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Histopathology of the human liver in yellow fever with special emphasis on the diagnostic role of the Councilman body.

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Liver specimens from 10 cases of yellow fever (YF) were studied by light and four by electron microscopy to review morphological aspects of the disease relevant to its diagnosis, with special emphasis on acidophilic bodies (AB) and on the possible presence of the virus within infected cells. The AB

Review of Cerliponase Alfa: Recombinant Human Enzyme Replacement Therapy for Late-Infantile Neuronal Ceroid Lipofuscinosis Type 2.

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The objective of this review is to summarize the pharmacology, efficacy, and safety of cerliponase alfa for the treatment of late infantile neuronal ceroid lipofuscinosis type 2 (CLN2). Cerliponase alfa is recombinant human tripeptidyl peptidase 1 enzyme replacement therapy. A phase 1/2 trial

[An unusual cause of pulmonary fibrosis in a 71-year-old patient].

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METHODS A 71-year-old woman, suffering from spinal stenosis presented interstitial lung disease, albinism, and severe loss of visual acuity. On physical examination she showed tic-like automatism predominantly periorbital in the face and in the fingers. A computed tomography of the chest revealed

Chronic granulomatous disease of childhood and sea-blue histiocytosis. A pathologic study of an autopsy case.

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A boy, who started remittent fever reaching 40 degrees C at the age of 4 years and 9 months and gradually developed hepatosplenomegaly, lymphadenopathy, pancytopenia, and hypergammaglobulinemia, is reported. Aspirated bone marrows contained sea-blue histiocytes, and nitroblue tetrazolium test was

[Mucocutaneous lymph node syndrome in Austria. Four cases with one fatal outcome. 2. Pathological findings].

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First pathoanatomic case report of mucocutaneous lymph-node syndrome (MCLS) from Austria: A 3 1/2 year old boy was admitted to the Pediatric Department with a six day history of fever and bilateral conjunctivitis. He showed a maculous exanthema, red and fissuring lips, reddened tonsils and a coated

Study of Intraventricular Cerliponase Alfa for CLN2 Disease.

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Recombinant human tripeptidyl peptidase 1 (cerliponase alfa) is an enzyme-replacement therapy that has been developed to treat neuronal ceroid lipofuscinosis type 2 (CLN2) disease, a rare lysosomal disorder that causes progressive dementia in children. In a multicenter, open-label study, we

Considering Valproate as a Risk Factor for Rapid Exacerbation of Complex Movement Disorder in Progressed Stages of Late-Infantile CLN2 Disease.

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Neuronal ceroid lipofuscinosis type 2 (CLN2 disease, OMIM 204500) is a rare autosomal-recessive lysosomal storage disorder. It is one of the most common neurodegenerative disorders in childhood. Symptoms include epilepsy, rapid motor and language regression, dementia, visual loss, and a complex

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