hypergammaglobulinemia/huvudvärk

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Location of the gene causing hyperimmunoglobulinemia D and periodic fever syndrome differs from that for familial Mediterranean fever. International Hyper-IgD Study Group.

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The hyperimmunoglobulinemia D and periodic fever (hyper-IgD) syndrome is typified by recurrent febrile attacks with abdominal distress, joint involvement (arthralgias/arthritis), headache, skin lesions, and an elevated serum IgD level (> 100 U/ml). This familial disorder has been diagnosed in 56

Incidence and clinical features of hyperimmunoglobulinemia D and periodic fever syndrome (HIDS) and spectrum of mevalonate kinase (MVK) mutations in German children.

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Autoinflammatory diseases (AIDs) are characterized by recurrent, self-limiting systemic inflammation. Disorders include hereditary recurrent fever (HRF) syndromes such as hyperimmunoglobulinemia D and periodic fever syndrome (HIDS). To determine the incidence of HIDS and report clinical and genetic

Cutaneous manifestations and histologic findings in the hyperimmunoglobulinemia D syndrome. International Hyper IgD Study Group.

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METHODS The hyperimmunoglobulinemia D (hyper-IgD) syndrome is characterized by recurrent febrile attacks with abdominal distress, headache, and arthralgias. Physical examination reveals cervical lymphadenopathy in most cases and, sometimes, splenomegaly. Skin lesions have been observed in isolated

Cytokine activation during attacks of the hyperimmunoglobulinemia D and periodic fever syndrome.

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The hyperimmunoglobulinemia D and periodic fever (hyper-IgD) syndrome is typified by recurrent febrile attacks with abdominal distress, joint involvement (arthralgias/arthritis), headache, skin lesions, and an elevated serum IgD level (> 100 U/mL). This familial disorder has been diagnosed in 59

Hyperimmunoglobulinemia D and periodic fever syndrome. The clinical spectrum in a series of 50 patients. International Hyper-IgD Study Group.

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We studied 50 patients (28 male and 22 female) with the hyper-IgD and periodic fever syndrome. Most patients originated from Europe, namely The Netherlands (28 cases; 56%), France (10 cases, 20%), and Italy (3 cases, 6%), but 1 patient was from Japan. A hereditary component is suggested by 18

Mixed connective tissue disease.

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A study was done that involved 46 patients with high-titer serum antibody to ribonucleoprotein (RNP). Common cutaneous manifestations included swollen hands or sclerodactyly (50 percent), cutaneous lupus erythematosus (48 percent), periungual telangiectasia (46 percent), alopecia (46 percent),

The influence of age on a clinical presentation of Toxocara spp. infection in children.

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Toxocariasis is a helminthozoonosis due to the infestation of humans by roundworms, Toxocara spp. Actual informations indicate it the most common worm infection in many countries, typically connected with rural areas. The authors analyzed the documentation of 84 children with positive serology to

Unusual case of inflammatory spinal epidural mass (Castleman syndrome).

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Castleman syndrome (giant lymph node hyperplasia) is a rare, heterogeneous lymphoproliferative disorder of unknown etiology and pathogenesis. Most cases occur as mediastinal masses, although extrathoracic involvement including nodal and extranodal locations have been reported. The localized variants

[IBL-like T cell lymphoma associated with early gastric cancer: a case report].

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A 67-year-old man was admitted in October 1987 with complaints of nausea, headache, dizziness and speech disturbance. Hematological examination showed pancytopenia. Bone marrow aspiration failed with a dry tap. A month later, the second aspiration showed hypocellular marrow containing 18.2% of

Extensive anterior cranial fossa idiopathic hypertrophic pachymeningitis: a case report and review of the literature.

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Idiopathic hypertrophic cranial pachymeningitis is a rare chronic inflammatory and fibrosing process of unknown etiology. This entity is characterized by fibrosis and thickening of the dura mater and resulting in neurological syndrome. The authors report a 72 year-old woman who presented with

[Vasculo-Behçet's disease with superior sagittal sinus thrombosis--case report (author's transl)].

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The patient is a 30-year-old man who has suffered from recurrent attacks of tonsilitis, oral aphthae and scrotal ulcerations, erythema nodosum and thrombophlebitis. In April, 1980, he gradually developed headache and visual disturbance. On April 14, 1980, he was pointed out remarked bilateral choked

Visual loss from idiopathic intracranial pachymeningitis.

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We report three patients with chronic headaches and optic neuropathy due to widespread meningeal thickening shown on enhanced MRI; all had biopsy-proven intracranial pachymeningitis (fibrosclerosis of the meninges). Two patients had bilateral optic neuropathy, elevated CSF protein, and polyclonal

Mevalonate kinase deficiency and Dutch type periodic fever.

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Dutch type periodic fever (DPF) is an autosomal recessive hereditary fever syndrome. Cases have been reported worldwide, the majority from France and The Netherlands. From infancy the patients suffer fever attacks that recur every 2-8 weeks, often precipitated by immunizations, infections or

Case report of intracranial Rosai-Dorfman disease.

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Rosai-Dorfman disease (RDD)-sinus histiocytosis with massive lymphadenopathy-represents a peculiar proliferation of histiocyte-like cells in patients. The condition was described by Rosai and Dorfman in 1969, after examining 4 cases, as an idiopathic histiocytic disorder. In 1972, they studied an

[Tuberculous meningitis and acquired immunodeficiency syndrome].

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A series composed by 13 patients suffering Tuberculous Meningitis (TM) and Acquired Immunodeficiency Syndrome (AIDS) is reported. The characteristics of the disease are compared with those obtained from a control group. TM reached a frequency of 18% in the first 66 patients with AIDS and neurologic

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