Results 111 to 120 of about 2,216 (148)
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Hyper-IgD syndrome or mevalonate kinase deficiency
Current Opinion in Rheumatology, 2011The hyper-IgD and periodic fever syndrome (HIDS) is one of the classical monogenetic hereditary autoinflammatory disorders, and together with the more severe mevalonic aciduria it is also known as 'mevalonate kinase deficiency' (MKD). In this study, we will give an overview of the primary research on mevalonate kinase deficiency published in the past 2
Stoffels, M., Simon, A.
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Hyper-IgD syndrome with novel mutation in a Japanese girl
Modern Rheumatology, 2008Hyperimmunoglobulin D and periodic fever syndrome (HIDS) is an autosomal recessive auto-inflammatory disorder characterized by recurrent febrile attacks with lymphadenopathy, abdominal distress, skin eruptions and joint involvement. We discuss the case of a 15-year-old Japanese girl who had presented with periodic fever, hepatosplenomegaly and ...
Takuya, Naruto +5 more
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A patient with hyper-IgD syndrome responding to anti-TNF treatment
Clinical Rheumatology, 2006The hyperimmunoglobulinemia D periodic fever syndrome (HIDS) is caused by recessive mutations in the mevalonate kinase gene, which encodes an enzyme involved in cholesterol and nonsterol isoprenoid biosynthesis. The pathogenesis and treatment remains unclear. We describe a 6-year-old Turkish girl with severe disease.
Hans R Waterham +2 more
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A patient with hyper-IgD syndrome responding to simvastatin treatment
European Journal of Internal Medicine, 2008The hyper-IgD syndrome (HIDS) is an autosomal recessively inherited auto-inflammatory syndrome, caused by deficient enzyme activity of mevalonate kinase (MVK), an enzyme in the isoprenoid pathway. Patients present with a long history of recurrent fever attacks, accompanied by abdominal pain, skin lesions, lymphadenopathy and arthralgia.
H, Attout +4 more
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A case series of three patients with hyper IgD syndrome
Journal of Allergy and Clinical Immunology, 2004Abstract Rationale This case series brings to light three newly diagnosed cases of Hyper IgD Syndrome (HIDS), a rare inherited periodic fever with only 193 reported cases world-wide through May 2003. Methods Three patients were diagnosed with HIDS and their clinical and laboratory data are presented.
Y. Padeh, A. Rubinstein
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Overlap of Familial Mediterranean Fever and Hyper-IgD Syndrome in an Arabic Kindred
Journal of Clinical Immunology, 2015Hyperimmunoglobulinemia D Syndrome (HIDS) has rarely been reported in Arabs. Moreover, the simultaneous presence of mutations in MEFV and MVK segregating in the same family is exceptional. We report an Arabic girl presenting since the age of 8-years with two patterns of recurrent episodes of fever, and associated with a spectrum of clinical features ...
Taha, Moussa +5 more
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“Hyper-IgD syndrome” or “mevalonate kinase deficiency”: an old syndrome needing a new name?
Rheumatology International, 2013We suggest that the use of HIDs name is obsolete, while MKD definition could suit better to the description of the disease.
CELSI, Fulvio +2 more
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Recenti progressi in medicina, 1995
In 1984, Van der Meer first reported six patients with a long history of recurrent attacks of fever of unknown cause and a constantly elevated polyclonal IgD (> 100 U/mL); he suggested the acronym of "hyper-IgD syndrome" (HIDS). A recent literature review identified 60 cases (59 from Europe and 1 from Japan).
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In 1984, Van der Meer first reported six patients with a long history of recurrent attacks of fever of unknown cause and a constantly elevated polyclonal IgD (> 100 U/mL); he suggested the acronym of "hyper-IgD syndrome" (HIDS). A recent literature review identified 60 cases (59 from Europe and 1 from Japan).
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[Periodic fever due to hyper-IgD syndrome].
Nederlands tijdschrift voor geneeskunde, 2000In a 45-year-old man who from early childhood had been suffering of periodic fever, which did not respond to any therapy attempted, the ultimate diagnosis was hyperimmunoglobulinaemia D syndrome (HIDS). HIDS attacks typically occur every 4-6 weeks and last 3-7 days.
I M, Wauters +2 more
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