Results 131 to 140 of about 2,267,031 (171)

A patient with hyper-IgD syndrome responding to anti-TNF treatment

Clinical Rheumatology, 2006
The 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, Rezan Topaloğlu, Erkan Demirkaya   +2 more
exaly   +5 more sources

Diagnostic challenge of hyper-IgD syndrome in four children with inflammatory gastrointestinal complaints

Scandinavian Journal of Gastroenterology, 2006
OBJECTIVE: Hyper-IgD syndrome (HIDS) is a rare autosomal recessive disease characterized by recurrent fever, lymphadenopathy, diarrhoea, abdominal pain, headache, arthralgia and skin rash. Abdominal symptomatology may mimic inflammatory bowel disease. We
Stefano Martelossi, Federico Marchetti, Alessandro Ventura   +2 more
exaly   +3 more sources

Das Hyper-IgD-Syndrom (The hyper-IgD-syndrome)

Zeitschrift f�r Rheumatologie, 1997
We report on a 6-year-old Romanian girl with recently diagnosed hyper-IgD-syndrome. The leading symptom of this rare disease are periodic pyrexia, joint involvements (arthralgias/arthritis) and swollen lymph nodes. A permanent increase of alpha 1-acid glycoprotein fucosylation indicates persisting inflammation.
J. Suschke, J.-U. Walther
openaire   +2 more sources

Crescentic glomerulonephritis in hyper IgD syndrome

Pediatric Nephrology, 1999
The hyperimmunoglobulinemia D syndrome (HIDS) is a well-defined entity resembling familial Mediterranean fever. HIDS is a systemic inflammatory disease associated with stimulation of T-cell-mediated immunity. These patients are at low risk for amyloidosis and are not known to develop nephropathy.
M, Tsimaratos, I, Koné-Paut, L, Daniel, M C, Gubler, B, Dussol, G, Picon   +5 more
openaire   +2 more sources

Effect of inflammatory attacks in the classical type hyper-IgD syndrome on immunoglobulin D, cholesterol and parameters of the acute phase response [PDF]

Journal of Internal Medicine, 2004
Background. Classical type hyper-immunoglobulin D (IgD) syndrome (HTDS) is an hereditary autoinflammatory disorder, characterized by recurrent episodes of fever, lymphadenopathy, abdominal distress and a high serum concentration of IgD.
Alberto Mantovani, Johan Bijzet, Anna Simon   +2 more
exaly   +3 more sources

Treatment of adult hyper-IgD syndrome with canakinumab

The Journal of Allergy and Clinical Immunology: In Practice, 2015
Hyper-IgD and periodic fever syndrome (HIDS) is a rare, autosomal-recessive autoinflammatory disease characterized by lifelong recurrent episodes of systemic inflammation. HIDS frequently presents in infancy with intermittent fevers lasting from 4 to 7 days, recurring every 4 to 6 weeks.
Casey D, Curtis, Charity C, Fox
openaire   +2 more sources

Hyper-IgD syndrome with novel mutation in a Japanese girl

Modern Rheumatology, 2008
Hyperimmunoglobulin 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, Yasuo, Nakagishi, Masaaki, Mori, Takako, Miyamae, Tomoyuki, Imagawa, Shumpei, Yokota   +5 more
openaire   +2 more sources

A patient with hyper-IgD syndrome responding to simvastatin treatment

European Journal of Internal Medicine, 2008
The 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, S, Guez, I, Ranaivo, N, Jameerbaccus, C, Series   +4 more
openaire   +2 more sources

A case series of three patients with hyper IgD syndrome

Journal of Allergy and Clinical Immunology, 2004
Abstract 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
openaire   +1 more source

Overlap of Familial Mediterranean Fever and Hyper-IgD Syndrome in an Arabic Kindred

Journal of Clinical Immunology, 2015
Hyperimmunoglobulinemia 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, Buthaina, Aladbe, Rowaida Z, Taha, Elaine F, Remmers, Hatem, El-Shanti, Basil M, Fathalla   +5 more
openaire   +2 more sources