diseminada aguda es una enfermedad inflamatoria-desmielinizante inmunomediada que suele manifestarse tras una infección o vacunación en niños en edad escolar. Típicamente presenta una fase prodrómica con un cuadro pseudogripal seguida de una fase con clínica muy variada, pudiendo aparecer alteraciones neurooftalmológicas como oftalmoplejía o neuritis óptica.La etiología es variada, incluyendo enfermedades tumorales, vasculares, infecciosas, inflamatorias y desmielinizantes. El diagnóstico se basa en la historia clínica y en las características de la resonancia magnética cerebral, prueba de imagen de elección. El estudio del líquido cefalorraquídeo puede servir de ayuda en la orientación del cuadro clínico.El pronóstico es favorable, con excelente respuesta a los corticoides e inmunoglobulinas y con mínimas secuelas a largo plazo en la mayoría de los casos.Presentamos el caso de un varón de 8años con enfermedad desmielinizante aguda por adenovirus cuya manifestación fue un síndrome del ocho y medio. (AU)

Acute disseminated encephalomyelitis is an immune mediated inflammatory-demyelinizing disease that usually manifests after infection or vaccination in school-age children. It typically presents a prodromal phase with flu-like symptoms, followed by a phase with varied clinical symptoms, neuro-ophthalmological alterations such as ophthalmoplegia or optic neuritis may occur.The differential diagnosis includes tumor, vascular, infectious, inflammatory and demyelinating diseases. Diagnosis is based on the clinical history and the characteristics of brain magnetic resonance imaging, the gold standard test. The study of the cerebrospinal fluid can help to guide the clinical picture.The prognosis is favorable, with an excellent response to corticosteroids and immunoglobulins, with minimal long-term sequelae in most cases.We report the case of an 8-year-old male with acute demyelinating disease due to adenovirus whose manifestation was an eight-and-a-half syndrome. (AU)

Eight-and-a-half syndrome as manifestation of acute disseminated adenovirus encephalomyelitis.

Acute disseminated encephalomyelitis is an immune mediated inflammatory-demyelinizing disease that usually manifests after infection or vaccination in school-age children. It typically presents a prodromal phase with flu-like symptoms, followed by a phase with varied clinical symptoms, neuro-ophthalmological alterations such as ophthalmoplegia or optic neuritis may occur. The differential diagnosis includes tumor, vascular, infectious, inflammatory and demyelinating diseases. Diagnosis is based on the clinical history and the characteristics of brain magnetic resonance imaging, the gold standard test. The study of the cerebrospinal fluid can help to guide the clinical picture. The prognosis is favorable, with an excellent response to corticosteroids and immunoglobulins, with minimal long-term sequelae in most cases. We report the case of an 8-year-old male with acute demyelinating disease due to adenovirus whose manifestation was an eight-and-a-half syndrome.

Vogt-Koyanagi-Harada: treatment of recurrence after administration of 3 intravenous bolus of 1 g of corticosteroids and mycophenolate mofetil.

The case is presented on a young Honduran female with no medical history of note, who presented with multiple areas of exudative retinal detachment (RD), and a best-corrected visual acuity of 1.3 logMAR in both eyes. She was diagnosed with incomplete Vogt-Koyanagi-Harada syndrome, and treated early with a combination of intravenous therapy with 1 g of prednisolone per day for 3 days, as recommended by published evidence, as well as mycophenolate mofetil (2 g per day). During the corticosteroids tapering, there was a recurrence of exudative retinal detachments, and megadoses of 1 g of intravenous corticosteroids per day were reintroduced for 6 days until the complete resolution of the fluid of the exudative RD, and cyclosporine (100 mg per day), subtenon triamcinolone (40 mg/mL), and intravitreal ranibizumab once a month in the both eyes were added to the treatment, with a great control of choroidal inflammation that resulted in the remission of symptoms and signs.

Vogt-Koyanagi-Harada: tratamiento de la recurrencia tras la administración de 3 bolos intravenosos de 1g de corticoides y micofenolato de mofetilo / Vogt-Koyanagi-Harada: treatment of recurrence after administration of 3 intravenous bolus of 1g of corticosteroids and mycophenolate mofetil

Presentamos el caso de una mujer hondureña de 27 años sin antecedentes médicos de interés que presentó múltiples áreas de desprendimientos de retina (DR) exudativos y una mejor agudeza visual corregida de 1,3logMAR en ambos ojos. Fue diagnosticada de síndrome de Vogt-Koyanagi-Harada incompleto y tratada con prednisolona intravenosa (1g/24h) durante 3 días, tal y como recomienda la evidencia publicada, junto con micofenolato de mofetilo (2g/24h). Durante el descenso paulatino de corticoesteroides, los DR recidivaron, por lo que se reintrodujeron las megadosis de 1g/24h de corticoesteroides intravenosos durante 6 días hasta la resolución completa de los DR y se añadieron ciclosporina (100mg/24h), triamcinolona subtenoniana (40mg/mL) e inyecciones intravítreas mensuales de ranibizumab en ambos ojos, con un adecuado control de la inflamación coroidea que se tradujo en la remisión de los síntomas y signos (AU)

The case is presented on a young Honduran female with no medical history of note, who presented with multiple areas of exudative retinal detachment (RD), and a best-corrected visual acuity of 1.3logMAR in both eyes. She was diagnosed with incomplete Vogt-Koyanagi-Harada syndrome, and treated early with a combination of intravenous therapy with 1g of prednisolone per day for 3 days, as recommended by published evidence, as well as mycophenolate mofetil (2g per day). During the corticosteroids tapering, there was a recurrence of exudative retinal detachments, and megadoses of 1g of intravenous corticosteroids per day were reintroduced for 6 days until the complete resolution of the fluid of the exudative RD, and cyclosporine (100mg per day), subtenon triamcinolone (40mg/mL), and intravitreal ranibizumab once a month in the both eyes were added to the treatment, with a great control of choroidal inflammation that resulted in the remission of symptoms and signs (AU)

Vogt-Koyanagi-Harada: treatment of recurrence after administration of 3 intravenous bolus of 1g of corticosteroids and mycophenolate mofetil. / Vogt-Koyanagi-Harada: tratamiento de la recurrencia tras la administración de 3 bolos intravenosos de 1g de corticoides y micofenolato de mofetilo.

The case is presented on a young Honduran female with no medical history of note, who presented with multiple areas of exudative retinal detachment (RD), and a best-corrected visual acuity of 1.3logMAR in both eyes. She was diagnosed with incomplete Vogt-Koyanagi-Harada syndrome, and treated early with a combination of intravenous therapy with 1g of prednisolone per day for 3 days, as recommended by published evidence, as well as mycophenolate mofetil (2g per day). During the corticosteroids tapering, there was a recurrence of exudative retinal detachments, and megadoses of 1g of intravenous corticosteroids per day were reintroduced for 6 days until the complete resolution of the fluid of the exudative RD, and cyclosporine (100mg per day), subtenon triamcinolone (40mg/mL), and intravitreal ranibizumab once a month in the both eyes were added to the treatment, with a great control of choroidal inflammation that resulted in the remission of symptoms and signs.

Arsenite alters heme synthesis in long-term cultures of adult rat hepatocytes.

Arsenite (As[III]) effects on the intermediate steps of heme biosynthesis were studied in adult rat hepatocytes seeded on a feeder layer of 3T3 cells (3T3-hepatocytes) and maintained for 2 weeks with culture medium non-supplemented or supplemented with 150 microM 5-aminolevulinic acid (ALA). The activities of the intracellular enzymes porphobilinogen deaminase (PBG-D), uroporphyrinogen III synthase (UROIII-S), and uroporphyrinogen III decarboxylase (URO-D), and the intermediary uroporphyrins (URO), coproporphyrins (COPRO) and protoporphyrin IX (PROTO) were determined in these cultures. The 3T3-hepatocytes maintained the activities of PBG-D, UROIII-S and URO-D during 2 weeks and ALA addition to the culture medium increased PBG-D (2-3-fold) and UROIII-S (50%) activities and porphyrin production, which accumulated as PROTO. Exposure to 3.9 microM As(III) inhibited UROIII-S activity (down to 34%), and PBG-D and URO-D activities to a lower extent; these effects were magnified by ALA supplementation. As(III) also produced an intracellular accumulation and a decreased excretion of PROTO, and a 31% reduction of the COPRO/URO ratio in the culture medium. Additionally, As(III) caused cytoplasmic vacuolization and lipid accumulation. Our results show that As(III) exposure selectively inhibits several intermediary enzymes of heme metabolism and affects the intra- and extracellular content of porphyrins and their ratio in the culture medium. They also confirm that 3T3-hepatocytes are a suitable in vitro model to study hepatic heme metabolism and its alterations by hepatotoxic chemicals.